Multistage surgical repair for split notochord syndrome with neuroenteric fistula: case report.

Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.

Small Bowel Hemangioma in a 2-Year-Old Female With Recurrent Anemia.

BACKGROUND: Hemangiomas of the small bowel are rare tumors that present with melena, hematochezia, or signs of anemia. Because of the rarity of gastrointestinal hemangiomas, they are not commonly considered in the differential diagnosis for pediatric patients with anemia. CASE REPORT: We present a case of small bowel hemangioma in a 2-year-old female with recurrent episodes of severe anemia. After resection of a 4.5 cm × 2 cm benign hemangioma, the patient's hemoglobin level steadily improved. The patient did not experience recurrence and has had no problems with activities of daily living. CONCLUSION: Small bowel hemangiomas can cause serious life-threatening anemia or obstruction of the small bowel and should be considered in cases of pediatric anemia unresponsive to medical management.

Robotic transaxillary thyroidectomy with gasless approach in a girl with goitre.

BACKGROUND: Robotic-assisted transaxillary thyroidectomy is a minimally invasive approach for the removal of the thyroid through the axilla. This technique eliminates a visible scar and affords excellent optics of the cervical anatomy. We sought to describe the technique and outcome for transaxillary gasless subtotal thyroidectomy in the paediatric population. METHODS: A 13 year-old female with an enlarged goitre underwent a transaxillary robot-assisted gasless subtotal thyroidectomy in an academic institution. The main outcome measures were feasibility of the robotic approach, patient and gland characteristics, operative time and complications. RESULTS: There was no conversion to laparoscopic or open surgery. The robotic docking time was 110 min and total operative time was 150 min. The patient tolerated the procedure well. Estimated blood loss was 10 ml. The patient was discharged within 24 h. There were no perioperative or postoperative complications. In addition there was no evidence of postoperative vocal cord palsy or paresis. CONCLUSIONS: This initial experience demonstrates that this technique can be a feasible, safe and effective method for subtotal thyroidectomy in the paediatric population. The use of robotic technology for endoscopic thyroid surgery could overcome the limitations of conventional endoscopic surgeries in the surgical management of thyroid disease.

Resection of a large, ruptured, undifferentiated (embryonal) sarcoma of the liver in a child: a case report and review of the literature.

BACKGROUND: Undifferentiated (embryonal) sarcoma (UES) of the liver is a malignant hepatic neoplasm accounting for 7% of pediatric hepatic tumors. Current use of multimodal therapy, including chemotherapy and surgery, has greatly improved survival. Tumor rupture is uncommon and, prior to the adjuvant use of sarcoma based chemotherapy regimens, was thought to be poor prognostic sign. CASE PRESENTATION AND DISCUSSION: A 10-year-old girl presented with acute worsening of abdominal pain while being worked up for liver mass. At surgery, she was found to have a ruptured, 20 x 15 x 5 centimeter (cm) UES, the largest reported ruptured UES to be resected primarily. The size and position of her tumor required very aggressive surgery for complete resection. She subsequently received adjuvant chemotherapy and radiation. Twenty-four months following surgery she is doing well with no evidence of disease. CONCLUSION: Despite tumor rupture, complete tumor resection gives the patient the best chance of long-term survival. Aggressive surgery is warranted if the tumor can be completely resected.