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Background: Contiguous gene deletion syndrome at Xp22.3 resulting in nullisomy in males or Turner syndrome patients typically encompasses the steroid sulfatase gene (STS) and contiguously located other genes expanding the phenotype. In large deletions, that encompass also the Kallmann syndrome 1 gene (KAL1), occasionally infantile hypertrophic pyloric stenosis (IHPS) and congenital anomalies of the kidney and urinary tract (CAKUT) have been reported., Patient Presentation: We report on a male newborn with family history in maternal uncle of renal abnormalities and short stature still without ichthyosiform dermatosis. The baby presented CAKUT with kidney failure and progressive vomiting. Renal bicarbonate loss masked hypochloremic and hypokalemic metabolic alkalosis classically present in IHPS and delayed its diagnosis. Antropyloric ultrasound examination and cystourethrography were diagnostic. After Fredet-Ramstedt extramucosal pyloromyotomy feeding and growing was regular and he was discharged home. Comparative whole-genome hybridization detected a maternal inherited interstitial deletion of 1.56 Mb on Xp22.31(6,552,712_8,115,153) × 0 involving the STS gene, but not the KAL1 gene., Conclusions: Aberrant cholesterol sulfate storage due to STS deletion as the underlying pathomechanism is not limited to oculocutaneous phenotypes but could also lead to co-occurrence of both IHPS and kidney abnormalities, as we report. Thus, although these two latter pathologies have a high incidence in the neonatal age, their simultaneous association in our patient is resembling not a chance but a real correlation expanding the clinical spectrum associated with Xp22.31 deletions., (© 2022. The Author(s).)

Background: Transanal irrigation (TAI) is employed for children with fecal incontinence, but it can present several problems which require a study of their outcomes among different pathologies and without a tailored work up. The aim of our study was to evaluate the effectiveness of an advanced protocol in order to tailor TAI, prevent complications, and evaluate outcomes., Methods: We included 70 patients (14 anorectal malformation, 12 Hirschsprung's disease, 24 neurological impairment, 20 functional incontinence) submitted to a comprehensive protocol with Peristeen ® : fecal score, volumetric enema, rectal ultrasound, anorectal 3D manometry, and diary for testing and parameter adjustment., Results: Among the patients, 62.9% needed adaptations to the parameters, mainly volume of irrigated water and number of puffs of balloon. These adaptations were positively correlated with pre-treatment manometric and enema data. In each group, the improvement of score was statistically significant in all cases ( p 0.000); the main factor influencing the efficacy was the rate of sphincter anomalies. The ARM group had slower improvement than other groups, whereas functional patients had the best response., Conclusions: Our results showed that TAI should not be standardized for all patients, because each one has different peculiarities; evaluation of patients before TAI with rectal ultrasound, enema, and manometry allowed us to tailor the treatment, highlighting different outcomes among various pathologies, thus improving the efficacy.

Necrotizing enterocolitis (NEC) is a typical disorder of preterm newborns, with a high mortality and morbidity rate. The therapeutic and nutritional management of disease depends on several factors. Its prognosis is linked, in addition to the severity of the disease and the need for surgery, to a correct enteral feeding in these patients. This study aims to identify the clinical characteristics of 18 patients with NEC, evaluating the different therapeutic paths undertaken, the type of formula used and the survival rate of this population. Average time of enteral nutrition before the NEC onset was 11,3 ± 11,6 days, with an average fasting period since the onset of 24 ± 18.9 days. 77.8% of patients received surgery and resumed enteral nutrition 17.7 ± 17.9 days after the intervention. The overall survival rate of our cohort was 55.5%. More prospective studies are needed to evaluate the long-term outcomes of survived children with NEC., (© 2021. The Author(s).)

Background: Hirschsprung disease, the most important congenital colonic dysmotility in children results from neural crest migration, differentiation, proliferation, or apoptosis defects where the rearranged during transfection (RET)-Protooncogene pathway has a central role. Although palatal and retinal anomalies in the context of chromosomopathies and some mono-/oligogenic syndromes are reported associated with Hirschsprung disease the role of inactivating RET mutations in these cases is not clarified., Case Presentation: We report on a dysmorphic newborn with cleft palate and palatal synechia, who showed intestinal obstruction after 24 h of life. Transient ileostomy and surgical biopsies were performed to diagnose aganglionosis of the colon and last ileal loop. No chromosomal anomalies or copy number variations were found. We identified a paternal heterozygous germline mutation c.1852 T > C, which results in the substitution of cysteine by arginine in the RET-receptor tyrosine kinase (p.C618R mutation). There was no family history of Hirschsprung disease, but the father underwent surgery for medullary thyroid carcinoma and was affected by retinal dystrophy., Conclusions: The occurrence of Hirschsprung disease and carcinoma shows how a single mutation may be responsible for adverse effects: gain and loss of function of the same receptor. Furthermore, it would be interesting to study its dual role in face and retina embryology, and to extend targeted investigations of RET hotspots in these developmental abnormalities to facilitate counselling, follow-up, and tumor prevention. Complex surgical procedures and genetic testing as well as socio-economic impact are a challenge for familiar compliance.

Background/introduction: Vesicoureteral reflux (VUR) potentially leads to renal damage, scarring, and eventually end-stage renal disease. Endoscopic treatment is well tolerated in children, it has reduced costs, and it effectively prevents urinary tract infections (UTIs), while avoiding long term antibiotics use., Objective: With the aim to investigate the time needed to reach the stabilization of the dextranomer/hyaluronic acid (Dx/HA) implants and to identify cut-off heights to ensure the success of the procedure, the authors analyzed ultrasonographic (US) intra-operative appearance of the mounds following endoscopic treatment for VUR and repeated the measurements during serial postoperative evaluations. The final clinical goal would be to obtain an alternative parameter that might reduce the need for postoperative voiding cystourethrogram (VCUG)., Study Design: The authors selected all children treated for moderate-high grade VUR with renal scarring or repeated UTI under antibiotic prophylaxis and followed with regular time points for at least 1 year (time points 1, 3, 6, and 9 months). Endoscopic injection performed with double-HIT/STING technique was combined with US to determine the intra-operative mound height and to calculate reabsorption rate. Mound height was measured as the maximal vertical diameter of the mound visualized at the ureteral orifice. Based on postoperative VCUG findings, patients were divided in group A (success of the endoscopic treatment) and group B (persistence of reflux)., Results: Thirty patients aged 1-7 years, counting for 47 ureters, completed the protocol and were included in the analysis. Mounds height had a significant difference between A and B at all time points (P < 0.005). However, height did not differ between 6 and 9 months in both groups. Percentage of reduction in A was significant from 1 to 6 months (P < 0.005) but not onward, while in B, it was never significant. Ultimately, both groups had a comparable trend of reabsorption, with a complete stabilization achieved in 6 months and an overall reduction of approximately 22%., Discussion and Conclusion: Following the endoscopic injection of Dx/HA, US mounds height was found to strongly correlate with VCUG, both intra-operatively and for months following the procedure. Compared with the available literature, the authors first report a fixed reabsorption rate, consistent with the results in animal models and a timeframe to achieve stabilization. The possibility to measure those parameters with US renders this approach useful in the clinical setting, and it justifies the reduced use of VCUG in the follow-up of endoscopic injection for VUR., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Solitary functioning kidney (SFK) may be associated to hypertrophy, hypertension and chronic kidney disease. We evaluated blood pressure (BP) of children with congenital SFK comparing agenesis to multicystic dysplastic kidney (MCDK) and correlated BP profiles with renal dimensions of affected and contralateral kidney., Methods: We compared 40 patients with MCDK, grouped for either treatment options (A: conservative vs B: nephrectomy) or involution time (A1: before 4 years-of-age vs A2: persistence-of-MCDK), to 10 unilateral agenesis (C). Patients were evaluated with ultrasound, scintigraphy, office-ambulatory BP monitoring., Results: Compensatory hypertrophy was demonstrated in most of the subjects, without differences between subgroups, with an increase over time (p < 0.001). A1-C showed an overall percentage of hypertrophy significantly higher than A2-B (83%-88% vs 70%-73%, respectively; p = 0.03); moreover, cumulative risk to develop hypertension in A1-C is significantly higher compared to A2-B in office and ambulatory BP monitoring (p = 0.03). Insufficient dipping in systolic and/or diastolic BP was found in 82% children, without differences between subtypes., Conclusions: Patients with a small/absent dysplastic kidney have an increased risk to develop hypertrophy and hypertension compared to patients with a large residual, regardless of nephrectomy. ABPM revealed absent dipping in most patients with SFK, warning further investigations in apparently not symptomatic patients.

