Sudden Appearance of a Palpable Chest Wall Mass Secondary to Macrocystic Lymphatic Malformation: A Case Report.

Chest wall lymphatic malformations are rare and can pose a diagnostic dilemma, particularly if they present abruptly. This case report describes a 15-month-old male toddler presenting with a left lateral chest mass. Histopathology of the surgically excised mass confirmed the diagnosis of a macrocystic lymphatic malformation. Furthermore, there was no recurrence of the lesion in the two-year follow-up period.

Perioperative management of Wilms' tumor with intracardiac extension: Report of two cases with review of literature.

Wilms' tumor (WT) is one of the solid tumor that affects children. It involves the kidney and may extend to the lungs and liver. WT conquers the adjacent blood vessels such as renal veins and inferior vena cava (IVC); its extension to IVC and right-sided heart chambers is unusual. Furthermore, when the tumor extends to heart chambers, its surgical management becomes challenging and demands multidisciplinary medical and surgical specialties including pediatric cardiac surgery. In this report, we discuss the surgical management and perioperative treatment of two unusual cases of WT with IVC and intracardiac extension.

Outcomes of transanal endorectal pull-through for rectal atresia.

Rectal atresia is a rare anorectal malformation, and it has been reported to represent 1%-2% of all anorectal malformations. We report three newborns who were admitted to the neonatal intensive care unit for abdominal distention, bilious vomiting and failure to pass meconium. The external anus and genitalia were normal and well formed. Digital rectal examination showed a blind-ending anal canal. All three infants were initially managed with diverting colostomy and then transanal resection of the rectal atresia with primary anastomosis, followed by colostomy closure. All patients eventually developed normal bowel habits and gained complete bowel control at 3-5 years of age, with mild constipation managed with laxatives. Contrast enema in a newborn with distal bowel obstruction is helpful to delineate the anatomy to show the gaps and to facilitate the procedure. In conclusion, transanal endorectal pull-through is a feasible and safe procedure with satisfactory clinical outcomes.

Hepatobiliary anomalies in conjoined twins.

INTRODUCTION: Conjoined twinning is an extremely rare anomaly. Very few diagrammatic descriptions are provided for the various hepatobiliary anomalies seen in these twins. We aimed to review our experience with the various subtypes of hepatobiliary anomalies and their association with the inability to separate as well as provide diagrammatic descriptions of these anomalies. METHODS: We retrospectively reviewed our experiences within separating twins. We reviewed patterns of hepatobiliary anomalies and the required investigations and intraoperative workups. RESULTS: Of the 60 cases we evaluated, 28 were successfully separated. The reasons for nonseparation were possession of: a single heart, major communicating hearts, or major chromosomal anomalies. The liver was involved in 17 cases (60.7%) in the operative group and 23 cases (71.8%) in the nonoperative group. All cases had a computed tomographic scan and ultrasound as preoperative workup. Only 2 cases required a magnetic resonance cholangiopancreatography for preoperative evaluation. Intraoperative ultrasound was not used, and only 3 cases required an intraoperative cholangiogram. Diagrammatic depictions of the various categories of anomalies are presented. CONCLUSION: In our experience, we did not find hepatobiliary anomalies to be the sole reason for inseparability in any of the conjoined sets. Hepatobiliary anomalies seem to be more frequent in the nonseparable group.

Delayed fistulization from esophageal replacement surgery.

We present two patients who developed delayed fistulization following esophageal replacement surgery. The first is a 13-year-old child who, at the age of 3 years, underwent a trans-mediastinal colonic esophageal replacement for a refractory corrosive injury followed by a retrosternal reverse gastric tube after an early catastrophic leak. Ten years later, he presented with a history of intermittent chest pain for 6 months. He developed a tension pneumo-pericardial tamponade caused by a fistula between gastric tube and pericardium. He recovered after sternotomy. The second was born prematurely with type C esophageal atresia and other malformations. After esophageal anastomosis, he developed a refractory stricture that was resected at 10 months. Despite a fundoplication at 4 years, the recurrent esophageal stricture required resection at 14 years, accomplished by mobilizing the stomach into the chest through a left thoracoabdominal incision. The postoperative course was complicated by a gastric leak in the chest with empyema, but the patient recovered and was able to eat. Five years later, he underwent an anterior spinal fusion to correct a worsening kyphoscoliosis. Postoperatively, he developed an ARDS picture, leakage of air through the gastrostomy, and a fatal pulmonary hemorrhage secondary to a gastro-bronchial fistula. Fistulization from esophageal replacement surgery represents a rare long-term complication that pediatric surgeons need to be aware of.

Intramuscular myxoma: a rare neck mass in a child.

Intramuscular myxoma (IM) is a true mesenchymal benign tumour which is rare in both adults and children. In children, it most commonly affects the heart, mandible or maxilla. We report a 5-year-old child with IM presenting as a posterolateral neck mass and review the literature. IM, although very rare, should be included in the differential diagnosis of neck masses in the paediatric age group.