J Pediatr Surg

BAPS prize lecture: New insight into mechanisms of parenteral nutrition-associated cholestasis: role of plant sterols. British Association of Paediatric Surgeons.

Iyer KR. Spitz L. Clayton P.
No information.
BACKGROUND PURPOSE: Infants on long-term parenteral nutrition frequently will have progressive cholestatic liver disease, the cause of which remains largely unknown. The aim of this study is to establish a possible role for plant sterols (phytosterols) in the pathogenesis of parenteral nutrition-associated cholestasis (PNAC). METHODS: Two experimental studies were used: (1) A study on neonatal piglets involved the daily injection of plant sterols; measurement of their concentrations in serum, liver, and bile during a 14-day period; and determination of serum bile acid concentrations, bile acid secretion, and bile flow at the end of the 14-day period. (2) Isolated rat hepatocyte couplets were used to study the effects of sterols on canalicular secretion. RESULTS: The daily injection of phytosterols (in amounts similar to those given to infants who receive parenteral nutrition) led to their progressive accumulation in the piglets' serum, liver, and bile. Serum bile acid levels were significantly higher in the sterol-treated piglets. Maximal bile acid excretion was significantly lower in the sterol group. Phytosterols caused a significant inhibition of secretory function in isolated rat hepatocyte couplets. CONCLUSION: Important contaminants of commercial lipid emulsions (phytosterols) have been identified as a possible cause of PNAC.

Infant pediatric liver transplantation results equal those for older pediatric patients.

Van der Werf WJ. D'Alessandro AM. Knechtle SJ. Pilli G. Hoffmann RM. Judd RH. Odorico JS. Kirk AD. Rayhill SC. Sollinger HW. Kalayoglu M.
Department of Surgery, University of Wisconsin School of Medicine, Madison 53792-7375, USA.
METHODS: From July 1984 to July 1995, 99 pediatric patients underwent 127 orthotopic liver transplants (OLT) at the University of Wisconsin Children's Hospital. The patients were divided into four groups according to age at time of transplant: group I, 0 to 6 months (n = 20); group II, 6 to 12 months (n = 18); group III, 1 to 2 years (n = 10); and group IV, 2 to 18 years (n = 51). A retrospective analysis was performed to compare these four groups with regard to preoperative indications and demographics, intraoperative technique, complications, and survival. All patients were followed up for 2 to 13 years. RESULTS: Biliary atresia was the most common indication for OLT in all four groups. The average waiting period varied from 19+/-18 days for group I to 44+/-64 days for group IV. Reduced-size liver transplant (I, 41%; II, 52%; III, 28%; IV, 21%), split-liver transplant (I, 0%; II, 7.4%; III, 17%; IV, 2.9%), or whole-liver transplant techniques were used. Although postoperative Intensive Care Unit stay was longer for the 0- to 6-month-old patients (I, 20+/-64; II, 7.6+/-9; III, 13+/-17; IV, 6.8+/-14 days), the total hospital stay (I, 43+/-63; II, 33+/-34; III, 32+/-20; IV, 29+/-31 days) was similar for all patients. The incidence of hepatic artery thrombosis (I, 19%; II, 19%; III, 27%; IV, 16%), biliary tract complications (I, 4.8%; II, 15%; III, 20%; IV, 14%), and retransplantation (I, 9.5%; II, 41%; III, 33%; IV, 14%) were not significantly different between the four groups. Portal vein thrombosis (I, 9.5%; II, 11%; III, 6.6; IV, 0%) and primary nonfunction (I, 9.5%; II, 7.4%; III, 0%; IV, 3.1%) occurred more frequently in the 0- to 6-month and 6- to 12-month groups, however, the 1-, 5-, and 10-year survival rate for patients (I, 85%, 79%, 79%; II, 89%, 74%, 74%; III, 80%, 80%, 80%; IV, 84%, 75%, 75%, respectively) and primary liver allografts (I, 69%, 69%, 69%; II, 72%, 72%, 63%; III, 70%, 70%, 70%; IV, 71%, 57%, 57%, respectively) were not significantly different (P = .98 and P = .83). CONCLUSION: These results demonstrate that OLT can be effectively performed on infants of all ages and that OLT should not be delayed because of age.

Experience with papillary and solid epithelial neoplasms of the pancreas in children.

Ky A. Shilyansky J. Gerstle J. Taylor G. Filler RM. Grace N. Superina R.
Department of Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
BACKGROUND: Papillary cystic tumour of the pancreas in children is a rare tumour. METHODS: Ten patients admitted to The Hospital for Sick Children, Toronto, have been reviewed, and presentation and management are documented. RESULTS: One patient who had disseminated disease at presentation died. CONCLUSION: Excision of the tumour irrespective of size is recommended.

Stenting for caustic strictures: esophageal replacement replaced.

De Peppo F. Zaccara A. Dall'Oglio L. Federici di Abriola G. Ponticelli A. Marchetti P. Lucchetti MC. Rivosecchi M.
Department of Pediatric Surgery, Bambino Gesu Children's Hospital, Rome, Italy.
METHODS: From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesu Children's Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption. RESULTS: No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03). CONCLUSION: Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

The failure rate of surgery for gastro-oesophageal reflux.

Kimber C. Kiely EM. Spitz L.
Department of Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, London, England.
PURPOSE: The aim of this study was to document the presenting symptomatology and radiological findings of failed fundoplication, to determine the risk factors involved in recurrent gastro-oesophageal reflux, and to assess the results of revisional surgery. METHODS: Sixty-six patients requiring redo fundoplication during the past 15 years were studied. Sixty children had their initial fundoplication performed at our institution. RESULTS: The median time from initial fundoplication to the diagnosis of failure was 1.5 years. The main presenting symptoms were severe retching (n = 33), recurrent vomiting and aspiration (n = 26) and intolerable gas bloat (n = 7). Severe dysphagia occurred in two children with tight Nissen fundoplications. Contrast radiographic studies demonstrated fundoplication failure and correlated with the operative findings in 85% of cases. Risk factors for failure comprised hypertonic cerebral palsy, severe learning difficulties, oesophageal atresia, CHARGE syndrome, and tracheomalacia. The cause for the fundoplication failure included herniation of the fundoplication into the posterior mediastinum (failed crural repair, n = 30), disruption of the wrap (n = 10), combination of herniation and disruption (n = 22), and a tight wrap (n = 4). After revisional surgery, 13 children had persisting symptoms, five of whom underwent a third antireflux procedure. CONCLUSIONS: The predominant cause of fundoplication failure is herniation into the posterior mediastinum, which occurred most frequently in children with hypertonic cerebral palsy. This may be prevented by meticulous attention to accurate apposition of the crura at initial procedure. Redo fundoplication failed to resolve the preoperative symptoms in 20%, with five children requiring a third fundoplication.

Effect of prenatal glucocorticoids on pulmonary vascular muscularisation in nitrofen-induced congenital diaphragmatic hernia.

Okoye BO. Losty PD. Lloyd DA. Gosney JR.
Department of Child Health and Pathology, University of Liverpool, England.
BACKGROUND/PURPOSE: Pulmonary hypertension (PH) contributes significantly to the mortality of congenital diaphragmatic hernia (CDH). Pulmonary vascular changes in CDH include a reduced vascular bed with increased arterial medial wall thickness and peripheral extension of muscle into intraacinar vessels. Antenatal steroids improve biochemical immaturity, lung compliance, and morphology in experimental CDH animals. The aim of this study was to examine the effects of prenatal glucocorticoid therapy on pulmonary artery muscularisation in CDH rats. METHODS: CDH was induced in fetal rats by the maternal administration of 100 mg of nitrofen by gavage on day 9.5 of gestation (term, day 22). Control animals received olive oil (OO). Dexamethasone (Dex, 0.25 mg/kg) or normal saline (NS) was given by intraperitoneal injection on days 18.5 and 19.5, and fetuses were delivered by caesarean section on day 21.5. Lung sections from five fetuses in each of four experimental groups were studied by a blinded investigator- OO-NS controls, CDH-NS, CDH-Dex, and non-CDH-NS. The external diameter (ED), medial wall thickness (MT), percent of medial wall thickness, and wall structure were evaluated from preacinar arteries accompanying conducting airways, and the intraacinar arterioles associated with the respiratory bronchi and saccules. RESULTS: In the preacinar arteries, CDH-NS animals had a significantly increased MT percentage compared with OO-NS controls (21.2+/-8.8 v 17.8+/-10.3, P = .0001). CDH-Dex rats had a lower MT percentage than CDH-NS rats (15.5+/-6.7 v 21.2+/-8.8, P = .0001). In the intraacinar region, CDH-Dex fetuses had a reduced percentage of muscularised intraacinar blood vessels compared with CDH-NS and OO-NS controls (10% v 24% and 28%, respectively, P = .01). Dexamethasone-treated CDH pups also displayed a significantly lower MT percentage of the intraacinar arteries compared with CDH-NS and OO-NS animals (15.7+/-13 v 23.4+/-9 and 25.4+/-12, P = .003). CONCLUSIONS: Medial hypertrophy is present in the preacinar but not the intraacinar blood vessels of CDH rats before birth. Dexamethasone inhibits medial hypertrophy and reduces the number of muscularised intraacinar vessels. Antenatal glucocorticoids may reduce the risk of PH developing in human newborns with antenatally diagnosed CDH.

Pulmonary vascular balance in congenital diaphragmatic hernia: enhanced endothelin-1 gene expression as a possible cause of pulmonary vasoconstriction.

Okazaki T. Sharma HS. McCune SK. Tibboel D.
Department of Pediatric Surgery, Sophia Children's Hospital, Rotterdam, The Netherlands.
BACKGROUND: Pulmonary hypoplasia and persistent pulmonary hypertension (PPH) are the principal causes of the ongoing mortality in congenital diaphragmatic hernia (CDH) presenting with respiratory insufficiency within 6 hours after birth. Endothelin-1 (ET-1) is an endothelial-derived vasoconstrictor, which could play an important role in modulating pulmonary vascular tone in PPH. ET-1 exerts its role in controlling vascular tone through two different subtype receptors, endothelin-A receptor (ETA) which is responsible for vasoconstriction and endothelin-B receptor (ETB) which is responsible for vasodilatation by induction of nitric oxide synthase. METHODS: We examined the pulmonary expression of ET-1, ETA and ETB mRNAs in a rat model of CDH. CDH was induced in rats by administration of 100 mg of nitrofen dissolved in olive oil on day 10 of gestation. Fetal lungs were collected after cesarean section on gestational day 22 (term) and processed for Northern blot analysis and quantitative polymerase chain reaction (PCR). RESULTS: Significantly (P

Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudoobstruction. Report of two cases.

Kenny SE. Vanderwinden JM. Rintala RJ. Connell MG. Lloyd DA. Vanderhaegen JJ. De Laet MH.
Department of Pediatric Surgery, Hopital Universitaire des Enfants Reine Fabiola, Universite Libre de Bruxelles, Brussels, Belgium.
The case histories of two neonates presenting with intestinal pseudoobstruction are presented. One boy infant was premature and the girl infant was full term. Both patients needed a defunctioning ileostomy, and biopsy findings of the intestine in both patients showed a lack of interstitial cells of Cajal (ICC). At the time of closure of the ileostomies to restore intestinal continuity, repeat biopsy results showed a normal pattern of distribution of ICC. Delay in the development of ICC in the gastrointestinal tract may be a cause of intestinal pseudoobstruction in the newborn.

GDNF deficit in Hirschsprungs disease.

Martucciello G. Thompson H. Mazzola C. Morando A. Bertagnon M. Negri F. Brizzolara A. Rocchetti L. Gambini C. Jasonni V.
Department and Chair of Pediatric Surgery, Giannina Gaslini Children's Hospital, University of Genoa, Italy.
BACKGROUND/PURPOSE: In 1996, the glial cell line-derived neurotrophic factor (GDNF) was identified as one of the ligands of the RET transmembrane receptor. In the same year, GDNF mutations were found in association with RET protooncogene mutations in Hirschsprung patients. Mutations in GDNF per se are thought neither necessary nor sufficient to cause Hirschsprung's disease (HD). To date, our study group has identified GDNF mutations only in 2 of 98 cases of intestinal dysganglionosis. The aim of our study was to investigate a possible expression deficit of GDNF in the enteric nervous system of Hirschsprung patients not mutated for the GDNF gene. METHODS: We used rabbit polyclonal antibodies raised against a peptide corresponding to amino acids 186-205 mapping within the carboxy-terminal domain of human GDNF. GDNF expression was studied immunohistochemically in surgical specimens from 30 HD cases (27 classic forms and 3 ultralong forms) and from 10 age-matched controls. Serial sections from the same full-thickness specimens were investigated with the following histochemical and immunohistochemical techniques: acetylcholinesterase, lactate dehydrogenase, succinic dehydrogenase, alpha-naphthyl-esterase, glial fibrillary acid protein, S-100 protein, and neuron-specific enolase. RESULTS: A high level of GDNF expression was found in normal intestine and in Hirschsprung ganglionic segment. Satellite elements of myenteric ganglia presented a strong immunoreactivity to GDNF. Conversely, the aganglionic segment showed cholinergic hyperinnervation and hypertrophic trunks of nerve fibers in the muscular interstitium with complete absence of GDNF expression. The small ganglia of the hypoganglionic segment showed a reduced GDNF immunoreactivity. CONCLUSIONS: GDNF, a distantly related member of the transforming growth factor-beta superfamily, is a potent neurotrophic and survival factor for neurons and enteric ganglion cells. Mutations of the GDNF gene or GDNF expression deficit interrupt the faithful GDNF signaling via Ret, contributing to HD pathogenesis.

Turnover of radioactive mucin precursors in the colon of patients with Hirschsprungs disease correlates with the development of enterocolitis.