Purpose: Despite minimal invasiveness and high success rate, guidelines still prescribe voiding Cystourethrogram (VCUG) after endoscopic treatment for vesicoureteral reflux (VUR) in children. The aim of this paper was to analyze whether intraoperative ultrasound (IO-US) could improve surgical accuracy and perioperative counseling, thus potentially decreasing the need for postoperative VCUG., Methods: We selected children treated for moderate to high grade VUR, renal scarring or repeated infections under antibiotic prophylaxis from January to December 2015. Endoscopic injection was combined with IO-US to detect optimal needle placement and to guide mound formation. IO-US findings were compared to surgeon opinion and postoperative VCUG, performed 3months after surgery. All patients were followed-up for 1year., Results: A significant relationship was found between IO-US mound height (p=0.003) or localization (p<0.0005) and VCUG. Success of endoscopic treatment vs persistence of reflux groups had a mean mound height of 10.62±1.36mm and 8.39±1.08mm respectively (p<0.0005). Height maintained a significant correlation with success in simple and multivariable regression analysis. ROC curve determined ≥9.8mm as predictor of reflux resolution (95% CI 0.825 to 0.998; p<0.0001)., Conclusions: IO-US facilitates pediatric urologists to find an optimal location, to reach a volcano mound morphology and height, thus increasing intraoperative accuracy. IO-US also helps evaluating high-risk recurrence and guiding prognostic counseling., Type of Study: Treatment study., Level of Evidence: II., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Endoscopic dextranomer/hyaluronic acid copolymer (Dx/HA) injection is a safe and efficacious treatment option for vesicoureteral reflux (VUR) in children. Endoscopic appearance, hydrodistention and amount of injected Dx/HA have been demonstrated not to be reliable predictors of outcome. Aim of this study was to evaluate Dx/HA mounds on ultrasound scans (US) and find out any eventual correlation with reflux resolution., Methods: We selected patients treated with endoscopic injection for moderate to high VUR, renal scaring or repeated infections under antibiotic prophylaxis. Success was defined by absence of VUR at control 3months after surgery; at 3months we also measured mound height ultrasonographically., Results: We considered a total of 32 children (15 male, 17 female; 53 ureters) with a median age of 3years (±24months). Overall success rate was 77% per ureter. Success rate correlates directly with age and inversely with VUR grade. Mound height is the major predictive parameter for reflux resolution (sensitivity 100%, specificity 65.9%); mean mound heights of success-group vs. persistence-of-reflux group were 9.97±1.61mm and 7.29±1.74mm respectively (p<0.0005)., Conclusion: A mound measuring at least 9.8mm at post-operative US scan is a predictor of reflux resolution. Age and grade also seems to influence success rate., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Abnormalities involving the skin coverage of the penis are difficult to define, but they can significantly alter penile appearance, and be a cause of parental concern., Data Sources: The present review was based on a nonsystematic search of the English language medical literature using a combination of key words including "penile skin anomalies" and the specific names of the different conditions., Results: Conditions were addressed in the following order, those mainly affecting the prepuce (phimosis, balanitis xerotica obliterans, balanitis, paraphimosis), those which alter penile configuration (inconspicuous penis and penile torsion), and lastly focal lesions (cysts, nevi and vascular lesions). Most of these anomalies are congenital, have no or minimal influence on urinary function, and can be detected on clinical examination. Spontaneous improvement is possible. In the majority of cases undergoing surgery, the potential psychological implications of genital malformation on patient development are the main reason for treatment, and the age generally recommended for surgery is after 12 months of age., Conclusion: This review provides the pediatrician with a handy tool to identify the most common penile skin anomalies, counsel parents adequately, make sensible and evidence based choices for management, and recognize complications or untoward outcomes in patients undergoing surgery.

The term 'inconspicuous penis' refers to a group of anatomical abnormalities in which the penis looks smaller than is expected. Micropenis can be defined as 'true micropenis'--which results from a defect in the hypothalamic-pituitary-gonadal axis--and 'micropenis secondary to congenital anatomical anomalies of the surrounding and overlying structures'--also known as 'concealed penis'. The different forms of concealed penis include webbed penis, congenital megaprepuce and partially hidden penis caused by prepubic adiposity. This disorder can also have iatrogenic causes resulting from adhesions that are secondary to circumcision--this type of concealed penis is known as 'trapped penis'. However, in both groups, micropenis is defined as a stretched penile length that is at least 2.5 SD below the mean for the patient's age, but without any other penile defects. Patients with true micropenis can be managed with testosterone, which has demonstrated good penile elongation results in the long term. Surgery also has a pivotal role in reconstruction for elongating the penis and for correction of anatomical abnormalities in concealed penis.

Background: Ileocecal (IC) duplication cysts are enteric duplications located at the IC junction, not clearly identified in all the published series. The reported treatment is IC resection and ileocolic anastomosis. It is well known that the loss of the IC valve has several adverse effects. This study is aimed at demonstrating that cyst removal together with the common ileal wall and following enterorrhaphy is possible, safe, and effective in preserving the IC region., Methods: Medical records of 3 patients who underwent surgery for IC duplication between 2003 and 2013 were retrospectively reviewed evaluating follow-up results., Results: All patients had an antenatal diagnosis of intraabdominal cystic mass. In two cases associated malformations were reported. The lesions presented at newborn age with intermittent small bowel obstruction and required removal. No patients underwent IC resection. The diagnosis of duplication cyst was confirmed by histo-pathologic examination. The postoperative course was uneventful, even in the long-term follow-up., Conclusions: Our conservative approach is a simple and safe technique, effective in avoiding the loss of the IC valve in children with duplication at the IC junction., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Failed hypospadias refers to any hypospadias repair that leads to complications or causes patient dissatisfaction. The complication rate after hypospadias repairs ranges from 5-70%, but the actual incidence of failed hypospadias is unknown as complications can become apparent many years after surgery and series with lifelong follow-up data do not exist. Moreover, little is known about uncomplicated repairs that fail in terms of patient satisfaction. Risk factors for complications include factors related to the hypospadias (severity of the condition and characteristics of the urethral plate), the patient (age at surgery, endocrine environment, and wound healing impairment), the surgeon (technique selection and surgeon expertise), and the procedure (technical details and postoperative management). The most important factors for preventing complications are surgeon expertise (number of cases treated per year), interposition of a barrier layer between the urethroplasty and the skin, and postoperative urinary drainage. Major complications associated with failed hypospadias include residual curvature, healing complications (preputial dehiscence, glans dehiscence, fistula formation, and urethral breakdown), urethral obstruction (meatal stenosis, urethral stricture, and functional obstruction), urethral diverticula, hairy urethra, and penile skin deficiency.

Objectives: The use of covering urethroplasty with flaps in hypospadias surgery has been well recommended. Various techniques have been described for flap harvesting. The aim of the present study was to compare the outcome and complication rate of dorsal preputial flaps and ventral dartos flaps., Methods: A total of 130 patients were prospectively evaluated from January 2008 to December 2011. Using the tubularized incised plate urethroplasty procedure, urethroplasty was carried out by a single surgeon. Patients were randomly divided in two groups: group A (57 patients), in which a preputial flap was carried out using three different techniques; and group B (73 patients), in which a single or a double ventral dartos flap was used., Results: A total of 41 complications occurred in 24 patients. Urethrocutaneous fistulas were observed in 14.9%, quite equally distributed between groups A and B. There was only one urethrocutaneous fistula in a patient treated with the double ventral dartos flaps. Five cases (3.8%) of glans dehiscence were observed: four after single ventral dartos flap and one after dorsal preputial flap. Six patients in group A and seven in group B experienced meatal stenosis. In three cases of iatrogenic torsion of the penis, a dorsal preputial flap was laterally transposed. Finally, a lower complication rate was observed for double ventral dartos flap versus the other techniques., Conclusions: The use of a double ventral dartos flap should represent the first-line technique for coverage of distal urethroplasty., (© 2013 The Japanese Urological Association.)

