Pathology and Surgical Management of Urinary Retention Manifesting After Anorectal Malformation Surgery.

Introduction: Associated congenital anomalies, operative structural injury, and postoperative neurovesical dysfunction combine to cause urinary retention in children with a history of surgery for anorectal malformation (ARM). Aim: To study the presentation and management of urinary retention in patients with a history of ARM surgery. Methodology: Retrospective study. Results and Discussion: Twenty-five children presented with urinary retention with a history of ARM surgery performed elsewhere. There were 17 males (high - 14, intermediate - 2, and low - 1) and 8 females (cloaca). Sixteen children had an abnormal spine. Eight children had urethral injury (total transection - 3) and three had a large residual rectal stump. 41/48 renal units were dilated and 27 refluxing. Seventeen children had abnormal estimated glomerular filtration rate and five had undergone urinary diversion. Management: Definitive surgical management was individualized, the most pertinent consideration being whether normal voiding would be feasible or whether a continent low pressure urinary reservoir with clean intermittent catheterization (CIC) would be a safer option. Operative management included excision of the rectal stump (3), urethral reconstruction (2), bladder augmentation (17), Mitrofanoff port (22), bladder neck closure (2), and antireflux surgery (13). Follow-up estimated glomerular filtration rate had improved/normalized in all but two patients. HUN resolved/improved in all and 25/27 refluxing units ceased refluxing. All are socially continent with ten voiding normally and the rest on CIC. Conclusion: Urinary retention after ARM surgery is multifactorial and requires prompt recognition and possibly urinary diversion. Final reconstruction aims at achieving continence with safe upper tracts. Urethral voiding is possible in the selected cases.

An approach to giant neonatal hydrocolpos with normally sited anus - Diagnosis and management including a novel one stage operation.

INTRODUCTION: Giant non cloacal neonatal hydrocolpos is a rare entity where early recognition of the underlying cause and drainage of the distended vagina is paramount. Conventionally, immediate management has been by abdominal tube vaginostomy or, in urogenital sinus by vaginal clean intermittent catheterisation (CIC). Though effective in providing temporary relief, the abdominal tube vaginostomy is replete with complications, making it imperative to search for alternatives. Here we describe our method of emergent and definitive management of these children, where definitive repair, depending on the aetiology, has been made possible in a single stage. MATERIAL AND METHODS: Seven girls who presented primarily or with history of giant non cloacal neonatal hydrocolpos at two tertiary care centres from January 2013 to March 2021 were studied retrospectively. None had imperforate anus or a bulging hymenal membrane. Four had high vaginal atresia (VA) and three had Urogenital sinus (UGS), one with high confluence. Four girls (2 VA, 2 UGS) presented to us as neonates and were managed by emergency drainage procedures other than tube vaginostomy: CIC in one (low UGS) and a novel abdominoperineal U flap vaginoplasty in three (2 VA, 1 high confluence UGS) which was also the definitive procedure. This procedure involved fashioning an inferiorly based inverted U flap from the posterior wall of distended vagina which is tubularised and flipped down by an abdomino-perineal procedure. The girl with a low UGS later underwent a perineal procedure. Three presented elsewhere as neonates (2 VA, 1 UGS) and had abdominal tube vaginostomy as the emergency drainage procedure. This was complicated by tube dislodgement and pyocolpos till successful reconstruction was performed by us. Successful reconstructions included the abdominoperineal U flap vaginoplasty in one (VA) and a perineal procedure in another (low UGS). One girl with high VA had an inflamed vagina after tube vaginostomy, precluding the U flap vaginoplasty, and underwent abdominal vaginal mobilisation and pull through complicated by partial vaginal necrosis with septic complications. RESULTS: Reconstruction resulted in a patent and a good calibre neo-vagina in all at a mean follow up of 48 months. CONCLUSION: Immediate management of giant non cloacal neonatal hydrocolpos may either be vaginal CIC or a surgical procedure. The abdomino-perineal U flap vaginoplasty is a one stage neonatal procedure that provides effective drainage and forms a definitive neo-vagina. An abdominal tube vaginostomy can be avoided except where neonatal surgical expertise is unavailable.

Profile and Epidemiology of Intussusception in Children Under-Two Years of Age: A Prospective Surveillance.

OBJECTIVE: To report background intussusception epidemiology in children under-two years in Coimbatore district of Tamil Nadu state, India, prior to rotavirus vaccine (RVV) introduction. METHODS: Prospective sentinel surveillance was done at four hospitals in Coimbatore during April 2016 to September 2017. The children aged >1 mo and < 24 mo with diagnosed intussusception were recruited and data on clinical, treatment, diet, immunization, and sociodemography were collected. RESULTS: Sixty-eight children with intussusception were recruited. Among them 76.5% were infants with male predominance (57.4%) and 48.5% were 4-8 mo old. Vomiting (83.8%), pain in abdomen (including excessive crying) (72.1%), and bloody stools (45.6%) were the main symptoms. Reduction was tried in 92.6% cases with success in 83.8%. Surgery was needed by 16.2% children. No child died. Any RVV dose was received by 24.1% children and 2 children received RVV in 8-21 d and none within 1-7 d window. The incidence of intussusception requiring hospitalization was estimated to be 36.4 and 11.7 cases/100,000 children/y for the first and second years, respectively. CONCLUSION: Intussusception occurred in infants even without RVV exposure. Continued surveillance is needed to document the changes in intussusception epidemiology after RVV introduction.

Paediatric bladder neurofibroma on bilateral ureterostomy: rare case report-strategic management.

Urogenital involvement in neurofibromatosis is extremely rare and thus is less documented. Herein, we discuss a 11-year-old boy who was referred with a diagnosis of neurofibroma bladder on bilateral flank ureterostomy with mild renal failure. A diagnosis of neurofibroma of the bladder with ureterohydronephrosis had been made and partial excision had been attempted, followed by bilateral ureterostomy because of increasing ureterohydronephrosis. He was 13 years and wanted to be dry as he wanted to go to school. With the plan of attaining dryness with stable upper tracts, he was posted for surgery. Near-total excision followed by a sigmoid conduit was performed. The biopsy was reported as plexiform neurofibroma and is on regular follow-up, dry on a stoma bag with stable renal parameters.

Urethral reconstruction in aphallia using transpubic exposure and colonic monti neo-urethra - An addition to the De Castro reconstruction.

INTRODUCTION: Fashioning a functioning neo urethra in a boy with aphallia is one of the unsolved problems in this condition. AIM: We present our technique and outcome of operative exposure and neo urethral construction in four aphallic boys. MATERIALS AND METHODS: Retrospective study of the records of four aphallic boys operated in the period 2015-2019 was undertaken. The demographics, presentation, operative procedure, current follow up and voiding outcome was noted. RESULTS: The neo phallus was constructed by the De Castro technique in four aphallic boys aged 1-8 years. Pre operative assessment revealed bladder outflow obstruction in two and urinary incontinence in one boy. The operative exposure afforded after reflection of the De Castro flap was utilized in doing a limited pubic symphysiectomy. This exposed the bladder neck, urethra and the urethral termination into the ano rectum. The healthy native urethra was isolated by disconnecting it from its ano rectal termination and a neo urethra constructed from a segment of proximal sigmoid colon by its reconfiguration into a Monti type tube. The neo urethra was then anastomosed to the native urethra and laid within the bed of the De Castro flap so as to reach the neo phallic termination. The De Castro flap was then tabularized over the neo urethra to form the new phallus. Appendicular or ileal Monti Mitrofanoff was also added to the reconstruction. Three boys are voiding well. One boy had a partial bladder outflow obstruction resulting from operative correction of incontinence and is dry on intermittent catheterization. Follow up was for 1-4 years and upper tracts are stable or improved. CONCLUSION: We describe the transpubic approach to the construction of a reliable neo urethra in the form of a Monti tube from the sigmoid colon in four boys with aphallia. We believe this to be a useful addition to the De Castro procedure for neo phallic construction.

Non-obstructive, non-refluxing uretero-colonic anastomosis by colonic sero-muscular excision and extra mural peri-ureteric wrap - A new technique in 17 children.