Aslam A. Spicer RD. Corfield AP.
Department of Paediatric Surgery, Bristol Royal Hospital for Sick Children, England.
BACKGROUND/PURPOSE: Mucin glycoproteins (mucins) in the colonic mucus gel layer interact with pathogens performing protective functions by a variety of mechanisms. It is recognised that patients with Hirschsprung's disease (HD) are prone to episodes of enterocolitis even after corrective surgery, the aetiology of which is poorly understood. The authors correlated the turnover of radioactive mucin precursors in organ culture of the proximal ganglionated colon at the time of pull-through with the development of postoperative enterocolitis. METHODS: The colonic mucins in the retained proximal ganglionated colon of nine HD patients at the time of pull-through were studied. Organ culture of intact mucosa was performed with radioactive mucin precursors 35S-sulphate and 3H-glucosamine. Mucins in the secretions and epithelial cells were then purified by gel filtration. Turnover of the isotopes was determined by relating radioactivity to tissue DNA content. These patients were followed up prospectively for a mean duration of 30.8 months. The patients were assigned to one of two groups according to the criteria of requiring hospital admission for enterocolitis during this period. There were five patients in the group that remained well after corrective surgery and four in the group that developed entercolitis. The turnover values of both radioisotopes were analysed for differences in the two groups of patients. RESULTS: Patients in the enterocolitis group had a median value for turnover of 331 dpm/microg DNA, and the group that was well had a median value of 2044 dpm/microg DNA. These differences were statistically significant (Mann-Whitney, P = .037). CONCLUSIONS: A reduced turnover of mucins as shown by incorporation of radioactive precursors will give rise to a defective colonic mucus-defensive barrier. It can be inferred that the lower the turnover, the more prone a patient is to postoperative enterocolitis. It is therefore possible that organ culture with radioactive mucin precursors of the proximal ganglionated mucosa performed at the time of pull-through has a predictive value in the development of postoperative enterocolitis.

Prognostic value of serum procollagen III peptide and type IV collagen in patients with biliary atresia.

Kobayashi H. Miyano T. Horikoshi K. Tokita A.
Department of Paediatric Surgery, Juntendo University School of Medicine, Tokyo, Japan.
BACKGROUND/PURPOSE: Progressive hepatic fibrosis, in spite of a successful Kasai procedure, is a major problem in patients with biliary atresia (BA). Early identification of patients at risk would be of great value. N-terminal procollagen-III peptide (PIIIP) (which is a marker of fibrogenesis and, therefore, of on going inflammation), and type IV collagen (found in basement membrane extracellular matrix), were measured in patients with BA to determine their potential as prognostic markers. METHODS: Thirty-three postoperative BA patients (11.0+/-3.7 years old) and 20 normal controls (10.5+/-2.8 years old) were studied. The BA patients were classified on the basis of their current liver function test results into three outcome groups. Group I (n = 9) had severe liver dysfunction, group II (n = 13) had moderate, and group III (n = 11) had good liver function. Serum P-III-P and type IV collagen values were determined by radioimmunoassays and one step sandwich enzyme immunoassay. RESULTS: In group I, serum PIIIP (1.93+/-0.64 U/mL) and type IV collagen levels (363.5+/-69.5 ng/mL) were significantly higher than in group II (PIIIP [1.32+/-0.25 U/mL], type IV collagen [225.3+/-45.4 ng/mL]; P < .01). There were increased levels in serum PIIIP and serum type IV collagen in group II compared with group III (PIIIP [1.01+/-0.25 U/mL], type IV collagen [171.3+/-47.2 ng/mL]; P < .01). There were no significant differences in serum PIIIP and type IV collagen levels between group III and controls. CONCLUSION: The authors conclude that serum levels of PIIIP and type IV collagen may be useful in the long-term follow-up of BA patients after Kasai's portoenterostomy.

Management of biliary atresia in the liver transplantation era: a 15-year, single-center experience.

Maksoud JG. Fauza DO. Silva MM. Porta G. Miura I. Zerbini CN.
Division of Surgery, Children's Institute, University of Sao Paulo Medical School, Brazil.
BACKGROUND/PURPOSE: The aim of this study was to address the perioperative aspects of hepatoportoenterostomy (HPE) for biliary atresia (BA), through the study of a 15-year, single-center experience of the management of this disease. METHODS: One hundred twenty-seven patients were divided into three groups, depending on the variant of HPE performed: group A (n = 53) underwent HPE with external diversion of the Roux-en-Y anastomosis; group B (n = 54) underwent HPE with a long (35 to 40 cm) Roux-en-Y anastomosis, without diversion; and group C (n = 20) underwent the same kind of HPE as group B, but with a modified, "super extensive" dissection of the porta hepatitis. Eleven children in group B had an intussusception type antireflux valve in the Roux-en-Y loop. The porta hepatitis of 105 children was histologically classified in types I to III and correlated with rate of postoperative bile flow and age at surgery. Liver transplantation was performed after HPE in 20 patients. RESULTS: Overall, biliary drainage was achieved in 72.5% of the children after HPE and 26.8% of all patients became jaundice free. Porta hepatitis type III was associated with a significantly higher rate of biliary drainage post-HPE then types I and II. There was no difference in the rate of bile drainage, relative number of jaundice-free patients, and mean number of episodes of cholangitis per year among surgical groups A, B, C. In group A, 43.7% of the patients had complications related to the stoma. The actuarial survival of children who underwent HPE followed by liver transplantation was 85%. CONCLUSIONS: (1) There is no correlation between type of porta hepatis and age at surgery for BA; (2) type III porta hepatis is associated with higher rates of bile drainage post-HPE; (3) children older than 16 weeks with BA should still be considered for HPE; (4) in these older patients, factors other than the type of porta hepatis, possibly the degree of liver damage, play a role in the lower rate of bile drainage observed; (5) external diversion of the Roux-en-Y in HPE is not beneficial and is detrimental because of stoma-related complications; (6) an antireflux valve in the Roux-en-Y loop does not reduce the incidence of cholangitis post-HPE; (7) Surgical reexploration does not restore biliary drainage; and (8) sequential therapy with HPE followed by liver transplantation remains the treatment of choice for BA.

Amebic liver abscess: spare the knife but save the child.

Moazam F. Nazir Z.
Division of Pediatric Surgery, Aga Khan University, Karachi, Pakistan.
BACKGROUND/PURPOSE: Amebic liver abscess (ALA), the most common extraintestinal manifestation of infection with Entameba histolytica, carries significant morbidity and mortality in the pediatric age group. The efficacy of metronidazole in the treatment of ALA is well established, but the role of surgical intervention remains controversial. Many investigators still advocate aggressive surgical therapy for complicated and ruptured ALA. Reports regarding management of ALA in children are sparse and deal with small numbers of patients. The objective of this study was to assess the effectiveness of parenteral metronidazole combined with judicious aspiration of ALA in obviating the need for surgical intervention. METHODS: The medical records of all children admitted with the diagnosis of ALA between 1986 and 1997 to the Aga Khan University Hospital were reviewed retrospectively. The diagnosis of ALA was confirmed in 48 patients by an ultrasound scan together with elevated indirect hemagglutination (IHA) titres (>250). Ages ranged from 3 weeks to 14.5 years. RESULTS: Thirty-seven (75%) children were below the fifth percentile for height and weight, and 45 (93.7%) presented with a hemoglobin level of less than 10.0 g/dL. Comorbid factors included pulmonary tuberculosis (n = 2, chicken pox (n = 1), tetralogy of Fallot (n = 1), and thalassemia major (n = 2). There was a mean delay of 13 days before presentation to the hospital. All patients were treated with parenteral metronidazole and broad-spectrum antibiotics. The latter were discontinued on confirmation of the diagnosis. The duration of treatment with metronidazole ranged from 2 to 5 weeks. Percutaneous aspiration of the ALA was performed under ultrasound guidance using sedation in 28 patients for one or more of the following indications; ALA greater than 7.0 cms (n = 20), left lobe involvement (n = 8), and no response after 48 hours of medical therapy (n = 6). Nine patients required more than one aspiration. One patient with peritoneal rupture of the ALA additionally underwent percutaneous aspiration of the peritoneal cavity under ultrasound guidance. One patient required insertion of a chest tube after rupture of the ALA into the right pleural cavity, and another underwent urgent bronchoscopy after rupture of the abscess into the tracheobronchial tree. The hepatobronchial fistula closed spontaneously with medical therapy. No patient required open surgical drainage, and all recovered without relapse. The mean duration of hospitalization was 12 days. CONCLUSION: Our experience suggests that parenteral metronidazole combined with timely aspiration of the abscess can obviate the need for surgical intervention in large and complicated ALA even in malnourished children who present late for treatment.

Abnormal colonic interstitial cells of Cajal in children with anorectal malformations.

Kenny SE. Connell MG. Rintala RJ. Vaillant C. Edgar DH. Lloyd DA.
Department of Paediatric Surgery, Alder Hey Children's Hospital, Liverpool, England.
BACKGROUND/PURPOSE: Constipation is a frequent functional problem in children after operation for all types of anorectal malformations. Although this has been assumed to be caused by hypomotility of the rectosigmoid colon, recent studies have demonstrated generalized colonic hypomotility in children with high or intermediate anomalies. The cause of this disorder is unknown. The aim of this study was to determine whether the observed colonic hypomotility seen in patients with anorectal malformations was caused by defects in distribution or density of interstitial cells of Cajal (ICC), recently identified as 'intestinal pacemaker cells'. METHODS: Colostomy specimens from 12 patients with high anorectal anomalies (ARM group; age 0 to 14 months) were compared with colostomy specimens from five control patients with nonmotility-related gastrointestinal pathology (age, 1 to 4 months). Specimens were immunohistochemically labelled with antibodies to PGP9.5, a marker for neural tissue, and antibodies to c-kit, a recently characterized marker for interstitial cells of Cajal (ICC). RESULTS: Ganglion cells were present in all histological specimens. Abnormalities in distribution and density of c-kit-positive ICC were present in 7 of 12 ARM patients. In two ARM patients, ICC were completely absent, and in five patients, ICC density was markedly reduced in circular muscle and at the submucosal border of circular muscle. Only five ARM patients had a distribution of ICC similar to that of control patients. CONCLUSION: Defects in the population of intestinal pacemaker cells may underlie the colonic hypomotility seen in high anorectal malformations and hence may contribute to refractory constipation.

Bowel management for fecal incontinence in patients with anorectal malformations.

Pena A. Guardino K. Tovilla JM. Levitt MA. Rodriguez G. Torres R.
Department of Surgery, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.
BACKGROUND/PURPOSE: Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. METHODS: Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. RESULTS: Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. CONCLUSION: Bowel management consisting of enemas, laxatives, and medications is successful when administered in an organized manner. It is vital to determine the type of fecal incontinence from which the patients suffer and to target their treatment accordingly.

How to achieve a successful Malone antegrade continence enema.

Curry JI. Osborne A. Malone PS.
Department of Paediatric Urology, Southampton General Hospital, England.
BACKGROUND/PURPOSE: The Malone antegrade continence enema (MACE) has proved invaluable in the management of children with faecal incontinence. The authors have reviewed their experience in depth to try and ascertain why some MACEs fail. METHODS: The records of 31 children who had a MACE procedure between 1990 and 1995 were reviewed. Their preoperative bowel management, the operative procedure, the postoperative washout regime, and the outcome were assessed. Success was graded either as full, described as totally clean or minor rectal leakage on the night of the washout; partial, described as clean but significant stomal or rectal leakage, occasional major leaks, still wearing protection, and perceived improvement by the parents or child; or failure, described as regular soiling or constipation, no perceived improvement, and the procedure abandoned. There were 18 boys and 13 girls in the series. The mean age at survey was 8.4 years. RESULTS: Overall success rate was 19 of 31 (61%) with a mean follow-up of 3.25 years. The diagnosis and success rates were: anorectal anomaly 11 (success, 8 of 11, 73%), neuropathic bowel 11 (8 of 11, 73%), chronic constipation 8 (3 of 8, 38%).

Anorectal malformations and Downs syndrome.

Year 1998
Torres R. Levitt MA. Tovilla JM. Rodriguez G. Pena A.
Department of Surgery Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.
BACKGROUND/PURPOSE: Down's syndrome is a common association in patients with anorectal malformations. The purpose of this study was to determine whether the anorectal defect in patients with Down's syndrome had specific characteristics and whether the presence of Down's syndrome represented a serious detriment to the patient's functional prognosis. METHODS: Nine hundred eighty-seven patients with anorectal malformations were studied retrospectively. Twenty patients (2%) had Down's syndrome. Nineteen of these (95%) had the same specific type of anorectal defect: imperforate anus with no fistula. This defect has a good prognosis, the rectum is located about 2 cm above the perineal skin, the sacrum is normal, and the sphincter mechanism is good. For comparison, a group of 34 patients with the same defect but without Down's syndrome was also studied. All patients were operated on via posterior sagittal approach by the same surgeon. RESULTS: Imperforate anus without fistula occurs in 5% of all patients with anorectal malformations and in 95% of those patients who also suffer from Down's syndrome. The characteristics of the defect were the same in both groups of patients, and surprisingly, the prognosis was good in both groups (80% to 96% of patients had voluntary bowel movement, 100% had urinary continence). CONCLUSIONS: The association of Down's syndrome with imperforate anus without fistula is not coincidental. This particular benign defect can be predicted to occur in most patients with Down's syndrome. The presence of Down's syndrome in cases of anorectal malformations should not be a contraindication to repairing the imperforate anus and to closing the colostomy.

Immediate and long-term results of surgical management of low imperforate anus in girls.

Year 1998
Javid PJ. Barnhart DC. Hirschl RB. Coran AG. Harmon CM.
C.S. Mott Children's Hospital, University of Michigan, Ann Arbor 48109-0245, USA.
BACKGROUND: The majority of girls with imperforate anus are reported to have a malformation of the low variety. Despite this, much of the literature has focused on the more complex, high lesions. METHODS: This study reviews our experience with 44 girls with low imperforate anus from a 22-year period. RESULTS: The incidence of associated anomalies was 61%, which is higher than generally reported. All patients in the study had anal fistulae. Fifty-seven percent had perineal fistulae, 23% had fourchette fistulae, and 20% had vestibular fistulae. Cutback anoplasty was performed in 55%, Potts transfer anoplasty was used in 27%, and 18% of patients were treated with either limited posterior sagittal anorectoplasty or anterior sagittal anorectoplasty. Surgical complications were uncommon. Long-term follow-up was carried out by telephone survey. This showed 89% of the girls to be successfully toilet trained. However, 47% of patients experience at least occasional soilage or episodic fecal incontinence. CONCLUSIONS: Low imperforate anus can be successfully treated using a variety of procedures without colostomy. Most girls with low imperforate anus are successfully toilet trained, but problems with continence persist in a significant number of these patients.

The Malone (antegrade colonic enema) procedure: early experience.

Year 1998
Wilcox DT. Kiely EM.
Great Ormond Street Children's Hospital NHS Trust, London, England.
PURPOSE:The aim of this study was to assess the results of the Malone (antegrade colonic enema) procedure for fecal incontinence. METHODS: By a retrospective review of patients treated between 1990 and 1996 in a tertiary referral center, 36 patients were treated with a Malone procedure. Age at operation was 8.3 (range, 3 to 14) years, the mean period of follow-up was 39 (range, 9 to 72) months. The indication was fecal soiling in 35 and chronic constipation in one. The underlying diagnosis was an anorectal anomaly in the majority of patients. The appendix was used in 30 patients and a cecal flap in six, and a submucosal antireflux procedure was also performed in 10. In 35 patients, a circular stoma was created and in one a V flap was used. Antegrade colonic enemas were performed daily in 10, alternate days in 23, and in three patients the stoma was no longer used. Enemas were performed with a 10F catheter using a mixture of phosphate enema (or liquorice) and saline. RESULTS: Fecal soiling was completely controlled in 28 patients, and eight children soiled more than once a week. Complications occurred in 15 patients; the main problem was stenosis of the conduit, which occurred in 9 of 30 appendiceal stomas and three of six cecal stomas. Stomal stenosis was treated with surgical revision in eight patients. Additional complications were reflux through the stoma (n=2), pain on catheterisation (n=1), and small bowel obstruction (n=1). In one patient the Malone has been converted to a colostomy. CONCLUSIONS: The Malone procedure is a simple technique that can effectively control fecal incontinence in the majority of cases. It appeared to be better in older children. Stomal stenosis is a frequently encountered problem that may require surgical revision.

Occurrence of MEN 2a in familial Hirschsprungs disease: a new indication for genetic testing of the RET proto-oncogene.

Year 1998
Decker RA. Peacock ML.
The Charlie Hays Division of Cancer Research, The Decker Foundation, St Louis, MO, USA.
PURPOSE: The association of the rare hereditary cancer syndrome, multiple endocrine neoplasia type 2a (MEN 2a) with Hirschsprung's disease, both linked to germline mutations in the RET proto-oncogene, has been reported recently. With the widespread availability of genetic screening for MEN 2a, it is necessary to define the indications for genetic testing of MEN 2a and population subgroups at high risk for inheriting the disease. The purpose of this study was to assess the prevalence of Hirschsprung's disease in MEN 2a and investigate the value of genetic analysis for MEN 2a in children with familial Hirschsprung's disease. METHODS: The ethnically diverse study group consisted of unselected consecutive patients (n=426) at risk for hereditary medullary thyroid cancer (MTC) referred to a single laboratory for genetic testing. Analysis used genomic DNA and a polymerase chain reaction-based heteroduplex mutation detection strategy for exons 10, 11, 13, and 14 of the RET proto-oncogene followed by direct DNA sequencing. Significance of RET genotype-phenotype correlation was determined by Fisher's two-tailed Exact test and a 2 x 2 contingency table. RESULTS: Thirty-six distinctly new MEN 2a kindreds were identified. Hirschsprung's disease cosegregated among siblings with MEN 2a in 15 patients from 6 of the 36 (17%) families. The extent of aganglionosis in the 15 patients ranged from midrectum to duodenum. Of the 15 patients with Hirschsprung's disease, 10 (six boys, four girls) underwent thyroidectomy for MTC (n=5) or C-cell hyperplasia (n = 5) at ages 2 to 47 years (mean, 15.6 years), and the remaining five patients died in childhood of complications related to the aganglionosis. In retrospect, Hirschsprung's disease was the presenting feature of MEN 2a in five of the six families rather than MTC or pheochromocytoma. In all six MEN 2a families expressing Hirschsprung's disease, the RET mutation predisposing to the combined phenotype occurred in exon 10 at codons 609 (n=2), 618 (n=3), or 620 (n = 1). By contrast, the MEN 2a with Hirschsprung's phenotype was not found in any of the 22 families with a RETexon 11, 13, or 14 mutation (P=.0007). CONCLUSIONS: The authors conclude that Hirschsprung's disease is a phenotypic marker for MEN 2a and possibly more common than originally appreciated. The expression of Hirschsprung's disease with MEN 2a may be uniquely linked to RETexon 10 mutations. The authors recommend that (1) patients affected with MEN 2a may be counseled regarding the potential risk of Hirschsprung's disease in offspring and (2) a family history of MTC be explored in children with familial Hirschsprung's disease and genetic screening for MEN 2a be considered.

The colon patch graft procedure for extensive aganglionosis: long-term follow-up.

Year 1998
Nishijima E. Kimura K. Tsugawa C. Muraji T.
Department of Surgery, Kobe Children's Hospital, Sumaku, Japan.
PURPOSE: During the last 17 years, the authors have used the colon patch graft (CPG) procedure to treat 11 patients with extensive aganglionosis. This study evaluates the effectiveness of the CPG procedure in treating patients with this disorder. METHODS: An initial ileostomy was created during infancy in all 11 patients. At 1 to 21 months (mean, 5.5 months) of age, an 11- to 40-cm (mean, 18 cm) side-to-side ileocolostomy (CPG) was created between the ileum and aganglionic ascending colon, (including aganglionic ileum in three patients). Ten patients later underwent a Swenson-type definitive operation, the CPG segment being brought down to the anus at 9 to 30 months (mean, 19 months) of age. These 10 patients have been followed up for growth, development, and bowel habit patterns for the subsequent 5 to 17 years (mean, 12 years). RESULTS: Within 1 month after the CPG was created, intravenous nutrition could be discontinued and the patients treated at home until time for the definitive procedure. During this period, one patient died of aspiration. After the definitive operation, body weight for age returned to a normal range in 2 to 4 years. Currently, seven older patients have one to three bowel movements per day, whereas three younger patients have explosive diarrheal defecations. None are incontinent of stool. Iron deficiency anemia developed in four patients. Two are mentally retarded, but the others are doing well in school. CONCLUSIONS: (1) The colon patch graft procedure effectively reduces "ileostomy diarrhea," shortening the period of intravenous nutrition to 1 month, (2) bowel habit patterns improve with the advance of time, (3) body weight for age returns to normal 2 to 4 years after the definitive procedure, and (4) during long term follow-up, patients must be evaluated for iron deficiency anemia.

Ileal exclusion for Bylers disease: an alternative surgical approach with promising early results for pruritus.

Year 1998
Hollands CM. Rivera-Pedrogo FJ. Gonzalez-Vallina R. Loret-de-Mola O. Nahmad M. Burnweit CA.
Division of Pediatric Surgery, Miami Children's Hospital, FL, USA.
BACKGROUND/PURPOSE: Progressive familial intrahepatic cholestasis (Byler's disease) is often characterized by pruritus-induced self-mutilation with minimal response to medical therapy. The causative cholestasis is likely to progress to cirrhosis necessitating transplantation. Partial external biliary diversion has been used with promising results for the jaundice and debilitating pruritus but all the potential complications and aesthetic concerns of long-term stomas attend this approach. METHODS: The authors describe a terminal ileal exclusion that was first developed for patients who had previously undergone cholecystectomy. Over a 3-year period, we identified for study seven children with liver histology characteristic of Byler's disease accompanying a clinical picture of chronic cholestasis without a defined metabolic or anatomic abnormality. The first two patients underwent a cholecystojejunal cutaneous stoma, until now, the recommended treatment for this condition. The third had previously undergone cholecystectomy so an ileocolonic anastomosis was performed excluding the distal 15% of the small bowel. This child had complete relief of pruritus without evidence of diarrhea. Two more terminal ileal exclusions were performed with similar results before standardizing this approach. The authors approximated small intestinal length using Siebert's graph relating crown-heel length to small intestinal length. The midpoint between the mean and one standard deviation below the mean was determined. Fifteen percent of the estimated small bowel length was measured back from the ileocecal valve and then divided using a linear stapling device. A stapled anastomosis was created between the proximal ileum and the cecum, bypassing the terminal ileum. RESULTS: Four of five children have had relief from their pruritus and self-mutilation with no evidence of diarrhea. Terminal ileal bypass offers a stoma-free, completely reversible "biliary diversion." CONCLUSION: Early results on a few patients are promising, but long-term evaluation of growth, development, and liver function and histology is needed before advocating this as the primary therapy for Byler's disease.

Gastrointestinal perforation after pediatric orthotopic liver transplantation.

Year 1998
Beierle EA. Nicolette LA. Billmire DF. Vinocur CD. Weintraub WH. Dunn SP.
Department of Pediatric Surgery, St Christopher's Hospital for Children, Temple University School of Medicine, Philadelphia, PA 19134-1095, USA.
PURPOSE: The aim of this review was to determine the incidence of gastrointestinal perforation after pediatric liver transplantation and to identify risk factors and clinical indicators that may lead to an earlier diagnosis. METHODS: A retrospective chart review of all children who presented with gastrointestinal perforation after liver transplantation at our institution between January 1, 1987 and August 1, 1996 was performed. RESULTS: One hundred fifty-seven orthotopic liver transplants were performed in 128 children. Fifty-eight reexplorations, excluding those for retransplantation, were performed in 38 children. Ten perforations occurred in six children (incidence, 6.4%). Two children required multiple reexplorations because of several episodes of perforation. The sites of perforation were duodenum (n=1), jejunum (n=8), and ileum (n=1). A single-layer closure was used to repair five perforations, two-layer closures in four, and resection with primary anastomosis in another. The type of repair did not affect the occurrence of subsequent perforations. All the children were less than 18 months old. Four children had undergone prior laparotomy. All children had choledochoenteric anastomoses, but only one had a perforation associated with it. One child sustained bowel injury during the dissection for the liver transplant, but none of the perforations occurred at this site. Bowel function had returned before perforation in five children. Five children were receiving systemic antibiotics at the time of their perforation, and none had been dosed with pulse steroids for rejection. All of the children had significant changes in their temperature. Acute leukopenia developed in one child. A leukocytosis developed in the rest of the children. Abdominal radiographs demonstrated pneumoperitoneum in only one child. All children had positive culture findings from their abdominal drains. Cytomegalovirus developed in one child. Although the diagnosis of gastrointestinal perforation after pediatric liver transplant remains difficult, positive drain culture findings and significant alterations in temperature and leukocyte counts suggest its presence. Pneumoperitoneum is rarely present. CONCLUSION: A high index of suspicion and timely laparotomy, especially in children less than 2 years of age, may be the only way to rapidly diagnose and treat this potentially devastating complication of liver transplant.

Current status of intestinal transplantation in children.

Year 1998
Reyes J. Bueno J. Kocoshis S. Green M. Abu-Elmagd K. Furukawa H. Barksdale EM. Strom S. Fung JJ. Todo S. Irish W. Starzl TE.
Thomas E. Starzl Transplantation Institute, University of Pittsburgh Medical Center, Children's Hospital of Pittsburgh, PA 15213, USA.
PURPOSE: A clinical trial of intestinal transplantation (Itx) under tacrolimus and prednisone immunosuppression was initiated in June 1990 in children with irreversible intestinal failure and who were dependent on total parenteral nutrition (TPN). METHODS: Fifty-five patients (28 girls, 27 boys) with a median age of 3.2 years (range, 0.5 to 18 years) received 58 intestinal transplants that included isolated small bowel (SB) (n = 17), liver SB (LSB) (n=33), and multivisceral (MV) (n=8) allografts. Nine patients also received bone marrow infusion, and there were 20 colonic allografts. Azathioprine, cyclophosphamide, or mycophenolate mofetil were used in different phases of the series. Indications for Itx included: gastroschisis (n=14), volvulus (n=13), necrotizing enterocolitis (n=6), intestinal atresia (n=8), chronic intestinal pseudoobstruction (n=5), Hirschsprung's disease (n=4), microvillus inclusion disease (n=3), multiple polyposis (n=1), and trauma [n=1). RESULTS: Currently, 30 patients are alive (patient survival, 55%; graft survival, 52%). Twenty-nine children with functioning grafts are living at home and off TPN, with a mean follow-up of 962 (range, 75 to 2,424) days. Immunologic complications have included liver allograft rejection (n=18), intestinal allograft rejection (n=52), posttransplant lymphoproliferative disease (n=16), cytomegalovirus (n=16) and graft-versus-host disease (n=4). A combination of associated complications included intestinal perforation (n=4), biliary leak (n=3), bile duct stenosis (n=1), intestinal leak (n=6), dehiscence with evisceration (n=4), hepatic artery thrombosis (n=3), bleeding (n=9), portal vein stenosis (n=1), intraabdominal abscess (n=11), and chylous ascites (n=4). Graft loss occurred as a result of rejection (n=8), infection (n=12), technical complications (n=8), and complications of TPN after graft removal (n=3). There were four retransplants (SB, n=1; LSB n=3). CONCLUSIONS: Intestinal transplantation is a valid therapeutic option for patients with intestinal failure suffering complications of TPN. The complex clinical and immunologic course of these patients is reflected in a higher complication rate as well as patient and graft loss than seen after heart, liver, and kidney transplantation, although better than after lung transplantation.

Injection sclerotherapy in the treatment of rectal prolapse in infants and children.

Year 1998
Chan WK. Kay SM. Laberge JM. Gallucci JG. Bensoussan AL. Yazbeck S.
Department of Pediatric Surgery, The Montreal Children's Hospital, McGill University, Quebec, Canada.
PURPOSE: The aim of this study was to examine the efficacy of injection sclerotherapy as treatment for persistent rectal prolapse. METHODS: The records of 28 patients with rectal prolapse treated with injection sclerotherapy over a 16-year period were reviewed. Initial management included assessment and correction of predisposing factors. If rectal prolapse persisted or if the prolapse required repeat emergency or operating room reduction, injection sclerotherapy was performed. The sclerosing agent included D50W in 21 patients (sole agent in 15, combined with ethanolamine oleate in four, and with phenol 5% in two). Phenol 5% alone was used in six patients, and 25% saline was used in one patient. Number of injections, recurrences, and complications were reviewed. RESULTS: Two patients were lost to follow-up, and one patient was cured once a polyp was recognized and removed. Of the remaining 25 patients, 21 were cured. Sixteen required one injection, three required two injections, and two required three injections (64% cure rate after one injection, 84% cure rate after three injections). There were 4 of 25 failures: two went on to low anterior resection after having failed two injections each; one patient was treated with Thiersch cerclage and injection after two failed injections; and one patient did not respond after three injections but had less severe prolapses. Of those injected with D50W alone, 13 of 14 were cured with injection sclerotherapy. Nine received one injection, two received two injections, and two received three injections (64% cure rate after one injection, 93% cure rate after three injections). The only complication was excessive oozing at the injection site in one patient. He was simply observed in hospital overnight. Follow-up averaged 33 months. The only significant underlying abnormality in our patient population was spina bifida in one patient. This patient was cured with injection therapy. Cystic fibrosis was ruled out by clinical examination and sweat chloride test in all patients. Constipation was the most common condition identified with the onset of rectal prolapse (15 of 28). CONCLUSIONS: Injection sclerotherapy is simple and should be considered as the first line treatment of recurrent rectal prolapse after failure of conservative measures. D50W is effective, easily available, inexpensive, and associated with few complications.

Complications of retained internal bolster after pediatric percutaneous endoscopic gastrostomy.

Year 1998
Mollitt DL. Dokler ML. Evans JS. Jeiven SD. George DE.
Department of Surgery, University of Florida Health Science Center, Jacksonville 32209, USA.
PURPOSE: Percutaneous endoscopic gastrostomy (PEG) has been widely accepted as an efficacious means of nutritional support in the infant and child. A well-described technique uses the Gauderer-Ponsky tube (CR Bard Incorporated, Tewksbury, MA) drawn antegrade through the gastric wall and secured by an internal and external SILASTIC (Dow Corning; Midland, MI) bolster. The majority of reported complications attendant to its use occur secondary to insertion. This report details a less well-described complication of tube removal. METHODS: Since 1992, 234 pediatric PEGs have been performed using a Gauderer-Ponsky tube. Approximately 6 weeks after the procedure, all catheters were removed and replaced with gastric buttons. The internal bolster was left within the stomach to pass spontaneously. RESULTS: Five children (2.1%), ages 6 months to 5 years, failed to pass this crossbar. Three subsequently presented with dysphagia and drooling with the internal bolster wedged in the proximal esophagus. All were left with significant residual stricture after endoscopic removal of the crossbar. Two required dilatation and the third underwent operative stricturoplasty. A fourth child returned with intermittent gastric outlet obstruction. The internal bolster was retained in the stomach 4 months after catheter removal. Endoscopic retrieval resulted in resolution of the symptomatology. The final case was found to have an asymptomatic bolster in the stomach approximately 18 months after catheter removal. CONCLUSIONS: These cases highlight a potential sequelae of pediatric percutaneous endoscopic gastrostomy not previously acknowledged. The significant complications associated with the retained bolster in four of these five children suggests that follow-up should be altered to monitor prompt passage of the crossbar after tube removal.

Experience with 220 consecutive laparoscopic Nissen fundoplications in infants and children.

Year 1998
Rothenberg SS.
Columbia Presbyterian/St Lukes Medical Center, Denver, Colorado, USA.
BACKGROUND/PURPOSE: Fundoplication for gastroesophageal reflux disease is a common procedure performed in infants and children. This report describes a 4-year experience with 220 consecutive laparoscopic Nissen fundoplications. METHODS: Ages ranged from 5 days to 18 years and weight from 1.4 to 100 kg. The procedures were performed using a five-trocar technique and with 5- or 3.4-mm instruments depending on the size of the patient. RESULTS: Two hundred eighteen fundoplications were completed successfully. Average operative time dropped dramatically from 109 to 55 minutes for the first 30 cases compared with the last 30. Intraoperative and postoperative complication rates were 2.6% and 7.3%, respectively. Average time to discharge postfundoplication was 1.6 days. The wrap failure rate is 3.4%. CONCLUSIONS: This study shows that although the learning curve for laparoscopic fundoplication may be steep, the procedure is safe and effective in the pediatric population. The clinical results are comparable to the traditional open fundoplication but with a significant decrease in morbidity and hospitalization.

Laparoscopic Ladds procedure in infants with malrotation.

Year 1998
Bass KD. Rothenberg SS. Chang JH.
Presbyterian St Luke's Columbia Medical Center and The Children's Hospital, Denver, CO, USA.
PURPOSE: This clinical study was undertaken to examine the feasibility of a laparoscopic approach for the treatment of documented malrotation. METHODS: From May 1994 through January of 1997, 12 patients, aged 5 days to 4 months, weighing 3 to 7 kg, underwent laparoscopic Ladd's procedure for malrotation. All patients had symptoms of intermittent upper intestinal obstruction, and malrotation was documented by an upper gastrointestinal contrast study. None of the patients had acute volvulus or compromised bowel. The procedure was performed using 3 trocars of 3.5 mm diameter. Ports were placed in the infraumbilical ring, and the right and left mid to lower quadrants. A standard Ladd's procedure with appendectomy was performed in all cases. RESULTS: All procedures were completed successfully through the laparoscope. Operative times averaged 58 minutes (35 to 120 minutes). One patient with Pierre-Robin underwent a laparoscopic Nissen fundoplication and gastrostomy tube placement at the same time requiring 120 minutes. Feedings were started on postoperative day (POD) 1 in 10 cases and POD 2 in two cases. Hospital stay ranged from 2 to 4 days (average, 2.2) in the patients with isolated malrotation. The patient with Pierre-Robin had a prolonged hospitalization because of chronic respiratory problems not associated with surgery. There were no complications. All patients had resolution of their symptoms. CONCLUSIONS: Laparoscopic Ladd's procedure is a safe and effective technique. It can be performed in neonates in times equivalent to standard open techniques, and it appears to allow for earlier feeds and decreased hospital stays.

Laparoscopic gastric antroplasty in children with delayed gastric emptying and gastroesophageal reflux.

Year 1998
Sampson LK. Georgeson KE. Royal SA.
The University of Alabama at Birmingham, Department of Surgery, The Children's Hospital of Alabama, 35233, USA.
BACKGROUND/PURPOSE: A significant number of children (50%) with gastroesophageal reflux (GER) have delayed gastric emptying (DGE). Although controversial, many pediatric surgeons use a gastric outlet procedure in conjunction with fundoplication for gastroesophageal reflux in these patients. This paper describes the technique and clinical outcome of 61 patients undergoing a laparoscopic gastric antroplasty at the time of the laparoscopic fundoplication. METHODS: The charts of 61 patients who underwent laparoscopic gastric antroplasty in conjunction with laparoscopic fundoplication between May 26, 1992 and October 17, 1996 were reviewed retrospectively. All patients had a documented DGE confirmed by a liquid isotope meal being retained in the stomach. After completion of the fundoplication, a laparoscopic antroplasty was performed by incising a 2 to 3.5-cm linear incision in the pylorus and distal gastric antrum. The seromuscular wall was divided to the level of the mucosa allowing the mucosa to bulge through the defect. The wound was closed transversely using interrupted 2-0 silk sutures. RESULTS: Four of the 61 patients underwent conversion to open antroplasty for technical reasons. The remaining 57 patients recovered uneventfully from the laparoscopic antroplasty with clinical resolution of both GER and DGE. Two of 57 patients had intermittent episodes of retching and were unable to tolerate large bolus feedings because of dumping. They were treated by dividing the feedings into two smaller portions. These symptoms cleared within 6 months. The remaining 55 patients have tolerated feedings well. Evaluation of the gastric emptying was performed randomly in selected patients with documented improvement of the emptying after antroplasty. An evisceration of omentum through the umbilical incision developed in one patient on the third postoperative day. CONCLUSIONS: Patients with delayed gastric emptying who need fundoplication can be treated with laparoscopic gastric antroplasty in conjunction with laparoscopic fundoplication. Laparoscopic antroplasty appears to be clinically efficacious in improving delayed gastric emptying.

Antenatal glucocorticoid therapy suppresses angiotensin-converting enzyme activity in rats with nitrofen-induced congenital diaphragmatic hernia.

Year 1998
Okoye BO. Losty PD. Fisher MJ. Hughes AT. Lloyd DA.
Department of Paediatric Surgery, Institute of Child Health, Alder Hey Children's Hospital, Liverpool, England.
BACKGROUND/PURPOSE: Neonates with congenital diaphragmatic hernia (CDH) have a high morbidity and mortality rate caused by pulmonary hypoplasia associated with pulmonary hypertension (PH). In experimental CDH, antenatal glucocorticoid therapy improves surfactant biochemical immaturity, enhances lung compliance, and induces morphological maturation in CDH rats. The effects of steroid therapy on preventing or treating PH in this condition have not been studied. Angiotensin converting enzyme (ACE), which is produced by the vascular endothelium, is implicated in the pathogenesis of pulmonary hypertension. The aim of this study was to evaluate the effect of antenatal glucocorticoid therapy on ACE activity and expression in CDH rat lungs. METHODS: CDH was induced in fetal rats by the maternal administration of 100 mg nitrofen on day 9.5 of gestation (term, day 22). Dexamethasone (Dex) (0.25 mg/kg) was given by intraperitoneal injection on days 18.5 and 19.5 before delivery of the fetuses by cesarean section on day 21.5. Control animals received olive oil (OO) by gavage and normal saline (NS) as vehicle injection. ACE activity was measured spectrophotometrically in the lungs of rats from four treatment groups: CDH-NS, non-CDH-NS, CDH-Dex, and OO-NS controls. Total lung ACE activity (mU per lung) was significantly lower in CDH-NS (P = .002) and CDH-Dex (P = .004) rats compared with non-CDH-NS and OO-NS controls (9.1 +/- 1.0 and 10.7 +/- 1.3 v 16.2 +/- 1.6 and 15.4 +/- 1.7). When specific ACE activity (mU/mg protein) was derived by expressing ACE activity per milligram of lung protein, CDH-NS animals showed elevated specific ACE activity (P = .05) compared with OO-NS controls (6.31 +/- 1.1 v 4.4 +/- 0.4). CDH-Dex animals had a significantly lower specific ACE activity (P = .01) compared with CDH-NS and Non-CDH-NS rats (4.0 +/- 0.4 v 6.31 +/- 1.1 and 5.83 +/- 0.54). The specific ACE activity levels of CDH-Dex rats were equivalent to those seen in the lungs of OO-NS controls (P = .24). CONCLUSION: Antenatal steroid therapy, by suppressing pulmonary ACE activity, may reduce the risk of pulmonary hypertension developing in human newborns with antenatally diagnosed CDH.

Abdominal packing for severe hemorrhage.

Year 1998
Stylianos S.
Division of Pediatric Surgery, Babies & Children's Hospital of New York, New York 10032, USA.
BACKGROUND: Diffuse abdominal or retroperitoneal hemorrhage is potentially lethal particularly when associated with coagulopathy, hypothermia, and acidosis. Temporary abdominal packing (PACKS) can control hemorrhage and provide crucial time to correct these physiological and metabolic derangements. METHODS: The author reviewed the combined experience of pediatric surgeons at 13 institutions to determine the efficacy of this technique. RESULTS: Twenty-two patients with refractory hemorrhage (ages, 6 days to 20 years) were treated with PACKS. The etiology of hemorrhage was trauma in 13, solid organ tumor bed in four, liver bleeding during necrotizing enterocolitis surgery in two, hemorrhagic pancreatitis in one, iliac artery injury while on extracorporeal membrane oxygenation (ECMO) in one, and biliary reconstruction after liver transplant in one. The anatomic site of hemorrhage was the liver or hepatic veins in 14, retroperitoneum or pelvis in seven, and the pancreatic bed in one. Twenty patients (91%) were coagulopathic, hypothermic, and acidotic at the time of packing. Fifteen patients (68%) had PACKS inserted during a primary operative procedure, whereas seven patients (32%) had PACKS inserted during a reexploration for persistent hemorrhage. The mean volume of intraoperative transfusion before PACKS was 190 mL/kg (range, 50 to 600). Primary fascial closure was accomplished in 12 (55%) patients, and temporary skin closure or prosthetic material was used in the other ten. PACKS controlled hemorrhage in 21 of 22 (95%) patients. Removal of PACKS was possible within 72 hours in 18 (82%) patients. No patient experienced rebleeding after PACKS removal; however, two patients died with PACKS in place. An abdominal abscess developed in seven patients (32%); all were successfully drained. Eighteen patients (82%) survived after abdominal packing. Two deaths were caused by multisystem organ failure, one was caused by cardiac failure from uncorrectable cardiac anomalies, and one was from exsanguination after blunt traumatic liver injury. There were no differences in volume of intraoperative blood product transfusion, time to initiate PACKS, physiological status, or type of abdominal closure between survivors and nonsurvivors. CONCLUSION: The author concludes that temporary abdominal packing can be life saving in children with refractory abdominal or retroperitoneal hemorrhage associated with coagulopathy, hypothermia, and acidosis.

Nonoperative management of pancreatic injuries in children.

Year 1998
Shilyansky J. Sena LM. Kreller M. Chait P. Babyn PS. Filler RM. Pearl RH.
Department of Surgery, University of Toronto, Hospital for Sick Children, Ontario, Canada.
PURPOSE: The safety and efficacy of nonoperative management of pancreatic contusions and transections was examined by reviewing the case histories of 35 consecutive children with pancreatic injuries treated over the past 10 years. METHODS/RESULTS: Surgical exploration was performed for the management of associated injuries only. The diagnosis of pancreatic trauma was suspected in children with abdominal pain, tenderness, elevated serum amylase levels and findings consistent with pancreatic injury on abdominal ultrasound scan or computerized tomography (CT) examination. After children were diagnosed with pancreatic injury, enteral feedings were withheld and total parenteral nutrition administered until abdominal pain resolved and serum amylase levels and radiographic findings improved. Twenty-three children received diagnosis within 24 hours of injury, and in 12, the diagnosis was delayed 2 to 14 days. Hyperamylasemia was found in 27 of 35 children. Twenty-eight children sustaining pancreatic injuries were treated nonoperatively. Abdominal imaging in these children demonstrated pancreatic contusion in 14, transection in 11, and pseudocyst in three. Enteral feeding resumed an average of 15 days after injury. The average hospital stay was 21 days. Pseudocysts formed in 10 children (2 of 14 with contusion; 5 of 11 with transection; three children presented late, and the type of pancreatic injury could not be determined), whose average hospital stay was 25 days. All pseudocysts were successfully managed nonoperatively, although percutaneous aspiration or drainage was required in six children. Children underwent follow-up for an average of 10 months after injury (range, 1 to 144 months). Abdominal pain and radiological abnormalities resolved in all children before discharge from the clinic. CONCLUSIONS: Nonoperative management of pancreatic contusion and transection diagnosed radiologically is effective and safe. Pseudocysts may form after pancreatic injury, and if large or symptomatic, can be managed successfully by percutaneous drainage.

Peritoneal drainage as definitive treatment for intestinal perforation in infants with extremely low birth weight (<750 g).

Year 1998
Lessin MS. Luks FI. Wesselhoeft CW Jr. Gilchrist BF. Iannitti D. DeLuca FG.
Division of Pediatric Surgery, Brown University School of Medicine and Hasbro Children's Hospital, Providence, Rhode Island, USA.
BACKGROUND: Advances in neonatal intensive care have improved the survival of the extremely premature infant. However, survival at less than 25 weeks' gestational age remains tenuous, with intestinal perforation presenting a significant mortality. METHODS: During an 18-month period from 1995 to 1996, nine patients weighing less than 750 g (range, 485 to 740 g; mean, 615 g) presented with intestinal perforation. All patients were treated with peritoneal drainage. Drains were removed after clinical improvement and the cessation of peritoneal drainage. RESULTS: Seven patients survived the initial drainage procedure (78%). At a mean follow-up of 12 months, the six long-term survivors are all tolerating full enteral feeds, and none developed intestinal strictures or intraabdominal abscess. No patient required subsequent celiotomy. Peritoneal drainage has previously been considered in some centers as temporary therapy in extremely ill neonates deemed unlikely to survive operation. The authors have adopted drainage as the sole treatment in selected patients. CONCLUSION: Peritoneal drainage alone may be considered definitive therapy for intestinal perforation in the majority of micropremature infants.

Increased insulin-like growth factor and platelet-derived growth factor system in the pyloric muscle in infantile hypertrophic pyloric stenosis.

Year 1998
Ohshiro K. Puri P.
Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.
BACKGROUND: Infantile hypertrophic pyloric stenosis (IHPS) is characterized by hypertrophy of the pyloric muscle. The etiology of IHPS is unknown. The growth of smooth muscle cells (SMC) is regulated by several growth factors. Insulin-like growth factor (IGF) and platelet-derived growth factor (PDGF) act synergistically to stimulate SMC proliferation. The effects of IGF-I and PDGF are mediated via their receptors. METHODS: Full-thickness muscle biopsy specimens were obtained from eight IHPS patients (age range, 14 to 46 days) at pyloromyotomy and from eight age-matched controls without gastrointestinal disease at autopsy performed within 4 hours after death. Indirect three-step immunohistochemistry was performed using anti-IGF-I, IGF-I receptor alpha (IGF-IR alpha), IGF-IR beta, PDGF-BB and PDGF receptor (PDGF-R) antibodies and visualized by peroxidase staining. RESULTS: The most striking difference between tissues from IHPS patients and controls was the marked increase in IGF-I, IGF-IR alpha, IGF-IR beta and PDGF-R in the hypertrophic circular muscle layer, and, to a lesser degree, in the longitudinal muscle in pyloric stenosis. CONCLUSION: The findings suggest that the upregulated local IGF and PDGF systems may play a role in the development of pyloric muscle hypertrophy in IHPS.

Adventitial changes in pulmonary vasculature in congenital diaphragmatic hernia complicated by pulmonary hypertension.

Year 1998
Taira Y. Yamataka T. Miyazaki E. Puri P.
Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
PURPOSE: The purpose of this study was to characterize structural changes in the pulmonary vasculature in congenital diaphragmatic hernia (CDH) complicated by persistent pulmonary hypertension (PPH) with particular emphasis on adventitial thickness. METHODS: Victorian blue Van Gieson (VVG) staining and immunostaining with antialpha smooth muscle actin (ASMA) were performed on lung tissues obtained at autopsy from 23 patients with CDH complicated by PPH and 11 age-matched control tissues of sudden infant death syndrome patients (SIDS). The degree of medial and adventitial thickening was measured in pulmonary arteries with an external diameter (ED) of less than 75 microm, 75 to 100 microm, 100 to 150 microm, 150 to 250 microm, 250 to 500 microm, and greater than 500 microm by IPS-4.01 image analyzer and compared statistically. The degree of medial thickening and adventitial thickening was also measured in pulmonary veins with an ED of less than 100 microm, 100 to 200 microm, and greater than 200 microm. To determine whether the characteristic structural changes were size related, each was related to ED. The area of adventitia and media of the pulmonary arteries and veins was measured using image analyzer. RESULTS: There was a significant increase in medial and adventitial thickness in arteries of all sizes in CDH patients compared with controls (P < .01). The degree of adventitial area was significantly increased for arteries of all sizes (P < .01) and the degree of medial area was significantly increased only for arteries less than 100 microm size (P < .05) in CDH patients compared with controls. Calculation of the areas of the various components in the wall of each artery showed that for small arteries (

Ventriculo-peritoneal shunts in children reveal the natural history of closure of the processus vaginalis.

Year 1998
Clarnette TD. Lam SK. Hutson JM.
F. Douglas Stephens Surgical Research Laboratory, Royal Children's Hospital, Parkville, Victoria, Australia.
PURPOSE: Little information is known about the natural history of closure of the processus vaginalis. The authors studied children who had ventriculoperitoneal shunts to determine the natural history of closure of the processus vaginalis and to better understand the role of intraabdominal pressure in the etiology of congenital inguinal hernia. MATERIALS AND METHODS: A retrospective review of all children undergoing insertion of ventriculoperitoneal shunts between 1985 and 1995 at the Royal Children's Hospital was undertaken. In each case, the sex, the cause of hydrocephalus, the age at insertion of the shunt, and the subsequent development of an inguinal hernia or hydrocele was recorded. RESULTS: There were 430 children in the study. An inguinal hernia developed in 15% of children after insertion of a ventriculoperitoneal shunt, and a hydrocele developed in an additional 6% of boys. Inguinal hernias were bilateral in 47% of boys and 27% of girls. The incidence of subsequent development of an inguinal hernia or hydrocele was closely related to the age of insertion of the ventriculoperitoneal shunt, being 30% during the last 8 weeks of gestation and the first few months of life, then falling quite sharply to reach about 10% at 1 year. CONCLUSIONS: The high occurrence of inguinal hernias and hydroceles after ventriculoperitoneal shunt insertion supports the role of raised intraabdominal pressure in the etiology of these conditions. It appears that raised intraabdominal pressure is associated with an increased incidence of clinical hernias, but not with increased incidence of patency of the processus vaginalis. The development of an inguinal hernia or hydrocele after insertion of a ventriculoperitoneal shunt can be used as an indirect marker of patency of the processus vaginalis at the time of insertion of the shunt. From this, we propose that the processus vaginalis remains patent in at least 30% of children in the first few months of life, after which time the patency rate appears to fall off quite sharply.

Pyogenic liver abscess in children--South Indian experiences.

Year 1998
Kumar A. Srinivasan S. Sharma AK.
Department of Paediatrics and Radiodiagnosis, Jawaharlal Institute of Post-Graduate Medical Education & Research, Pondicherry, India.
PURPOSE: Eighteen cases of pyogenic liver abscess (PLA) admitted at JIPMER hospital, South India, over a 6-year period were analyzed to document the clinical profile and to evaluate the management of PLA among children. METHODS: Records of all these patients were reviewed for presenting signs and symptoms, any associated condition, investigative results, management, and follow-up findings. RESULTS: The overall incidence of PLA was 78.9 per 100,000 pediatric (under 12 years) admissions. One patient had aplastic anemia and was on long-term steroid therapy, whereas another had measles in recent past. Moderate to severe malnutrition was present in five (27.8%) and ascariasis in seven (38.9%) children. Common presentations were fever (100%), abdominal pain (76.9%), and tender hepatomegaly (83.3%). Ultrasonography results were positive in all cases. Fourteen patients (77.8%) had solitary liver abscess, and four had multiple abscesses. Organism was isolated in 11 cases (63.6%), and Staphylococcus aureus was the commonest isolate (66.7%). All patients received antibiotics. Twelve cases were managed conservatively with antibiotics alone, of these only two (16.7%) required drainage later on. Percutaneous aspiration was also undertaken in four additional (22.2%) cases and open drainage in two (11.1%), at presentation. The overall mortality rate was 11.1%. Time taken for complete resolution ranged from 10 days to 40 days. CONCLUSIONS: Any child presenting with fever, abdominal pain, and tender hepatomegaly should be subjected to ultrasound scan for early detection of PLA. S aureus is the commonest causative agent. Enterobacteriaceae contribute significantly during infancy. A combination of cloxacillin and gentamicin or a third generation cephalosporine and gentamicin, especially in infants, is a satisfactory initial coverage. Therapeutic drainage is not a must in all cases of PLA. When required, percutaneous needle aspiration is safe and effective. Resolution and significant reduction in mortality has been made possible by early detection and optimum antibiotics therapy.

Effects of anal invasive treatment and incontinence on mental health and psychosocial functioning of adolescents with Hirschsprungs disease and low anorectal anomalies.

Year 1998
Diseth TH. Egeland T. Emblem R.
Division of Child and Adolescent Psychiatry, The National Hospital, Oslo, Norway.
BACKGROUND/PURPOSE: Recent studies of adolescents with Hirschsprung's disease (HD) and low anorectal anomalies (LARA) showed persistent impairment of fecal control in both groups, but very different mental and psychosocial outcome. METHODS: To explore possible reasons for these differences, 19 adolescents with HD (aged 10 to 20 years; median, 16) operated on by the Duhamel technique were compared with 17 adolescents with LARA (aged 12 to 20 years; median, 15). The 36 adolescents were assessed for treatment procedures, bowel function, and mental and psychosocial outcome by data collected from medical records, physical examination, semistructured interview, and standardized questionnaires. The parents of 30 adolescents were also interviewed and completed questionnaires. RESULTS: Duration of anal invasive treatment procedure and current bowel function were associated with mental and psychosocial outcome. The treatment variable, duration of anal dilation, was the most significant predictor of the adolescents's mental health (R2 = .41, P < .01), whereas chronic family difficulties and parental warmth together with the current bowel function variables, fecal and flatus continence function, best explained the variance in psychosocial outcome (R2 = .77, P < .0001). Thus, the differences in treatment procedures and continence function between the HD and LARA groups may partially explain differences in mental and psychosocial outcome. CONCLUSIONS: These findings suggest that anal dilatation and continence dysfunction may have negative impact on mental health and psychosocial functioning. Indications for and ways of performing the procedure of dilation, and the treatment of persistent incontinence problems, are questioned.

Impaired gastric emptying in children with repaired esophageal atresia: a controlled study.

Year 1998
Montgomery M. Escobar-Billing R. Hellstrom PM. Karlsson KA. Frenckner B.
Department of Pediatric Surgery, St Goran's/Karolinska Hospital, Karolinska Institute, Stockholm, Sweden.
BACKGROUND: Scintigraphy is considered the "gold standard" for investigating gastric emptying. The lack of standards regarding registration technique and meal composition has been a problem especially in pediatric patients. METHODS: In this study, gastric emptying of a solid meal was assessed by scintigraphy in 10 patients with repaired esophageal atresia (5 to 10 years old), and the results were compared with those in 11 healthy control children (5 to 11 years old). The meal consisted of pancakes with a fixed energy composition labeled with Tc-99m. Fractional meal retention values were plotted as a function of time. RESULTS: Half-emptying time and lag phase values were longer in the patient group, whereas the emptying rate was slower and the retention values at 60 and 90 minutes were higher than in the control group. Extremely long lag phase and slow emptying rates were seen in two patients with reflux symptoms and abdominal complaints. Gastric emptying in healthy children has not previously been studied by scintigraphy. The results of this study show that values for gastric emptying of solids in healthy children correspond well to those reported in healthy adults. CONCLUSION: Scintigraphy is an easy and reliable method for gastric emptying studies in children. The radioactive dose can be kept very low, which makes it a safe method even for pediatric patients. Delayed gastric emptying can occur in patients who have repaired esophageal atresia, and may be associated with reflux symptoms and abdominal complaints.

Multisystem organ failure and capillary leak syndrome in severe necrotizing enterocolitis of very low birth weight infants.

Year 1998
Sonntag J. Wagner MH. Waldschmidt J. Wit J. Obladen M.
Department of Neonatology, Clinic of Pediatric Surgery, Charite-Virchow-Klinikum, Humboldt University, Berlin, Germany.
BACKGROUND: Classification systems for necrotizing enterocolitis (NEC) in preterm infants have been developed to define severity grades relevant for treatment and prognosis. Multisystem organ failure (MSOF) and capillary leak syndrome (CLS) also have prognostic value in these patients. The aim of this retrospective study was to investigate the incidence and predictive value of MSOF and CLS according to the classification criteria. METHODS: The records of 1,022 very low birth weight infants admitted from 1982 to 1996 were reviewed for diagnosis of NEC stage IIA or higher (classification of Walsh and Kliegman). Among those patients (n = 50) the incidence of MSOF and CLS was determined, separately for surgical or conservative treatment. RESULTS: Twelve patients were assigned to stage II, 22 to stage IIIa, and 16 to stage IIIb; 31 infants underwent operation. Mortality rate was not influenced by the grade. In eight patients only gastrointestinal symptoms were found, whereas in 23 patients, up to three organ systems and in 19 patients, four or more organ systems were affected. Mortality depended on the number of involved organ systems. CLS occurred postoperatively in 10 of the 31 infants; eight of them died. CONCLUSION: The prognostic values of MSOF and CLS are higher than that of classification criteria in NEC of VLBW infants.

Exteriorization of the distal esophagus in the abdomen in esophageal atresia.

Year 1998
Bhatnagar V. Agarwala S. Chattopadhyay A. Mitra DK.
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi.
METHODS: The distal esophagus was exteriorized on to the left upper abdominal wall (abdominal esophagostomy) in 15 babies who had esophageal atresia with or without tracheo-esophageal fistula. The indications for this procedure were long gap atresia with or without tracheoesophageal fistula in which primary anastomosis was not possible and a major anastomotic dehiscence requiring cervical esophagostomy and gastrostomy. In all these patients a decision to replace the esophagus had been made, and a cervical esophagostomy was constructed. The distal esophagus was mobilized either from the thorax if thoracotomy had been done or by a transhiatal abdominal route. CONCLUSIONS: Advantages of the abdominal esophagostomy include absence of gastroesophageal reflux, no indwelling catheter, early institution of enteral feeds, intermittent catheterization for feeding, easy nursing care, and no stomal complications. In addition, this procedure allows the entire stomach to be available for esophageal replacement and retains the natural gastroesophageal junction and the lower esophagus for anastomosis to any bowel segment being used for the esophageal replacement.

Serial lung volume measurements during the perinatal period in infants with abdominal wall defects.

Year 1998
Laubscher B. Greenough A. Dimitriou G. Davenport M. Nicolaides KH.
Department of Child Health, Harris Birthright Research Centre for Fetal Medicine, King's College Hospital, London, England.
METHODS: Daily measurements of lung volume (functional residual capacity, FRC) were made during the perinatal period in eight infants (median gestational age, 37 weeks; range, 34 to 38 weeks) with abdominal wall defects. RESULTS: On the first day of life and before surgical intervention, four infants had FRCs below the reference range; the occurrence of low lung volumes was not significantly related to gestational age or diagnosis. Lung volume was further, but only temporarily, impaired by surgical closure of the abdominal wall defect, with a reduction in the median FRC from 25 mL/kg (range, 18 to 36) preoperatively to 12 mL/kg (range, 5 to 19) on the first postoperative day (P < .02). CONCLUSION: These data are consistent with abnormal antenatal lung growth in certain infants with abdominal wall defects.

Chylothorax after repair of congenital diaphragmatic hernia--risk factors and morbidity.

Year 1998
Kavvadia V. Greenough A. Davenport M. Karani J. Nicolaides KH.
Children Nationwide Regional Neonatal Intensive Care Unit, Department of Child Health, King's College Hospital, London, England.
BACKGROUND/PURPOSE: Chylothoraces have been reported rarely after congenital diaphragmatic hernia (CDH) repair, but that is contrary to the authors' experience. The aim of this study was to audit the outcome of antenatally diagnosed CDH cases to determine the incidence, possible risk factors, and morbidity associated with chylothorax in CDH patents. METHODS: Twenty-four of 35 consecutive infants with CDH (69%) underwent surgical repair and survived the immediate postoperative period. Results: Effusions developed in all 22 for whom chest radiographs were available. Six of the eight infants whose effusions required drainage had a chylothorax. Those six infants required a mean of 8 days of thoracentesis, during which a mean total volume of 358 mL/kg of fluid was removed. The infants remained on a medium-chain triglyceride (MCT) formula for a mean of 81 days. The six infants with a chylothorax differed from the rest with respect to their duration of oxygen dependence (a median of 28.5 versus 15 days, P < .05) and hospital stay (a median of 5.5 versus 4 weeks, P < .05), but no significant risk factors for the development of a chylothorax were identified. CONCLUSION: Chylothorax is a relatively common cause of effusion after CDH repair and is associated with increased morbidity.

All mechanical low rectal anastomosis in children.

Year 1998
Mattioli G. Buffa P. Martinelli M. Ivani G. Jasonni V.
Department of Pediatric Surgery, University of Genoa School of Medicine, Giannina Gaslini Scientific Institute, Italy.
PURPOSE: The aim of this study is to demonstrate the feasibility and usefulness of mechanical suturing in children for low rectal anastomosis. METHODS: The study group includes 31 children operated on from January 1993 to July 1996 by the same senior surgeon, performing the modified Duhamel procedure for Hirschsprung's disease in 17 children, intestinal neuronal dysplasia in seven, and the Knight-Griffen procedure in seven pediatric patients with chronic ulcerative colitis. RESULTS: In all the cases the technique of "viscero-synthesis" was performed using the mechanical stapler. A circular stapler has been used for the end-to-end and the end-to-side anastomosis between the anal canal or the back wall of the rectum with the pulled viscus, while a linear endoscopic stapler (GIA) has been used for the consolidation of the rectocolic wall in the modified Duhamel technique. CONCLUSIONS: The results obtained demonstrate that the mechanical staplers in children are safe and effective in low rectal anastomosis, sparing operative time and reducing the risk of anastomotic dehiscence; however, the size of circular instruments limits its use in neonates and small infants.

Congenital diaphragmatic hernia--does the side of the defect influence the incidence of associated malformations?

Year 1998
Losty PD. Vanamo K. Rintala RJ. Donahoe PK. Schnitzer JJ. Lloyd DA.
Institute of Child Health, Royal Liverpool Children's Hospital (Alder Hey), England.
BACKGROUND/PURPOSE: Patients with congenital diaphragmatic hernia (CDH) frequently have associated anomalies. Experiments in the nitrofen CDH model have shown differential embryonic cell death patterns in rodents suggesting unique mechanisms in the formation of right-sided (RCDH) or left-sided (LCDH) diaphragmatic hernia. These findings provide insight into the pathogenesis of CDH and may aid our understanding on the spectrum of associated anomalies commonly observed in humans. This study therefore set out to test the hypothesis that the side of the diaphragmatic defect in humans is related to the incidence and severity of coexistent organ malformations. METHODS: The medical and autopsy records of 301 CDH patients presenting to two institutions over a 23-year period were examined to analyze these factors. RESULTS: One hundred patients (33%) were found to have one or more associated anomalies. The incidence of multiple-RCDH (10%) versus LCDH (7.3%) and cardiac anomalies-RCDH (10%) versus LCDH (8.5%) was similar in both groups of patients. However, the hypoplastic heart syndrome was a unique feature in 5 of 22 patients (23%) with LCDH who had cardiac abnormalities. This cardiac anomaly may be related developmentally to LCDH. CONCLUSION: The cellular mechanisms underlying the genesis of this spectrum of abnormalities in humans and the nitrofen CDH model warrant further study to elucidate factors governing embryonic cell fate and phenotype expression.

Quality of life more than 20 years after repair of esophageal atresia.

Year 1998
Ure BM. Slany E. Eypasch EP. Weiler K. Troidl H. Holschneider AM.
Department of Pediatric Surgery, The Children's Hospital of Cologne, University of Cologne, Germany.
PURPOSE: To examine the quality of life after repair of esophageal atresia, follow-up studies were performed in 58 of 71 surviving patients (81.7%). METHODS: Fifty patients with primary anastomosis and all eight surviving patients with colon interposition were seen. The mean age was 25.3 years (range, 20 to 31). Symptoms were evaluated by a standardized interview. Quality of life assessment was performed using a visual analogue scale (0 to 100 points), the Spitzer Index (5 dimensions, 10 points), and the Gastrointestinal Quality of Life Index (GIQLI, 5 dimensions, 128 points). RESULTS: After primary anastomosis the estimated meal capacity was unrestricted in 46 patients (92%), but numerous symptoms such as recidivating cough (60%), hold up (48%), and short breath (30%) were reported. All symptoms except cough were seen more frequently in patients with colon interposition, and all of these patients suffered from periods of short breath. Quality of life scores were higher in patients with primary anastomosis compared with colon interposition. The difference in the visual analogue scale score did not reach statistical significance, but the mean Spitzer Index was 9.7 compared with 8.8 after colon interposition (P < .05). The GIQLI after primary anastomosis was similar to that in healthy controls and was significantly lower in patients with colon interposition. This was because of specific symptoms, which scored 49.3 after colon interposition compared with 61.7 after primary anastomosis (P < .05) and to 54.8 (SD 5) in healthy controls (P < .05). Physical and social functions, emotions, and inconvenience of a medical treatment scored similar in patients with primary anastomosis, colon interposition, and healthy volunteers. CONCLUSIONS: The long-term quality of life after primary anastomosis was excellent. Patients with colon interposition suffer more frequently from various gastrointestinal and respiratory symptoms, but they lead an otherwise normal life.

Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia.

Year 1998
Fu T. Cui X. Wang X. Fu Z.
Department of Pediatric Surgery, Affiliated Hospital of Binzhou Medical College, Shandong, China.
A full-term baby boy with a segmental defect of the ileal musculature associated with terminal ileal and biliary atresia is presented. The newborn had a dilated loop of the ileum 30 cm proximal to the ileal atretic site. Pathological study results showed absence of the intestinal musculature with relatively intact mucosa. Foci of recent muscular necrosis were found in the lesion. One month later, relaparotomy was performed because of persistent jaundice and hepatic duct atresia was confirmed. Segmental defect of the intestinal musculature associated with ileal atresia and biliary atresia has not been reported in the literature. The authors emphasize that in the management of this unusual defect, one should pay attention to the multiple associated malformations.

Intraabdominal leg: unique variant of split notochord syndrome.

Year 1998
Fowler CL.
Department of Surgery, University of Kentucky Medical Center, Lexington 40536-0084, USA.
An infant was born with a spectrum of anomalies representing a unique variant of the split notochord syndrome. The major anomalies included giant omphalocele and duplicated lower spine, between which developed a posterior lumbosacral mass that was contiguous with an intraabdominal, skin-covered "leg" within a saccular cecum. Features of this case overlap aspects of fetiform teratoma, fetus-in-fetu, conjoined twins, and caudal duplication, suggesting an etiologic relation between these entities and split notochord syndrome.

Gastric diverticulum: an uncommon cause of abdominal pain in a 12 year old.

Year 1998
Ciftci AO. Tanyel FC. Hicsonmez A.
Department of Pediatric Surgery, Hacettepe University Medical Faculty, Ankara, Turkey.
A 12-year-old girl presented with long-lasting intractable abdominal pain associated with a gastric diverticulum. The clinicopathologic features of this rare entity are discussed with emphasis on pathogenesis, diagnosis, and treatment. The authors emphasize that congenital diverticulum of the stomach should be considered in the differential diagnosis of abdominal pain in childhood. Surgical treatment consisting of simple excision of the diverticulum is warranted in cases presenting with long-lasting symptoms after failure of medical treatment.

Eighteen years experience with neonatal Hirschsprungs disease treated by endorectal pull-through without colostomy.

Year 1998
So HB. Becker JM. Schwartz DL. Kutin ND.
Long Island Jewish Medical Center, Schneider Children's Hospital, New Hyde Park, NY, USA.
METHODS: In the past 18 years, the authors have treated 84 patients with Hirschsprung's disease. Of these, 43 patients were under 1 month of age and underwent endorectal pull-through without colostomy. Some have undergone follow-up for as long as 18 years. RESULTS: Thirty-four of these 43 (79%) newborn patients were available for follow-up. Twenty-two were totally continent. The remaining 12 have normal sphincter tone. Of the 41 patients above 1 month of age, 34 (83%) were available for follow-up. Some have undergone follow-up for as long as 18 years. CONCLUSION: Twenty-two of this latter group (79%) have normal bowel control.

The role of ultrasonography in the diagnosis of pyloric stenosis: a decision analysis.

Year 1998
Olson AD. Hernandez R. Hirschl RB.
Department of Pediatrics and Surgery, C.S. Mott Children's Hospital, University of Michigan Medical Center, Ann Arbor 48109-0245, USA.
BACKGROUND/PURPOSE: The appropriate role for ultrasonography (US) as a replacement for the upper gastrointestinal series (UGI) in vomiting infants remains undefined. The authors have used decision analysis techniques to determine whether the use of ultrasonography as an initial screen in vomiting infants is cost effective when compared with the UGI as the only study. METHODS: Two diagnostic strategies were compared: 1) UGI alone and 2) ultrasonography followed by an UGI series in 50% of cases when ultrasonography scan was negative for pyloric stenosis. The test sensitivity (US, 0.9; UGI, 1.0) and test specificity (US, 1.0; UGI, 1.0) and the incidence of pyloric stenosis among vomiting infants presenting to the community pediatrician (0.30) or after a negative examination by an experienced examiner (0.02 to 0.18) were obtained from a review of the literature. The relative charges for ultrasonography and UGI were obtained from a national survey from which the cost ratio of US to UGI was estimated to range from 0.67 to 1.81 with a median of 1.06. RESULTS: Under these baseline assumptions, UGI only was the preferred strategy. The results of the decision analysis were sensitive to, or dependent on, assumptions made regarding the incidence of pyloric stenosis, the US to UGI cost ratio, the sensitivity of the US, and the proportion of patients that proceed to UGI when the US scan was negative for pyloric stenosis. When at least 50% of patients whose US scan was negative for pyloric stenosis proceeded to a UGI, UGI remained the preferred strategy for all cost ratios examined (0.6 to 1.7). Even when no patients proceeded to UGI, the cost ratio of US to UGI had to be less than 0.7 under the typical incidence (0.30) of pyloric stenosis among vomiting infants presenting to the community pediatrician for US to be cost effective. Finally, only UGI was indicated when an olive was not appreciated by an experienced examiner. CONCLUSION: Under assumptions that fit most clinically relevant circumstances, the UGI as the initial study is the most cost-effective radiological diagnostic test in the evaluation of the vomiting infant.

A quantitative study of the morphological and histochemical changes within the nerves and muscle in infantile hypertrophic pyloric stenosis.

Year 1998
Abel RM. Bishop AE. Dore CJ. Spitz L. Polak JM.
Department of Histochemistry and the Medical Statistics Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, England.
PURPOSE: The aim of this study was to quantify changes in dimensions of nerves and muscle and the proportionate expression of neural antigens in infantile hypertrophic pyloric stenosis (IHPS). METHODS: Twenty specimens of pylorus from children with IHPS and age/sex-matched controls were examined using conventional histology and immunohistochemistry for a range of nerve and muscle antigens. The changes in the proportion of nerves expressing each antigen were quantified and statistically analyzed. RESULTS: The longitudinal muscle was found to be hypertrophic and protein gene product 9.5-stained nerves appeared longer and thicker in the myenteric plexus and shorter in the longitudinal muscle layer in IHPS. The proportion of nerves that expressed neural nitric oxide synthase (nNOS) was found to be diminished in all the IHPS tissues examined. In the circular muscle and myenteric plexus, the proportion of nerves that expressed vasoactive intestinal polypeptide (VIP) and nNOS was almost identically diminished. The expression of calcitonin gene-related polypeptide and substance P was proportionately reduced in the myenteric plexus. Conclusions: The results of this study represent the first quantitative analysis of nerves and muscle in IHPS. The muscle hypertrophy is not restricted to circular muscle layer. The changes in nerve morphology cannot be attributed to a dilutional effect of the muscle hypertrophy. The selective changes in nerve and ganglion morphology varies between tissue layers and neural antigen expressed. The findings of reduced proportions of nerves expressing, in particular, nNOS may shed some light on the etiology of this condition.

Antenatal diagnosis of congenital anomalies of the biliary tract.

Year 1998
Redkar R. Davenport M. Howard ER.
Department of Pediatric Surgery, King's College Hospital, London, England.
BACKGROUND: The accuracy of the technique of antenatal ultrasonography in the diagnosis of congenital bile duct lesions is unknown. METHODS: Thirteen patients with proven biliary disease who had abnormal antenatal scans were reviewed. Two infants had type I cystic biliary atresia and one had a noncommunicating segmental dilatation of the bile duct in a type 3 biliary atresia. The remainder had choledochal cysts and included two patients with intrahepatic cysts. The correct diagnosis was made antenatally in only two (15%) cases. Of the remaining patients, seven received a diagnosis of intraabdominal cysts of unknown etiology, three of duodenal atresia, and one ovarian cyst. The median gestational age at the antenatal diagnosis was 20 weeks. RESULTS: Jaundice developed in 11 infants, and dilatation of intrahepatic biliary radicals was noted in four of the choledochal cysts. Obstructive jaundice and increasing cyst size were indications for early surgery, and twelve infants underwent a laparotomy at a median age of 4 weeks. During the median follow-up period of 2 years, 12 of the 13 patients have lost their jaundice or remained anicteric. Antenatal diagnosis offers the possibility of early definitive surgery for uncomplicated choledochal dilatation and the chance of improved outcome for surgically treated biliary atresia. An algorithm is suggested for the management of antenatally detected cystic biliary lesions.

Necrotizing enterocolitis after gastroschisis repair: a preventable complication?

Year 1998
Jayanthi S. Seymour P. Puntis JW. Stringer MD.
Department of Paediatric Surgery and Neonatology, Leeds General Infirmary, England.
BACKGROUND: Necrotizing enterocolitis (NEC) has been documented in up to 20% of infants after repair of gastroschisis and is responsible for significant morbidity. NEC is reported to occur up to 10 times more in preterm infants receiving standard formula compared with those who have been fed exclusively with breast milk. Does breast milk confer a similar protection against NEC in infants who have undergone surgery for gastroschisis? METHODS: All newborns with gastroschisis delivered between 1990 and 1996 and treated in a single neonatal unit were analyzed retrospectively. Clinical data, details of feeding regimens, and episodes of definite NEC were recorded. RESULTS: Of 60 infants with gastroschisis, 6 (10%) died but none had evidence of NEC. Of the remaining 54 infants, clinical and radiological signs of NEC developed in 8 (15%). All recovered with medical treatment including the three patients with recurrent episodes. NEC developed in none of the 12 babies exclusively fed with expressed breast milk (EBM) in contrast to 1 (5%) of the 19 who received both EBM and formula, and 7 (30%) of the 23 who were fed solely on formula. There was no significant difference in gestation, incidence of primary versus silo closure, or incidence of intestinal atresia/stenosis in those with NEC (n=8) compared with those without (n=46), but birth weight in the NEC group was lower. NEC was less likely to develop in infants who received EBM than those who were exclusively formula fed (P < .02). CONCLUSION: After gastroschisis repair, feeding with maternal expressed breast milk may help to protect the infant from developing NEC.

Perianal abscess and fistula-in-ano in infants.

Year 1998
Festen C. van Harten H.
Pediatric Surgical Center, University Hospital Nijmegen, The Netherlands.
PURPOSE: The aim of this study was to obtain insight into the short- and long-term results of treatment of perianal abscess and fistula-in-ano in infants. METHODS: This is a retrospective study of the records of patients treated over a 21-year period from January 1974 until December 1994 in a Pediatric Surgical Center. A long-term (1 to 24 year, mean 7.74 year) follow-up by questionnaire (response 81%) is also included. RESULTS: Drainage of a perianal abscess is followed in 35% of cases by a fistula. Fistulotomy or fistulectomy is followed in 13% of cases by a recurrence. There were two long-term recurrences that both healed spontaneously. The persisting scar sometimes gives problems with anal cleaning. All children aged 3 years and older were continent for feces. In two, there was soiling for some time. One had constipation and one was incontinent during the night. CONCLUSIONS: Simple drainage of a perianal abscess is followed frequently by a fistula. Fistulotomy or fistulectomy of a fistula-in-ano in infants has a reasonable chance of recurrence in the short term. Long-term recurrences are exceptional. There are no serious disabilities in the long run.

The pathological evaluation of the pediatric inguinal hernia sac.

Year 1998
Wenner WJ Jr. Gutenberg M. Crombleholme T. Flickinger C. Bartlett SP.
Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, 19104, USA.
BACKGROUND: The College of American Pathologists has suggested that institutions should establish guidelines for the evaluation of the hernia sac. In addition, some states require the submission of this tissue for pathological evaluation. Yet, neither evidence-based guidelines nor published reviews for the evaluation of the pediatric hernia sac are available. Therefore, this retrospective study was conducted to document experience with the evaluation of the pediatric hernia sac. METHODS: All reports of the evaluation of hernia sacs submitted to the Department of Pathology during an 8-year period were reviewed. The case of any report that differed in any way from "consistent with hernia sac" was evaluated for the effect of the findings on the clinical course. RESULTS: A total of 7,924 hernia sacs were submitted on 6,034 patients. Microscopic evaluation was performed on 534. A total of 7,567 (95.4%) submitted specimens on 5,743 patients were "consistent with hernia sac" and demonstrated no other findings. Three hundred fifty-seven specimens contained findings in addition to hernia sac. In no patient did the results of the evaluation have an effect on the patient care. CONCLUSIONS: There is strong evidence that the routine pathological evaluation of pediatric hernia sacs offers little relevant clinical information. Mandatory tissue submission of hernia sacs should be reconsidered.

Acquired umbilical fistula after repair of inguinal hernia: a case report.

Year 1998
Okuyama H. Fukuzawa M. Nakai H. Okada A.
Department of Pediatric Surgery, Osaka University Medical School, Suita, Japan.
A 2-year, 9-month-old boy had an umbilical fistula after repair of an inguinal hernia at 8 months of age. Fistulography findings showed a duct running from the umbilicus toward the inguinal wound. Pathological finding of the surgically removed fistula demonstrated granulomatous tissues containing silk ligature. Acquired umbilical fistula is a rare complication of inguinal herniorrhaphy. Its clinical details as well as a review of the previously reported four cases are presented.

Regression of childhood Barretts esophageal mucosa by antireflux surgery and bipolar electrocoagulation.

Year 1998
Garcia Montes C. Brandalise NA. Deliza R. Servidoni MF. Ferraz JG. Magalhaes AF.
Endoscopy Unit of the Gastrocentro, Faculty of Medical Sciences, State University of Campinas, Unicamp, Sao Paulo, Brazil.
The authors report a case of a 13-year-old girl with Barrett's esophagus who underwent antireflux surgery and was subsequently treated with endoscopic thermal coagulation using bipolar electrocoagulation. Follow-up endoscopy 15 months after completion of the endoscopic therapy showed normal esophageal mucosa without intestinal metaplasia. Longer follow-up is needed to assess the long-term effects of endoscopic treatment of the Barrett's mucosa with thermal coagulation, and this procedure should still be considered under investigation.

Intraoperative localization of small intestinal bleeding in an infant by methylene blue injection: a case report.

Year 1998
Mazziotti MV. Muldowney S. Darcy M. Hackett BP. Skinner MA.
Department of Surgery, Washington University School of Medicine, St Louis, MO 63110-1077, USA.
During the evaluation of patients with profuse gastrointestinal bleeding, it is often difficult to accurately localize bleeding sites in the small intestine. Moreover, during laparotomy, there may be no intraoperative findings to allow identification and resection of the bleeding lesion. Here the authors report a case of severe intestinal bleeding in an infant in whom the intraoperative injection of methylene blue dye into a terminal branch of the superior mesenteric artery was critical in determining the exact location of bleeding. After accurate localization of the bleeding source and segmental intestinal resection, the child recovered uneventfully with no recurrence of gastrointestinal bleeding. To the authors' knowledge, this is the first reported use of this technique in infancy.

Fibrous fusion between the liver and the lung: an unusual complication of right congenital diaphragmatic hernia.

Year 1998
Katz S. Kidron D. Litmanovitz I. Erez I. Dolfin Z.
Department of Pediatric Surgery, Sapir Medical Center, Meir General Hospital, Kfar Saba, Israel.
A congenital right diaphragmatic hernia was diagnosed in a full-term newborn who presented with respiratory distress at the age of 10 hours. The patient required respiratory support, and was operated on at age 6 days. During surgery, a central defect of the diaphragm was found. The herniated liver was only partially reducible because of intrathoracic adhesions. The patient died at 10 days of age as a result of persistent fetal circulation. Postmortem pathological examination demonstrated a central diaphragmatic defect and a nonseparable fibrous fusion between the liver and the lung.

Association of Klinefelter syndrome and abdominal teratoma: a case report.

Year 1998
Czauderna P. Stoba C. Wysocka B. Iliszko M.
Clinic of Pediatric Surgery and the Department of Biology and Genetics of the Medical University of Gdansk, Poland.
Extragonadal germ cell tumors are rare. The association with Klinefelter syndrome has become observed recently. A case of an 11-month-old infant with Klinefelter syndrome and a retroperitoneal mature teratoma is presented. In the tumor and lymphocytes, a 47,XXY karyotype was found. The association of Klinefelter syndrome with germ cell tumors and its possible explanations are discussed.

Successful right trisegmentectomy for ruptured hepatoblastoma with preoperative transcatheter arterial embolization.

Year 1998
Chan KL. Tam PK.
Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Hong Kong.
This is the first report of the successful use of percutaneous transcatheter arterial embolization (TAE) in controlling hemorrhage from ruptured hepatoblastoma, allowing early major hepatic resection to be performed safely in a young infant. A 6-month-old girl presented with a huge abdominal mass and was found to have a hepatoblastoma that measured 15 x 10 x 12 cm and arose from the right lobe of her liver on computed tomography (CT) scan examination. The tumor spontaneously ruptured, and she went into shock. TAE with gelfoam cube particles successfully arrested the tumor bleeding and allowed stabilization of her blood pressure with blood transfusion. Right trisegmentectomy was performed 12 hours later. The postoperative course was uneventful. With three courses of cisplatin, vincristine, and 5-fluorouracil after the hepatectomy, the serum alpha-fetoprotein level returned to normal, and the patient has remained well 4 months postoperation.

Cystic dysplasia of the testis associated with ipsilateral renal agenesis and high anorectal anomalies.

Year 1998
Ngai RL. Yeung BK. Tsui WM. Cheng FY.
Department of Surgery, Caritas Medical Centre, Kowloon, Hong Kong.
Scrotal masses in young children are often caused by hydrocoeles and hernias. When they arise from the testis, testicular tumor or orchitis, although rare in this age group, is often the diagnosis. Cystic dysplasia of the testis, a rare condition frequently associated with renal anomalies, is another possible differential diagnosis. Herein the authors describe a case in which cystic dysplasia of the testis is associated with ipsilateral renal agenesis as well as high anorectal anomalies.

Pancreas divisum and stenosis of the major and minor papillae in an 8-year-old girl: treatment by dual sphincteroplasty.

Year 1998
O'Rourke RW. Harrison MR.
Department of Pediatric Surgery, University of California, San Francisco 94143-0570, USA.
Pancreas divisum is the most common congenital anomaly of the pancreas. Its relationship to the development of pancreatitis is controversial. The authors report on an 8-year-old girl who presented with recurrent bouts of acute pancreatitis and multiple failed attempts at endoscopic retrograde cholangiopancreatography (ERCP) who was referred for surgical exploration. She was found to have marked stenoses of both major and minor papillae and an intraoperative pancreaticogram consistent with pancreas divisum. She underwent sphincteroplasty of both major and minor papillae and remains symptom-free after 22 months. It is believed that in a patient with pancreatitis and pancreas divisum, or in a patient with pancreatitis and multiple failed attempts at ERCP, transduodenal exploration and intraoperative pancreaticogram are appropriate next steps in management. If pancreas divisum in association with minor papilla stenosis is found, sphincteroplasty is appropriate therapy. If major papilla stenosis is also present, we recommend sphincteroplasty of both the major and minor papillae.

Mesenteric cyst--an unusual presentation.

Year 1998
Mohanty SK. Bal RK. Maudar KK.
Department of General Surgery, Armed Forces Medical College, Pune, India.
Mesenteric and retroperitoneal cysts develop from ectopic lymphatic tissue. Most of them present as symptomless abdominal swellings or are found coincidentally during abdominal operations. One third of patients may present with acute abdominal pain, and few may present with chronic abdominal pain. However, mesenteric cyst presenting as inguinal hernia is extremely rare. Only four cases have been reported in the English-language literature. The present case is the fifth, and only the second from this subcontinent. The treatment of choice is complete surgical excision.

Hereditary multiple atresias of the gastrointestinal tract: report of a case and review of the literature.

Year 1998
Lambrecht W. Kluth D.
Department of Pediatric Surgery, University Hospital, Hamburg, Germany.
Hereditary multiple atresia of the gastrointestinal tract is an extremely rare subgroup of intestinal atresia. The aim of this study was to report a new case, to review the literature, and to describe the unique features of this malformation. A computer-generated list of articles on this subject was obtained, and all articles relative to this malformation were reviewed. Thirty-five other well-documented cases were found in the literature. Hereditary multiple atresias have several unique features: (1) the abdominal x-ray shows signs of gastric or duodenal atresia combined with typical large rounded or oval homogeneous calcifications in the abdominal cavity, (2) intraoperatively widespread atresias (exclusively type I and II) extending mostly from stomach to rectum are found, (3) cystic dilatation of the bile ducts can be present in cases with both complete pyloric and duodenal or proximal jejunal atresia, (4) the pathogenesis is still speculative; a combined immunodeficiency should be excluded, and (5) a fatal outcome is the rule.

Prenatal magnetic resonance imaging enhances fetal diagnosis.

Year 1998
Quinn TM. Hubbard AM. Adzick NS.
Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, 19104, USA.
BACKGROUND: Ultrasound (US) evaluation of some fetal anomalies provides limited information. Anatomic details that affect prognosis and selection for fetal therapy, such as liver herniation and pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) and airway patency in giant neck masses, may be difficult to delineate using conventional sonographic methods. The authors evaluated the utility of prenatal magnetic resonance imaging (MRI) with new ultrafast imaging sequences in the diagnosis and management of fetal anomalies. METHODS: From April 1996 to April 1997 45 MRI scans were performed in 31 pregnant women with an US diagnosis of a fetal anomaly. The US diagnoses included CDH, giant neck masses, lung masses, abdominal and pelvic abnormalities, twin anomalies, and central nervous system (CNS) anomalies. The fetuses ranged in age from 18 to 39 weeks' gestation (mean, 28.7 weeks). Using a 1.5-T magnet, a variety of ultrafast imaging sequences were performed including fast gradient-echo, half-fourier single shot turbo spin-echo (Haste) and echo-planar imaging yielding images with T1 to T2 type weighting. RESULTS: With CDH, MRI demonstrated liver herniation into the chest in 11 of 14 cases. In four cases, US findings had not been definitive. In two cases of CDH detected by MRI, the primary diagnosis by US had been congenital cystic adenomatoid malformation (CCAM). With lung masses, MRI accurately distinguished between CCAM and bronchopulmonary sequestration (BPS). For giant neck masses with potential airway obstruction, MRI scans permitted differentiation of teratoma from cystic hygroma and allowed delineation of fetal airway involvement. The accurate anatomic evaluation facilitated planning for the ex utero intrapartum treatment (EXIT) procedure, a technique for securing the airway while the term fetus is still on placental support. With huge abdominal masses such as enterogenous cyst and lymphangioma, MRI scanning clarified the diagnosis. Fourteen of the 31 (45%) patients underwent fetal treatment after US and MRI evaluation. CONCLUSIONS: Prenatal MRI enhances fetal anatomic evaluation and facilitates perinatal management and family counseling. Ultrafast imaging sequence MRI is helpful to corroborate and refine US diagnoses. Fetal MRI is a valuable adjunct to US for prenatal diagnosis before fetal surgical intervention for selected life-threatening birth defects.

