Optimising the management of children with concomitant bladder dysfunction and behavioural disorders.

Bladder dysfunction and behavioural disorders in children are commonly concomitant; hence, it is difficult to treat each in isolation. Pharmacotherapy is common treatment for behavioural disorders, and these medications may have intended or unintended positive or negative bladder sequelae. This review identifies the literature regarding the effects of behavioural pharmacotherapy on bladder functioning and possible bladder management strategies in children with concomitant behaviour and bladder disorders to enable clinicians to better manage both conditions. A PROSPERO registered PRISMA-guided review of three major databases was performed. After an initial scoping study revealed significant heterogeneity, a narrative approach was undertaken to discuss the results of all relevant cases relating to children being treated with pharmacotherapy for behaviour disorders and outcomes related to bladder function. Studies were screened to identify those that described effects of commonly prescribed medications in children with behavioural disorders such as stimulants, alpha 2 agonists, tricyclic antidepressants (TCA), serotonin and noradrenergic reuptake inhibitors (SNRI), selective serotonin reuptake inhibitors (SSRI) and antipsychotics, and the findings and implications were summarised. The review identified 46 studies relevant to behavioural pharmacotherapy and bladder function (stimulants (n = 9), alpha 2 agonists (n = 2), TCAs (n = 7), SNRIs (n = 8), SSRIs (n = 8) and antipsychotics (n = 6). Six studies focused specifically on bladder management in children with behavioural disorders with concurrent behavioural pharmacotherapy. This review identifies useful factors that may assist clinicians with predicting unintended bladder effects following initiation of behavioural pharmacotherapy to facilitate the best approach to the treatment of bladder dysfunction in children with behavioural disorders. With this evidence, we have provided a useful decision-making algorithm to aide clinicians in the management of these dual pathologies.

Culture-positive urinary tract infection following micturating cystourethrogram in children.

Objective: To determine the incidence of culture-positive urinary tract infection (UTI) after micturating cystourethrogram (MCUG). We further wanted to identify risk factors for developing a culture-positive UTI following MCUG. Methods: A retrospective review of the available medical records of 500 paediatric patients who underwent MCUG in Perth, Western Australia was performed. Results: Seven (1.4%) patients comprised of four females and three males developed a febrile, culture-positive UTI within 14 days following MCUG. Significant association was found for female patients, patients with neurogenic bladder, and patients with previous culture-positive UTI as developing a culture-positive UTI following MCUG. Multivariate logistic regression determined that patients were more likely to develop culture-positive UTI within 14 days following MCUG if they had a known history of UTI (odds ratio: 5.0, 95% confidence interval: 1.5-17.3, p=0.010) or had a neurogenic bladder (odds ratio: 4.2, 95% confidence interval: 1.0-17.9, p=0.049). Conclusion: The incidence of patients who developed a febrile, culture-positive UTI following MCUG was low at 1.4%. Statistically significant and independent associations for the development of culture positive UTI were found in patients with neurogenic bladder and patients with previous culture-positive UTI. Further prospective studies are necessary to determine necessity of prophylactic antibiotics for high-risk patients, e.g., patients with neurogenic bladder or previous culture-positive UTI.

Persistent Müllerian Duct Syndrome: Understanding the Challenges.

Persistent Müllerian duct syndrome (PMDS) is a rare autosomal recessive condition defined by the presence of Müllerian duct-derived structures in an otherwise normally masculinized phenotypical and genotypical (46,XY) male. We describe the case of an infant diagnosed with PMDS, managed and followed up for 7 years. The diagnosis of PMDS was made at laparoscopy at 6 months of age for investigation and management of bilateral impalpable testes. A Müllerian structure resembling a uterus with bilateral fallopian tube-like structures was seen in the pelvis, along with bilateral intra-abdominal testes. Gonadal biopsy confirmed normal testicular tissue. The child underwent successful bilateral two-stage Fowler-Stephens orchidopexies. The Müllerian remnant was preserved to maintain testicular vascularity. At the most recent follow-up, the testes are intrascrotal and normal on palpation. There have been no clinical symptoms or concerns with the Müllerian remnant during surveillance with ultrasound and MRI. To date, there are less than 300 cases described in the medical literature, with limited consensus on management. We reflect on challenges the condition poses, including fertility preservation in PMDS, testicular and Müllerian malignancy risk in PMDS, and optimal management and surveillance of PMDS.

