Flodmark P, Wattsgård C
Department of Diagnostic Radiology, Central Hospital Halmstad, Sweden.
Pediatr Radiol. 2001 Jan;31(1):36-7. doi: 10.1007/s002470000363.
Cerebro-costo-mandibular syndrome is a rare disorder characterized by rib malformations, various degrees of cerebral maldevelopment, mental deficiency, palatal defects, and micrognatia. This syndrome was first described in 1966. The majority of cases are sporadic, but a few instances of familial occurrence have been reported, some with an autosomal recessive pattern of inheritance. Mortality in early age has been high, probably mostly due to respiratory insufficiency secondary to rib abnormalities and flail chest. We report a mother and son with this disorder, suggesting autosomal dominant transmission.
脑-肋-下颌综合征是一种罕见的疾病,其特征为肋骨畸形、不同程度的脑发育不全、智力缺陷、腭裂和小颌畸形。该综合征于1966年首次被描述。大多数病例为散发性,但也有少数家族性发病的报道,部分呈常染色体隐性遗传模式。早年死亡率较高,可能主要是由于肋骨异常和连枷胸继发的呼吸功能不全。我们报告了一对患有该疾病的母子,提示为常染色体显性遗传。