M.L. Poli-Merol

Is ilioinguinal/iliohypogastric nerve block always totally safe in children?

Summary We report a case of accidental puncture of the small bowel during an ilioinguinal/iliohypogastric nerve block procedure for hernia repair. The diagnosis was made a few days later during a laparoscopic exploration owing to the progressive onset of clinical and radiological intestinal obstruction. A large, obstructing subserosal haematoma was found without any apparent perforation of the mucosa, and the damaged loop was resected. Such a complication has already been reported once in the literature after use of a long bevel needle for the puncture. This case is the first reported using an atraumatic short bevel needle. We discuss the technical aspects of the procedure and underline the fact that regional anaesthesia in children is never totally risk free.

Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype

BACKGROUND There is a wide difference of opinion between the medical-surgical community and advocacy group regarding Disorders of Sexual Development (DSD) secondary to congenital adrenal hyperplasia (CAH) being ranked in the intersex category. This rupture is even more evident when the issue of genitoplasty is brought up. For physicians it is obvious and unequivocal that a person with CAH and an XX karyotype has a female gender identity, whereas associations tend to rank persons with CAH in the intersex category and advocate holding-off on surgical management. MATERIAL/METHODS A retrospective case study vs. control group, spanning over 40years, included 21 patients who were treated in 3 different centers. Each patient and their parents were contacted independently and interviewed regarding interpersonal relationships, psychological impact of genitoplasty, gender identity and opinion on optimal care management for this disorder. Three couples controls (parent-child) per CAH patients were used and matched according to age, sex assigned at birth and ethnic origin. RESULTS Sex assigned at birth seemed to concord with the gender identity perceived by the patients in 85.7% of cases. In fact, 89.7% of patients and 100% of parents felt that feminizing genitoplasty should be performed within the first year of life. There is however a significant difference compared to controls who felt that surgical management should occur later on in life. No difference was highlighted during childhood regarding parents-child relationships or social integration. However, during adolescence, the parents-child relationship tended to be significantly more painful for the CAH group. Integrating their parenting role was significantly harder for patients in the CAH-DSD group. In the population of CAH-DSD patients who had genitoplasty the level of sexual fulfillment was not lower to the one reported by the control group. CONCLUSION Female sex assignment seems legitimate according to this study and the development of gender identity in these patients matches the sex assigned at birth. Resolving early on the adequacy of the genital anatomy with the sex assigned is promoted by patients as well as their parents. Proper psychomotor development and sexual satisfaction underline the absence of complications related to the surgical technique and the relevance of early surgical management.

Botulinum toxin injections in the management of non-neurogenic overactive bladders in children

INTRODUCTION Non-neurogenic detrusor overactivity in children leads to varying degrees of functional impairments (urinary urgency, pollakiuria, urge incontinence, nocturia). Botulinum toxin has shown its effectiveness in the management of detrusor overactivity in neurological patients. OBJECTIVES To evaluate the relevance of intravesical Botulinum toxin injections for the treatment of non-neurogenic overactive bladders in children. These pediatric patients were resistant to all the usual therapeutics (e.g. bladder/bowel rehabilitation, anticholinergic drugs, management of diet/hygiene habits and constipation, percutaneous posterior tibial nerve stimulation). MATERIALS AND METHODS 8 children (mean age: 12.5years), 5 girls, 3 boys with daytime and/or nighttime incontinence and non-neurogenic detrusor overactivity validated by urodynamic testing. Urodynamic testing was conducted before the injections as well as 6weeks and 1year post injections. We used Dysport® 8 Speywood Units/kg injected via cystoscopy into 25 different sites. RESULTS We noted improvements without any complaints during bladder voiding for all patients, in 6 patients the overactivity disappeared after 1 injection. Compliance was improved early-on in half the cases and at 1year for all cases (from 12% to 61%, p=0.01). Noninhibited contractions decreased constantly in both frequency and intensity. Clinical symptoms improved: mean of 7.75 daytime urinary incontinence episodes (IE) per week before the injection vs. 3 after the procedure (p=0.04). For nighttime IE the improvement was even more noticeable with 7.38 nighttime IE episodes per week before the injection vs. 2.06 after the procedure (p=0,02). CONCLUSION Intradetrusor Botulinum toxin injections are a potential therapeutic option for the management of non-neurogenic detrusor overactivity in children resistant to the usual treatments.

Role of angiogenesis-related genes in cleft lip/palate: Review of the literature

OBJECTIVES Cleft lip and cleft palate (CLP) are the most common congenital craniofacial anomalies. They have a multifactorial etiology and result from an incomplete fusion of the facial buds. Two main mechanisms, acting alone or interacting with each other, were evidenced in this fusion defect responsible for CLP: defective tissue development and/or defective apoptosis in normal or defective tissues. The objective of this work was to study the implication and role of angiogenesis-related genes in the etiology of CL/P. METHODS Our methodological approach included a systematic and thorough analysis of the genes involved in CL/P (syndromic and non-syndromic forms) including previously identified genes but also genes that could potentially be angiogenesis-related (OMIM, Pub Med).We studied the interactions of these different genes and their relationships with potential environmental factors. RESULTS TGFβ, FGA, PDGFc, PDGFRa, FGF, FGFR1, FGFR2 growth factors as well as MMP and TIMP2 proteolytic enzymes are involved in the genesis of CLP (P>L). Furthermore, 18 genes involved in CLP also interact with angiogenesis-related genes. DISCUSSION Even if the main angiogenesis-related genes involved in CLP formation are genes participating in several biological activities and their implication might not be always related to angiogenesis defects, they nevertheless remain an undeniably important research pathway. Furthermore, their interactions with environmental factors make them good candidates in the field of CLP prevention.

Mast Cells and MCPT4 Chymase Promote Renal Impairment after Partial Ureteral Obstruction

Obstructive nephropathy constitutes a major cause of pediatric renal progressive disease. The mechanisms leading to disease progression are still poorly understood. Kidney fibrotic lesions are reproduced using a model of partial unilateral ureteral obstruction (pUUO) in newborn mice. Based on data showing significant mast cell (MC) infiltration in patients, we investigated the role of MC and murine MCPT4, a MC-released chymase, in pUUO using MC- (Wsh/sh), MCPT4-deficient (Mcpt4−/−), and wild-type (WT) mice. Measurement of kidney length and volume by magnetic resonance imaging (MRI) as well as postmortem kidney weight revealed hypotrophy of operated right kidneys (RKs) and compensatory hypertrophy of left kidneys. Differences between kidneys were major for WT, minimal for Wsh/sh, and intermediate for Mcpt4−/− mice. Fibrosis development was focal and increased only in WT-obstructed kidneys. No differences were noticed for local inflammatory responses, but serum CCL2 was significantly higher in WT versus Mcpt4−/− and Wsh/sh mice. Alpha-smooth muscle actin (αSMA) expression, a marker of epithelial–mesenchymal transition (EMT), was high in WT, minimal for Wsh/sh, and intermediate for Mcpt4−/− RK. Supernatants of activated MC induced αSMA in co-culture experiments with proximal tubular epithelial cells. Our results support a role of MC in EMT and parenchyma lesions after pUUO involving, at least partly, MCPT4 chymase. They confirm the importance of morphologic impairment evaluation by MRI in pUUO.

Use of Cutaneous Flap for Continent Cystostomy (Daoud Technique)

PURPOSE We present the results of a new technique using a pedicled cutaneous flap for continent cystostomy. MATERIALS AND METHODS A total of 15 boys and 8 girls (mean +/- SD age 13.4 +/- 6.4 years) underwent continent cystostomy for neurogenic bladder (20), bladder exstrophy (2) and sequelae of hypospadias (1) between 1999 and 2008. In this procedure a rectangular pedicled flap is surgically elevated from a hairless area on the abdomen. The flap is tubularized and passed through the anterior abdominal wall directly into the bladder. A submucosal detrusor incision is made to expose the bladder mucosa, and the distal part of the flap is anastomosed to the bladder mucosa in a circular manner. The tube is positioned along the incised detrusor, which is closed over. Viability of the flap, self-catheterization management and continence status are then evaluated. RESULTS Mean +/- SD followup was 4.5 +/- 3.1 years. There was 1 case of distal necrosis of the flap, which required a secondary surgery using the Mitrofanoff technique. The 22 remaining flaps were initially viable, although 2 patients were eventually lost to followup and 3 subsequently presented with false-passage incidents requiring a few days of calibration using a balloon catheter. Dryness was achieved immediately in 73% of the cases. After adding a complementary bulking agent the dryness rate reached 77%. CONCLUSIONS We present a novel approach to continent cystostomy that is safe and easy to perform. This technique is a less invasive and more efficient alternative to other commonly used approaches.

Visceral Involvement in Stiff Skin Syndrome

Dermatitis artefacta or factitious disease may be unrecognized in children. We present a 12-yearold girl who had an unusual facial lesion on the chin, which was self-inflicted but histologically mimicked cutaneous T-cell lymphoma. Our report emphasizes both the potential diagnostic pitfalls and the importance of clinicopathologic correlation.

Article originalSite donneur de greffe de peau mince : quel pansement utiliser ?Split-thickness skin graft donor site: Which dressing use?

INTRODUCTION The management of split-thickness skin graft donor sites is targeted towards promoting the healing process, reducing pain. This has been an inconclusive topic. The aim of this study was to list and to discuss the French practices in term of split-thickness skin graft (STSG) donor site dressing. MATERIALS AND METHODS Multicentric national study by questionnaire (Google Drive(®)) for the attention of the plastic and/or pediatric surgeons. The type of dressing used on skin and sclap and the rhythm of dressing changes were analyzed. RESULTS The study included 26 surgical centers on 40 contacted. The alginate is mainly used (Algostéril(®)) (17/26). It is left in position until healing (13/17). Five other types of dressings have been reported: paraffin gauze (3/26), lipidocolloides (1/26), Mepitel(®) (1/26), Mepilex(®) (1/26), indifferent use of gauze or alginate dressings (4/26). Twenty-two out of 26 centers make no difference in dressing choice between skin and scalp. Medical practices did not differ between adult or pediatric departments. CONCLUSION Cost-effectiveness has become an important issue in wound management, requiring judicious use. The lack of consensus regarding split-thickness skin graft donor site dressing and our clinical practices force us to reconsider the best therapeutic option. This study coupled with the analysis of the literature highlights the difficulties of the practitioner in choosing the best dressing. The alginate seems to get the preference of our practices by its ease of use, its absence of change (reduces pain by limiting manipulations) and its moderate cost.

P356 - Double localisation de tératomes cervico-faciaux et fente palatine

Les teratomes foetaux cervico-faciaux sont des tumeurs rares, de decouvertes antenatales ou neonatales, souvent benins. Ils peuvent toutefois engager le pronostic vital a la naissance par obstruction des voies aeriennes. Ces tumeurs sont le plus frequemment isolees, mais de rares cas d’anomalies cranio-faciales associees sont decrits. Nous rapportons l’observation clinique d’une petite fille qui presentait a la naissance une fente palatine, ainsi qu’une lesion de 15×10mm d’allure kystique cervicale droite faisant suspecter un lymphangiome kystique. Selon le protocole du service et devant la stabilite de la lesion cervicale, il est decide de realiser la fermeture de la fente, sans geste sur la lesion cervicale. A 18 mois, devant la persistance d’une fistule palatine residuelle, une reprise chirurgicale est decidee. Lors de ce geste l’exerese de la lesion cervicale droite evolutive est realisee. De plus, au decours de la chirurgie palatine, est decouvert une lesion pediculisee du cavum. L’examen anatomopathologique retrouvait un teratome mature kystique (tissu bronchique, gastrique et musculaire) en cervical et teratome mature, solide et polypoide au niveau du cavum. Nous discuterons sur le plan clinique et therapeutique, a l’aide de la litterature, cette association rare.

Monitoring Cerebral and Renal Oxygenation Status during Neonatal Digestive Surgeries Using Near Infrared Spectroscopy

Background Depending on the initial pathology, hypovolemia, intra-abdominal hypertension, and sepsis are often encountered in neonatal digestive surgery. Accurate newborn monitoring during and after surgery is essential to adapt resuscitation protocols. Near infrared spectroscopy (NIRS) is non-invasive and can detect hypoperfusion which indicates a low circulatory blood flow, regardless of the cause. Objective Evaluating changes in cerebral and renal regional oxygen saturation during neonatal digestive surgeries, conducted according to normal practices, with commonly used monitoring parameters. Analyzing retrospectively the inter-relationships between NIRS values and mean arterial pressure (MAP) values as well as pre-ductal SpO2. Methods Prospective, descriptive, monocentric study. All neonates referred for surgery were included. NIRS allows the measurement of cerebral and renal oxygenation fluctuations, as well as calculating difference in intraoperative and postoperative values. Results Nineteen patients were included. Cerebral regional oxygen saturation (C rSO2) values were stable while renal regional oxygen saturation (R rSO2) values tended to decrease with time during surgery. Indeed, 72% of rSO2 decline episodes occurred after the first 30 min of surgery, without any significant statistical differences for the next 90 min of surgery. After surgery, the lowest average C and R rSO2 values were evidenced during the first 6 h, with 60% of C rSO2 and R rSO2 anomalies occurring in that time frame. There was no significant statistical difference observed in the following 18 h. There was a significant correlation between R rSO2 and SpO2 values (p < 0.01), but not with C rSO2 values. There was no correlation with the MAP either for the C rSO2 values or R rSO2 ones. Conclusion NIRS is a promising non-invasive bedside tool to monitor cerebral and tissue perfusion, analyzing tissue microcirculation. NIRS has its interest to guide neonatal digestive surgeries (bowel manipulation, viscera reduction) and may represent an early warning for identifying patients requiring resuscitation during or after these surgeries.