| الوصف: |
A monozygotic twin pair is concordant for craniofacial malformations of Tessier number 0-14 cleft. Craniofacial findings were confirmed by anthropometric and cephalometric analyses: a short and protruded upper lip with wide philtrum, a short nose with short and narrow columella, platyrrhine like nostril with wide interalar distance, a retruded midface, and a brachycephlic head. Other findings were a small notch of the vermilion border, a diastema between the maxillary central incisors, a duplicated anterior nasal spine, bilateral aplasia of the alar and upper lateral cartilages, a notch of their caudal nasal bone, and a low placed cribriform plate. These findings suggest a modest form of true median cleft lip of Tessier number 0-14 cleft. Other physical findings were clinodactyly of little fingers, melanonychia on all the finger nails, and unilateral breast deformities. In the case of elder brother, Millard type cleft lip repair for correction of vermilion-lip-nose deformities, alar advancement for narrowing of alar bases, resection of duplicated anterior nasal spine, rolled ear cartilage graft for caudal support of the nose, excision of supernumerary nipple and areola were done simultaneously. In the cases of younger brother, in addition to lip surgeries, augmentation rhinoplasty using calvarial bone onlay graft and resection of duplicated nipple and areolar were performed. Most craniofacial measurements are turned to the range of age-matched normal boy : width of the caudal nose ; width of the columella ; width of the philtrum ; and inclination of the nostril axis. However, nasal tip is protruded so much in relation to columellar elongation that disharmony of the basal nose results. ; 저자들은 14세 남자 일란성 쌍생아에서 발생한 Tessier 0-14번 두 개안면열을 치험하였다. 형제 사이의 징후는 서로 일치하였으며, 두개와 안면의 연조직과 골조직을 모두 침범하였지만 그 정도는 매우 경미하였다. Kawamoto가 기술한 특징적인 징후 외에 본 형제에서 공통적으로 더 발견된 소견은 단두, 짧은 코, 짧고 좁은 비축주, 흑인형의 비공 및 양쪽 비공저의 절흔, 넓은 비익간격, 양쪽 이상구 주위를 포함한 중안면골의 후퇴, 짧은 상구순의 전출, 중앙부 홍순의 상구순 침범, 넓은 인중, 구리빛 손톱 및 제5지의 만지증이었으며, 형제 사이에 일치하지 않는 소견은 안검하수증, ... |