MHAC - microhydranencephaly
PCH4 - pontocerebellar hypoplasia type 4
Bilateral generalized polymicrogyria
Joubert syndrome
PCH6 - pontocerebellar hypoplasia type 6
Micropolygyria
Orofaciodigital syndrome type 6
Congenital pontocerebellar hypoplasia type 13
Congenital porencephalia
PCH12 - pontocerebellar hypoplasia type 12
Micromelic dwarf
Joubert syndrome with renal defect
Bilateral polymicrogyria
Cerebellar hypoplasia, intellectual disability, congenital microcephaly, dystonia, anaemia, growth retardation syndrome
Bilateral generalised polymicrogyria
Congenital hypoplasia of brain stem
Spinal cord hypoplasia
Congenital hypoplasia of brainstem
nudE neurodevelopment protein 1-related microhydranencephaly
Polymicrogyria due to tubulin beta 2B class IIb mutation
Lethal hydranencephaly, diaphragmatic hernia syndrome
Unilateral polymicrogyria
Hypoplasia of part of brain
Congenital absence of part of brain (disorder)
Cerebral cortical dysgenesis
Cortical dysgenesis with pontocerebellar hypoplasia due to tubulin beta 3 class III mutation
Permanent neonatal diabetes mellitus with cerebellar agenesis syndrome
Pontocerebellar hypoplasia type 2
Total agenesis of cerebellum
Floppy infant
Short stature, pituitary and cerebellar defect and small sella turcica syndrome
Absence of part of brain
Lissencephaly (disorder)
Fatal pontocerebellar hypoplasia, hypotonia, respiratory insufficiency syndrome
Endosteal hyperostoses
PCH5 - pontocerebellar hypoplasia type 5
Respiratory insufficiency syndrome of newborn
Lethal pontocerebellar hypoplasia, hypotonia, respiratory insufficiency syndrome
CIMDAG syndrome
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type D
Nephronophthisis
Posterior-predominant lissencephaly, broad flat pons and medulla-midline crossing defects syndrome
Cerebral pachygyria
microgyria
Lissencephaly with cerebellar hypoplasia type E
Neonatal diabetes mellitus
Polymicrogyria due to TUBB2B mutation
Varadi Papp syndrome
Lissencephaly with cerebellar hypoplasia
Congenital hypoplasia of basal part of pons
Lissencephaly type 3 metacarpal bone dysplasia syndrome
Lissencephaly type 3 familial foetal akinesia sequence syndrome
Microlissencephaly type A
Type 2 lissencephaly
Congenital cerebellar hypoplasia co-occurrent with tapetoretinal degeneration
Early-onset progressive encephalopathy, hearing loss, pons hypoplasia, brain atrophy syndrome
PCH2 - pontocerebellar hypoplasia type 2
Hemispheric cerebral hypoplasia
Congenital chorioretinal degeneration
Bilateral frontoparietal polymicrogyria
Congenital pontocerebellar hypoplasia type 8
Short rib dysplasia
Fetus with hereditary disease
Microcephaly, corpus callosum and cerebellar vermis hypoplasia, facial dysmorphism, intellectual disability syndrome
Vascular Ehlers-Danlos, polymicrogyria syndrome
Congenital hypoplasia of cerebral hemisphere
Short rib syndrome
Congenital pontocerebellar hypoplasia type 14
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type E
Agyrias
Lissencephaly due to TUBA1A (tubulin alpha 1A) mutation
Nanomelia
Congenital hypoplasia of inner granular layer of cerebellum
Congenital cerebellar hypoplasia
PCH1 - pontocerebellar hypoplasia type 1
PTCD - pontine tegmental cap dysplasia
X-linked intellectual disability, cerebellar hypoplasia, spondyloepiphyseal dysplasia syndrome
Granular cell hypoplasia
Congenital pontocerebellar hypoplasia type 6
PAFAH1B1-related lissencephaly
Cortical dysgenesis with pontocerebellar hypoplasia due to TUBB3 mutation
Bonnemann Meinecke syndrome
Pontocerebellar hypoplasia type 1
Isolated unilateral hemispheric cerebellar hypoplasia
Pachygyria/lissencephaly
Autosomal recessive medullary cystic disease
Pontocerebellar hypoplasia type 6
Foetal microcephaly
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type F
Congenital pontocerebellar hypoplasia type 1
Isolated hypoplasia of cerebellar vermis
Arima syndrome
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type B
X-linked intellectual disability with cerebellar hypoplasia syndrome
Autosomal recessive frontotemporal pachygyria
Epileptic spasms
Pontine tegmental cap dysplasia
Type 3 lissencephaly
Norman disease
Agenesis of cerebellum and hydrocephalus syndrome
Fetal onset olivopontocerebellar hypoplasia
Congenital anomaly of the meninges
lissencephaly
Agenesis of right hemisphere of cerebellum
Joubert syndrome with oculorenal defect
Bilateral frontal polymicrogyria
Congenital pontocerebellar hypoplasia type 5
Congenital hypoplasia of frontal lobe
Hydranencephaly
Lissencephaly with cerebellar hypoplasia type D
Congenital hypoplasia of cerebral white matter
Congenital pontocerebellar hypoplasia type 4
Pachygyria
Floppy baby
Congenital muscular dystrophy with cerebellar involvement
Jeune syndrome
Lissencephaly with cerebellar hypoplasia type A
Asphyxiating thoracic dystrophy
Congenital absence of part of brain
Hydranencephaly (disorder)
SCAR20 - autosomal recessive spinocerebellar ataxia type 20
Cobblestone lissencephaly without muscular or eye involvement
Congenital hypoplasia of pars basalis of pons
Cobblestone lissencephaly without muscular or ocular involvement
Floppy infant syndrome
Pontocerebellar hypoplasia type 8
Permanent diabetes mellitus of infancy
Joubert syndrome with Jeune asphyxiating thoracic dystrophy
COACH (cerebellar vermis hypoplasia, oligophrenia, congenital ataxia, coloboma, hepatic fibrosis) syndrome
Neonatal neuromuscular disorder
Cerebellar aplasia
MPPH syndrome
X-linked lissencephaly type 1
Garcia Lurie syndrome
Hypoplasia of the cerebellum
X-linked lissencephaly with agenesis of corpus callosum and genital anomaly syndrome
Fewer or absent grooves in brain
Microgyria (disorder)
ABSENCE OF CEREBRAL HEMISPHERES CONGEN
Basel Vanagaite Sirota syndrome
CEREBRAL HEMISPHERES ABSENCE CONGEN
COASY gene related pontocerebellar hypoplasia
Pontocerebellar hypoplasia due to CHMP1A (charged multivesicular body protein 1A) mutation
Lissencephaly syndrome
Aprosencephaly
Lissencephaly type 1 due to doublecortin gene mutation
Isolated lissencephaly type 1 without known genetic defect
Hemispheric cerebellar agenesis
Hydranencephaly with proliferative vasculopathy
Pancreatic and cerebellar agenesis syndrome
Congenital small cerebrum
Lissencephalies
Fetal microcephaly
Macrogyria (disorder)
Isolated cerebellar vermis hypoplasia
Microcephalus, cerebellar hypoplasia, cardiac conduction defect syndrome
MARCH syndrome
XK aprosencephaly syndrome
Polymicrogyria with optic nerve hypoplasia
Vascular EDS (Ehlers-Danlos) with polymicrogyria
Jeune thoracic dystrophy
Congenital malformation of anterior pituitary
Pontocerebellar hypoplasia type 7
Congenital hypoplasia of adenohypophysis
Partial agenesis of corpus callosum
Aplasia of the vermis
Congenital hypoplasia of cerebellum
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type A
Lissencephaly co-occurrent with congenital cerebellar hypoplasia type C
Joubert syndrome with hepatic defect
Micromelus
Agyria diffuse
CLP1-related pontocerebellar hypoplasia
Large Gyri of Cerebrum
Kuzniecky syndrome
Partial corpus callosum agenesis, cerebellar vermis hypoplasia with posterior fossa cysts syndrome
Congenital anomaly of anterior pituitary
Bilateral parasagittal parieto-occipital polymicrogyria
Congenital pontocerebellar hypoplasia type 3
Macrogyria
Cerebellar hypoplasia and tapetoretinal degeneration
CONGEN ABSENCE OF CEREBRAL HEMISPHERES
Fewer and broader ridges in brain
Congenital agenesis of brainstem nuclei
Congenital bilateral perisylvian syndrome
Type 1 lissencephaly
Microcephaly, cerebellar hypoplasia, congenital heart conduction defect syndrome
Lissencephaly co-occurrent with congenital cerebellar hypoplasia
Aplasia of part of brain
Isolated cerebellar vermis agenesis
Congenital cerebral porosis
Classic lissencephaly
BFPP - bilateral frontoparietal polymicrogyria
Congenital hypoplasia of anterior pituitary
Dyke-Davidoff-Masson syndrome
Pontocerebellar hypoplasia type 4
Fetal hereditary disease
Congenital small cerebellum
Isolated agenesis of cerebellar vermis
Congenital hypoplasia of part of brain
Congenital conduction defect
Cerebellum agenesis with hydrocephaly
MPPH (megalencephaly, polymicrogyria, polydactyly, hydrocephalus) syndrome
Foetal onset olivopontocerebellar hypoplasia
Craniotelencephalic dysplasia
Anterior pituitary hormone deficiency
Congenital malformation of the meninges
Cerebral Hemispheres, Absence, Congenital
Congenital absence of cerebellum
PCH14 - pontocerebellar hypoplasia type 14
Cerebellar vermis hypoplasia, oligophrenia, congenital ataxia, coloboma, hepatic fibrosis
Lissencephaly type 2 without muscular or ocular involvement
Aicardi syndrome
X-linked lissencephaly with ambiguous genitalia
Oligophrenin-1 syndrome
Congenital porencephaly
Hereditary disorder of fetus
Curatolo Cilio Pessagno syndrome
Polymicrogyria
PCH8 - pontocerebellar hypoplasia type 8
Congenital pontocerebellar hypoplasia type 7
Lissencephaly with cerebellar hypoplasia type B
Jeune thoracic dysplasia
Lissencephaly pachygyria
