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. DESIGN, SETTING, AND PARTICIPANTS: A clinical randomized trial that ran between August 2012 and July 2017 was conducted. The trial was multicentric, including 13 referral centers for pediatric urology. Male boys, aged 1-28 d, diagnosed with posterior urethral valves, confirmed by voiding cystogram, were included. The exclusion criteria included presence of a genital malformation contraindicating performing a circumcision. INTERVENTION: Participants were randomized to neonatal circumcision + antibiotic prophylaxis (CATB) or antibiotic prophylaxis alone (ATB), and followed for 2 yr. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The primary outcome was a risk of presenting fUTIs in each group. An fUTI was defined as fever (>38.5 °C) with evidence of pyuria and culture-proven infection on urinalysis, obtained by urethral catheterization or suprapubic aspiration. A bivariate analysis of the primary outcome was performed using the Kaplan-Meier method. RESULTS AND LIMITATIONS: In total, 91 patients were included: 49 in group CATB and 42 in group ATB. The probability of presenting an fUTI was 20% in group ATB versus 3% in group CATB. The hazard ratio of presenting an fUTI within 2 yr in the ATB group compared with that in the CATB group was 10.3 (95% confidence interval: 1.3-82.5). Sixty-four children (70.3%) had a complete follow-up at 2 yr of age. CONCLUSIONS: Circumcision significantly decreases the risk of presenting an fUTI in boys with PUVs. PATIENT SUMMARY: In this report, we compared, in a multicentric trial, the number of febrile urinary tract infections (UTIs) in boys with posterior urethral valves who had either antibiotic prophylaxis alone or antibiotic prophylaxis and circumcision. We found that those who had a circumcision had a significantly lower risk of febrile UTIs.

Bilateral methachronous testicular germ cell tumor and testicular microlithiasis in a child: Genetic analysis and insights. A case report.

OBJECTIVES: To report our experience with a case of a child with bilateral testicular micro-lithiasis (TML) who developed bilateral metachronous testicular germ cell tumor (TGCT) and determine the most appropriate follow-up and care management in children with testicular micro calcifications in regards to the theoretical risk of testicular cancer. CASE REPORT: A 12 year-old boy was diagnosed with TGCT and TML. Ten years after complete remission, he presented with a recurrence on the contralateral testis. Genetic screening was performed on both resected and the patient's karyotype was analyzed. RESULTS: Blood karyotype was normal. Aberrations were found in the tumor karyotype. CGH array showed alterations in chromosome arm 12p. DISCUSSION: TML is frequently associated with testicular malignancy in adults: in 16.9% of cases the normal contralateral testicle develops TML in TGCT. Recent works of literature find no relationship between TML and cancer in general, but in patients with additional risks, the relationship becomes stronger. Some authors suggest that environmental components and genetics are determinant factors. This is highly suspected in our reported case. It would seem that TML is not a precancerous lesion per se, but rather a marker of an at-risk situation. Long term evolution is uncertain and regular self-palpation that starts before puberty is the only way to ensure proper screening and monitoring. CONCLUSION: TML have been suspected to be a sign of testicular dysgenesis syndrome, which yields a risk of developing TGCT in case of noxious associations. In patients with a history of TGCT contralateral TML is alarming and aggressive surgical management should be discussed. Therapeutic education of these patients on self-palpation is the best way to ensure proper follow-up.

New in vivo model to analyse the expression of angiogenic genes in the borders of a cleft lip.

Defects in the fusion of facial buds can result from an anomaly in tissue development or apoptosis, or both. Our working hypothesis was that anomalies in the development of tissues could be caused by a genetic angiogenic defect. Our main objective was to design a reproducible experimental model to study the expression of angiogenic genes in the borders of cleft lips with or without cleft palate. We therefore prospectively studied seven non-syndromic patients, three with a cleft lip (2 right, 1 left), and four with a cleft lip and palate (1 bilateral, 2 right, 1 left), with no CGH (comparative genomic hybridisation) array, who had primary operations to repair their clefts. We also used four controls (cultured fibroblasts from healthy skin samples). The mean (range) age at operation was 44 (13-77) days. We studied the lateral and medial borders histologically and did qPCR (quantitative real-time polymerase chain reaction) analysis for gene expression with 22 genes of interest (and two housekeeping genes) involved in cleft lip and angiogenesis. The qPCR analysis found significant (p<0.05) overexpression of eight genes in the medial border and seven in the lateral border, and underexpression of nine genes in the medial, and ten in the lateral border. The difference in expression between the two borders was not significant. This preliminary study has enabled us to develop a new method to analyse the expression of angiogenic genes in the borders of cleft lips.

[Therapeutic mega-ureter primitive before one year of life, retrospective study of 20years]. / Stratégie thérapeutique des méga-uretères primitifs avant 1 an de vie, étude rétrospective de 20 ans.

INTRODUCTION: What is the proper way to manage complicated primary mega-ureter in infants under the age of one. This has already been discussed in the literature but the controversy remains. OBJECTIVE: Evaluate the long-term results of the management of mega-ureter based support under the age of one. MATERIAL AND METHODS: Single-center retrospective study from 1990 to 2010. All children under one year found were evaluated including clinical examination, ultrasound, scintigraphy and cystography. They were divided into two groups: group 1: children operated on before the age of one year, group 2 non-operated or operated children after the age of one year. We analyzed the long-term evolution of these children on the following criteria: reflux, pyelonephritis, changes in dilation, renal function, need for surgical revision or secondary surgery, and impact on bladder function. RESULTS: In total, 54 patients were included in group 1 and 56 patients in group 2. In a median follow-up of 12 years. A total of 101 boys and 9 girls (sex-ratio 11.22). There were 57 left MUP (52%), 22 right (20%) and 31 bilateral (28%). A total of 71% of antenatal diagnosis. No difference on the emergence of complications: 25 (group 1) versus 31 (group 2) OR=0.69; 95% (0.307; 1.574); P=0.44. No difference between secondary surgery and revision surgery: group 1=12, group 2=22, OR=0.45; 95% CI (0.17, 1.09); P=0.06. No difference for daytime incontinence: OR=1.04; 95% CI (0.14; 7.64); P=0.67. Seventy-six children (69%) were finally made, 12 children operated twice (10.9%) and 34 children (31%) never made. CONCLUSION: The main challenge of the MUP of management is the preservation of renal function. Sixty-nine percent of our children received surgery due to impaired renal function lower than 30% of urethral dilatation greater than 10mm associated with reflux or recurrent pyelonephritis. Clinical monitoring, regular ultrasound and isotopic testing are necessary and should be extended to adulthood. LEVEL OF EVIDENCE: 5.

[Secondary megaureter: A rare complication of Deflux® endoscopic management of vesicoureteral reflux in children]. / Le méga-uretère secondaire : une complication rare du traitement endoscopique du reflux vésico-urétéral par Deflux® chez l'enfant.

Endoscopic management is the gold standard for symptomatic low-grade vesicoureteral reflux (VUR) in children. Deflux® (hyaluronic acid/dextranomer) injection is highly effective and has very few complications. We report on two cases of secondary megaureter after Deflux® injections. In the first case, a boy presented with Grade 4 VUR. He received a bilateral Deflux® injection with a total of three syringes. The postoperative ultrasound was normal. However, a check-up ultrasound 3 years later showed a significant ureteropyelocalyceal dilatation, with stasis and decreased renal function on scintigraphy, the reason why antireflux surgery (Cohen procedure) was performed. In the second case, a girl diagnosed with bilateral VUR at birth received bilateral injections with one syringe on each side at the age of 12 months. One month later, the ultrasound showed a dilation of the distal ureters (diameter of the right ureter, up to 10mm; left ureter, up to 6.7mm). The child underwent surgery 8 months later (Cohen procedure) because of iterative pyelonephritis and persistent ureter dilatation. Only one previous case has been described in the literature. In our experience, this complication has occurred only twice in 452 injections (4‰). In conclusion, endoscopic treatment with hyaluronic acid/dextranomer injection is a minimally invasive procedure that improves the situation in cases of VUR. It has few complications. Other than failure, there is a low risk of secondary expansion requiring, in our opinion, ultrasound verification over the long term.

Papillary endothelial hyperplasia (Masson's tumor) in children.

The intravascular papillary endothelial hyperplasia (IPEH/Masson's tumor) is a rare benign tumor of the skin and subcutaneous vessels. We report, in four pediatric cases, clinical presentation, care (diagnostic and surgical) of Masson's tumor in children. Two boys (two years) and two girls (four and six years) showed a pain subcutaneous tumor (one to five centimeters). They were in the transverse abdominal muscle, between two metatarsals, at the front of thigh and in the axilla. Imaging performed (MRI, Doppler ultrasound) evoked either a hematoma, a lymphangioma or hemangioma. The indication for removal was selected from pain and/or parental concern. The diagnosis was histologically. A lesion persisted in residual form (incomplete initial resection), and is currently not scalable for eleven years. DISCUSSION: This tumor is characterized by excessive proliferation and papillary endothelial cells in the vessels, following a thrombotic event. It is found mainly in adults (no specific age), and preferentially localizes in the face and limbs. The clinical differential diagnosis of this tumor is angiosarcoma. The imagery has not allowed in our series to diagnose but still essential to eliminate differential diagnoses. Only surgical excision with histological examination can differentiate. Our study emphasizes the possibility of pediatric cases with two cases of unusual locations (abdominal and axilla). Clinical presentations we met, now lead us to direct our histologist looking for a Masson tumor in any child with a subcutaneous tumor and/or intramuscular pain, sudden onset, and vascular appearance (after excluding an arteriovenous malformation).

[Umbilicus in children]. / L'ombilic chez l'enfant.

The umbilicus is our first scar. It is the last remain of our life in utero. Besides the umbilical hernia, a common pathology during the first three years of life that rarely requires surgery, there are some rare congenital abnormalities such as gastroschisis and omphalocele, which occur in about 1-5/10,000 births. Gastroschisis is a birth defect of the anterior abdominal wall, through which the fetal intestines freely protrude and are not covered by any membranes. During the 13th week prenatal ultrasound, the umbilical cord can be seen to be properly attached while the intestines float freely in the amniotic fluid. This defect is most common in young women who smoke and/or use cocaine and is not typically associated with genetic disorders. Omphalocele is an average coelosomy, in which a visceral hernia protrudes into the base of the umbilical cord. Omphalocele is typically diagnosed during the prenatal phase, and occurs most commonly in older mothers. It is frequently associated with genetic and morphologic abnormalities, therefore a karyotype should automatically be performed. For these two pathologies, the surgical problem lies in managing, during the reintegration, the conflict container/content responsible to lower vena cava syndrome and disorders ventilatory. Deciding on the technique will depend on the clinical form, and on the tolerance to reinsertion. The success of the surgery is directly linked to the postoperative emergence care for the pre-, per- and postnatal phases. The umbilical cord is preserved in the case of a gastroschisis. A primary or secondary umbilicoplasty will be performed for an omphalocele closure. The umbilicoplasty aims to create an umbilicus in a good position by giving it a shape, ideally oval, but also and especially an umbilication. The primary or secondary umbilicoplasty remains a challenge in a growing abdomen (change in position, deformation, loss of intussusception with growth). Many techniques are described: cutaneous flaps randomly placed, excision and skin plasty, resection and controlled wound healing. The choice of technique is a matter of practice but must be done in a rational way, depending on the scar condition when secondary reconstruction, and with minimal scarring, for primary reconstruction. To avoid morphological changes associated with growth, secondary umbilicoplasty should be proposed after the age of five.

Hypospadias.

