Salvatore Cascio

Laparoscopy versus ultrasonography for the evaluation of Mullerian structures in children with complex disorders of sex development

PurposeThe 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.MethodsWe 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.ResultsEighteen patients were identified. Age at diagnosis ranged from birth to 14xa0years (mean 2.5xa0years). 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 (47xa0%) patients, in one (7xa0%) patient US and L confirmed the absence of Mullerian structures, while in six (40xa0%) 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.ConclusionsPelvic ultrasonography failed to identify Mullerian structures in 40xa0% of patients with complex DSD. On the contrary, laparoscopy allowed excellent visualization of pelvic structures and gonads in children with complex DSD.

Increased Occurrence of Disorders of Sex Development, Prematurity and Intrauterine Growth Restriction in Children with Proximal Hypospadias Associated with Undescended Testes

PURPOSEnProximal hypospadias represents 20% of hypospadias cases, which are considered to have a higher incidence of associated urological, nonurological, developmental and sexual development disorders, and chromosomal anomalies. We compared associated anomalies in boys with proximal hypospadias and undescended testis with those in boys with proximal hypospadias and descended testes.nnnMATERIALS AND METHODSnWe reviewed the medical records of 69 boys who underwent 2-stage hypospadias repair for proximal hypospadias at a single institution during the 11-year period of 2001 to 2011. Collected data included demographics, birth history, associated urological and extra-urological anomalies, karyotype analysis and gonad palpability. Patients were divided into group 1-those with proximal hypospadias and undescended testis, and group 2-those with proximal hypospadias and descended testes. Statistical analysis was performed using the 2-tailed Fisher exact test.nnnRESULTSnThere were 17 patients (25%) in group 1 with a median age of 2.2 years and 52 in group 2 (75%) with a median age of 2 years. Children in group 1 had a higher incidence of XY nondysgenetic testicular sexual development disorder (8 vs 11, p = 0.06), premature birth (9 vs 10, p = 0.01) and intrauterine growth restriction (8 each) than children in group 2 (p = 0.01).nnnCONCLUSIONSnPrematurity and intrauterine growth restriction are significantly associated with proximal hypospadias and undescended testis. Also, due to the 28% incidence of an underlying sexual development disorder, male infants with proximal hypospadias should undergo multidisciplinary evaluation.

Assessment of the introduction of an adolescent transition urology clinic using a validated questionnaire

BACKGROUNDnAdolescents with complex urological conditions are at risk of bladder dysfunction, metabolic disturbances, neoplastic changes and deterioration in renal function. Hence they require appropriate transition to the adult service to ensure lifelong urological care is adequately provided.nnnOBJECTIVEnBarriers and difficulties to the transition process have been identified in the literature. To overcome these difficulties an Adolescent Transition Urology Clinic (ATUC) was established in 2009 where the patients are seen by a paediatric and an adult urologist, a urology nurse and if needed an adolescent gynaecologist. Our aim was to assess the ATUC and present the patients perspective to this new service using a validated questionnaire.nnnMATERIALS AND METHODSnData of all patients seen at the ATUC over the study period were prospectively recorded. Patients who completed the transition were contacted and asked to complete two online urological transition questionnaires: 1) The Care Transition Measure 15 (CTM-15) which is a validated questionnaire to assess the quality of care during transition from the patients perspective. 2) The Transition Care Experience (TCE) which was created for the purpose of this study.nnnRESULTSnThirty patients attended the ATUC over 4.5 years, with 26 (87%) completing the transition to the adult urological care. Of the 26 patients there were 14 (54%) males with a mean age of 18 years. The most common underlying urological condition was neurogenic bladder (85%). Nineteen (73%) patients completed the questionnaires. Only 1 (5%) patient thought that attending the ATUC was not beneficial, 17 (89%) would recommend the ATUC to other adolescents and 9 (47%) patients considered 18 as the appropriate transition age. Overall, 74% thought that written information would have been useful and 21% considered the adult hospital as an inappropriate environment for young adults. After attending the ATUC, 2 (11%) patients thought their preferences were not met and 3 (16%) patients were not confident that they can take care of their health.nnnDISCUSSIONnTransition of patients with complex urological conditions should aim to facilitate transferring the care from the parent to the patient and preparing the adolescent to adult life by addressing their sexual and reproductive functions. Inadequate transition can have serious health consequences. On-going communication between the paediatric and the adult urologist facilitates the transition process even after the transfer of care has occurred. The CTM-15 has been considered as the only available measure of quality of care during transition from the patients perspective. Our study suggests that the large majority of adolescent attending the clinic are confident in looking after their complex urological condition. In addition one patient in 5 found the adult hospital environment an inappropriate place for adolescents and they would have preferred longer follow up in a childrens hospital. The value of written information after clinic consultation seems to be a useful adjunct that might facilitate the complex transition process.nnnCONCLUSIONnThis is the first report evaluating the transition of adolescents with complex urological conditions using a validated transition questionnaire. The combined paediatric/adult urology clinic is beneficial in addressing the patients needs and allowing smooth transition of these complex patients to the adult service.

