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The National Children's Research Centre (NCRC), the single largest paediatric research centre in Ireland, has been in existence for over 50 years and is located on the grounds of the largest children's hospital in Ireland; Children's Health Ireland at Crumlin. Professor Puri was appointed as the Director of the Research in 1989 and became President of the NCRC in 2009, a position he held until 2016. Professor Puri is one of the most cited paediatric surgical researchers in the world. His research work has been cited over 23,500 times in peer-reviewed articles with an h-index of 76 and i10-index of 494. The aim of this review is to analyse the most relevant areas of academic research at the NCRC, Dublin, during the years when Prof. Puri was Director/President of the NCRC. In addition, the relevant factors essential to create a successful paediatric surgical research centre will be discussed. A literature search using PubMed/Medline was carried out using the search terms "Prem Puri" over a 40-year period (1980-2020). Articles were analysed to identify the most significant research areas in the field of paediatric surgical research and the relevant laboratory and clinical findings. In addition, a separate analysis of successful funding and human factors, such as research fellows working at the NCRC, was also carried out. During the period under review, Professor Puri's team published 750 articles in peer-reviewed journals. Three main areas of research were reviewed with a total number of 391 articles: congenital diaphragmatic hernia (CDH) was the topic with the largest number of publications (153), followed by Hirschsprung's disease (HSCR) (144) and Vesicoureteral reflux (VUR) (94). Eighty research fellows, all paediatric surgeons, from 18 different countries were trained in basic science under the supervision of Professor Puri at the NCRC. Over the last three decades, the NCRC has been one of the most successful centres for paediatric surgical research in the world. The three areas of research with the largest number of publications were identified as CDH, HSCR and VUR. Various factors can explain the success of the NCRC: (a) the constant presence, for over 30 years, of a very successful paediatric surgeon leading the centre, (b) a multicultural laboratory with research fellows from all over the world and (c) grants of more than 15 million raised over the years, which guaranteed a constant flow of resources for laboratory research., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Megaprepuce (MP) is a rare and challenging condition characterised by an excessive inner prepuce, paucity of penile skin and an extremely narrow phimotic ring. The aetiological factors leading to its development are poorly understood. A variety of surgical techniques have been described in the last 26 years mostly with small number of patients and short follow-up. It is also highly likely that some series have in the past included different variants of inconspicuous penis combining concealed penis, MP and webbed penis. This article is a systematic review of the literature on Megaprepuce; in particular the embryology, history, aetiology, and the surgical techniques available for the correction of this unique penile anomaly will be presented and discussed in this study.

Aim: To compare the quality of life (QoL) in children with spina bifida with a control group of their peers using a validated questionnaire, the Neurogenic Bowel Dysfunction Score (NBDS)., Methods: The NBDS questionnaire was prospectively distributed to children attending a multi-disciplinary Spina Bifida clinic and healthy controls attending pediatric urology clinics. A score (out of 41) was assigned to each child based on their responses to the validated questionnaire. A lower score indicates better bowel function-related quality of life. SPSS software (v.25) was used for all statistical analysis., Results: There were 98 respondents to the questionnaire, 48 children with spina bifida and 50 controls. The average age of respondents was 7.88 years (3-16 years). Of those with Spina Bifida, 33 (69%) were on retrograde rectal irrigations, [19 (58%) Peristeen® system, 11 (33%) tube rectal irrigations, and 3 (9%) Willis system], 6 (12%) were on laxatives, and 9 (19%) were on no treatment. The median NBDS for Spina Bifida patients was significantly higher 13.5 (2-32) compared to the control group 2 (0-26, p < 0.001). Amongst Spina Bifida patients, there was no difference in quality of life between the modalities of bowel management (p = 0.203)., Conclusions: Despite active bowel management, children with spina bifida report a worse quality of life compared to the control group. In those with spina bifida, the lack of a difference between various bowel management strategies, including no treatment, indicates the need for a longitudinal study to evaluate the basis for this unexpected finding.

Purpose: To examine the sonographic measurements of pyloric muscle and pyloric thickness in premature infants with hypertrophic pyloric stenosis (HPS) and to correlate these measurements with patients variables. We aimed also to evaluate the clinical features and short-term outcome of HPS in premature compared to term infants., Methods: The medical notes and the pyloric ultrasounds of all premature infants (<37 weeks) admitted to the Neonatal Surgical Unit with HPS over a 20-year period (1990-2010) were retrospectively reviewed. Sonographic measurements of the pyloric length, muscle thickness and the pyloric ratio (muscle thickness divided by pyloric diameter) were correlated to weight at presentation, duration of symptoms and corrected gestational age. Multiple regression and correlation analysis was carried out., Results: Seventy-five preterm infants were identified (51M:24F). Median gestational age was 34 weeks. Median weight at presentation was 2.74 kg. Forty-two had an ultrasound (US) before surgery, 37 measurements were analysed. Pyloric length, muscle thickness and pyloric ratio were not affected by weight at presentation (p = 0.67, p = 0.122, p = 0.849), corrected gestational age (CGA) (p = 0.921, p = 0.678, p = 0.076), or duration of symptoms (p = 0.827, p = 0.268, p = 0.281). Mean length of stay was 6 days and overall complication rate was 27 %, both higher than reported in term infants (p < 0.001)., Conclusions: Premature infants with HPS have a higher female preponderance than expected. Ultrasound measurements in premature infants with HPS are not affected by weight, corrected gestational age or duration of symptoms. In addition, they have a longer hospital stay and increased complication rate compared to term infants.

