Preperitoneal Bladder Augmentation: Feasibility and Results.

INTRODUCTION: Bladder augmentation is an important part of pediatric reconstructive urology. This study was conducted to assess the feasibility and results of our technique of preperitoneal bladder augmentation. MATERIALS AND METHODS: Thirty-three children underwent preperitoneal bladder augmentation for small inelastic bladders who had failed medical management or needed undiversion. The underlying diagnosis included neurogenic bladder, valve bladder, bladder exstrophy, non-neurogenic neurogenic, ectopic ureters, and urogenital sinus. The operative procedure involved placing the entire augmentation in the preperitoneal or subcutaneous space after bivalving the native bladder. The augment segment of the bowel with its pedicle was brought into the preperitoneal space through a small opening in the parietal peritoneum. A Mitrofanoff port was also provided where needed. RESULTS: Preperitoneal augmentation provided an adequately compliant, good volume bladder except in children with bladder exstrophy or previous abdominal surgery. There was a good cystometric recovery, with resolution of hydronephrosis and incontinence. Vesicoureteral reflux resolved in 24 of 26 units. In the 13 children who were uremic preoperatively, there was a significant decrease in serum creatinine levels, although 9 children continued to have supra-normal serum creatinine. Surgical complications seen were within expectations. There was no incidence of intraperitoneal leak, which is the main projected benefit of this procedure over the traditional "intraperitoneal" method of augmentation. CONCLUSIONS: The preperitoneal augmentation provides an adequate, safe, and low-pressure reservoir of urine except in cases of bladder exstrophy and previous abdominal surgery.

Long-term outcome following splenectomy for chronic and persistent immune thrombocytopenia (ITP) in adults and children : Splenectomy in ITP.

The purpose of this research is to study the outcomes of splenectomy for chronic and persistent immune thrombocytopenia (ITP). This study is a retrospective analysis of 254 patients with chronic or persistent ITP who underwent splenectomy at CMC, Vellore, India between 1995 and 2009. Responses were assessed based on standard criteria. One hundred and sixty seven adults and 87 children with a median age of 29 years (range 2-64) with persistent (n = 103) or chronic ITP (n = 151) was studied. Response was seen in 229 (90.2 %) including CR in 74.4 % at a median time of 1 day (range 1-54). Infections following splenectomy were reported in 16 %. Deaths related to post splenectomy sepsis occurred in 1.57 % and major bleeding in 0.78 %. At median follow-up of 54.3 months (range 1-290), 178 (70.1 %) remain in remission. The 5-year and 10-year overall survival (OS) is 97.4 ± 1.2 % and 94.9 ± 2.1 %, respectively, while the 5-year and 10-year event-free survival (EFS) is 76.5 + 2.9 % and 71.0 + 3.9 %, respectively. Splenectomy is associated with long-term remission rates of >70 % in chronic or persistent ITP.

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.

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

INTRODUCTION AND HYPOTHESIS: Absence 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. METHODS: Bowel 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). RESULTS: Thirty-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. CONCLUSIONS: Genital 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.

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.

Rectovestibular fistula with vaginal atresia: our experience and a proposed course of management.

BACKGROUND: Rectovestibular fistula with coexisting vaginal atresia poses a surgical dilemma with regard to the timing and type of reconstruction. We present our experience and suggest an appropriate course of management. METHODS: Seven patients with rectovestibular fistula and coexisting vestibular atresia were operated in our hospital during January 2004 through December 2013. The details of their bowel, menstrual and sexual functions were recorded. RESULTS: Five of the seven patients who underwent anoplasty in childhood presented to us in their teens with primary amenorrhea and cyclical abdominal pain. All five had sigmoid colon neovaginoplasty. Four of these had the uterus or its remnants anastomosed to the neovagina. All four have regular menstrual cycles. One patient is sexually active and has satisfactory sexual function. The bowel function in all the five patients is good. The remaining two patients presented in their infancy and had the anorectovestibular fistula left as the neovagina. The recto-sigmoid was pulled down to form the neoanus. Both these patients have bowel incontinence. CONCLUSION: We recommend the rectovestibular fistula be used as the neoanus and not as the neovagina. Delayed bowel vaginal replacement has excellent results and allows for optimal assessment of functioning uterine body or remnants.

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

BACKGROUND: Cervicovaginal 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. METHODS: Twenty 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. RESULTS: Eighteen 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. CONCLUSION: Patients 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.

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

PURPOSE: To determine the degree of stress in parents of children operated for intermediate anorectal malformations, and their quality of life (QOL) at follow-up. METHODS: Forty-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. RESULTS: The 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. CONCLUSION: Managing 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.

