From Big Medical Encyclopedia

OPERA SYNDROME (E. Apert, 1868 — 1940, fr. pediatrician; synonym: acrocephalosyndactylia, acrosphenosyndactylia, acrocraniodysphalangia) — the symptom complex of hereditary anomalies which is characterized by a combination of deformations of a front part of a skull to a syndactylia and other defects of bone system.

The combination of deformation of a skull to a syndactylia was for the first time described in 1886. R. Troquart. Aper, having generalized literary data and the observations, described a syndrome of the combined malformations in 1906.

Outward of the child with a syndrome Aper. The high head, distance between eyes it is increased, an exophthalmos, lack of a part of fingers on the right brush.

Etiology Ampere-second. it is not established, it is connected with unfavorable conditions of pre-natal fetation. Ampere-second. it is more often observed at children of elderly parents. Some authors recognize the hereditary nature of a disease.

At Ampere-second. peculiar is characteristic type of the child (fig.): the head high with a ledge it is necessary a forehead (see. Tower skull ), the slanting arrangement of palpebral fissures, distance between eyes is increased, an exophthalmos, a nose hook-shaped, the basis its wide, the syndactylia, a polydactylia, is more rare a synostosis between beam and elbow bones. Syndactylia (see) it can be observed in various forms: full merge of all fingers on hands and legs or their partial connection in various options.

Also other defects of bone system are observed: malformations of a backbone, a tutopodvizhnost in an elbow joint, the high sky with its splitting. The first teeth appear late, sometimes in 4 years. There can be a lag in intellectual development, an atrophy of an optic nerve, fusion of an anus. Internals without pathology.

At differential diagnosis it is necessary to consider that from other dysostoses of Ampere-second. differs in existence of a syndactylia.

Treatment Ampere-second. symptomatic. Forecast for life adverse.

See also Dysostosis .

Bibliography: Apert E. De l’acrocephalosyndactylie, Bull. Soc. M£d. Hop. Paris, 3. ser., t. 23, p. 1310, 1906; B öök J. A. a. Hesselvik L. Acrocephalosyndactyly, Acta paediat. (Uppsala), v. 42, p. 359, 1953.

M. F. Deshchekina.