Pediatr Radiol

Umbilical metastasis (Sister Mary Joseph''s nodule) in a child.

Norman JT. Wagner ML. Chintagumpala M.
Department of Diagnostic Imaging, Texas Children's Hospital, 6621 Fannin Street, Houston, TX 77030, USA.
Metastatic malignancy to the umbilicus, also known as Sister Mary Joseph's nodule, is a rare condition, which to our knowledge has not been reported in a child. We present a case of an umbilical metastasis from carcinoma of the colon in a boy who was also receiving chemotherapy for mediastinal lymphoblastic lymphoma.

A lethal osteochondrodysplasia with mesomelic brachymelia, round pelvis, and congenital hepatic fibrosis: two siblings born to consanguineous parents.

Nishimura G. Nakayama M. Fuke Y. Suehara N.
Department of Radiology, Dokkyo University of School of Medicine, 880 Kitakobayashi, Mibu, Shimotsuga-gun, Tochigi-ken 321-02, Japan.
We report a hitherto unknown, lethal osteochondrodysplasia in two Japanese siblings born to consanguineous parents. The skeletal abnormalities are characterised by mesomelic brachymelia with bowed forearms, a round pelvis with shortened greater sciatic notches, an ossification defect of the pubic bones, and absence of ossification centers in the cervical vertebral bodies. The associated visceral anomalies comprised periportal fibrosis and cystic dysplasia of the intrahepatic bile ducts, pancreatic ductal ectasia, a simple renal cyst, microcephaly with multifocal laminar necrosis and ectopic gray matter, dysplastic tracheobronchial cartilage, abnormal lobulation of the lung, diaphragmatic hernia, and stenotic pulmonary valve. Thrombocytopenia was present but megakaryocytes were slightly increased in the bone marrow. The patients showed various dysmorphic features including aniridia, a long palpebral fissure, prominent nasal bridge, beaked nose, flat philtrum, low-set fleshy ears, micrognathia with submucosal cleft palate, and multiple joint contractures.

A case of perforated cystic duplication of the transverse colon.

Dutheil-Doco A. Ducou Le Pointe H. Larroquet M. Ben Lagha N. Montagne J.
Service de Radiologie, Hopital d'Enfants Armand-Trousseau, 26 avenue du Docteur Arnold-Netter, F-75 571 Paris Cedex 12, France.
A 7-year-old girl presented with an abdominal cystic mass close to the pancreas. US and CT could not identify its origin. The diagnosis of duplication of the transverse colon was made at surgery. This location is rare. The differential diagnosis in this case included a pancreatic cystadenoma, a pseudocyst or a mesenteric cyst.

Non-operative management of traumatic pancreatic pseudocysts associated with pancreatic duct laceration in children.

Lucaya J. Vazquez E. Caballero F. Chait PG. Daneman A. Wesson D.
Department of Pediatric Radiology, Hospital Universitari Materno-Infantil Vall d'Hebron, Ps. Vall d'Hebron, 119-129, E-08 035 Barcelona, Spain.
Objective. To assess the successful non-operative management in traumatic pancreatic pseudocysts (TPP) associated with duct laceration in children. Surgical therapy (cystogastrostomy or distal pancreatectomy with splenic salvage) has been classically considered the treatment of choice for those pseudocysts. Materials and methods. This report presents the clinical and imaging findings in two children with TPP and pancreatic duct disruption observed either on endoscopic retrograde cholangiopancreatography or injection via catheter drainage. Results. Both children responded to long-term cyst drainage. Conclusion. Although the experience is limited, the authors suggest that pancreatic injury associated with duct laceration can respond to non-operative management.

Hepatobiliary rhabdomyosarcoma in children: diagnostic radiology.

Roebuck DJ. Yang WT. Lam WW. Stanley P.
Chinese University of Hong Kong, Sha Tin, New Territories, Hong Kong, China.
Rhabdomyosarcoma (RMS) occurs infrequently in the liver and biliary tree. Although the radiological diagnosis may be simple when the tumour involves the extrahepatic bile ducts, no specific imaging features are known for hepatic RMS. We present four cases and discuss the role of diagnostic radiology in the management of this tumour.

Necrotizing myofasciitis: an atypical cause of acute abdomen in an immunocompromised child.

Donnelly LF. Frush DP. O'Hara SM. Bisset GS 3rd.
Department of Radiology, Section of Pediatric Radiology, Box 3808, Duke University Medical Center, Durham, NC 27710, USA.
We report the case of an immunocompromised 15-year-old boy who presented with symptoms mimicking an "acute abdomen" related to necrotizing myofasciitis of the anterior abdominal wall. CT demonstrated the abdominal wall process as the cause of the patient's symptoms and sonographically guided aspiration confirmed the diagnosis. Despite prompt diagnosis and aggressive surgical debridement, the infection continued to progress and the patient died within 24 h of presentation.

Sonography of acute appendicitis in children: 7 years experience.

Year 1998
Hahn HB. Hoepner FU. Kalle T. Macdonald EB. Prantl F. Spitzer IM. Faerber DR.
Department of Paediatric Radiology, Kinderklinik, Technischen Universitat, Munchen, Germany.
OBJECTIVE: The objective of this prospective study was to determine the accuracy in diagnosing appendicitis in children by using high-resolution graded compression sonography. MATERIALS AND METHODS: During a 7-year period, 3859 children (mean age 9.9 years) with clinically suspected appendicitis were evaluated by sonography with the graded compression technique. The ultrasound results were correlated with the intraoperative and histopathological findings or clinical outcome. RESULTS: High-resolution sonography was performed in 3859 children. Of the 610 patients who underwent a laparotomy, 494 had histologically proven acute or perforated appendicitis (prevalence 13%). In these children, sensitivity, specificity and overall accuracy of sonography were 90%, 97% and 96%, respectively. The reasons for false-negative and false-positive results are discussed. CONCLUSION: Although the use of ultrasound to diagnose acute appendicitis in children has excellent results, the decision for surgery remains a clinical one because of the continuing false-negative and false-positive results from sonography.

Torsion of an ovarian cyst mimicking enteric duplication cyst on transabdominal ultrasound: two cases.

Year 1998
Godfrey H. Abernethy L. Boothroyd A.
Department of Radiology, Royal Liverpool Children's NHS Trust, UK.
We describe two girls with abdominal cysts which demonstrated the "double wall" or "muscular rim" sign. On the basis of this, the diagnosis of duplication cyst was made in each case. At surgery, both were found to have ovarian cysts, and histology revealed prominent haemorrhage within the cyst wall. To our knowledge, there has been no previous report of a false-positive "double wall sign".

Multiple atresias with extensive intraluminal calcifications in a newborn with cystic fibrosis.

Year 1998
Oguzkurt P. Tanyel FC. Kotiloglu E. Hicsonmez A.
Department of Pediatric Surgery, Hacettepe University School of Medicine, Ankara, Turkey.
A newborn patient with cystic fibrosis and multiple intestinal atresias demonstrated intraluminal calcifications on a plain abdominal radiograph. Cystic fibrosis may be an aetiological factor for intraluminal calcifications secondary to multiple intestinal atresias.

Ultrasonographic follow-up of the healing process of medically treated hypertrophic pyloric stenosis.

Year 1998
Yamamoto A. Kino M. Sasaki T. Kobayashi Y.
Department of Pediatrics, Kansai Medical University, Osaka, Japan.
In hypertrophic pyloric stenosis (HPS), prompt pyloromyotomy is, in general, the treatment of choice. There has been no information available as to the natural history of the pyloric tumour. We present four infants with medically treated HPS who were followed by sonography to observe the anatomical changes that occur with atropine sulfate. The initial change was shortening of the pyloric canal, followed by thinning of the muscular layer as clinical symptoms improved.

Magnetic resonance cholangiography--feasibility and application in the paediatric population.

Year 1998
Chan YL. Yeung CK. Lam WW. Fok TF. Metreweli C.
Department of Diagnostic Radiology and Organ Imaging, The Chinese University of Hong Kong, Prince of Wales Hospital, Ngan Shing Street, Shatin, Hong Kong.
OBJECTIVE: To assess the feasibility of magnetic resonance cholangiography (MRC) in paediatrics. MATERIALS AND METHODS: MRC was attempted in 41 children from 1 week to 14-years. There were three groups: (1) children studied with MRI for non-biliary problems as controls; (2) infants with jaundice; and (3) older children suspected of biliary disease. The examination was successfully performed in 35 children, which included 12 children studied for non-biliary problems, 12 infants with jaundice, and 11 older children with suspected biliary disease. RESULTS: In group 1, the entire common duct was visualised in all 12 children. In group 2, successful demonstration of the common duct was achieved in two of the ten infants with subsequent confirmation of normal bile duct patency. In the last group of patients with suspected biliary disease, MRC gave good anatomical display of six choledochal cysts but failed to demonstrate the anomalous choledochopancreatic channel. It helped to confirm the diagnosis of two cases of pancreatic head cysts, and one case of Caroli's disease. CONCLUSION: MRC can be applied to the paediatric population, but its value depends on the type of problem to be evaluated. With current spatial resolution, its value in the diagnosis of biliary atresia is probably limited because it cannot reliably demonstrate the bile ducts in young infants with non-obstructive jaundice. In older children, it could provide a non-invasive anatomical display of the biliary tree and its disease. It has a potential role in addressing problematic cases encountered during ultrasonography.

Retropsoas hernia as a cause of chronic abdominal pain: CT diagnosis.

Year 1998
Benson JE. Strauch ED.
Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, 601 N. Wolfe Street, Baltimore, MD 21287, USA.
Congenital retropsoas small bowel herniation is reported as the cause of long-standing recurrent abdominal pain in a teenage girl. Knowledge of this entity is important for differential diagnosis of abdominal pain, mass, or retroperitoneal gas and fluid, and for avoiding complications of percutaneous renal interventions.

CT appearances of the Rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction.