Clinical spectrum and management options for prostatic utricle in children.

PURPOSE: Prostatic utricle (PU) has been described in children with hypospadias, posterior urethral valves (PUV), variances of sex characteristics and normal external genitalia. Intervention may be required in symptomatic cases, but clinical characteristics vary, requiring individualisation of management. We describe our experience with this condition. METHODS: Retrospective review of males noted to have PU on cystoscopy (2009-2020) at a single centre. Presentation, management and outcomes were recorded (Variances of sex characteristics excluded). RESULTS: Of 1060 male children who underwent cystoscopy for various reasons, PU was recorded in 36 (3.4%), indications for cystoscopy being hypospadias in 28, PUV in 3, urinary tract infections (UTI) in 3 and hydronephrosis in 2. Six (16.7%) developed epididymo-orchitis. All 6 had Ikoma Grade II PU. Case 1 (16 years,UTI) was managed with intravenous antibiotics. Case 2 (8 years,hypospadias) underwent laying open of urethral stricture. Case 3 (5 years,PUV) underwent laparoscopic PU excision. Cases 4 and 5 (3 and 6 years, hypospadias) underwent cystoscopic injection of bulking agent near the insertion of the vasa. Case 6 (3 years,hypospadias) underwent laparoscopic PU excision with vasal disconnection (vasal openings at dome of utricle rather than base) following failed endoscopic management. Median follow-up was 36 (0-206) months. All remained asymptomatic with normal testicular volumes for age at current follow-up. CONCLUSION: Multiple treatment options to address a symptomatic PU have been described and enable surgeons to individualise treatment based on clinical circumstances. It is important to identify variations in anatomy of vasal connection to PU to plan appropriate management. LEVEL OF EVIDENCE: IV (Retrospective study).

Effectiveness of standard urotherapy (basic bladder advice) and combination therapies in managing bladder dysfunction in children with treated behavioral disorders: Results of a prospective cohort (DABBED) study.

AIMS: We evaluated the efficacy of standard urotherapy and combination therapies in treatment of bladder dysfunction in children with treated behavioral disorders. METHODS: Prospective study of children (6-16 years) with bladder dysfunction and behavioral disorders was conducted between March 2018-2020. Eligible children were initially offered standard urotherapy and those with no response at 3 months were offered combination therapies. Symptomatic response, changes in Akbal score and PinQ score were reported at 6 months and outcomes were correlated to behavioral diagnoses and medications. RESULTS: Thirty-nine consecutive children (male = 27, mean age [SD] 10.3 [±2.0] years) were recruited, of whom 29 completed the study (five lost to follow-up, three non-compliant to treatment, two excluded). Thirty-four (87%) children had attention deficit/hyperactivity disorder. Monosymptomatic nocturnal enuresis (n = 11) and non-monosymptomatic enuresis (n = 17) were the commonest diagnoses. Following 3-month review, 14 (38%) children continued to receive standard urotherapy, while 15 (41%) children were transitioned to combination therapy. At 6-month review, complete/partial response was seen in 62% (23/37) and no response in 16% (6/37); with 32% (12/37) responding to standard urotherapy alone. Akbal symptom scores (15.9-11.5; P < 0.01) and PinQ scores (26.0-19.5; P = 0.008) improved significantly at 6-month follow-up. Type of underlying behavioral disorder(s) or medications for behavioral disorder did not influence the outcomes. CONCLUSION: This study confirms that children with underlying behavioral disorders are able to have a good response to the appropriate therapy for their bladder dysfunction with a third of children responding to standard urotherapy alone.

Page Kidney in a Child with Severe Pelviureteric Junction Obstruction.

