Reju Joseph Thomas

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.

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.

Surgical approach to the palpable undescended testis.

An undescended testis may be associated with abnormal testicular development and function. A number of testes that are undescended at birth have been observed to descend spontaneously. The goal of orchidopexy remains to place those testes in the scrotum that would not have descended spontaneously. How long should the surgeon wait for spontaneous descent? By what age should the testis be positioned in the scrotum to avert further damage? Does earlier intervention result in a better functioning testis? Do different surgical techniques differ in their success in retaining the testis in the scrotum when dealing with the palpable undescended testis? This article reviews the current information on the timing and types of surgical intervention of the palpable undescended testes and their outcomes.

Bladder calculi in the augmented bladder: A follow-up study of 160 children and adolescents

INTRODUCTION Bladder augmentation (BA) has been used for various congenital and acquired conditions to create a low pressure, continent catheterizable reservoir. The prevalence of calculi within the BA have been reported to be from 3 to 52.5%. The present study reports the prevalence and risk factors of bladder calculi in patients with BA. MATERIAL AND METHODS A retrospective review of 160 patients was performed from January 1997 through December 2012. The various risk factors for the formation of bladder calculi such as the nature of the anatomical defect, presence of preoperative urinary calculi, type of bowel augmentation, addition of a mitrofanoff and/or bladder neck procedure, prevalence of post-operative urinary tract infections (UTIs), need for mitrofanoff revision due to stenosis/difficulty catheterization, postoperative significant hydronephrosis and bladder calculi were recorded for analysis. The children underwent open removal or endoscopic cystolithotripsy. One hundred and eight males and 52 females (average age 6.3 years) were followed up for a median of 70.5 months. All patients performed daily bladder irrigation with tap or drinking water. RESULTS Post-operative bladder calculi were noted in 14 (8.8%) of 160 patients following BA. Median time to stone formation was 37.5 months (11-120 months). Recurrent febrile UTIs were noted in 16 of the 160 patients following BA. The various risk factors and their outcomes are summarized in table. Eight patients underwent open cystolithotomy and four patients were treated by cystolithotripsy. Post-operative recurrent bladder calculi were noted in 2 patients. Multivariate analysis revealed that exstrophy/epispadias (OR 17.2) and recurrent UTI (OR 55.4) were independent risk factors for developing postoperative calculi in bladder augmentations. All other risk factors did not achieve statistical significance. DISCUSSION There seemed to be no difference in the prevalence of calculi in the ileal or colonic augmentations. Mucus secreted by the bowel segment blocks catheters leading to incomplete drainage, stagnation and UTIs. Our protocol consists of daily bladder irrigation till the effluents are clear of mucus. This is probably the key to the low prevalence of postoperative calculi (8.8%) in our patients. CONCLUSION Bladder exstrophy/epispadias and UTIs are independent statistically significant risk factors for the formation of bladder calculi in BA patients. Other risk factors such as preoperative calculi, bladder neck procedures and the use of mitrofanoff though not statistically significant, may contribute to the overall risk. The performance of daily bladder irrigation is an important part of our management of mucus.

Cervicovaginal atresia with hematometra: restoring menstrual and sexual function by utero-coloneovaginoplasty

BackgroundCervicovaginal atresia is a rare Mullerian anomaly. The management of cervicovaginal atresia has evolved from historical recommendations of hysterectomy to various reconstructive procedures more recently. The latter carries a risk of significant morbidity and unknown fertility. We present our experience in the management of this complex anomaly.MethodsTwenty patients with cervicovaginal atresia were operated in our hospital from January 2004 through December 2013. The details of their anatomical variations and functional outcomes were analyzed.ResultsEighteen out of twenty patients had cervical agenesis. Two patients had cervical hypoplasia. All patients underwent utero-coloneovaginoplasty. Post operatively, all patients have regular menstrual cycles. One patient is married, sexually active and has satisfactory coital function. One patient had a bowel anastomotic leak that required a diversion ileostomy. Two patients developed mild stenosis. One patient has mild neovaginal mucosal prolapse. No patient has developed pyometra.ConclusionPatients with cervicovaginal atresia need to be counselled about the various reconstructive options available and the potential risks. Social and economic factor play a significant role in determining the plan of management. For patients from conservative societies, utero-coloneovaginoplasty provides a safe conduit for the passage of menstrual flow and coitus, at the cost of permanent infertility.

Enteric duplication in children: Experience from a tertiary center in South India

Background: Enteric duplications (EDs) are rare aberrations of the embryonic gut. This study was undertaken to define the clinical characteristics and management challenges of this unusual entity in the Indian population. Materials and Methods: Hospital records of 35 children with 38 ED operated between 2003 and 2014 were analyzed and followed up. Results: The median age at presentation was 285 days (range 1-day to 16 years) with male preponderance (71%). Small bowel duplications were the most common (44%), and thoracoabdominal duplications were seen in 8% children compared to 2% in the literature. The median duration of symptoms was 18 days (interquartile range [IQR] 3-210 days). Associated anomalies were seen in 49% children with vertebral and spinal anomalies being the most common. Ultrasonogram (US) was done in 83% children and had a sensitivity of 55%. In the presence of a gastrointestinal bleed, Technetium 99m pertechnetate scintigraphy scan had a positive predictive value of 80%. Thirty-five lesions were completely removed. Mucosectomy was done in two children, and one total colonic duplication was left in situ after providing adequate internal drainage. There was no postoperative mortality. The follow-up was possible in 66% children. Conclusions: EDs are uncommon and have varied, nonspecific symptoms. Thoracoabdominal duplications are more common in the Indian population. The US is a good screening tool but requires a high index of suspicion where complete excision is not possible; the provision of adequate internal drainage is an acceptable alternative. The long-term prognosis of children with ED depends on the extent of physiological disturbance due to associated anomalies.

