Abstract

Materials and Methods: Clinical data was obtained from subjects in a long-standing natural history study of FD/MAS (Screening and Natural History Study of Fibrous Dysplasia, NIH 98-D-0145). Indications for surgery were categorized as: facial deformity, visual loss, hearing impairment, nasal airway obstruction, malocclusion, or aneurysmal bone cyst (ABC). Surgeries were divided as: debulking (partial removal and/or recontouring of FD), reconstruction (resection of FD bone with introduction of hardware and/or grafting material), optic nerve decompression, aneurysmal bone cyst enucleation, or biopsy. Methods of Data Analysis: Statistical analysis was performed with unpaired t-test and Fisher’s exact test. Results and Outcomes Data: Of 169 patients with FD/MAS, 133 (79%) had CF-FD. Of these, 33 patients (25%) underwent a total of 95 craniofacial operations. The most common indication for initial operation was facial deformity (24%). Of the 33 patients, 23 (70%) underwent a secondary procedure in the same anatomic location. The distribution of surgeries was as follows: debulkings 36 (42%), reconstructions 26 (30%), optic nerve decompressions 15 (17%), aneurysmal bone cyst enucleations 9 (10%), biopsies 9 (10%). The mean length of postoperative follow-up was 13.6 years (range 0–39, SD 10.6). The most common indication for re-operation was FD regrowth, which occurred significantly more frequently after debulking procedures (26/36, 72%) than reconstructions (10/30, 33%) (p=0.0027). Theprevalence of MAS-associated GH excess was higher in the surgically treated group (12/36, 36%) than in patients who were managed non-operatively (14/100, 14%) (p = 0.0098). Reoperations for FDregrowthweremore common inpatients with GH excess (16/34, 47%) than in patients without GH excess (18/50, 36%) (p = 0.0465). Conclusions: Facial deformity was the most common indication for surgical intervention in our patient cohort. Postoperative FD regrowth and re-operation are common after craniofacial surgery; in our patient population, FD regrowth with facial deformity was the most common reason for secondary operation. Our study corroborated previous observations, which indicate that resection and reconstruction with hardware and/or grafting material may result in less regrowth and fewer re-operations as compared to more conservative debulking and recontouring techniques. Finally, we also found that MAS-associated GH excess is a risk factor for craniofacial morbidity and postoperative FD regrowth.

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