Hypospadias is a hypoplasia of the tissues forming the ventral side of the penis responsible of an ectopic meatus of the urethra. This congenital anomaly results in a fusion defect of the two epithelial surfaces of the urethral groove between the 11th and the 18th weeks of development. The earlier this process arrests, the more the form is proximal and severe. This is the second genital malformation in boys with 1 case per 250 male births. Its origin is often multifactorial (genetic, endocrine, placental and environmental). Three anatomical forms exist: proximal, middle and distal (the most common). Additional exams (endocrine, genetic and morphological) are realized early, before surgery, in case of severe hypospadias, familial, associated with cryptorchidism, bifid scrotum, micropenis and/or skeletal, kidney, and/or heart abnormalities. It clarifies pubertal prognosis. The surgical management is made between 6months and 12months: it limits the functional and aesthetic impact of this malformation. Many surgical techniques are described. They all have in common the three operating time: penile straightening, urethroplasty, reconstruction of the ventral side of penis. They are based on direct sutures, local flaps pedicled, and grafts (skin or mucosa). The rate of postoperative complications is between 6 and 30 %. The two main complications are fistulae and stenoses. The psychological follow-up of these children is necessary to adulthood. Surgery of hypospadias remains a delicate surgery and must be performed by experienced surgeons.

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.

[The role of balneology in plastic surgery]. / Place du thermalisme en chirurgie plastique.

UNLABELLED: Balneology can be part of the plastic surgery care sector. The objectives of this study were firstly to the state of knowledge about the hydrotherapy and specify the place reserved for hydrotherapy by surgeons as an adjunct in plastic and reconstructive surgery (adult and child). MATERIALS AND METHODS: Multicentric national study by poll (Google Drive®) focused at plastic and/or pediatric surgeons. The following information was analyzed: frequency, timing of prescription, indications, the surgeon's feelings towards hydrotherapy and the differences between adult's and children's prescriptions. RESULTS: Fifty-four teams were contacted: 22 responses were received (15 "adult" plastic surgeons, 9 "pediatric" plastic surgeons, 6 pediatric surgeons, with 12 out of 22 working with burnt patients). Eighteen out of 22 prescribed hydrotherapy. Twenty out of 22 thought that hydrotherapy had a role as adjuvant therapy in plastic surgery. The indications were: burns (11/20), skin-graft hypertrophy (10/20), inflammatory and pruritic scar and cutaneous trophic disorders (9/20), psychological (3/20), retractions (2/20), weight loss and smoking (1/20). The timing of the prescription was: < 3 months (2/20), < 6 months (7/20), > 6 months and < 1 year (15/20), > 1 year (8/20) after surgery/trauma. Twenty out of 22 found a beneficial effect: physical (19/20): reduction of inflammatory signs, pruritus and pain, scar maturation, skin thinning improvement; psychological (14/20): positive for patient/family. Five out of 17 made the difference between child/adult, 10/17 made no difference but only treated adults or children. CONCLUSION: The respondents in the study are probably more sensitive to the effects of hydrotherapy that non-respondents. It is difficult to assess the real impact of hydrotherapy in plastic surgery because distinguishing spontaneous favorable evolution of a scar from one only due to the hydrotherapy or multidisciplinary management is difficult. However, hydrotherapy seems to have its role among multidisciplinary management.

[Primary surgery for cleft palate and short hospital stay (48 h): Are they compatible?]. / Chirurgie primaire des fentes vélaires et/ou vélo-palatines et hospitalisation de courte durée (48h) : sont-elles compatibles ?

UNLABELLED: The aim of this study was to evaluate our practices by studying the duration of hospitalization and the parental real-life experience after a primary surgery of a cleft palate. MATERIALS AND METHODS: Monocentric retrospective study by analysis of the patients files and phone interview of the parents whose children were operated for a primary surgery of a cleft palate isolated, or associated with a labial cleft, or included in a syndromic form. RESULTS: Forty-nine patients (25 B-24 G) were performed by 44 Wardill and five Furlow procedures (average age: 11 months ½) between 2010 and 2012. The average duration of the post-operative stay was 1.5 days. Thirty-three parents were contacted (67%). The return was "very well" or "well done" in 82% of the cases. The pain at home was estimated by the parents as "worthless" or "little intense" in 73% of the cases. For 16% of the parents, the child seemed "uncomfortable". The prescription of analgesic was followed only in 70% of the cases. The duration of hospitalization was considered by the families as "good one" in 70% of the cases, "too long" for 12% and "too short" for 18% in particular because of difficulty in eating or parental anxiety. CONCLUSION: Even if palatine surgery is considered to be painful, anaesthetic techniques and current analgesic protocols allow to envisage very simple and fast consequences, authorizing an early return of the children at home.

[Recurrence of pregnancies with gastroschisis: a case report]. / Récidive de grossesse avec fœtus atteint de laparoschisis : à propos d'un cas.

Gastroschisis, a parietal anomaly belonging to median celosomia, is characterized by right lateral-umbilical evisceration. Its frequency, estimated at 1 birth in 2700, shows an excellent final prognosis conditioned by the digestive ischemic damage found at birth whose therapeutic management is often limited. No genetic cause is currently incriminated in the genesis of gastroschisis, except syndromic group associations. In this original observation, we report the case of a couple with a normal history whose obstetric management of the first pregnancy showed a fetus with gastroschisis at 12 WG, with primary closure at birth. The same couple began a second pregnancy in post-partum with on the first-quarter echography showing gastroschisis. A boy was delivered vaginally with extraction, who died of a multivisceral failure at 48h of life. Although no genetic factor is currently identified in gastroschisis onset and although only environmental factors such as illicit substance consumption, young maternal age, and socio-economic precariousness explain the genesis of this parietal anomaly, our observation is original because of the recurrence of this anomaly in the same woman during two successive pregnancies. The early characteristic of the second pregnancy, starting as soon as menstruation returned, can explain the organogenesis of this second gestation in a similar environmental context.

Frequency and socio-psychological impact of taunting in school-age patients with cleft lip-palate surgical repair.

OBJECTIVES: Cleft lip-palate (CLP) is a "social" pathology because of its impact on the child's facial appearance and speech. School is the first place where children are confronted to others and when they start socializing. Taunting and bullying are common and their psychological impact remains hard to assess. The aim of this study was to evaluate the importance of taunting in school and its impact in CLP patients who had surgical repair. METHODS: We conducted a multicenter prospective study where we consecutively included patients ≥ 12 years who had CLP repair. During a multidisciplinary consultation they were asked to complete a questionnaire (3 parts: surgical outcomes, taunting and its impact, socio-economic status) previously approved by our psychologists. RESULTS: 55 patients were included (37 B, 18 G) (mean age 15.5 years): 11 CL, 13 CP and 31 CLP. 69% of patients reported having suffered from taunting and peer victimization in school. In 84% of the cases, taunting was linked to the CLP defect itself. The teasing started in primary school to reach a peak of aggressiveness in middle school. 42% of patients reported that bullying occurred at least once a day (16/38). Regarding the psychological impact of taunting, 50% of patients reported sadness, 31% depression and 26.3% were marked for life. At one time or another 29% of patients did not want to attend school because of the teasing. The grade retention rate amounted to 37.7% (20/53), and 2 patients were in special education classes. As a matter of fact, 50% of these children repeated their 1st or 2nd year of primary school. Furthermore, 47% of patients wanted to change something to their face, but 63% of them never spoke to their surgeon about additional surgeries even though they were teased in school. CONCLUSIONS: Taunting is common in children with CLP. This study highlights the high frequency and impact of taunting on the daily lives and self-perception of patients with CLP or CLP repair. It is important for healthcare professionals to be aware of this issue in the context of a multidisciplinary approach.

[Split-thickness skin graft donor site: which dressing use?]. / Site donneur de greffe de peau mince : quel pansement utiliser ?

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.

[Cleft lip and palate: Health-related quality of life (French VSP-A scale) for patients and their family. About 51 families]. / Fentes labio-palatines : qualité de vie (VSP-A) patients et parents. À propos de 51 familles.

PURPOSE, OBJECTIVE: The aim of our study was to investigate the quality of life of patients undergoing cleft lip or cleft lip and palate as well as the perception of quality of life of patients perceived by their parents slot. PATIENTS AND METHODS: Fifty-one adolescents and young adults (29 boys and 22 girls) who underwent cleft lip (FL 22) or lip and palate (CLP 29) and their parents have received psychological and surgical joint consultation. The minimum age for inclusion was 10 years (mean age 15.5 years). A quality of life questionnaire (VSP-A) "children" was given between 10 and 11 years and a questionnaire "adolescents" beyond. Parents have them answered a questionnaire of perceived quality of life of their children. The results were analyzed and compared to a control population (Statistics Student test). Perceived parents lived in relation to their children was obtained through the study of linear regression curves. RESULTS: The response rate to the questionnaires was 66.7% for parents, 85.7% for children and 63.6% for teenagers. The quality of life of the patients was assessed by the patients to 65.1/100 on average. The index of overall quality of life was superimposed on the control population (p=0.66). Perceived quality of life of patients by their parents was fairly close to the quality of life described by patients (66.5). On the areas of family, education, recreation, quality index was proportionately less than for other areas. For each of these areas, parents overestimated the quality of life of their child. Compared to the control group the fields of education, leisure, vitality quality index were significantly lower in the/FL population P.

[Recurrent distal ileal obstruction by a trichobezoar in a 5-year-old child]. / Occlusion iléale récidivante par trichobézoar chez une enfant de 5ans.

The diagnosis of trichobezoar can be difficult, due to its low prevalence in the pediatric population, limited knowledge of behavioral disorders in children and their prevention, and the difficulty of the clinical diagnosis. We report a case of intestinal occlusion in a 5-year-old child, whose diagnosis was delayed and the trichobezoar, revealed intraoperatively, confined 15cm from the ileocecal valve. Diverging management strategies are proposed in the literature concerning the indication of CT for diagnostic and prognostic purposes, and fibroscopy, still considered as the diagnostic reference. Surgical exploration can lead to the final diagnosis. Bowel obstruction requires emergency surgery to avoid intestinal complications and must be followed by psychological therapy to limit recurrence.

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: TGFß, 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.

VAC® therapy a therapeutic alternative in giant omphalocele treatment: a multicenter study.

Giant omphalocele is associated to morbidity and mortality because of the strain the reintegrated herniated mass places on the hemodynamic equilibrium and breathing functions of affected infants. Currently, care management consists in a reintegration in one time or progressive reintegration. We report here a multicenter retrospective study about alternative management by VAC® therapy for giant omphaloceles. The study included three patients (1 girl, 2 boys) presenting with giant omphaloceles, born at full term in three different University Hospitals (prenatal diagnosis, normal karyotype). VAC® therapy was implemented at different times according to the cases (at Day 11, Month 1 and Month 5 after birth). The initial pressure applied was -10 mmHg progressively increased to -50 mmHg. A middle size VAC GranuFoam Silver® Dressing was used in all cases. Wound healing occurred at Month 4 for the first case, Month 6 and Month 8 for the other two. VAC® therapy is a good alternative for the care management of giant omphaloceles with more advantages especially when using prosthetic material. We also aimed at refining the most adapted indications in these specific situations, and finally we envisioned a harmonization of care for these children.

Cleft palate associated with cervico-facial teratoma: report of two cases and review of the literature.

We report two original cases of association of cleft palate and lateral cervico-facial teratoma. We discuss the embryological explanation. The first child presented a cleft palate associated with two cervico-facial localisations of teratoma. The other had Pierre Robin sequence associated with lateropharyngeal teratoma with an extra sub maxillary localisation. Most reported cases were of midline teratomas, leading different authors to advance a mechanical origin to the cleft. Our cases are different: we could hardly find reported cases of associate lateral tumours and cleft palate, which would suggest two different embryologic mechanisms, or at least a combination of more complicated phenomenons.