Laparoscopic Cystogastrostomy in Children with Pancreatic Pseudocysts: A Preliminary Experience of Eight Cases.

Introduction: Pancreatic pseudocysts (PPCs) and walled-off necrosis (WON) in children following acute pancreatitis are uncommon. The various modalities of therapy possible are conservative treatment, external drainage, endoscopic stenting, and internal surgical drainage procedures. There are no existing guidelines for the management of PPC in children. We evaluate the outcomes of laparoscopic cystogastrostomy (LCG) performed at our center. Materials and Methods: Eight children (median age: 10 years) underwent LCG for large PPC (median size: 12.5 cm). There were seven patients with PPC and one with WON. Seven underwent LCG by a transgastric approach and one underwent LCG by a retrogastric approach. Results: Seven out of the eight patients had complete resolution of symptoms and the PPC. The median follow-up period was 32 months (interquartile range: 9.5-55.5 months). There were no conversions. There was one patient with a WON who developed a recurrence. Conclusion: LCG is a safe and effective treatment option for large PPC/WON in children. A posterior retrogastric approach, when indicated, is a safe approach with a comparable outcome.

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.

Working classification and the quest for an effective, reconstructive management strategy in Y duplication of male urethra.

INTRODUCTION: Y-duplication is a rare subtype of urethral duplication whose surgical correction has traditionally yielded less than satisfactory results. Herein we report 18 patients, 17 of whom have completed successful urethral reconstruction. MATERIALS AND METHODS: A retrospective analysis was done on 18 children who have undergone repair of Y-duplication urethra in two tertiary care institutes from 2013 to 2020. The various subtypes encountered were classified in order to develop a rationale for reconstruction based on the underlying pathology. The various modalities used for reconstruction and the outcomes of the repair were studied. All but one boy voided mainly via the posterior (ventral) channel opening in the ano-rectum or perineum. These boys had the urethral reconstruction incorporating the healthy proximal ventral urethra at its origin. Further reconstruction up to the glans tip depended on the degree of dorsal (orthotropic) urethral patency, which forms the basis of our classification into Types I (completely stenotic), II (penile urethra patent), III (penile and bulbar urethra patent) and IV (fully patent dorsal urethra). RESULTS: Reconstruction resulting in voiding via a glanular or coronal meatus was completed in 17 boys. One boy is awaiting further surgery while being dependent on Mitrofanoff CIC. While urethral continuity could be achieved using patent segments of native urethra alone into two boys (Types II and III), all other children needed a neo-urethral segment to replace (Type I) or supplement (Type II) the dorsal urethra. Neo urethra was constructed from a tubularized preputial island flap (n = 11), Monti tube constructed from ileum (n = 3) or sigmoid colon (n = 4). A perineal operative exposure alone sufficed in 9, while the rest required an additional trans pubic approach. The only boy with Type IV anomaly underwent excision of the ventral urethral limb. Three boys with initially elevated serum creatinine have normal levels after reconstruction. CONCLUSION: Successful reconstruction of boys with Y- duplication of the urethra entailed elucidation of anatomical variations, adequate operative exposure (trans pubic and/or perineal) and innovative utilisation of local and/or distant tissues (preputial island flap, ileal/sigmoid Monti tubes).

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.

Outcome of end cutaneous ureterostomy (ECU) as a non conservative option in the management of primary obstructive megaureters (POM).

INTRODUCTION: Primary obstructive megaureters (POM) can be treated with one of the following options: conservative management with antibiotic chemoprophylaxis and active observation of the hydroureteronephrosis (HUN) until suspicion of renal deterioration; refluxing/non-refluxing ureteric reimplantation with antibiotic suppression; temporary double-J stenting; endoscopic balloon dilatation; endoureterotomy; and end cutaneous ureterostomy (ECU). OBJECTIVE: To study the profile of patients with POM and assess the efficacy, safety and outcome of ECU as an interim procedure. METHODS: A retrospective review was performed of patients who underwent ECU for POM between January 2004 and December 2014. Demographics, surgical details, and outcomes were studied. RESULTS: A total of 25 patients (19 males, six females) underwent ECU of 25 renal units for POM at a mean age of 7 months (range 23 days to 2.5 years). Of these, nine had presented with radiological worsening of antenatally detected HUN, 12 had symptoms (urosepsis in nine and palpable hydronephrosis in three), three had a solitary kidney in renal failure, and one had incidentally diagnosed renal cortical thinning as shown in Table below. Following diversion, renal failure had resolved in all, and febrile urinary tract infection (UTI) developed in one while awaiting reimplantation. Undiversion was performed in 21/25 patients at a mean duration of 12 months after diversion and a mean age of 19 months. In these, the ureteric size had decreased significantly at reimplantation. In 4/25, undiversion was not performed due to loss of follow-up in two and a subsequent nephrectomy in two. Two out of 21 developed febrile UTI after undiversion. The overall mean follow-up period was 34.2 months (n = 25), while the mean follow-up after undiversion was 41.5 months (n = 21). There was no incidence of stomal complications. CONCLUSIONS: End cutaneous ureterostomy was a safe and effective temporary procedure for the treatment of progressive primary obstructive megaureters.

