Ocular adnexal problems in craniofacial deformities

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Summary Ocular adnexal problems occured in 86% (145) of 170 patients having major craniofacial reconstructive procedures at our medical centre in the past six years. Those without problems included certain minimal manifestations with craniofacial clefting phenomena. Evaluations were done by measurements and evaluation directly on the patient, life-size photographs, and cephalometric or 2 metre X-rays. Detailed ophthalmological evaluations were carried out on each patient. The syndromes were broken into six broad categories. These with the number of patients in parentheses were: orbital hypertelorism (24), craniofacial dysostosis (36), isolated craniofacial dysostosis or plagiocephaly (36), mandibulofacial dysostosis (10), hemifacial microsomia (24), and other deformities (40). Specific ocular adnexal problems related to the syndromes were evaluated. Vertical canthal displacement occurred in 66% of patients, horizontal canthal displacement in 33%, colobomas and coloboma-like conditions in 8%, nasolacrimal apparatus problems in 13%, ptosis in 15%, exorbitism in 14%, blepharophimosis in 1.7%, and palpebral fissure alterations in 53%. Certain problems tend to be most frequently associated with certain diagnoses. Vertical canthal displacement occurred in 100% (36/36) of patients with craniofacial dysostosis or its variants; there was also a high incidence of patients with isolated craniofacial dysostosis, and patients in the “other” classification category. Horizontal canthal displacement occurred in 100% of patients with orbital hypertelorism, and there was a high incidence of this in patients with craniofacial dysostosis. Colobomas and coloboma-like conditions occurred most consistently in orbital hypertelorism and mandibulofacial dysotosis. Nasolacrimal apparatus problems were most frequently associated with orbital hypertelorism and craniofacial dysostosis. Ptosis occured most consistently in craniofacial dysostosis, as did exorbitism. Methods for correction included several variations of known and newer techniques. Vertical canthal displacement up to 3 mm was corrected by displacing the lateral canthus through a coronal incision without preliminary dissection. Beyond 3 mm, preliminary dissection and orbital movement or bone grafts were necessary. Horizontal canthal displacement was best corrected utilizing preliminary dissection of the medial canthal ligament, and bone movements if the bony interorbital distance was more than 5 mm above normal. Colobomas, nasolacrimal apparatus problems, and ptosis were corrected by more standard ophthalmological procedures. Exorbitism was corrected by orbital advancement. Inadequately corrected ocular adnexal problems particularly in the canthal regions severely compromised the end result of the craniofacial procedure. Attention to their specific anatomical deformity and details of correction results in a high rate of success in handling these problems.