There are various causes of Reno Vascular Hypertension in children reported in the literature. Amongst these, Page kidney gets a rare mention. This phenomenon is a result of the accumulation of blood or urine in the perinephric or subcapsular space, resulting in compression of renal parenchyma, microvascular ischemia, alteration in the renin-angiotensin apparatus, and high renin hypertension. It has been well documented and studied in adults. Only a few cases are reported in the paediatric population. We report a rare presentation of Page kidney in a 5 year 8 months old girl. She initially presented with Dietl's crisis secondary to left Pelviureteric Junction obstruction (PUJO) causing massive hydronephrosis. She developed Page kidney phenomenon after spontaneous rupture of the pelvicalyceal system formed a tight compressive urinoma. She was managed successfully with internal JJ stenting and ultrasound-guided aspiration of the urinoma followed by elective delayed Pyeloplasty. To our knowledge, this is the first documented case of Page kidney in a child with severe PUJO.

Multicystic dysplastic kidney - treat each case on its merits.

OBJECTIVES: To assess outcomes of unilateral multicystic dysplastic kidney (MCDK) managed at an Australasian centre over a 15 year period. To assess if MCDK involution could be predicted based on change noted between first two postnatal ultrasound scans 6 months apart. SUBJECTS AND METHODS: A retrospective study was performed. RESULTS: One-hundred-and-six cases of unilateral MCDK were studied. Eighty-four of these presented antenatally. Twenty-two MCDK cases presented postnatally. Urological anomalies associated with MCDK included vesicoureteric reflux (VUR), ureterocele and contralateral pelviureteric junction obstruction (PUJO). Children undergoing surgical intervention for these anomalies were offered concurrent MCDK nephrectomy. Morbidity associated with MCDK under surveillance included febrile culture-positive urinary tract infection in 20 cases (20.7%), hypertension in four (3.7%) and Wilms' tumor in one (0.9%). Thirty-six cases (34%) underwent complete involution, 32 (30.2%) were in the process of involuting and 38 cases (35.8%) underwent nephrectomy because of failure of involution or associated morbidity. If the MCDK reduced in cranio-caudal interpolar length by 20% or more between the first postnatal USS and the next one 6 months later, then it was very likely to involute spontaneously. If the MCDK did not reduce in cranio-caudal interpolar length by 20% between the first postnatal scan and the next one 6 months later, then it was highly likely to fail to involute, and in our study, correlated with the outcome of nephrectomy. CONCLUSION: Although MCDK is a benign condition, it should be carefully investigated and followed-up, as involution may not occur in over a third. In some cases, morbidity may occur. Each case of MCDK should be managed on its own merits. LEVEL OF EVIDENCE: Level II - Prognosis study, Retrospective.

Long-term medical and psychosocial outcomes in congenital diaphragmatic hernia survivors.

OBJECTIVE: Survival rates for congenital diaphragmatic hernia (CDH) are increasing. The long-term outcomes of CDH survivors were compared with a healthy control group to assess the morbidity for guidance of antenatal counselling and long-term follow-up programmes. PARTICIPANTS AND DESIGN: Participants born with CDH in Western Australia 1993-2008 were eligible with matched controls from the general population. Participants had comprehensive lung function tests, echocardiogram, low-dose chest CT scan and completed a Strengths and Difficulties Questionnaire (SDQ) and quality of life (QOL) questionnaire. RESULTS: 34 matched case-control pairs were recruited. Demographic data between groups were similar. Cases were smaller at follow-up (weight Z-score of -0.2vs0.3; p=0.03; height Z-score of -0.3vs0.6; p=0.01). Cases had lower mean Z-scores for forced expiratory volume in 1 s (FEV1) (-1.49 vs -0.01; p=0.004), FEV1/forced vital capacity (-1.92 vs -1.2; p=0.009) and forced expiratory flow at 25-75% (FEF25-75) (-1.18vs0.23; p=0.007). Cases had significantly worse respiratory mechanics using forced oscillation technique. Subpleural triangles architectural distortion, linear opacities and scoliosis on chest CT were significantly higher in cases. Prosthetic patch requirement was associated with worse lung mechanics and peak cough flow. Cases had significantly higher rates of gastro-oesophageal reflux disease (GORD) and GORD medication usage. Developmental delay was significantly higher in cases. More cases had a total difficulties score in the high to very high range (25% vs 0%, p=0.03) on the SDQ and reported lower objective QOL scores (70.2 vs 79.8, p=0.02). CONCLUSION: Survivors of CDH may have significant adverse long-term medical and psychosocial issues that would be better recognised and managed in a multidisciplinary clinic.