Congenital ureteric stenosis: a study of 17 children.

AIM: To review cases of congenital ureteric stenosis treated in the period between 1999 and 2007. We propose to analyze the type of presentation, management and results. MATERIAL AND METHODS: We report 17 children aged 20 days to 8 years with obstructive uropathy due to congenital stenosis of the ureter at one or more levels. This condition could be mistaken for the more common pelviureteric junction obstruction (PUJO) or primary megaureter, but it is a distinct and more serious anomaly. 13 of the 17 children had one or more associated anomalies, the most significant of which was a contralateral multicystic dysplastic kidney. Other associated anomalies included PUJO, megacalyx, vesicoureteric reflux, urogenital sinus, duplicate vagina, anorectal malformation and agenesis of the bladder. 16 children were symptomatic at presentation, with uremia (serum creatinine >1 mg/dl) in 5, while 1 was diagnosed antenatally. The correct preoperative diagnosis was made in only three children. Reconstruction included ureteroureteral anastomosis, ureteric reimplantation or ureteral substitution. RESULTS: There is follow up for 15 of the 17 patients. Length of follow up ranges from 1 to 7 years (average 2.7 years). There was satisfactory urinary drainage established in all 17 cases and uremia has resolved 3 of the 5 children. The children with solitary functioning kidney are at risk of uremia in later life. CONCLUSION: Congenital ureteric stenosis is a rare condition, but distinct anomaly with possible grave consequence and has been distinguished from other causes of congenital ureteric obstruction.

Use of vein-viewing device to assist intravenous cannulation decreases the time and number of attempts for successful cannulation in pediatric patients.

Every child who contacts a healthcare setting has a potential for intravenous cannulation (IV) procedure and related pain, fear, and distress. Many of the healthcare professionals recognize that there is a lack of intervention to prevent multiple cannulation attempts and to reduce pain and distress inflicted to children during IV cannulation. A quasi-experimental study was undertaken in pediatric patients to study the effect of a vein-viewing device (VTorch) on IV cannulation procedure. The number of cannulation attempts and time taken for successful cannulation were assessed with the use of this device (experimental group, n = 159) and compared it with the standard procedure (control group, n = 159). The effect of this device in cannulation associated pain, fear, and behavioral distress were also evaluated among these children. Using Vein-viewing device as an aid for IV cannulation significantly reduced the time taken for cannulation (P = .003) and the number of cannulation attempts (P = .03). In addition, there was a significant increase in the first-attempt cannulation success rate with the use of this device (P = .04). The use of vein-viewing device did not have any direct effect on cannulation associated pain, fear, or behavioral distress among the study participants. The results of this study may aid in improving the quality of intravenous access procedure in pediatric patients.

Pneumonostomy in the Surgical Management of Hydatid Cyst of the Lung.

Background Pneumonostomy in the surgical treatment of bilateral hydatid cyst of the lung(HCL) was described by Anand et al. This study presents the comparative long-term results of pneumonostomy for simple and complicated HCL. Methods and Patients The pneumonostomy technique was applied to both open and minimally invasive operations. The cyst was opened, endocyst removed, and any bronchial openings closed. The pericyst was closed over a 20-French Malecot tube, which was exteriorized and connected to an underwater seal. The tube was removed after 3 weeks by which time a well-established tract had formed. Hospital records of 26 children with 30 HCL who underwent pneumonostomy between 2001 and 2014 were reviewed and followed up. Patients were analyzed in two groups:group1 comprised uncomplicated and group2 complicated HCL. There was a statistically significant difference in the age at presentation in the two groups. The groups were comparable with respect to presenting symptoms, sex ratio, and side or size of cyst. Results Six(20%) children with surgical complications were graded by Clavien-Dindo classification. Three(10%) children qualified as grade 1 and did not require pharmacologic or surgical therapy. Three(10%) children had grade 3 complications; two developed empyema and one pneumothorax. There were no prolonged air leaks. Children with complicated cysts did not require longer hospitalization. Follow-up was possible in 80.76% of the children. The mean duration of follow-up was 21.3 months (interquartile range, 5-63 months). There were no postoperative recurrences or disease-related mortality. Conclusion Pneumonostomy is a safe and effective technique for dealing with the residual cavity in large complicated cysts and bilateral HCL.

Acute gastric volvulus: Late-onset ischemic consequences and their management.