INTRODUCTION: Uretero-enteric anastomosis with concomitant neobladder/augmentation/conduit becomes necessary when the bladder is unavailable or unfit for reimplantation or the ureters are short after high diversion or resection of lower ureteric pathology. Aiming to prevent both obstruction and reflux, we report a novel technique of sero-muscle denuded extra mural uretero-colic anastomosis. OPERATIVE TECHNIQUE: (Fig 1) The ureter was brought through the colonic mesentery. An adjacent zone of colon "abcd" was chosen to receive the ureter and colonic sero-muscular layer was excised from this zone. The ureter was placed on the raw area and uretero-colic anastomosis was done (a-d).The edges of the sero-muscular layer (ab and cd) were sutured over the ureter to complete a submucosal tunnel. The augmentation/neo bladder was completed such that the implanted ureter lay within it between two regions of re-configured bowel, this adding to the anti-reflux mechanism created by the extra mural peri-ureteric wrap. AIM: Surgical audit of this technique. MATERIALS AND METHODS: 17 children (exstrophy - 8, ectopic ureter with bladder agenesis/hypoplasia - 3, prune belly - 2, neurogenic bladder -2, eosinophilic cystitis -1 and posterior urethral valve -1) underwent colonic implantation of 23 ureters while 6 ureters were drained via a trans uretero-ureterostomy into the reimplanted ureter. Nine augmentation, seven neo bladders and one sigmoid conduit was performed. The colon was available for ureteric re-implant in all patients. RESULTS: Pre-operatively 21 had hydroureteronephrosis. The bladder was agenetic/hypoplastic, small and fibrosed or poorly compliant. Eleven ureters had been diverted. Post operatively (mean follow up - 3.4 years) 16 of 30 renal units were normal, 11 had mild and three had moderate residual hydronephrosis, with no new hydronephrosis. Cystogram showed no reflux in 14 children and unilateral reflux in three (one re-diverted). Of 29 renal units drained directly or via trans uretero-ureterostomy by the uretero-colic reimplantation, none are obstructed, 26 have no vesico-ureteric reflux while three (10%) have reflux. DISCUSSION: We report results comparable to other extra mural techniques into the bowel, mainly from adult literature. Pediatric refluxing ureters are often large megaureters with or without preliminary diversions. The technique described avoids obstruction although having a slightly higher incidence of recurrent VUR. The ureters with recurrent reflux were massively dilated preoperatively and showed decrease in ureteric diameter postoperatively. The wrap, made for a larger ureter could have become too roomy and allowed reflux. CONCLUSION: This technique of non refluxing non obstructive uretero-colonic anastomosis has proven useful in selected situations.

A Rare Case of Pediatric Osteochondroma Presenting as Hemothorax.

Isolated osteochondroma presenting as hemothorax is a rare entity. A 7-year-old boy presented with respiratory distress and diagnosed with hemothorax, and computed tomography showed osteochondroma and removal with resection of the rib was curative.

Management and outcome in dehisced exstrophy with a simplified bladder re-closure and further reconstruction.

INTRODUCTION: The traditionally accepted method of bladder re closure in dehisced exstrophy involves osteotomy assisted pubic bone approximation (PBA). Continent voluntary voiding is achieved in a small proportion of children sometimes after many operative procedures. We propose a simple yet reliable method of repair of the dehisced bladder using Rectus abdominis muscle flap (RAM) instead of PBA to bridge the inter-pubic gap, followed by concomitant or subsequent bladder augmentation (BA). AIM: The aim of this study is to assess the outcome of dehisced exstrophy, using a RAM flap assisted redo closure (without PBA) with concomitant or subsequent further reconstruction. MATERIALS AND METHODS: This is a retrospective analysis of children who presented with dehisced exstrophy after repair in other institutions and who have undergone redo repair using RAM flap in two tertiary care centers from 2001 to 2019. The outcome of the redo closure and subsequent or concomitant further reconstruction as regards dryness, stability of the upper tracts and resolution of vesico ureteric reflux (VUR) was studied. RESULTS: Fifty five children (34 boys) underwent redo exstrophy repair for dehisced exstrophy using the RAM flap. Epispadias repair was performed concomitantly in 31 boys. In 26 children (group1) of mean age 12 months further surgery was deferred while in 29 children (group 2) of mean age 69 months underwent concomitant BA. Nine group 1 children underwent BA subsequently. Ureteric reimplantation was done at the time of BA in 54 ureters, 40 into the bladder plate and rest into the bowel segment of BA. 22 ureters were not reimplanted. Bladder neck surgery including 18 bladder neck closure and Mitrofanoff port for Clean Intermittent catheterization (CIC) were done along with BA. The RAM assisted bladder closure was event free and none needed redo operation. 35/38 augmented children are dry on Mitrofanoff CIC and one unaugmented boy voids normally. The upper tracts remain stable on ultrasound and VUR has resolved in 67/76 ureters. At current follow up, after a mean period of 53 months eGFR was normal in all except 3 who had initially presented with severe hydro uretero nephrosis. CONCLUSION: We present a simple and reliable method of repair of dehisced exstrophy using RAM flap with the feasibility of concomitant BA. Dryness was achieved with stable upper tracts in 36/39 children, 27 of them with a single reconstructive attempt.

