Autosomal recessive hereditary disorder
Congenital cerebral ventriculomegaly
Developmental hereditary disorder
Hereditary disorder of nervous system
Hereditary nephropathy
Multiple congenital cysts of kidney
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Cerebral ventriculomegaly, cystic kidney disease (disorder)
Cerebral ventriculomegaly, cystic kidney disease
VMCKD - ventriculomegaly with cystic kidney disease
Congenital nephrosis, cerebral ventriculomegaly syndrome
A rare genetic syndrome with a central nervous system malformation as a major feature, and characteristics of a triad of high alpha-fetoprotein levels in both maternal serum and amniotic fluid, cerebral ventriculomegaly, renal macro and microcysts. Variable findings include congenital nephrotic syndrome, aqueductal stenosis, gray matter heterotopias and cardiac malformations among others.
Id1216942009
StatusPrimitive
Associated morphologyPolycystic change
Finding siteKidney structure
OccurrenceCongenital
Associated morphologyEnlargement
Finding siteEntire ventricle of brain
OccurrenceCongenital
Pathological processPathological developmental process
ICD-10 complex map reference set
TargetQ07.8
RuleTRUE
AdviceALWAYS Q07.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified