Balloon dilatation of oesophageal strictures in children.
Sandgren K. Malmfors G.
Department of Paediatric Surgery, University Hospital, Lund, Sweden.
During the last ten years balloon dilatation has become increasingly frequent in the therapy of oesophageal strictures, both for diagnosis and treatment. From 1983 to 1994, balloon catheterization was performed in 36 children (oesophageal atresia 28, tracheo-oesophageal fistula 3, congenital stenosis 1, acquired oesophageal stricture subsequent to gastro-oesophageal reflux 1, to caustic ingestion 3). Age at treatment varied from 2 weeks to 15 years. Thirty-nine (3 double) strictures were dilated a total of 171 times. Balloon dilatation was successful in 31 cases (79%). In two children therapy was changed to conventional bouginage and six strictures were resected. Advantages of the method may include that forces are exerted radially and that the procedure may be performed under better control since fluoroscopy is used.
Ultrasonographic triangular cord: the most definitive finding for noninvasive diagnosis of extrahepatic biliary atresia.
Choi SO. Park WH. Lee HJ.
Department of Pediatric Surgery, Keimyung University Dongsan Medical Center, Taegu, Korea.
Early diagnosis of extrahepatic biliary atresia (EHBA) is very important for a successful bile drainage procedure. An urgent and extensive investigation is warranted to make a differential diagnosis of EHBA from other cholestatic disease, especially neonatal hepatitis (NH). The diagnosis may be made by laparotomy with cholangiogram and liver biopsy but these procedure are invasive. Because a cone-shaped fibrous tissue was always found at the porta hepatis during Kasai's procedure and that type of fibrous tissue has never been seen in other types of cholestatic jaundice, the authors have used ultrasonography (US) to detect fibrous remnants at the porta hepatis in infants clinically suspected of having EHBA and identified a triangular- or tubular-shaped echogenic density in the bifurcation of the portal vein at the porta hepatis in EHBA and called it an ultrasonographic "triangular cord (TC)". The authors determined that the presence of TC denoted the EHBA. On the other hand, nonvisualization of TC was interpreted as consistent with NH. In the present study, the authors reviewed ultrasonographic examination and hepatobiliary scintigraphy in a series of 41 infants suspected of having EHBA or NH. The TC was identified in thirteen infants. In twelve of thirteen infants with TC found by US, the diagnosis of EHBA was confirmed at the time of Kasai's Roux-en-Y hepatoportojejunostomy operation. The remaining one died at 15 months of age without having treatment. TC was not visualized in 28 infants. Twenty-seven of 28 infants with absent TC improved clinically through medical treatment for NH. The other, diagnosed to have NH by needle and wedge liver biopsies, eventually showed a TC on follow-up US examination performed 40 days after the initial examinations. Moreover, follow-up percutaneous liver biopsy specimen showed typical findings of biliary atresia with bile duct proliferation and portal fibrosis. The patient underwent a Kasai hepatoportoenterostomy. On review of the hepatobiliary scintigraphy, all 13 infants with TC had no intestinal excretion of isotope. Thirteen of 28 infants (46%) without TC also had no intestinal excretion of isotope in the 24hour follow-up but all of them were confirmed to have NH by percutaneous liver biopsy and their subsequent clinical course confirmed NH with the detection of bile in their stool except one. On the basis of these results, the authors conclude that ultrasonographic TC is a very specific finding representing the fibrous cone at the porta hepatis and is a quick, simple, and definitive tool in the noninvasive diagnosis of EHBA. If TC is visualized, no further studies are necessary and exploratory-laparotomy can be done. If TC is not visualized, hepatobiliary scintigraphy is recommended to demonstrate bile duct patency. Percutaneous liver biopsy is only required if neither TC nor intestinal excretion of isotope is seen. However, early exploration or close US follow-up study is recommended in any patient suspected of having EHBA clinically, even if liver biopsy confirms the presence of NH.
Thirty-eight years experience of malignant hepatic tumors in infants and childhood.
Okada A. Fukuzawa M. Oue T. Kohmoto Y. Kusafuka T. Fukui Y. Kamata S. Imura K.
The Department of Pediatric Surgery, Osaka University Medical School, Suita, Japan.
A description is given of therapeutic experiences with 39 cases of malignant liver tumors in infancy and childhood during the past 38 years. Of these patients, 9 not undergoing hepatic resection all died, while 18 (60%) of 30 patients treated by hepatic resection survived. When only patients with hepatoblastoma are considered, 14 of 24 patients are alive, although 3 of them had local recurrence and had lung metastasis. Among patients with other types of liver tumor, those with hepatocellular carcinomas (2 cases) and vascular neoplasms all died in a short period of time, whereas 2 with yolk sac tumor and one with metabolic pancreatic tumor are alive despite of tumor recurrence. In summary, the results of surgical treatment of malignant liver tumors in infancy and childhood, which formerly were poor, have been improved remarkably, which we owe mainly to: 1) advances in diagnostic imaging techniques (e.g., angiography, ultrasonography, CT and MRI) permitting early diagnosis, localization of tumor, visualization of the coursing of major vessels (particularly, hepatic artery & vein) and more accurate definition of resectability, 2) technical improvement of hepatic resection and 3) progress of chemotherapy mainly with cisplatin and adriamycin.
Spontaneous perforation of choledochal cyst: a study of 13 cases.
Ando K. Miyano T. Kohno S. Takamizawa S. Lane G.
Department of Pediatric Surgery, Juntendo University, School of Medicine, Shizuoka Children's Hospital Tokyo, Japan.
Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.
Meckels diverticulum and laparoscopy of children. Whats new?
Valla JS. Steyaert H. Leculee R. Pebeyre B. Jordana F.
Fondation Lenval, Nice, France.
Laparoscopy has not changed the diagnostic approach in Meckel's diverticulum (MD). Preoperative echography and scintigraphy are still indicated in case of symptomatic diverticulum. During each submesocolic laparoscopy we must meticulously look for Meckel's diverticulum. There are two surgical procedures for MD resection: 1. Short intestinal resection after exteriorization (celio-assisted surgery) is advocated in young patients or in complicated MD. 2. Laparoscopic resection by the Endo GIA stapler is advocated in older patients and latent MD with a narrow base.
A gastroschisis-like abdominal wall defect in the left hypochondrium. Case report and literature review.
Melikoglu M. Karaguzel G. Ogus M. Aslan A. Inan M.
Department of Pediatric Surgery, Akdeniz University School of Medicine, Antalya, Turkey.
Congenital abdominal wall defects are exceedingly rare on the left side. The presented patient had an upper abdominal wall defect located just lateral to the left rectus muscle. Additionally, upper parts of the abdominal flat muscles were defective on that side. Because no report was found in the literature about the defect described here, both its terminological and embryological backgrounds are discussed.
Abdominal situs inversus with congenital duodenal stenosis: rare association.
Akel S. Halabi J. Shawis R.
American University of Beirut Medical Center, Lebanon.
Abdominal situs inversus carries a significant mortality because the majority of cases (95 %) have associated cardiac and splenic defects. A review of all cases reported in the English literature confirms in addition the presence of significant gastro-intestinal pathologies, mainly annular pancreas, midgut volvulus and duodenal atresia. We report on an additional case of abdominal situs inversus without cardiac or splenic abnormalities, who had partial duodenal obstruction secondary to a mucosal diaphragm. This seems to be the thirteenth case associated with duodenal obstruction and the sixth case secondary to a mucosal duodenal diaphragm, to date, in this setting.
Spleno-caval shunt in children using internal jugular vein graft: a case report.
Yandza T. Mayer S. Dubois J. Blanchard H.
Department of Surgery, Hopital Sainte-Justine, Montreal, Quebec, Canada.
Several techniques for decompression of portal hypertension have been described. The spleno-caval shunt is an easy and safe technique described in adult patients. However, the procedures described in adults are not adapted to children. We report an adaptation of this technique in a child, using the internal jugular vein as an interposition graft for relief of portal hypertension.
A critique of systemic steroids in the management of caustic esophageal burns in children.
Ulman I. Mutaf O.
Department of Pediatric Surgery, Ege University Faculty of Medicine, Izmir, Turkey.
Five hundred and nineteen NaOH ingestion cases were admitted to our department between 1975 and 1994, and examined via esophagoscopy in the first 48 hours. Two hundred and forty-six patients in this series were diagnosed as severe burns endoscopically. This group of 246 patients were evaluated in a retrospective study to determine whether systemic steroid treatment had any place in preventing stricture formation following severe esophageal burns. Seventy-nine patients in this group were divided into three subgroups and they received methyl prednisolone parenterally in three different regimens. The control group consisted of 167 patients admitted between the years 1986 and 1994 who did not receive any form of steroid treatment. There were no statistically significant differences between the healing rates of the subgroups and the control group (p > 0.01). The authors concluded that systemic steroid treatment has no beneficial effect on esophageal wound healing following caustic esophageal burns.
Modified Toupet wrap for gastroesophageal reflux in childhood.
Mayr J. Sauer H. Huber A. Pilhatsch A. Ratschek M.
Department of Pediatric Surgery, University of Graz, Austria.
It was the aim of our study to follow up our clientele of infants and children who had undergone a partial (posterior) Toupet wrap or modified Toupet wrap. METHODS: All 22 children who had undergone a posterior partial wrap within a 4-year period were followed up for 4.0 years (0.6-5.7 years). 36.4% of children had associated anomalies such as operated esophageal atresia, operated congenital diaphragmatic hernia, Gregg's syndrome or arthrogryposis multiplex congenita. 14% of children suffered from some form of neurological impairment. Prior to operation the 4 main examinations for detection and documentation of gastroesophageal reflux (GER) disease were carried out where possible. Five children underwent Toupet fundoplication and in 17 children a modified Toupet fundoplication with reinforcement of the wrap fixation using a Vicryl-mesh was applied. A pH-metric study and upper gastrointestinal series with reflux testing were done in all 22 children at least 6 months postoperatively. Successful control of GER was documented in 21 of 22 children (95.5%). In the remaining child a reoperation was necessary to correct recurrence of a sliding hiatal hernia and GER. In three children (13.6%) postoperative complications occurred within the first 2 weeks following fundoplication and were managed medically. Following the postoperative 24-hour pH-metric study and the upper gastrointestinal series with reflux testing, one child with normal results at these investigations was lost to follow-up. The remaining 21 children were followed up for another 10 months to 5 years. Four children (18.2%) were found to suffer from functional complications (mild dumping syndrome, retrosternal pain, vomiting during episodes of asthma, dysphagia). No gas bloat syndrome occurred within the follow-up interval and all children were able to belch and vomit. There was no mortality in our limited series. Our series indicates that the partial (posterior) Toupet wrap and the modified Toupet fundoplication are safe and effective procedures for surgical correction of GER in children which preserve the ability of infants and children to belch and vomit.
Surface umbilical pyloromyotomy.
Misra D. Mushtaq I.
Department of Paediatric Surgery, Queen Elizabeth Hospital for Children, London, UK.
BACKGROUND: Despite much initial enthusiasm, umbilical pyloromyotomy has failed to achieve widespread popularity. This mostly relates to difficulty with delivering the pyloric tumour through the relatively small incision. METHOD: A new technique for umbilical pyloromyotomy is described in which a circumumbilical skin incision with a right lateral extension is combined with transverse division of the linea alba and part of the right rectus muscle. The pyloric tumour is easily delivered to the skin surface through this generous minilaparotomy. After muscle closure the lateral skin extension is swung medially as described and incorporated within the umbilical scar. RESULTS: We report our experience of 30 cases in which this technique was successfully employed. The pyloromyotomy was done on the surface in all cases and the resulting scar was barely visible in the folds of the umbilicus.
Morphologic alterations of the enteric nervous system and deficiency of non-adrenergic non-cholinergic inhibitory innervation in neonatal necrotizing enterocolitis.
Sigge W. Wedel T. Kuhnel W. Krammer HJ.
Department of Pediatric Surgery, Medical University of Lubeck, Germany.
Although damage to intramural nervous tissue should be expected in neonatal necrotizing enterocolitis (NNEC), as the pathology of NNEC is defined by substantial destruction of the bowel wall, only a few studies have considered its implication. Thus, the aim of the study has been to examine morphological alterations of the enteric nervous system (ENS) in intestinal segments affected by NNEC. Immunohistochemical methods allowed the demonstration of both neuronal and glial elements and the assessment of an altered localization of non-adrenergic non-cholinergic (NANC) inhibitory mediators within the intramural plexuses. Intestinal segments from patients with NNEC (n = 8) and control subjects (n = 3) were obtained and submitted to immunohistochemical examination incubating with antibodies against protein gene product (PGP) 9.5, protein S-100, vasoactive intestinal polypeptide (VIP) and nitric oxide synthase (NOS). The most severe damage of nervous tissue was found within the plexus mucosus and plexus submucosus internus. The ganglionated plexuses showed a loss ot both glial and nerve cells with various stages of cell deterioration and the formation of central lesions within the myenteric ganglia. The observed neuropathologic changes correspond to the group of acquired segmental hypoganglionosis. Specimens from patients with NNEC were also characterized by an absence of immunoreactive VIP and NOS in the plexus submucosus and within the circular muscle layer. The deficiency in NANC inhibitory innervation may contribute to the formation of functional obstructions following acute NNEC. Furthermore, it is likely that the neuropathological lesions induced in early stages of NNEC may result in dysfunctional intestinal motility facilitating intraluminal bacterial overgrowth and translocation, and therefore, possibly promote the self-perpetuating pathophysiologic cycle culminating in progressive NNEC. As an additional finding, two patients with NNEC showed typical features of intestinal neuronal dysplasia (IND). The association of NNEC and IND is reviewed in the literature and possible causalties are discussed.
