Enteric duplication in children: Experience from a tertiary center in South India.

BACKGROUND: Enteric duplications (EDs) are rare aberrations of the embryonic gut. This study was undertaken to define the clinical characteristics and management challenges of this unusual entity in the Indian population. MATERIALS AND METHODS: Hospital records of 35 children with 38 ED operated between 2003 and 2014 were analyzed and followed up. RESULTS: The median age at presentation was 285 days (range 1-day to 16 years) with male preponderance (71%). Small bowel duplications were the most common (44%), and thoracoabdominal duplications were seen in 8% children compared to 2% in the literature. The median duration of symptoms was 18 days (interquartile range [IQR] 3-210 days). Associated anomalies were seen in 49% children with vertebral and spinal anomalies being the most common. Ultrasonogram (US) was done in 83% children and had a sensitivity of 55%. In the presence of a gastrointestinal bleed, Technetium(99m) pertechnetate scintigraphy scan had a positive predictive value of 80%. Thirty-five lesions were completely removed. Mucosectomy was done in two children, and one total colonic duplication was left in situ after providing adequate internal drainage. There was no postoperative mortality. The follow-up was possible in 66% children. CONCLUSIONS: EDs are uncommon and have varied, nonspecific symptoms. Thoracoabdominal duplications are more common in the Indian population. The US is a good screening tool but requires a high index of suspicion where complete excision is not possible; the provision of adequate internal drainage is an acceptable alternative. The long-term prognosis of children with ED depends on the extent of physiological disturbance due to associated anomalies.

A rare case of bilateral cystic partially differentiated nephroblastoma recurring as bilateral cystic Wilms tumour.

Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.