Sudipta Sen

The appendix as ureteral substitute: A report of 10 cases

BACKGROUND Ureteric replacement in part or in total is rarely needed in children. We present our experience in using the appendix to replace the ureter. METHODS A retrospective case note review was carried out at Sheffield Childrens Hospital (UK), Ekta Institute of Child Health (Raipur, Chhattisgarh, India) and Christian Medical College Hospital (Vellore, India) of all cases of ureteric substitution using the appendix. RESULTS Ten patients were identified, operated in 2002-2007: seven males and three females with a median age of 2.5 years (range 2.5 months to 12 years). The reasons for ureteric replacement were traumatic ureteric avulsion (n=1), congenital ureteric stenosis (n=5), non-drainage following previous pyeloplasty for pelvi-ureteric junction obstruction (n=3) and ureteric stricture following reimplantation for vesico-ureteric reflux (n=1). The appendix was used in an anti-peristaltic manner in all cases, and in one case a transureteroureterostomy was performed. At a median follow up of 16months (1-72 months), all the patients were well except one whose kidney function had deteriorated. CONCLUSIONS Total or partial replacement of the ureter using the appendix, even in the first year of life, preserved renal function in nine cases. Ureteric continuity can be successfully restored in children using the appendix.

Varied presentations of unilateral lung hypoplasia and agenesis: a report of four cases

Abstract Unilateral lung hypoplasia or agenesis can be asymptomatic or␣present with recurrent respiratory symptoms. The latter may be amenable to surgical treatment in␣selected cases. Of four children in this report, two are being managed without surgery. A third was relieved of his symptoms by pneumonectomy. The fourth presented with acute foreign-body inhalation into the healthy right main bronchus, and coexistent left pulmonary agenesis was discovered at bronchoscopy. Bronchoscopy and computed tomography were found to be the most useful investigations in management.

Abdominal muscle flap repair for large defects of the diaphragm

Repair of a large diaphragmatic defect in congenital diaphragmatic hernia (CDH) and eventration of the diaphragm (DE) is difficult, especially when this is an unexpected finding at surgery. A patch of synthetic material may not be available at short notice, especially in developing countries. We describe the repair of nine such defects by using an abdominal muscle flap comprising the transversus abdominis and internal oblique muscles based on the intercostal and subcostal vessels. Although reports of similar flap repairs have been published in the literature [1–5], these employed a subcostal incision for abdominal entry, which we believe jeopardizes the flap’s vascularity. Thus, we prefer to use an upper abdominal midline incision for abdominal entry in all cases of CDH and DE.

Results of Wilms’ tumour management in two tertiary-care hospitals in Asia

In the period 1985–1995, 87 children underwent surgery for Wilms’ tumour; 16 were lost to follow-up. Of the remaining children, 27 presented with stage I disease, 11 with stage II, 12 with stage III, 14 with stage IV, and 6 with stage V. One child was not staged. The histology was favourable Wilms’ tumour in 44, anaplastic in 12, unclassified in 8, clear-cell sarcoma in 4, and rhabdoid tumour in 3. Although a total nephrectomy was generally performed, partial renal surgery was performed for 6 bilateral and 4 unilateral tumours, the latter including 2 fused kidneys. Preoperative chemotherapy was employed with benefit in massive tumours, tumour in fused kidneys, bilateral tumours, and preoperatively diagnosed inferior vena caval tumour thrombi. Postoperative chemotherapy, employed in all cases, consisted of actinomycin D and vincristine with the addition of adriamycin in anaplastic and advanced-stage tumours. Ten children underwent second-line chemotherapy for disease unresponsive to the above management, but only 1 of these is currently free of disease. Postoperative tumour-bed radiotherapy, used in selected cases, prevented local recurrence in stage I and II disease. However, 20% of stage I and II patients not receiving radiotherapy developed tumour-bed recurrence. Twenty-three children have died and 5 with advanced disease and incomplete follow-up are presumed to be dead. Nine children are currently on treatment; 34 have successfully completed treatment, the disease-free survival in stages I–V being 81%, 75%, 42%, 14%, and 50%, respectively. Overall disease-free survival was 69% for Wilms’ tumour of favourable histology and 50% for anaplastic tumours. The 3 patients with rhabdoid tumours and 3 of 4 with clear-cell sarcomas have died. Wilms’ tumour management in the developing world is compromised by cases lost to follow-up and late presentation with massive tumours and advanced stage. Preoperative chemotherapy is advantageous in a number of cases, and postoperative radiotherapy should be deployed more frequently.

