Genetic disease
Primary lymphedema
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Ephrin receptor B4-related lymphatic-related hydrops fetalis (disorder)
EPHB4-related lymphatic-related hydrops fetalis
EPHB4-related generalized lymphatic dysplasia with non-immune hydrops fetalis
EPHB4-related generalized lymphatic dysplasia with atrial septal defect
Ephrin receptor B4-related lymphatic-related hydrops fetalis
A rare primary lymphedema characterized by a highly variable lymphatic phenotype ranging from severe lymphatic-related hydrops fetalis, which may cause perinatal demise or fully resolve to become completely asymptomatic, to a mild presentation in older patients with persistent varicose veins, peripheral edema and impaired lymph drainage in the lower limbs. Atrial septal defect has been described in association and may be the only anomaly in some patients.
Id1222666002
StatusPrimitive
Associated morphologyLymphatic edema
Finding siteLimb structure
Pathological processPathological developmental process
ICD-10 complex map reference set
TargetI89.0
RuleTRUE
AdviceALWAYS I89.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified