Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.

Background: Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years.

Material And Methods: Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences.

Results from the French National Esophageal Atresia register: one-year outcome.

Background: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA).

Methods: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database.

Severe sex differentiation disorder in a boy with a 3.8 Mb 10q25.3-q26.12 microdeletion encompassing EMX2.

The molecular basis of male disorders of sex development (DSD) remains unexplained in a large number of cases. EMX2 has been proposed to play a role in the masculinization process for the past two decades, but formal evidence for this causal role is scarce. The aim of this study is to yield additional support to this hypothesis by reporting on a male patient who presented with 46,XY DSD, a single kidney, intellectual disability, and the smallest microdeletion including EMX2 reported to date.

Esophageal atresia: data from a national cohort.

Purpose: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan.

Methods: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts.

Long-term incidence of febrile UTI after DxHA treatment of VUR.

Purpose: To assess the long-term incidence of febrile urinary tract infection (fUTI) in children treated by endoscopic injection of dextranomer/hyaluronic acid (DxHA) for vesicoureteral reflux (VUR).

Materials And Methods: Prospective study from January 2002 to December 2009 in children treated at our institution for VUR by endoscopic injection of DxHA. All children underwent clinical and renal/bladder ultrasound follow up at 3 months after procedure, then annually.

Robot-assisted surgery: current status evaluation in abdominal and urological pediatric surgery.

Purpose: The last two decades have witnessed a dramatic development of minimally invasive surgery (MIS). Robotic-assisted surgery is currently the latest step in the evolution of MIS. MIS was initially developed for adult surgery, but its use has progressively been extended to pediatrics.

Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study.

Purpose: The aim of the study was to assess quality of life (QOL) of patients born with bladder exstrophy (BE) and reconstructed during early childhood in 7 French university hospitals (QUALEX study: QUAlity of Life of bladder EXstrophy).

Methods: Patients from 6 to 42 years old answered self-administered Short-Form 36 (SF-36), VSP-A (Vécu et Santé Perçue de l'Adolescent), VSP-AE (Vécu et Santé Perçue de l'Enfant), AUQUIE (AUto-QUestionnaire Imagé de l'Enfant), and general questionnaires about functional and socioeconomic data. Dimension scores were compared between adults and adolescents using SF-36 and adolescents and children using VSP-AE.

Follow-up and surgical management of Peutz-Jeghers syndrome in children.

Background: Peutz-Jeghers syndrome (PJS) is an autosomal dominant syndrome with an increased risk of polyposis complications and intestinal and extraintestinal tumours.

Methods: During the last 15 years, we reviewed a series of 11 children with PJS, with special attention to evolution and follow-up. Diagnosis was based on at least 1 hamartomatous polyp associated with 2 of the 3 following criteria: family record of PJS, polyposis localised on small bowel, and mucocutaneous pigmentation.

Prenatal diagnosis of a fetal abdominal eventration: a rare congenital abdominal wall defect.

We report a case of abdominal eventration associated with cystic fibrosis, diagnosed by mid-trimester ultrasonography. The defect concerned the abdominal muscles and their aponevrotic sheath, but respected the skin. There was no associated malformation.

[Malignant rhabdoid tumor of the liver with spontaneous rupture: a case report].

We report the case of an 8-year-old child, presenting a rhabdoid tumor of the liver with spontaneous rupture, revealed by an intra-abdominal bleeding with rapidly fatal course. This clinical and pathological report raises the problem of the differential diagnosis of primary malignant hepatic tumors of the child with no alpha-foetoprotein increase.

Congenital paravertebral arteriovenous fistula: a case report.

Congenital paraspinal arteriovenous fistulae are rare and usually diagnosed after neurologic or cardiovascular manifestations. They may be discovered unexpectedly in children during clinical examination, which reveals the presence of a vascular murmur. The association with multicystic kidney is exceptional.

An exceptional type of anorectal malformation: anorectal atresia with posterior fistula in a newborn male.

The authors report the case of a newborn male with an exceptional type of anorectal atresia with a fistula that passes posterior to the muscle complex and exits at a site just to the tip of the coccyx. This anorectal malformation was associated with other abnormalities including agenesis of the right kidney, pyelic dilatation of the left kidney, duplicity, and left megaureter. There was no defect in the spine.

[Gestational diabetes and urinary tract malformations: a hospital case-control study].

Introduction: Urinary tract malformations represent the leading cause of renal failure in children. Gestational diabetes plays a controversial role in the development of urinary tract malformations. The purpose of this study was to assess the role of insulin-requiring gestational diabetes on the development of ureteric malformations in children.

[Bladder augmentation cystoplasty with continent urinary diversion by ileo-caeco-appendicular graft in children].

Objective: Description of augmentation cystoplasty combined with continent urinary diversion using an ileo-caeco-appendicular graft in children.

Material And Method: Augmentation cystoplasty combined with Mitrofanoff appendicostomy and reimplantation of the ureters into the ileal stump was performed during a single operation in two patients (a 3-year-old girl and an 8-year-old boy). The indications were a pseudoneurogenic bladder with major dilatation of the upper tract due to high-grade reflux in one case and a small scarred bladder after repeated failure of repeated surgery for duplicated ureterocele in another case.

[Vesicoureteric reflux in children: long-term results of endoscopic treatment by Macroplastique injection].

Objective: To evaluate the long-term efficacy (from the fifth year after injection and up to seven years) of endoscopic treatment by Macroplastique implant for vesicoureteric reflux (VUR) in children, sometimes associated with urological malformations or renal complications. The secondary objective was to evaluate the local tolerance of the Macroplastique implant and the development of any local (haematuria, stones, ureteric stasis) or distant complications.

Material And Methods: Retrospective analysis of the medical files of 389 patients, representing a total of 590 refluxing ureters treated by Macroplastique injection in eight different centres.

Current management of infants with fetal renal pelvis dilation: a survey by French-speaking pediatric nephrologists and urologists.

To analyze the current management recommendations among French-speaking physicians treating infants with antenatal renal pelvis dilatation, we surveyed 83 pediatric nephrologists and 68 pediatric urologists by questionnaire. A total of 45 (54%) pediatric nephrologists and 38 (56%) pediatric urologists responded. The threshold for the diagnosis of abnormal fetal renal pelvis dilatation was significantly higher among pediatric urologists than nephrologists.

Interobserver reliability in the interpretation of radiologic signs in Legg-Calvé-Perthes disease.

An accurate interpretation of radiographs is crucial in determining therapeutic choices in Legg-Calvé-Perthes disease. The aim of this study was to measure the interobserver reliability of a group of surgeons in this condition. Twenty-three radiographs were shown to nine pediatric orthopedic surgeons at nine different medical centers.