Macroencephaly
Neonatal hypotonia
agyria
Fatal infantile encephalopathy with olivopontocerebellar hypoplasia
Abnormality of neurogenesis
Congenital aplasia of part of brain
Macrogyrias
PAFAH1B1 (platelet activating factor acetylhydrolase 1b regulatory subunit 1) related lissencephaly
Congenital pontocerebellar hypoplasia type 10
Joubert syndrome with oro-facial-digital syndrome
Hemispheric cerebral agenesis
Micromelia
Congenital pontocerebellar hypoplasia type 9
Multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, hydranencephaly syndrome
Intellectual disability, coarse face, macrocephaly, cerebellar hypotrophy syndrome
White matter hypoplasia, corpus callosum agenesis, intellectual disability syndrome
Congenital pontocerebellar hypoplasia
Congenital hypoplasia of part of brain (disorder)
COACH syndrome
Megalencephaly
Zaki Gleeson syndrome
Atelencephaly
Rhombencephalosynapsis
Pachygyria, intellectual disability, epilepsy syndrome
OPHN1 syndrome
Microlissencephaly
Congenital Absence of Cerebral Hemispheres
Congenital hepatic fibrosis
Megalencephaly, polymicrogyria, postaxial polydactyly, hydrocephalus syndrome
CLP1 (cleavage and polyadenylation factor I subunit 1) related pontocerebellar hypoplasia
Congenital hypoplasia of ventral portion of pons
PCH13 - pontocerebellar hypoplasia type 13
Congenital hypoplasia of cerebrum
Agenesis of part of brain
Joubert syndrome with retinopathy
Joubert syndrome with orofaciodigital defect
Hypoplasia of brain gyri
Foetus with hereditary disease
Familial juvenile nephronophthisis
Fatal pontocerebellar hypoplasia, hypotonia, respiratory distress syndrome
Hydrancephaly
XK syndrome
Microcephaly, polymicrogyria, corpus callosum agenesis syndrome
Porencephaly, cerebellar hypoplasia, internal malformations syndrome
Complete agenesis of vermis
Aprosencephaly/atelencephaly spectrum
Familial aplasia of the vermis
Cerebello-oculo-renal syndrome
Congenital pontocerebellar hypoplasia type 11
Aicardi's syndrome
Endosteal hyperostoses with cerebellar hypoplasia
Dysgenesis of the brainstem
Familial juvenile medullary cystic kidney disease
Absence of Cerebral Hemispheres, Congenital
NDE1-related microhydranencephaly
Lissencephaly syndrome Norman Roberts type
Congenital hypoplasia of spinal cord
CIMDAG (cerebellar hypoplasia, intellectual disability, congenital microcephaly, dystonia, anaemia, growth retardation) syndrome
Pachygyrias
Lissencephaly type 2 without muscular or eye involvement
Varadi syndrome
Partial absence of septum pellucidum
Lissencephaly with cerebellar hypoplasia type F
Absence of septum pellucidum
Congenital hypoplasia of inferior vermis
Autosomal recessive spinocerebellar ataxia type 20
Agenesis of cerebellum
Cerebellar atrophy with progressive microcephaly
Cerebellar hypoplasia
Occipital pachygyria and polymicrogyria
Joubert syndrome with ocular defect
Fatal infantile encephalopathy with mitochondrial respiratory chain defect
Joubert syndrome with JATD (Jeune asphyxiating thoracic dystrophy)
Broad Gyri of Cerebrum
Atelomyelia
Combined malformation of central nervous system and skeletal muscle
Occipital MCD (malformation of cortical development)
Pontocerebellar hypoplasia type 5
Lissencephaly due to tubulin alpha 1A mutation
Joubert syndrome with congenital hepatic fibrosis
Congenital pontocerebellar hypoplasia type 2
CIMDAG (cerebellar hypoplasia, intellectual disability, congenital microcephaly, dystonia, anemia, growth retardation) syndrome
Lissencephaly with cerebellar hypoplasia type C
Anomalies of hypothalamus
Dysgenesis of the cerebellum
Gentile syndrome
X-linked lissencephaly with abnormal genitalia syndrome
PCH11 - pontocerebellar hypoplasia type 11
Pontocerebellar hypoplasia type 3
Hydranencephalies
Pontocerebellar hypoplasia due to TBC1D23 mutation
Microlissencephaly micromelia syndrome
Congenital pontocerebellar hypoplasia type 12
PCH3 - pontocerebellar hypoplasia type 3
PCH7 - pontocerebellar hypoplasia type 7
Lissencephaly due to LIS1 mutation
Schizencephalic porencephaly
Permanent neonatal diabetes mellitus
Lissencephaly type 3 familial fetal akinesia sequence syndrome
Agenesis of cerebrum
Aplasia of cerebellum
Isolated bilateral hemispheric cerebellar hypoplasia
Lissencephalia
CDH - Congenital diaphragmatic hernia
Arachnoid cyst
Congenital diaphragmatic hernia
Agenesis of left hemisphere of cerebellum
AIC - Aicardi syndrome
Cerebellar hypoplasia, intellectual disability, congenital microcephaly, dystonia, anemia, growth retardation syndrome

