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.

[Cayler syndrome: A case report and review of the literature]. / Syndrome de Cayler : à propos d'un cas et revue de la littérature.

BACKGROUND AND PURPOSE: Facial asymmetries to the tears are rare. We report a pediatric original case that may fall within the framework of a Cayler syndrome. Through its clinical presentation, we will discuss differential diagnoses, associated forms, its etiology, and its management. CASE REPORT: At the maternity unit, in a male infant, after vaginal delivery at term without extraction, was discovered a lack of mobility of the labial commissure on the right side, only when crying. The rest of the examination was unremarkable, except ipsilateral microtia. Genetically, karyotype was 46,XY, 22q11 without microdeletion. The head and neck MRI and echocardiogram were normal. DISCUSSION AND CONCLUSION: Asymmetry with tears has been described in the literature, through association with microdeletion 22q11 syndrome. The originality of this case was the presence of an isolated muscle abnormality. Muscles affected by this syndrome are: Musculus depressor labii inferioris, the Depressor anguli oris, and Mentalis musculus. The three muscles can be affected concomitantly. Isolated involvment of the Depressor anguli oris muscle has also been described. The mechanical dysfunction can be either linked to muscle innervation agenesis or to a defect thereof. There is no specific treatment. The symptoms improve with age by decreasing the frequency of crying. However, it is important to know this pathology in order to seek an optimum balance further in search of associated abnormalities (FISH 22q11, cardiac Doppler ultrasound) but also to educate, to reassure families often worried by the situation.

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.

Interest of umbilical fold incision for pyloromyotomy.

We report the results of a study concerning 80 infants undergoing a pyloromyotomy over a 3-and-a-half-year period. 40 infants had a right upper quadrant transverse rectus incision and 40 infants had an umbilical fold incision. Morbidity and cosmetic aspect of the two techniques were compared. We insist on the umbilical fold incision which presents the same advantages as the classic one and a better cosmetic result.