Management of urinary incontinence in girls with congenital pouch colon.

PURPOSE: This study describes the management of urinary incontinence (UI) in eight girls with congenital pouch colon (CPC) associated with anorectal malformation (ARM). METHODS: From 2013 to 2015, six girls with CPC and UI underwent bladder neck reconstruction (BNR). Four girls had complete UI (CUI) and two girls partial UI (PUI). From 2019 to 2023, four girls, including two with failed BNR, underwent bladder neck closure (BNC) and augmentation cystoplasty (AC) with a continent stoma. Subtypes of CPC were Complete CPC (n = 7) and Incomplete CPC (n = 1). All girls had a double vagina; short, wide urethra; and reduced bladder capacity with an open, incompetent bladder neck (BNI). During BNR, a neourethra was constructed from a 1.5-2 cm-wide and 1.5-3-cm-long trigonal strip. During BNC, AC was performed using a 20 cm ileal segment (n = 3) and by a colonic pouch segment, preserved during earlier colorraphy (n = 1). Continent stoma included a Monti's channel (n = 3) and appendicovesicostomy (n = 1). RESULTS: BNR produced moderate improvement of UI (n = 2), while UI was still very severe (n = 4). During BNC, intraoperative complications included iatrogenic vaginal tears (n = 4). Early complications included partial dehiscence of the ileocystoplasty (n = 1), partial adhesive small bowel obstruction (n = 1), and difficulty in stomal catheterization with prolonged drainage from the pelvic drain (n = 1). Late complications included unilateral grade II vesicoureteric reflux (n = 2) and vesicovaginal fistula (VVF) (n = 2) needing trans-vaginal closure in one girl. Urinary stones (n = 2) with stomal leakage of urine in one girl needed open cystolithotomy twice (n = 1), and endoscopic lithotripsy (n = 1). At follow-up, all patients have high overall satisfaction with the procedure and their continence status. CONCLUSIONS: BNC with AC and a catheterizable stoma satisfactorily achieves continence in girls with CPC and UI, vastly improving quality of life. If lower urinary tract (LUT) anatomy is favorable, BNR with/without AC can be the initial surgical procedure. BNC should be the primary procedure in girls with unfavorable LUT anatomy and for failed BNR. LEVEL OF EVIDENCE: IV.

Management of non-functioning kidney due to pelvi-ureteric junction obstruction in pediatric age group: an observational study.

INTRODUCTION: A non-functional kidney (NFK) has been defined as one having paper-thin parenchyma, and split renal function (SRF) of < 10% on a nuclear scan. There are differences of opinion about nephrectomy or pyeloplasty in these patients. The present study was conducted to assess our management strategy of renal salvage for NFK. MATERIALS AND METHODS: It was a retrospective cohort study from January 2015 to July 2022, patients having SRF < 10% were included. These patients underwent ultrasound-guided percutaneous nephrostomy (PCN). A repeat nuclear scan was performed after 3 months. If SRF increased to > 10%, pyeloplasty was performed. RESULTS: Fifteen patients were managed. The mean age was 24.67 ± 23.61 months. Male to female ratio was 4:1. The initial mean SRF was 6.67 ± 2.85, which improved to 16.80 ± 4.69 after 3 months of placing the PCN (p < 0.001). The corresponding changes in the mean effective renal plasma flow (ERPF) were 60.13 ± 24.08 to 106.53 ± 24.61 (p < 0.001). There was no complaint after the placement of PCN. All patients underwent dismembered pyeloplasty. CONCLUSION: In NFK due to PUJO, expectant treatment in form of PCN followed by pyeloplasty appears to be the primary treatment modality, and nephrectomy may not be needed in any of them.

Evaluation and management of "low" anorectal malformation in male children: an observational study.

PURPOSE: ARM with perineal fistula has been traditionally defined as low ARM (LARM). This study was conducted to evaluate LARM in male patients with an emphasis on the role of various factors on the outcome and follow-up of them. MATERIALS AND METHODS: It was a retrospective cohort study. The clinical presentation, associated anomalies, and complications were assessed. The operative procedures included cutback anoplasty and others. The patients were followed in the outpatient department. The complications were assessed and managed accordingly. RESULTS: During the study period of 8 years, 301 patients were admitted. The complaints included absent or abnormal anal opening, abdominal distension, constipation, and peritonitis. Most of the children (n = 214) presented in the neonatal period. The most common clinical presentation was the perineal fistula. The most common associated anomaly was urologic. Fourteen patients were referred from other centers after complications. The most common problem in follow-up was constipation. CONCLUSION: LARM in male patients may have a diverse presentation. The associated anomalies need proper assessment. Awareness may avoid delayed presentation and unwanted complications. When managed by an expert, the condition can be effectively managed. Regular follow-up is important.

Study the Possibility of Creating Mucosal Valve Mechanism at Ureteric Orifice without Obstructing the Urine Outflow but Preventing the Urine Backflow into the Ureters.

Objective: To study the possibility of creating mucosal valve mechanism at ureteric orifice without obstructing the urine outflow but preventing the urine backflow into the ureters. Materials and Methods: Ethical waiver was obtained from the institutional ethical committee. Prospective experimental study was conducted on the post-mortem specimen of intact bladder with urethra and bilateral ureters retrieved from the already slaughtered lamb available in the meat market. Feeding tube inserted via urethral opening into the bladder lumen and bladder inflated with saline demonstrated no reflux of urine via transverse cut opening of ureters. Bladder lumen opened, ureteric orifices incised backwards to eliminate the obliquity. After closing the bladder opening, saline inflation test demonstrated bilateral reflux of saline via cut openings of bilateral ureters. Bladder was re-opened. The upper limb of horizontal U started 10 mm lateral and 8 mm above the refluxing ureteric orifice. Distal most curvature of horizontal U was kept 5 mm medial to ureteric orifice continuing along the lower limb of horizontal U terminating 10 mm lateral and 8 mm below the refluxing ureteric orifice, mucosal flaps from superior and inferior incision mobilized and edges joined to cover the ureteric orifice creating a flap valve mechanism. Influx of saline via cut end of ureters demonstrated no obstruction. Bladder was closed. Saline inflation test and contrast study demonstrated abolition of reflux on flap side and persistence of reflux on another side. Results: Five such experiments were conducted. On the side where the valve was created, Vesicoureteral reflux was abolished in four but in one minimal reflux still persisted. Conclusion: Creating a mucosal flap valve around the ureteric orifice can prevent reflux in 80% of cases without obstruction and without the necessity of ureteric mobilization or creating submucosal tunnel.

The wandering ventriculoperitoneal shunt and the scope of its salvage.

PURPOSE: Distal shunt tube migration following ventriculoperitoneal (VP) shunt placement in children is mostly managed by an initial shunt diversion/removal and subsequent replacement. Lately, shunt salvage is being used as an alternative in certain conditions. We have focused on the situations where one can consider or disregard shunt salvage in such cases. METHOD: A retrospective study of children treated for distal shunt migration following VP shunt placement between January 2013 and December 2019. RESULT: Seventeen children were managed for over 7 years. These included cutaneous extrusions (n = 4), hollow viscus perforation (n = 6), inguinal hernias (n = 5), and umbilical extrusion (n = 2). The surgical treatment varied from a cutaneous wound closure (with a tube in situ), temporary external shunt diversion, and laparotomy with shunt reposition into the peritoneal cavity. Shunt salvage was possible in three cases, whereas in 2 cases even though shunt salvage was possible, it was not feasible due to a short residual shunt length. CONCLUSION: VP shunt salvage is possible in certain cases of distal shunt migration with a functional uninfected shunt. Small cutaneous extrusions can be covered by a local skin flap. Also, one should consider the residual intraperitoneal shunt length before its salvage in small children.

Isolated Penile Injury in Boys: Accident, Negligence, or Abuse?

AIM: The aim of the study was to highlight the etiology, spectrum of presentation, and management of isolated penile trauma in boys. METHODS: A retrospective review of boys treated for isolated penile trauma between January 2015 and June 2019 at a tertiary-level hospital. RESULTS: Nine children were admitted over 4½ years. Etiology: The mechanism of penile injury (PI) was penile hair tourniquet (n = 5), postcircumcision (n = 2), dog bite (n = 1), and scald injury (n = 1). Extent of injury includes complete urethral transection at corona (n = 4); loss of urethral plate in a case of hypospadias (n = 1); complete loss of glans (n = 1); penile transection at corona (n = 1); total penile amputation (n = 1); and deep partial-thickness burns of penile shaft and adjacent suprapubic skin (n = 1). Management: One patient absconded. The remaining patients were managed as follows: calibration of urethral meatus (n = 1); penile burn was managed with dressing and antibiotics; coring of glans with urethral end-end anastomoses (n = 4); Bettocchi's quadrangular lower abdominal flap phalloplasty (n = 1); and Bracka's staged urethroplasty (n = 1). Complications include wound infection following trauma (n = 4), postsurgical infection (n = 3), urethrocutaneous fistula (n = 2), and reapplication of penile hair tourniquet (n = 1). CONCLUSION: Isolated PI in boys is not uncommon. Most are preventable if the parents are apprized and watchful. The clinician should also be vigilant regarding child maltreatment. A staged approach tailored to the type of injury provides a satisfactory outcome.

Meningocele tourniquet syndrome.

Tourniquet syndrome is a rare condition where a tourniquet applied to an appendage leads to an obstructed blood flow and subsequent ischemic injury. Meningomyelocele and meningocele are common birth defects, and involvement of meningocele in tourniquet syndrome is never mentioned in the literature. We managed a 10-day-old male child presenting with infected lumber meningocele with a tourniquet tied at its base. It is being presented with review of relevant literature.

Radiologic Evaluation of Uterovaginal Abnormalities in Girls with Congenital Pouch Colon.

BACKGROUND/PURPOSE: The radiologic investigations of 25 girls with congenital pouch colon (CPC), managed over 17 years, were retrospectively reviewed. In 13 girls who form the study group, the investigations provided information about the anomalous uterovaginal (UV) anatomy and these findings were studied. MATERIALS AND METHODS: Age at presentation was 2 months to 10 years. The subtypes of CPC were Type I (n = 2), Type II (n = 9), and not recorded (n = 2). All patients had a double vagina and a unicornuate uterus on each side in the pelvis. The radiologic studies, performed at varying periods after surgery, included an intravenous urogram (IVU) (n = 4), micturating cystourethrogram (MCU) (n = 3), distal ileostogram/colostogram (n = 6), and magnetic resonance imaging (MRI) (n = 7). RESULTS: IVU and MCU showed retrograde filling of the vaginas with contrast during micturition with a small-capacity urinary bladder and a relatively open bladder neck. The two vaginas were quite apart, but symmetrical in appearance and position. A distal dye study showed filling of the colonic pouch, its terminal fistula, and the two vaginas in six patients. Opacification of the bladder was seen in 3/6 girls, including one girl with left-sided Grade IV vesicoureteral reflux. MRI (n = 7) showed a monocornuate uterus on each side in the pelvis. The upper vaginas on each side were apart, being widely apart (n = 5) and somewhat closer (n = 2). The lower vaginas were closer with an intervaginal septum. Other findings were a widely open bladder neck and urethra in two girls with urinary incontinence and visualization of the terminal fistula of the colonic pouch (n = 2). The lumbosacral spine was normal in all patients. CONCLUSIONS: In girls with CPC, retrograde reflux of contrast into the vaginas during a distal dye study or an IVU/MCU may provide useful details of the anatomy of the vaginas. An MRI scan is recommended as essential for comprehensive evaluation of the anomalous UV anatomy.

Anorectal Malformations in Adolescent Females: A Retrospective Study.

