Simultaneous Presentation of Wilms' Tumor and Contralateral Ganglioneuroma in a Child: Case Report and Literature Review.

We demonstrate a 4-year-old girl who presented with progressive, asymmetrical, firm abdominal distention and was diagnosed with synchronous Wilms' tumor and left para-aortic ganglioneuroma (GN). Although synchronous tumors in the pediatric population are commonly associated with malignancy-predisposing syndromes, the patient in question was found to be otherwise healthy and had no clinical evidence nor family history of a syndrome. This case is the second one in the literature diagnosed with synchronous presentation of Wilms' tumor and GN in a previously healthy child. In addition, a GN foci presumed to be a previous metastasis of a neurogenic tumor that subsequently matured to GN was depicted within a left para-aortic lymph node. We aimed to emphasize an extremely rare synchronous occurrence of these embryonal tumors, increase the awareness of physicians, and discuss the radiologic differential diagnosis and management.

Long-term outcomes of six patients after partial internal biliary diversion for progressive familial intrahepatic cholestasis.

BACKGROUND: Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive familial intrahepatic cholestasis (PFIC). In these patients quality of life can be improved and progression of liver disease can be delayed while waiting for liver transplantation. The aim of our study was to evaluate six patients with PFIC who have undergone PIBD in long-term follow-up. METHODS: Retrospective review of the records of six patients who underwent PIBD for PFIC between 2008 and 2010 was conducted to evaluate age, growth, clinical and laboratory studies for long-term outcome. RESULTS: Serum postoperative bile acid levels were reduced from a mean 340.1µmol/L (range 851-105) preoperatively to a mean of 96.3µmol/L at postoperative fifth year. The difference between pre- and postoperative bile acid levels was statistically significant (p=0.018). AST decreased from 79.1U/L (range 43-150U/L) to 64.6U/L (range 18-172U/L), ALT decreased from 102.8U/L (range 35-270U/L) to 84.6U/L and total bilirubin decreased from 2.9µmol/L (range 0.35-6.4µmol/L) to 1.53µmol/L (range 0.3-2.4). Again, the decrease in total bilirubin levels was significant (p=0.043). Pruritus was diminished from a mean of +4 (range 4-4) preoperatively to a mean of +2 (4-0). One patient who underwent liver transplantation owing to relapsing pruritus died from postoperative sepsis in the early postoperative period at the fifth year after PBID. Five symptom-free patients have not required liver transplantation at a mean period of 6.1±0.83years (5.1-7.0years) follow-up. CONCLUSION: PBID is an effective surgical procedure in the long-term and can delay the need for liver transplantation in children with PFIC by reducing jaundice and pruritus.

Enteric Duplication Cysts in Children: A Single-Institution Series with Forty Patients in Twenty-Six Years.

PURPOSE: The purpose of the study was to evaluate our experience with enteric duplication cysts in 40 children during the past 26 years, while assessing the variability of their presentations and to propose an algorithm for surgical management. METHODS: We retrospectively analysed sex, age, clinical presentations, duplication site, surgical treatment, presence of ectopic tissue, complications, associated anomalies, and prognosis of 40 patients with gastrointestinal tract duplications who were surgically treated in our clinic. RESULTS: Overall, there was a predominance of boys (28 males, 70 %; 12 females, 30 %). The presenting symptom was vomiting in 23 patients, rectal bleeding in 11 patients, abdominal mass in 10 patients, abdominal pain in 9 patients, constipation in 6 patients, cough in 2 patients, and respiratory distress in 2 patients. In 30 patients, a complete excision of the cyst with additional segmental intestinal resection and anastomosis was performed. Cystectomy was performed in seven patients, while complete excision of the cyst with additional wedge resection was performed in two. A Wrenn procedure (mucosectomy) was performed in one patient. CONCLUSION: Due to the variability in the site of enteric duplications, a wide range of presenting symptoms can exist, which is challenging for diagnosis. In children with a diagnosis of acute abdomen, enteric duplication cysts should be considered, and these children should be further investigated for additional skeletal, urogenital, and gastrointestinal system pathologies. Surgical treatment depends on the site and type of the cyst.

