Interstitial cells of Cajal: clinical relevance in pediatric gastrointestinal motility disorders.

Interstitial cells of Cajal (ICCs) are pacemaker cells of gastrointestinal motility that generate and transmit electrical slow waves to smooth muscle cells in the gut wall, thus inducing phasic contractions and coordinated peristalsis. Traditionally, tyrosine-protein kinase Kit (c-kit), also known as CD117 or mast/stem cell growth factor receptor, has been used as the primary marker of ICCs in pathology specimens. More recently, the Ca2+-activated chloride channel, anoctamin-1, has been introduced as a more specific marker of ICCs. Over the years, various gastrointestinal motility disorders have been described in infants and young children in which symptoms of functional bowel obstruction arise from ICC-related neuromuscular dysfunction of the colon and rectum. The current article provides a comprehensive overview of the embryonic origin, distribution, and functions of ICCs, while also illustrating the absence or deficiency of ICCs in pediatric patients with Hirschsprung disease intestinal neuronal dysplasia, isolated hypoganglionosis, internal anal sphincter achalasia, and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome.

Delayed primary anastomosis for repair of long-gap esophageal atresia: technique revisited.

The operative management of patients born with long-gap esophageal atresia (LGEA) remains a major challenge for most pediatric surgeons, due to the rarity and complex nature of this malformation. In LGEA, the distance between the proximal and distal esophageal end is too wide, making a primary anastomosis often impossible. Still, every effort should be made to preserve the native esophagus as no other conduit can replace its function in transporting food from the oral cavity to the stomach satisfactorily. In 1981, Puri et al. observed that in newborns with LGEA spontaneous growth and hypertrophy of the two segments occur at a rate faster than overall somatic growth in the absence of any form of mechanical stretching, traction or bouginage. They further noted that maximal natural growth arises in the first 8-12 weeks of life, stimulated by the swallowing reflex and reflux of gastric contents into the lower esophageal pouch. Since then, creation of an initial gastrostomy and continuous suction of the upper esophageal pouch followed by delayed primary anastomosis at approximately 3 months of age has been widely accepted as the preferred treatment option in most LGEA cases, generally providing good functional results. The current article offers a comprehensive update on the various aspects and challenges of this technique including initial preoperative management and subsequent gap assessment, while also discussing potential postoperative complications and long-term outcome.

Multidisciplinary Treatment Strategies for Wilms Tumor: Recent Advances, Technical Innovations and Future Directions.

Significant progress has been made in the management of Wilms tumor (WT) in recent years, mostly as a result of collaborative efforts and the implementation of protocol-driven, multimodal therapy. This article offers a comprehensive overview of current multidisciplinary treatment strategies for WT, whilst also addressing recent technical innovations including nephron-sparing surgery (NSS) and minimally invasive approaches. In addition, surgical concepts for the treatment of metastatic disease, advances in tumor imaging technology and potentially prognostic biomarkers will be discussed. Current evidence suggests that, in experienced hands and selected cases, laparoscopic radical nephrectomy and laparoscopic-assisted partial nephrectomy for WT may offer the same outcome as the traditional open approach. While NSS is the standard procedure for bilateral WT, NSS has evolved as an alternative technique in patients with smaller unilateral WT and in cases with imminent renal failure. Metastatic disease of the lung or liver that is associated with WT is preferably treated with a three-drug chemotherapy and local radiation therapy. However, surgical sampling of lung nodules may be advisable in persistent nodules before whole lung irradiation is commenced. Several tumor markers such as loss of heterozygosity of chromosomes 1p/16q, 11p15 and gain of function at 1q are associated with an increased risk of recurrence or a decreased risk of overall survival in patients with WT. In summary, complete resection with tumor-free margins remains the primary surgical aim in WT, while NSS and minimally invasive approaches are only suitable in a subset of patients with smaller WT and low-risk disease. In the future, advances in tumor imaging technology may assist the surgeon in defining surgical resection margins and additional biomarkers may emerge as targets for development of new diagnostic tests and potential therapies.

Genetically Modified Mouse Models of Congenital Diaphragmatic Hernia: Opportunities and Limitations for Studying Altered Lung Development.

Congenital diaphragmatic hernia (CDH) is a relatively common and life-threatening birth defect, characterized by an abnormal opening in the primordial diaphragm that interferes with normal lung development. As a result, CDH is accompanied by immature and hypoplastic lungs, being the leading cause of morbidity and mortality in patients with this condition. In recent decades, various animal models have contributed novel insights into the pathogenic mechanisms underlying CDH and associated pulmonary hypoplasia. In particular, the generation of genetically modified mouse models, which show both diaphragm and lung abnormalities, has resulted in the discovery of multiple genes and signaling pathways involved in the pathogenesis of CDH. This article aims to offer an up-to-date overview on CDH-implicated transcription factors, molecules regulating cell migration and signal transduction as well as components contributing to the formation of extracellular matrix, whilst also discussing the significance of these genetic models for studying altered lung development with regard to the human situation.

Prenatal Detection of Congenital Duodenal Obstruction-Impact on Postnatal Care.

BACKGROUND: Duodenal obstruction is a rare cause of congenital bowel obstruction. Prenatal ultrasound could be suggestive of duodenal atresia if polyhydramnios and the double bubble sign are visible. Prenatal diagnosis should prompt respective prenatal care, including surgery. The aim of this study was to investigate the rate and importance of prenatally diagnosed duodenal obstruction, comparing incomplete and complete duodenal obstruction. METHODS: A retrospective, single-center study was performed using data from patients operated on for duodenal obstruction between 2004 and 2019. Prenatal ultrasound findings were obtained from maternal logbooks and directly from the investigating obstetricians. Postnatal data were obtained from electronic charts, including imaging, operative notes and follow-up. RESULTS: A total of 33/64 parents of respective patients agreed to provide information on prenatal diagnostics. In total, 11/15 patients with complete duodenal obstruction and 0/18 patients with incomplete duodenal obstruction showed typical prenatal features. Prenatal diagnosis prompted immediate surgical treatment after birth. CONCLUSION: Prenatal diagnosis of congenital duodenal obstruction is only achievable in cases of complete congenital duodenal obstruction by sonographic detection of the pathognomonic double bubble sign. Patients with incomplete duodenal obstruction showed no sign of duodenal obstruction on prenatal scans and thus were diagnosed and treated later.

Surgical Management of Pediatric Inguinal Hernia: A Systematic Review and Guideline from the European Pediatric Surgeons' Association Evidence and Guideline Committee.

INTRODUCTION: Inguinal hernia repair represents the most common operation in childhood; however, consensus about the optimal management is lacking. Hence, recommendations for clinical practice are needed. This study assesses the available evidence and compiles recommendations on pediatric inguinal hernia. MATERIALS AND METHODS: The European Pediatric Surgeons' Association Evidence and Guideline Committee addressed six questions on pediatric inguinal hernia repair with the following topics: (1) open versus laparoscopic repair, (2) extraperitoneal versus transperitoneal repair, (3) contralateral exploration, (4) surgical timing, (5) anesthesia technique in preterm infants, and (6) operation urgency in girls with irreducible ovarian hernia. Systematic literature searches were performed using PubMed, MEDLINE, Embase (Ovid), and The Cochrane Library. Reviews and meta-analyses were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement. RESULTS: Seventy-two out of 5,173 articles were included, 27 in the meta-analyses. Laparoscopic repair shortens bilateral operation time compared with open repair. In preterm infants, hernia repair after neonatal intensive care unit (NICU)/hospital discharge is associated with less respiratory difficulties and recurrences, regional anesthesia is associated with a decrease of postoperative apnea and pain. The review regarding operation urgency for irreducible ovarian hernia gained insufficient evidence of low quality. CONCLUSION: Laparoscopic repair may be beneficial for children with bilateral hernia and preterm infants may benefit using regional anesthesia and postponing surgery. However, no definite superiority was found and available evidence was of moderate-to-low quality. Evidence for other topics was less conclusive. For the optimal management of inguinal hernia repair, a tailored approach is recommended taking into account the local facilities, resources, and expertise of the medical team involved.

Persistent bowel dysfunction after surgery for Hirschsprung's disease: A neuropathological perspective.

Hirschsprung's disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.

Impact of the Global COVID-19 Pandemic on the Incidence, Presentation, and Management of Pediatric Appendicitis: Lessons Learned from the First Wave.