Frequently Asked Questions

What is the ICD-10 code for other reduction deformities of brain?

The ICD-10-CM code for other reduction deformities of brain is Q04.3. The full clinical description is "Other reduction deformities of brain". Q04.3 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code Q04.3 mean?

ICD-10-CM code Q04.3 represents “Other reduction deformities of brain”. It is classified under Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities and is a billable/specific code that can be used on a claim.

Is Q04.3 a billable code?

Yes, Q04.3 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is Q04.3 in?

Q04.3 is in Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (codes Q00-Q99).

What codes cannot be used with Q04.3?

Q04.3 has Excludes1 notes indicating codes that cannot be used together with it, including: cyclopia (Q87.0); macrocephaly (Q75.3); congenital malformations of corpus callosum (Q04.0).

What SNOMED CT codes does Q04.3 map to?

Q04.3 maps to 156 SNOMED CT concepts: 80651009, 253128003, 253143001, 253172000, 715990006, and 151 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for Q04.3?

Q04.3 is linked to 8 UMLS Concept Unique Identifiers: C0020225, C0266461, C1879312, C0266483, C0266462, and 3 more. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does Q04.3 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like other reduction deformities of brain affect a person's functioning: body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of Q04.3?

Q04.3 maps to the ICD-11 code: LA05.Z (Cerebral structural developmental anomalies, unspecified).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.