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

PurposeTo 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.MethodsThe medical notes and the pyloric ultrasounds of all premature infants (<37xa0weeks) 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.ResultsSeventy-five preterm infants were identified (51M:24F). Median gestational age was 34xa0weeks. Median weight at presentation was 2.74xa0kg. 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 (pxa0=xa00.67, pxa0=xa00.122, pxa0=xa00.849), corrected gestational age (CGA) (pxa0=xa00.921, pxa0=xa00.678, pxa0=xa00.076), or duration of symptoms (pxa0=xa00.827, pxa0=xa00.268, pxa0=xa00.281). Mean length of stay was 6xa0days and overall complication rate was 27xa0%, both higher than reported in term infants (pxa0<xa00.001).ConclusionsPremature 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.

Open versus prone retroperitoneoscopic partial nephrectomy in children: A comparative study

PURPOSEnTo compare the outcomes of open (OPN) and prone retroperitoneoscopic partial nephrectomy (PRPN) in children.nnnMATERIALS AND METHODSnThe medical and radiological records of all children undergoing OPN and PRPN over a 6-year period (2002-2008) were reviewed.nnnRESULTSnThirty-nine (11 boys/28 girls) partial nephrectomies were performed in a single institution. There were 24 OPNs (15 upper, 9 lower) and 15 PRPNs (13 upper, 2 lower) with a median age at surgery of 2.3 years and 3.4 years, respectively. Median duration of surgery was 50 min (range 30-180) for the OPN and 150 min (range 70-205) for the PRPN (P < 0.001). Median postoperative hospital stay was 4 days (range 2-7) for the OPN and 2 days (range 1-5) for the PRPN (P < 0.001). One patient in the OPN underwent a total nephrectomy with a loss of the remaining moiety. In 20 (87%) patients of the OPN group an epidural infusion was administered and four required a morphine infusion, while only five patients in the PRPN group required a morphine infusion.nnnCONCLUSIONSnOpen partial nephrectomy had a significantly shorter operative time, but it was associated with a longer postoperative stay and higher analgesia requirements when compared to PRPN.

'Targeted top down' approach for the investigation of UTI: A 10-year follow-up study in a cohort of 1000 children.

INTRODUCTIONnInvestigations following urinary tract infection (UTI) aim to identify children who are prone to renal scarring, which may be preventable. In 2002, in an attempt to reduce unnecessary intervention, the present institution standardised the investigation of children with a confirmed UTI.nnnOBJECTIVEnThis study aimed to identify the significance of urological abnormalities on investigations following a UTI in children, prior to the introduction of the National Institute for Health and Care Excellence (NICE) guidelines.nnnMETHODSnClinical information on the first 1000 patients was retrieved from a prospective UTI hospital database. The follow-up period was 10 years.nnnRESULTSnThere were 180 males and 820 females (M:F = 1:4.5). The median age of presentation was 5 years (range 11 days-16 years). A renal ultrasound (US) was performed on all patients, and was normal in 93% of cases (n = 889) (see Figure). Of the 7% who had an abnormal US (n = 71), 54 were female and 17 male (M:F = 1:3). A total of 372 DMSA scans were requested and 350 attended their appointment. Of these, 278 cases (79%) were reported as normal, while 72 had an abnormality documented. Of these 72 patients with abnormalities on DMSA scan, 49 had a repeat DMSA scan: 30 demonstrated permanent scarring, while the DMSA scan became normal in 19. Sixteen of the 278 patients whose DMSA scan was initially normal had a repeat DMSA scan due to symptoms, and all scans were normal. Twelve (1.2%) patients required surgical intervention: three underwent circumcision for recurrent UTIs; three underwent endoscopic treatment of VUR; one had a PUV resection; one underwent a cystoscopy; three had a pyeloplasty for pelvi-ureteric junction obstruction; and one had a ureteric reimplantation for vesico-ureteric junction obstruction. After initial investigations and management, 936 patients were discharged from the UTI clinic: 47 of them re-presented - 40 with recurrent UTIs and seven with dysuria. Thirty-five of the 47 children who re-presented with urological symptoms underwent a DMSA scan, which showed scarring in three (6%).nnnDISCUSSIONnOnly 12% of children have a significant radiological abnormality picked up on investigation following a UTI. The present investigation approach differed from the NICE guidelines, where imaging is based on patient age and characteristics of the UTI. All children had a renal US, while DMSA scans were reserved for those children <1 year of age or those with upper tract symptoms. The present protocol recommended a renal US in all children presenting with a UTI. This promptly identified those with pelvi-ureteric junction obstruction and those with PUV, who all presented >6 months of age with a single UTI and, therefore, based on the NICE guidelines would not have undergone a renal US. Of the children who re-presented with further UTIs, a significant number were found to have dysfunctional voiding. As this link is well reported, it may be appropriate to screen for this in older children at initial presentation. Only three patients, who had a US at presentation, were subsequently found to have scarring on DMSA. After 10 years of follow-up, this could represent a false negative rate of 0.3% for the screening programme. None of the girls were found to have VUR or needed any surgical intervention, which suggested that early identification of the scarring might not have altered management. Few patients required surgical intervention, all of whom were identified early. No patient who re-presented required intervention. This would suggest that the present protocol is effective at picking up abnormalities that require surgical management.nnnCONCLUSIONnThis study suggested that after a childhood UTI, the liberal use of renal ultrasound and a focused top down approach to investigation is likely to identify the vast majority of children who require intervention.

I12 Laparoscopic Appendectomy

We describe the conventional laparoscopic appendectomy which, in our hands, has proven to be suitable for appendicitis of all grades of severity, regardless of whether the appendix is high retrocaecal in position.

I22 Retroperitoneoscopic Nephrectomy

There are fewer indications for nephrectomy in children than in adults. These include non or minimally functioning kidneys due to congenital renal dysplasia/hypoplasia, end stage reflux nephropathy, pelvic ureteric junction obstruction and non involuting multicystic dysplastic kidney.

I25 Button Vesicostomy

The button vesicostomy (BV) is a well-described technique which allows short- and medium-term bladder drainage. Originally described to assess bladder function prior to closing a long-standing vesicostomy, its use in recent years has been extended to children with poor bladder emptying, children with bilateral high-grade vesicoureteric reflux and children unsuitable for the formation of an appendicovesicostomy.

I26 Laparoscopic-Assisted Insertion of a Peritoneal Dialysis Catheter

Peritoneal dialysis is an alternative to haemodialysis and is used to treat patients with end-stage renal disease. Different techniques have been developed for catheter placement. We describe a single-port laparoscopic technique which allows good visualization of the pelvis and accurate positioning of the peritoneal catheter, with a low complication rate and excellent cosmetic result.