Autosomal recessive hereditary disorder	Developmental hereditary disorder	Hereditary disorder of nervous system	Lissencephaly	Micromelia	Multiple malformation syndrome with facial-limb defects as major feature
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Microlissencephaly micromelia syndrome (disorder)
	Microlissencephaly micromelia syndrome
	Basel Vanagaite Sirota syndrome
	A syndrome of abnormal cortical development with characteristics of severe prenatal polyhydramnios, postnatal microcephaly, lissencephaly, upper limb micromelia, dysmorphic facies (coarse face, hypertrichosis, and short nose with long philtrum), intractable seizures, and early death. Hypoparathyroidism was noted in one case.
	syndroom van microlissencefalie en micromelie
	syndroom van Basel-Vanagaite-Sirota

Id	723405001
Status	Primitive

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

Associated morphology	Congenital smallness
Finding site	Entire limb
Occurrence	Congenital
Pathological process	Pathological developmental process

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

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q04.3
Rule	TRUE
Advice	ALWAYS Q04.3 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified