Why adolescents delay with presentation to hospital with acute testicular pain: A qualitative study.

BACKGROUND/PURPOSE: Adolescents have poor outcomes following testicular torsion directly attributable to delay from onset of symptoms to presentation to hospital. The aim of this study was to investigate the barriers to urgent presentation in young men. METHODS: Semistructured interviews were undertaken with young men (11-19 years), using a topic guide exploring issues surrounding testicular pain and health. Thematic analysis was undertaken using a framework approach. RESULTS: Twenty-seven adolescents were recruited, data saturation was reached at sixteen participants, and median age was 13.5 years (range 11-18). The process by which an adolescent gets to hospital with testicular pain is slow. They must recognize the problem and alert their parents, who then use a 'watch and wait' policy to assess need for medical review, often leaving it 'a day' or overnight. Adolescent males do not engage with healthcare services independently of their parents. Additional factors preventing early presentation include: absence of knowledge about testicular pathology from adolescents and their parents; concern from the young people about raising a false alarm and family concerns about burdening healthcare services. CONCLUSIONS: Recommendations include designing a testicular health education campaign for young men and educating parents regarding the medical conditions where a 'watch and wait' policy may be harmful to their child. LEVEL OF EVIDENCE: VI.

Data and data illustrations supporting the analysis of transcripts from interviews exploring the views and experiences of young men and their parents/guardians regarding testicular health.

Evidence shows young men have poor outcomes from testicular torsion directly attributable to delay in presentation to hospital [1]. Only a third to a half of adolescents present within 6 h with testicular pain, [2,3] There is poor understanding of why adolescents delay in presenting with testicular pain. The authors started without an a-priori hypothesis and designed a thematic qualitative research protocol to explore the phenomena is a naturalistic setting [4,5] . Sixteen young men (11-19 years) and their parents or guardians underwent semi-structured interviews, directed by a topic guide which evolved with subsequent interview findings. Young men were recruited from out of school clubs to minimise the bias associated with schools or hospital recruitment, and were naïve to testicular disease. Verbatim transcriptions were coded, categories and themes formed and final concepts derived utilising a framework methodology. The figure included shows the initial topic guide. The data tables presented show the emergent themes and the final code book. The authors have utilised the analysis to explore the factors impeding young men in presenting early to hospital with testicular pain [6]. The authors feel the data tables and raw data will be of interest to other researchers interested in adolescent health, health access, public health, linguistics and healthcare qualitative methodology.

Malrotation in infancy and childhood.

BACKGROUND AND AIMS: The cardinal features of bilious vomiting and abdominal distension assist in the diagnosis of malrotation and volvulus, an often fatal condition in the neonate. When these symptoms are absent, however, diagnosis becomes trickier. In the older child, these classic symptoms are less likely to occur, or occur at a later stage, meaning these children suffer from a delay in diagnosis and consequently do poorer. There is a need, therefore, to identify the common presenting features in these older children in order to facilitate earlier diagnosis and improve outcomes. METHODS: We reviewed the current literature (Appendix 1) and then undertook a retrospective study in our own department to explore the presenting complaint, time to diagnosis, intraoperative findings and outcome of all children over the age of 28 days who underwent a Ladd's procedure for malrotation at Royal Hospital for Sick Children, Glasgow (RHSC) between 1998 and 2014. RESULTS: It was found that children between the ages of 28 days and 15 years are more likely to display signs and symptoms such as chronic abdominal pain and non-bilious vomiting. Their complication rate was found to be significant. CONCLUSION: Education surrounding the timely diagnosis of malrotation in the post-neonatal child is crucial.

Factors associated with passing the European Board of Paediatric Surgery Exam.

PURPOSE: The European Board of Paediatric Surgery (EBPS) includes a written Part 1 and an oral/practical Part 2 component. The goal of this study was to describe the EBPS examination candidate pool, and to determine factors associated with successfully passing the examination. METHODS: A database including all registered candidates for the EBPS exams since 2005 was constructed. Queried information included demographics, training location, language proficiency, and written/oral scores. Logistic regression analysis was performed to elucidate variables predictive of examination success. RESULTS: Until 2013, a total of 370 candidates registered for the part 1 examination and 147 successfully passed part 2. Pass rates for part 1 were 68, 65, 20, and 0 % on first, second, third, and forth attempts, respectively. Pass rates for part 2 were 79 % for both first and second attempts. Training in a single country was associated with passing Part 1 (p = 0.048), while having completed at least some training in an English-speaking country increased the chance of passing Part 2 (p < 0.01). CONCLUSION: The pool of EBPS examination candidates is highly diverse and international. First- and second-attempt pass rates are similar for both parts. Candidates who completed all their training in non-English-speaking countries may wish to consider additional English language practice to increase their chances of success.

Transition into adult healthcare services in Scotland: findings from a study concerning service users at the Scottish Spina Bifida Association.

BACKGROUND AND AIMS: Literature on interventions that enable young people with spina bifida and/or hydrocephalus to have smooth transition, into adult healthcare services, stress the need for the process to start early and to include all family members. The study reported here was set to quantify and articulate the experiences of service users who are or due to be going through the transition process in Scotland today. METHODS AND RESULTS: Focus group sessions, in the North of Scotland and in the 'Central Belt', captured rich qualitative data. A survey, sent to eligible participants on the Spina Bifida National database, offered complimentary data source. Despite the fact that the number of returned questionnaires was low (n = 20), data analysis identified a number of core recurring themes. These include issues concerning Communications, Respect, Choice and Control. Findings suggest that there is a significant chasm between the political rhetoric and the reality faced by young people with spina bifida moving to adult healthcare services. CONCLUSION: A possible way to facilitate successful transition of young people is using personal healthcare information as the locus for needed change. More research is needed to ascertain whether a 'Person-Centred Record', which is set to empower young people on their transition pathway, is an appropriate transition tool.

Familial tumoral calcinosis.

INTRODUCTION: Tumoral calcinosis is a rare condition characterised by progressive, ectopic, periarticular deposits of calcium. These tumour-like growths often infiltrate muscle and tendon, usually presenting as multiple masses or as a painless, solitary mass. Our case report will focus on familial tumoral calcinosis, an autosomal recessive metabolic disorder generally observed in patients within the first two decades of life. CASE PRESENTATION: Our case report introduces two Sudanese siblings from consanguineous parents who presented with simultaneous recurrent multiple soft tissue swellings. CONCLUSION: The cases discussed highlight the main features of familial tumoral calcinosis and indicate the need for improved clinical guidance on the investigations, treatment and genetic screening of familial tumoral calcinosis.

Wilms' tumor with intravascular extension: A review article.

Intravascular extension of Wilms' tumor is a well-recognized phenomenon. Intravascular extension into the vena cava occurs in only 4-8% of patients with Wilms' tumors and intraatrial extension occurs in around 1-3% of patients. This review of the published literature in this cohort aims to summarize the findings of different case series to provide an optimum management plan. A literature search was performed and index papers were retrieved for review. The search included the following terms: Intracaval, intravascular, intraatrial and intracardiac extension of Wilms' tumor or nephroblastoma. The management of patients with intravascular tumor thrombus in Wilms' tumor is complex. A skilled multi-disciplinary team at a tertiary referral center with cardiothoracic surgery available should manage these patients. Multi-modal diagnostic and preoperative imaging are required to confirm and define the extent of the extension. Preoperative chemotherapy is advocated for all but exceptional circumstances and must be followed closely. Surgical resection should be planned according to the stage of intravascular extension with possible need for cardiopulmonary bypass and deep hypothermia with cardiac arrest if required. Surgical complications are more common in this group of patients, but outcome is comparable to those without intravascular extension, and is more closely correlated with the histological subtype then stage of intravascular extension. Operative imaging are required to confirm and define the extent of the extension.

Audit of ventriculoperitoneal shunt infections in paediatric patients, 2006-2013.

BACKGROUND: Infection following ventriculoperitoneal shunt placement remains a significant complication with an incidence of 3-27% cited in literature. Infections cause significant morbidity, and it is important that empirical antibiotic therapy for management is guided by accurate knowledge of prevailing aetiologies and local antibiotic sensitivity patterns. AIMS: To establish the incidence of shunt infections in our paediatric population, to identify the causative micro-organisms, and to determine the antibiotic resistance patterns of the responsible micro-organisms. METHODS: Retrospective data collection utilising existing databases in the Royal Hospital for Sick Children, Yorkhill, between 1 January 2006 and 30 September 2013. RESULTS: Total number of shunt operations was 308 with 28 episodes of infection involving 27 patients (male = 12, female = 15). The incidence of infection was 9%. In all, 79% of the episodes involved a single pathogen with 21% being mixed pathogens. Coagulase-negative staphylococci were the most common cause of infection (44%). Gram-positive sensitivity to flucloxacillin and gentamicin was noticeably low at 22% and 14%, respectively with 87% of coagulase-negative staphylococci resistant to gentamicin and 81% resistant to flucloxacillin. CONCLUSIONS: The changing spectrum of Gram-positive organisms has impacted on antibiotic sensitivity patterns, and our local prescribing policy has been adapted in order to manage shunt infections most effectively.

The long-term outcome of patients diagnosed with sacrococcygeal teratoma in childhood. A study of a national cohort.

BACKGROUND/PURPOSE: The improved survival of sacrococcygeal teratoma (SCT) has led to increased awareness of its long-term sequelae. Our aim was to assess the long-term outcome of a national cohort using detailed questionnaires. METHODS: The three paediatric surgery centres in Scotland were contacted to identify all SCT patients ≥5 years of age. Case notes were reviewed. Detailed separate questionnaires were used to assess long-term bowel, urinary and obstetric outcomes and were completed during an arranged interview. Groups were statistically compared using Z-tests or Fisher's exact test. RESULTS: Overall, 48 patients were identified but only 31 were available for follow-up. Age ranged from 5-35 years (median 12 years and 8 months). There were 25 (81%) females and 5 (16%) patients had malignant disease. Abnormal bowel function was noted in 42% of patients, with constipation being the commonest complaint (39%) with no obvious predictive features at presentation. Urinary symptoms were reported in 55% of the patients. A total of nine (29%) patients suffered from urgency and/or wetting. Confirmed urinary tract infections (UTIs) were reported by nine patients. Successful pregnancies were reported by two females and neither of their children had SCT. CONCLUSIONS: This is one of the largest national studies assessing the long-term outcome of patients with SCT. It highlights the significant gastrointestinal and urological long-term morbidities of SCT patients, which is useful for counselling families.

Langerhans cell histiocytosis: 23 years' paediatric experience highlights severe long-term sequelae.

PURPOSE: To review the presentation and outcome of patients with Langerhans cell histiocytosis attending The Royal Hospital for Sick Children, Glasgow over a 23-year period. METHOD: Thirty-one children were diagnosed with Langerhans cell histiocytosis between January 1990 and December 2012. Retrospective information from medical records was gathered on age at diagnosis, presenting symptoms, classification of disease, treatment and long-term outcome. RESULTS: There were 17 boys and 14 girls; median age at diagnosis 2 years 9 months (interquartile range: 1 year 6 months to 4 years 4 months). Eleven were below 2 years and two were below 6 months of age at diagnosis. Eighteen (58%) children had single system disease of which four were multifocal; 13 (42%) had multisystem disease. Seventeen children improved with conservative treatment. Fourteen required steroids and dual agent chemotherapy; three required further chemotherapy. One child died. Two children had successfully treated relapses. Ten developed diabetes insipidus, seven were growth hormone deficient, two suffered from hypothyroidism and one panhypopituitarism. Median follow-up of the cohort was 8 years 10 months (interquartile range: 5 years 5 months to 12 years 7 months). CONCLUSION: Langerhans cell histiocytosis is a rare disease in infants and young children, with a variable course ranging from self-limiting to life threatening. In very young children (under 2 years of age), multisystem disease is more common, requiring intensive chemotherapy and lifelong follow-up. Lasting sequelae were identified in over a third of patients, including endocrine dysfunction, hearing difficulties, neurological and psychological problems.

The long-term psychosocial outcomes following excision of sacrococcygeal teratoma: a national study.

OBJECTIVE: The overall effect of the reported long-term sequelae following sacrococcygeal teratoma (SCT) excision on the psychosocial and sexual development has not been addressed appropriately in the literature. The aim of this study was to evaluate the psychosocial adjustment of a national cohort of SCT patients using a validated psychosocial questionnaire. METHODS: Three paediatric surgery centres in Scotland were contacted to identify those SCT patients who were now 5 years or older. The main outcome measure was the Derriford Appearance Scale 59 (DAS-59) which assesses concerns of everyday living, personal relationships, self-esteem and emotional distress. Following ethical approval, age appropriate invitation letters and information sheets for both parents and patients were sent to prospective participants. Parents/patients were contacted after 2 weeks to arrange an interview to complete the questionnaire. RESULTS: 31/48 (65%) of patients identified with SCT completed the DAS-59. Participant ages ranged from 5 to 35 years (median 12 years). 5/31 (16%) were malignant cases. There were 25 (81%) female participants and 12 (39%) patients older than 16 years. 9/31 (29%) participants indicated concern over their appearance. However, we found low levels of appearance-related distress and overall participants showed positive adjustment to personal relationships and everyday living on the DAS-59. CONCLUSIONS: This is the first study looking at the psychosocial adjustment of patients with SCT using a validated psychosocial questionnaire. Encouragingly, we found low levels of appearance-related distress. Future research could identify those factors associated with increased risk of poorer outcomes and highlight those in need of psychological intervention.

Case-based anatomy teaching: a viable alternative?

BACKGROUND: Over the last two decades, there has been a decline in the amount of time available for anatomy teaching in the medical undergraduate curriculum, and new methods of anatomy teaching have been adopted for pragmatic reasons, with little evidence base to support their proposed educational benefits. This study seeks to establish the effect of a case-based teaching method on students' confidence in anatomy. METHODS: Forty-three student volunteers in the clinical phase of the Glasgow medical course were given weekly anatomy teaching sessions based on clinical case presentations over 4 weeks. The students were given an anatomy test, and were asked to rate their confidence in their anatomy knowledge before and after the teaching sessions. RESULTS: There was a two-point increase in students' self-rated confidence, and a 10.9 per cent increase in average test score after the case-based anatomy teaching sessions. Both of these increases were statistically significant (p < 0.001 and p < 0.001, respectively). DISCUSSION: Case-based anatomy instruction can significantly improve students' confidence and knowledge of anatomy. This method of teaching was also highly rated by students, which may make it a viable option for the teaching of anatomy in the modern medical curriculum.

Hypertrophic pyloric stenosis in premature infants: evaluation of sonographic criteria and short-term outcomes.

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.

A meta-analysis of the risk of boys with isolated cryptorchidism developing testicular cancer in later life.

BACKGROUND: Significant variability exists for the relative risk (RR) of testicular malignancy in isolated cryptorchidism. OBJECTIVE: To perform a meta-analysis to clarify the true magnitude of this risk, allowing clinicians to better counsel patients and their families. SETTING: Secondary research conducted by undergraduate researchers, clinical academics and a clinical statistician. DESIGN, DATA SOURCES, AND METHODS: A search of the English literature was performed for studies relating to testicular cancer and cryptorchidism, published between 1 January 1980 and 31 December 2010, using Embase and Medline databases. 735 papers were identified and analysed by four authors independently in accordance with our inclusion and exclusion criteria. Studies reporting an association between cryptorchidism and subsequent development of testicular malignancy were included. Genetic syndromes or other conditions which predisposed to the development of cryptorchidism were excluded. Pooled estimates and 95% CIs for the RRs were calculated. RESULTS: Nine case-control studies and three cohort studies were selected. The case-control studies included 2281 cases and 4811 controls. Cohort studies included 2 177 941 boys, with a total of 345 boys developing testicular cancer (total length of follow-up was 58 270 679 person-years). The pooled RR was 2.90 (95% CI 2.21 to 3.82) with significant heterogeneity (p<0.00001; I(2)=89%). CONCLUSION: Boys with isolated cryptorchidism are three times more likely to develop testicular cancer. The limitations of this study must be acknowledged, in particular, possible publication bias and the lack of high-quality evidence focusing on the risk of malignancy in boys with isolated cryptorchidism.

Urogenital anomalies in girls with sacrococcygeal teratoma: a commonly missed association.

BACKGROUND: The association of urogenital (UG) anomalies and sacrococcygeal teratoma (SCT) has not been widely reported. Our aim was to look at the national incidence and presentation of this anomaly in patients with SCT and to provide the first report of a clear anatomical description of this commonly missed association. METHODS: Sacrococcygeal teratoma cases in Scotland during the last 30 years were identified. Patients with associated UG anomalies were reviewed in detail to identify their presentation, anatomy, and management. RESULTS: Fifty-three patients with SCT were identified, including 41 girls. Five girls (12%) subsequently had a UG anomaly diagnosed, which was not apparent at the initial surgery. Two patients presented with retention, and their anomaly was diagnosed at 6 weeks and 7 months of age. The other 3 presented with incontinence, and despite thorough assessment, including cystoscopy, their UG anomalies were not recognized until the ages of 7, 9, and 13 years. CONCLUSIONS: Urogenital anomalies are surprisingly common in girls with SCT. The reason for this association is unclear. None of these cases were diagnosed initially, which means that it was either missed or acquired. Urogenital anomalies should be suspected in girls with SCT and actively excluded in those with voiding difficulties.

Age-related medical decision limits for urinary free (unconjugated) metadrenalines, catecholamines and metabolites in random urine specimens from children.

BACKGROUND: Neuroblastoma is the most common extracranial solid tumour in childhood (8% of all childhood cancers), the most frequently diagnosed in infancy, and has one of the highest death rates, while chromaffin tumours rarely present in childhood. Both tumour types produce catecholamines and their metabolites. It is difficult to produce reference ranges for tests in children, and currently, no age-related medical decision limits for free metadrenalines (free metanephrines) in random urine specimens exist in the paediatric literature. METHODS: Results of vanillylmandelic acid (VMA), 5-hydroxyindoleacetic acid, homovanillic acid (HVA), noradrenaline (NA), adrenaline, dopamine (DA), free normetadrenaline (fNMA), free metadrenaline and free 3-methoxytyramine (f3MT) in 1658 random urines obtained from infants, children and young adults were measured by high performance liquid chromatography with electrochemical detection. Specimens were excluded from consideration if obtained from the following categories, i.e. (a) harbouring neuroblastic, chromaffin, carcinoid or other tumours or malignancies; (b) medical conditions having known association with excess catecholamine excretion; (c) patients administered catecholamine or paracetamol; (d) overly dilute urine; and (e) manifesting outlying values following visual inspection. RESULTS: There remained 872 specimens that were grouped into seven age ranges (<1; 1 or 2; 3 or 4; 5-7; 8-10; 11-13; 14-19 y) for which medical decision limits were determined for each analyte. There was no significant difference between the results for boys or girls. In 55 patients harbouring neuroblastic tumours, HVA (54/55), f3MT (14/16), VMA (45/53) and DA (43/53) were the most frequently elevated analytes at time of diagnosis. In 11 patients presenting in childhood with chromaffin tumours, fNMA (11/11) followed by NA (10/11) were the most frequently elevated. Discussion The likely reasons for outlying or missing values, together with the reasons for variation in the distinctive biochemical patterns of analytes exhibited in individuals harbouring either neuroblastic or chromaffin tumours are discussed.

The role of a paediatric day surgery unit at the Royal Hospital for Sick Children, Glasgow between 1993 and 2006.

A retrospective analysis was carried out on all day surgery admissions at Glasgow's Royal Hospital for Sick Children between 1993 and 2006. The aim of the study was to analyse the total number of operations per day, month and year; specialties involved; and age range of the children admitted, to examine how these factors changed over the 13-year study period. We also studied the specific operations performed in 2006 by each specialty.

Childhood phaeochromocytoma and paraganglioma: 100% incidence of genetic mutations and 100% survival.

INTRODUCTION: The aim is to identify the incidence of genetic mutations and outcome of children presenting with phaeochromocytoma/paraganglioma (PGL) to a single paediatric surgical service to determine the need for genetic counselling in associated kindreds. METHODS: A retrospective case note review was undertaken of all cases treated between 1998 and 2008 with particular reference to presentation, management, and predisposing genetic conditions. RESULTS: Seven cases (4 male, 3 female) were identified (median age, 13 years; interquartile range, 9-16). Three cases had a family history of phaeochromocytoma/PGL. All presented with neurologic symptoms related to hypertension, including headaches (n = 5), hemiparesis (n = 2), facial palsy, and hemianopia. All underwent surgical resection. Five patients had meta-iodobenzylguanidine (MIBG) therapy for apparently malignant features. All cases were found to have a predisposing genetic mutation: von Hippel-Lindau (n = 3), succinate dehydrogenase mutations (n = 3), and multiple endocrine neoplasia (n = 1). All patients are alive after a median follow-up of 5 (interquartile range, 2-7) years. CONCLUSIONS: All 7 cases had a familial genetic mutation identified, and none arose de novo. We advocate genetic counselling for all families of children diagnosed with phaeochromocytoma/PGL with lifelong surveillance tailored to the underlying syndrome because of the increased risk of synchronous and metachronous tumours associated with these genetic syndromes.