Hydrometrocolpos is a rare condition in which the uterus and the vagina are grossly distended with a retained fluid other than pus or blood. It may present during the neonatal period or later at puberty. Most cases reported earlier were stillbirths and were diagnosed only on autopsy. Antenatal diagnosis is now possible with the advent of ultrasound. An early diagnosis and speedy management is the key to survival. Many previous case reports have focused on the varied clinical presentations, multiple causes, associated syndromes and/or the radiological diagnosis of this condition. However, management options for different types of hydrometrocolpos have not yet been concisely discussed. We have reviewed the literature and tried to summarize the management options applicable to most case scenarios of hydrometrocolpos.

Major genetic aberrations proposed in the pathogenesis of ARM affect members of the Wnt, Hox (homebox) genes, Sonic hedgehog (Shh) and Gli2, Bmp4, Fgf and CDX1 signalling pathways; probable targets of future molecular gene therapy.

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A day-old neonate presented with bowel obstruction and an abdominal mass. Exploratory laparotomy revealed complete tubular midgut duplication from duodeno-jejunal junction up to terminal ileum which was communicating with the ileum distally. At the proximal end, another 5×5 cm duplication cyst was identified and excised. Postoperatively, complaints were relieved. During follow-up, 99m-Tc-pertechnetate-SPECT scan showed ectopic gastric mucosa in lower abdomen and in the right hemithorax. CECT-chest showed a 3×3 cm foregut duplication cyst, but there were no respiratory symptoms. While being planned for an elective surgery, he presented at 6 months of age in emergency with massive bleed per-rectum and shock. He underwent Wrenn procedure without any injury to the normal bowel. The thoracic foregut duplication cyst was excised later. Total midgut duplication, though benign, may present with life-threatening haemorrhage if left untreated. Presence of one such lesion warrants a search for others. Mucosal stripping is a simple and safe alternate to resection.

Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung’s disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.