Roshkow J E, Haller J O, Berdon W E, Sane S M
Department of Radiology, State University of New York Health Science Center at Brooklyn.
Pediatr Radiol. 1988;19(1):45-9. doi: 10.1007/BF02388410.
The term neurocristopathy has been applied to the association of Hirschsprung's disease, Ondine's curse (Congenital Hypoventilation Syndrome) and congenital neuroblastoma. Eight newborns with Hirschsprung's disease and Ondine's curse are discussed. Five of these have been seen by the authors. The remaining three patients are reported in the literature. In six of the infants (5 of ours, 1 from the literature) total colonic aganglionosis was found. Congenital neuroblastoma was present in two of the infants. In infants presenting with Hirschsprung's disease (especially of the long segment type) and breathing difficulties, the presence of a neurocristopathy should be considered.
神经嵴病这一术语已被用于指先天性巨结肠、翁丁氏咒诅(先天性低通气综合征)和先天性神经母细胞瘤之间的关联。本文讨论了8例患有先天性巨结肠和翁丁氏咒诅的新生儿。其中5例是作者所诊治的。其余3例患者来自文献报道。在6例婴儿中(我们诊治的5例,文献报道的1例)发现了全结肠无神经节症。2例婴儿患有先天性神经母细胞瘤。对于患有先天性巨结肠(尤其是长段型)且有呼吸困难的婴儿,应考虑存在神经嵴病。