[Goldenhar syndrome. Oculoauriculovertebral dysplasia. 2 new pediatric cases]. / Syndrome de Goldenhar. Dysplasie oculo-auriculo-vertébrale. Deux nouvelles observations pédiatriques.

The authors report two new cases of Goldenhar's syndrome, as they are described in Gorlin's scheme of oculo-auriculo-vertebral dysplasia. Therapeutical problems (plastic and orthopedic surgery, ocular prothesis and hearing aid) due to Goldenhar's syndrome are very acutely pointed out in these cases. Generalized convulsions, not yet described in the medical literature, are found in these two cases. These congenital malformations are caused by branchial arches organogenesis anomalies. The authors consider that accurate and early dermatologic diagnosis is important for the correct management of children with oculo-auriculo-dysplasia.

[MacCune-Albright syndrome. Apropos of a case]. / Syndrome de MacCune-Albright. A propos d'une observation.

The MacCune-Albright's syndrome as described by these authors in 1936 and 1937 is extremely rare if one considers the complete form consisting in the triad: café-au-lait spots, fibro-osseous dysplasia, endocrine disturbances. We report an additional case in a 5 year old girl with an precocious puberty. Within a 10 year follow up occurred a polyostotic fibrous dysplasia with pathologic bone fractures in cystic areas, and a right exophthalmos induced by a cyst of the large wing of the sphenoid bone. All these lesions led to orthopaedic and neural surgical procedures. The early puberty of central origin (as proved by the LH-RH test) was treated during 7 years by medroxyprogesterone acetate with an excellent result. No peripheral endocrine syndrome was evidenced. This case stresses the multiple problems arising in the disease and the necessity of a long lasting careful follow up.

[Pathophysiology of gastroesophageal reflux and esophagitis (author's transl)]. / Physiopathologie du reflux gastro-oesophagien et de l'oesophagite. Applications diagnostiques et thérapeutiques.

Two new data explain pathophysiology of gastroesophageal reflux and esophagitis: first, the low esophagus pressure is altered in the reflux, second, the biliary salts act a part in the determination of esophagitis. New methods of explorations are proposed: esophageal manometric study, measure of intraluminal pH and soon perhaps radio isotope. That better understanding, those new explorations should allow to choose better than yesterday, therapeutic schemes. But many questions remain without answer.

[Painful Tolosa-Hunt ophthalmoplegia. Apropos of a case in a child]. / Ophtalmoplégie douloureuse de Tolosa-Hunt. A propos d'une observation chez l'enfant.

Painful ophthalmoplegia occurred in a twelve-year-old boy, with repeated attacks over five years. This fits the description of Tolosa-Hunt syndrome which has only infrequently been described in children. Tolosa-Hunt syndrome combines severe lasting orbital pain and paralysis of one or more oculomotor nerves (extrinsic or intrinsic III, IV, VI). Pain is dramatically alleviated by high doses of corticosteroids, while the ophthalmoplegia improves more slowly. For some authors, this effect of corticosteroids is diagnostic. Repeated attacks occur over several years and resolve spontaneously with or without sequellae. Diagnosis can only be ascertained after careful ruling out of a general or locoregional etiology through clinical and paraclinical investigations. Differential diagnosis with ophthalmoplegic migraine may be difficult.