Mamta Sengar

Gastric teratoma with predominant nephroblastic elements

Gastric teratoma is an uncommon neoplasm in infancy. Most of the gastric teratomas described have mature elements while some have immature elements. Occurrence of nephroblastic rests in gastric teratoma has not been described in available literature earlier.

Prolapse of the rectum associated with spontaneous rupture of the distal colon and evisceration of the small intestine through the anus in an infant

Prolapse of rectum of varying degrees is a well-known entity in children. Spontaneous rupture of the rectum along with massive ileal evisceration because of increased intraabdominal pressure is a rare complication of rectal prolapse in the adults. Rectal prolapse in children is usually a benign condition. Known complications of the rectal prolapse in children include recurrent mucosal ulceration, bleeding, and proctitis. Spontaneous rupture of the rectum with or without ileal evisceration has not been previously reported in infants.

Congenital pouch colon in female subjects

Over a period of 5-year (May 2000 to April 2005) 29 patients of congenital pouch colon (CPC) were managed by single pediatric surgeon in the Department of Pediatric Surgery of a tertiary hospital. Of these, 11 were girls (M: F: 1.6:1). Detailed anatomy could be studied in nine patients, were included in this study. Age of presentation in female subjects ranged 1 day to 8 years. On examination, eight of the nine patients had single perineal opening suggesting a very high incidence of association of cloaca in female subjects with CPC. Four had short urogenital sinus with colonic pouch opening in the posterior wall of urinary bladder. In other two patients, CPC terminated in short cloaca. Anomalies of mullerian structures such as uterus didelphus and septate vagina ware commonly encountered. Proximal diversion with or without pouch excision was done as the initial preliminary treatment for all those patients who presented in early life. Of the nine girls, two died after the preliminary surgery. Only five patients have undergone definitive surgery. Definitive surgery included abdomino-perineal pull-through of proximal normal colon ( n =2), tubularization of pouch with abdomino- posterior sagittal- pull through ( n =2) and abdomino-posterior sagittal urethra-vaginoanorectoplasty with pull through of tapered pouch colon in one patient. Two of these patients had concomitant bowel vaginoplasty. Three patients with tubularized pouches had constipation and spurious diarrhea. However, good pseudo-continence of bowel was achieved on regular bowel washes. The cosmetic appearance of perineum in all these three patients was acceptable. The patients in whom pouch excision was done had diarrhea and severe perineal excoriation resistant to conservative management.

Gastrointestinal mucormycosis: A success story and appraisal of concepts

Mucormycosis is an opportunistic, life-threatening fungal infection caused by fungi of the class Zygomycetes. The disease has traditionally been reported in immunocompromised patients, premature infants, diabetics, transplant recipients, prolonged use of corticosteroids or in condition associated with increased availability of serum iron such as acidosis or deferoxamine administration. The infection is progressive and associated with a high mortality unless treatment is initiated promptly. The number of cases of gastrointestinal mucormycosis indexed on PubMed over the past 2 decades has shown an alarming rise. Moreover, the infection is being increasingly reported in patients without the traditional risk factors. We report successful management of an immunocompetent child with gastrointestinal mucormycosis who responded to aggressive treatment with surgical debridement and antifungal agents. The fungicidal activity of colistin (polymyxin E) has also been highlighted.

Bowel vaginoplasty in children

OBJECTIVES: To describe our experience with bowel vaginoplasty done in children. MATERIALS AND METHODS: This is a retrospective study of eight children aged 10 months to 8 years, who underwent bowel vaginoplasty over a period of 5 years (2000-2005). The indications of bowel vaginoplasty included anorectovestibular fistula (ARVF) associated with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (n=6) and cloaca (n=2). The bowel segment used for vaginoplasty included colon (n=3), ileum (n=2) and duplicated rectum (n=1). In two patients of ARVF associated with uterovaginal agenesis, the distal- most part of ARVF was transected at the level of peritoneal reflection and left as neovagina, whereas the proximal bowel was pulled through at the proposed neo-anal site. All the patients were advised daily home dilatation of the neo vaginal orifice with Hegars dilators, for a period of six weeks. RESULTS: Bowel vaginoplasty was done in eight patients. None had any significant per-operative complication. Two patients had abdominal wound dehiscence, requiring secondary suturing. Two patients had mucosal prolapse of the neovagina, which required trimming. One patient died two months after discharge, because of meningitis. Out of the eight patients, seven are in regular follow-up. Six patients have neovagina, cosmetically acceptable to the parents; all have been radiologically proven to have adequate length. One patient had unacceptable perineal appearance with nipple-like vaginal orifice and scarred perineal wound, that merits a revision. None of the patients had vaginal stenosis and excessive mucus discharge, during follow-up visits. Although post surgical results are acceptable to the parents cosmetically, the sexual and psychological outcome is yet to be assessed. Conclusions: Bowel vaginoplasty is a safe and acceptable procedure to treat the pediatric patients of uterovaginal agenesis and cloaca.

Colorectal duplication with prostatorectal fistulae.

Tubular colonic duplications are rare malformations and its association with prostatorectal fistula is extremely rare with only one case reported to date. We report the successful management of an 8 year old child with communicating tubular colonic duplication with prostatorectal fistulae. Tubular colonic duplications may be difficult to diagnose due to varied presentations, therefore a high degree of suspicion should be present while managing children with isolated congenital recto urinary fistulas.

Intercostal variant of lumbar hernia in lumbocostovertebral syndrome: our experience with 6 cases

Lumbocostovertebral syndrome (LCVS) is a rare type of congenital lumbar hernia. Its features include lumbar hernia associated with genitourinary, vertebral, and rib anomalies. Less than 25 cases have been reported to date. We describe the clinical manifestations and associated anomalies in 6 cases of LCVS managed by us. The patients with suspected LCVS syndrome should be evaluated by detailed clinical history, examination, conventional radiography, magnetic resonance imaging spine, ultrasound abdomen, and echocardiography. The defect can be repaired using local tissues in infancy. There is a need for long-term follow-up for possible late recurrence and scoliosis.

Mature Teratoma in a Supernumerary Ovary in a Child: Report of the First Case

BACKGROUND Supernumerary ovary (ie, ovarian ectopia having no anatomic connection with the normally placed ovaries) is a rare gynecologic condition. To the best of our knowledge, only 1 pediatric case of supernumerary ovary has been reported to date in the English literature. CASE A 4-year-old girl was assessed for foul-smelling vestibular discharge and was found to have a fistulous tract with opening near the vaginal orifice. Fistuloscopy revealed hair in the lumen of the tract. Computed tomography scan showed a retroperitoneal mass in continuation of the tract. Excision of the mass revealed a mature teratoma in a retroperitoneal supernumerary ovary. SUMMARY AND CONCLUSION Supernumerary ovary, a gynecologic rarity, is even more uncommon in children. Hence, a thorough clinical-radiological-pathological correlation is mandatory to diagnose extragonadal ovarian tumors arising in supernumerary ovaries.

Delayed repair of posttraumatic bronchial transection.

Tracheobronchial injuries (TBI) are uncommon but potentially fatal injuries that can occur following blunt trauma to the thoracic region. Occasionally the diagnosis is not made at initial presentation, and patients present late with various sequelae. A 3 year old boy sustained blunt thoracic injury and developed respiratory distress, pneumothorax, and subcutaneous emphysema, which were managed conservatively with intercostal tube. Bronchoscopy performed to assess the cause of persistent collapse of the right lung and continued respiratory distress showed complete block of right main bronchus one cm away from the carina. Thoracotomy was performed and the transected ends of the bronchus were anastomosed. Postoperatively the right lung expanded with relief in respiratory distress. This report highlights the success of delayed repair of TBIs as an alternative to pneumonectomy.

Use of intralesional bleomycin as primary therapy in macrocystic lymphangiomas

A prospective study was conducted to determine the efficacy and short term adverse effects of intralesional bleomycin sclerotherapy in children with lymphangioma. The diagnosis of lymphangioma was made on clinical examination and was supplemented with Doppler ultrasonography. Children with ultrasonographically proven, subcutaneous, macrocystic lymphangiomas with no/ minimal vascularity were included in the study. Before the procedure hemogram and baseline chest radiograph were performed. The procedure was performed after taking consent for the parents, under mild sedation (oral Triclofos) and aseptic conditions. A 20 G needle was used to perform the aspiration and attempt was always made to traverse the needle in subcutaneous plane initially and then puncture the lymphangioma in order to prevent egress of bleomycin from the puncture site at the end of the procedure. After complete aspiration of the lesion bleomycin was injected through the same needle. If the cyst could not be aspirated properly then injection under ultrasonographic guidance was performed. Bleomycin (1 mg/ml) was administered at dose of 0.6 mg/kg with a maximum dosage of 1 mg/ kg or 15 mg per session. A compression dressing, if feasible, was applied after the procedure. The parents were instructed to review with the child after 2 days in the outpatient department for removal of dressing and evaluation of any adverse effects. Patient was again examined at the end of 3 weeks and need for repeat injection assessed.