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Metopisk kraniosynostose
Engelsk titel: Metopic craniosynostosis Läs online Författare: Meling TR ; Due-Tönnessen BJ ; Helseth E Språk: Nor Antal referenser: 39 Dokumenttyp: Fallbeskrivning UI-nummer: 00112135

Tidskrift

Tidsskrift for Den Norske Laegeforening 2000;120(26)3147-50 ISSN 0029-2001 E-ISSN 0807-7096 KIBs bestånd av denna tidskrift Denna tidskrift är expertgranskad (Peer-Reviewed)

Sammanfattning

BACKGROUND : Premature closure of the metopic suture leads to inhibited growth of the frontal bones, producing a keel-shaped forehead (trigonocephaly). Simple metopic synostosis is usually sporadic. Trigonocephalic patients account for 8-16% of the referrals to craniofacial centers, with a marked male predominance. Intracranial pressure (ICP) may be increased, whereas shunt-dependent hydrocephalus is infrequent. Infrequently, patients have intra- or extracerebral anomalies; one third have varying degrees of neuropsychological problems. The treatment is primarily surgical. MATERIAL AND METHODS : We present two patients who during infancy developed increasingly keel-shaped foreheads, retruded orbital rims, increased biparietal diameter and close-set eyes (hypothelorism). Both had raised ICP, but normal psychomotoric development. They were operated using radical fronto-orbital surgical remodelling. RESULTS : Recovery was uneventful. Three months post-operatively, they had pleasing cosmetic results with no symptoms of increased ICP. INTERPRETATION : Where metopic craniosynostosis is suspected, the infant should be examined clinically with palpation of fontanelles and sutures, evaluated with respect to the shape and development of the facial skeleton, as well as by X-ray of the skull sutures. Radical fronto-orbital surgical remodelling gives a stable correction of the craniofacial deformity and generally a satisfactory cosmetic result.