"Spectaclesplasty" periorbital rotation advancement in Crouzon syndrome.
Facial deformity in Crouzon syndrome is characterized by exophthalmos, exorbitism, mild hypertelorism, and maxillary hypoplasia with a Class III occlusion. The Göteborg craniofacial unit corrects this deformity in selected patients by the 2-stage procedure of "spectaclesplasty" followed by a Le Fort I maxillary osteotomy at skeletal maturity. "Spectacles" is a reference to the bilateral circumferential periorbital bony skeleton, and a spectaclesplasty is a differential rotation and advancement of this complex. Spectaclesplasty has been integrated as part of our protocol in managing Crouzon syndrome from birth to maturity. All patients who have undergone spectaclesplasty were retrospectively reviewed. A composite scoring system analyzing periorbital anatomy and aesthetics was used to compare the preoperative and most recent postoperative photographs. Complications and the need for ancillary procedures were recorded. Twenty-one patients have undergone spectaclesplasty since this technique was introduced in 1984. Mean follow-up time is 5 years 10 months. Compared with their preoperative aesthetics, the improvement was rated as excellent for 8 patients (38%), very good for 7 patients (33%), good for 5 patients (24%), and minimal for 1 patient (5%). No patient was rated as having no improvement. Mean perioperative blood loss was 111% of estimated red cell mass. Mean operative time was 6.4 hours. The mean duration of stay in the intensive care unit was 28 hours, and the mean hospital stay was 11 days. There were few complications and no incidences of mortality. Spectaclesplasty yields high-quality aesthetic results in most cases. It is our impression that spectaclesplasty en bloc rotation advancement of the periorbital bony skeleton can be safely performed before skeletal maturity of the lower face. Correction of the bony periorbital anatomy in early adolescence is important in alleviating psychosocial distress in this age group. In our hands, spectaclesplasty produces a more normal anatomic position of the periorbital soft tissues facilitating both function and aesthetics.
Davis
Charles
C
Central & Southern New Zealand Craniofacial Program, Wellington, New Zealand. info@craniofcialsurgery.co.nz
Lauritzen
Claes
C
D000293:Adolescent; D000328:Adult; D002648:Child; D003394:Craniofacial Dysostosis / Q000601:surgery*; D004954:Esthetics; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006972:Hypertelorism / Q000601:surgery; D008297:Male; D013508:Ophthalmologic Surgical Procedures / Q000379:methods*; D009915:Orbit / Q000601:surgery*; D019340:Osteotomy, Le Fort; D012189:Retrospective Studies