Small Bowel Obstruction due to Anomalous Congenital Bands in Children.

Introduction. The aim of the study was to evaluate our children who are operated on for anomalous congenital band while increasing the awareness of this rare reason of intestinal obstruction in children which causes a diagnostic challenge. Patients and Methods. We retrospectively reviewed the records of fourteen children treated surgically for intestinal obstructions caused by anomalous congenital bands. Results. The bands were located between the following regions: the ascending colon and the mesentery of the terminal ileum in 4 patients, the jejunum and mesentery of the terminal ileum in 3 patients, the ileum and mesentery of the terminal ileum in 2 patients, the ligament of Treitz and mesentery of the jejunum in one patient, the ligament of Treitz and mesentery of the terminal ileum in one patient, duodenum and duodenum in one patient, the ileum and mesentery of the ileum in one patient, the jejunum and mesentery of the jejunum in one patient, and Meckel's diverticulum and its ileal mesentery in one patient. Band excision was adequate in all of the patients except the two who received resection anastomosis for intestinal necrosis. Conclusion. Although congenital anomalous bands are rare, they should be considered in the differential diagnosis of patients with an intestinal obstruction.

Management of a Wilms' tumor with intracardiac extension using extracorporeal circulation and deep hypothermic circulatory arrest: Case report and review of the literature.

Wilms' tumor is a relatively common malignancy among childhood cancers. However, intracardiac extension of the lesion is rare and challenging. In this report, the authors present a successful management of intracardiac extension of Wilms' tumor in a 3-year-old child using cardiopulmonary bypass and deep hypothermic circulatory arrest. The authors also reviewed the published literature on Wilms' tumor with cardiac extension, which were managed by cardiopulmonary bypass and deep hypothermic circulatory arrest to provide an optimum management plan in this challenging condition.

Assessment of 35 children with abdominal tuberculosis.

BACKGROUND/AIMS: To contribute to the diagnosis and treatment of pediatric abdominal tuberculosis cases by assessing the clinical, laboratory, and radiological features of patients who presented at our clinic and were diagnosed with abdominal tuberculosis. MATERIALS AND METHODS: Clinical, laboratory, and radiological features were reviewed retrospectively for 35 patients diagnosed with abdominal tuberculosis and followed up at the Pediatric Infectious Diseases Clinic between January 1987 and August 2012. RESULTS: The study group included 16 female (45.7%) and 19 male (54.3%) patients with an age range of 6 months to 16 years (mean: 9.77±4.36 years). Twenty-nine patients were diagnosed with tuberculosis peritonitis, five patients with intestinal tuberculosis, and one patient with pelvic tuberculosis. The most common signs and symptoms were ascites, abdominal pain, abdominal distention, weight loss, and fever. Mean duration of the complaints was 109 days (range: 10 days to 3 years). CONCLUSION: Abdominal tuberculosis is a disease with an insidious course without disease-specific clinical and laboratory signs. When the disease is suspected, laparoscopy or laparotomy could be helpful in diagnosis. Employing ultrasound and computed tomography signs, abdominal tuberculosis should be included in differential diagnoses in regions with a high incidence of tuberculosis when there is abdominal pain, weight loss, ascites, history of contact with individuals with tuberculosis, and positive tuberculin skin test when patients have not been Bacillus Calmette Guerin BCG vaccinated.

Rupture of a congenital diaphragmatic eventration in a child: report of a case.

Diaphragmatic rupture may be traumatic or may occur spontaneously. Spontaneous diaphragmatic rupture is a rare entity seen almost entirely in adults. We report here a case with spontaneous rupture of a congenital diaphragmatic evantration in a child.