An unusual complication after craniofacial surgery for Apert syndrome

• Main Authors: Abhay A Lune, Bharat B Dogra, Sonali A Lune
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2014-01-01
• Series: Medical Journal of Dr. D.Y. Patil University
• Subjects: Apert syndrome; brachycephaly; craniofacial surgery; ectropion; syndactyly
• Online Access: http://www.mjdrdypu.org/article.asp?issn=0975-2870;year=2014;volume=7;issue=1;spage=91;epage=94;aulast=Lune

Apert syndrome is a rare genetic disorder, characterized by premature fusion of skull sutures, mid-face hypoplasia and syndactyly of the hands and feet. It is inherited as autosomal dominant or sporadic and is associated with increased paternal age. It arises from mutations in the fibroblast growth factor receptor 2 gene on chromosome 10q26. A case of Apert syndrome who had undergone craniofacial surgery elsewhere 4 years back presented to us with purulent discharge near the lateral orbital margin of right orbit, watering and redness of the right eye. He had telltale signs of this syndrome in the form of skull deformities such as brachycephaly, frontal bony prominence, mid-face hypoplasia, proptosis and syndactyly of both hands and feet. There was a surgical scar of previous craniofacial surgery over the bi-coronal region. He had a discharging granuloma over the lateral orbital margin and the adjacent lower eyelid had developed cicatricial ectropion. X-ray and computed tomography scan orbit confirmed the clinical suspicion of osteomyelitis of the underlying zygomatic bone at the site of miniplate and screw fixation of the earlier surgery. He was treated with excision of granuloma and extraction of loose screw and infected miniplate while ectropion was corrected by rotation advancement of temporal skin flap.