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Background: Endoscopic treatment of vesicoureteral reflux (VUR) is an important minimally invasive surgical approach in patients undergoing surgical treatment of VUR. In our past experience, we observed that a bulking agent mound sagittal diameter of 10 mm is the main predictor of effectiveness of the procedure. Moreover we noticed that the use of intraoperative ultrasound, allows the surgeon to better identify the site, volume and shape of the bulking agent injected, finally reducing operative time., Objective: We aimed to evaluate if the intraoperative ultrasound assistance could definitively improve effectiveness of the endoscopic procedure., Methods: We retrospectively compared two series treated with endoscopic procedures for intermediate and high grade primary VUR, respectively without (series A) and with (series B) intraoperative ultrasound (IO-US). In all patients VCUG was performed to assess VUR grade and to verify resolution or VUR downgrading during the follow-up., Results: A total of 177 ureteric units were treated. Endoscopic procedures globally were effective in 68/96 ureters (70.8 %) in series A and in 68/81 ureters (83.9 %) in series B. No significant differences in effectiveness were observed comparing the series with regard to VUR grades, but a significant difference is shown (p < 0.05) when grouping grades III-V VUR. No significance in differences of volume injected were detected, but operative time was significantly lower in series B (27.5 min vs 19.6 min, p < 0.05). Mean sagittal mound diameter measured during cystoscopy in series B was 10.45 mm (range 8.5-14.2 mm)., Discussion: The intraoperative ultrasound assistance during endoscopic treatment of VUR could represent a valid tool for surgeons to better identify location, volume and shape of the bulking agent. Furthermore, the use of an objective parameter of evaluation of the implant can overcome the subjective intraoperative evaluation of the implant itself, improving results for experienced surgeons and reducing the learning-curve for inexperienced ones., Conclusions: Results of endoscopic injection of bulking-agent can be improved with intraoperative ultrasound, allowing at the same time a significant reduction of operative time., Competing Interests: Conflict of Interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Tumors are rare in neonatal age. Congenital mesoblastic nephroma (CMN) is a usually benign renal tumor observed at birth, or in the first months of life. It may also be identified prenatally and associated with polyhydramnios leading to preterm delivery. Effective treatment is surgical in most cases, consisting in total nephrectomy. In literature, very few studies report on the neonatal management of such a rare disease, and even less are those describing its uncommon complications., Cases Presentation: We report on two single-center newborns affected with CMN. The first patient is a preterm female baby, born at 30 + 1 weeks of gestation (WG) due to premature labor, with prenatal (25 WG) identification of an intra-abdominal fetal mass associated with polyhydramnios. Once obtained the clinical stability, weight gain, instrumental (computed tomography, CT, showing a 4.8 × 3.3 cm left renal neoformation) and histological/molecular characterization of the lesion (renal needle biopsy picture of classic CMN with ETV6-NTRK3 translocation), a left nephrectomy was performed at 5 weeks of chronological age. The following clinical course was complicated by intestinal obstruction due to bowel adherences formation, then by an enterocutaneous fistula, requiring multiple surgical approaches including transitory ileo- and colostomy, before the conclusive anastomoses intervention. The second patient is a 17-day-old male term baby, coming to our observation due to postnatal evidence of palpable left abdominal mass (soon defined through CT, showing a 7.5 × 6.5 cm neoformation in the left renal lodge), feeding difficulties and poor weight gain. An intravenous diuretic treatment was needed due to the developed hypertension and hypercalcemia, which regressed after the nephrectomy (histological diagnosis of cellular CMN with ETV6-NTRK3 fusion) performed at day 26. In neither case was chemotherapy added. Both patients have been included in multidisciplinary follow-up, they presently show regular growth and neuromotor development, normal renal function and no local/systemic recurrences or other gastrointestinal/urinary disorders., Conclusions: The finding of a fetal abdominal mass should prompt suspicion of CMN, especially if it is associated with polyhydramnios; it should also alert obstetricians and neonatologists to the risk of preterm delivery. Although being a usually benign condition, CMN may be associated with neonatal systemic-metabolic or postoperative complications. High-level surgical expertise, careful neonatological intensive care and histopathological/cytogenetic-molecular definition are the cornerstones for the optimal management of patients. This should also include an individualized follow-up, oriented to the early detection of any possible recurrences or associated anomalies and to a better quality of life of children and their families., (© 2023. Società Italiana di Pediatria.)