We report three infants who presented with acute gastric volvulus and recovered initially after de-torsion, but later presented with sequelae due to ischemia of gastroesophageal junction, stomach and gastroduodenal junction. The first two infants could not be fed orally or by gastrostomy tube because of microgastria and stricture of the lower esophagus and gastroduodenal junction, and were managed on jejunostomy feeds, while the third child was managed on gastrostomy feeds till the gastric substitution surgery. The first case was treated nonsurgically with repeated dilatations, but ultimately succumbed to sepsis and malnutrition. In the second child, attempted dilatation resulted in esophageal perforation and she was reconstructed using ileocecal segment as a substitute for stomach and lower esophagus, and has done well. The third child was managed surgically by the Hunt Lawrence J pouch as stomach substitute and has also done well.

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 Children's 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.

Neurogenic bladder decompressing via a spontaneous colovesical fistula--a case report.

A 3-year-old girl with operated meningomyelocele and urinary incontinence presented with recurrent attacks of watery diarrhea and anuria, which were relieved by urethral catheterization. Investigations revealed a poorly compliant neurogenic bladder that periodically decompressed via a spontaneously developed colovesical fistula. The fistula was repaired with concomitant bladder augmentation. To our knowledge, this is the only report of such a complication from neurogenic bladder in childhood.

Bilateral cystic adrenal neuroblastoma with cystic metastasis in the liver.

A 2 1/2-month-old infant presented with a massive hepatomegaly. Ultrasound and computerized tomography showed a large cystic lesion in the right adrenal, small cysts in the left adrenal, and multiple cystic liver metastases. The right adrenal cyst, on excision, turned out to be a cystic neuroblastoma with hemorrhage. On follow-up, the cysts in the left adrenal and the liver metastases are regressing.

Does bladder augmentation stabilize serum creatinine in urethral valve disease? A series of 19 cases.

OBJECTIVE: This study evaluates the results of bladder augmentation (BA) in 19 boys with posterior urethral valves, especially as regards its efficacy in stabilizing serum creatinine. PATIENTS AND METHODS: In the period 1995-2005, 188 patients with urethral valves were surgically managed. Nineteen of these had undergone BA as a part of their surgical management after initial endoscopic valve ablation in 15 and diversion in four boys. The mean serum creatinine at the time of BA was 2.11 mg/dl. RESULTS: BA stabilized the serum creatinine in 14 but failed to do so in five boys. A serum creatinine level of more than 2mg/dl at the time of BA was associated with a significantly worse rate of success. BA as part of an undiversion procedure in three boys was unsuccessful. CONCLUSION: In an economic milieu where renal transplantation is not available for the majority of deserving children, careful selection is required before BA is considered as a surgical solution for the valve bladder. BA, when otherwise indicated, has been beneficial in children with pre-augmentation creatinine up to 2 mg/dl.

The leaking pancreatic duct in childhood chronic pancreatitis.

The leaking pancreatic duct in childhood chronic pancreatitis presents with ascites and pleural effusion and is a potentially lethal condition. Seven children with this condition were seen in the period 2003-2006. The correct diagnosis was not entertained till a raised serum amylase was discovered. The diagnosis was confirmed by very high levels of amylase in the aspirated abdominal or pleural fluid. Computerized tomogram was the most useful imaging study and demonstrated a dilated pancreatic duct. All children were operated within 6 days of diagnosis by a Puestow's procedure in six and peripancreatic drainage in one. Six children made a prompt and lasting recovery after a Puestow's procedure while one child, also suffering from metastatic neuroblastoma, died in the immediate post operative period after peripancreatic drainage. We recommend prompt and definitive surgical management of this potentially lethal condition.

The use of refluxing ureter in the creation of a Mitrofanoff channel in children undergoing bladder augmentation: is a formal reimplantation necessary?

To study the fate of a refluxing ureteral stump used as a Mitrofanoff channel (MC) for clean intermittent catheterization (CIC), without reimplantation in augmented bladders. Ten boys and seven girls, 1-14 years old (mean 6.5 years) seen in period 1998-2005, underwent creation of MC using refluxing or potentially refluxing ureters. Concomitant bladder augmentation (BA) was performed for various abnormalities of lower urinary tract, including exstrophy epispadias complex, neurogenic bladder cloaca and posterior urethral valve. The uretero-vesical junction was maintained intact during creation of the MC. Follow-up period ranged from 2-72 months (mean 32.5 months). Satisfactory CIC was possible in 14 children while in two children the stoma became obstructed due to non-usage. Urinary leak via the ureteric MC was seen in only one patient who partially responded to anticholinergic therapy and a shortened CIC interval but this needed to be formally closed subsequently. A refluxing ureter can be used in the creation of a MC without formal reimplantation in the majority of children undergoing BA.