Samir Akel

Double zonal aganglionosis in long segment Hirschsprung's disease with a “skip area” in transverse colon

The case of an infant with Hirschsprungs disease involving the rectum, entire colon, cecum, appendix, terminal ileum and sparing the midtransverse colon is described. The purpose of the report is to submit an additional illustration of this rarely documented aspect of intestinal aganglionosis.

One-lung ventilation of the newborn with tracheoesophageal fistula.

Intermittent positive pressure ventilation (IPPV) of the newborn having esophageal atresia associated with distal tracheoesophageal fistula (TEF) may result in marked distention of the gastrointestinal tract, which can be complicated with serious cardiovascular embarrassment (1). The present report shows that, in two newborns having surgical repair of TEF, selective bronchial intubation can be safely used to achieve one-lung ventilation (OLV) while the sidewall of the tube serves to occlude the fistula, thereby retarding gas insufflation into the stomach.

Limb salvage surgery for children and adolescents with malignant bone tumors in a developing country

To describe the 5‐year experience of a multidisciplinary limb salvage program for children and adolescents with malignant bone tumors in Lebanon.

Intestinal polypoid arteriovenous malformation: unusual presentation in a child and review of the literature.

Aim:  We report a jejuno‐jejunal intussusception with a polypoid arteriovenous malformation as a lead point in a 12‐year‐old boy, presenting with lower abdominal pain and non‐bloody non‐projectile vomiting.

Respiratory failure in a former preterm infant following high spinal anesthesia with bupivacaine and clonidine.

children. The CPB flows and the cannulae for its conduct were based on BSA computed by height and weight based Mosteller formula and found appropriate. CPB-flows computation based on age nomogram is almost double as compared with the one based on Mosteller formula. Praveen Kumar Neema Manikandan Sethuraman Arun Vijayakumar Ramesh Chandra Rathod Department of Anaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, 695011 Kerala, India (email neema@sctimst.ac.in, and praveenneema@yahoo.co.in)

Primary malignant peripheral nerve sheath tumor of the lung in a young child without neurofibromatosis type 1

Malignant peripheral nerve sheath tumors (MPNST) are uncommon in children and almost half of the cases occur in patients with neurofibromatosis 1 (NF1). We report a child with a primary MPNST of the lung without NF1. MPNST of the lung has similar clinical and radiologic characteristics as pleuropulmonary blastoma. We suggest to include MPNST of the lung in the differential diagnosis of intrapulmonary masses in children. Pediatr Blood Cancer 2006; 47:636–638.

Isolated renal haemangioma in children: presentation and management

A 28-day-old baby boy presented with progressive jaundice and pallor of 3 weeks’ duration. On examination he had moderate abdominal distension with a large rightsided firm immobile abdominal mass reaching the right iliac fossa. Laboratory studies showed severe anaemia (haemoglobin 52 mg/L), thrombocytopenia (platelet count 26 000/ μ L) and unconjugated hyperbilirubinaemia (total/direct bilirubin 193/7mg/L); Coombs test was negative. CT of the abdomen revealed a 10 × 7.5 × 5.5 cm heterogeneous mass arising from the right kidney, with areas of increased density suggestive of acute haemorrhage (Fig. 1). This large tumour was consistent with Wilms’ tumour although mesoblastic nephroma was another possibility. The patient underwent emergency right nephrectomy. The kidney contained a 6.5 × 6.0 × 4.5 cm mass, the inner edge of which abutted the renal pelvis without invading it. The outer aspect of the mass compressed the surrounding renal cortex. The overlying renal capsule was focally ruptured and there was extensive haemorrhage into the perirenal fat. The cut surface of the mass was dark brown and friable, and the bulk of it appeared to be composed of clotted blood (Fig. 2). Microscopy of the mass revealed a cavernous haemangioma with extensive intratumoral and perirenal haemorrhage. Identifiable features of haemangioma were limited to a thin rim along the interface between the mass and normal renal tissue. They consisted of anastomosing vascular spaces separated by a scant connective tissue stroma, as well as large cystic spaces containing clotted blood (Fig. 3a). Entrapped normal tubules were occasionally seen within the stroma (Fig. 3b). The cells lining the vascular spaces were benign and expressed endothelial markers (factor VIII and CD34). Extensive sampling of the mass showed no evidence of malignancy. The patient had an uneventful postoperative course, and normal haematological and biochemical profiles upon discharge from hospital. Fig. 1. CT of the abdomen showing a large heterogeneous mass arising from the upper pole of the right kidney and displacing the rest of the renal parenchyma medially, associated with areas suggestive of acute haemorrhage and necrosis.

Traumatic injuries of the alimentary tract in children

Abstract Traumatic alimentary tract (AT) perforations in children secondary to instrumentation, though rare, can occur at any age, especially in neonates and young infants. Awareness of such a possibility is essential for prompt management to be initiated. Over a 10-year period (1986–1995), eight pediatric patients with AT perforations secondary to instrumentation were managed at the American University of Beirut Medical Center. Five had upper AT injuries that included three hypopharyngeal, one esophageal, and one gastric lesion. The remaining three patients had lower AT injuries involving the transverse colon in one and the rectum in two. All the patients but two (one with a hypopharyngeal and one with an esophageal injury) were managed surgically and all survived. These rare injuries are discussed in addition to the preventive measures to be taken.

Gallstone pancreatitis in children: atypical presentation and review

Pancreatitis in children is less common than in adults and is mostly related to trauma, infection and anatomical anomalies. Gallstone pancreatitis is an even rarer entity in infants and children. We present an unusual case of gallstone pancreatitis in a 17- month-old girl who presented with jaundice of 1 month duration. The laboratory studies showed conjugated hyperbilirubinaemia with associated acute pancreatitis and pseudo-hyponatraemia secondary to the markedly elevated lipid profile. Further evaluations revealed obstruction at the Ampulla of Vater with no evidence of a choledochal cyst and no mass lesion seen in the pancreas. The patient initially underwent urgent decompressive cholecystostomy with intraoperative cholecystography. When the biochemical parameters improved, the patient underwent formal cholecystectomy with common bile duct exploration, extraction of multiple impacted stones in the ampullary region and Fogarty balloon sphincterotomy. The post-operative course was uneventful. Conclusion:Acute gallstone pancreatitis in children may present as jaundice or abdominal pain. Recognition, early diagnosis and surgical intervention are the mainstay for a good outcome.

Outcome of Ewing sarcoma in a multidisciplinary setting in Lebanon

Treatment of Ewing sarcoma (ES) necessitates coordinated multi‐disciplinary care. We analyzed outcome for 39 patients treated at a single institution in Lebanon, a developing country with available multidisciplinary treatment modalities, where financial barriers to care are overcome by a fundraising system. Median follow‐up was 58 months. Five‐year overall and event‐free survival were 76% and 58%, respectively, for localized disease, and 40% and 38%, respectively, for metastatic disease. We conclude that, in a country with emerging economy, by following international protocols and ensuring availability of needed resources, outcome of patients with ES is similar to that in developed countries. Pediatr Blood Cancer 2014; 61:1472–1475.