Krushnakumar Kesan

Closure of Large Meningomyelocele Wound Defects with Subcutaneous Based Pedicle Flap with Bilateral V-Y Advancement: Our Experience and Review of Literature

BACKGROUND Meningomyelocele is a defect of the spinal cord, vertebrae, and the overlying skin and is the most common form of open spinal dysraphism. Rapid closure of the back defect in the early postnatal period is mandatory to reduce the frequency of infection-related complications of the central nervous system. Majority of the cases present with small defects, which can be closed primarily, with or without subcutaneous dissection. However, direct closure is not possible in 25% of the cases. Different types of local flaps (skin or muscle flaps) are widely used for covering the skin defects; and with varying results. PATIENTS AND METHODS A prospective nonrandomized study was conducted in the department of pediatric surgery at a tertiary hospital, from September 2007 to October 2011. Overall 35 patients with large meningomyelocele defects that could not be closed primarily were included in the study. All patients were treated using subcutaneous tissue based pedicle flap with bilateral V-Y advancement. RESULTS There were 27 neonates, 7 infants, and 1 child, with a male:female ratio of 1.19:1. There were 3 thoracolumbar, 14 lumbar, 14 lumbosacral, 3 sacral, and 1 multiple meningomyelocele defects with an average size of 8.5 cm (range 6.5-11 cm). Average intraoperative blood loss was 8 mL (range 6-10.5 mL). Average operative time which included flap reconstruction time, after closure of dura, was 38.6 min. Total seven patients had wound complications viz. fat necrosis (n = 2), flap necrosis (n = 2), hematoma (n = 1), cerebrospinal fluid leak followed by wound dehiscence (n = 1), wound infection which led to meningitis (n = 1). Average healing time for flap repair was 7.52 days. Overall 80% (n = 28) of the patients had good flap texture and contour with satisfactory cosmesis. CONCLUSION Closure of large meningomyelocele wound defects with subcutaneous based pedicle flap with bilateral V-Y advancement is an effective technique. The main advantages of this technique are its simplicity, short operative time, good tolerance, early healing, and good cosmetic outcome with an excellent flap texture and contour match with minimal complications.

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.

Intra-abdominal cystic lymphangiomas in children: A case series

Background: Lymphangiomas are hamartomas of lymphatic vessels commonly arising in the head, neck, and axilla. Intra-abdominal cystic lymphangiomas are rare and are located in the retroperitoneum, the mesentery, omentum, or visceral organs. Radiological imaging in the form of ultrasound and computed tomography (CT) scan helps in preoperative diagnosis. The objective of this study is to present the management of children with intra-abdominal cystic lymphangioma in our institution. Materials and Methods: Six cases of intra-abdominal cystic lymphangiomas comprising three males and three females, which presented over 15 months, were studied. Clinical presentation, location, mode of surgical intervention, and outcome were studied. Results: The site of origin was retroperitoneum, omentum, and the mesentery, with two cases of each. Age of presentation ranged from 5 days to 7 years. Abdominal lump was the most common presenting feature. All the patients underwent complete surgical resection - four had open excision, one had a laparoscopy converted to open excision, and one had a complete laparoscopic excision. Postoperative recovery was uneventful in all of them, with no recurrence at 6 months follow-up. Conclusion: Intra-abdominal cystic lymphangiomas in children are usually symptomatic. CT scan is more accurate than ultrasound to diagnose the lesion. Complete excision of the lesion with or without intestinal resection provides symptomatic relief and prevents recurrence.

Conservative management of type 2 gallbladder perforation in a child

Gallbladder perforation (GBP) is a rare but serious complication of cholecystitis and needs to be managed promptly. Acalculus cholecystitis leading to GBP is frequently associated with enteric fever and found in critically ill patients, and a surgical approach is not always feasible in such patients. Use of percutaneous tube cholecystostomy (PTC) in such patients is a known entity but it is usually followed by interval cholecystectomy. Here we report a case of perforated gallbladder in a child managed conservatively and successfully with PTC as the definitive treatment wherein cholecystectomy was avoided. The functionality of the gallbladder was confirmed by a Tc99m-HIDA scan.

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.

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.

Bilateral pharyngeal apparatus (branchial) sinuses in a set of identical twins — a case report

Defective closure of the pharyngeal apparatus during the second week of gestation results in pharyngeal arch anomalies. Although pharyngeal apparatus (branchial) anomalies are frequently seen, bilateral cases are rare (only 2% to 3%). Bilaterality has a familial predisposition. Our patients, a set of identical twins, presented with bilateral discharging cervical sinuses noticed at 3 months of age. Physical examination was suggestive of bilateral pharyngeal apparatus (branchial) sinuses. Both patients were managed with surgical excision in the same operative sitting. To the best of our knowledge, ours is the first case of bilateral pharyngeal apparatus (branchial) sinuses in a set of identical twins.

Delayed primary presentation of a male child with high anorectal malformation: A case report and review of relevant literature

Anorectal malformation (ARM), one of the most common congenital anomalies, may present with a wide spectrum of defects. Although, female ARM and male children with low ARM may present late, almost all male patients with high ARM present within first few days of life. We present a rare case of a male child with a high ARM with a wide recto-prostatic urethral fistula who presented at one year of age. He had an associated left radial club hand deformity. He underwent 3 staged repair viz. right transverse colostomy, posterior sagittal anorectoplasty and stoma closure. Our patient was wrongly informed to undergo surgical intervention at one year of age by a village doctor. The parents were also ignorant about the pathology. This case highlights the requirement for improvement in not only literacy, but also basic health care, awareness of the general population and the medical fraternity about common congenital anomalies, and an efficient referral system to a tertiary care center, in India.