CONTEXT: While most of the females with anorectal malformations (ARMs) present in either neonatal period or early infancy, a small percentage presents in their adolescence. AIM: The aim is to study the causes of delay in presentation, management, and postoperative outcome in these cases. SETTINGS AND DESIGN: This is a retrospective observational study conducted in the Department of Paediatric Surgery, King George's Medical University, Lucknow. SUBJECTS AND METHODS: Records of females with ARM managed in one unit of the department from 2010 to 2015 were scrutinized. Of these, record of females who primarily presented in adolescence was reviewed. Data regarding the demographics, clinicoradiological presentation, management, postoperative stay, and follow-up were analyzed. Causes for delay in presentation were looked for. STATISTICAL ANALYSIS USED: Statistical analysis used was not required. RESULTS: Out of 627 cases of ARM managed over 5 years, ten girls (5.3%) presented between 12 and 18 years (average 14.4 years). Main reasons for the delay in presentation were misinformation, illiteracy, and poverty. These comprised of anovestibular fistula (n = 5), anterior ectopic anus (n = 3), perineal canal (n = 1), and rectovestibular fistula (n = 1). Three (30%) girls had no problem apart from an abnormal anal orifice. Five (50%) girls had constipation as a major additional symptom. Four cases had a normal to mildly enlarged rectum on contrast study, whereas the rectum was moderately to hugely dilated in the remaining six cases. Six cases were managed with posterior sagittal anorectoplasty while an anterior sagittal anorectoplasty was done in four. Minor wound dehiscence developed in two cases. There was no mortality. In a mean follow-up of 16 months (8-26), constipation and soiling were seen in 5 (50%) girls. The overall outcome in these girls was satisfactory. CONCLUSION: Presentation of females with ARM in adolescence is not uncommon in the third world. A primary pull through is possible in these girls with mild-to-moderate rectal dilatations. A diverting colostomy before pull through is always a safe option in cases with severely dilated rectum and also otherwise. Moreover, a greater awareness regarding these malformations in the general public is required.

Ectopic Salivary Gland Cyst in the Neck: Association with Congenital Plunging Ranula.

Congenital ranula is rare. We report a large, symptomatic, congenital plunging ranula associated with a salivary gland cyst in the neck. To the best of our knowledge, this is the first such reported case. Even though both the cysts had their origin from the sublingual gland, only the cervical cyst had a capsular covering. Herniation of a part of the immature sublingual gland anlage through a congenital mylohyoid defect, its separation, and subsequent maturation could explain this occurrence.

Type V Congenital Pouch Colon: An Extremely Rare Variant of Anorectal Malformations.

Type V is the rarest form of congenital pouch colon with only four cases reported till date. We report this anomaly in a 6-month-old boy. He was managed successfully with excision of distal pouch and coloplasty of proximal pouch along with abdominoperineal posterior sagittal anorectoplasty. We recommend preservation of proximal pouch in such cases.

Migration of Ventriculoperitoneal Shunt into a Hernia Sac: An Unusual Complication of Ventriculoperitoneal Shunt Surgery in Children.

We report 2 cases of ventriculoperitoneal (VP) shunt migration into an inguinal hernia sac. In both cases hernia manifested itself on the right side in late infancy. We attempted to analyse the anatomical and mechanical factors leading to shunt migration as seen in the X-rays of our cases.

Congenital pouch colon in girls: Genitourinary abnormalities and their management.