Primary buried (BP) penis is describes as a small penis caused by a penile ligaments anomaly; it is unclear if a primary BP could reach a normal length. We selected 49 patients treated at our institution between 2015 and 2020 in order to post-operatively evaluate the SPL after one year. SPL was evaluated according to the PH Tanner staging system for pre-pubertal patients according to age-normalized values. A micropenis was detected if the SPL was below 2.5 SD. A normal SPL was found in thirty-two patients, eighteen were in PH Stage 1, four were in PH Stage 2, six were in PH Stage 3, and four were in PH Stage 5. Seventeen patients showed a reduced SPL; in seven of these (four in PH Stage 4 and three in PH Stage 5), their SPL was <2.5 ST. The difference in micropenis prevalence between the pre-pubertal and post-pubertal patients was significant ( p = 0.038). A primary BP grows normally during the pre-pubertal period, where patients frequently showed a normal SPL, but it seems to be unable to reach a normal length in the higher PH stages, where the SPL is used to detect a micropenis. We suggest that a primary BP should be considered not as a simple defect of the penile ligaments and surrounding tissues, but as an incomplete manifestation of a micropenis due to a growth slowdown of the organ in late puberty.

Background: Duplications of the long arm of chromosome 3 are rare, and associated to a well-defined contiguous gene syndrome known as partial trisomy 3q syndrome. It has been first described in 1966 by Falek et al., and since then around 100 patients have been reported. Clinical manifestations include characteristic facial dysmorphic features, microcephaly, hirsutism, congenital heart disease, genitourinary anomalies, hand and feet abnormalities, growth disturbances and intellectual disability. Most of cases are due to unbalanced translocations, inherited from a parent carrying a balanced aberration (reciprocal translocation or inversion), and rarely the genomic anomaly arises de novo. Very few studies report on the prenatal identification of such rearrangements., Case Presentation: Hereby, we report on a newborn with a rare pure duplication of the long arm of chromosome 3. Noninvasive prenatal test (cell free fetal DNA analysis on maternal blood), performed for advanced parental age and use of assisted reproductive technique, evidenced a partial 3q trisomy. Then, invasive cytogenetic (standard and molecular) investigations, carried out through amniocentesis, confirmed and defined a 3q27.1-q29 duplication spanning 10.9 Mb, and including about 80 genes. Our patient showed clinical findings (typical facial dysmorphic features, esotropia, short neck, atrial septal defect, hepatomegaly, mild motor delay) compatible with partial trisomy 3q syndrome diagnosis, in addition to pre- and postnatal overgrowth., Conclusions: Advanced parental age increases the probability of chromosomal and/or genomic anomalies, while ART that of epigenomic defects. Both conditions, thus, deserve more careful prenatal monitoring and screening/diagnostic investigations. Among the latter, cell free fetal DNA testing can detect large segmental aneuploidies, along with chromosomal abnormalities. It identified in our patient a wide 3q rearrangement, then confirmed and defined through invasive molecular cytogenetic analysis. Neonatologists and pediatricians must be aware of the potential risks associated to duplication syndromes. Therefore, they should offer to affected subjects an adequate management and early and careful follow-up. These may be able to guarantee to patients satisfactory growth and development profiles, prevent and/or limit neurodevelopmental disorders, and timely recognition of complications., (© 2023. The Author(s).)