Purpose: The incidence of GER, related symptoms and complications in patients treated for congenital diaphragmatic hernia (CDH) are poorly defined. The aim was to evaluate incidence and development of GER in children treated for CDH in a short- and long-term follow-up period, identifying potential risk factors of morbidity., Methods: Thirty-six patients were evaluated with pH-MII at a median age of 6 months (T1) and re-evaluated with pH-MII and endoscopy at a median age of 5 years (T2)., Results: The incidence of reflux was 83 % in T1 and 61 % in T2; the incidence of symptoms was 62 % in T1 and 38 % in T2. In both groups the reflux was mainly non-acidic. Patch, intrathoracic stomach and esophageal dysmotility were risk factors for GER., Conclusions: The incidence of GER and symptoms decrease over the time but it was higher than in the literature, probably because it is mainly non-acidic and evaluable only with MII. The esophageal dysmotility was found to be the main risk factor. An high incidence of reflux and esophagitis was found also in asymptomatic patients, and so a close follow-up is recommended in all patients even if it is asymptomatic.

Background: Anastomotic stricture is an important problem after esophageal atresia (EA) repair. This study evaluates a technique of oblique esophageal anastomosis without use of a flap in order to prevent stricture formation., Methods: Medical records of 16 patients (14 with EA type III and 2 with EA type IV Ladd-Gross classification) who underwent primary repair of EA at birth without anastomotic tension were reviewed, evaluating long-term follow-up results. All patients were studied with esophageal contrast study, pH-multichannel intraluminal impedance, and endoscopy. The incidence of complications and their management were analysed., Results: Contrast esophagogram and esophagoscopy always showed regular patency of the suture line., Conclusions: Our technique of oblique anastomosis is simple, safe, and effective in preventing stricture formation even in the long-term follow-up., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Gastroesophageal reflux (GER) is frequently observed in children treated for congenital diaphragmatic hernia (CDH) at birth, as well as esophageal dysmotility, that has been hypothesized to be caused by innervatory anomalies. The aim of this study is to evaluate GER and dysmotility in young patients with CDH using pH-multichannel intraluminal impedance., Methods: Thirty children (17 boys and 13 girls) who underwent repair for CDH between 2002 and 2007 with a median age of 5.2 years (range, 3-10 years) were included in the study. All patients were operated on with a subcostal laparotomy incision and had a left-sided diaphragmatic defect. The defect repair required an artificial patch (Goretex, Gore Medical, Flagstaff, AZ) in 8 patients (27%) because of its size. We described impedance reflux parameters and some specific motility parameters studied on 10 standardized swallows., Results: The incidence of GER was 86%. Reflux was mainly nonacidic, postprandial, and short-term and reached only the distal esophagus. Esophageal dysmotility was observed only in the distal esophagus., Conclusions: With the use of pH-multichannel intraluminal impedance, both GER and esophageal motility in patients with congenital malformations can be analyzed. In patients with CDH, impaired motility seems to involve only the distal esophagus. In this group, the specific pattern of reflux is probably caused by the involvement of gastroesophageal junction, without significant intrinsic innervation abnormalities as observed in patients with esophageal atresia., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII)., Methods: Fifteen patients with a mean age of 7.5 years (group 1) have been studied and compared with 15 children without congenital malformation, submitted to pH-MII for suspected GER (group 2). These latter patients serve as a control group of healthy subjects. The following impedance reflux and motility parameters have been studied on 10 standardized swallows: number of reflux episodes, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity., Results: In the group of EA patients, mean acid clearing time and median bolus clearing time were pathological. In the control group, all reflux parameters were normal. Patients with EA had significantly longer median bolus presence time at each measuring site, median total bolus transit time, and median segmental transit time and slower total propagation velocity (P < .001)., Conclusions: pH-multichannel intraluminal impedance evaluates both GER and motility patterns. Our report studies impedance parameters of esophageal motility in healthy children and in EA patients using only pH-MII., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Management of ovarian cysts in infants is controversial; it can be conservative or surgical, and the management is determined by the cyst's size and sonographic features., Methods: A surgical approach using a 10-mm umbilically placed operative laparoscope was taken in 3 female infants with antenatally diagnosed large, simple ovarian cysts. The contents of the cysts were partially aspirated and the cyst walls were stripped off the remaining ovarian parenchyma. No intraoperative or postoperative complications were recorded., Conclusions: The one-trocar video-assisted stripping technique for large ovarian cysts in infants appears to be an ovarian-tissue-preserving procedure, and it sidesteps the disadvantages of large scars and formation of adhesions., (Copyright 2010 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of study was to evaluate if pelvic ultrasound can be useful in managing children with chronic idiopathic constipation., Methods: A total of 270 children with idiopathic chronic constipation were enrolled in the study. At baseline and at monthly checkups children were evaluated by clinical score and pelvic ultrasound (US). Patients have been divided in 2 groups, based on pelvic US results: group A with a rectal diameter >3 cm, group B with a rectal diameter <3 cm or rectum not visualized. Both groups were subsequently randomly divided in two subgroups (A1, A2, B1, B2) on the basis of the prescribed treatment (disimpaction for the first week and daily laxative or only daily laxative)., Results: After 1 month of therapy all clinical features improved in group A1 and at pelvic US, rectal size reduced and became not visualized; group A2 showed poor clinical response and transverse diameter of rectum did not modify significantly; B1 and B2 groups showed significant improvement only after 2-3 months., Conclusions: Rectal disimpaction is necessary only in presence of MR. Pelvic US is a useful to diagnose MR and to set up the most appropriate treatment protocol for different chronic constipation cases.