Laparoscopic cholangiogram in biliary atresia: a refinement in the gallbladder hitch technique.

INTRODUCTION: The study describes a refinement in the gallbladder hitch stitch and assesses the value of the laparoscopic cholangiogram in children with suspected biliary atresia. METHODS: Twenty children with neonatal jaundice and no drainage as shown on the HIDA scan underwent a diagnostic laparoscopy through an umbilical 5 mm port. A 3 mm laparoscopic needle holder inserted through a 3.5 mm port to the left of the umbilicus was used to hitch the gallbladder to the abdominal wall. The stylet of a large bore 16F IV cannula then was used to penetrate the gallbladder to perform the laparoscopic cholangiogram. RESULTS: There was no need for conversion in all 20 children by this technique. Patent biliary anatomy was demonstrated in 11 children (11/20). These children had no further procedures. In 3 (3/20) children, the common bile duct was demonstrated, while the hepatic ducts were not. These children had a laparotomy for Kasai procedure after an open cholangiogram with a vascular bulldog clamp on the CBD confirmed the finding. Six (6/20) had no demonstrable patency; 3 had it confirmed when the abdomen was opened for the Kasai procedure; only those proceeding to Kasai portoenterostomy (3 hepatic duct atresia, 3 complete biliary atresias) had an epidural catheter placed by the anesthetist. The remaining 3 had no further procedure performed due to the advanced nodular liver with ascites and evidence of portal hypertension. CONCLUSION: The findings of laparoscopic cholangiogram were confirmed in all six children who underwent laparotomy for Kasai procedure. The laparoscopic cholangiogram using gallbladder hitch reliably demonstrates a patent biliary system (11/11) and was valuable in avoiding further invasive procedures in 70% (14/20) of babies.

Orbit technique in malrotation with non-obstructive volvulus: A novel technique of devolvulation.

INTRODUCTION: Volvulus may be noted in up to two-thirds of cases involving malrotation beyond infancy. Laparoscopic devolvulation has been described as a frustrating procedure because of restricted visualization. Here, a setup and technique that address these concerns are proposed. MATERIALS AND SURGICAL TECHNIQUE: Three boys (median age: 7 years) who had been diagnosed preoperatively with malrotation underwent laparoscopic exploration and devolvulation for volvulus found intraoperatively. The children were placed in a dorsal supine modified lithotomy position. Four 5-mm ports were inserted-one umbilically, one in the suprapubic region, and one in both the right and left iliac. After the volvulus was inspected, the right iliac atraumatic grasper was placed at the root of the mesentery. The bowel was devolvulated counterclockwise with the grasper used as a pivot-that is, the orbit technique. Once derotated, the rest of the operation proceeded with the division of Ladd's bands, the widening of the mesentery, and appendectomy. DISCUSSION: Devolvulation was successful in all three boys. The orbit technique is a useful devolvulation technique in non-obstructive volvulus when other techniques fail.

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.

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.

A retroperitoneal enteric duplication cyst communicating with the right upper ureter in an infant.

We report an extremely rare case of isolated retroperitoneal enteric duplication cyst with gastric mucosa causing haematuria and dysuria by communicating with the urinary system. A 9-month-old male child was admitted to our hospital with persistent haematuria, dysuria and anaemia. Investigations revealed a retroperitoneal cyst abutting the hydronephrotic non-functioning right kidney. At surgery an isolated retroperitoneal cyst communicating with the right pelviureteric junction was found. The kidney and associated cyst were excised. Histology of the cystic lesion revealed an enteric duplication cyst lined by ectopic gastric mucosa. Isolated retroperitoneal enteric duplication cyst communicating with the urinary tract has not been previously reported in the English literature. We propose that acid secretion into the right renal system was the cause of the haematuria-dysuria syndrome which promptly resolved postoperatively.

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.

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 peculiar cause for metabolic acidosis in the newborn.

Metabolic acidosis is often encountered in a sick neonate and intestinal duplication with heterotopic gastric mucosa is a well-established condition. We present a previously unreported relationship between neonatal metabolic acidosis, resulting from transperitoneal absorption of hydrochloric acid, and a ruptured non- communicating ileal duplication cyst with gastric mucosal heterotopia. The neonate recovered rapidly after resection of the ileal duplication. We present this case to highlight a rare but surgically correctable cause of neonatal metabolic acidosis.

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.