Unusual presentation of superior mesenteric artery syndrome in a child.

Superior mesenteric artery syndrome (SMAS), also known as Wilkie's syndrome, is a rare cause of upper gastrointestinal tract obstruction. We report a case of a 10-year-old girl with persistent abdominal pain for over 3 months, who on extensive investigations was diagnosed with SMAS. She underwent a surgical procedure to bypass the obstructed portion of the intestine for relief of her symptoms.

Role of Chemoports in Children with Hematological/Solid Tumor Malignancies - Technical Implications and Complications: An Institutional Experience.

AIM: The aim of this retrospective analysis was to identify the variously related complications and to study preventive and therapeutic measures for these complications. MATERIALS AND METHODS: A total of 72 catheters were inserted in 69 patients (mean follow-up of 1140 days) from December 2002 to May 2017. Sixty-four children were diagnosed to have hematological malignancies, and five children had solid tumors. The youngest child was 2 months of age, and the oldest was 15 years, 5 months. Records were analyzed retrospectively for the age, indication, route of insertion, and postoperative complications. A protocol-based insertion and postinsertion handling by trained nursing staff/doctors were instituted, including a periodic training program for those concerned. RESULTS: Chemoport-related complications were infection in 3 (4.16%), necessitating port removal in one patient. The rest were managed by antibiotic-lock therapy. The other problems were catheter tip occlusion in 1 (1.38%) and extravasation in two patients (2.77%) leading to a sterile collection around the port chamber. An unsightly scar in 4 (5.55%) and granuloma formation at scar site in 1 (1.38%) patient were noted. CONCLUSION: Totally implantable chemoports are preferred in children with solid and hematological malignancies because of decreased pain, the rate of infection, and ability to maintain patency for the long term. Despite significant advantages over other types of central venous access, chemoports have their own complications. It was also noted that the rate of complications could be minimized by periodic training of all the personnel concerned and following protocol-based handling of ports.

Pediatric Budd-Chiari Syndrome: A Case Series.

OBJECTIVE: To study the diagnostic methods and treatment outcomes in children with Budd- Chiari syndrome. METHODS: Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management. RESULTS: 21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived. CONCLUSIONS: The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.

Role of ano rectal myomectomy in children with chronic refractory constipation.

BACKGROUND: Chronic refractory constipation (CRC) is an uncommon type of constipation. These children have persistent symptoms even after treatment with high dose laxatives, which may cause abdominal distension, vomiting, cramping and bloating. We conducted this study to assess the diagnostic and therapeutic role of anorectal myomectomy in children with CRC. MATERIALS AND METHODS: This study includes 107 patients who fit the criteria of CRC. Complete bowel preparation with polyethylene glycol solution, enemas and antibiotics was carried out before surgery in all patients. The anorectal myomectomy was carried out under general anaesthesia with the patient in the high lithotomy position. The patients were followed up from 6 months to 13 years postoperatively. The success of myomectomy was based on the daily and complete passage of stools without the need for medication or enemas. RESULTS: A total of 99 patients were included in the study, of which, 86 (86.86%) patients showed a good response to anorectal myomectomy. Of these, 32 patients had normal histology, 14 had histology suggestive of Hirschsprung's disease, 8 had hypoganglionosis, 10 had ultra-short segment Hirschsprung's disease and 22 had hypertrophic nerves with immature ganglia. Poor response was seen in 13 (13.13%) patients of whom 5 had normal histology, 5 had Hirschsprung's disease, 2 had hypoganglionosis and 1 had ultra-short segment Hirschsprung's disease. CONCLUSION: Anorectal myomectomy is an effective and technically simple procedure in selected patients with CRC for both diagnostic and therapeutic purposes.

Outcome of Biliary Atresia After Kasai's Portoenterostomy: A 15-year Experience.

BACKGROUND: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: Medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year). RESULTS: 121 patients (61.9% males) were included; 32 (26.5%) were lost to follow-up at 1 year. At last follow-up, out of the 89, 42 (47.2%) were alive, 29 (32.6%) were jaundice-free, and 47 (52.8%) had died. The native liver survival rate at last follow up was 43.8%. 42 (47.2%) patients had complete clearance of jaundice at 3 months post-procedure. Jaundice-clearance rate was significantly high in patients alive (83.3% vs 16.7%, P<0.001)) as compared to those who died later. CONCLUSION: Jaundice clearance at 3 months post surgery is a good early indicator of long term success.