Abstract Hirschsprung's disease is characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. It is managed by excision of the aganglionic segment and anastomosis of the ganglionated bowel just above the dentate line. The level of aganglionosis is determined by performing multiple seromuscular biopsies and/or full thickness biopsy on the antimesenteric border of the bowel to determine the level of pullthrough. The transition zone is described as being irregular, and hence a doughnut biopsy is recommended so that the complete circumference can be assessed. Herein, we described a child in whom there was a selective absence of ganglion cells in 30% of the circumference of the bowel along the mesenteric border for most of the transverse colon. This case defies the known concept of neural migration in an intramural and transmesenteric fashion and emphasizes the importance of a doughnut biopsy of the pulled-down segment.
Background: Solid pseudopapillary neoplasm of the pancreas (SPN) in children is rare tumor with low malignant potential. Some tumors however behave aggressively. There is very little literature on the management of these variants especially in children. We share our experience of managing large and recurrent SPN and explore the clinicopathological findings correlating to the risk of recurrence. Methods: This is a retrospective study of children who were treated for SPN between 2012 and 2022 at a tertiary care center in India. The clinicopathological features and management strategies in these children were evaluated. Results: 16 children with SPN were treated during this period (88% girls). The median age of presentation was 12 years (IQR 9-14). All children presented with abdominal pain. Computed tomography gave a definitive diagnosis in 81% of cases. The tumor predominantly involved the head of the pancreas (n=9, 56%). Eight of nine children classified as high-grade malignant had a benign course. One child had a recurrence of the tumor 4 years after the initial resection and further recurrence on chemotherapy. She required radiation therapy in addition to reoperation following which she is disease free for 77 months. The overall median follow-up was 46 months (IQR 18-72 months). Conclusion: Complete resection of the tumor provides a cure in most patients with SPN. Recurrent tumors require a multi-modality approach. Long-term survival is good. Better prognostic criteria with immunohistochemistry are required to predict the behavior of these tumors as the WHO criteria for malignancy correlate poorly with clinical outcomes in childhood SPN.
Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible.A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015.The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems.Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN.
Congenital anomalies of the kidney and urinary tract (CAKUTs) occur in 3–6 per 1000 live births, accounting for most cases of paediatric end-stage kidney disease. 1 However, the molecular basis of CAKUT and anomalies of the external genitalia is poorly understood. We, herein, describe a case with left recurrent epididymo-orchitis with a coexistent urethral duplication and an ectopic ureter with an ipsilateral non-functioning kidney, which is, to the best of our knowledge, the first reported case of its kind. This case may bring about a paradigm shift in our comprehension of the development of the two entities. Understanding the pathogenesis may help develop preventive and renal preservation strategies. The Sonic hedgehog gene and bone morphogenetic protein 4 play crucial roles in preventing anomalies of the ureters and the external genitalia. In this article, we look at possible molecular pathways that could explain the synchronicity of this rare entity.
Wireless capsule endoscopy (WCE) is one of the great milestones in the field of gastroenterology. It is versatile in image acquisition, painless and can reach parts of the small bowel not amenable to conventional endoscopy. The commonest complication with WCE is retention of the capsule. We report a case of retained capsule in a child who was being investigated for obscure gastrointestinal bleeding (OGIB). Operative intervention was required for its retrieval after two weeks of expectant management.
Solid pseudopapillary neoplasm (SPN) of the pancreas in children is a rare tumor with low malignant potential. Some tumors, however, behave aggressively. There is very little literature on managing these variants, especially in children. We share our experience of managing large and recurrent SPN and explore the clinicopathological findings correlating to the risk of recurrence.
Aim: The aim of the study is to review 7 patients with congenital esophageal stenosis treated in our institution from a diagnostic and therapeutic point of view. Materials and Methods: This is a retrospective cohort study of 7 patients treated in Christian Medical College, Vellore from 2008 to 2014. The data were analyzed with regards to age at onset of symptoms, investigative findings, age at definitive treatment, pathology, modalities of treatment, and outcomes. Results: Symptoms started within the 1 st year of life in all children with a median age of 4 months. The time of delay in diagnosis ranged from 8 months to 81 months with a mean period of 37 months. About 6 patients had a lower esophageal stenosis and 1 patient had a mid-esophageal stenosis. About 4 of the 7 children underwent endoscopic balloon dilatation from elsewhere, with 2 of the above 4 undergoing a myotomy for a wrongly diagnosed achalasia. The number of dilatations ranged from 2 to 7 with a mean of 4 dilatations. Resection of the stenotic segment with end to end anastomosis was employed in 6 of the 7 patients, and a transverse colon interpositioning was done in 1 patient. An antireflux procedure was performed in one patient. Histopathological examination of the resected specimen revealed tracheobronchial remnant in 3 patients, fibromuscular thickening in 3 patients, and membranous web in 1 patient. Postoperatively, 2 of the 7 patients had asymptomatic gastroesophageal reflux and 1 patient had postoperative stricture requiring one session of endoscopic balloon dilatation. The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or gastroesophageal reflux. There was a statistically significant increase in the weight for age after the operation. Conclusion: Congenital esophageal stenosis is rare and often confused with other causes of esophageal obstruction. Although endoscopic balloon dilatation offers an effective temporary relief, we feel that definitive surgery is curative. Long-term results following definitive surgery have been good, especially with respect to symptoms and weight gain.
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