Objective: This study aimed to investigate clinical, surgical, and genetic data of neonates with anorectal malformation (ARM)., Study Design: A retrospective observational study was conducted on neonates with ARM as an isolated type (group 1), with ≤2 (group 2), and with ≥3 associated malformations (group 3), born between 2009 and 2020. Distribution of ARM, associated abnormalities and genetic testing were analyzed, and risk factors for adverse outcomes were identified., Results: The 45 ARM cases (36% females) were divided as follows: 13 neonates belonging to group 1 (29%), 8 to group 2 (18%), and 24 to group 3 (53%). Cases were equally distributed over 11 years. Krickenbeck anatomy was: without fistula/imperforate anus (18%), perineal fistula (36%), rectourethral fistula (4%), rectovesical fistula (2%), vestibular fistula (4%), cloaca (4%), and rare ARMs (31%). Groups showed differences in anthropometric data, Krickenbeck anatomy, and intensive care burden. Additional major congenital abnormalities were prevalent specific of VATER/VACTERL spectrum (vertebral/anorectal/cardiac/tracheoesophageal/renal/limb defects), but also Hirschsprung disease was found in 3/20 biopsies (15%). The most frequent minor abnormality was a single umbilical artery. In group 3, we identified four de novo microdeletions at 8p23.2, 8q13.3, Xp22.31-p22.2, Xq28, four de novo microduplications at 1p36.32, 6p24.1-p23, 13q14.11, 15q11.2, one microdeletion at 9q33.1 inherited from the affected mother, one microdeletion at 7q35 inherited from the unaffected father, one structurally uncharacterized rearrangement involving 9p23-q34.3. Thus, we attributed the Xq28 deletion with inactivated FAM58A gene in one girl to the X-linked dominant STAR syndrome (toe syndactyly-telecanthus-anogenital/renal malformations)., Conclusions: Despite the great physical and social burden on ARM patients and their parents, in the majority of cases, the etiology is largely unknown and attributed to be multifactorial. In females, STAR syndrome should be part of the differential diagnosis. Associated malformations of other organ systems interact in outcome parameters.

Background: Cat eye syndrome (CES) is a rare chromosomal disease, with estimated incidence of about 1 in 100,000 live newborns. The classic triad of iris coloboma, anorectal malformations, and auricular abnormalities is present in 40% of patients, and other congenital defects may also be observed. The typical associated cytogenetic anomaly relies on an extra chromosome, derived from an inverted duplication of short arm and proximal long arm of chromosome 22, resulting in partial trisomy or tetrasomy of such regions (inv dup 22pter-22q11.2)., Case Presentation: We report on a full-term newborn, referred to us soon after birth. Physical examination showed facial dysmorphisms, including hypertelorism, down slanted palpebral fissures, and dysplastic ears with tragus hypoplasia and pre-auricular pit. Ophthalmologic evaluation and heart ultrasound identified left chorioretinal and iris coloboma and ostium secundum type atrial septal defect, respectively. Based on the suspicion of cat eye syndrome, a standard karyotype analysis was performed, and detected an extra small marker chromosome confirming the CES diagnosis. The chromosomal abnormality was then defined by array comparative genome hybridization (a-CGH, performed also in the parents), which identified the size of the rearrangement (3 Mb), and its de novo occurrence. Postnatally, our newborn presented with persistent hypoglycemia and cholestatic jaundice. Endocrine tests revealed congenital hypothyroidism, cortisol and growth hormone (GH) deficiencies, which were treated with replacement therapies (levotiroxine and hydrocortisone). Brain magnetic resonance imaging, later performed, showed aplasia of the anterior pituitary gland, agenesis of the stalk and ectopic neurohypophysis, confirming the congenital hypopituitarism diagnosis. She was discharged at 2 months of age, and included in a multidisciplinary follow-up. She currently is 7 months old and shows a severe global growth failure, and developmental delay. She started GH replacement treatment, and continues oral hydrocortisone, along with ursodeoxycholic acid and levothyroxine, allowing an adequate control of glycemic and thyroid profiles as well as of cholestasis., Conclusions: CES phenotypic spectrum is wide and highly variable. Our report highlights how among the possible associated endocrine disorders, congenital hypopituitarism may occur, leading to persistent hypoglycemia and cholestasis. These patients should be promptly assessed for complete hormonal evaluations, in addition to major malformations and midline anomalies. Early recognition of such defects is necessary to decrease fatal events, as well as short and long-term related adverse outcomes., (© 2022. The Author(s).)

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&#95;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).)

Introduction: Autosomal recessive polycystic kidney disease (ARPKD; MIM#263200) is one of the most frequent pediatric renal cystic diseases, with an incidence of 1:20,000. It is caused by mutations of the PKHD1 gene, on chromosome 6p12. The clinical spectrum is highly variable, ranging from late-onset milder forms to severe perinatal manifestations. The management of newborns with severe pulmonary insufficiency is challenging, and causes of early death are sepsis or respiratory failure. In cases of massive renal enlargement, early bilateral nephrectomy and peritoneal dialysis may reduce infant mortality. However, there is no conclusive data on the role of surgery, and decision-making is driven by patient's clinical condition and expertise of the center., Patient Presentation: We hereby describe a preterm female newborn with perinatal, rapid and bilateral, abnormal growth of both kidneys, respiratory failure and initial signs of liver disease. She was subsequently confirmed to be affected by a rare and severe homozygous mutation of the PKHD1 gene, inherited from both her consanguineous parents. Our patient died 78 days after birth, due to a fungal sepsis which worsened her respiratory insufficiency., Conclusions: This patient report shows some of the clinical and ethical issues of neonatal ARPKD, and the need of multidisciplinary approach and good communication with the family. Target next generation sequencing (NGS) techniques may guide and support clinicians, as well as guarantee to these patients the most appropriate clinical management, avoiding unnecessary and/or disproportionate treatments.

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.

Introduction: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy., Methods: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units., Results: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

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.)

Background: Congenital pelvic skeletal anomalies (CPSA) may appear as isolated defects or in association with other anomalies like congenital malformations of the digestive system (CMDS). Minor CPSA in non-syndromic patients are often overlooked. We aimed to assess the frequency of CPSA in newborns with CMDS to review the diagnostic approaches., Study Design: A retrospective review of medical records of 201 newborns who underwent X-rays for different neonatal indications was conducted. In 122 patients CMDS were diagnosed and classified according to the ICD-10 classification; 79 non-CMDS patients acted as controls. Pelvic skeletal segments were examined by X-rays., Results: Patients with CMDS, showed a higher risk of CPSA (Odds ratio 2.89; 95% CI 1.34 6.23) and other associated malformations in comparison to non-CMDS patients. Newborns with malformations of the large intestine have the highest risk of adjacent CPSA (48%), as it is a developmental defect originating from the same somite. In addition to skeletal agenesis/hypoplasia, we reported dysmorphic and bifid vertebras, trident ileum, and elongated neural arches., Conclusions: The high incidence of CPSA in CMDS suggests performing a routine radiographic pelvic evaluation in cases of CMDS in order to identify complex phenotypes that could originate from the same developmental field., Competing Interests: Declaration of competing interest None., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Purpose: Undetected refluxing venous systems could cause persistence/recurrence of varicoceles in patients undergoing varicocelectomy. Color Doppler ultrasound (CDUS) is an important tool in the diagnosis and follow-up of varicocele, and could be successfully used to detect a venous reflux in the iliac-deferential district, usually involved in the recurrence/persistence of varicocele., Materials and Methods: We compared 2 historical series of patients treated with Palomo laparoscopic varicocelectomy between 1994 and 2018. In group 1, preoperative scrotal CDUS was obtained, while in group 2, additional inguinal CDUS was performed in order to detect a refluxing deferential vein (DV). When a deferential reflux was found, the DV and internal spermatic vein were interrupted during the same Palomo laparoscopic varicocelectomy., Results: A total of 449 patients underwent left laparoscopic varicocelectomy; 146 of them were not studied for deferential reflux with CDUS (group 1), while in the remaining 303, routine CDUS research of deferential reflux was obtained (group 2). The persistence/recurrence rate was significantly higher in group 1 (13.7 vs. 1%, p < 0.000)., Conclusions: The research of a refluxing DV revealed a useful, cost-effective, and simple tool, allowing a better comprehension of the vascular anatomy of varicocele and, thus, a significant reduction of varicocele persistence/recurrence rate., (© 2020 S. Karger AG, Basel.)

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.

Background: Female vaginoplasty reconstruction, by choice, is usually performed with adjacent tissue. However in some clinical conditions such as high urogenital confluence sinus, cloacal malformation with extreme vaginal hypoplasia, local tissue may not be available. When vaginal replacement is performed in pediatric patients intestinal segments is preferred to non-operative procedures that require continuative dilations. However mucus production, malignant transformation risk and diversion colitis are important side effects., Technique: We present a nouvel technique for vaginoplasty in a female child presenting with an isolated urogenital sinus malformation without virilization. The patient at 20 months underwent vaginoplasty using tubularized bladder mucosal graft., Results: Surgical procedure was devoid of complications. Pubertal development occurred at age of 15. She underwent regular follow up until 18 years of age. At this age we performed clinical evaluation: absence of vaginal introitus stenosis and good cosmetic results were observed. Then she underwent vaginoscopy with multiple biopsies. Pathology examination of the bladder mucosal graft evidenced a normal structure of the mucosa, with a stratified squamous epithelium., Discussion: Different techniques are taken into account for vaginal reconstruction according to the severity and to the type of malformation. We describe the use of bladder mucosal graft with favorable results after long term follow-up., (Copyright © 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study was to define different characteristics of infants with esophageal atresia and correlations with neonatal level of care, morbidity and mortality occurring during hospital stay., Methods: Charts of all newborns with esophageal atresia (EA) admitted to our University NICU between January 2003 and November 2016 were reviewed and subdivided in four groups related to different clinical presentations; EA as an isolated form (A), with a concomitant single malformation (B), as VACTERL association (C), and in the context of a syndrome or an entity of multiple congenital anomalies (D)., Results: We recruited 67 infants with EA (with or without tracheoesophageal fistula), distributed in groups as follows: A 31.3%, B 16.4%, C 26.8% and D 25.3%. Type of atresia was not statistically different among different groups. Mortality was higher in groups C and D, especially if associated with congenital heart defects. In survivors, we found different auxological evolution and prognostic profiles considering duration in days of invasive mechanical ventilation and total parenteral nutrition, as well as length of stay and corrected gestational age at discharge., Conclusions: In the context of genetic and syndromic entities, subjects with VACTERL association showed a lower mortality rate although a higher and more complex level of intensive care was noted in comparison to infants without VACTERL genetic and syndromic entities.

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.)

Objective: In principle, the prepuce can be reconstructed during hypospadias repair, but the procedure has not gained wide acceptance and preputial reconstruction (PR) is surrounded by several controversies., Material and Methods: A review is provided of the technique for PR, how PR combines with the other steps of hypospadias repair, the risks of complications related to the urethroplasty and specific to PR, and the results of PR with particular regard to the relevance for the patient and his family., Results: PR can be important for patients requiring hypospadias repair and their parents. It can be performed in almost all patients with distal hypospadias except perhaps those with the most asymmetrical prepuces or severe ventral skin deficiency. PR does not seem to increase urethroplasty complications, but combination of PR with tubularisation of the urethral plate urethroplasty seems to offer the best chance of success. Specific complications occur in around 8% of patients and include partial or complete dehiscence of the prepuce and secondary phimosis. To prevent the latter, the reconstructed prepuce should be easily retractile at the end of surgery. Technical modifications can help to achieve this goal. Cosmetically, reconstructed prepuces are not fully normal, but the abnormality could be less important for a patient and his parents that the complete absence of the prepuce., Conclusion: On the basis of the evidence summarised above, an algorithm for PR in patients with distal hypospadias is proposed. PR can be offered to the vast majority of distal hypospadias patients, although some modification of the technique for hypospadias repair can be required. Retractility of the reconstructed prepuce at the end of surgery seems paramount for final success., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective This report discusses the role of both congenital and acquired risk factors in the pathogenesis of portal vein thrombosis (PVT). Study Design We describe the clinical management and treatment of PVT in a preterm newborn with a homozygous mutation of the methylenetetrahydrofolate reductase (MTHFR) and plasminogen activator inhibitor-1 (PAI-1) genes and sepsis by Candida parapsilosis. Results Although literature data suggest a minor role of genetic factors in thrombophilia in the case of only one mutation, we hypothesize that combined thrombophilic genetic defects may have a cumulative effect and significantly increase the thrombotic risk. Conclusion It could be appropriate to include more detailed analyses of procoagulant and fibrinolytic factors in the diagnostic workup of neonatal thrombosis, also through the investigation of genetic polymorphisms. The anticoagulant therapy and the removal of concurrent risk factors remain basic steps for the adequate management and prevention of complications., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

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.)

Visceral solitary myofibromas are uncommon in childhood. We report a case of a solitary asymptomatic visceral myofibroma of the bladder trigone occurring in a 3-month-old boy. Once malignancies were ruled out by cystoscopy, radical excision was performed in order to avoid any potential impairment of bladder dynamic. Postoperative course was uneventful and patient was discharged on day 3 after surgery. After 36 months of follow-up, the patient is toilet-trained and remains well; bladder function is normal.

Mature cystic teratoma is the most frequent benign ovarian neoplastic lesion in adolescents and is generally composed of fully differentiated tissue arising from multipotential three germinal layers. It accounts for approximately 50% of benign ovarian tumors in childhood. Rarely, a bilateral, synchronous, or metachronous presentation can be observed, supporting a conservative approach as the first surgical approach. We report a case of an ovarian mature cystic incarcerated in indirect inguinal hernia in a 15-year-old girl undergoing ovary-sparing surgery. To our knowledge this is the first case of such lesion incarcerated in an inguinal hernia reported in literature.

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.)

Observation is a conservative management option in infants with nonrefluxing hydronephrosis, primary nonrefluxing megaureter and ureterocele diagnosed postnatally following antenatal detection of hydronephrosis. Antibiotic prophylaxis might be a sensible regimen under these circumstances to prevent UTI in this population who are potentially at increased risk. However, studies examining the efficacy of prophylactic antibiotics are sparse in this setting. For each condition, prophylactic policies seem extremely variable, and UTI rates vary widely with comparable rates reported between patients followed on and off antibiotics. Overall, antibiotic prophylaxis seems unnecessary in patients with isolated low-grade hydronephrosis. Patients with high-grade nonrefluxing hydronephrosis seem at increased risk of UTI, with risk further increasing in patients with associated ureteral dilatation (hydroureteronephrosis) irrespective of the presence of a ureterocele. Obstruction might be an additional independent risk factor, but the diagnosis of obstruction is often possible only in retrospect. The data available suggest that infants are the most at risk of UTI during the first 6 months of life, particularly if they undergo catheterization during workup examinations. Thus, antibiotic prophylaxis might be prudent during the first 6-12 months of life in patients with high-grade hydronephrosis and hydroureteronephrosis with or without ureterocele, and particularly before completion of the diagnostic workup. Paediatric urologists are urged to embark on controlled trials to compare patients followed with and without antibiotic prophylaxis.

During adolescence, the risk of developing a varicocele increases. Prevalence is less than 1% in boys aged younger than 10 years, but approaches that of the general adult population (about 15%) during puberty. For adolescent males with varicoceles, surgical risk factors have not yet been clearly delineated and clinical severity correlates poorly with prognosis. Fortunately, the widespread use of Doppler ultrasonography is transforming the diagnostic work-up for this demographic. A continuous reflux detected by color Doppler ultrasound (CDUS) is thought to have a negative prognostic value and evidence suggests that a peak retrograde flow above 38 cm per second is a powerful predictor of lack of spontaneous improvement in adolescent patients with ≥ 20% asymmetry between testes. CDUS also enables the detection of varicocele resulting from reflux in the deferential vein adjunctive to a refluxing internal spermatic vein; a causality that accounts for approximately 15% of cases. In addition to a diagnostic role, hemodynamic parameters can be used to predict the risk of persistence or worsening asymmetry. Although further studies are necessary to validate single parameters, it seems that the more severe the reflux, the greater the likelihood that the patient will develop testicular asymmetry.

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.)

Hydrocele is defined as an abnormal collection of serous fluid in the potential space between the parietal and visceral layers of the tunica vaginalis. In the majority of affected adolescents, hydrocele is acquired and is idiopathic in origin. The pathogenesis of idiopathic hydrocele is thought to be an imbalance in the normal process of fluid production and reabsorption. The diagnosis is usually clinical. Taking a thorough history is essential to rule out any fluctuation in size, which is an indication of a patent processus vaginalis. Scrotal ultrasonography is mandatory in nonpalpable testicles to rule out a subtending testicular solid mass requiring inguinal exploration. Otherwise, open hydrocelectomy via a scrotal incision is the standard treatment of idiopathic hydroceles. The second most common cause of hydrocele in adolescents is varicocelectomy. The risk of hydrocele formation is higher with non-artery-sparing procedures or those performed without microsurgical aid, and in surgery requiring cord dissection. If hydrocele occurs after varicocelectomy, initial management should include observation with or without hydrocele aspiration. Large persistent hydroceles are best served by open hydrocelectomy.

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.

Unlabelled: Postoperative pain is less intense after laparoscopic than after open surgery. However, minimally invasive surgery is not a a pain-free procedure. Many trials have been done in adults using intraperitoneal and/or incisional local anesthetic, but similar studies have not yet been reported in the literature in children., Aim: The aim of this study was to evaluate the analgesic effect of periportal infiltration and intraperitoneal instillation of ropivacaine in children undergoing laparoscopic surgery., Materials and Methods: Thirty patients who underwent laparoscopic surgery were randomly allocated to one of three groups. Group A (n = 10) received local infiltration of port sites with 10 mL of ropivacaine. Group B (n = 10) received both an infiltration of port sites with 10 mL of ropivacaine and an intraperitoneal instillation of 10 mL of ropivacaine. Group C did not receive any analgesic treatment. The local anesthetic was always administered at the end of surgery. The degree of postoperative abdominal parietal pain, abdominal visceral pain, and shoulder pain was assessed by using a Wong-Baker pain scale and a Visual Analog Scale (VAS) at 3, 6 12, and 24 hours postoperatively. The following parameters were also evaluated: rescue analgesic treatment, length of hospital stay, and time of return to normal activities., Results: Three hours after operation, patients had low pain scores. Six and 12 hours postoperatively, the abdominal parietal pain was significantly higher (P < 0.0005) in group C than in the other two groups, both treated with an infiltration at the trocar sites; mean intensity of abdominal visceral pain was significantly lower (P < 0.0005) in group B than in groups A and C; the overall incidence of shoulder pain was significantly lower (P < 0.0005) in group B patients than in patients of groups A and C. At 20 hours postoperatively, pain scores were significantly reduced of intensity in all groups. Rescue analgesic treatment was significantly higher in group C, if compared to groups A and B 12 hours after the operation. No statistically significant difference was found in length of hospital stay, but children who received analgesic treatment had a more rapid return to normal activities than untreated patients (P < 0.0005)., Conclusions: Our study demonstrates that the combination of local infiltration and intraperitoneal instillation of ropivacaine is more effective for pain relief in children after laparoscopic surgery than the administration of ropivacaine only at the trocar sites.

Background: This study aimed to assess whether deferential reflux in pediatric and adolescent varicocele can be successfully treated laparoscopically., Materials and Methods: Since 2001 at our institution, 148 boys were evaluated for a left varicocele. Preoperatively,all the patients underwent ultrasound scan assessment of testicular volume and color-Doppler US (CDUS)to rule out reflux into the internal spermatic vein (ISV), deferential vein, or cremasteric vein. Boys with ISV reflux were treated by laparoscopic transperitoneal Palomo; boys with isolated deferential reflux or associated to ISV reflux were laparoscopically managed adding to the former procedure, coagulation or clipping of refluxing deferential veins., Results: Reflux in both the ISV and the deferential vein was observed in 21 (14.1%) out 148 boys with varicocele.Only one case (0.6%) of varicocele was caused by an isolated deferential reflux. No reflux in the cremasteric vein was observed. After a median follow up period of 2 years (range, 6 months-5 years), none of our patients with deferential reflux experienced varicocele recurrence either clinically or according to CDUS scanning.No testicular atrophy was observed., Conclusion: Our diagnostic approach is a rigorous standard for identifying all the venous systems concurring with the varicocele. Our proposed technique with laparoscopic interruption or coagulation of deferential veins when proved by CDUS to be refluxing may allow successful treatment for most varicoceles. This method allows reduction in recurrence of varicocele due to a missed deferential reflux.

The authors present a previously unreported association of different types of intestinal atresia in identical low-birth-weight twins. Both babies were affected by duodenal atresia, associated in the first case with a complete mucosal duodenal membrane and in the second one with an "apple-peel" jejunal atresia. These occurrences may suggest that they were either the consequence of linkage of 2 genes or a pleiotropic expression of a single gene responsible for such rare conditions.

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.

Gartner's duct cysts associated with renal dysgenesis are rare malformations and represent a diagnostic challenge. We report on one such case in which final diagnosis was achieved by laparoscopy and discuss the possible role of minimally invasive surgery in the management of this condition.

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.

Background/aims: The hydrocele rate is very low after microsurgical subinguinal varicocelectomy, but microsurgical expertise is not always available in pediatric centers. We describe a modified technique to reduce the hydrocele rate after subinguinal varicocelectomy performed without an operating microscope., Methods: A retrospective review was performed of 142 non-microsurgical subinguinal varicocelectomies performed at a single pediatric center. In 96 patients, varicocelectomy was combined with the excision and eversion of the tunica vaginalis ('pre-emptive hydrocelectomy') while in the remaining 46 cases the vaginalis was left untouched., Results: Pre-emptive hydrocelectomy allowed a significant reduction in the hydrocele rate in comparison to cases in whom the vaginalis was left untouched, hydrocele rate 4.3 vs. 13% (p = 0.04). In patients undergoing preemptive hydrocelectomy, hydrocele occurred in 3 of 54 (5.5%) cases in whom the vaginalis was only excised and only 1 of 42 (2.4%) in whom it was also everted. No testicular complications were observed., Conclusions: In centers performing subinguinal varicocelectomy without the aid of an operating microscope, pre-emptive hydrocelectomy with eversion of the vaginalis might be offered as an adjunctive treatment with limited associated morbidity that might be able to reduce the incidence of postoperative hydrocele., ((c) 2008 S. Karger AG, Basel.)

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.