The fast-evolving nature of the coronavirus disease 2019 (COVID-19) pandemic has led to unprecedented clinical, logistical, and socioeconomical challenges for health-care systems worldwide. While several studies have analyzed the impact on the presentation and management of acute appendicitis (AA) in the adult population, there is a relative paucity of similar research in pediatric patients with AA. To date, there is some evidence that the incidence of simple AA in children may have decreased during the first lockdown period in spring 2020, whereas the number of complicated AA cases remained unchanged or increased slightly. Despite a worrying trend toward delayed presentation, most pediatric patients with AA were treated expediently during this time with comparable outcomes to previous years. Hospitals must consider their individual capacity and medical resources when choosing between operative and non-operative management of children with AA. Testing for severe acute respiratory syndrome coronavirus type 2 is imperative in all pediatric patients presenting with fever and acute abdominal pain with diarrhea or vomiting, to differentiate between multisystem inflammatory syndrome and AA, thus avoiding unnecessary surgery. During the further extension of the COVID-19 crisis, parents should be encouraged to seek medical care with their children early in order that the appropriate treatment for AA can be undertaken in a timely fashion.

C-reactive protein/albumin ratio is a prognostic indicator for predicting surgical intervention and mortality in neonates with necrotizing enterocolitis.

PURPOSE: The role of hypoalbuminemia and raised C-reactive protein (CRP) levels in predicting critical prognosis has been described extensively in adult literature. However, there are limited studies in pediatrics, particularly neonates. The CRP/albumin (CRP/ALB) ratio is often associated with higher mortality, organ failure and prolonged hospital stay. We hypothesized that the serum CRP/ALB ratio has a prognostic value in predicting surgery and mortality in neonates with necrotizing enterocolitis (NEC). METHODS: Retrospective review of all neonates with clinical and radiological evidence of non-perforated NEC that were treated in a tertiary-level referral hospital between 2009 and 2018. General patient demographics, laboratory parameters and outcomes were recorded. Receiver operating characteristics analysis was performed to evaluated optimal cut-offs and area under the curve (AUC) with 95% confidence intervals (CI). RESULTS: A total of 191 neonates were identified. Of these, 103 (53.9%) were born at ≤ 28 weeks of gestation and 101 (52.9%) had a birth weight of ≤ 1000 g. Eighty-four (44.0%) patients underwent surgical intervention for NEC. The overall survival rate was 161/191 (84.3%). A CRP/ALB ratio of ≥ 3 on day 2 of NEC diagnosis was associated with a statistically significant higher likelihood for surgery [AUC 0.71 (95% CI 0.63-0.79); p < 0.0001] and mortality [AUC 0.66 (95% CI 0.54-0.77); p = 0.0150], respectively. CONCLUSIONS: A CRP/ALB ratio of ≥ 3 on day 2 is indicative of a critical pathway in neonates with radiologically confirmed, non-perforated NEC. This could be used as an additional criterion to guide parental counselling in NEC for surgical intervention and mortality.

Ovarian lesions and tumors in infants and older children.

Objectives: Ovarian lesions are rare but frequent in children. Patients could present with abdominal pain, but ovarian lesions could also be incidentally found on ultrasound. Awareness is required in cases with acute, severe lower abdominal pain, as ovarian torsion could be the cause. Other lesions can be cysts or benign or malignant ovarian tumors. Thus, the aim of this paper is to review typical ovarian lesions according to age, imaging and laboratory findings, and surgical management. Methods: We retrospectively analysed the patient charts of 39 patients aged 10.4 ± 6.1 years (from 3 months to 18 years) with ovarian lesions treated in our institution between 01/2009 and 08/2020. All clinical and pathological findings of infants and children operated on for ovarian lesions were included. Results: Ovarian lesions in children younger than 2 years of age were typically ovarian cysts, and ovarian tumors were not observed in this age group. In older children over 10 years of age, tumors were more common - with mostly teratoma or other germ cell tumors, followed by epithelial tumors. Moreover, acute or chronic ovarian torsion was observed in all age groups. In general, ovarian tumors were much larger in size than ovarian cysts or twisted ovaries and eventually showed tumor marker expression of AFP or ß-HCG. Simple ovarian cysts or twisted ovaries were smaller in size. Surgery for all ovarian lesions should aim to preserve healthy ovarian tissue by performing partial ovariectomy. Conclusions: In adolescent girls with acute abdominal pain, immediate laparoscopy should be performed to rule out ovarian torsion. Careful imaging evaluation and the assessment of tumor markers should be performed in painless ovarian lesions to indicate an adequate surgical ovarian-sparing approach.

Pbx1, Meis1, and Runx1 Expression Is Decreased in the Diaphragmatic and Pulmonary Mesenchyme of Rats with Nitrofen-Induced Congenital Diaphragmatic Hernia.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) and associated pulmonary hypoplasia (PH) are thought to originate from mesenchymal defects in pleuroperitoneal folds (PPFs) and primordial lungs. Pre-B-cell leukemia homeobox 1 (Pbx1), its binding partner myeloid ecotropic integration site 1 (Meis1), and runt-related transcription factor 1 (Runx1) are expressed in diaphragmatic and lung mesenchyme, functioning as transcription cofactors that modulate mesenchymal cell proliferation. Furthermore, Pbx1 -/- mice develop diaphragmatic defects and PH similar to human CDH. We hypothesized that diaphragmatic and pulmonary Pbx1, Meis1, and Runx1 expression is decreased in the nitrofen-induced CDH model. MATERIALS AND METHODS: Time-mated rats were exposed to nitrofen or vehicle on gestational day 9 (D9). Fetal diaphragms (n = 72) and lungs (n = 48) were microdissected on D13, D15, and D18, and were divided into control and nitrofen-exposed specimens. Diaphragmatic and pulmonary gene expression levels of Pbx1, Meis1, and Runx1 were analyzed by quantitative real-time polymerase chain reaction. Immunofluorescence-double-staining for Pbx1, Meis1, and Runx1 was combined with mesenchymal/myogenic markers Gata4 and myogenin to evaluate protein expression. RESULTS: Relative mRNA expression of Pbx1, Meis1, and Runx1 was significantly decreased in PPFs (D13), developing diaphragms/lungs (D15), and muscularized diaphragms/differentiated lungs (D18) of nitrofen-exposed fetuses compared with controls. Confocal-laser-scanning-microscopy revealed markedly diminished Pbx1, Meis1, and Runx1 immunofluorescence in diaphragmatic and pulmonary mesenchyme, associated with less proliferating mesenchymal cells in nitrofen-exposed fetuses on D13, D15, and D18 compared with controls. CONCLUSION: Decreased Pbx1, Meis1, and Runx1 expression during diaphragmatic development and lung branching morphogenesis may reduce mesenchymal cell proliferation, causing malformed PPFs and disrupted airway branching, thus leading to diaphragmatic defects and PH in the nitrofen-induced CDH model.

Anorectal manometry in pediatric settings: A systematic review of 227 studies.

BACKGROUND: There is wide variation in the clinical use of diagnostic tools for children with chronic constipation and functional/structural fecal incontinence (CCFSFI). Anorectal manometry (ARM) is a well-recognized technique to assess the function of the anorectum. PURPOSE: Our aim was to perform an up-to-date review on ARM in pediatric patients with CCFSFI, with specific focus on the indication of use and protocol. Variation of its use in pediatrics will be explored. METHODS: A systematic search was conducted for empirical studies utilizing ARM with a pediatric sample. A keyword search of literature published in English before July 2018 was conducted and updated to October 2019. Data on demographics, clinical information, study aims, ARM parameters and use of sedation/anesthesia were collected. KEY RESULTS: A total of 227 studies were included in this systematic review. The age of study participants at the time of ARM ranged from birth to 18 years. ARM was most commonly used in patients with organic conditions (65%) compared to functional constipation (41%). In almost half [108/227 (48%)] of the studies, ARM was performed awake. The ARM parameters most frequently assessed were the rectoanal inhibitory reflex, which was evaluated in 198/227 studies (87%) and the anal resting pressure [166/227 studies (73%)]. CONCLUSIONS AND INFERENCES: This systematic review has highlighted the vast variation of ARM use within pediatrics and the need to strive toward standardization and use of consensus guidelines. We anticipate this will further advance our understanding of the pathophysiological mechanisms involved in children with defecation disorders.

Gastroesophageal Reflux Disease and Need for Antireflux Surgery in Children with Cystic Fibrosis: A Systematic Review on Incidence, Surgical Complications, and Postoperative Outcomes.

