Harriet J. Corbett

Recent advances in congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is a common birth defect which continues to challenge paediatric surgeons and intensivists. Affecting approximately 1:2500 births, a baby with CDH is born every 24–36 hours in the UK.1

Congenital diverticulum of the bladder mimicking tumour

The incidence of bladder diverticula is low and identification is usually straightforward with contrast studies. We present a case of a child who had a clearly identified bladder diverticulum as well as a soft-tissue lesion in the posterior wall of the bladder that was suspected to be a rhabdomyosarcoma. Despite ultrasound scanning, micturating cystourethrogram, computed tomography and cystoscopy, open surgery was required to rule out tumour.

Enterostomy-related complications and growth following reversal in infants

Background Infant enterostomies are used to manage various neonatal surgical conditions where it is not suitable or safe to form a primary anastomosis. Complications are common and there is no consensus regarding optimal timing of enterostomy reversal. Stoma reversal is thought to allow patients to thrive; however, this has not been demonstrated robustly. Aim The study aimed to identify risk factors for enterostomy-related complications and to determine the relationship between enterostomy complications, enterostomy reversal and weight gain in infants with enterostomies. Methods A retrospective case note review of 58 infants who underwent enterostomy formation and reversal during a 6-year period was undertaken; demographic data, diagnosis, enterostomy complications and serial weights were noted. Standardised growth charts were used to calculate z scores. Results Enterostomy complications were documented in 24 infants (41%). Infants of low birth weight and low gestational birth age were significantly more likely to have an enterostomy-related complication (1110 vs 2125 g, 28.5 vs 35 weeks, respectively); they were more likely to have longer inpatient stays and remain dependent on parenteral nutrition prior to closure (median 92.5 vs 52 days, 40% vs 16%, respectively). Irrespective of diagnosis, gestation and presence of an enterostomy complication, the mean z score prior to enterostomy closure was −0.747 vs +0.892 following closure. Conclusions Around 40% of infants with an enterostomy will have an enterostomy-related complication. Whatever their weight, gestation or underlying pathology, most infants thrive after enterostomy closure and this should be considered when planning the optimal timing for this procedure.

ANG-1 TIE-2 and BMPR Signalling Defects Are Not Seen in the Nitrofen Model of Pulmonary Hypertension and Congenital Diaphragmatic Hernia

Background Pulmonary hypertension (PH) is a lethal disease that is associated with characteristic histological abnormalities of the lung vasculature and defects of angiopoetin-1 (ANG-1), TIE-2 and bone morphogenetic protein receptor (BMPR)-related signalling. We hypothesized that if these signalling defects cause PH generically, they will be readily identifiable perinatally in congenital diaphragmatic hernia (CDH), where the typical pulmonary vascular changes are present before birth and are accompanied by PH after birth. Methods CDH (predominantly left-sided, LCDH) was created in Sprague-Dawley rat pups by e9.5 maternal nitrofen administration. Left lungs from normal and LCDH pups were compared at fetal and postnatal time points for ANG-1, TIE-2, phosphorylated-TIE-2, phosphorylated-SMAD1/5/8 and phosphorylated-ERK1/2 by immunoprecipitation and Western blotting of lung protein extracts and by immunohistochemistry on lung sections. Results In normal lung, pulmonary ANG-1 protein levels fall between fetal and postnatal life, while TIE-2 levels increase. Over the corresponding time period, LCDH lung retained normal expression of ANG-1, TIE-2, phosphorylated-TIE-2 and, downstream of BMPR, phosphorylated-SMAD1/5/8 and phosphorylated-p44/42. Conclusion In PH and CDH defects of ANG-1/TIE-2/BMPR-related signalling are not essential for the lethal vasculopathy.

Outcomes of endopyelotomy for pelviureteric junction obstruction in the paediatric population: A systematic review

INTRODUCTION Dismembered pyeloplasty is the gold standard treatment for pelviureteric obstruction (PUJO) although endourological techniques are also employed. Outcomes and success rates for paediatric endopyelotomy are variably reported. OBJECTIVE The study aimed to systematically analyse published literature to give an overall success rate for endopyelotomy in children. STUDY DESIGN Medline and Embase databases were searched using relevant key terms to identify reports of paediatric endoplyelotomy. Literature reviews, case reports, series of <3 children and adult studies (age >20 years) were excluded. Primary and secondary procedures were considered separately. The procedure was considered successful if [a] the author reported success AND [b] there was no immediate conversion to open pyeloplasty OR a subsequent procedure to the PUJ 3 or more weeks after endopyelotomy. RESULTS One hundred and fourteen studies were assessed, 15 were included in the final review. Overall, 220 endopyelotomies were performed in 216 patients; 128 had primary PUJO, 92 underwent secondary endopyelotomy. Median success rate was 71% (range 46-100) in the primary group and 75% (25-100) in the secondary group. Previously undetected crossing vessels were found at subsequent open pyeloplasty in 12 failures (11 primary = 31% of failed primary endopyelotomies). Complications were reported in 14.8% of primary and 14.1% of secondary procedures. DISCUSSION This study is limited by the data given in the individual series: varied criteria used for patient selection and outcome as well as inconsistent pre and post operative imaging data precluded a meta-analysis. Designating procedures as failures if there were subsequent procedures to the PUJ lowered success rates from author-given figures for some studies. Complications rates after endopyelotomy are higher than those for open and minimally invasive pyeloplasty. Success rates for endoplyelotomy do not compare favourably with pyeloplasty and crossing vessels should be excluded before considering the procedure.

The impact of an operative note proforma at a paediatric surgical centre

Rationale, aims and objectives With expectations for standardization and evidence-based practice, the Royal College of Surgeons (RCS) published the ‘Good Surgical Practice’ in 2008. The document sets standards for operative records anticipating improved documentation, audits, medico-legal review and quality and safety of handover. We evaluated (1) documentation against RCS standards; (2) whether RCS standards are applicable to and adequate for paediatric surgery; and (3) the impact of a standardized operative proforma. Methods All general surgery and urology admissions during July 2011 and August 2012 were retrospectively reviewed using 23 set criteria. An operative note proforma was introduced in February 2012. Results were compared and statistically analysed using two-tailed Fishers exact test, with Bonferroni correction where appropriate (SPSS 20.0, IBM SPSS Statistics, Armonk, NY, USA). Results There were 345 children admitted; 63.2% underwent an operation (119 in 2011 versus 99 in 2012); 55% of operations were day cases. The initial audit noted poor documentation in 7 of 23 criteria. Following introduction of the operative note proforma, documentation improved significantly: patient identifiers by 12.3%, procedure time 43.3%, assistants name 31.9%, procedure type 21.4%, closure 6.2%, anaesthetists name 15.9%, anaesthetic type 56.8% and surgeons grade 65.8% (P < 0.05). Subgroup analysis in the latter cohort shows most of these effects to be related to the use of the proforma rather than education alone (P < 0.05). Quality of documentation was better by consultants versus trainees in 2 of 23 criteria (P < 0.05). Conclusion We have demonstrated a simple and cost-effective way of improving the quality of operative documentation, in line with guidelines set out by the RCS, in a climate of increasing economic austerity.

Crohn's disease presenting as enterovesical fistula.

Enterovesical fistulae in Crohns disease are relatively rare. We present the first report of a child presenting with an enterovesical fistula as the initial presentation of Crohns disease. Management comprises of timely diagnosis, and treatment involving surgical resection with adjunctive medical management including immunomodulators. This case highlights the need to be aware of the rare but important occurrence of Crohns enterovesical fistula as a cause for urinary symptoms in a child with inadequate weight gain.

Allantoic cyst – an unusual umbilical cord swelling

We report a baby with an unusual umbilical cord swelling. On the antenatal scans, a cystic area within the umbilical cord near its insertion onto the abdominal wall was detected. Postnatally an unusually thick umbilical cord with a yellow fluid filled cyst at the base was noted. The fluid from the cyst was confirmed as urine and ultrasound confirmed patent urachus. The baby underwent a cystoscopy and excision of patent urachus with associated allantoic cyst. Allantoic cyst is a rare swelling formed at the base of umbilicus associated with a patent urachus which results from an allantoic remnant. Paediatricians need to be aware about this condition as investigation is required to differentiate allantois cysts from umbilical pseudocysts. A patent urachus with allantoic cyst requires surgical excision.

Colon atresia and frontal encephalocele: a rare association

The association of colonic atresia with craniofacial anomalies has been well described and probably represents a malformative event that occurs in the early embryonal period. We present a case of an infant with colonic atresia and a frontal encephalocele and believe this to be a newly reported association. We review possible pathogenic mechanisms.

Management of paediatric high‐grade blunt renal trauma: a 10‐year single‐centre UK experience

To report the management and outcome of paediatric patients sustaining high‐grade blunt renal trauma.