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PURPOSE:
Hypertelorbitism is an abnormal increase in the bony interorbital distance and has been associated with a variety of congenital deformities including syndromic craniosynostosis and Tessier midline clefts. Early surgical correction may result in more pronounced midface growth disturbance or relapse. Thus, timing for surgical correction remains controversial. To address some of the debate over timing of surgery and postoperative relapse, we therefore evaluated our own experience with correction of hypertelorbitism. We present 33 patients undergoing facial bipartition or orbital box-osteotomies for hypertelorbitism and report on their degree of relapse seen after a mean follow-up of 14.0 years.
METHODS:
Patients with hypertelorbitism diagnosed by our multidisciplinary team at UCLA between the years 1975 to 2009 and treated with either facial bipartition or orbital box osteotimy and repositioning who had long-term follow-up were studied (n=33). Pateints with asymmetric or non-midline rare craniofacial clefts, incomplete records, or follow-up less than 7 years were excluded in this study. Age at time of first surgery, pre-operative interdacryon distance, and immediate post-operative interdacryon distance were recorded. Measurements were made based on computed tomographic scans and intra-operatively with calipers. For analysis, patients were grouped according to age (< 6 or >6) and preoperative interdacryon distance: mild, 30-34 mm; moderate, 35-40 mm; severe, greater than 40mm. Relapse (interdacryon distance greater than 15% above the norm) was determined on postoperative follow-up and need for secondary correction was noted. Physician satisfaction score (0-4) was also assessed.
RESULTS:
Diagnoses included Crouzon syndrome (n=13), Apert (n=8), Pfeiffer (n=3), Saethre-Chotzen (n=1), Jackson-Weiss (n=1), Antley-Bixler (n=1), non-syndromic craniosynostosis (n=1), and median craniofacial dysplasia (n=5). The average age at time of surgery was 9.2 ± 3.0 years. The mean pre-operative interdacryon distance was 36.5 ± 4.8 mm and the immediate intra-operative distance achieved was 18.3 ± 1.4 mm. With regard to age at the time of initial procedure, patients less than 6 years of age were all noted to have relapse and 83% underwent revisionary surgery. In patients 6 years of age or greater, only 11% had relapse and required reoperation. Yet, satisfaction scores were similar (3.2 vs 3.5). With regard to severity of hypertelorbitism, there was no relapse noted among patients with mild hypertelorbitism (IOD=30-34mm). Among those with moderate hypertelorbitism (IOD=35-40mm), 29.4% developed relapse. By contrast, all patients with severe hypertelorbitism (IOD>40mm), were noted to have relapse requiring repeat correction. Satisfaction scores were similar (3.4 vs 3.3 vs 3.1).
CONCLUSION:
Relapse following surgery for hypertelorbitism is related to age of the patient at correction and the preoperative severity. Depending on the psychosocial situation, surgical repositioning of the orbits should be delayed until later childhood.