Levine C
Department of Radiology, University of Missouri-Columbia Hospital 65212.
J Pediatr Surg. 1989 Mar;24(3):233-40. doi: 10.1016/s0022-3468(89)80001-6.
Congenital defects of lymphatics constitute a spectrum of disorders that may manifest with a variety of clinical presentations including lymphedema, chylous effusions, lymphangiomatous malformations with cystic masses and localized gigantism, and intestinal lymphangiectasia with malabsorption. These entities constitute a relatively rare group of disorders, the origin of which remains somewhat controversial, but in some it appears to be due to early lymphatic obstruction. Five cases are described, which demonstrate the anatomical pathology of these entities. A classification and description of the defects is also presented. An attempt is made to present a unified theory of origin for this seemingly diverse group of diseases. While these entities may be challenging from a diagnostic and therapeutic standpoint, a wide variety of imaging modalities, which includes lymphography, computed tomography scanning, and ultrasound, may be used to diagnose the extent and internal structural characteristics of the abnormalities.
先天性淋巴管缺陷构成了一系列疾病，其临床表现多样，包括淋巴水肿、乳糜性积液、伴有囊性肿块和局部巨大症的淋巴管瘤样畸形，以及伴有吸收不良的肠淋巴管扩张症。这些病症构成了一组相对罕见的疾病，其起源仍存在一定争议，但在某些情况下似乎是由于早期淋巴管阻塞所致。本文描述了5例病例，展示了这些病症的解剖病理学。还对缺陷进行了分类和描述。试图为这一看似多样的疾病群体提出一个统一的起源理论。虽然从诊断和治疗角度来看，这些病症可能具有挑战性，但多种成像方式，包括淋巴管造影、计算机断层扫描和超声，可用于诊断异常的范围和内部结构特征。
