Search

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.)

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: Patients with anorectal malformation (ARM) need long-term follow-up, in order to evaluate fecal continence; the main predictors of longer-term success are the type of ARM, associated anomalies and sacral integrity. Three-Dimensional High Resolution Anorectal Manometry (3D-HRAM) gives detailed information on pressure on the anal complex profile. Our objective was to analyze anal sphincter activity in ARM patients with 3D-HRAM establishing the correlation between manometric and clinical data., Methods: Forty ARM patients were submitted to 3D-HRAM: manometric, anatomical and clinical scores were correlated with each other and with the bowel management response (BM)., Results: A positive correlation between all scores and types of ARM was found: in high ARM and in patients with spinal anomalies (regardless to ARM type) lower scores were reported and even after BM they did not achieve good continence., Conclusions: 3D-HRAM gives detailed data on the functional activity of the anal sphincter complex. Our study revealed a correlation between manometric parameters and clinical outcomes, confirming spinal malformations and ARM type as the most important prognostic risk factors for a bad outcome. Specific sphincteric defects can also be explored with manometry, allowing for tailored bowel management strategies.

Fosfomycin in intravenous (IV) formulation has re-emerged as a valuable tool in the treatment of multi-drug resistant (MDR) and extensively drug-resistant (XDR) infections because of its broad spectrum of antibacterial action and pharmacokinetic characteristics. This retrospective study aimed to evaluate how fosfomycin was used in patients admitted to the Polyclinic of Palermo between January 2017 and July 2022. Clinical indications, therapeutic associations, clinical outcomes, and any side effects were analyzed. Intravenous fosfomycin was used in 343 patients, 63% male, with a mean age of 68 years (range 15-95). Urinary tract infections (UTIs) and hospital-acquired pneumonia (HAP) were the main indications for treatment (19% and 18% of the total cases, respectively), followed by skin and soft tissue infections and sepsis. IV fosfomycin was administered in combination with other antibacterial agents, the most common of which were ceftazidime/avibactam (35%), meropenem (17%), and colistin (14%). Nineteen patients received it as monotherapy for UTIs. About 66% had resolution of the infectious process with clinical remission (cure or discharge). Electrolyte disturbances occurred in 2.6% and gastrointestinal symptoms occurred in 2.9%. The data showed that IV fosfomycin is a safe and effective therapeutic option in the treatment of infections with multidrug-resistant microorganisms.

Background: Congenital syphilis (CS) depends on the placental transmission of Treponema pallidum (TP) spirochetes from an infected mother to fetus during pregnancy. It shows a wide clinical variability with cutaneous and visceral manifestations, including stillbirths, neonatal death, and asymptomatic cases. Preterm infants with CS may have more severe features of disease than the term ones, due to the combined pathogenic effect of both CS and prematurity., Case Presentation: We report on a female preterm (32 +6  weeks of gestation) newborn showing most of the typical CS manifestations, in addition to gastrointestinal disorders including feeding difficulties, colon stenosis and malabsorption leading to postnatal growth restriction. The mother resulted positive at the syphilis screening test of the first trimester of pregnancy, but she did not undergo any treatment. At birth, our newborn was VDRL positive (antibody titer four times higher compared to the mother), and she was treated with intravenous benzathine benzylpenicillin G for 10 days (50,000 IU/Kg three times per day). Poor tolerance to enteral nutrition (abdominal distension, increased biliary type gastric secretions) was observed. A barium enema X-Ray identified a colon stenosis within the descending tract. However, the poor general conditions due to a concurrent fungal sepsis did not allow to perform any surgical procedure, and a conservative approach with total parenteral nutrition was started. The following evolution was marked by difficulties in enteral feeding including refusal of food and vomiting, to which also contributed the neurological abnormalities related to a perinatal asphyxia, and the affective deprivation for the physical absence of the mother during hospitalization. At 5 months of age, after the introduction of an amino acid-based formula (Neocate LCP Nutricia ®), an improvement of enteral feeding was observed, with no further and significantly decreased episodes of abdominal distension and vomiting respectively, and regular stool emission. A psychological support offered to the family allowed a more stable bond between the mother and her baby, thus providing a significant additional benefit to food tolerance and growth. She was discharged at 5 months of age, and included in a multidisciplinary follow-up. She at present shows global growth delay, and normal development apart from mildly increased tone of lower limbs., Conclusions: Our report highlights less common clinical CS manifestations like gastrointestinal disorders including feeding difficulties, colon stenosis and malabsorption leading to postnatal growth delay. Moreover, it underlines how prematurity may worsen the clinical evolution of such congenital infection, due to the additional pathogenic effect of possible associated diseases and/or conditions like sepsis, hypoxic/ischemic injury, or use of drugs. CS may be observed also in high-income countries, with high rates of antenatal screening and availability of prenatal treatment. A multidisciplinary network must be guaranteed to the affected subjects, to ensure adequate care and improve the quality of life for patients and their families., (© 2022. The Author(s).)