Neurovesical dysfunction in patients with anorectal malformations.
Emir H. Soylet Y.
Department of Pediatric Surgery, Cerrahpasa Medical Faculty, University of Istanbul, Turkey.
The aim of the study is to investigate the incidence of neurovesical dysfunction (NVD) in patients with operated anorectal malformations (ARM) and its relationship with sacral abnormalities. Between September 1992 and April 1994 cystomictiometric and electromyography investigations were performed for 33 patients operated on for anal atresia. The age of these 14 male and 19 female patients ranged between 1-17 years (mean age: 7.7). The level of ARM was supralevator in 10 patients and infralevator in 23 patients. The x-ray of 14 patients showed lumbosacral abnormalities. In urodynamic investigation, NVD was observed in 15 patients (45.4%). 4 of the patients had signs developed secondary to organic subvesical obstruction. The urodynamic results of 14 patients were in the normal range. NVD rate was found to be 70% in patients with supralevator anal atresia, 34.7% in patients with infralevator anal atresia, 64.2% in patients with sacral abnormalities and 31.5% in patients with no sacral abnormality. CONCLUSION: NVD incidence increased in patients with supralevator ARM and in patients with sacral abnormalities. However, presence of infralevator abnormality or absence of sacral abnormality does not eliminate NVD. We think that, whenever NVD is suspected in a patient with ARM, urodynamic investigations should be performed as a routine investigation of the urinary tract.
Tubular esophageal duplication associated with esophageal stenosis, pericardial aplasia, diaphragmatic hernia, ramification anomaly of lower lobe bronchus and partial pancreas anulare.
Fuchs J. Grasshoff S. Schirg E. Gluer S. Burger D.
Department of Pediatric Surgery, Hannover Medical School, Germany.
Alimentary tract duplications are rare anomalies, which are often associated with other congenital malformations. We report on a newborn with the combination of tubular esophageal duplication, congenital esophageal stenosis, ramification anomaly of lower left lobe bronchus, complete pericardial aplasia and malrotation of the bowel with a partial pancreas anulare. The diagnoses are based on esophagogram, bronchogram, bronchoscopy, gastroscopy, explorative thoracotomy and laparotomy. The treatment was a resection of the duplication per thoracotomy, esophageal myotomy with fundoplicatio.
Gastroschisis and total intestinal atresia.
Morris-Stiff G. al-Wafi A. Lari J.
University Hospital of Wales, Cardiff, UK.
Gastroschisis is an uncommon condition which is now being detected at an earlier stage due to the use of routine antenatal ultrasound scanning. The presence of intestinal atresia secondary to impairment of the vascular supply is well recognised and is associated with a poorer outcome because of the potential for loss of bowel length. We report a case of gastroschisis in which there was resorption of the bowel and closure of the abdominal wall defect. At laparotomy there was total intestinal atresia.
Sarihan H. Abes M. Yildiz K. Turgutalp H. Arslan Y.
Department of Pediatric Surgery, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey.
Two rare cases of mesenteric fibromatosis are presented. The first patient had a right upper quadrant mass and colicky abdominal pain. The tumor originated from the mesentery of the colon and it infiltrated the gallbladder, cystic duct, and the liver. The second patient had severe hematemesis and melena. The origin of the tumor, which infiltrated 3/4 part of the stomach, the gastrohepatic ligament, the first part of the duodenum, and the liver could not be determined. In the first patient, partial colectomy and cholecystectomy were performed and the liver lesion was completely excised. The second patient underwent subtotal gastrectomy and left lobectomy of the liver. The first part of the duodenum was excised. Gastrojejunostomy was performed. No recurrence was recorded in either patients during 16 and 13 months follow-up, respectively. In this report, diagnostic aids, differential diagnosis and treatment of this rare disease are discussed.