Purpose: The diagnosis of children with disorders of sex development (DSD) requires a karyotype, different biochemical and radiological investigations in the context of a multidisciplinary team. The aim of this study was to compare the diagnostic accuracy of laparoscopy (L) versus ultrasonography (US) in the assessment of children with complex DSD., Methods: We retrospectively examined the theatre database searching for children with DSD who underwent laparoscopic surgery from 1999 to 2011. The medical and radiological records were reviewed., Results: Eighteen patients were identified. Age at diagnosis ranged from birth to 14 years (mean 2.5 years). There were seven patients with 46XY dysgenetic testicular DSD (4 mosaic Turner, 3 mixed gonadal dysgenesis), seven patients with 46XY non-dysgenetic testicular DSD (4 persistent Mullerian duct syndrome, 2 complete androgen insensitivity syndrome, one unknown), two patients with ovotesticular DSD, one patient with 46XX DSD (congenital adrenal hyperplasia) and one patient with 46XY DSD complete sex reversal. Fifteen underwent ultrasonography prior to laparoscopy. Both modalities identified Mullerian structures in seven (47 %) patients, in one (7 %) patient US and L confirmed the absence of Mullerian structures, while in six (40 %) patients there was discordance, with US failing to visualize pelvic Mullerian structures. In the last patient with 46XY non-dysgenetic testicular DSD, the rectum was thought to be a dilated uterus on ultrasonography., Conclusions: Pelvic ultrasonography failed to identify Mullerian structures in 40 % of patients with complex DSD. On the contrary, laparoscopy allowed excellent visualization of pelvic structures and gonads in children with complex DSD.

The Malone antegrade enema (MACE) and the caecostomy button (CB) are two methods of achieving colonic lavage in constipated children with faecal soiling. We reviewed our experience with the MACE and CB, aiming to compare results, complications, and outcomes. Between June 1998 and August 2002, 37 children (15 boys) underwent MACE and 12 children (9 boys) underwent CB for idiopathic constipation that had failed conventional treatment. Rectal biopsy was ganglionic in all cases. Mean age at surgery was 9.9 years for the MACE patients and 9.8 years for the CB patients. All children are under continuous review, and mean follow-up is 18 months. Statistical analysis of proportions used Fisher's exact test. Soiling stopped completely in 30 children with MACE and in 9 with CB. Occasional soiling is still present in two children with a CB and in one with MACE. One child with a CB had resumed regular bowel activity, and the CB was removed. MACE failed in 5 (14%) patients because of ineffective colonic lavage, and in one patient (3%) the appendix was replaced by a CB because of perforation of the appendicostomy. CB failed in one patient (8%) because of faecal leak around the button; the child was subsequently converted to MACE (P = >0.5). Complications requiring operative intervention were seen in 9 (24%) of the 37 patients who underwent MACE and none of the 12 patients who underwent CB (P = 0.09). The main complication requiring surgical intervention was stoma stenosis (11%). Complications not requiring operative intervention were seen in 7 (19%) patients after MACE and 11 (92%) of the 12 patients who underwent CB (P < 0.001). The MACE and CB procedures are reliable and effective with high success rates. The MACE has a higher incidence of complications requiring operative intervention. Conversely, complications not requiring operative intervention are more frequent with CB. CB is a safe and effective alternative to MACE in children with faecal soiling.

Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction (BOO) in infancy. Bladder instability, poor compliance and myogenic failure are responsible for the poor long-term prognosis in these patients. Previous studies have reported abundance of sensory neuropeptides, e.g. substance P (SP), calcitonin gene-related peptide (CGRP), vasoactive intestinal polypeptide (VIP) and acetylcholinesterase (AchE) nerves in the urinary bladder. We hypothesized that the functional changes in the bladder following BOO are due to alteration in cholinergic and sensory neuropeptide innervation. We therefore investigated cholinergic and sensory innervation of urinary bladder following BOO. Fifteen immature male guinea pigs (Hartley strain) 3-4 weeks old and weighing approximately 250 g. underwent placement of a silk ligature around the bladder neck to induce BOO. Controls included 5 sham-operated animals. The animals were killed 1, 2 and 4 weeks following obstruction, respectively. Whole-mount preparation and conventional sections of bladder wall were performed. AchE histochemistry, and single-label immunofluorescence histochemistry for SP, CGRP and VIP were utilized. Light microscopy and laser scanning confocal microscopy were used to assess the results. AchE staining showed marked increase in cholinergic innervation density within the suburothelial region following BOO. The staining for SP, CGRP and VIP demonstrated marked reduction in sensory nerve density within the suburothelial region 1 week following BOO and the lack of sensory innervation 4 weeks after BOO. The marked reduction in sensory innervation of the bladder and simultaneous increase in cholinergic innervation following BOO may lead to bladder instability and decrease in bladder compliance.

Urinary tract infection (UTI) is one of the most common causes of unexplained fever in neonates. The aim of this study was to determine the incidence of urinary tract anomalies and acute renal damage in neonates who presented with first urinary tract infection in the first 8 weeks of life. We reviewed the records of 95 infants, who were hospitalised with UTI during a 6-year period (1994-1999). Patients with antenatally diagnosed hydronephrosis and incomplete radiological investigations were excluded from the study. Of the remaining 57 patients, 42 were boys and 15 girls. The mean age at diagnosis was 32 days (range 5-60 days). All patients underwent renal ultrasonography (US), voiding cystourethrogram (VCUG) and (99m)Tc-dimercaptosuccinic acid (DMSA) scan. Urinary tract abnormalities were detected in 20 (35%) patients. Vesicoureteral reflux (VUR) was found in 19 (33%) neonates, 7 girls and 12 boys. Acute cortical defects on DMSA scan were present in 19 kidneys of patients with VUR and in 25 of those without reflux. Only one-third of neonates after first symptomatic UTI had VUR. We recommend that US, VCUG, and DMSA scan should be routinely performed after the first UTI in infants younger than 8 weeks.