A novel, homozygous mutation in desert hedgehog (DHH) in a 46, XY patient with dysgenetic testes presenting with primary amenorrhoea: a case report.

BACKGROUND: Desert hedgehog (DHH) mutations have been described in only a limited number of individuals with 46, XY disorders of sex development (DSD) presenting as either partial or complete gonadal dysgenesis. Gonadal tumours and peripheral neuropathy have been associated with DHH mutations. Herein we report a novel, homozygous mutation of DHH identified through a targeted, massively parallel sequencing (MPS) DSD panel, in a patient presenting with partial gonadal dysgenesis. This novel mutation is two amino acids away from a previously described mutation in a patient who presented with complete gonadal dysgenesis. Adding to the complexity of work-up, our patient also expressed gender identity concern. CASE PRESENTATION: A 14-year-old, phenotypic female presented with primary amenorrhoea and absent secondary sex characteristics. Investigations revealed elevated gonadotrophins with low oestradiol, testosterone of 0.6 nmol/L and a 46, XY karyotype. Müllerian structures were not seen on pelvic ultrasound or laparoscopically and gonadal biopsies demonstrated dysgenetic testes without neoplasia (partial gonadal dysgenesis). The patient expressed gender identity confusion upon initial notification of investigation findings. Formal psychiatric evaluation excluded gender dysphoria. Genetic analysis was performed using a targeted, MPS DSD panel of 64 diagnostic and 927 research candidate genes. This identified a novel, homozygous mutation in exon 2 of DHH (DHH:NM_021044:exon2:c.G491C:p.R164P). With this finding our patient was screened for the possibility of peripheral neuropathy which was not evident clinically nor on investigation. She was commenced on oestrogen for pubertal induction. CONCLUSION: The evaluation of patients with DSD is associated with considerable psychological distress. Targeted MPS enables an affordable and efficient method for diagnosis of 46, XY DSD cases. Identifying a genetic diagnosis may inform clinical management and in this case directed screening for peripheral neuropathy. In addition to the structural location of the mutation other interacting factors may influence phenotypic expression in homozygous DHH mutations.

Intravesical ureteric reimplantation for primary obstructed megaureter in infants under 1 year of age.

PURPOSE: This study aimed to assess the outcome of intravesical ureteric reimplantation for primary obstructed megaureter (POM) performed in infants under 1 year of age. MATERIALS AND METHODS: A retrospective cohort study was carried out of all infants undergoing intravesical ureteric reimplantation surgery for POM at two pediatric centers in the 12 years between 2003 and 2014. Outcomes were compared with those of children aged over 1 year undergoing intravesical reimplantation for POM during this period. Follow-up was until November 2015. RESULTS: Thirty-seven megaureters in 34 infants were reimplanted, at median age 5.2 months (Table). Operative success was 97%, with one out of 34 patients (3%) requiring reoperation for recurrent ureteric obstruction. Renal preservation was high. One patient of 34 (3%), who had bilateral congenital renal dysplasia, showed evidence of decline in renal function. Five patients of 34 (15%) had postoperative urinary tract infections (UTIs). Four (13%) of those toilet trained had some symptoms of lower urinary tract dysfunction (LUTD). Three patients (9%) who had congenitally dysplastic kidneys developed hypertension. Comparatively, 14 children with POM underwent reimplantation over 1 year of age. Operative success was 86%, with two children requiring reoperation for recurrent stenosis. Both had evidence of bladder dysfunction before surgery, and thickened bladder walls noted during surgery. One of 14 (7%) had a decline in ipsilateral renal function after recurrent stenosis. Two (14%) had postoperative UTI. Three (25%) had evidence of LUTD. DISCUSSION: There are few data about feasibility and long-term outcomes specifically of ureteric reimplantation for POM in infancy. This is the first study to document long-term outcomes of intravesical ureteric reimplantation in infancy for POM. The recently published British Association of Paediatric Urologists consensus statement supports delaying definitive reimplantation surgery for POM that requires intervention until after 1 year of age. There is little evidence presented for this recommendation. Our study confirms the safety and feasibility of intravesical ureteric reimplantation for POM in infants under age 1, with an operative success rate of 97%, and high renal function preservation rate. UTI risk improved after surgery in children >6 months old and remained stably low in those <6 months of age. Postoperative rates of LUTD were low. Hypertension occurred in only three cases with congenital renal dysplasia. CONCLUSIONS: Ureteric reimplantation in infants under 1 year of age is a safe and effective option for managing POM that requires intervention, and compares favorably with those undergoing reimplantation over 1 year of age.

