Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome
차봉근, 최동순, 장인산, Yook Hyun-Tae, 이승엽, 이상신, 이석근,
소속 상세정보
차봉근 ( Cha Bong-Kuen ) - Gangneung-Wonju National University College of Dentistry Department of Orthodontics
최동순 ( Choi Dong-Soon ) - Gangneung-Wonju National University College of Dentistry Department of Orthodontics
장인산 ( Jang In-San ) - Gangneung-Wonju National University College of Dentistry Department of Orthodontics
( Yook Hyun-Tae ) - Chonbuk National University School of Dentistry Department of Orthodontics
이승엽 ( Lee Seung-Youp ) - Chonbuk National University School of Dentistry Department of Orthodontics
이상신 ( Lee Sang-Shin ) - Gangneung-Wonju National University College of Dentistry Department of Oral Pathology
이석근 ( Lee Suk-Keun ) - Gangneung-Wonju National University College of Dentistry Department of Oral Pathology
Abstract
Background: A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagnosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a short nose with bulbous tip.
Methods: Fundamental aberrant growth may be associated with the cranial base structure in radiological observation.
Results: The Apert syndrome patient had a shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view.
Conclusion: Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.
키워드
Apert syndrome; Midface hypoplasia; Anterior cranial base; Retruded zygomatic axes
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