Psychosocial aspects of follow-up of children operated for intermediate anorectal malformations

PurposeTo determine the degree of stress in parents of children operated for intermediate anorectal malformations, and their quality of life (QOL) at follow-up.MethodsForty-two of the 166 children who had undergone a sacroperineal pullthrough operation for an intermediate type of anorectal malformation, between 1996 and 2005, in the department of paediatric surgery at Christian Medical College, Vellore, responded to follow-up. The psychosocial well-being of the parents and the QOL of the children were assessed by an independent observer.ResultsThe main factor which aggravated the stress and caused dissatisfaction with the final outcome was fecal soiling. Mothers bore the brunt of the care of these children, with some help from the fathers and grandparents. The QOL was also significantly affected by soiling, and improvement in soiling resulted in a dramatic improvement in the QOL.ConclusionManaging fecal soiling aggressively in the child with anorectal malformation, and providing social support to the family, are crucial for achieving a better QOL in these children and their families.

Calcifying fibrous tumour: an unusual omental lesion

Calcifying fibrous tumour (CFT) is a recently described distinct clinicopathological entity characterized by calcifying lesions usually occurring in soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum of children and adults. Most reported cases involving the peritoneum have been in adults. We present the imaging, surgical and pathology findings of CFT in a 7-year-old child who presented with an incidental finding of a large omental mass.

Gender assignment in male pseudohermaphroditism: an Indian perspective

Parents of 21 out of 30 children with male pseudohermaphroditism (MPH) opted for a male upbringing for their child and appropriate management was instituted. The phallic size in these 21 children varied from adequate to microphallus, although at least one externally visible testis was present in all cases. Nine children who were assigned a female gender were being reared as girls at home prior to medical consultation and 7 of them had a female-type vulvar outlet. This predominance of male gender assignment in MPH is in striking contrast to the Western experience. The reasons for this difference in India are discussed in the light of the Indian social and economic background.

Blastoschizomyces capitatus infection after contamination of fluids for intravenous application

Summary. A six‐month‐old child died after infusion of fluids containing Blastoschizomyces capitatus. This represents a fatality due to this organism in a human with no proven immunosuppression.

Bowel vaginoplasty in children and young women: an institutional experience with 55 patients

Introduction and hypothesisAbsence of a vagina owing to congenital Mullerian defects or other acquired causes requires reconstruction of the female genital passage. We present our experience using various bowel segments.MethodsBowel vaginoplasty was performed in 55 patients from January 2004 through May 2014 for cervicovaginal atresia (20), Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome (20), distal vaginal atresia (8), cloaca (2), cervical atresia (1), complex urogenital sinus (1), transverse vaginal septum (1), rhabdomyosarcoma of the vagina (1), and traumatic stricture of the vagina (1). The bowel segments used were sigmoid (50), ileum (2), anorectovestibular fistula ( 2), and loop rectovaginoplasty (1).ResultsThirty-nine patients who had the proximal vagina or uterus anastomosed to the bowel segment reported regular menstrual flows. Three patients are sexually active with satisfactory coital function. None of our patients developed pyometra. Five patients had neovaginal mucosal prolapse. Two patients had severe stenosis requiring excision of the neovagina. Seven patients had mild stenosis requiring dilatations in 6 patients and V-Y meatoplasty for 1 patient. One patient had a descending colon anastomotic leak requiring a diversion ileostomy.ConclusionsGenital reconstruction with bowel vaginoplasty is a highly skilled operation that provides a durable and lubricated replacement of the vagina with good outcomes. Utero-coloneovaginoplasty is a safe procedure preserving the menstrual flow in patients with a functional uterine fundus.

Colon replacement of vagina to restore menstrual function in 11 adolescent girls with vaginal or cervicovaginal agenesis.

AimCervicovaginal or vaginal agenesis with functioning endometrial tissue is rare. We report the construction of a colon conduit which is anastomosed to posterior uterine wall or upper vaginal pouch to allow menstruation.Materials and methodsWe report seven girls with cervicovaginal agenesis and four with lower vaginal agenesis (aged 12–20 years) who presented with painful cryptomenorrheoa. All the girls wanted to conserve their uterus and menstruate normally. A colon conduit was constructed for the egress of menstrual blood. The colon conduit was anastomosed to the posterior uterine wall in the seven girls with cervicovaginal agenesis and to the distended upper vaginal pouch in the four girls with vaginal agenesis. Utero-colonic neovaginal anastomosis was performed only after excising a circular portion of the posterior myometrium to prevent stenosis.ResultsThe colon conduit functioned effectively, providing an egress for regular painless menstruation. One patient had stenosis of the perineal neovaginal orifice for which dilations were done. One girl has married and reports satisfactory intercourse. The mean follow up is 2.2 years.ConclusionsThis group of patients forms a separate subgroup needing a conduit not only for sexual function but also for menstruation. However, if treated by the method described herein, they should be cautioned against pregnancy if they have cervicovaginal agenesis and against vaginal delivery if they have vaginal agenesis.