Purpose: To assess the influence of the method for stent placement, the duration of stenting, and the presence of bladder drainage on the complication rate of open pyeloplasty., Patients and Methods: Complications were, retrospectively, compared in 228 consecutive open pyeloplasties performed at institution A using a trans-pyelostomic 6-Fr splint/stent for 5 days and no bladder drainage, and 150 consecutive open pyeloplasties performed at institution B using a trans-nephrostomic 6-Fr splint/stent for 9 days plus bladder drainage., Results: Median age at surgery was comparable between groups. The overall complication rate was 13% and was comparable at the two institutions, but for the presence of perioperative bleeding that was more common when the stent was placed trans-nephrostomically (institution B) and the rate of stent dislodgements, which was lower at institution A, perhaps due to some technical details aiming to prevent any inadvertent traction on the stent. Additional procedures, such as double J internal stent insertion, were required in <2% of cases., Conclusions: A 5-day period of stenting after open pyeloplasty is generally enough. Trans-nephrostomic and trans-pyelostomic stent placement is equally effective. However, the former can be associated with a slightly higher bleeding rate. Details during stent placement are keys to avoid postoperative dislodgement and malfunctioning. Systematic bladder drainage seems unnecessary.

Background: Stripping of the cystic wall is performed by gynecologists to treat large ovarian cysts. Information in the pediatric population is poor. We prospectively evaluated the pathologic specimens of large ovarian cyst to determine whether the stripping technique is a tissue-sparing procedure even in this age., Methods: We evaluated 5 patients. Samples were taken from the intermediate part of the cystic wall and from the layer covering the cyst during excision. The presence of ovarian tissue adjacent to the cyst wall, and the morphological features of the surrounding tissue were both evaluated. Pelvic ultrasound follow-up was also performed., Results: Patients' mean age was 4.5 years (7 days to 12 years). All cysts were removed because all were symptomatic. The mean diameter was 86.6 mm (74-100 mm). Cysts were follicular in 2 cases, serous in other two, and endometriotic in 1 case. Adjacent ovarian tissue was present in 1 of 5 specimens and was approximately 1 to 2 mm in thickness. The layer adjacent to the cystic wall always appeared as normal ovarian tissue. Ultrasound scans at follow-up revealed presence of ovarian tissue., Conclusion: The stripping procedure for large ovarian cyst excision allows to spare the adjacent normal ovarian tissue even in pediatric age because ovarian tissue is rarely excised with the cyst wall during the procedure.

