Autosomal recessive hereditary disorder	Congenital anomaly of the kidney	Developmental hereditary disorder	Hereditary disorder of nervous system	Hereditary nephropathy	Hydranencephaly	Multiple system malformation syndrome
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Multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, hydranencephaly syndrome (disorder)
	MARCH syndrome
	Multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, hydranencephaly syndrome
	A rare genetic lethal multiple congenital anomalies/dysmorphic syndrome with characteristics of severe hydranencephaly and renal dysplasia or agenesis. Pregnancy is complicated by oligo or anhydramnios, leading to features of Potter sequence (including typical facies and microretrognathia, limb contractures, talipes equinovarus, and pulmonary hypoplasia) in the fetus. Affected fetuses either die in utero or shortly after birth. Histology of the brain shows widespread presence of multinucleated neurons and glial cells.
	syndroom van multinucleaire neuronen, anhydramnion, dysplasie van nier, hypoplasie van cerebellum en hydranencefalie
	syndroom van multinucleaire neuronen, anhydramnion, renale dysplasie, cerebellaire hypoplasie en hydranencefalie syndroom van multinucleaire neuronen, anhydramnion, nierdysplasie, hypoplasie van cerebellum en hydranencefalie MARCH-syndroom

Id	1169358003
Status	Primitive

Associated morphology	Absence
Finding site	Cerebral hemisphere structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Morphologically abnormal structure
Finding site	Kidney structure
Occurrence	Congenital
Pathological process	Pathological developmental process

ICD-10 complex map reference set

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified