[Necrotizing fasciitis of the eyelids and orbit: a life-threatening ophthalmological emergency]. / Fasciite nécrosante orbito-palpébrale: une urgence ophtalmologique à pronostic vital.

INTRODUCTION: Necrotizing fasciitis is an extremely serious exceptional infectious process affecting subcutaneous soft tissues with skin gangrene and vascular thromboses. Starting from a case repat of necrotizing fasciitis of the eyelids and orbit, which occurred in a child of 4, we analyse the early diagnostic elements and the urgency of treatment of a pathology which can rapidly be life-threatening. OBSERVATION: A child, aged 4 presented a necrotizing fasciitis of the eyelid and orbit with serious toxic shock, after minor skin abrasion. RESULT: Resuscitation and parenteral antibiotic therapy were not sufficient to stop the necrotizing process, which required surgical debridement of gangrenous tissues. DISCUSSION: Necrotizing fasciitis is a rare affection with fatal local and general prognosis. Location on the orbit and eyelids is extremely rare, with only a score of cases recorded in the literature, principally in adults. Our observation shows the possibility of occurrence in a young child and found the typical features of the pathology: minor initial trauma, beta hemolytic streptococcus, extremely fast progression requiring resuscitation, extensive antibiotic therapy and surgical excision of the gangrenous tissues. Eyesight remained intact, but cases of blindness have been described in the literature. Secondary reconstructive surgery on the eyelids may be necessary. CONCLUSION: Urgent diagnosis of this exceptional pathology that may affect the young child and prompt antibiotic therapy and surgical debridement are the only guarantees of survival of infected patients.

Management of seat-belt syndrome in children. Gravity of 2-point seat-belt.

We present our experience with a management of seat-belt syndrome in three children and draw particular attention to the severity of two-point fixation seat-belt injuries after a motor vehicle accident with 5 passengers whose vehicle was struck head-on by an oncoming vehicle. The parents were sitting in front, Adeline had a 2-point lap seat-belt, the 2 other children had 3-point seat-belts. The parents both had humerus fractures. The 4-year-old brother suffered a cervical and abdominal trauma with renal and splenic contusions and intestinal perforations. Adeline suffered multiple injuries, notably to the head, spine and abdominal viscera with erosions at the site of lap-seat-belt contact. The spinal injury was an L2 angular Chance fracture associated with paraplegia on the 7th day. Operative findings included a transverse tear of the left rectus abdominus muscle, an incomplete transection of the stomach and perforation of the ileum. The injuries were ultimately fatal. Given associated abdominal pain, skin erosions at the site of seatbelt contact, spinal fracture, and rectal muscle disruption apparent on emergency laparotomy, early diagnosis is important for better prognosis.

[Value of thoracoscopy in purulent pleuresies in children younger than four years]. / Intérêt de la thoracoscopie dans les pleurésies purulentes de l'enfant de moins de quatre ans.

UNLABELLED: Video-assisted thoracoscopic surgery is widely performed in adults but there are few publications concerning the paediatric population. The objective is to effect optimal adhesiolysis of post-pneumonic loculated empyema with lower morbidity. PATIENTS AND METHODS: Over a 4-year period we used thoracoscopic debridement in five children younger than 4 years of age with loculated thoracic empyema. All patients failed initial treatment, including antibiotics and chest tube drainage. Early sonographic evaluation of the empyema organization guided the most appropriate moment for the intervention. The average duration of tube drainage after thoracoscopy was 4 days (range: 1 to 7 days). RESULTS: All patients made an uneventful postoperative recovery. At a follow-up visit 1 month after discharge, the children were clinically asymptomatic; however, some degree of pleural thickening was still visible on chest X-rays. CONCLUSION: In skilled hands, thoracoscopy is a safe procedure for post-pneumonic empyema in young children, providing a rapid clinical and radiological recovery with a good cosmetic result.

Duplication of the leg--renal agenesis: congenital malformation syndrome.

A case is given of a male born with a duplication of the left leg and ipsilateral kidney agenesis. Although the etiology is unknown, we believe this association represents a congenital malformation syndrome. It is a polytopic developmental field defect. The growth factor IGF-I produced by the intermediary mesoderm or mesonephros (embryonary kidney) could be implicated in the induction limb development and the application of FGF-8 protein to the flank of young chick embryos inducing the development of additional limbs. It is possible that one or more of the growth factors produced by the mesonephros take some cells of the intermediary mesoderm out of their renal way to form a supernumerary limb.

[Sertoli-Leydig tumors in children. 2 case reports]. / Tumeurs de Sertoli-Leydig de l'enfant. A propos de deux observations.

Sertoli-Leydig tumors stem from the mesenchyma and sexual cords of the embryonic gonad. Two cases are reported. One manifested as symptoms of virilization in a 12 year old girl. The other patient developed adnexal torsion at the age of five years. Pelvic ultrasonography visualized the tumor in both cases. Increased production of ovarian androgens suggested the diagnosis in the first case. Histological studies disclosed intermediate differentiation in the first case and tubular differentiation in the second. These tumors usually exhibit low-grade malignancy and unilateral salpingo-oophorectomy ensures recovery in most instances.

Lethal junctional epidermolysis bullosa with normal expression of BM 600 and antro-pyloric atresia: a new variant of junctional epidermolysis bullosa?

A newborn girl is described with a lethal junctional epidermolysis bullosa (Herlitz form) (JEB) associated with a congenital localized absence of skin, and a pyloric atresia (PA). The post-mortem examination of the digestive tract showed a widespread cleavage between the epithelium and the chorion. Immunohistological and electron microscopical examination showed a cleavage occurring through the lamina lucida of the digestive basement membrane, as for the skin blisters. Despite the lethal character of this form of JEB, the BM 600 glycoprotein was normally recognized at the dermo-epidermal junction by the monoclonal antibody GB3. This rare association of lethal JEB-PA-localized absence of skin, with a quite unusual GB3 positive immunophenotype could correspond to a new variant of JEB.

[Pygomelus. Apropos of 2 cases and a review of the literature]. / La pygomélie. A propos de deux cas et d'une revue de la littérature.

The authors report two rare cases of pygomelus monster. The appearance and operative procedures undertaken are described. Pygomelus and caudal duplication are often mixed in literature. This monstruosity is classified in the twin monsters, but minor forms any be discussed with sacro-coccygeal teratomas and reduplication of lower limbs.