Document Detail


Postmenarchal development of chylous ascites in acrocephalosyndactyly with congenital lymphatic dysplasia.
MedLine Citation:
PMID:  11336770     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
R E Batt; S R Michalski; T Mahl; J C Reynhout
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Obstetrics and gynecology     Volume:  97     ISSN:  0029-7844     ISO Abbreviation:  Obstet Gynecol     Publication Date:  2001 May 
Date Detail:
Created Date:  2001-05-04     Completed Date:  2001-05-24     Revised Date:  2009-10-26    
Medline Journal Info:
Nlm Unique ID:  0401101     Medline TA:  Obstet Gynecol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  829-31     Citation Subset:  AIM; IM    
Affiliation:
Department of Gynecology-Obstetrics, State University of New York at Buffalo, 5648 Broadway, Buffalo, NY 14086-2317, USA. ronbatt@localnet.com
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MeSH Terms
Descriptor/Qualifier:
Acrocephalosyndactylia / complications*
Adult
Chylous Ascites / complications,  diagnosis*,  diet therapy
Diagnosis, Differential
Female
Humans
Lymphatic Diseases / complications*,  congenital
Menarche

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