Servelle M
Centre Chirurgical Val d'Or, Saint Cloud, France.
J Cardiovasc Surg (Torino). 1991 Mar-Apr;32(2):159-65.
Of 300 congenital malformations of the lymphatics of the small intestine investigated, 120 were operated upon. Intestinal lymphography shows no injection of the cisterna chyli and histology proves that the mesenteric lymph nodes are abnormal. The induced hyperlipidemia test permits a biochemical diagnosis. Modifications of the flow of the chyle secondary to the hypoplasia of the cisterna chyli were studied: (1) in the abdominal cavity, (2) in the extraperitoneal region and the lower limb, (3) in the thorax, especially the chyle drainage channels from the diaphragm towards the cervical region. Our investigations have established that the following diseases are produced by malformation of the lymphatics of the small intestine: protein losing enteropathy, chyloperitoneum, chyluria, lymphedema with chyle reflux, chylothorax, chylopericardium, chyle reflux in the pulmonary lymphatics, hypoproteinemia and food allergies. A better understanding of the pathophysiology of the malformations of the intestinal lymphatics permits a more rational treatment of the diseases produced by this anomaly.
在对300例小肠淋巴管先天性畸形进行调查的病例中，120例接受了手术治疗。肠道淋巴造影显示乳糜池无造影剂注入，组织学检查证实肠系膜淋巴结异常。诱导性高脂血症试验可进行生化诊断。研究了因乳糜池发育不全继发的乳糜流动改变：（1）在腹腔内；（2）在腹膜外区域和下肢；（3）在胸部，尤其是从膈肌至颈部区域的乳糜引流通道。我们的研究已证实，小肠淋巴管畸形可导致以下疾病：蛋白丢失性肠病、乳糜性腹水、乳糜尿、伴有乳糜反流的淋巴水肿、乳糜胸、乳糜心包、肺淋巴管乳糜反流、低蛋白血症和食物过敏。对肠道淋巴管畸形病理生理学的更好理解有助于对由这种异常引起的疾病进行更合理的治疗。
