지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한악안면성형재건외과학회 Maxillofacial Plastic Reconstructive Surgery Maxillofacial Plastic Reconstructive Surgery 제40권 제41호
- 발행연도
- 2018.12
- 수록면
- 1 - 8 (8page)
- DOI
- https://doi.org/10.1186/s40902-018-0179-8
이용수
초록· 키워드
오류제보하기
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.
목차
등록된 정보가 없습니다.
참고문헌 (31)
참고문헌 신청
Athanasiadis AP / 2008 / Papasozomenou P et al(2008)Apert syndrome : the current role of prenatal ultrasound and genetic analysis in diagnosis and counselling / Fetal Diagn Ther 24:495~498
Martelli H Jr / 2008 / Apert syndrome: report of a case with emphasis on craniofacial and genetic features / Pediatr Dent 30:464~468
Hutson LR Jr / 2007 / Tracheal anomalies complicating ventilation of an infant with Apert syndrome / J Clin Anesth 19:551~554
Bochukova EG / 2009 / Rare mutations of FGFR2 causing Apert syndrome: identification of the first partial gene deletion, and an Alu element insertion from a new subfamily / Hum Mutat 30:204~211
Hajihosseini MK / 2009 / Evidence that Fgf10 contributes to the skeletal and visceral defects of an Apert syndrome mouse model / Dev Dyn 238:376~385
Martelli H Jr / 2008 / Apert syndrome: report of a case with emphasis on craniofacial and genetic features / Pediatr Dent 30:464~468
Hutson LR Jr / 2007 / Tracheal anomalies complicating ventilation of an infant with Apert syndrome / J Clin Anesth 19:551~554
Bochukova EG / 2009 / Rare mutations of FGFR2 causing Apert syndrome: identification of the first partial gene deletion, and an Alu element insertion from a new subfamily / Hum Mutat 30:204~211
Hajihosseini MK / 2009 / Evidence that Fgf10 contributes to the skeletal and visceral defects of an Apert syndrome mouse model / Dev Dyn 238:376~385
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