Corsois L, Boman F, Sfeir R, Mention K, Michaud L, Poddevin F, Mestdagh P, Gottrand F
Anatomie et cytologie pathologiques, site Eurasanté, CHRU, 59037 Lille cedex, France.
Arch Pediatr. 2004 Oct;11(10):1205-8. doi: 10.1016/j.arcped.2004.06.027.
Hirschsprung's disease (HD) involves the entire colon in less than 5% of cases, and the association of extensive HD with intestinal malrotation is very rare. This association of symptoms may delay both diagnosis and treatment. An infant presented with an intermittent occlusive syndrome that began neonatally. Intestinal malrotation was diagnosed radiologically, and treated surgically when the child was 2 months old. However, a chronic occlusion persisted. Biopsies of the rectum and the appendix demonstrated an absence of neurons in intestinal plexi. When the child was 17 months old, ileostomy and surgical excision of the segment affected by HD (the colon and terminal ileum) were performed. An ileoanal anastomosis was performed at the age of 29 months, with favorable outcome. The persistence of symptoms of intestinal occlusion after attempted treatment of intestinal malrotation must therefore suggest the possibility of associated HD in a young child.
先天性巨结肠（HD）在不到5%的病例中累及整个结肠，广泛型HD与肠旋转不良的关联非常罕见。这种症状关联可能会延迟诊断和治疗。一名婴儿自出生起就出现间歇性肠梗阻综合征。经放射学诊断为肠旋转不良，并在患儿2个月大时进行了手术治疗。然而，慢性肠梗阻持续存在。直肠和阑尾活检显示肠神经丛中无神经元。患儿17个月大时，进行了回肠造口术以及对受HD影响的节段（结肠和回肠末端）进行手术切除。29个月大时进行了回肠肛管吻合术，结果良好。因此，在尝试治疗肠旋转不良后肠梗阻症状持续存在，必须提示幼儿存在相关HD的可能性。
