Ravi Prakash Kanojia

Delayed presentation of anorectal malformations

Aims and Objectives: Delayed management of anorectal malformation (ARM) increases the surgical and functional complications for the patient. We defined “delayed presentation of ARM” and reviewed our patients with ARM to find out the incidence and causes of delayed presentation. Materials and Methods: Patients satisfying the criteria of “delayed presentation of ARM” were involved. Detailed information of each patient including the mode of presentation, associated anomalies, plan of management and follow-up was obtained from the hospital records. Results: Between 2003 and 2006, 43 patients satisfied our criteria of “delayed presentation of ARM”. There were 21 males and 22 females. Seventeen of these males presented with low-type ARM. Eleven of them were managed by a single-stage procedure. These “delayed presenters” had to live with constipation, inadequate weight gain and parental anxiety for a greater time. Analysis of the outcomes showed more functional complications in patients who had undergone failed perineal surgery previously. In females with low ARM, the procedure of choice was anterior sagittal anorectoplasty (ASARP). Single stage surgery provides good outcomes for most of low type of ARMs. High-type ARMs in males and females were managed by a staged procedure. Conclusion: “Delayed presentation of ARM” is a major group of ARM in our setup. The management and results of their treatment are not different from those of the early presenters. The most common cause of delayed ARM is wrong advice given by the health care providers followed by inadequate treatment elsewhere. Corrective surgeries taking second attempt in perineum always produces poor outcomes.

Unusual Ventriculoperitoneal Shunt Extrusion: Experience with 5 Cases and Review of the Literature

Distal migration of ventriculoperitoneal shunt is rare. We present this unusual complication in 5 patients. The lower end of the shunt was extruded from right lumbar region, cervical area, umbilicus and rectum. The cause of such extrusion is not known. The patients were managed by shunt removal followed by shunt replacement on the opposite side.

Diagnosis and management of congenital bladder diverticulum in infancy and childhood: experience with nine cases at a tertiary health center in a developing country

ObjectivesThe purpose of the study is to present the authors’ clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center.Patients and methodsRecords of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up.ResultsAll were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up.ConclusionPediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

Empyema thoracis: Surgical management in children

Empyema thoracis can produce significant morbidity in children if inadequately treated. Correct evaluation of the stage of the disease, the clinical condition of the child and proper assessment of the response to conservative treatment is crucial in deciding the mode of further surgical intervention. This ranges from intercostal chest tube drainage and video-assisted thoracoscopic surgery to open decortication. Surgical decortication becomes mandatory in neglected cases; it gives very gratifying results ameliorating the disease rapidly and is well tolerated by young patients. This article reviews the current literature and discusses the important considerations while managing these patients. Indications for surgery are highlighted, based on our large experience at a tertiary care center.

Isolated cleft sternum: Neonatal surgical treatment

Isolated cleft sternum is a rare congenital defect of the anterior chest wall and is the result of failed ventral midline fusion of sternal bands. We present two cases operated 18 and 24 days after birth with satisfactory results. Surgery is indicated to protect the heart and mediastinal contents.

A rare case of isolated Cushing syndrome in a 3-month-old boy.

Abstract Adrenocortical carcinoma (ACC) is a rare malignancy in children. Most of these are endocrinologically active tumors, with virilizing features being typically dominant. Its presentation with isolated Cushing syndrome is rare. We report a 3-month-old infant with Cushing syndrome without the clinical features of androgen or mineralocorticoid excess consequently diagnosed with ACC. We discuss the different presentations, diagnosis, and management of this rare tumor with a rarer presentation.

Vesicoureteral reflux: Endoscopic therapy and impact on health related quality of life.

Aim: To evaluate the health related quality of life (HRQOL) after endoscopic injection treatment for vesico ureteral reflux (VUR) in children. Materials and Methods: Fifty four children received treatment and were prospectively evaluated for their quality of life scores, according to resolution of reflux on cystograms and status of renal scars. Results: Of the 81 refluxing units, 72 (89%) had resolution of reflux whereas 9 (11%) did not resolve. The total average QOL was higher for the patients in the resolved group as compared to the non resolved group. Comparison of pre and post procedure renal DMSA scans in 44 patients showed status quo in 26, regression of scars in six, progression in two and formation of new scars in 6. The total increase in HRQOL was highest in regression group (67.91), and lowest in progression group (36.45). Conclusions: Successful endoscopic treatment of VUR is associated with improved quality of life, as indicated by higher HRQOL scores in the resolved group.

Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula: Basics of technique and its nuances.

Aim: To review the technique of thoracoscopic repair of esophageal atresia with tracheoesophageal fistula (TREAT) and results reported in literature and with authors′ experience. Patients and Methods: The technique of TREAT was reviewed in detail with evaluation in patients treated at authors′ institution. The patients were selected based on selection criteria and were followed postoperatively. The results available in literature were also reviewed. Results: A total of 29 patients (8 females) were operated by TREAT. Mean age was 2.8 days (range 2-6 days). Mean weight was 2.6 kg (range 1.8-3.2 kg). There was a leak in four patients, and two patients had to be diverted. They are now awaiting definitive repair. Twenty-one patients have completed a mean follow-up of 1.5 years and are doing well except for two patients who had a stricture and underwent serial esophageal dilatations. The results from current literature are provided in tabulated form. Conclusions: TREAT is now a well-established procedure and currently is the preferred approach wherever feasible. The avoidance of thoracotomy is a major advantage to the newborn and is proven to benefit the recovery in the postoperative patient. The technique demonstrated, and the tweaks reported make the procedure easy and is helpful to beginners. The outcome is very much comparable to the open repair as proven by various series. Various parameters like leak rate, anastomotic stricture are the same. The outcome is comparable if you TREAT these babies well.

Dorsal enteric sinus with spina bifida: a rare form of split notochord syndrome.

Split notochord syndrome is a rare congenital malformation that results in spinal anomalies associated with anomalies of the gastrointestinal tract and central nervous system. This report presents an infrequently described variant of this syndrome. A 9-month-old female presented with a dorsal midline mass since birth which was partially covered by skin, and part of the lesion gave a gross appearance of intestinal mucosa. MRI was suggestive of partial sacral agenesis with spinal dysraphism with tethered cord. Excision of the mass with repair of the spinal defect was done. Histopathology confirmed the presence of gastrointestinal mucosa.

NON SURGICAL MANAGEMENT OF CYSTIC LYMPHANGIOMA

Aim/Purpuse: To evaluate our experience of 19 patients of lymphangioma who were treated by intralesional Bleomycin.Materials and Methods: Nineteen patients of lymphangioma aged between 16 days to 11 years were managed in the department. The male-female ratio was of 2:1. Commonest sites were in the neck (58%) followed by axilla (21%). The patients were treated by intralesional bleomycin injection. Bleomycin was given at a dose not exceeding 0.5 unit/kg/dose at interval of 2 weeks. Reduction in size of the mass was noted in between 2 weeks to 16 weeks and number of injections required for each patient varied from 1 to 6. Follow up ranged from 1– 7 Year.Result: In injection group, significant reduction of mass was noted in 84% (n=l6) and 57% (n=11) of them showed complete disappearance. No serious complications were noted in any patient.Conclusion: Our experience showed that Bleomycin in aqueous solution is a good sclcrosing agent in the management of lymphangioma.