Postmenarchal development of chylous ascites in acrocephalosyndactyly with congenital lymphatic dysplasia.

Abstract:

BACKGROUND:Acrocephalosyndactyly is a syndrome characterized by congenital malformation of the skull with craniosynostosis, midface hypoplasia, and symmetrical webbed fusion of the fingers and toes. We describe a possible pathophysiologic mechanism for chylous ascites that developed several months after menarche in a woman with acrocephalosyndactyly and congenital lymphatic dysplasia. CASE:A 25-year-old nulligravid woman with acrocephalosyndactyly, at 18 months after menarche, developed persistent abdominal distension at age 18 years. Laparoscopy at age 25 years revealed chylous ascites with marked chronic peritoneal inflammation, and lymphatic dysplasia with lymphocysts. With hormone manipulation, the chylous ascites fluctuated. CONCLUSION:After menarche in a woman with acrocephalosyndactyly, ovarian steroid hormones might have increased lymph production and hydrostatic pressure, causing rupture of congenitally dysplastic lymph vessels resulting in chylous ascites.

journal_name

Obstet Gynecol

authors

Batt RE,Michalski SR,Mahl T,Reynhout JC

doi

10.1016/s0029-7844(00)01162-5

subject

Has Abstract

pub_date

2001-05-01 00:00:00

pages

829-31

issue

5 Pt 2

eissn

0029-7844

issn

1873-233X

pii

S0029784400011625

journal_volume

97

pub_type

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