Background: Osteopathia Striata with Cranial Sclerosis (OS-CS), also known as Horan-Beighton Syndrome, is a rare genetic disease; about 90 cases have been reported to date. It is associated with mutations (heterozygous for female subjects and hemizygous for males) of the AMER1 gene, located at Xq11.2, and shows an X-linked pattern of transmission. Typical clinical manifestations include macrocephaly, characteristic facial features (frontal bossing, epicanthal folds, hypertelorism, depressed nasal bridge, orofacial cleft, prominent jaw), hearing loss and developmental delay. Males usually present a more severe phenotype than females and rarely survive. Diagnostic suspicion is based on clinical signs, radiographic findings of cranial and long bones sclerosis and metaphyseal striations, subsequent genetic testing may confirm it., Case Presentation: Hereby, we report on a female newborn with frontal and parietal bossing, narrow bitemporal diameter, dysplastic, low-set and posteriorly rotated ears, microretrognathia, cleft palate, and rhizomelic shortening of lower limbs. Postnatally, she manifested feeding intolerance with biliary vomiting and abdominal distension. Therefore, in the suspicion of bowel obstruction, she underwent surgery, which evidenced and corrected an intestinal malrotation. Limbs X-ray and skull computed tomography investigations did not show cranial sclerosis and/or metaphyseal striations. Array-CGH analysis revealed normal findings. Then, a target next generation sequencing (NGS) analysis, including the genes involved in skeletal dysplasias, was performed and revealed a de novo heterozygous nonsense mutation of the AMER1 gene. The patient was discharged at 2 months of age and included in a multidisciplinary follow-up. Aged 9 months, she now shows developmental and growth (except for relative macrocephaly) delay. The surgical correction of cleft palate has been planned., Conclusions: Our report shows the uncommon association of intestinal malrotation in a female newborn with OS-CS. It highlights that neonatologists have to consider such a diagnosis, even in absence of cranial sclerosis and long bones striations, as these usually appear over time. Other syndromes with cranial malformations and skeletal dysplasia must be included in the differential diagnosis. The phenotypic spectrum is wide and variable in both genders. Due to variable X-inactivation, females may also show a severe and early-onset clinical picture. Multidisciplinary management and careful, early and long-term follow-up should be offered to these patients, in order to promptly identify any associated morbidities and prevent possible complications or adverse outcomes., (© 2022. The Author(s).)

Background: Dysphagia in neurologically impaired children is associated with feeding difficulties, malnutrition and aspiration pneumonia. Esophageal high-resolution manometry (HRM) has been used in the diagnosis of motility disorders affecting the swallowing process. The aim of this study was to analyze swallowing functions in NI children by using HRM in order to establish swallow parameters identifying inhalation risk., Methods: Twenty-five NI children with cerebral palsy were submitted to esophageal HRM with UES analysis, comparing the results with non-NI children. The following parameters were evaluated: maximum pressure and duration of contraction of the velopharynx (VP) and tongue base (TB), and maximal, minimal, resting pressure and relaxation duration of the upper esophageal sphincter (UES)., Results: pVP max, pTB max, pUES max and resting pressure were lower, while p UES minimal was higher and relaxation duration was shorter in NI children vs. the control group. Predictive values of inhalation risk were evaluated., Conclusions: This study evaluates inhalation risk in NI children using HRM to study UES function. Our results confirm the alterations described in NI children: insufficient contraction and clearing force for bolus transmission through the pharynx and incomplete UES relaxation can predispose to pharyngeal residues and inhalation independently of swallowing because of lower values of UES resting.

