Teyton L, Mora J J, Loyau G, Mahoudeau J, L'Hirondel J L, Lebrun E, Dumas M, Ollivier D
Rev Med Interne. 1984 Mar;5(1):76-82. doi: 10.1016/s0248-8663(84)80084-3.
Cleidocranial dysostosis is a rare embryopathy affecting the whole osseous system. Anomalies predominate on skull, chest and dentition but the whole skeleton can be affected especially pelvic girdle and spine. An autosomal transmission is often demonstrable; isolated forms are very uncommon. We present two cases fortuitously diagnosed in our ward: a familial cleidocraniopelvic major form and an incomplete isolated form. The treatment must be especially oriented towards dental malformations.
锁骨颅骨发育不全是一种罕见的影响整个骨骼系统的胚胎病。异常主要出现在颅骨、胸部和牙列,但整个骨骼都可能受到影响,尤其是骨盆带和脊柱。常可证实为常染色体遗传;孤立病例非常罕见。我们展示了在我们病房偶然诊断出的两例病例:一例家族性锁骨颅骨骨盆主要型和一例不完全孤立型。治疗必须特别针对牙齿畸形。