Benign scrotal masses in children - some new lessons learned.

INTRODUCTION: A preponderance of benign intratesticular masses in pre-pubertal males encourages testicular-sparing surgery (TSS). OBJECTIVE: To review outcome of benign testicular lumps in children managed at a tertiary pediatric center more than 7.5years. METHODS: A retrospective review of pediatric benign testicular lesions from January 2008 to June 2015 was performed. RESULTS: There were twelve benign intratesticular tumors. Of these, 11 were in pre-pubertal males; comprising four teratomas, two epidermoid cysts, one dermoid cyst, two cases of Leydig cell hyperplasia, one cystic dysplasia of the rete testis and one large simple intratesticular cyst. We illustrate a case of Leydig cell hyperplasia presenting with precocious puberty limited to the ipsilateral hemi-scrotum. TSS was attempted in all 11 pre-pubertal cases, but successfully performed in seven. TSS was possible for a large testicular cyst seemingly replacing the entire testis, with evidence that the testis reconstituted itself after surgery. Recurrence of an epidermoid cysts reported. CONCLUSION: For the first time in the literature, this series reports Leydig cell hyperplasia presenting with ipsilateral hemi-scrotal changes of precocious puberty; shows evidence that the testis reconstitutes itself after TSS for a large cyst; and reports recurrence of an epidermoid cyst after TSS.

A modern era comparison of right versus left sided congenital diaphragmatic hernia outcomes.

BACKGROUND/PURPOSE: This study aims to retrospectively review outcomes, including neurodevelopmental outcomes, of neonatal right sided congenital diaphragmatic hernias (RCDH) compared with left sided congenital diaphragmatic hernias (L-CDH) treated surgically at our institute. METHODS: A retrospective review was undertaken of all cases of congenital diaphragmatic hernia (CDH) treated at Princess Margaret Hospital for Children (PMH), Perth, born between 1st January 2002 and 1st August 2012. The outcomes of R-CDH cases were compared with L-CDH cases. We examined duration of ventilatory support, use of patch versus primary closure, the CDH recurrence rates, the number of reoperations and neurodevelopmental follow-up at one year of age. RESULTS: Forty-nine cases of CDH were operated on at PMH during the 10-year period. Of these, ten cases were R-CDH with 39 L-CDH cases. Of 49 cases, 34 were diagnosed antenatally, 5 R-CDH versus 29 L-CDH. Only 8/39 cases of L-CDH required patch repair for larger defects, while 5/10 R-CDH required patch repair. Postoperative mortality was 6/49 (1/10 right sided versus 5/39 left sided). Recurrence was observed in 5/10 R-CDH versus 6/39 L-CDH with p=0.03. Thirty-three of 43 surviving patients received one-year follow-up with Griffiths general quotient (GQ) assessment demonstrating a median score of 98 for L-CDH (IQR 86 to 104.25) and 91 for R-CDH (IQR 76.5 to 93). CONCLUSIONS: R-CDH required patch repair more commonly than L-CDH because of larger defect size or complete agenesis. The rate of recurrent herniation was the only morbidity significantly higher in the R-CDH group. Survivors of R-CDH did not have a significant difference in neurodevelopmental outcome compared to L-CDH cases, with both groups exhibiting normal median GQ scores at one year of age.

Chronic orchalgia after surgical exploration for acute scrotal pain in children.

OBJECTIVES: The aim was to review the pediatric cohort undergoing surgical exploration for acute scrotal pain at our institution and assess the entity of chronic orchalgia post exploration in this cohort. MATERIALS AND METHODS: A retrospective review of all pediatric patients who underwent surgery for acute scrotal pain at a single institution between 1 January 2001 and 1 January 2012 was conducted. RESULTS: A total of 1084 patients underwent scrotal exploration for acute scrotal pain where the underlying cause could not be clinically ascertained. Causes found at exploratory surgery are shown in the table. Forty-four children (4.1%) re-presented with another episode of acute scrotal pain and underwent re-exploration. A hundred of the 772 children with testicular appendage torsion at initial exploration had unilateral exploration only. Seven (7%) of these re-presented with contralateral appendage torsion. The complication rate of initial scrotal exploration was 5.6% and that of re-exploration was 6.8%. All complications were managed conservatively except for a painful reactive hydrocele that underwent the Jaboulay procedure. Fifteen (1.4%) children in this cohort developed chronic orchalgia. Thirteen (87%) of these had definite pathology found at initial exploration. One of 61 (2%) with postoperative complications (a reactive hydrocele) developed chronic orchalgia. Pediatric chronic pain specialists were consulted for all patients. In 10 of the 15 (67%), significant comorbidities included constipation, anxiety, somatization, hydrocele, dysfunctional voiding, and multiple joint pain. The Jaboulay procedure for reactive hydrocele and re-exploration to pex the testes due to suspected intermittent testicular torsion resolved chronic orchalgia in one patient each. DISCUSSION: Pediatric chronic orchalgia post exploration is uncommon. It has a multifactorial etiology. Comorbidities are common. It is possible that some unexplored patients labeled as chronic orchalgia in the literature may have underlying correctable pathology. Surgically correctable pathology such as intermittent testicular torsion, metachronous testicular appendage torsion, and symptomatic hydrocele or varicocele should be excluded in children with chronic orchalgia. Chronic pain specialists should be consulted and associated comorbidities managed. Prior surgical exploration and testicular fixation in children with chronic orchalgia helped reassure patients and families that there was no underlying surgical cause for the pain and facilitated compliance with chronic pain management. CONCLUSIONS: Pediatric chronic orchalgia has a multifactorial etiology and is uncommon after scrotal exploration surgery. Comorbidities are common and must be managed. Surgical exploration helps reassure patients that there is no correctable cause for the pain and facilitates engagement with chronic pain management.

Inflammatory myofibroblastic tumour of the bladder in children: a review.

INTRODUCTION: Inflammatory myofibroblastic tumours of the bladder (IMTB) are rare, and feature a benign and reactive proliferation of myofibroblasts. 25% of the reported IMTB cases in the literature occur in children. The present study presents a review of IMTB in children. DISCUSSION: The data from 42 reported cases of paediatric IMTB in the world literature are summarised, including two recent cases from the present centre. Paediatric IMTB equally affects males and females. It mainly presents with haematuria, dysuria or abdominal pain. Lesions can vary in size, but mean size is 5.5 cm. Mean age is 7.5 years. The aetiology of IMTB is poorly understood, but includes infective or traumatic aetiologies, or a possible clonal lesion. IMTB may specifically show clonal gene rearrangements involving the anaplastic lymphoma kinase (ALK-1) gene. To differentiate IMTB from rhabdomyosarcoma, tissue diagnosis and careful histological analysis are essential. Tumour biopsy can be achieved by a transurethral approach or a transcutaneous approach with ultrasound guidance. Between 35 and 89% of cases of IMTB express ALK-1 by immunohistochemistry. ALK-1 expression is much less common in other bladder soft tissue tumours. ALK-1 is thus useful in the diagnosis of IMTB. The treatment of choice for IMTB is complete surgical resection of the lesion. In children, no proven recurrent or metastatic IMTB episodes are reported after excision. However IMTB recurrences are reported in adults, likely due to incomplete excision. Follow-up after excision is therefore recommended. CONCLUSIONS: Paediatric IMTB is uncommon. Tissue biopsy is essential for diagnosis. Careful histological assessment is required to differentiate IMTB from malignant paediatric bladder tumours such as rhabdomyosarcoma. ALK-1 expression is useful in confirming the diagnosis of IMTB. Treatment of choice is complete surgical resection of the lesion. Recurrence is reported in adult IMTB. Follow-up is therefore recommended.

Management of duplex-system ureterocele.

AIM: To analyse different treatment modalities, functional outcome and continence in children treated for duplex-system ureterocele and to review the relevant literature. METHODS: The medical records of patients with duplex-system ureterocele treated between 2001 and 2011 were reviewed retrospectively. RESULTS: Twenty-two cases were identified. Five patients underwent incision of the ureterocele as initial procedure. It was curative in only one patient. Seven patients underwent upper-pole nephroureterectomy. It was curative in 4 cases. Five patients underwent excision of ureterocele and common-sheath reimplant, and the remaining 5 patients had upper-pole nephroureterectomy and simultaneous excision of ureterocele with lower-moiety ureteric reimplantation. These surgeries were curative in all patients. Follow-up ranged from 4 to 84 months. Functional outcome was good in all patients. Fourteen patients were continent at follow-up, and continence was not assessed in the other 8 because of young age. CONCLUSIONS: Our data suggest a higher rate of secondary procedures if there is retained ureterocele. Data also suggest that complete reconstruction can be safely performed in a young infant without any adverse effect on continence.

Peritoneal drainage versus laparotomy as initial surgical treatment for perforated necrotizing enterocolitis or spontaneous intestinal perforation in preterm low birth weight infants.

BACKGROUND: Standard surgical management of infants with perforated necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP) is laparotomy with the resection of the necrotic or perforated segments of the intestine. Peritoneal drainage is an alternative approach to the management of such infants. OBJECTIVES: To evaluate the benefits and risks of peritoneal drainage compared to laparotomy as the initial surgical treatment for perforated NEC or SIP in preterm infants. SEARCH STRATEGY: Cochrane Central Register of Controlled Trials (CENTRAL), (The Cochrane Library 2010, Issue 3), MEDLINE (1966 to July 2010), EMBASE (1980 to July 2010), CINAHL (1982 to July 2010), previous reviews and cross-references were searched. Abstracts of paediatric academic society meetings were also searched (online: 2000 to 2009; handsearching Pediatric Research: 1995 to 2000). SELECTION CRITERIA: All randomised or quasi-randomised controlled trials in preterm (< 37 weeks gestation), low birth weight (< 2500 g) infants with perforated NEC or SIP allocated to peritoneal drainage or laparotomy as initial surgical treatment. DATA COLLECTION AND ANALYSIS: Data were excerpted from the trial reports and analysed according to the standards of the Cochrane Neonatal Review Group. MAIN RESULTS: Only two randomised controlled trials (RCT) met the eligibility criteria. Overall, no significant differences were seen between the peritoneal drainage and laparotomy groups regarding the incidence of mortality within 28 days of the primary procedure (28/90 versus 30/95; typical relative risk (RR) 0.99, 95% CI 0.64 to 1.52; N = 185, two trials); mortality by 90 days after the primary procedure (typical RR 1.05, 95% CI 0.71 to 1.55; N = 185, two trials) and the number of infants needing total parenteral nutrition for more than 90 days (typical RR 1.18, 95% CI 0.72 to 1.95; N = 116, two trials). Nearly 50% of the infants in the peritoneal drainage group could avoid the need for laparotomy during the study period (44/90 versus 95/96; typical RR 0.49, 95% CI 0.39 to 0.61; N = 186, two trials). One study found that the time to attain full enteral feeds in infants ≤ 1000 g was prolonged in the peritoneal drainage group (mean difference (MD) 20.77, 95% CI 3.62 to 37.92). AUTHORS' CONCLUSIONS: Evidence from two RCTs suggests no significant benefits or harms of peritoneal drainage over laparotomy. However, due to the very small sample size, clinically significant differences may have easily been missed. No firm recommendations can be made for clinicians. Large multicentre randomised controlled trials are needed to address this question definitively.