Background: Cardio-facio-cutaneous syndrome (CFCS) belongs to RASopathies, a group of conditions caused by mutations in genes encoding proteins of the rat sarcoma/mitogen-activated protein kinase (RAS/MAPK) pathway. It is a rare syndrome, with about 300 patients reported. Main clinical manifestations include facial dysmorphisms, growth failure, heart defects, developmental delay, and ectodermal abnormalities. Mutations (mainly missense) of four genes (BRAF, MAP 2 K1, MAP 2 K2, and KRAS) have been associated to CFCS. However, whole gene deletions/duplications and chromosomal microdeletions have been also reported. Specifically, 19p13.3 deletion including MAP 2 K2 gene are responsible for cardio-facio-cutaneous microdeletion syndrome, whose affected subjects show more severe phenotype than CFCS general population., Case Presentation: Hereby, we report on a female newborn with prenatal diagnosis of omphalocele, leading to further genetic investigations through amniocentesis. Among these, array comparative genomic hybridization (a-CGH) identified a 19p13.3 microdeletion, spanning 1.27 Mb and including MAP 2 K2 gene. Clinical features at birth (coarse face with dysmorphic features, sparse and friable hair, cutaneous vascular malformations and hyperkeratotic lesions, interventricular septal defect, and omphalocele) were compatible with CFCS diagnosis, and further postnatal genetic investigations were not considered necessary. Soon after discharge, at around 1 month of life, she was readmitted to our Neonatal Intensive Care Unit due to repeated episodes of vomiting, subtending a hypertrophic pyloric stenosis (HPS) which was promptly identified and treated., Conclusions: Our report supports the 19p13.3 microdeletion as a contiguous gene syndrome, in which the involvement of the genes contiguous to MAP 2 K2 may modify the patients' phenotype. It highlights how CFCS affected subjects, including those with 19p13.3 deletions, may have associated gastrointestinal defects (e.g., omphalocele and HPS), providing further data on 19p13.3 microdeletion syndrome, and a better characterization of its genomic and phenotypic features. The complex clinical picture of such patients may be worsened by additional, and even precocious, life-threatening conditions like HPS. Clinicians must consider, anticipate and/or promptly treat possible medical and surgical complications, with the aim of reducing adverse outcomes. Extensive diagnostic work-up, and early, continuous, and multidisciplinary follow-up, as well as integrated care, are necessary for the longitudinal clinical evolution of any single patient., (© 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.

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: 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: 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.)

Background: VATER association was first described in 1972 by Quan and Smith as an acronym which identifies a non-random co-occurrence of Vertebral anomalies, Anal atresia, Tracheoesophageal fistula and/or Esophageal atresia, Radial dysplasia. It is even possible to find out Cardiovascular, Renal and Limb anomalies and the acronym VACTERL was adopted, also, embodying Vascular, as single umbilical artery, and external genitalia anomalies., Methods: Data on patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) admitted in the Neonatal Intensive Care Unit (NICU) between January 2003 and January 2013 were evaluated for the contingent occurrence of typical VACTERL anomalies (VACTERL-type) and non tipical VACTERL anomalies (non-VACTERL-type). The inclusion criterion was the presence of EA with or without TEF plus two or more of the following additional malformations: vertebral defects, anal atresia, cardiovascular defects, renal anomalies and lower limb deformities, like radial dysplasia., Results: Among 52 patients with EA/TEF, 20 (38,4%) had isolated EA and 7 (21,8%) had a recognized etiology such a syndrome and therefore were excluded. Among 32 infants with EA and associated malformations, 15 (46,8%) had VACTERL association. The most common anomalies were congenital heart defects (73,3%), followed by vertebral anomalies (66,6%). Many patients also had additional non-VACTERL-type defects. Single umbilical artery was the most common one followed by nervous system abnormalities and anomalies of toes. Between the groups of infants with VACTERL type and non-VACTERL-type anomalies, there are several overlapping data regarding both the tipically described spectrum and the most frequently reported non-VACTERL-type malformations. Thus, it is possible to differentiate infants with a full phenotype (VACTERL full phenotype) and patients that do not meet all the criteria mentioned above, but with some homologies with the first group (VACTERL partial phenotype)., Conclusion: The high frequency of non-VACTERL-type anomalies encountered in full and partial phenotype patients would suggest the need for an extension of the clinical criteria for the diagnosis of VACTERL association and also for pre- and post-operative management and follow-up in the short and long term.

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.)

Objective: Multichannel intraluminal impedance (MII) directly evaluates esophageal bolus transport. There is a good correlation between MII and manometry in healthy adults, but there are no reports concerning children.The aim of the present study was to determine normal values of esophageal motility using only impedance measurements in healthy children and in a pediatric population with gastroesophageal reflux (GER)., Patients and Methods: We described in the present study 60 children submitted to pH-MII for 24 hours for suspected GER. Patients were divided into 2 different groups on the basis of their pH-MII report. Group 1 patients showed acid GER, whereas group 2 patients had negative pH-MII analysis for GER despite symptoms. We described impedance reflux and motility parameters on 10 standardized swallows: number of reflux, mean acid clearing time, median bolus clearing time, bolus presence time, total bolus transit time, segmental transit time, and total propagation velocity., Results: In group 1, the median mean acid clearing time was 151 seconds, whereas the median mean bolus clearing time was 25 seconds. In group 2 patients, all of the reflux parameters were normal. In group 1 the median bolus presence time at each measuring site, the median total bolus transit time, and the median segmental transit time were significantly greater and total propagation velocity lower than values reported in group 2 (P < 0.001), if compared with those described for adult patients., Conclusions: The pH-MII is an ideal test in children because it studies GER with its characteristics and motility pattern. Our report summarizes for the first time impedance motility parameters in healthy children.

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.

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.

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.

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.

Unlabelled: In a study from Italy, colour-Doppler ultrasonography was a reliable diagnostic tool in the preoperative assessment of patients with varicocele. The authors also found that it helped to distinguish those who could be treated laparoscopically from those who should be treated by microsurgical subinguinal ligature., Objective: To investigate whether colour Doppler ultrasonography (CDUS) is a reliable diagnostic tool for selecting patients with varicocele to undergo either laparoscopy or open microsurgical subinguinal ligation., Patients and Methods: In a 3-year period, 42 boys affected by left varicocele were evaluated before surgery by inguinal and scrotal CDUS. Using this method it was possible to distinguish Coolsaet type-1 varicocele (due to isolated renal-internal spermatic vein reflux) and Coolsaet type-3 varicocele (due to associated renal-internal spermatic reflux and iliac-deferential reflux). Boys with Coolsaet type-1 varicocele were treated by a laparoscopic transperitoneal Palomo procedure, whereas those with Coolsaet type-3 varicoceles were treated by lymphatic-sparing microsurgical subinguinal ligation., Results: The varicocele was Coolsaet type-3 in six patients (14%), who had microsurgical open surgery, and the remaining 36 (86%) had Coolsaet type-1 and had laparoscopic surgery. At the follow-up there was no venous scrotal reflux. In two patients in the laparoscopic group a hydrocele developed after surgery, which resolved spontaneously., Conclusions: This study showed that CDUS was a reliable diagnostic tool for assessing boys with varicocele. It clearly distinguished Coolsaet-type 1 varicoceles that can be treated laparoscopically, from Coolsaet type-3 varicoceles that should be treated with microsurgical subinguinal ligature.