Congenital lymphangiectasia with fetal cystic hygroma: Report of two cases with coexistent Down's syndrome
✍ Scribed by Gail R Weingast; Maj. Kenneth D Hopper; Stuart A Gottesfeld; Michael L Manco-Johnson
- Publisher
- John Wiley and Sons
- Year
- 1988
- Tongue
- English
- Weight
- 743 KB
- Volume
- 16
- Category
- Article
- ISSN
- 0091-2751
No coin nor oath required. For personal study only.
✦ Synopsis
Fetal cystic hygromata (lymphangiomata) are commonly seen in association with Turner's syndrome, and have been previously described as diagnostic of that disorder.' However, in recent years, cystic hygromata have been found in association with several other genetic malformations, including 6 fetuses with Down's synd r ~m e . ~-~ Differentiating between Turner's syndrome with its low risk of recurrence in subsequent pregnancies vs other malformation syndromes with a higher recurrence risk is important. We report 2 cases of cystic hygroma diagnosed in fetuses with Down's syndrome who demonstrated diffuse skin thickening with septations and cutaneous cysts indicative of severe lymphatic involvement. Congenital lymphangiectasia is representative of a severe fatal form of cystic hygroma. Amniocentesis with karyotyping is recommended in any fetus with congenital lymphangiectasia.
CASE REPORTS Case 1
A 22-year-old G2P1 female was referred for a second opinion to our department at 12 weeks' menstrual age for evaluation of a possible cystic hygroma. The patient's previous pregnancy and From the '