Kedar Mudkhedkar

Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate.

Solitary crossed renal ectopia (SCRE) is an exceedingly rare anomaly of the urinary tract. So far, only 34 cases have been reported in the literature. It usually presents after infancy. Most of these cases are diagnosed incidentally while patients are undergoing evaluation for associated genitourinary, cardiovascular, hematological or vertebral abnormalities. We report the first case of SCRE presenting in neonatal age with impaired renal function and vesico-ureteric reflux.

Gluteal flap for omphalocele repair in a case of epigastric heteropagus: A novel approach for surgical management

Epigastric heteropagus is an extremely rare congenital anomaly, in which there is asymmetrical conjoined twinning, with the parasitic twin attached to the epigastrium. A 2-day-old male, with epigastric heteropagus and omphalocele, was operated in our institution. After excision of the parasitic twin, omphalocele was covered with a gluteal skin flap available from the parasitic twin. Post-operative course was uneventful, except for infection along the edges of the skin flaps, which was managed conservatively. Only 44 cases of epigastric heteropagus twins have been reported previously in world literature. We present a novel surgical approach for the repair of the omphalocele in a case of epigastric heteropagus twins, probably the 45th case to be reported in the world literature.

Immediate and long-term outcome analysis of lipomeningomyelocele repair in asymptomatic infants in a tertiary care center.

Objective: To analyze immediate and long-term results of lipomeningomyelocele (LMM) repair in asymptomatic patients. Materials and Methods: Seventeen patients of LMM presented to Department of Paediatric Surgery over a period from 2011 to 2015 were evaluated preoperatively by magnetic resonance imaging of whole spine, and pre- and post-operative Ultrasound of kidney, ureter, bladder, and neurosonogram. Surgical procedure involved total excision of lipoma in 15 patients and near total excision in 2 patients. Division of filum terminale could be done in 15 out of 17 patients. Follow-up varied from 1 to 3.5 years (mean 1.9 years). Results: This study included 10 (58.8%) patients of lumbosacral LMM, 5 (29.4%) patients of sacral, and 2 (11.7%) patients of thoracolumbar LMM. About 13 (76.4%) patients were operated before 3 months of age, 2 (23.5%) patients were operated between 3 and 6 months, and two patients were operated between 6 and 11 months. None of the patients had bladder/bowel dysfunction preoperatively. Preoperative lower limb power was normal in all patients. Objective improvement in lower limb motor function was observed in 3 (17.6%) patients and three patients had decreased lower limb power. Two patients developed altered sensations and weakness of lower limb about 2.5-3 years after initial LMM repair. They needed repeat detethering of cord. Two patients had fecal pseudoincontinence, whereas one patient developed constipation. Bowel dysfunction was managed by rectal washouts, and oral laxatives were added if required. One (5.8%) patient of lumbosacral LMM and 1 (5.8%) patient of sacral LMM had urinary incontinence postoperatively. This was managed by clean intermittent catheterization with continuous overnight drainage. Conservative management of bladder and bowel dysfunction was effective in all patients till the last follow-up. Two patients developed hydrocephalus after LMM repair for which low-pressure ventriculoperitoneal shunt was inserted. Wound infection occurred in 1 (5.8%) patient, whereas 7 (41.1%) patients developed seroma in wound which responded to repeated aspirations under aseptic precautions. Conclusion: With total excision of lipoma and division of filum terminale satisfactory outcome for asymptomatic patients of LMM can be achieved. Authors recommend early surgery for LMM even in asymptomatic patients. Patients with residual lipoma and undivided filum terminale should be observed closely for the development of progressive neurological changes.

Passage of nasogastric tube through tracheo-esophageal fistula into stomach: A rare event

Esophageal atresia with tracheo-oesophageal fistula (TEF) occurs in 1 in 3500 live births. Anorectal malformation is found to be associated with 14% of TEF. Esophageal atresia with TEF is a congenital anomaly which classically presents as excessive frothing from the mouth and respiratory distress. Rarely gastric position of the feeding tube in a case of TEF can be obtained delaying the diagnosis of TEF. We had an uncommon situation where a nasogastric tube reached the stomach through the trachea and tracheo-esophageal fistula, leading to misdiagnosis in a case of esophageal atresia with tracheoesophageal fistula. By using a stiff rubber catheter instead of a soft feeding tube for the diagnosis of esophageal atresia and TEF, such situation can be avoided.

Posterior urethral polyp with type I posterior urethral valves: a rare association in a neonate.

Urethral polyp is a rare cause of bladder outlet obstruction, voiding dysfunction, and hematuria in the pediatric age group. Urethral polyps are rarely associated with other congenital urinary tract anomalies. In this study, we report a case of solitary posterior urethral polyp with type I posterior urethral valve in a 7-day-old neonate presented with urinary retention and deranged renal function. The polyp was diagnosed on cystoscopy. Transurethral resection of the polyp with posterior urethral valve fulguration was performed. Pathologic assessment revealed a fibroepithelial lesion, which was consistent with congenital posterior urethral polyp.

Unusual presentation of metanephric stromal tumor in a neonate

Metanephric stromal tumor (MST) of kidney is an under-reported benign stromal specific renal neoplasm with good prognosis. This tumor is to be differentiated from congenital mesoblastic nephroma and clear-cell sarcoma of the kidney. In this case report, we describe the imaging, gross, and microscopic features of MST with ischemic left kidney in a 1-month-old child and discuss treatment with relevant literature.

Palmoplantar keratoderma and pleomorphic adenoma of submandibular salivary gland: A rare association in a pediatric patient

Palmoplantar keratoderma (PPK) is a rare disorder in which dry, thick patches of skin develop on the soles of the hands and feet. Our patient, an 11-year-old boy was undergoing treatment for progressive PPK by a dermatologist since the age of 1 year. He presented to us with swelling in the left submandibular region. Incision biopsy was suggestive of pleomorphic adenoma (PA). Patient was managed with complete surgical excision of the left submandibular gland. The association of both the diffuse and punctate forms of PPK with internal neoplasia has been described by a number of reports. We report the occurrence of PA of the submandibular salivary gland in a case of diffuse PPK. To the best of our knowledge this association has never been described before.