Lymph vessel defects in patients with ovarian dysgenesis.
Lymphangiography of the lower extremities was performed in 21 patients with ovarian dysgenesis. Seventeen subjects turned out to have hypoplastic superficial lymph vessels of the lower limbs. Aplasia of the lymphatics of the legs occurred in four instances. Hypoplastic lymph pathways were also observed in the pelvis and in the retroperitoneal space. Although the impairment of the lymphatic channels occurred in all cases of ovarian dysgenesis, it appears that it is more severe in patients with streak gonads. There was no correlation between the lymphatic defect and the karyotype. Patients with and without lymphedema had similar lymphatic defects. Our findings suggest that hypoplasia of the superficial lymph vessels of the lower limbs is a common abnormality in patients with ovarian dysgenesis irrespective of their chromosomal complements. The hypothesis that lymphedema in ovarian dysgenesis is mainly attributable to hypoplasia of the lymphatic channels is supported by this study. However, it appears that other factor(s) may also play a role in the pathogenesis of the lymphedema, since, despite lymphatic hypoplasia, lymphedema does not develop in all instances.
D000293:Adolescent; D000328:Adult; D005260:Female; D006059:Gonadal Dysgenesis / Q000235:genetics*; D006801:Humans; D008208:Lymphatic System / Q000002:abnormalities*; D008209:Lymphedema / Q000235:genetics; D010053:Ovary / Q000002:abnormalities*; D014424:Turner Syndrome / Q000235:genetics