Objective: To review the evolution in indications for treatment and treatment modalities for adolescent varicocele at our centre, and evaluate the impact of varicocelectomy on final outcome., Patients and Methods: Between 1995 and 2006, we treated 242 left varicoceles. Preoperative assessment included clinical evaluation, measurement of testicular volumes, and colour-Doppler ultrasound (CDUS). A subinguinal varicocelectomy was performed in 124 patients (group A), and a laparoscopic non-artery-sparing Palomo procedure in the remaining 118 (group B). In group B patients, CDUS was also used to investigate the functional anatomy of varicocele, and all the veins found to be refluxing were divided during surgery. The two groups were compared with regard to indications for surgery and outcome., Results: Over time the proportion of patients operated on because of testicular growth retardation increased. Persistence/recurrence rate was comparable between the two groups. In 13% of group B patients, the deferential vein was found to be refluxing on preoperative CDUS and was divided at surgery. Hydrocele rate was higher in group A, unless the vaginalis was excised and everted during varicocelectomy. About 75% of patients with preoperative left testicular growth failure experienced postoperative catch-up growth, irrespective of treatment., Conclusion: Indications for treatment are still evolving. Varicocele can successfully be treated in the majority of cases by either a laparoscopic or subinguinal approach. Both techniques require care, and CDUS can aid in the decision making. Most patients with preoperative testicular growth failure experience postoperative catch-up growth.

Aim: Testicular torsion (TT) requires prompt diagnosis and treatment to avoid testicular loss. Most studies have focused on the ideal work up to rule TT out in cases of acute scrotum. We attempted here to define objective criteria to select between orchidopexy and orchidectomy in patients undergoing surgery for TT., Patients and Methods: Fifteen boys with a median age at presentation of 7.8 (range 6.4-12) years undergoing surgical treatment for TT underwent color-Doppler ultrasound (CDU) preoperatively, and a bleeding test intraoperatively. Duration of preoperative history, degree of torsion, CDU findings and degree of bleeding were analyzed., Results: Salvageability was independent of the degree of torsion. In patients with a history longer than 10h, no flow on CDU and no bleeding, after orchidectomy all the testicles were necrotic on pathology. When all these variables were negative, all the testicles did well during follow up. In the group of patients with no agreement among the analyzed variables, the outcome was unpredictable. Five out of six underwent orchidopexy, but in two cases the testicle atrophied (in spite of flow on CDU in one)., Conclusions: No predictive parameters were found for testicular salvageability. Taken as a whole, the parameters studied can be of help in treatment choice. In patients with no agreement among the parameters, orchidopexy seems the appropriate option, but parents should be informed of the risk of testicular atrophy during follow up.

Prenatal exposure to antiepileptic drugs (AEDs) increases the risk of major congenital malformations (MCM) in the fetus. AED-related abnormalities include heart and neural tube defects, cleft palate, and urogenital abnormalities. Among the various congenital anomalies of the kidney and urinary tract (CAKUT), multicystic dysplastic kidney (MCDK) disease is one of the most severe expressions. Although prenatal ultrasound (US) examination has increased the prenatal diagnosis of MCDK, the pathogenesis is still unclear. We report on four cases of MCDK in infants of epileptic women treated with AEDs during pregnancy. From October 2003 to June 2006, we observed four infants with unilateral MCDK born to epileptic women. Three patients were considered to have typical features of multicystic dysplastic kidney, and one infant was operated because of a cystic pelvic mass in the absence of a kidney in the left flank. The macroscopic appearance of this mass showed an ectopic multicystic kidney confirmed by histological findings. All patients have been studied by US scans, voiding cystourethrogram (VCUG), and radionuclide screening isotope imaging. The prenatal exposure to AEDs increases the risk of major congenital malformations from the background risk of 1-2% to 4-9%. AEDs may determine a defect in apoptosis regulation that could lead to abnormal nephrogenesis, causing MCDK. Carbamazepine (CBZ) and phenobarbital (PHB) during pregnancy should be used at the lowest dosage compatible with maternal disease. The reduction, or even suspension, of drug dosage should be achieved from the periconceptional period to the first 8 weeks of gestation to avoid any interference with organogenesis.