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Lymphatic malformations are rare benign developmental anomalies of the lymphatic system that can be diagnosed by prenatal ultrasound. Depending on their anatomical site and size, the lesions can cause a variety of aesthetic and functional deficits. Several treatment options are available, the most suitable is still under debate. The experience gained at our Centre and the review of the literature can be useful to improve prenatal counseling, that is challenging due to the heterogeneity of clinical presentation and treatment.

Introduction: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients., Materials and Methods: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire., Results: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient., Conclusions: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors., Type of the Study: retrospective case series., Level of Evidence: IV., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase., Objective: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC. The main goal of the intervention program was to educate the adolescents and re-frame how they see themselves or feel about themselves, especially in relation to BEEC. In particular it was predicted that the program could: (1) improve the perception of pleasure concerning the body, particularly regarding the genital area, giving proper and specific information on pleasure, masturbation and medical history of BEEC; and (2) elicit a more relational-affective perspective on sexuality., Study Design: 13 adolescent patients took part in the 1-year program. The effects of the intervention program were verified through a test-retest methodology using Sexuality Evaluation Schedule Assessment Monitoring (SESAMO)., Results: The results showed that participants changed their attitude in several psychosexual areas, more specifically: psycho-environmental situations, body experience, areas of pleasure, medical and sexual history, and motivation and conflict areas (Summary Table)., Discussion: This study demonstrated, for the first time, that a targeted program may significantly improve the psycho-sexual condition of adolescents with BEEC. In particular, this research showed that adolescents need to be able to discuss and tackle topics of a psychological and sexual nature, as well as receive understandable answers that can be put into practice in their everyday lives. The study had several methodological limitations, especially owing to the limited number of participants, the absence of a follow-up period of a few months after the intervention, and the overall exploratory nature of the program., Conclusion: This intervention methodology may be considered a first attempt at improving the self-esteem of adolescents with BEEC, by contrasting forms of psychological difficulties in order to improve the quality of life of these young people., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Study Objective: To report the clinical findings and treatment results of a series of patients with epithelial tumors of the ovary, registered and treated prospectively in a multi-institutional Italian network (TREP project) on rare tumors in children and adolescent between 2000 and 2014., Design, Setting, and Participants: Data on 16 patients, aged 22 to 206 months, from 7 centers were reviewed. All patients were grouped on the basis of the results of the first surgical approach, according to the Children Oncology Group staging system., Results: The most frequent symptom was abdominal pain; 3 patients were hospitalized for acute pain after ovarian torsion. Initial surgical treatment was complete in 15 of 16, and a biopsy was performed in 1 of 16. The histology examination revealed 8 benign tumors (7 mucinous cystadenomas and 1 serous cystadenoma) and 8 borderline tumors (2 serous and 6 mucinous). Fifteen of 16 patients maintained the complete remission after surgical treatment alone; 1 affected by Proteus syndrome died consequent to the progression of a synchronous Wilms tumor, after a delayed incomplete surgery., Conclusion: Our analysis documented the rarity of these tumors in children and adolescents. No malignant histologies were found. Surgery alone was effective to cure the majority of patients. Considering the rarity of malignant entities, ovary-sparing surgery may be planned when an epithelial tumor is suspected on the basis of the preoperative work-up and intraoperative findings., (Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

A lower urinary tract fistula consist in an abnormal connection between bladder, urethra and adjacent abdominal organs or skin. There are several types of urinary fistulas in paediatric age and they may be congenital or acquired. Etiology may be due to embriological defects, infectious processes, malignant tumours, pelvic irradiation as well as complications following surgical procedures, especially postsurgical repair of hypospadia or epispadia. Clinical presentation depends on the type of fistula and diagnosis is based on signs, symptoms and radiological or endoscopic examinations. We performed PubMed research using terms such as lower urinary fistulae, urology and paediatrics and we consulted medical texts. We reviewed selected articles and used the relevant ones to perform our study concentrating on classification, diagnosis and treatment of different types of fistulas. Paediatric lower urinary fistulas are an uncommon pathology, but the knowledge of their etiology and classification is important to recognise them and lead the physician to an appropriate treatment, which is surgical in most cases.

Background: Pediatric studies reported that the combined use of the anti-neutrophil cytoplasm autoantibodies (ANCA) and the anti-Saccharomyces cerevisiae mannan antibodies (ASCA) may be a specific useful noninvasive test in the diagnosis of inflammatory bowel diseases (IBD)., Aims: To evaluate the diagnostic accuracy of ANCA and ASCA in children with suspected IBD, and to see whether different commercially available assays (indirect immunofluorescence vs. ELISA) agree well enough in terms of analytical performance., Patients and Methods: Sixty-nine children (30 males, 39 females, age range 2-18 years) with suspicion of IBD entered the study. Before colonoscopy, a blood sample was also drawn to assess ASCA and ANCA., Results: A diagnosis of IBD was established in 47 patients; the remainder had infective or other causes of colitis. For ulcerative colitis, the association ASCA-/ANCA+ had 70% sensitivity and 86% specificity, with a positive predictive value of 82%. The association ASCA+/ANCA- had 86% sensitivity and 93% specificity for Crohn's disease, with a positive predictive value of 75%., Conclusion: Although more experience is needed to state the diagnostic power of serologic assay, determination of ANCA and ASCA in IBD children may help both in distinguish these conditions from other entities and ulcerative colitis from Crohn's disease, particularly in doubtful cases., ((c) 2007 S. Karger AG, Basel)

Background: We previously reported that stage 3 neuroblastoma comprises (i) a low-risk group including all infants (age 0-11 months) as well as older children with non-abdominal primaries, and (ii) a high-risk group made up of children >1 year of age with abdominal primaries. Aggressive chemotherapy was effective only in the latter group., Patients and Treatment: On this basis, in 1990 we designed a new protocol by which all low-risk patients received standard-dose chemotherapy, while the high-risk ones received very aggressive chemotherapy., Results: Between November 1990 and December 1997 a total of 95 eligible and evaluable children were enrolled: 47 were low-risk (35 infants and 12>1 year of age at diagnosis and having non-abdominal primaries), and 48 were high-risk (being >1 year of age and having abdominal primaries). Of the 47 low-risk patients, five relapsed and four subsequently died. The 5-year overall survival (OS) was 91%. Of the 48 patients in the high-risk group, 22 relapsed or progressed, 18 of whom died from their disease and two from toxicity, and one was lost to follow-up. The 5-year OS was 60%. Univariate analysis showed that age, site of primary, risk-group, urine vanillylmandelic excretion, plasma levels of lactate dehydrogenase, ferritin and neurone-specific enolase, and MYCN status correlated with outcome. However, multivariate analysis showed that only MYCN status retained prognostic value., Conclusions: In low-risk stage 3 neuroblastoma, standard-dose chemotherapy is associated with an excellent chance of being cured. Aggressive chemotherapy is effective for high-risk patients, but results are still unsatisfactory. MYCN gene amplification is a prognostic indicator for most, but not all, treatment failures.