Management of urethral fistula after hypospadias repair with particular reference to purse-string sutures: a 24-year review.

PURPOSE: To review our management of urethral fistulae following hypospadias repair over a 24-year period. To showcase our innovations, particularly the purse-string closure technique. METHODS: We reviewed our prospectively maintained database from 1997 to 2020 to identify patients with fistula. Two main surgical techniques were used: traditional linear closure and purse-string suture. Other innovations included anchoring skin to corpora and intersectional skin closure. RESULTS: Sixty two patients presented with 73 urethral fistulae after hypospadias repair. 55/62 were operated: 28-purse-string technique, 23-linear closure, 4-redo urethroplasty. Cure after the first attempt was achieved in 26/28 (93%) in the purse-string group and 16/23 (70%) in the linear group (p = 0.015). Spontaneous resolution occurred in 6/62 patients (9.6%), in 3 spontaneously and in 3 after a single dilation. 1 patient awaits surgery. Closure after first fistula repair was 22/24 (92%) in distal hypospadias and 20/27 (74%) in proximal hypospadias (p = 0.051). CONCLUSION: Remarkably, conservative management was succesful in almost 10% of urethral fistulae, either spontaneously or after a single dilation. Purse-string closure, rarely described in the literature, coupled with our other innovations, helped bring down our recurrence rates significantly with no patient needing more than two surgeries for cure.

Technical Innovations to Reduce Complication Rates in Esophageal Atresia with Particular Reference to Long-term Outcomes: A Single Surgeon's Experience of 22 Years.

Background: Following esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, the standard leak rate reported in the literature is 5%-10%, and stricture rate is 40%-72%. There is a global quest for surgical innovations to drive down these complication rates which can cause considerable morbidity. Methods: A prospectively maintained database of the senior author's patients who had esophageal atresia repair from 1995 to 2016 was reviewed. Two distinct innovations were implemented: (1) adequate or generous mobilization of the lower esophageal pouch and (2) a 2-5 mm slit in distal esophagus to widen its circumference. Results: Forty-three patients with EA/TEF were reviewed. Of those, 40 underwent primary repair. The median follow-up was 12.5 years (range 4-26 years). There were no anastomotic leaks and only 8 (20%) patients developed anastomotic strictures requiring dilations (1-5 dilations/patients). One patient (2.5%) had a recurrent fistula. One early mortality was recorded. At the latest follow-up, 35 (87.5%) patients had normal oral feeding, while 1 (2.5%) patient had occasional food sticking episodes. Four syndromic patients (10%) were on jejunal or gastrostomy feeding. Conclusion: An adequate or generous mobilization of the distal esophageal pouch, together with a 2-5 mm slit in the distal esophagus, achieves a tension-free and wide anastomosis. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale with a small slit, helps. These seemingly minor innovations, when used together, contributed to a substantially lower complication rate sustained over a 22-year period - no leaks and only 20% stricture rate.

Necrotizing Enterocolitis in Neonates: Has the Brain Taken a Hit 10 Years Later?

BACKGROUND: The neonate with necrotizing enterocolitis (NEC) is at risk of developing poor neurodevelopmental outcomes. There is a dearth of long-term follow-up studies in this field, with a majority of studies reporting a follow-up duration of 2 years. The aim of this study was to assess neurodevelopment of babies diagnosed with NEC more than a decade ago. This study was carried out in a tertiary hospital with neonatal surgery and intensive care units. MATERIALS AND METHODS: Retrospective review of notes and telephone interviews with parents of babies diagnosed with NEC between January 2007 and December 2008 was conducted. Evidence of motor, cognitive, and sensory impairment was recorded. Fisher's exact, χ2, and unpaired t-tests were used. P-values <0.05 were considered significant. RESULTS: Overall mortality in this cohort was 31%. Eighteen patients were followed up to an average age of 11.2 years. Of the 18 patients, 11 (61%) had a neurological impairment. Of the 15 surgically managed patients, 10 (67%) had an impairment and, of the 3 medically managed patients, 1 (33%) had an impairment. Cognitive impairment was the most common (10/18, 56%), followed by motor (6/18, 33%). Ten of 18 (56%) had special education needs, 9 of 18 (50%) had learning difficulties, 6 of 18 (33%) had speaking difficulties, and 4 of 18 (22%) had cerebral palsy. Patients also had behavioral conditions (3/18, 17%), visual impairment (2/18, 11%), and seizures (2/18, 11%). CONCLUSION: In the field of NEC, there is a hidden neurological burden that neonatal surgeons bequeath to the community. Sixty-one percent of patients are neurologically impaired, affecting the quality of life and function in the long-term. There should be appropriate parent counseling at the point of diagnosis and regular development checks for children with NEC.

