Alice Faure

Management and outcome challenges in newborns with gastroschisis: A 6-year retrospective French study.

Abstract Objective: To identify the gestational age (GA) at which risk of mortality and severe outcome was minimized comparing preterm delivery and expectant management. Methods: Retrospective study performed between 2009 and 2014 of newborns with gastroschisis in three large French level III neonatal intensive care units. Each department followed two distinct strategies: elective delivery at 35 weeks’ GA and a delayed approach. Results: We included 69 gastroschisis cases. The lengths of stay lasting more than 60 days were significantly greater in the planned delivery group than in the expectant approach group (18/30 (60%) vs. 8/39 (20.5%), p = 0.001). Gastroschisis cases receiving antenatal corticoids during the last two weeks of gestation required significantly less surgeries during their initial stay (p = 0.003) as well as shorter parenteral feedings (p = 0.002). A multivariate logistic regression showed that a GA of less than 36 weeks’ GA was is a pejorative factor for a stay above 60 days, regardless of whether it was a simple or complex gastroschisis, (OR= 3.8; p = 0.021). A complex gastroschisis was a risk factor for significantly longer parenteral feedings, regardless of the center where patient is treated (Beta = −0.3, p = 0.035). Conclusions: Future research should focus on decisions about delivery timing by incorporating risk of neonatal morbidity.

Value of diagnosis imaging in the evaluation of the severity of histological lesions in duplex systems.

OBJECTIVE In order to determine the effectiveness of imagery in the assessment of the severity of histological lesions in duplex systems in children we compared histology results from heminephrectomies with diagnosis imaging findings [renal ultrasound (US), scintigraphy, unenhanced and contrast-enhanced magnetic resonance imagery (MRI)]. MATERIALS AND METHODS Between 2007 and 2013, 34 children with duplex system underwent surgery. The results from US (n = 34), dimer captosuccinic acid scintigraphy (n = 23) and MRI (n = 16) were compared with histological data. Five histological lesions were found (chronic interstitial inflammation, interstitial fibrosis, tubular atrophy, glomerulosclerosis and dysplasia) and categorized as severe (>25%) or moderate (≤ 25%). RESULTS Severe histological lesions were found in 76.5% and moderate lesions in 23.5%. Radiological features were compared with histological results. In US, severe parenchymal thinning was associated with chronic interstitial inflammatory. The absence of parenchymal enhancement and/or severe cortical thinning in MR urography (MRU) was significantly associated with interstitial fibrosis. All poorly functioning poles were associated with severe histological lesions (p = 0.091), but not to a specific category of lesions. CONCLUSIONS MRI sensibility was excellent (90%) in the diagnosis of poorly functioning pole. Severe thinning on US and minimal pole function on MRU can be used to predict the severity of histological lesions.

Long-Term Efficacy of Young-Dees Bladder Neck Reconstruction: Role of the Associated Bladder Neck Injection for the Treatment of Children With Urinary Incontinence

OBJECTIVE To compare the long-term efficacy of Young-Dees bladder neck reconstruction (YDBNR) alone versus YDBNR plus bladder neck injection (BNI) in patients with urinary incontinence caused by urethral sphincter insufficiency. PATIENTS AND METHODS Between 1987 and 2006, we assessed the continence rates obtained with YDBNR and BNI as a supplementary treatment for persistent outlet insufficiency in patients with neurogenic bladder (group 1, n = 35) and bladder exstrophy (group 2, n = 20). Median postoperative follow-up was 16 years (range: 5-29). RESULTS A total of 55 children (23 males and 32 females) underwent YDBNR at the median age of 7.6 years (range: 1.9-17.25). Only 10 patients (18%) were considered continent after the isolated YDBNR; 17% (n = 6/35) from group 1 and 20% (n = 4/20) from group 2 (p = 1). Because of unsatisfactory results after YDBNR, 81.8% (n = 45/55) received BNI. Fifteen patients (33%) became continent with an average of 2.29 injections (±1.1); 44.8% (n = 13/29) from group 1 and 12.5% (n = 2/16) from group 2. A significant difference was found on comparing the social continence rate attained with YDBNR plus BNI between patients from group 1 and group 2 (54% vs 30%, P = .04). The difference between males and females in terms of continence rates was not statistically significant. CONCLUSION Long-term results of YDBNR are modest. BNI does increase the results of YDBNR, especially in patients with neurogenic bladder.

Predicting postnatal renal function of prenatally detected posterior urethral valves using fetal diffusion‐weighted magnetic resonance imaging with apparent diffusion coefficient determination

The objective of this study was to evaluate the accuracy of fetal diffusion‐weighted magnetic resonance imaging with apparent diffusion coefficient (ADC) determination to predict postnatal renal function (nadir creatinine at 1 year and eGFR) of men with posterior urethral valves (PUV).

Neurodevelopment and Health-Related Quality of Life in Infants Born with Gastroschisis: A 6-Year Retrospective French Study.

Introduction Quantify quality of life (QoL) outcomes in gastroschisis children is little assessed. The primary objective was to describe the long‐term outcome of newborns with gastroschisis treated in three tertiary care hospitals of France in terms of neurodevelopment and QoL. Materials and Methods The study reported was a cross‐sectional, descriptive multicentric retrospective study assessing the outcome of newborns with gastroschisis, born between January 1, 2009, and December 31, 2014, treated at two large and French level III neonatal intensive care units. Long‐term outcome data were assessed by questionnaires sent to the infants’ parents. Questionnaires explored global health, neurological development, and quality of life (overall assessment including socio‐economic and medical), Age & Stages Questionnaire, infants’ quality of life (KIDSCREEN), and quality of parents’ life (General Questionnaire Short Form‐36). Results In this study, 50% of the survivors families answered the assessment form (n = 33). The average follow‐up age was 40 months, ranging from 8 months to 6 years. Cases of simple gastroschisis more often had a normal score for “communication” (p = 0.033), while patients who received morphine for a longer duration had significantly lower scores for the items “communication” and “problem resolving” (p = 0.024 and p = 0.011, respectively). Childrens QoL was significantly lower for patients with gestational age younger than 36 weeks (p = 0.023) and for patients born following “fetal cause delivery” (p = 0.022). Parents had a significantly higher physical composite score if their child underwent primary closure (p = 0.012). Conclusion Our analyses confirm the idea that cases of complex gastroschisis and preterm delivery may lead to poorer outcome. Such hindsight (40 months in mean) allowed for an interesting assessment of development long after the patients initial hospitalization and to confirm these results, a standardized neuropsychological evaluation of patients should be done when at least 6 years old. An accurate assessment of the social environment and its impact on the development and QoL of children will be fundamental to avoid selection bias.

What is the outcome of paediatric gastrocystoplasty when the patients reach adulthood

To document the long‐term outcomes of paediatric augmentation gastrocystoplasty (AGC) in terms of preservation of renal function and maintenance of dryness, and to analyse the rate of complications.

Family History is Underestimated in Children with Isolated Hypospadias: A French Multicenter Report of 88 Families

Purpose: While familial forms of complex disorders/differences of sex development have been widely reported, data regarding isolated hypospadias are sparse and a family history is thought to be less frequent. We aimed to determine the frequency of hypospadias in families of boys with hypospadias, to establish whether these familial forms exhibit a particular phenotype and to evaluate the prevalence of genetic defects of the main candidate genes. Materials and Methods: A total of 395 boys with hypospadias were prospectively screened for a family history with a standardized questionnaire, extensive clinical description, family tree and sequencing of AR, SF1, SRD5A2 and MAMLD1. Results: Family history of hypospadias was more frequent than expected (88 patients, 22.3%). In 17 instances (19.3%) familial hypospadias cases were multiple. Familial hypospadias was related to the paternal side in 59.1% of cases, consisting of the father himself (30.7%) as well as paternal uncles and cousins. Premature birth, assisted reproductive techniques, other congenital abnormalities and growth retardation were not more frequent in familial hypospadias than in sporadic cases. The severity of phenotype was similar in both groups. The results of genetic analysis combined with previous data on androgen receptor sequencing revealed that familial cases more frequently tend to demonstrate genetic defects than sporadic cases (5.68% vs 1.63%, p = 0.048). Conclusions: Familial forms of hypospadias are far more frequent than previously reported. Even minor and isolated forms justify a full clinical investigation of the family history. Detecting these hereditary forms may help to determine the underlying genetic defects, and may improve followup and counseling of these patients.

Heterotopic pancreas without Meckel’s diverticulum in children as unique cause of gastrointestinal bleeding: think about it!

Ectopic pancreas also known as heterotopic pancreas (HP) is a rare congenital anomaly, mainly found as incidental finding during autopsy or abdominal exploration for an other condition. Incidence rate is probably underestimated as patients are mostly asymptomatic; otherwise, it is capable of producing symptoms depending on its location, size, often appearing in the 4th-to-6th decades. Complications such as inflammation, obstruction, bleeding, and malignancy degeneration must be considered. Pediatrics cases are very rare, generally concerning HP within Meckel’s diverticulum, manifesting by gastrointestinal bleeding and intussusception. We report a rare case of jejunum bleeding, due to an isolated HP in a 15-year-old adolescent. Endoscopic and computed tomographic scan were normal, in particular did not found Meckel’s diverticulum. Diagnosis and treatment have been apprehended performing a laparoscopic exploration. It is a singular location for HP, predominantly found in upper gastrointestinal tractus. So far, there have been no case reports of jejunal bleeding from ectopic pancreas without Meckel’s diverticulum in children.

Estenosis ureterales intrínsecas y extrínsecas

Las estenosis del ureter son un problema frecuente en urologia. La diversidad de los tipos de estenosis, unicas o multiples, largas o cortas, intrinsecas o extrinsecas, refleja la variedad de las etiologias de estenosis ureterales (iatrogenicas, postirradiacion, traumaticas, infecciosas, extrinsecas, etc.). El diagnostico de una obstruccion es facil en caso de estenosis completa, pero puede ser mas dificil en caso de estenosis intermitente o parcial. En esta ultima situacion, es preferible limitar el numero de pruebas complementarias. El objetivo del tratamiento es evitar una perdida de nefronas y la atrofia renal. A la diversidad de las etiologias y de los tipos de estenosis ureterales responde la pluralidad de las tecnicas de tratamiento, desde la endoscopia hasta la cirugia abierta o laparoscopica. La eleccion de la tecnica depende de la evaluacion de las capacidades de recuperacion del rinon, de las caracteristicas de la estenosis, de la etiologia de la estenosis, de la experiencia del cirujano y de la operabilidad del paciente. Asi, a pesar de una tasa de exito claramente inferior a la de la cirugia, la endoscopia permitiria tratar las estenosis ureterales cortas con una menor morbilidad. Para la cirugia abierta o laparoscopica hay diversas tecnicas: reimplantacion ureteral, reemplazo ileal, autotrasplante o nefrectomia. Es preciso conocer bien la anatomia del ureter y en particular su vascularizacion. El principio de la anastomosis sin tension es prioritario para aumentar las posibilidades de exito de esta cirugia.

SFCP CO-03 - Involution anténatale de l’anse intestinale primitive chez trois laparoschisis

Objectifs Apport de l’IRM fœtale dans le pronostic digestif des laparoschisis compliques afin de determiner la nature de la malformation, l’etat de l’intestin d’amont et d’aval (grele court potentiel). Sujets Nous rapportons 3 cas de laparoschisis compliques : - Un cas d’involution complete antenatale de l’anse intestinale primitive entre 25–30 SA. Enfant actuellement âge de 7 ans, ayant subi 4 interventions chirurgicales pour grele court, en alimentation parenteral exclusive et en attente de greffe. - Deux cas (27 SA et 29 SA) d’ischemie diffuse des anses herniees a l’echographie et l’IRM conduisant a une interruption medicale de grossesse avec necrose digestive confirmee en anatomopathologie. Resultats Pour le premier cas, la grossesse a ete poursuivie (refus d’IRM par la mere) avec involution digestive complete et fermeture parietale. Pour les 2 cas suivants, ischemie severe de l’anse intestinale primitive a l’IRM. L’ensemble des anses exteriorisees etaient tres peu hydratees en hyposignal T2, hypersignal T1 avec un aspect infiltre de la racine du mesentere. Conclusions L’IRM fœtale confirme le caractere severe et etendu de l’ischemie de l’anse intestinale primitive et permet d’argumenter le mauvais pronostic potentiel.