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BACKGROUND: Hirschsprung's disease is one of the most important causes of functional intestinal obstruction in the paediatric age group. The clinical characteristics of patients with Hirschsprung's disease at a tertiary referral centre were studied over a 22-month period. Aims and Objectives: To study the demography, including Age, Sex, various clinical presentations, types, segment involved, surgery, complications and survival in patients with Hirschsprung's disease at a tertiary care referral centre in Rajasthan, India. Materials and methods: This was a prospective observational study between October 2018 and June 2020, over 22 months, at tertiary referral centre in North-Western India. Clinical data were tabulated, various presentations of the disease were studied, intra-operative ndings were noted and post-operative course was studied. Results: 82 cases of Hirschsprung disease were admitted, of which 66 were male and 16 were female. Of these, 10 presented in the neonatal age group, 25 during infancy (1 month to 1 year of age) and the rest – 47 patients presented beyond infancy. The commonest symptoms in infants and older children was chronic constipation, whereas delayed passage of meconium, abdominal distension and bilious vomiting were among the common symptoms in neonates. The patients underwent exploratory laparotomy with multiple biopsies and diversion colostomies. 43 patients underwent the denitive procedure. In all the 43 patients, Martin's modication of Duhamel's procedure, was the surgery performed. Complications included persistence of constipation, enterocolitis, and a few cases of anastomotic leak.

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A rare case of clear cell sarcoma of kidney (CCSK) especially in a neonate presented as abdominal mass in left flank. He was hospitalized, investigated and operated upon. Histopathology confirmed the diagnosis of clear cell sarcoma of the kidney. Post-operatively, chemotherapy was given according to the NWTS-5 protocol. During follow-u, the patient has shown good recovery after 7 months of surgery.

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