AIMS: To discuss the assessment and management of genitourinary (GU) tract abnormalities in 21 girls with Types I-III congenital pouch colon (CPC), studied over a period of 10 years. MATERIALS AND METHODS: Assessment included clinical and radiological assessment, examination under anesthesia (EUA), endoscopy of the lower GU tract, and evaluation of the surgical findings, operative procedures for the GU anomalies, and the results of management. RESULTS: Initial examination of the external genitalia showed a "clover-leaf" appearance (n = 6) and a single perineal opening (n = 6). In 9 patients, the openings of the urethra and double vagina were seen, of which a vestibular fistula was seen in 5 and an anterior perineal fistula in 1. Seventeen patients (81%) had urinary incontinence (UI) - partial in 10, and complete in 7. Renal function tests, X-ray sacrum, and abdominal US were normal in all patients. Micturating cystourethrogram (n = 9) showed a wide, bladder neck incompetence (BNI) with reduced bladder capacity in seven patients. EUA and endoscopy revealed a septate vagina in all patients and the urethral opening at a "high" position (n = 14) or at a relatively normal or "low" position (n = 7). In 8 patients, the intervaginal septum was thick and fleshy. Endoscopy showed a short, wide urethra, an open incompetent bladder neck, poorly developed trigone, and reduced bladder capacity in the patients with UI. The fistula from the colonic pouch opened in the proximal urethra (n = 4), high in the vestibule (n = 3), low in the vestibule (n = 8), perineum just posterior to the vestibule (n = 1), and undetermined (n = 5). Vaginoscopy (n = 8) showed normal cervices in all and cervical mucus in 4 patients. The subtypes of CPC were Type I CPC (n = 4), Type II CPC (n = 16), and Type III CPC (n = 1). All 21 patients had uterus didelphys. In four patients with UI, during tubular colorraphy, a segment of the colonic pouch was preserved for later bladder augmentation if required. A Young-Dees bladder-neck repair (BNR) was performed in four older girls for treatment of UI, with marked improvement in urinary continence in two girls, some improvement in one girl with complete urinary incontinence, and minimal improvement in one child. Division of the intervaginal septum was performed in three girls. CONCLUSIONS: GU abnormalities in girls with CPC need to be assessed and managed by a tailored protocol. UI is frequent, and its correction may require BNR. A segment of the colonic pouch can be preserved for possible future bladder augmentation. All girls have a septate vagina, often widely separated, and uterus didelphys. Gynecologic assessment and monitoring is required throughout adult life. Considering the wide opening of the vestibule, surgical management of the urogenital component by division of the intervaginal septum and if required, the vagino-fistula septum on each side results in a normal or a hypospadiac urethral opening and an adequate vaginal inlet.

Extraperitoneal Pelvic laparoscopic disconnection of accessory urethra from normal urethra in a case of urethral duplication.

We report an extraperitoneal pelvic laparoscopic approach to disconnect accessory urethra from normal urethra in complete urethral duplication. First stage consisted of chordee correction, partial excision of the accessory urethra and glansplasty. In the second stage the remaining accessory urethra was disconnected from the normal urethra through a pre-peritoneal minimal access approach to the retropubic space. The remaining distal mucosa was ablated using monopolar cautery.

Laparoscopic repair of hernia in children: Comparison between ligation and nonligation of sac.

AIM: The essence of the current techniques of laparoscopic hernia repair in children is suture ligation of the neck of the hernia sac at the deep ring with or without its transection. Some studies show that during open hernia repair, after transection at the neck it can be left unsutured without any consequence. This study was aimed to see if the same holds true for laparoscopic hernia repair. MATERIALS AND METHODS: Sixty patients (52 boys and eight girls, 12-144 months) with indirect inguinal hernia were randomized for laparoscopic repair either by transection of the sac alone (Group I) or transection plus suture ligation of sac at the neck (Group II). Outcome was assessed in terms of time taken for surgery, recurrence, and other complications. RESULT: Thirty-eight hernia units in 28 patients were repaired by transection alone (Group I) and 34 hernia units in 29 patients were repaired by transection and suture ligation (Group II). Three patients were found to have no hernia on laparoscopy. Recurrence rate and other complications were not significantly different in the two groups. All recurrences occurred in hernias with ring size more than 10 mm. CONCLUSION: Laparoscopic repair of hernia by circumferential incision of the peritoneum at the deep ring is as effective as incision plus ligation of the sac.

Analysis of the Efficacy of Elastography in Comparison with Dynamic Renal Nuclear Scintigraphy in the Evaluation of Unilateral Pelvi-Ureteric Junction Obstruction.