Spin on perinatal testicular torsion.

We describe a recent case of perinatal testicular torsion at our institution. The presentation, management and outcome of perinatal testicular torsion are quite different to testicular torsion in the general paediatric population. The literature describes a variety of management options for perinatal testicular torsion and these are briefly reviewed. In cases of unilateral perinatal testicular torsin, there is controversy over whether surgery to fix the contralateral testis is required, and if so, the appropriate timing for the surgery. A good understanding of the issues unique to perinatal torsion will facilitate appropriate counseling of parents of affected neonates.

Timing of morphologic and apoptotic changes in the sheep fetal kidney in response to bladder outflow obstruction.

PURPOSE: Posterior urethral valves are the main cause of bladder outflow obstruction in human fetuses. Thirty per cent of boys with valves develop end-stage renal disease, despite intervention in the postnatal period. The timing and mechanisms of renal damage in bladder outflow obstruction are unknown. We investigated the timing of changes in morphology and apoptosis in the fetal sheep kidney in response to obstruction. MATERIALS AND METHODS: Thirty-three fetal lambs at day 70 of gestation underwent surgical creation of bladder outflow obstruction. Twenty-nine fetal lambs had sham surgery. Fetal kidneys were collected 2, 5, 10, 20 and 30 days after surgery. Renal histology was examined. Real-time PCR was used to quantify the renal cortical expression of the pro-apoptotic gene Bax and anti-apoptotic gene Bcl-X. The TUNEL technique was used to assess regional renal apoptosis in response to obstruction. RESULTS: Changes in renal morphology were evident as early as 2 days after surgery in fetuses with bladder outflow obstruction, and progressed over 20-30 days to cystic renal dysplasia. Bladder outflow obstruction increased the renal cortical expression of Bax relative to Bcl-X. Tubular apoptosis peaked after 2 days of obstruction. Blastemal apoptosis peaked after 5 days of obstruction. CONCLUSIONS: Changes in pro- and anti-apoptotic gene expression in the fetal renal cortex, and alterations in the number of apoptotic cells and renal morphology are evident soon after the onset of bladder outflow obstruction. These findings suggest that damage to the developing fetal kidney begins to occur at the onset of obstruction. Attempts to preserve renal function by antenatal interventions may best be achieved by early treatment.

Rates for mastectomy are lower in women attending a breast-screening programme.

BACKGROUND: Mammographic screening for breast cancer facilitates earlier recognition of lesions, thus potentially allowing for breast-conserving surgery. Few studies have compared the final surgical outcomes of women presenting through breast screening programmes with those presenting via other sources. Are breast cancer patients presenting through BreastScreen more likely to undergo breast-conserving surgery than those presenting from other sources? METHODS: Using the Royal Perth Hospital (RPH) Multidisciplinary Breast Service Database, the final surgical outcomes were reviewed for 723 women treated for breast cancer at RPH between January 2000 and August 2002. During this period, 397 patients were referred to the RPH Multidisciplinary Breast Clinic from BreastScreen WA, and 326 were referred from other sources. RESULTS: Of all patients undergoing surgery for breast cancer, 58% in the screen group and 36% in the non-screen group had breast-conserving surgery (P < 0.0001). When surgical outcomes for women in the BreastScreen target age range of 50-69 years were analysed, 59.5% in the screen group and 42.3% in the non-screen group had breast-conserving surgery (P < 0.001). Patient choice was second only to disease extent as a factor determining the outcome of mastectomy. In both cohorts, more than 40% of patients who underwent re-excisional surgery for positive margins, after initial breast-conserving surgery, had residual invasive or in situ disease present. CONCLUSIONS: At RPH, BreastScreen patients were more likely to undergo breast-conserving surgery than those who presented from other sources. A significant proportion of women with positive margins after initial breast-conserving surgery had residual in situ or invasive disease. Re-excision for positive margins was thus warranted.