Year 1998
West WM. Duncan ND.
Section of Radiology, University Hospital of the West Indies, Mona, Kingston 6, Jamaica, West Indies.
The CT appearances of an unusual type of trichobezoar which causes the Rapunzel syndrome are described. This syndrome is characterized by intestinal obstruction secondary to a bezoar possessing a tail which extends to or beyond the ileo-cecal valve. Most cases of bezoar are diagnosed with plain films or barium meal, but CT may be requested in patients who present with abdominal masses. The Rapunzel syndrome may be diagnosed with a combination of the CT findings and the history.

Malignant intraperitoneal neoplasms of childhood.

Year 1998
Chung CJ. Bui V. Fordham LA. Hill J. Bulas D.
Department of Radiology, CB 7510, UNC School of Medicine, Chapel Hill, NC 27599-7510, USA.
OBJECTIVE: The purpose of this paper is to review and categorize the CT appearances of primary and secondary intraperitoneal neoplasms in children. MATERIALS AND METHODS: We retrospectively reviewed the CT images of 14 cases of intraperitoneal neoplasms. They were reviewed for bowel wall thickening, mesenteric nodules, ascites, calcification, peritoneal nodules, omental caking, and contrast enhancement. RESULTS: Computed tomography images of 14 cases of intraperitoneal neoplasms were evaluated: rhabdomyosarcoma (5), lymphoma (3), neuroblastoma (2), germ cell tumor (1), mesothelioma (1), Wilms tumor (1), and hepatocellular carcinoma (1). Nodular enhancement was seen in all cases, ascites in most, significant bowel wall thickening only with lymphoma, and calcification only with germ cell tumors. Omental caking was present both with rhabdomyosarcoma and lymphoma. CONCLUSIONS: While rare in pediatrics, intraperitoneal neoplasms occur in children, and CT is useful in identifying these peritoneal neoplasms.

Fetal surgery: a brief review.

Year 1998
Farmer DL.
Department of Surgery, Fetal Treatment Program, Children's Hospital of Michigan, 3901 Beaubien Blvd., Detroit, MI 48201, USA.
Fetal therapy is a logical extension of fetal diagnosis. Fetal surgery has been performed in humans for the past 10 years. During this time, technical obstacles have been overcome and the natural history of many fetal disorders has been defined. A select group of disorders amenable to potential improvement by fetal treatment has been identified including fetal urinary tract obstruction, fetal diaphragmatic hernia, fetal congenital cystic adenomatoid malformation and fetal sacrococcygeal teratoma. The fetal surgical experience with each of these lesions is reviewed and the maternal risk of fetal surgery is discussed.

Pyloric muscle in asymptomatic infants: sonographic evaluation and discrimination from idiopathic hypertrophic pyloric stenosis.

Year 1998
Rohrschneider WK. Mittnacht H. Darge K. Troger J.
Department of Paediatric Radiology, University of Heidelberg, Im Neuenheimer Feld 153, D-69 120 Heidelberg, Germany.
OBJECTIVE: To compare the morphological and functional US appearance of the pylorus in healthy infants with those suffering from idiopathic hypertrophic pyloric stenosis (IHPS) in order to determine the pathological limits and to find out the most discriminating morphometric parameter. MATERIALS AND METHODS: The pylorus of 84 asymptomatic infants was prospectively evaluated with respect to morphology (pyloric length, pyloric diameter, muscle thickness and pyloric volume) and function (gastric peristalsis and emptying, pyloric opening and the fluid passage). Results were compared with 85 patients with proven IHPS. RESULTS: In every normal infant we observed frequent pyloric opening with passage of gastric contents and quick gastric emptying. All infants with proven IHPS presented with a permanently closed pylorus and exaggerated, retrograde gastric peristalsis. For each of the four parameters, highly significant differences (P < 0.0001) were found between the control and IHPS groups. Pathological limits were 3 mm for muscle thickness (accuracy 100 %), 15 mm for pyloric length (accuracy 94 %), 11 mm for pyloric diameter (accuracy 92 %) and 12 ml for pyloric volume (accuracy 96 %). CONCLUSIONS: Evaluation of pyloric function plays an important role in the diagnosis of IHPS. The morphometric parameters are highly accurate in differentiating IHPS from a normal pylorus, muscle thickness being the most discriminating parameter.

Gallbladder contraction in biliary atresia: a pitfall of ultrasound diagnosis.

Year 1998
Ikeda S. Sera Y. Ohshiro H. Uchino S. Akizuki M. Kondo Y.
Department of Paediatric Surgery, Kumamoto University Medical School, Honjo 1-1-1, Kumamoto 860, Japan.
In 3 (9 %) of 34 children with biliary atresia, US revealed gallbladder contraction following an oral feed, given on admission, but not with subsequent feeds. Surgery revealed a Kasai type IIIa biliary atresia with a patent communication between the gallbladder and duodenum. We propose that the bile ducts may initially have been patent, but then gradually became obliterated secondary to inflammation. These cases may explain the development of one type of biliary atresia.