Ray S, Bowen J R
Clin Orthop Relat Res. 1984 May(185):77-82.
In the early part of this century sporadic reports of campomelic dysplasia appeared in the literature, along with reports of other entities, under the term "congenital bowing of the long bones." In 1970 Spranger et al. considered campomelic dysplasia a distinct entity. The terms "camptomelic" and "campomelic" have been used interchangeably in the literature since that time. The patient in the present report is a 6 1/2-year-old white girl with classic long-limbed campomelic dysplasia. The patient has had severe respiratory problems throughout her life. As an infant she was treated by a Pavlik harness for subluxation of the hips. She later required two cervical and two thoracic posterior spinal fusions, as well as release of complex foot deformities and osteotomy of the right tibia. Perioperative complications have been significant. Currently, her height and weight are those of a 2 1/2-year-old child, and she has the bone age of a 3 1/2-year-old child. Nevertheless, she is in first grade at a regular school. Certainly, the prognosis for patients with campomelic dysplasia is guarded, but some survivors may be helped by judicious orthopedic care.
在本世纪初，有关弯肢性发育异常的零星报道与其他病症的报道一同出现在文献中，统称为“先天性长骨弯曲”。1970年，施普朗格等人将弯肢性发育异常视为一种独特的病症。从那时起，“弯肢性的”和“弯肢的”这两个术语在文献中一直交替使用。本报告中的患者是一名6岁半的白人女孩，患有典型的长肢型弯肢性发育异常。该患者一生都有严重的呼吸问题。婴儿期时，她因髋关节半脱位接受了帕夫利克吊带治疗。后来，她需要进行两次颈椎和两次胸椎后路脊柱融合手术，以及矫正复杂的足部畸形和右胫骨截骨术。围手术期并发症严重。目前，她的身高和体重相当于2岁半儿童的水平，骨龄相当于3岁半儿童的水平。然而，她在一所普通学校读一年级。当然，弯肢性发育异常患者的预后不容乐观，但一些幸存者可能会因明智的骨科护理而得到帮助。
