PUVs are more common in boys with hypospadias.

INTRODUCTION: The association between posterior urethral valves (PUVs) and hypospadias has previously been reported in case reports. After the identification of this twin pathology in a number of patients, a national retrospective review of all patients with this dual diagnosis was performed. PATIENTS AND METHODS: All patients were identified in each centre from surgical databases of prospectively collated information on all surgical procedures. The medical notes were reviewed to ascertain demographics, the type of hypospadias, the mode of presentation of the valves and the outcome. RESULTS: Twenty-eight patients who had the dual diagnosis of hypospadias and PUV between 2002 and 2017 in the four tertiary paediatric centres where specialist paediatric urology is undertaken in our country were identified. Most patients (n = 24) had the valves diagnosed after hypospadias surgery. The median age at the time of hypospadias surgery was 1.4 years (range 1-4 years). There were 12 proximal and 16 mid or distal hypospadias. The commonest presentation was with problems voiding after surgery in 14 cases with a further seven boys who had urinary tract infections. Four patients had a urethro-cutaneous fistula after repair that initiated further assessment. Two boys had distal dehiscence of their repair. There was one boy presented with new onset daytime incontinence. The median time of follow-up after valve incision surgery was 4.9 years (range 0.1-12.3 years). Twenty-two patients (three pre toilet training) had no ongoing urinary symptoms. Twenty-one boys have normal renal function with one patient in stage 3b chronic kidney disease. The incidence of this dual diagnosis in Scotland is estimated at one in 100 cases of hypospadias in the paediatric population. CONCLUSION: The incidence of PUV in boys with hypospadias is estimated at 1% patients.

The risk of failure after primary orchidopexy: an 18 year review.

OBJECTIVE: To review the primary orchidopexy failure rate and outcome of repeat orchidopexy in a tertiary paediatric surgical centre and identify risk factors. METHODS: A prospectively collected and validated audits system was used to identify all boys having a repeat orchidopexy from August 1990 to December 2008 (18 years). RESULTS: In total, 1538 boys underwent orchidopexy with 1886 testicles operated on. Of these 348 (22.6%) patients had bilateral cryptorchidism. A need for repeat orchidopexy was identified in 31 boys resulting in a primary failure rate of 1.6% over the 18 years. Unilateral orchidopexy as the primary operation had a 1.5% failure rate. The failure rate for bilateral cryptorchidism was 1.87% per testicle rising to 1.93% per testicle when the primary operation was synchronous bilateral orchidopexy. Orchidopexy failure occurred in 9 patients (1.97%) who were under 24 months, 15 (2.67%) who were between 24 and 72 months and 7 (0.8%) over 72 months at time of first operation. CONCLUSION: Possible risk factors for primary orchidopexy failure are bilateral operation and older age at time of operation. Failure in achieving a satisfactory scrotal position (and testicular loss) following orchidopexy has been postulated as a potential surgical standard for revalidation of paediatric surgeons. This study adds important contemporary data to inform that process.

Bile vomiting in paediatrics: what do we really know?

Bile vomiting is clinically significant in neonates and children, indicating intestinal obstruction until proven otherwise. The aim of this study was to assess whether nursing staff within a children's hospital were able to accurately identify bilious vomiting and if a deficiency existed, whether educational posters could rectify this problem. A primary audit was conducted in the Royal Aberdeen Children's Hospital evaluating the ability of nursing staff to identify bile vomit and its significance. Educational posters were distributed and a secondary audit was conducted after six months to complete the audit cycle. The second audit also compared the knowledge of different medical professionals. In the primary audit, 41% of nurses selected the colour yellow, compared with 18% of nurses in the second audit with approximately 70% selecting dark-green. Thirty-three percent of nurses in the primary audit confirmed intestinal obstruction as the cause of bile vomiting, compared with 64% of nurses in the secondary audit. In conclusion, this study identified a deficiency in the recognition of bile vomiting among nurses, but demonstrates that the use of educational posters can significantly improve knowledge. This can be beneficial in the early recognition of this potential surgical emergency.

Factors that influence the decision to perform a karyotype in suspected disorders of sex development: lessons from the Scottish genital anomaly network register.

BACKGROUND: Although a karyotype represents the first objective evidence of investigating a case of suspected disorder of sex development (DSD), the factors that influence the clinician's decision to initiate investigations are unclear. METHODS: Cases of suspected DSD in Scotland were identified through the Scottish Genital Anomaly Network (SGAN) Register. The timing as well as other factors that influence the decision to perform a karyotype were subsequently studied. RESULTS: Out of the 572 cases on the register, 383 (67%) were classified as having a non-specific disorder of under-masculinisation. In 463 (81%) cases, the sex of rearing was male, in 71 (12%) female, and in 38 (7%) cases data regarding sex of rearing were unavailable. A karyotype was reported to have been performed in 153/572 (27%) cases. Infants in Scotland with suspected DSD are more likely to have a karyotype performed in the presence of a low external masculinisation score, bilateral impalpable testes, proximal hypospadias, or associated malformations. DISCUSSION: These data represent the first attempt at benchmarking the decision to check a karyotype and investigate infants with suspected DSD. Whilst this decision may be related to the complexity of the genital anomaly, there are other factors that may influence this decision, and these require further exploration through more rigorous systems for data collection.

The accuracy of clinical data: a comparison between central and local data collection.

BACKGROUND: The recent liberalisation of public access to information, including surgical performance, emphasises the necessity for accurate data collection. The Information and Statistics Division of the Scottish Executive (ISD) collect such data for each patient episode, but there is concern about the reliability of this information compared with that collected in local surgical departmental audit. AIM: To determine if diagnostic and operative details were consistent between local audited and national non-audited data sets. METHODS: Three surgical units comprising eight consultants were studied. Epidemiological, diagnostic and operative data for each consultant were accessed from the eScrips Internet resource (ISD Data) and from the departmental database. A unique patient number and date of birth matched individual patient episodes and the correlation between datasets graded for accuracy and consistency. RESULTS: 8375 individual data entries were recorded (ISD 4642, local databases 3733). 3402 pairs, 6408 (76.5%) of the total, matched accurately. 742 (16%) of the ISD entries were duplicates, and in 21% of unpaired entries the wrong consultant was recorded. Overall a clinically acceptable match occurred in 86.9% of paired entries for diagnosis and 84.0% for operation. The highest match with ISD data for diagnosis (88.8%) and operation (91.8%) occurred in the unit which holds a weekly audit meeting to validate information. DISCUSSION: There are significant discrepancies in surgical data between the local audit databases and central data. There is significant duplication of entries and inaccurate consultant allocation in ISD data. The promulgation of inaccurate information could threaten reputation or career and clinicians should play a more active role in ensuring clinical data are correct.

Unusual hindgut malrotation and associated mesenteric vascular abnormality presenting as large bowel obstruction in an adult.

We report an unusual and not previously described congenital hindgut malrotation presenting as large bowel obstruction in an adult.

A two-center experience with the exclusive use of laparoscopic transperitoneal nephrectomy for benign renal disease in children.

BACKGROUND: This study aimed to evaluate a two-center experience with pediatric transperitoneal laparoscopic nephrectomy, specifically focusing on the outcome parameters of operative time, complication, analgesic requirement, and postoperative stay. METHODS: This ambispective study was conducted over a 4-year period between May 2001 and May 2005 in two tertiary pediatric surgical centers. Data were prospectively recorded from an in-house expanded medical audit system (EMAS) and a Microsoft Excel database. Information on patient demographics, operative time, complications, analgesic requirement, and length of hospital stay were retrieved and analyzed. RESULTS: A total of 30 consecutive patients with a mean age of 4.43 years (range, 3 months to 15 years) underwent laparoscopic nephrectomy. All the patients underwent unilateral nephrectomy/nephroureterectomy for multidysplastic kidney (n = 12), reflux nephropathy (n = 13), pelvicoureteric junction obstruction (n = 4), or cystic disease of indeterminate cause (n = 1). The mean operative time was 93 +/- 30 min. The principal hemostatic devices used were the Harmonic Scalpel (20 cases), liga clips (5 cases), and hook diathermy and endoshears exclusively (4 cases). There were no conversions, but the intraoperative complications of bleeding (n = 2), difficult location (n = 1), difficult extraction (n = 1), and requirement for a liver retractor (n = 2) were encountered. An additional five patients had problems in the immediate postoperative period, two of whom went on to have long term difficulties with recurrent urinary tract infections resulting from a residual ureteric stump, which required surgery. Nearly one-third of the patients required morphine for analgesia in the immediate postoperative period, with the figure falling to 20% by day 1. The median postoperative hospital stay was 1 day (range, 0-16 days). At this writing, all the patients remain under surveillance with a mean follow-up period of 2.88 years, and no patients have experienced complications secondary to intraabdominal adhesions. CONCLUSION: Transperitoneal laparoscopic nephrectomy is technically feasible in most cases of benign renal disease. The intraoperative complications are minimal, and recovery for most is robust. Two-thirds of the patients are discharged within 24 h. In this study, narcotic analgesics were prescribed in about a one-third of all the cases for a limited period. Further problems may be seen when refluxing ureters are incompletely excised. However, the transperitoneal approach does not mitigate against complete excision because the exposure to the pelvis is adequate. At the midterm follow-up assessment, adhesive obstruction was not encountered, confirming this approach as a tenable alternative to other laparoscopic approaches for nephrectomy.

Operative management of testicular torsion: current practice within the UK and Ireland.

Several methods have been described and are currently used for fixation of testes in the operative treatment of testicular torsion. Although recurrence of torsion is generally viewed as a technical failure, the factors contributing to this failure remain unclear. This survey was conducted to establish current practice amongst paediatric surgeons in the UK and Ireland, in relation to testicular fixation, in an attempt to reflect the level of concern over the potential for recurrence as expressed in choice of procedure. The questionnaire survey indicated that 85% of paediatric surgeons use suture fixation of the testis alone or in combination with additional steps, such as eversion of the tunica vaginalis or creation of a dartos pouch, and 15% use a sutureless technique. The great majority (95%) fix the contralateral testis routinely and 85% routinely excise the appendix testis. Of the respondents, 17% have operated on a torted testis that had been previously fixed. In the absence of data from comparative trials, the method used for fixation remains a matter of personal preference. It was not possible to identify the definitive risk factors for recurrence from this study, but the use of absorbable sutures accompanied recurrence in most instances.

Using a 360 degrees diagnostic screening tool to provide an evidence trail of junior doctor performance throughout their first postgraduate year.

New medical graduates in the UK are known as Preregistration House Officers (PRHOs). At the end of this first postgraduate year, the Postgraduate Dean is responsible for allowing the PRHO to be fully registered with the General Medical Council. During the period 1999-2002 Professor Miriam Friedman Ben-David designed a more robust approach to appraisal and assessment of PRHOs, which provided educational feedback to all trainees, allowed any poor performers to be detected at an early stage, and provided 'hard' observable evidence for certification decisions. This paper describes the more recent development of her work resulting from further piloting of her system. The key tool, a 360 degrees diagnostic questionnaire, is designed to identify strengths and weaknesses in individual performance. It is presently being used as a screening tool to identify any trainees needing additional support and further assessment. The tool also forms part of an evidence trail for all PRHOs and helps inform formative assessment as well as contributing, along with other evidence, to full registration decisions. The evaluation of this tool, also described in this paper, shows the feasibility of implementing such a system on a wider scale and illustrates the successful balance made between robustness and feasibility.

Laparoscopic nephrectomy for unilateral multicystic dysplastic kidney in children.

The contemporary management of children with unilateral multicystic dysplastic kidney remains controversial. With the potential risks of hypertension, infection, and malignant transformation of the dysplastic kidney, conservative management necessitates long-term review with frequent ultrasound scans, urine analyses, and blood pressure checks. Operative management has traditionally used open nephrectomy with its associated patient morbidity. The introduction of laparoscopic procedures has allowed the development of techniques that reduce patient morbidity, hospital stay, and analgesia requirement. This article reports a series of 13 children who underwent elective laparoscopic nephrectomy for unilateral multicystic dysplastic kidney and discusses the advantages this procedure has to offer for their management.

Bilateral congenital diaphragmatic hernia.

Bilateral congenital diaphragmatic hernia (CDH) is a rare condition, with the literature suggesting a bleak prognosis. We describe a case of bilateral CDH that, despite confirming the challenges of diagnosis, demonstrates that the condition can have a favourable outcome.

Niemann-pick disease type C and Crohn's disease.

A five-year-old girl with Neimann-Pick disease type C subsequently developed Crohn's Disease. This association has only been presented once previously in the literature. This report discusses the options for managing one chronic disease in the presence of another life limiting condition.

Congenital funnel anus: investigation and novel management strategy.

Congenital funnel anus is a rare and poorly documented condition in which treatment strategies are still evolving. The authors present 2 cases and describe the findings after magnetic resonance imaging along with the treatment regime used successfully in these children.

Outcome after intra- and extra-corporeal laparoscopic appendectomy techniques.

BACKGROUND: This study aimed to assess the outcome after intracorporeal (IC) and extracorporeal (EC) laparoscopic appendectomy technique in a single institution over a 5-year period. METHODS: Records of all children ( n = 60) who underwent laparoscopic appendectomy at the Royal Aberdeen Children's Hospital between February 1997 and March 2002 were retrieved and evaluated. Observations were made regarding operative technique, anesthetic time, intra- and postoperative complications, postoperative analgesic requirement, and postoperative hospital stay. RESULTS: There were 30 children in each group who had a similar demographic profile. The magnitude of appendicitis severity in both groups was similar. The mean anesthetic time was 67.8 min for the IC group and 50.7 min for the EC group (p = 0.001). There were no recorded intraoperative complications, although a single case in the EC group required conversion to open procedure. The postoperative analgesic requirement in both groups was similar. The mean postoperative stay was 2.1 days in the IC group and 2.5 days in the EC group. Two postoperative complications were noted in the IC group; one intraperitoneal collection and one postoperative chest infection. Four complications occurred in the EC group; one intraperitoneal collection and three minor port-site wound infections. CONCLUSIONS: The authors' experience shows the EC technique to be significantly quicker, although with a slightly increased complication rate. Either technique can be applied safely for acute appendicitis.

Computed tomographic cholangiography in the diagnosis of bile duct injury in children.

A case of avulsion of the extrahepatic bile duct from the duodenum is described in association with severe liver trauma in a child. The value of computed tomographic (CT) cholangiography and laparoscopy in the diagnosis of this rare condition is outlined, and a paradigm for management is described.

The anocutaneous reflex and urinary continence in children with myelomeningocele.

OBJECTIVE: To examine the hypothesis that because most patients with myelomeningocele and a positive anocutaneous reflex have a competent sphincteric mechanism they are more likely to be continent on intermittent catheterization than those with a negative reflex, and to become so with less need for adjunctive surgical treatment. PATIENTS AND METHODS: The study included 111 patients with myelomeningocele treated for urinary incontinence; their present means of treatment and the outcome achieved were assessed and related to the presence or absence of an anocutaneous reflex. RESULTS: Among 29 patients with a positive anocutaneous reflex, 26 (90%) are currently dry on a regimen of intermittent catheterization, as opposed 41 (50%) of the 82 with a negative reflex (P < 0.001). Two (7%) of those with a positive and 23 (28%) of those with a negative reflex have undergone adjunctive surgery (P=0.024). CONCLUSION: These findings are consistent with both aspects of the hypothesis detailed in the Objective.

Double blind randomised controlled trial of topical glyceryl trinitrate in anal fissure.

AIMS: To determine the effectiveness and safety of topical glyceryl trinitrate (GTN) in the management of acute anal fissure in children. METHODS: Individual children were randomised to receive GTN paste or placebo for six weeks in addition to oral senna and lactulose. Patients took laxatives alone for a further 10 weeks. Each week a research nurse telephoned families to assess pain scores and give advice. Main outcome measures were validated standardised pain scores and time to painless defaecation. RESULTS: Forty subjects were recruited from 46 eligible children; 31 children completed the trial (13 in the GTN group and 18 in the placebo group). No differences in the proportion of those achieving pain free defaecation with relation to time were seen between the two groups. Similarly, there were no significant differences in pain scores between the two groups over the 16 week study period. However, in both groups pain scores had decreased significantly. There were no differences in the incidence of rectal bleeding, faecal soiling, presence of visible fissure, skin tag, or faecal loading at outpatient review at the time of recruitment, or at 6 weeks and 16 weeks. No serious adverse effects were observed. CONCLUSIONS: This study suggests that 0.2% GTN paste is ineffective in the treatment of acute anal fissures in childhood. However the overall fissure healing rate is high (84%) with associated reduction in pain scores, suggesting that a nurse based treatment programme can achieve a high rate of fissure healing.

Phenotypic presentation and outcome of esophageal atresia in the era of the Spitz classification.

PURPOSE: The aim of this study was to investigate contemporary patterns of presentation and outcome in newborns with esophageal atresia with or without tracheoesophageal fistula (OA) using modern prognostic criteria to appraise survival. METHODS: Over a 12-year period (1986 through 1997), 134 patients with OA were admitted to a single institution. Patient demographics, the presence of cardiac defects, other associated abnormalities (VACTERL), surgical intervention, and patient outcome were recorded. To identify and evaluate changes in the pattern of clinical presentation, frequency of associated anomalies and outcome, patients were analyzed during 4 consecutive time periods, 1986 to 1988, 1989 to 1991, 1992 to 1994, and 1995 to 1997. RESULTS: A primary repair or delayed primary repair was performed in 113 (84%) patients, with a staged procedure in 19 (14%). Two babies with trisomy 18 did not undergo surgery. Thirty-eight newborns (28%) had a major cardiac malformation (excluding patent ductus arteriosus, unless needing ligation), and 25 (19%) had recognized VACTERL associations. There was a significant increase in the proportion of infants with major cardiac defects diagnosed over the study period, 5 of 34 patients between 1986 and 1988 to 19 of 41 patients between 1995 and 1997 (chi(2) test, P <.001), but the incidence of VACTERL associations remained unchanged. Overall survival rate was 86% in those who underwent surgery. The relative risk of mortality in patients with major cardiac disease and VACTERL associations was 3.47 (95% CI; 1.51 to 7.96) and 2.54 (95% CI; 1.14 to 4.86), respectively. Birth weight was significantly higher in infants who survived (2.68 kg) compared with those who died (2.16 kg, P =.003). Thirty percent of infants with more than one system abnormality died compared with 8% of infants with 1, system abnormality (P =.004). CONCLUSIONS: This study has found a significant increase in the frequency of cardiac abnormalities encountered in a cohort of OA patients during the period under review. Cardiac disease and multiple abnormalities carried a substantial increased risk of mortality. In the era of the Spitz classification, the phenotypic presentation is important to accurately assess caseload severity and prognosis.