Urethral fistulae following surgery for scrotal or perineal hypospadias: A 20-year review.

INTRODUCTION: Urethral fistulae (UF) following hypospadias surgery can be a frustrating complication with reports of even 15 attempts to close a difficult fistula (Richter 2003). UF occurring in scrotal or perineal hypospadias (SPH) pose a further challenge because of the under-virilised penis. OBJECTIVE: To review the outcomes of a single surgeon's experience over 20 years of managing UF in SPH. To analyse the traditional approach of fistula closure and three alternative techniques. MATERIALS AND METHODS: A prospectively maintained database of patients who underwent hypospadias surgery for SPH from January 1997 to September 2018 was reviewed. Patients with UF were identified and their data recorded. The techniques of fistula closure were: a-Traditional approach. b Purse-string closure. c. Right angle intersection technique where the skin and urethra are closed at right angles to each other. d Anchoring skin to corpora away from the fistula closure. RESULTS: 32 patients with 41 fistulae were identified. Follow-up ranged from 1 to 18 years. 10/32 (31%) had concomitant meatal stenosis or urethral strictures. The sites of UF were: penoscrotal 19/41 (46%), midpenile 14/41 (34%), coronal or subcoronal in 8/41 (20%). One fistula resolved spontaneously after a single urethral dilatation. 4 patients with a coronal fistula were laid open to the glandular meatus creating a coronal hypospadias, with redo-urethroplasty later (in 2 a buccal graft was used). Of the rest, 29 fistulae were cured after one surgery, while 7 needed two attempts. No patient needed more than two surgeries to close the fistula. The recurrence rates were as follows- Purse-string suture: 10%, Right angle intersection technique: 14.3%, Anchoring skin to corpora: 16.7%, Traditional approach: 21.7%. Although the purse-string suture technique had the lowest recurrence rate, the figures did not reach statistical significance (P-0.95). CONCLUSION: Urethral fistulae occurring in SPH pose challenges because of the under-virilised penis. A third of patients may have meatal stenosis or urethral strictures which must be identified and dealt with. The three novel techniques we employed helped drive down our recurrence rate. Purse string sutures reduce the weak area to a dot and are an excellent way to deal with small fistulae (<5 mm). Other innovations include offsetting the skin suture line by anchoring it to the penile shaft well away from the fistula repair or closing the fistula and skin at right angle to each other. The fact that no patient needed more than two operations to lose the UF, was gratifying.

Treatment of megarectum in anorectal malformation with emphasis on preventive aspects: 17 years experience.

PURPOSE: Megarectum in anorectal malformation (ARM) causes severe morbidity. To compare conservative management (CM) of megarectum with excision (EX), to propose a new classification and to analyse management strategies. METHODS: Between 2000-2016, we reviewed all ARM to identify megarectum, defined by radiological recto-pelvic ratio > 0.61. A new classification was proposed: primary megarectum (PM) pre-anorectoplasty, and secondary megarectum (SM) post-anorectoplasty, with sub-types. Complications and Krickenbeck bowel function were compared between CM and EX. RESULTS: Of 124 ARM, 22 (18%) developed megarectum; of these, 7 underwent EX. There was no difference in functional outcomes when comparing CM vs EX-voluntary bowel movement (both 86%), soiling (40% vs. 57%) and constipation (both 86%). However, EX was associated with major complications (43%) and the requirement for invasive bowel management, compared to CM (85% vs. 27%, P = 0.02). 6/7 EX needed antegrade continence enema (ACE), one of these has a permanent ileostomy. With strategic changes, incidence of megarectum reduced from 20/77 (26%) to 2/47 (4%) after 2013 (P = 0.002). CONCLUSION: EX did not confer benefit in the functional outcome but carried a high risk of complications, often needing ACE or stoma. By adhering to strategies discussed, we reduced the incidence of megarectum and have avoided EX since 2013.

Challenges in managing proximal hypospadias: A 17-year single-center experience.

BACKGROUND: There are only a few publications in the medical literature reporting on complication rates in proximal hypospadias surgery, particularly with regard to long-term follow-up. METHODS: Over a 17.5-year period, we operated 100 patients with penoscrotal, scrotal and perineal hypospadias. Sixty-four had a single-stage repair, including 15 who received a buccal mucosa inlay "Snodgraft" repair. Thirty-six had a two-stage Bracka repair of which 19 received buccal or lower lip grafts and 17 had preputial grafts. Overall, 34 patients received buccal grafts. The median follow-up was eight years (range 1-16 years). Three patients were operated for residual chordee years later. RESULTS: Urethral fistulae occurred in a total of 26/100 (26.0%) cases, meatal stenosis in 16/100 (16.0%), wound breakdown in six (6.0%) and graft failure in one (1.0%). The fistula rate after the single-stage approach was 15/64 (23.4%), whereas it was 11/36 (30.6%) following two-stage repair (P = 0.4811). CONCLUSIONS: Proximal hypospadias remains a challenging condition to treat. It is possible to perform a single-stage repair in 64.0% of cases. This brings down the median number of operations to only two. Lower lip grafts were used in 34.0% but are now used in redo-surgeries only. Our fistula rate was 26.0% but has decreased significantly in recent years. LEVEL OF EVIDENCE: Level III.

Post-operative strictures in anorectal malformation: trends over 15 years.

AIM: For decades, paediatric surgeons have employed the standard posterior sagittal anorectoplasty (PSARP) approach to deal with patients with anorectal malformations (ARM). In recent years, we noted an apparent increase in the incidence of anal stricture after surgical repair of ARM following the introduction of laparoscopic pull-through and techniques aiming to preserve the internal sphincter-the internal sphincter sparing approach (ISSA). We decided to analyse our data to find out if these new trends had added to the problem of post-operative strictures. METHODS: All patients with ARM at our institution from January 2000 to December 2015 were identified. A retrospective case note review was carried out. Data collected included patient demographics, type of ARM, operative details, and post-operative outcomes. RESULTS: 114 patients were identified. Ten patients were excluded. Of the remaining 104 children, 48 (46%) were female. Median age was 8.3 (range 1.2-16.8) years. Types of ARM were as follows: perineal fistula (15 patients), anterior stenotic anus (12), imperforate anus without fistula (10), vestibular fistula (32), rectourethral (bulbar) fistula (11), rectourethral (prostatic) fistula (14), rectovesical fistula (7), and cloaca (3). Twenty-seven patients with a perineal fistula or anterior stenotic anus underwent perineal procedures that were variably described by the different operating surgeons. The majority (15 patients) had an anoplasty, 5 had anal transposition, 5 had limited PSARP, and 2 patients had ISSA. Two patients with a cloacal anomaly underwent open cloacal reconstruction. Of the remaining 75 patients, 45 had a PSARP approach, 6 had a laparoscopic-assisted pull-through, and 18 had ISSA. Four girls with vestibular fistula had anal transposition and two boys with imperforate anus without fistula had anoplasty. 15 (14%) children developed anal stricture. Stricture incidence differed according to operation type. PSARP was the most commonly performed procedure, with only 6% developing a stricture. In contrast, 30% of ISSA patients and 50% of children who had laparoscopic pull-through developed a stricture. Strictures also occurred in 11 and 12% of children having anal transposition and anoplasty, respectively. CONCLUSION: The laparoscopic-assisted pull-through involves tunnelling the sphincter muscle complex. We found that often the tunnels were not wide enough, resulting in narrowing not just at the ano-cutaneous junction but also at the deeper level. 50% developed strictures. We have modified our technique by ensuring that the tunnels are generous enough to allow the rectum to be pulled through without any resistance. ISSA unfortunately resulted in 30% of our patients developing strictures. This approach, started in 2004, was, therefore, abandoned in 2013. The standard Pena's PSARP, with or without a laparotomy, has stood the test of time. Any modification of this approach must be carefully thought through and audited meticulously. Strictures can cause significant morbidity, which may need several revisions, and the resulting redo anoplasties run the risk of sphincter damage, ironically which the newer modifications of ISSA were trying to conserve.

Surgical management of urethral prolapse in girls: 13 years' experience.

UNLABELLED: Study Type - Therapy (case series) Level of Evidence 4 What's known on the subject? and What does the study add? Urethral prolapse (UP) is a rare condition, with a suggested incidence of one in 3000. It occurs most often in prepubertal, primarily Black, girls. The underlying cause of this condition remains uncertain, although a lack of oestrogen is thought to have a role, owing to the preponderance of the condition in the prepubertal and postmenopausal age groups. A popular theory is that the problem arises as a consequence of poor attachments between the two layers of smooth muscle surrounding the urethra, combined with episodic increases in intraabdominal pressure. The most common presentation of UP is genital bleeding or a mass. The classical appearance of UP (i.e. the 'doughnut' sign) enables diagnosis to be made easily on clinical grounds alone. Optimum management of UP is less certain, with opinion divided on the merits of conservative therapy vs surgical excision. Conservative therapy aims to reduce mucosal oedema, improve local hygiene and counteract lack of oestrogen by using a combination of any or all of the following: Sitz baths, topical oestrogen cream, antibacterial wash/soap and topical antibiotics. Surgical management of UP involves excision of the prolapsed mucosa circumferentially. Several authors have reported success with surgical excision, but it carries a risk of developing stenosis of the urethral opening. The present study supports previously reported findings by other authors in terms of demographics and clinical presentation. Patient ages ranged from 2 to 15 years and all girls were of Black race. They most commonly presented with a mass (8/21 patients) or bleeding (6/21 patients) and diagnosis was confirmed on clinical examination, although one required a general anaesthetic (GA) to complete the examination. The present study shows that, in mild cases (usually where there is a mass without symptoms), UP can be successfully managed using conservative measures. In our practice, this involves the use of Sitz baths. More importantly, the study shows that in cases with more symptomatic prolapse or with evidence of vascular compromise, there is an alternative to a surgical procedure and its potential complications. We have found reducing the prolapse under a GA to be beneficial. Complete reduction was achieved in 3/7 patients, with no recurrence. The remaining four patients with partial reduction had improvement in symptoms, allowing conservative therapy to continue and resulting in complete or almost complete resolution of prolapse at follow-up. This approach has not been described previously in published literature on UP. OBJECTIVE: To review our experience of managing urethral prolapse (UP) in girls. PATIENTS AND METHODS: A total of 21 girls, all of whom were Black and whose age range was 2-15 years, were diagnosed with UP between 1995 and 2008. Case notes were reviewed for age, symptoms, clinical findings, predisposing factors, management and outcomes. RESULTS: Presenting symptoms were: mass (n= 8), bleeding (n= 6), dysuria/straining at micturition (n= 6), discharge (n= 1) and constipation (n= 1). In all, 13 patients were managed conservatively because their symptoms were mild. Seven patients underwent prolapse reduction under general anaesthetic (GA). In one patient, an examination under anesthesia was done to confirm the diagnosis as bedside examination was not possible. Prolapse reduction was complete in only three patients. Two patients had partial reduction, which resolved over the next 3 months. Two patients continue to have minimal residual prolapse. A causative/precipitating factor was found in only one patient (severe chronic constipation). She had a recurrence 2 years after reduction. There were no other recurrences. CONCLUSIONS: UP in girls can be diagnosed clinically in most cases. Girls with mild symptoms can be managed conservatively. For girls with more significant symptoms, we recommend a simple reduction under GA. This may be curative, or may reduce the prolapse significantly. Surgical excision is almost never required.

Neonatal functional intestinal obstruction and the presence of severely immature ganglion cells on rectal biopsy: 6 year experience.

PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. CONCLUSION: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.