Diethylstilbestrol syndrome

Orpha code: 1916OMIM code:

Definition

A malformation syndrome reported in offspring (children and grandchildren) of women exposed to diethylstilbestrol (DES) during pregnancy and is characterized by reproductive tract malformations, decreased fertility and increased risk of developing clear cell carcinoma of the vagina and cervix in young women. Reproductive malformations reported in DES syndrome include small, T-shaped uteri and other uterotubal anomalies that increase the risk of miscarriages in women and epididymal cysts, microphallus, cryptorchidism, or testicular hypoplasia in men. DES, a synthetic nonsteroidal estrogen was widely prescribed from 1940-1970 to prevent miscarriage.

Disease data
Classification

Malformation syndrome

Synonyms
DES embryofetopathy
Embriofetopatia dietylstilbestrolowa
Embriofetopatia DES
Zespół DES
DES syndrome
Diethylstilbestrol embryofetopathy
Distilbene embryofetopathy
ORPHA code
1916
OMIM code
-
ICD10 code
Q86.8
ICD11 code
LB44.6

No additional description.

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