Perry H D, Cossari A J
Br J Ophthalmol. 1986 May;70(5):396-9. doi: 10.1136/bjo.70.5.396.
A 3 1/2-year-old female presented with Turner's syndrome and Nonne-Milroy-Meige disease. Ocular findings included strabismus and bilateral chemosis which was unchanging and persisted throughout the four years the patient was followed up. Histopathological findings included diffuse lymphangiectasia and dense connective tissue surrounding the dilated lymph channels. Although the association between congenital lymphoedema and Turner's syndrome is common, the lymphoedema usually disappears by the first year of life. The persistence of the lymphoedema beyond this age is rare, as is the presence of the persistent chemosis. This report represents the first histopathological documentation of congenital lymphangiectasis in association with Turner's syndrome.
一名3岁半的女性患有特纳综合征和农内-米尔罗伊-梅热病。眼部检查结果包括斜视和双侧球结膜水肿，在对该患者进行随访的四年中，球结膜水肿一直没有变化且持续存在。组织病理学检查结果包括弥漫性淋巴管扩张以及扩张的淋巴管周围有致密的结缔组织。虽然先天性淋巴水肿与特纳综合征之间的关联很常见，但淋巴水肿通常在出生后第一年内消失。超过这个年龄淋巴水肿仍持续存在的情况很罕见，持续性球结膜水肿的情况也很罕见。本报告是先天性淋巴管扩张与特纳综合征相关的首例组织病理学记录。
