Surgical management of lower lip pits in Van der Woude syndrome.

Van der Woude syndrome (VDWS) is characterized by the presence of lower lip pits which may be of concern to patients due to aesthetic considerations. By presenting three clinical cases, we provide an overview of the surgical techniques currently available to treat labial pits. Fusiform excision with dissection of the entire pit is still the most commonly used procedure and it generally yields good functional and aesthetic outcomes. The split-lip advancement technique and the inverted T-lip reduction nonetheless represent good surgical alternatives. Proper management of the lower pits that occur with VDWS requires thorough knowledge of the available surgical procedures.

[Incontinentia pigmenti]. / Incontinentia pigmenti.

Incontinentia pigmenti (IP) is a hereditary syndrome characterized by specific skin lesions occurring mostly during the neonatal period (96% of the cases before 6 weeks of age). These skin lesions have four steps of evolution: inflammatory or erythemato-bullous stage (very often associated with peripheral blood hyper-eosinophilia), proliferative or verruco-lichenoid stage, pigmentary or terminal stage characterized by "fountain" or "firework" features (with a picture of pigmentary incontinence at histological examination), sometimes there is a fourth stage referred to as "involutive". Ocular and neurological involvement is the main determinant in the prognosis. Eye lesions include corneal flecks, cataracts, uveitis or optical atrophy with retrolental fribroplasia. The neurological involvement includes pyramidal syndrome, cerebral ataxia, microcephalia, and mental retardation. The disease has mainly an X-linked dominant transmission and is usually lethal for males. Rare cases are observed in boys, some being associated with Klinefelter syndrome. Research is ongoing to identify the IP gene on the X chromosome. In the family form of IP, the gene has been located on chromosome Xq28, which allows prenatal diagnosis using trophoblast biopsy.

[Burns and pregnancy. A case of severe burn occurring at the beginning of pregnancy. Maternal and fetal survival]. / Brûlures et grossesse. Une observation de brûlure grave survenue en début de grossesse. Survies maternelle et foetale.

The authors report a case of a severe thermal burn (70% of the surface of the body) in a pregnant woman who had 15 weeks amenorrhoea. The survival of the mother and the continuation of the pregnancy to term allowed a normal live baby to be born. As far as the authors know this is the fourth case history of a severe (more than 60% of the body) burn published occurring in the first trimester of pregnancy and carried through successfully to term. A study of the literature has shown that the physiopathological results of severe burns do explain the usually poor prognosis for mother and fetus. Large quantities of prostaglandins are released into the circulation of the mother because of the thermal insult to tissues and because of the infection that usually accompanies such burns. These prostaglandins can stimulate the myometrium, which gives rise to fatal obstetric complications. The urgent acute treatment for a severe burn in a pregnant woman, which should preferably be carried out in a special centre, consists in water and electrolyte resuscitation, assisted respiration and prevention of bacterial contamination. These measures are just as important for the fetus. At the same time obstetric care should be started. Good collaboration between the obstetricians and those who carry out the resuscitation all bring about the measures that are needed to allow the mother and fetus to survive.