A consensus process to identify research priorities in paediatric urology in the United Kingdom.

INTRODUCTION: Paediatric urologists manage a spectrum of conditions, much of the evidence for relevant treatment pathways is of low quality. For many conditions treatment varies according to location and surgeon; children with the same condition might have surgery in one unit but watchful waiting in another. Underlying this variation are differences in opinion, and insufficient high-level evidence with few prospective randomized studies. Such studies may be challenging to design, fund and recruit into, and are more likely to succeed if there is a collaborative approach. Research prioritization is a tool to identify the research of most value. Delphi methodology is an interpretive technique aiming to gain the consensus view of interested parties. The British Association of Paediatric Urologists (BAPU) set out to ascertain consensus on what paediatric urologists, working in the UK, consider to be areas of priority for research. This paper describes the process used, and the resulting list of research questions. METHODS: A scoping survey of paediatric urologists in the UK was undertaken to identify an initial set of research questions. These were refined by the BAPU research committee (BAPU RC), then prioritized using a modified Delphi process. During Stage 1a multiple new research questions were submitted leading to Stage 1b, an interim process. All UK paediatric urologists were invited to take part in Stage 2 of the prioritization process. RESULTS: Sixty-five questions were submitted to the scoping survey by 24 paediatric urologists. The BAPU RC refined these to 60 questions, which were submitted to Stage 1a of the modified Delphi process. Sixty-seven people completed Stage 1a, at the same time submitting 224 additional research questions. The BAPU RC revised the entire question set, ensuring the key subject of the original question was not altered and novel questions were retained. The BAPU RC undertook interim scoring of the resultant 79 questions, the top scoring 25 questions plus 5 lower scoring 'wild card' questions (to ensure the breadth of the specialty was represented) were put forward to Stage 2. A total of 65 people completed Stage 2, including a lay representative. A list of 30 priority research questions was generated; the top 10 includes management of neuropathic bladder, posterior urethral valves, antibiotic prophylaxis, DSD & CAH, continence, male external genitalia, VUR and transition care (Table). CONCLUSION: This process has provided BAPU, paediatric urologists in the UK, and funders with areas of research considered a priority in the specialty.

One size does not fit all: Impact of hand size on ease of use of instruments for minimally invasive surgery.

BACKGROUND: Consideration of ergonomic factors is important for the practice of safe and efficient minimally invasive surgery (MIS). Surgeons with smaller glove sizes have previously been reported to have increased difficulties with some minimally invasive instruments. We aim to investigate hand anthropometrics and their relationship to surgeon comfort when using MIS instruments. METHODS: Male and female surgeons from two centres were surveyed on their experience of handling MIS instruments and images obtained of the dorsal and palmar aspects of their dominant hand. Photographs of hands were transformed to calibrated coordinates to enable anthropometric measurements of finger length and width as well as palm width and hand span photogrammetrically. Surgeon-perceived discomfort, fatigue, pressure points and techniques to mitigate difficulty handling instruments were compared to hand measurements. RESULTS: Questionnaires were completed by 58 surgeons; 20 (34%) were consultants, 17 (29%) were women. Glove size ranged from 6 to 8 (median 7.5). Male participants had significantly larger hands than females in all measured dimensions. Female surgeons and those with smaller finger and hand dimensions were significantly more likely to experience difficulty or discomfort across a range of variables when using MIS instruments. CONCLUSIONS: Surgeons with smaller hands reported increased problems handling MIS instruments. This represents an issue of equity in surgery, with women being more significantly affected than men. Hand size varies greatly between surgeons and anthropometric variability should be considered in design of MIS instruments.

Single-center review of staged restorative proctectomy for ulcerative colitis.

AIM: The purpose of this study was review results of pediatric patients undergoing total colectomy and restorative surgery for ulcerative colitis (UC) in a regional pediatric surgical center. METHODS: A retrospective case note analysis of consecutive patients undergoing colectomy for UC between 1995 and 2014 was performed. Early complications were defined as occurring within 30 days of surgery. Data was expressed as median (range). RESULTS: Of 39 children who had colectomy (59% female), 21 J-pouch procedures (20 three-staged) were undertaken at our center at median age 13 years (6-17 years). Length of stay (LOS) after colectomy was 8 days (6-21) with five early and eight late complications. LOS after J-pouch ileo-anal anastomosis was 7 days (5-19 days) with two early and three late complications. After ileostomy closure LOS was 6 days (4-16 days) with three early and two late complications. The most frequent complication following each surgical stage was bowel obstruction. There was only one pouch loss, which was secondary to development of Crohn's disease (CD), and three other patients with J-pouches also subsequently developed CD 1.5 to 10 years after their original colectomy but retained their pouch. Bowel frequency at 1-2 years post J-pouch in 14 patients was a median of 4 per 24 h (2-7 per 24 h). CONCLUSION: When all three postoperative stages were considered, two thirds of patients developed complications resulting in 15 additional visits to the operating theater. One in five patients subsequently developed CD after J-pouch. This should be carefully considered during pre-operative counseling. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: Level IV.

Clinical and genetic analysis of patients with cystinuria in the United Kingdom.

BACKGROUND AND OBJECTIVES: Cystinuria is a rare inherited renal stone disease. Mutations in the amino acid exchanger System b(0,+), the two subunits of which are encoded by SLC3A1 and SLC7A9, predominantly underlie this disease. The work analyzed the epidemiology of cystinuria and the influence of mutations in these two genes on disease severity in a United Kingdom cohort. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Prevalent patients were studied from 2012 to 2014 in the northeast and southwest of the United Kingdom. Clinical phenotypes were defined, and genetic analysis of SLC3A1 and SLC7A9 combining Sanger sequencing and multiplex ligation probe-dependent amplification was performed. RESULTS: In total, 76 patients (42 men and 34 women) were studied. All subjects had proven cystine stones. Median age of presentation (first stone episode) was 24 years old, but 21% of patients presented after 40 years old. Patients had varied clinical courses, with 37% of patients having ≥10 stone episodes; 70% had evidence of CKD, and 9% had reached ESRD as a result of cystinuria and its complications. Patients with cystinuria received a variety of different therapies, with no obvious treatment consensus. Notably, 20% of patients had staghorn calculi, with associated impaired renal function in 80% of these patients. Genetic analysis revealed that biallelic mutations were present in either SLC3A1 (n=27) or SLC7A9 (n=20); 22 patients had only one mutated allele detected (SLC3A1 in five patients and SLC7A9 in 17 patients). In total, 37 different mutant variant alleles were identified, including 12 novel mutations; 22% of mutations were caused by large gene rearrangements. No genotype-phenotype association was detected in this cohort. CONCLUSIONS: Patients with cystinuria in the United Kingdom often present atypically with staghorn calculi at ≥40 years old and commonly develop significant renal impairment. There is no association of clinical course with genotype. Treatments directed toward reducing stone burden need to be rationalized and developed to optimize patient care.

Time course variations in the mechanisms by which cerebral oxygen delivery is maintained on exposure to hypoxia/altitude.

Normal cerebral function is dependent upon an adequate and continuous supply of oxygen. This study calculated cerebral blood flow based on assessment of the right middle cerebral artery (MCA) velocity (MCAVel) and MCA diameter (MCADiam) by trans-cranial Doppler and trans-cranial Duplex in normoxia, during acute exposure to 12% normobaric hypoxia for up to 6 hours, and after 3 days exposure to the equivalent altitude, 4392 m, in nine subjects. Mean (SD) MCAVel increased both after 6 hours hypoxia from 76.8 (11.4) to 97.2 (17.4) cms/sec (p<0.001), and after 3 days at altitude from 68.1 (7.5) [sea level] to 76.2 (10.2) [4392 m] (p=0.015). MCADiam increased from 5.07 (0.6) to 6.1 (0.6) mm (p<0.001) after 6 hours of 12% hypoxia. Calculated mean MCA blood flow increased after 6 hours of 12% hypoxia from 5.0 (0.6) mL/sec to 8.9 (1.2) mL/sec, but there was no difference between sea level and 4392 m. Calculated mean cerebral oxygen delivery increased from 72.4 (14.4) to 107 (20.1) mL/sec (p<0.001) after 6 hours of 12% hypoxia and was maintained unchanged at 4392 m. An increase in MCA caliber, rather than blood velocity, was a major contributor to increased oxygen delivery accompanying within the first few hours of exposure to acute hypoxia. During more long-term exposure, increases in MCA velocity and a rise in hemoglobin appeared to be the more important mechanisms in maintaining cerebral oxygen delivery. The implication of this observed change in MCA diameter questions the widely held assumption that MCA velocity is a surrogate for flow during acute hypoxic exposure.

Neonatal testicular torsion: a survey of current practice amongst paediatric surgeons and urologists in the United Kingdom and Ireland.

PURPOSE: Management of neonatal testicular torsion (NTT) is controversial, with varied opinion regarding the merit(s) and role of "emergent" testicular exploration and/or contralateral orchidopexy of the healthy testis. METHODS: A survey of consultant paediatric surgeons and urologists working in the United Kingdom and Ireland was conducted to ascertain views to guide best practice. RESULTS: A total of 148 questionnaires were mailed, of which 110 were returned (74% response rate). Of these, 60 (54.5%) surgeons considered NTT secondary to torsion of the spermatic cord and 8 (7.2%) thought primary vascular infarction of the testis responsible. Twelve (10.9%) use Doppler ultrasound to guide management and exclude tumour. Eighty-two surgeons (74.5%) explore the scrotum, and 59 (71.9%) perform ipsilateral orchidectomy and contralateral orchidopexy of the "healthy" testis. Few surgeons undertake emergent exploration. Only 11 (10%) surgeons have ever found a viable testis. Seven (6.4%) cases of synchronous NTT were reported. Twenty-four (21.8%) surgeons do not perform contralateral orchidopexy with concerns of damaging a healthy testis. Orchidopexy is favoured by 89 surgeons, with 46 (52%) using nonabsorbable suture fixation and 28 (31.4%) creating a sutureless extradartos pouch. In boys later found to have a "solitary scrotal testis" and a contralateral testicular remnant, 38 (36.5%) of 104 would always "pex" the testis to avert anorchia. CONCLUSIONS: Surgeons' opinions with NTT in the United Kingdom and Ireland remain diverse. Strong argument can be made for scrotal exploration with/without contralateral orchidopexy. Parents should be counselled on the merits of varied strategies to gain better understanding of the long-term outcomes for their male child.