Intussusception in children aged under two years in India: Retrospective surveillance at nineteen tertiary care hospitals.

OBJECTIVE: Intussusception has been linked with rotavirus vaccine (RVV) as a rare adverse reaction. In view of limited background data on intussusception in India and in preparation for RVV introduction, a surveillance network was established to document the epidemiology of intussusception cases in Indian children. METHODS: Intussusception in children 2-23 months were documented at 19 nationally representative sentinel hospitals through a retrospective surveillance for 69 months (July 2010 to March 2016). For each case clinical, hospital course, treatment and outcome data were collected. RESULTS: Among the 1588 intussusception cases, 54.5% were from South India and 66.3% were boys. The median age was 8 months (IQR 6, 12) with 34.6% aged 2-6 months. Seasonal variation with higher cases were documented during March-June period. The most common symptoms and signs were vomiting (63.4%), bloody stool (49.1%), abdominal pain (46.9%) and excessive crying (42.8%). The classical triad (vomiting, abdominal pain, and blood in stools) was observed in 25.6% cases. 96.4% cases were diagnosed by ultrasound with ileocolic location as the commonest (85.3%). Management was done by reduction (50.8%) and surgery (41.1%) and only 1% of the patients' died. 91.1% cases met Brighton criteria level 1 and 3.3% Level 2. Between 2010 and 2015, the case load and case ratio increased across all regions. CONCLUSION: Intussusception cases have occurred in children across all parts of the country, with low case fatality in the settings studied. The progressive rise cases could indicate an increasing awareness and availability of diagnostic facilities.

Urinary Tract Infection in Pediatric Patients on Clean Intermittent Catheterization via a Mitrofanoff port with Reused Catheters - Any Association with Catheter Sterility?

OBJECTIVE: The objective of this study is to find out whether the reused catheters for clean intermittent catheterization (CIC) are colonized before insertion and its association with urinary tract infection (UTI). MATERIALS AND METHODS: This is a study conducted on 28 pediatric surgery patients who are on CIC via a Mitrofanoff port and who were reusing catheters, in a tertiary care private medical college hospital for 6-month period. Catheters to be used for the next catheterization were sent for culture along with urine culture. A questionnaire was utilized to assess CIC practice and UTI. RESULTS: Diseases of patients were: neurogenic bladder and exstrophy-epispadias and posterior urethral valve. Twenty-one of them had an augmented bladder. Hydronephrosis was present in ten and vesicoureteral reflux (VUR) in five. Their mean duration of CIC was 5.3 years. Of 28 catheter tip samples, 16 catheters were colonized with organism. Of the 28 urine culture samples, 17 cultures were positive and all were asymptomatic except one. Of the 16 positive catheter samples, only 9 had positive urine culture; four of them had grown different organisms and five of them had the same organism, and even in these five, single organism was seen only in three. Urine culture grew Gram-negative organism in 85%, but catheter grew Gram-positive organism in 46%. No difference was found in the variables between both groups such as hydronephrosis, VUR, and augmented bladder. CONCLUSION: Reused catheters were colonized in 57% of the study patients, but reused catheter may not be the cause of culture positivity or UTI in the study population.

Transureteroureterostomy as an adjunctive antireflux procedure in children undergoing bladder augmentation for neurogenic bladder with major reflux.