Are scintiscans accurate in the selection of reflux patients for pyloroplasty?

Year 1998
Johnson DG. Reid BS. Meyers RL. Fry MA. Nortmann CA. Jackson WD. Marty TL.
Division of Pediatric Surgery, Primary Children's Medical Center and University of Utah School of Medicine, Salt Lake City 84113, USA.
BACKGROUND: Gastric emptying scintiscans are currently used to select reflux patients for added pyloroplasty at the time of fundoplication. The accuracy of this scan selection approach has been assumed. If preoperative scintiscans do not reliably predict postfundoplication gastric emptying, however, the decision to add pyloroplasty to the fundoplication operation may be inappropriate and even harmful. METHODS: The authors studied 27 children prospectively before and after gastric fundoplication. Gastric emptying at 60 minutes was measured by double isotopic labeling of liquid (111In) and solid (99mTc) phases of a test meal specifically designed for label fixation. The authors' question involved the accuracy of preoperative gastric scintiscans in predicting postfundoplication delay of gastric emptying (DGE). An evaluation of pyloroplasty as an effective treatment for DGE was not part of the study design. Pyloroplasty was performed as a secondary operation in three of the study children, however, because they persisted with unrelieved symptoms of retching, fullness, and abdominal discomfort. Scintiscan-documented postfundoplication delay in gastric emptying was present in all three patients at 18, 58, and 12 weeks, respectively. Additional scintiscans were performed in these patients after pyloroplasty. RESULTS: Gastric emptying of solids at 60 minutes did not show a significant change after a gastric fundoplication operation, although the trend was in the direction of a decrease (paired t test, P= .13). Liquid emptying at 60 minutes, however, was significantly increased (paired t test, P = .01). The variation in values between patients was wide, and the correlation between pre- and postoperative study results in the same patient was poor (r2 = 0.337 for solids and r2 = 0.116 for liquids). Most unexpectedly, scintiscans after postfundoplication pyloroplasty in the three patients with persistent symptoms showed no improvement in delayed gastric emptying on repeat scintiscan 42 to 117 weeks later. CONCLUSIONS: The data suggest that preoperative scintiscan evidence for postfundoplication DGE is probably accurate for solid emptying but not for liquids, at least as measured by the double isotope methodology of our study. Preoperative scintiscans that use a liquid phase label only may be highly misleading for the prediction of postfundoplication DGE. Furthermore, pyloroplasty may not be useful as treatment even when postfundoplication delay in gastric emptying can be accurately anticipated or confirmed. A fundamental motility disorder of the gastric body seems to be more important than muscular resistance at the gastric outlet as a cause for postfundoplication DGE, and the most effective treatment approach remains unclear.

Interstitial hernia: a diagnostic dilemma in infants and children.

Year 1998
Iskit SH. Dagli TE. Kiyan G. Durakbasa CU.
Department of Pediatric Surgery, Marmara University School of Medicine, Istanbul, Turkey.
The features of the rare interstitial hernia, as seen in four children, are described. All were boys ranging in age from 7 days to 7 years. The presenting sign was swelling in the right side of the abdomen associated with undescended testis. The hernias arose at the deep inguinal ring and expanded superiorly between the internal and external oblique muscles. Repair involved a standard herniotomy and orchidopexy through an oblique inguinal incision-except in one patient with a vanishing testis. The importance of being aware of this rare type of hernia is stressed.

Direct bypassing of extrahepatic portal venous obstruction in children: a new technique for combined hepatic portal revascularization and treatment of extrahepatic portal hypertension.

Year 1998
de Ville de Goyet J. Alberti D. Clapuyt P. Falchetti D. Rigamonti V. Bax NM. Otte JB. Sokal EM.
St Luc University Clinics, Louvain Medical School in Brussels, Belgium.
BACKGROUND: Decompression of extrahepatic portal hypertension by directly bypassing the thrombosed portal vein has never been reported in cases of children with idiopathic (or neonatal) portal vein obstruction and cavernoma. METHODS: Seven children (15 years or younger) with portal vein obstruction requiring surgical decompression (urgently in two cases), and in whom preoperative Doppler had shown that the intrahepatic portal branches were hypoplastic but free of thrombus, were included in a pilot study. The cavernoma was bypassed by interposing a venous jugular autograft between the superior mesenteric vein and the distal portion of the left portal vein. Patients received follow-up using routine clinical parameters, upper gastrointestinal endoscopy, and Doppler ultrasound. RESULTS: The mesenterico-portal bypass restored a direct (physiological) hepatopetal portal flow. The operation resulted in effective portal decompression as demonstrated by decrease of the pressure gradient, rapid regression of clinical signs of portal hypertension, and definitive control of bleeding. CONCLUSIONS:This study shows that direct bypassing of portal cavernoma is possible and results in effective portal decompression. Restoration of the hepatic portal flow is a major advantage compared with conventional surgical shunting procedures. This new technique is potentially applicable to two thirds of children with portal vein thrombosis and should be considered when shunting procedures are indicated.

Surgical treatment of diaphragmatic eventration caused by phrenic nerve injury in the newborn.

Year 1998
de Vries TS. Koens BL. Vos A.
Pediatric Surgical Center, Amsterdam, The Netherlands.
BACKGROUND: Phrenic nerve palsy in infants and young children usually results from birth injury or iatrogenic damage. The newborn almost invariably presents with severe respiratory distress, diaphragmatic elevation, and paradoxical movement at the affected side. METHODS/RESULTS: In this retrospective analysis a group of 23 patients below the age of 1 year with an obstetric or postoperative phrenic nerve injury was studied and compared with cases in the literature. All patients were admitted between 1986 and 1997 to the Pediatric Surgical Center, Amsterdam. Thirteen of 18 patients with an obstetric phrenic nerve injury underwent plication of the diaphragm after an average observation period of 100 days. In the remaining five children with an obstetric phrenic nerve injury, spontaneous recovery appeared within 1 month. Only one of five patients with a phrenic nerve palsy after a cardiac surgical procedure underwent plication of the diaphragm. Fifteen of the 34 patients described in the literature underwent plication of the diaphragm after an average of 54 days. CONCLUSIONS: If after 1 month no spontaneous recovery of the diaphragmatic paralysis caused by a phrenic nerve injury occurs, plication of the diaphragm is indicated. This operation proved to be successful for relief of symptomatic phrenic nerve injury in all cases. If the condition of the patient clinically deteriorates during this first month of life, the patient should be operated on immediately.

A case of resection under the IVC-atrial venovenous bypass of a hepatoblastoma after intraarterial chemotherapy.

Year 1998
Uotani H. Yamashita Y. Masuko Y. Shimoda M. Murakami A. Sakamoto T. Tazawa K. Tsukada K.
Department of Surgery, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Japan.
An 11-month-old infant with a huge hepatoblastoma occupying almost the entire liver was admitted to the hospital. Serum alpha fetoprotein (AFP) level was elevated to 685,120 ng/mL. Combination chemotherapy with continuous arterial infusion of tetrahydropyranyl Adriamycin (THP-Adriamycin) and cisplatin based on the 91B1 protocol of the Japanese Study Group For Pediatric Liver Tumor (JPLT) was administered as the adjuvant chemotherapy. The tumor responded to three courses of chemotherapy and shrank in size, although venocavography showed that the inferior vena cava (IVC) was completely occluded below the entry of the hepatic veins. Right hepatic trisegmentectomy was performed with an IVC-atrial venovenous bypass that prevented massive bleeding. In this case, it was recognized that the IVC-atrial venovenous bypass was advantageous in an infant. The procedure is very simple and the blood flow obtained by the bypass was sufficient. Two weeks postoperatively, three courses of chemotherapy were initiated after the protocol. The patient remains well without signs of recurrence 39 months postoperatively, and the AFP value has remained within 10 ng/mL.

Successful arterial switch operation in a low-birth-weight neonate who had transposition of the great arteries and advanced necrotizing enterocolitis.

Year 1998
Lin YT. Teng RJ. Wang JK. Chang MH. Chen CC. Chang CI.
Department of Pediatrics, National Taiwan University Hospital, Taipei, ROC.
A low-birth-weight (LBW; 1,940 g) girl was born at the gestational age of 36 weeks without any perinatal insult. Transposition of the great arteries (TGA) with a large patent ductus arteriosus (PDA) and interatrial shunt was detected. Stage IIIB necrotizing enterocolitis (NEC) developed 46 hours after birth. She received ileostomy and drainage. Arterial switch operation was successfully performed at 23 days of age when her weight was 1,900 g. The enterostomy was successfully repaired at 104 days of age.

Chylous ascites: a manifestation of blunt abdominal trauma in an infant.

Year 1998
Beal AL. Gormley CM. Gordon DL. Ellis CM.
Department of Surgery, North Memorial Health Care, University of Minnesota, Robbinsdale, USA.
This report presents an infant in whom a unique case of chylous ascites developed after blunt abdominal trauma. Unfortunately, this case was complicated by Pseudomonas peritonitis, likely from a distant source. Our patient was treated medically and had a good overall outcome.

Isolated cavernous hemangioma of the stomach in a neonate.

Year 1998
Nagaya M. Kato J. Niimi N. Tanaka S. Akiyoshi K. Tanaka T.
Department of Pediatric Surgery, Central Hospital, Aichi Prefectural Colony, Kasugai, Japan.
This is a report of an extremely rare case of isolated cavernous hemangioma of the stomach found in a neonate. Hematemesis developed in a 7-day-old baby boy weighing 2.8 kg at birth. This was followed by melena. Endoscopic examination findings showed a large hemorrhagic mass on the lesser curvature of the stomach. During laparotomy, a large vascular tumor was identified and subtotal gastrectomy was carried out. On section, the tumor extended through the submucosa to the serosal surface. Histological diagnosis was cavernous hemangioma. The authors could collect only 10 cases of hemangioma of the stomach diagnosed in childhood from the world literature.

Elective transplant pneumonectomy.

Year 1998
DeAnda A Jr. Cahill JL. Bernstein D. Starnes VA. Reitz BA.
Department of Pediatrics, Stanford Health Services, CA, USA.
The use of a single lung transplant, modified with removal of the middle lobe of the donor right lung, has been described for a term neonate with respiratory distress secondary to right-sided congenital diaphragmatic hernia. The successful transplant allowed the patient to be successfully weaned from extracorporeal membrane oxygenation. Because of the early age of the patient at transplantation (3 weeks), it was unclear how the patient's left lung would develop, and there was uncertainty regarding the risk of life-time immunosuppression. By the age of 4 years, 10 months, she was demonstrating some failure to thrive, hypertension, and hirsutism, obvious side effects of chronic immunosuppression. The question was raised as to the potential for transplant pneumonectomy. A ventilation-perfusion scan demonstrated a decrease of right lung ventilation compared with the immediate postoperative period (27% versus 43%); right heart catheterization with balloon occlusion of the right main pulmonary artery suggested that the patient would tolerate right pneumonectomy. After discussion with the family, the patient underwent transplant pneumonectomy via a right posterolateral approach. Findings at the time of operation included mild to moderate adhesions as well as recurrence of the diaphragmatic hernia. She tolerated the procedure well and was discharged home on the fifth postoperative day with cessation of her immunosuppression. The immediate and medium-term success of this procedure suggests the potential for temporizing transplantation as a palliation to promote survival until the remaining native lung can provide sufficient ventilation.

Median sternotomy and use of a pedicled sternocleidomastoid muscle flap in the management of recurrent tracheoesophageal fistula.

Year 1998
Holland AJ. Ford WD. Guerin RL.
Department of Paediatric Surgery, Women's and Children's Hospital, Adelaide, South Australia, Australia.
Recurrent tracheoesophageal fistula may complicate primary repair of congenital tracheoesophageal fistula. Standard treatment involves repair via a right lateral thoracotomy and use of adjacent soft tissues to separate the suture lines of the fistulous openings. The authors describe an alternative approach via a median sternotomy, which improves access, reduces the operating time required to identify the recurrent fistula, and enables the use of a pedicled sternocleidomastoid muscle flap to decrease the risk of refistulization.

A case of zinc chloride ingestion.

Year 1998
Yamataka A. Pringle KC. Wyeth J.
Department of Surgery, Wellington School of Medicine, New Zealand.
Zinc chloride is a powerful corrosive agent. Reports of zinc chloride ingestion are uncommon, and there is little information about its toxicity and management. The authors report the clinical course of a 10-year-old girl who accidentally ingested an acid soldering flux solution (pH, 3.0; zinc chloride, 30% to < 60%). Systemic effects after the ingestion were unremarkable except for lethargy. Thus, chelation therapy was not considered. Severe gastric corrosion was caused by local caustic action. An antral stricture of the stomach approximately 3 weeks after the ingestion developed, and she underwent a modified Heineke-Mikulicz antropyloroplasty. Postoperatively, she made an uneventful recovery. On follow-up, although she was tolerating a normal diet, results of a barium meal showed her stomach to be totally aperistaltic. Results of a nuclear medicine study showed moderately delayed gastric emptying. Careful long-term follow-up is necessary, because there is potential risk for malignancy in the damaged stomach.

Congenital short bowel syndrome associated with appendiceal agenesis and functional intestinal obstruction.

Year 1998
Sarimurat N. Celayir S. Elicevik M. Dervisoglu S. Yeker D.
Department of Paediatric Surgery, Cerrahpasa Medical Faculty, University of Istanbul, Turkey.
Congenital short bowel with functional intestinal obstruction and absence of appendix vermiformis is a very rare condition with poor prognosis. Seventeen cases of congenital short bowel have been reported previously in the literature. The etiology is unknown. In this report, a case of congenital short bowel, combined with functional intestinal obstruction, mal-rotation, and absence of the appendix vermiformis, is presented and the pathogenesis discussed.