Necrotizing soft tissue infection of the scalp after fronto-facial advancement by internal distraction in a 7-year old girl with Gorlin-Chaudhry-Moss syndrome--a case report.

摘要

In 1960, Gorlin, Chaudhry and Moss described a syndrome consisting of craniofacial dysostosis in association with hypertrichosis, cardiac, genital, dental and ocular anomalies. Diagnosis is based on typical clinical findings and cannot be performed by molecular genetic analysis until now. There is little in the clinical literature concerning this rare craniofacial syndrome. For functional and psychosocial reasons, surgical correction of the complex craniofacial malformation in a 7-year old Hungarian girl with Gorlin-Chaudhry-Moss syndrome was performed by fronto-facial advancement using internal distraction devices. Postoperatively necrotizing soft tissue infection of the scalp developed leading to termination of the distraction process ahead of schedule and requiring aggressive surgical management. Typical physiological and clinical characteristics were observed both during the initial craniofacial correction as well as during the management of the infectious complication suggesting that the linking of different conditions (surgical trauma plus the selection of toxic microorganisms) has caused tissue destruction rather than the syndromal disorder or the surgical technique of distraction osteogenesis. Although skeletal improvement was achieved residual damage from the infectious complication must be considered as severe.