INTRODUCTION: Gastroesophageal reflux disease (GERD) is associated with accelerated decline in lung health in children with cystic fibrosis (CF). Thus, antireflux surgery (ARS) is offered to a selected CF cohort with refractory GERD, but outcomes remain poorly investigated. This study aimed to determine the incidence of GERD in children with CF and to evaluate complications and outcomes of ARS. MATERIALS AND METHODS: A systematic literature-based search was conducted using various online databases according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The number of GERD cases in pediatric CF cohorts who underwent diagnostic investigation(s) was recorded. Data on postoperative complications and outcomes (including symptoms, lung function, and nutritional status) following ARS were analyzed. RESULTS: Ten articles (n = 289 patients) met the defined inclusion criteria (51% male; age range, 0.5 month-36 years). The overall incidence of GERD was 46% (range, 19-81%), derived from seven studies (n = 212 patients). Four publications (n = 82 patients) reported on ARS due to uncontrolled GERD. All ARSs were Nissen fundoplication (majority with gastrostomy placement). Major postoperative complications occurred in 15 (18%) patients, two required redo-ARS. Median follow-up time was 2 years (range, 3 months-6 years); 59% showed symptom improvement, and pulmonary exacerbations and decline in lung function were reduced. Nutritional status mainly improved in milder CF cases. There were no deaths related to ARS. CONCLUSION: Approximately half of pediatric CF patients have GERD. Published data for children with CF are limited and heterogeneous in terms of GERD diagnosis and outcomes following ARS. However, ARS has shown to slow the deterioration of lung function in CF.

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome.

INTRODUCTION: The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise. MATERIALS AND METHODS: An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association. RESULTS: Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively; p = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A (p = 0.09 and p = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively; p = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) (p = 0.02). CONCLUSION: Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.

Diagnostic Workup of Neonates With Esophageal Atresia: Results From the EUPSA Esophageal Atresia Registry.

Aim: Controversies exist on the optimal diagnostic workup for neonates with esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). Aim of this study was to describe the current diagnostic policies in EA/TEF patients enrolled in an International multicenter registry. Methods: All patients consecutively registered from July 2014 to December 2017 in the EUPSA Esophageal Atresia Registry (EUPSA-EAR) were included in the study. Data related to diagnostic investigations among Centers forming the EUPSA-EAR were analyzed. Main Results: During the study period, 374 consecutive patients were recorded by 23 Centers. The majority of patients underwent chest X-rays, echocardiography, abdominal ultrasound, and abdominal X-rays. Preoperative bronchoscopy and esophageal gap measurement were performed in one third of the patients. Conclusions: Present data from a large cohort of patients from the EUPSA-EAR show both inter-institutional and intra-institutional variability in diagnostic workup of patients with EA/TEF. Efforts should be made to develop guidelines on the diagnostic workup for EA/TEF patients.

Low serum albumin concentration predicts the need for surgical intervention in neonates with necrotizing enterocolitis.

PURPOSE: To investigate whether serum albumin (SA) concentration can predict the need for surgical intervention in neonates with necrotizing enterocolitis (NEC). METHODS: Retrospective review of all cases with NEC Bell's stage 2 and 3 that were treated in a single center between 2009 and 2015. Data on patient demographics, clinical parameters, laboratory findings and surgical status were recorded. Receiver operating characteristics analysis was used to evaluate optimal cutoffs and predictive values. RESULTS: Overall, 151 neonates with NEC were identified. Of these, 132 (87.4%) had confirmed NEC Bell's stage 2. The median gestational age was 28.4 (range, 23.1-39.0) weeks and 69 (52.3%) had a birth weight of ≤1000 g. Sixty-eight (51.5%) underwent surgery, showing a sustained reduction in SA over time with significantly lower median SA levels compared to 64 (48.5%) cases that responded well to medical treatment (18.3 ±â€¯3.7 g/L vs. 26.0 ±â€¯2.0 g/L; P < 0.001). SA concentration of ≤20 g/L on day 2 of NEC diagnosis was a significant predictor for surgery (OR 3.41; P = 0.019) with a positive predictive value of 71.4%. CONCLUSIONS: An SA concentration of ≤20 g/L on day 2 of the NEC disease process is associated with a higher likelihood for surgical intervention in neonates with NEC Bell's stage 2. SA, in combination with other clinical parameters and serological markers, may be a useful predictive tool for surgery in NEC. LEVEL OF EVIDENCE: II.

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.