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.

Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature.

Congenital hyperinsulinism (HI) is characterized by profound hypoglycemia caused by inappropriate insulin secretion. HI is a heterogeneous disorder with at least two histologic lesions and several implicated genes. If HI is caused by a focal lesion, elective surgery is the treatment of choice because it leads to complete recovery without diabetes. On the contrary, near-total pancreatectomy though recommended for diffuse HI, long-term risks of endocrine and exocrine deficiencies are present. Between the years 2006-2011, three patients of HI were referred to and operated by a single surgeon. The preoperative diagnosis was confirmed by recurrent hypoglycemia, inappropriately high insulin levels, and augmented glucose requirements. The medical records of all three patients were reviewed to study their clinical features, medical and surgical treatment, and postoperative outcome (short- and long-term). There were three patients in this series (male/female ratio, 1:2), all presenting in the neonatal age. All patients failed medical treatment, and radiological imaging did not reveal any pancreatic lesion. All patients underwent subtotal (80 %) pancreatectomy. Two patients had diffuse type of HI and one focal HI. One patient had transient hyperglycemia for 3 months, which needed insulin supplementation. No patient has developed recurrent hypoglycemia, malabsorption syndrome, or any neurological sequelae until the last follow-up. Doing subtotal or near-total pancreatectomy in diffuse type of HI still remains controversial as one has a higher risk of recurrent hypoglycemia, while the other has a higher rate of insulin dependent diabetes mellitus. Subtotal (80 %) pancreatectomy may be considered as the primary modality of surgical intervention in diffuse type of HI, especially when the diagnostic facilities are limited or diagnosis is not known after preliminary investigations. This minimizes the chances of postoperative diabetes mellitus, and redo surgery can always be considered if there is recurrent hypoglycemia.

Myxoid lipoblastoma.

A rapidly growing soft tissue mass in the axilla of an infant raises the suspicion of a lipoblastoma or a liposarcoma. Excisional/incisional biopsy is vital in confirming the diagnosis and hence avoiding aggressive extirpation. This case report highlights the role of histopathology and immunohistochemistry as the gold standard in differentiating a lipoblastoma from a liposarcoma. In some cases where the histopathology is inconclusive, genetic rearrangement of the PLAG1 (pleomorphic adenoma gene 1) oncogene on chromosome 8q12 helps in confirming the diagnosis of lipoblastoma.