Financial and relational impact of having a boy with posterior urethral valves.

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care.

Patients And Method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards.

Risk factors for febrile urinary tract infection in boys with posterior urethral valves.

Objective: Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors could allow for tailoring UTI prevention. The aim of this study was to use the data from the CIRCUP randomized controlled trial data to identify patient characteristics associated with a higher risk of fUTI.

Circumcision and Risk of Febrile Urinary Tract Infection in Boys with Posterior Urethral Valves: Result of the CIRCUP Randomized Trial.

Background: Boys with posterior urethral valves (PUVs) have an increased risk of febrile urinary tract infections (fUTIs). Circumcision is believed to reduce the risk of fUTIs in boys, although there are no randomized trials demonstrating this.

Objective: To determine the effect of circumcision on the risk of fUTIs in boys with PUVs.

Biliary and duodenal complications after « en bloc» liver-small bowel transplantation in children. A single center cohort study.

Background: The technique of « en bloc» liver and small bowel transplantation (L-BT) spares a biliary anastomosis, but does not protect against biliary complications. We analyze biliary and duodenal complications (BDC) in our pediatric series.

Methods: Between 1994 and 2020, 54 L-BT were performed in 53 children.

ZORRO: Z Omphaloplasty Repair for Omphalocele.

Purpose: To suggest a novel technique for omphalocele closure which uses the circular base of the umbilical cord, thus allowing for a more physiological healing process and natural-looking scar.

Methods: Among 16 neonates operated for omphalocele between 2011 and 2016, 12 were closed with a one-stage procedure using a Z omphaloplasty (ZORRO). Median gestational age was 36.

Incidence and survival of childhood cancer in the French islands of Reunion and Mayotte (2005-2011).

The aim of this study is to describe childhood cancer incidence and survival in the French islands of Reunion and Mayotte for the period 2005-2011. Data were taken from the population-based Cancer Registry of Reunion Island. All incident cases of malignant tumours and benign tumours of the Central Nervous System diagnosed between 2005 and 2011 in children under the age of 15 and living in Reunion or Mayotte were included.

Long-term results of the transmanubrial osteomuscular-sparing approach for pediatric tumors.

Background: The complete and safe resection of pediatric cervicothoracic tumors, mostly represented by neurogenic tumors, remains a surgical challenge because of the complex anatomy of this region. The transmanubrial osteomuscular-sparing approach (TOSA) is an alternative to isolated or combined cervical and thoracic approaches enabling the control of supra-aortic vessels and nerves through the thoracic inlet.

Methods: We retrospectively reviewed the tumor characteristics, completeness of resection, morbidity, and long-term outcome of patients with cervicothoracic tumors removed by TOSA between 2000 and 2012 in our institution.

Management of nonparasitic splenic cysts in children: A French multicenter review of 100 cases.

Background: The management of nonparasitic splenic cysts (NPSC) remains controversial. Surgical resection is indicated for symptomatic or complicated forms, but no guidelines are available for asymptomatic NPSC. The aims of this study were to evaluate the management of NPSC in French hospitals and to analyze the results of management.

Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center.

Our aim was to describe our achievements in pediatric intestinal transplantation (ITx) and define areas for improvement. After a period (1987-1990) of nine isolated small bowel transplants (SBTx) where only one patient survived with her graft, 110 ITx were performed on 101 children from 1994 to 2014: 60 SBTx, 45 liver-small bowel, four multivisceral (three with kidneys), and one modified multivisceral. Indications were short bowel syndrome (36), motility disorders (30), congenital enteropathies (34), and others (1).

Laparoscopic Splenectomy in Children: A Modified Technique with Joint Sealing of Segmental Splenic Arteries and Veins Favorably Compared with the Classic Technique.

 Splenectomy in children is nowadays widely performed by laparoscopy, the major risk being operative hemorrhage. We hereby compare the results of two different techniques of vascular control.  We retrospectively reviewed all patients undergoing a laparoscopic splenectomy (LS) for hematologic disease at a single institution from 2004 to 2013.

Preliminary experience using a tunica vaginalis flap as the dorsal component of Bracka's urethroplasty.

Objective: To evaluate clinical use of a tunica vaginalis flap as the dorsal component of a two-stage urethroplasty in boys with cripple hypospadias.

Patients And Methods: We performed the first stage of a Bracka two-stage urethroplasty, using a tunica vaginalis flap as the dorsal component in six boys with cripple hypospadias. We analysed their clinical characteristics and the results of this technique.

A novel approach to evaluating the benefit of post-urinary tract infection renal ultrasonography, using decision curve analysis.

Background: The benefit of post-urinary tract infection (UTI) sonography to detect clinically significant renal abnormalities remains a subject open to debate. Decision curve analysis (DCA) is a novel method for evaluating the clinical usefulness of diagnostic tests. Our objective was to determine, using DCA, the benefit of post-UTI sonography and of post-UTI sonography with biological markers of inflammation to predict the risk of recurrence of febrile UTI in children aged 2 to 24 months without known uropathy.

Male infants with hypospadias and/or cryptorchidism show a lower 2D/4D digit ratio than normal boys.

Background: In humans the ratio of the index finger to the ring finger is sexually dimorphic, with the mean ratio being larger in women than in men. It has been suggested that this difference is related to prenatal androgen exposure. This has been further demonstrated in children with congenital adrenal hyperplasia.

Assessment and outcome of children with intestinal failure referred for intestinal transplantation.

Background & Aims: Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF.

Delayed diagnosis of neurological bladder following spinal cord injury without radiological abnormality.

Spinal cord injuries are a well-known cause of neurological bladder and a well-known complication of high-velocity trauma. They can, however, be difficult to diagnose in young children, in whom the absence of initial neurological symptoms and normality of the radiological workup can be falsely reassuring. Management of associated injuries can further delay diagnosis.

MYO5B and bile salt export pump contribute to cholestatic liver disorder in microvillous inclusion disease.

Unlabelled: Microvillous inclusion disease (MVID) is a congenital disorder of the enterocyte related to mutations in the MYO5B gene, leading to intractable diarrhea often necessitating intestinal transplantation (ITx). Among our cohort of 28 MVID patients, 8 developed a cholestatic liver disease akin to progressive familial intrahepatic cholestasis (PFIC). Our aim was to investigate the mechanisms by which MYO5B mutations affect hepatic biliary function and lead to cholestasis in MVID patients.