INTRODUCTION: Transureteroureterostomy (TUU) provides urinary drainage of both renal systems to the bladder via a single ureter and is useful in selected situations of complex urological reconstructions. Herein we discuss its use, advantages and complications in children with neurogenic bladders and high-grade (4/5) reflux who have undergone augmentation cystoplasty. PATIENTS AND METHODS: Children with neurogenic bladder complicated by unilateral or bilateral high-grade vesicoureteric reflux (VUR), who underwent TUU along with augmentation cystoplasty (BA), were selected from two institutions. Eighteen children with an average age of 5 years at presentation were identified from a retrospective chart review. RESULTS: All had bilateral hydroureteronephrosis (HUN) of which there were 30 refluxing megaureters. While BA reduced bladder pressure, VUR was managed by refluxing to non-refluxing TUU in six cases with unilateral VUR and unilateral reimplantation with TUU to the reimplanted ureter in 12 cases of bilateral VUR, thus minimizing reimplantation to 12 of 30 ureters. The average time of follow-up was 51 months. Follow-up cystograms showed complete resolution of VUR in all. HUN improved/stabilized in all but one child. Serum creatinine remained normal in all but two cases. Other advantages of TUU include the use of the distal ureter as a catheterizable channel and ease of undiversion when the ureter has been diverted as a ureterostomy. An unusual complication of a TUU site stricture is discussed and the innovative technique of using a cecal patch to salvage the anastomosis is detailed. CONCLUSION: We conclude that a TUU is a safe and useful adjunctive procedure in children undergoing BA for neurogenic bladder with high-grade VUR, minimizing the need for ureteric reimplant in an unhealthy bladder.

Parental Satisfaction with Pediatric Day-Care Surgery and its Determinants in a Tertiary Care Hospital.

OBJECTIVE: The objective is to assess the level of parental satisfaction of pediatric day-care surgery and its different determinants. MATERIALS AND METHODS: This is a descriptive study performed in a tertiary care hospital in India among parents of pediatric day-care surgery patients from June 2013 to March 2015. The core questionnaire for the assessment of patient satisfaction for general day care (COPS-D) was used. Variables related to surgery, overall satisfaction, one open-ended question, and socio-demographic data were also collected. Calculated sample size was 121. RESULTS: The mean and standard deviation of parental satisfaction were estimated in eight domains of day care (COPS-D) using Likert scale 1-5. Preadmission visit had a mean of 4.63 (0.52), day of surgery 4.65 (0.58), operating room 4.76 (0.51), nursing care 4.46 (0.79), medical care 4.89 (0.48), information 4.51 (0.68), autonomy 4.64 (0.56), and discharge 4.50 (0.72). In elder children, there was less satisfaction on the information and discharge domains. Overall satisfaction was good in 88% of patients and was less than satisfactory when they had significant pain. CONCLUSION: Perception of quality of pediatric day-care surgery was assessed with a questionnaire and was found to be good. Variables related to surgery such as pain may be included in the questionnaire for assessing satisfaction in the day-care surgery.

Urogenital Management in Cloaca: An Alternative Approach.

INTRODUCTION: In the management of cloaca, there is concern that dissection of the urogenital sinus in early childhood with the aim of total anatomical correction is hazardous. Avoiding such mobilization and providing mitrofanoff channel, when needed, till peripubertal period reduces complications and is technically easier. MATERIALS AND METHODS: Forty-three cases of cloaca were managed in the period 2004-2016. Case records and radiology were reviewed retrospectively. The follow-up evaluation was done by looking into voiding history, bowel movements, and menstruation history. RESULTS: There were three groups of children, namely, those with no reconstruction done elsewhere except a diverting fecal stoma (Group I, n = 25), those who had undergone anorectal correction elsewhere with no attempt at urogenital reconstruction (Group IIA, n = 13), and those with attempted bowel and genitourinary reconstruction elsewhere (Group IIB, n = 5). The Group I children (one still awaiting reconstruction) underwent early rectal reconstruction followed by expectant management of the urogenital apparatus. The 18 referred cases had multiple problems, chiefly urogenital, of congenital or iatrogenic origin. While urinary reconstruction included bladder augmentation, ileal neobladder, bladder neck closure, and ureteric reimplantation, the foundation of urinary management was intermittent catheterization through mitrofanoff stoma and the avoidance of any dissection of the cloacal common channel. Surgery on the genital tracts included drainage of hydrocolpos, perineal surgery for low vaginae and abdominoperineal vaginoplasty for high vaginae in the peripubertal period with or without bowel supplementation. Spontaneous voiding was maintained in 17 of 25 (68%) Group I girls (including one death later from intestinal complications), 7 of 13 (54%), Group IIA girls, and 1 of 5 (20%) Group IIB girls. Painless menstruation was noted in eight postpubertal girls, three through the cloacal channel (awaiting reconstruction) and five through the reconstructed vagina. Most of the children are on a bowel management program for fecal cleanliness with washouts through the neoanus or Malone's stoma. CONCLUSION: We report a nonconventional approach to cloaca based on avoiding dissection of or around the common channel for urethrovaginal reconstruction, opting for mitrofanoff stoma for intermittent catheterization, when needed, and late vaginal reconstruction. We believe this approach has reduced the overall need for intermittent catheterization.