An Uncommon Twist: Isolated Fallopian Tube Torsion in an Adolescent

We report a 13-year-old girl with bilateral paratubal cysts and left isolated fallopian tube torsion (IFTT). Paratubal cysts are uncommon in children, and IFTT is a rare complication. Awareness of this entity and prompt surgical intervention could potentially salvage the fallopian tube preserving fertility.

A helping clamp for thoracoscopic plication of eventration of the diaphragm.

Background and Aim: It is difficult to suture an extremely thin and billowed up congenital eventration of the diaphragm thoracoscopically, without insufflation. Materials and Methods: The authors describe their technique using an intestinal clamp to control the redundant tissue and a feeding tube as a flexible knot pusher, to perform the thoracoscopic plication without risking hypercapnia. Results: A satisfactory result was obtained in all the four children. Conclusion: This is a useful adjunctive for thoracoscopic plication of diaphragmatic eventration.

An assessment of quality of life of operated cases of esophageal atresia in the community.

Aims: To evaluate the outcome of the operated children of esophageal atresia (EA) focusing on their early and late morbidity and mortality and quality of life (QoL) of survivors. Settings and Design: A cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA. Materials and Methods: The medical records of the children who underwent repair for EA during the period from 2000 to 2011 at the Christian Medical College Hospital, Vellore, were collected retrospectively. Patients with parents were invited to visit the hospital for follow-up and nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL assessment of children done. QoL assessment was done using the PedsQL™ 4.0 generic core scales questionnaire comprising 4 scale scores: physical, emotional, social functioning, and school functioning. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL. Statistical Analysis Used: Statistical Package for Social Sciences (SPSS) version 16 using Chi-square or Fisher′s exact test. Results: Of 79 patients operated during the said period, there were 10 deaths and a total of 69 (87%) children survived. Of the 66 patients available for follow-up, we interviewed 30 parents and children while for the remaining 36 children, out-patients charts were reviewed retrospectively. Mean follow-up duration was 3.56 years. The height and weight for age measurement showed 47% and 56% of children respectively as below the 5 th percentile. Main problems faced by operated EA children were of the respiratory (26%) and gastroesophageal (36%) tracts. In spite of the mentioned problems faced, the overall QoL of this group appeared good. In 23 of 30 patients, who answered PedsQL™ , more than 70% had scores >85 out of 100 in QoL scoring. Conclusions: While survivals of the children born with EA have improved, these children still face nutritional, respiratory, and gastroesophageal problems during their early childhood. In spite of this, the overall QoL of this patient group appears good.

Clinical profile and management options of children with congenital esophageal stenosis: A single center experience

Aim: The aim of the study is to review 7 patients with congenital esophageal stenosis treated in our institution from a diagnostic and therapeutic point of view. Materials and Methods: This is a retrospective cohort study of 7 patients treated in Christian Medical College, Vellore from 2008 to 2014. The data were analyzed with regards to age at onset of symptoms, investigative findings, age at definitive treatment, pathology, modalities of treatment, and outcomes. Results: Symptoms started within the 1 st year of life in all children with a median age of 4 months. The time of delay in diagnosis ranged from 8 months to 81 months with a mean period of 37 months. About 6 patients had a lower esophageal stenosis and 1 patient had a mid-esophageal stenosis. About 4 of the 7 children underwent endoscopic balloon dilatation from elsewhere, with 2 of the above 4 undergoing a myotomy for a wrongly diagnosed achalasia. The number of dilatations ranged from 2 to 7 with a mean of 4 dilatations. Resection of the stenotic segment with end to end anastomosis was employed in 6 of the 7 patients, and a transverse colon interpositioning was done in 1 patient. An antireflux procedure was performed in one patient. Histopathological examination of the resected specimen revealed tracheobronchial remnant in 3 patients, fibromuscular thickening in 3 patients, and membranous web in 1 patient. Postoperatively, 2 of the 7 patients had asymptomatic gastroesophageal reflux and 1 patient had postoperative stricture requiring one session of endoscopic balloon dilatation. The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or gastroesophageal reflux. There was a statistically significant increase in the weight for age after the operation. Conclusion: Congenital esophageal stenosis is rare and often confused with other causes of esophageal obstruction. Although endoscopic balloon dilatation offers an effective temporary relief, we feel that definitive surgery is curative. Long-term results following definitive surgery have been good, especially with respect to symptoms and weight gain.