INTRODUCTION: In hydronephrosis due to pelviureteric junction obstruction (PUJO), an obstruction to urine flow may lead to increased pelvic pressure, which may cause interstitial fibrosis and renal impairment. Recently, there have been reports on renal pelvic assessment using ultrasound elastography (USE). This study was conducted to see if USE can evaluate PUJO and if it can be correlated to the findings of the dynamic renal nuclear scan. MATERIAL AND METHODS: In this observational study, only patients with unilateral PUJO underwent acoustic radiation force impulse (ARFI) elastography. A rectangular region of interest (ROI) measuring 5 × 10 mm was positioned on the cortex region of the upper, mid, and lower poles of the affected kidney. Three valid measurements were obtained, from which a mean value was calculated. A dynamic renal nuclear scan using Technetium-99m ethylene dicysteine (EC or TC99 m EC) was obtained and split renal function (SRF) was used for comparison. RESULTS: In the group of 20 patients, the mean age was 3.37 years. The mean SRF of the affected kidney was 26.65 %, and the corresponding USE value was 0.45 kpa. The Spearman's rho correlation coefficient for SRF and USE was 1 and 0.672, respectively (p = 0.001). Elastography was not feasible if SRF was less than 20 %. CONCLUSION: USE may be able to comment on the renal functional status of hydronephrosis. If USE is reported as non-feasible, it may suggest that renal function is grossly compromised. It may serve as an alternative diagnostic modality for renal functional evaluation. LEVEL OF EVIDENCE: Level II, Prospective Cohort Study.

Retrieval of a retained umbilical vein catheter at 6 months.

Umbilical venous catheterisation is carried out for the care of neonates who have very low birth weight and are critically ill. It is uncommon for catheter fragments to become fractured and retained. Here, we describe a case of a retained fractured umbilical vein catheter in an infant. An endovascular procedure to retrieve a catheter is the usual management option when the catheter has been accidentally cut and has moved proximally into a major vessel or inside the heart. It is rare to need open exploration and retrieval.

Intralesional steroid in the era of propranolol for infantile hemangioma-Do we need it?

INTRODUCTION: Despite its effectivity, there are reports of poor response to propranolol in the treatment of infantile hemangioma (IH). The literature is limited to the type of IH that fails to respond to propranolol. This study was conducted to analyze which types of hemangiomas respond poorly to propranolol and the effects of intralesional triamcinolone (IL TMC) in them. MATERIAL AND METHODS: In this prospective cohort study, IH was classified as superficial, deep, and mixed. The clinical details were recorded. Propranolol was started in the patients at a dose of 1 mg/kg/day and increased to 2-3 mg/kg/day. The response to the treatment was evaluated as excellent, good, poor, and no response. IL TMC was given in the non-responding group at a dose of 1-2 mg/kg at one-month interval for a total of six doses after stopping propranolol. RESULTS: Ninety-six patients (median age, 7 months; M/F = 2:1) were treated. Superficial hemangioma was present in 40 (41.7%), deep in 10 (10.4%), and mixed in 46 (47.9%) patients. The response was statistically better if initiated within four months of age. It was not influenced by the sex, number, site, or size. The response was statistically better in superficial hemangioma. IL TMC was administered in the 16 patients. The response was good or excellent in 10 patients. CONCLUSION: Propranolol will be used as a first-line drug for IH. All superficial IHs are likely to respond. There will be a possibility of non-responding mixed or deep IH. Use of IL TMC seems reasonable for IH not responding to propranolol.

Study of Thoracoscopic Repair of Diaphragmatic Eventration in Children: A Case Series.

Background: To present a case series of children with eventration of diaphragm who underwent thoracoscopic repair highlighting the technical points and surgical modifications to prevent a recurrence. Settings and Design: This is an observational study of patients with diaphragmatic eventration admitted to a tertiary care institute. The study was designed following CARE guidelines endorsed by EQUATOR Network. Pediatric patients who had undergone thoracoscopic repair between January 2010 and March 2019 were included in the study. Materials and Methods: Age at surgery, gender, weight, site of the lesion, operating time, need for postoperative drain, and complications were assessed. Results: Twenty-six patients had thoracoscopic repair of the eventration of diaphragm. The male-female ratio was 12:1 and mean weight at the time of surgery was 6.3 kg (2.2-22 kg) with most patients having left side congenital diaphragmatic eventration (n = 21) as compared with the right side (n = 5). The average operating time was 66 minutes (37-144 minutes). Conclusions: Diaphragm plication by a thoracoscopic approach is safe and feasible in neonates and pediatric patients.