Females with Externally Visible but Stenosed Fecal Orifice - Low or High Anorectal Malformation.

We report four female children with externally visible but stenosed fecal orifice which proved to be a high anorectal malformation (Type IV pouch colon). They were managed by an abdominoperineal approach with excellent results. We emphasize the correct recognition of this pathology.

Repair of stenosed neourethra using the proximal neourethral diverticulum - A technique in redo hypospadias surgery.

Neourethral diverticulum secondary to distal neourethral stenosis is not uncommon after proximal hypospadias repair. We describe a technique using the neo urethral diverticular roof and overlying skin as a composite flap to reconstruct the urethral stenosis distal to the diverticulum. The composite flap is advanced distally for reconstruction of the stenosed urethra either as a tube or as an onlay. Urethra at the diverticular bed is tubularized to reduce its caliber. This technique has yielded excellent results in three children. The tissue expansion caused by the obstructed urinary stream has been utilized to correct both neourethral diverticulum and stenosis.

Residual terminal bowel fistulating into the alimentary tract: a hitherto unreported complication of surgery for anorectal malformation.

The residual terminal bowel after pull-through surgery for anorectal malformation has been reported to cause urinary complications. We report two boys where residual bowel has fistulated postoperatively into the alimentary tract causing metabolic and septic complication in one and a large pelvic mass with urinary and rectal obstruction in the other.

Epididymo-orchitis complicating anorectal malformations: collective review of 41 cases.

PURPOSE: Epididymo-orchitis is a devastating complication of imperforate anus. Without proper treatment the condition may result in infertility. Nevertheless, the exact etiology and optimal management of epididymo-orchitis in anorectal malformation are unknown. MATERIALS AND METHODS: Retrospective review of our hospital records between 1997 and 2010 identified 6 cases of epididymo-orchitis in 82 boys with anorectal malformation. Extensive search of the literature revealed 35 additional cases. Clinical details of these 41 patients were analyzed using chi-square testing. RESULTS: Epididymo-orchitis occurred predominantly (86%) in high or intermediate anorectal malformation with rectourinary fistula. Median age at onset of epididymo-orchitis was 6 months and there was no side predilection. Urinary tract infection was noted in 54% of patients. Persistent mesonephric duct syndrome (27%), urethroejaculatory duct reflux (22%), vasovesical ectopia (10%), neurovesical dysfunction (34%) and urethral stricture/stenosis (17%) were frequently associated risk factors for epididymo-orchitis. In 73% of patients epididymo-orchitis recurred with exclusive antibiotic treatment. Diverting colostomy was ineffective in preventing epididymo-orchitis. Division of rectourinary fistula was curative in 36% of patients, while in 34% epididymo-orchitis recurred even after division of rectourinary fistula. Division of rectourinary fistula is likely to resolve epididymo-orchitis in the absence of additional risk factors. Vasectomy was required in 22% of patients to prevent recurrence. CONCLUSIONS: In addition to rectourinary fistula, urinary reflux into the vas deferens and neurovesical dysfunction are major risk factors for epididymo-orchitis in anorectal malformation. Division of rectourinary fistula is curative in only a third of cases. In the remaining cases some form of surgical disruption of the vas deferens is needed to resolve recurrent epididymo-orchitis.