Q43.1
BillableHirschsprung's disease
Hirschsprung's disease
Coding Notes
Inclusion Terms
Alternative clinical terms for this condition
- Aganglionosis
- Congenital (aganglionic) megacolon
Excludes 2
Conditions not included here, but the patient may have both
Related Codes(9)
Q43.0Meckel's diverticulum (displaced) (hypertrophic)
Q43.2Other congenital functional disorders of colon
Q43.3Congenital malformations of intestinal fixation
Q43.4Duplication of intestine
Q43.5Ectopic anus
Q43.6Congenital fistula of rectum and anus
Q43.7Persistent cloaca
Q43.8Other specified congenital malformations of intestine
Q43.9Congenital malformation of intestine, unspecified
ICD-11 Equivalents(1)
ICD-11 Equivalents
View full mappingCorresponding ICD-11 codes from the WHO crosswalk mapping
Also Known As / Clinical Terms(186)
SNOMED CT
- Congenital dilatation of colon24291004
- Congenital megacolon24291004
- Central alveolar hypoventilation syndrome24825006
- Waardenburg syndrome47434006
- Waardenburg's syndrome47434006
- Secondary congenital megacolon197140007
- Secondary megacolon - congenital197140007
- Aganglionic megacolon204739008
- Aganglionosis204739008
- Congenital aganglionic megacolon204739008
- HD - Hirschsprung's disease204739008
- Hirschsprung disease204739008
- Hirschsprung's disease204739008
- Long segment Hirschsprung disease204740005
- Long segment Hirschsprung's disease204740005
- Short segment Hirschsprung disease204741009
- Short segment Hirschsprung's disease204741009
- Total intestinal aganglionosis204745000
- TCA - Total colonic aganglionosis253780003
- Total colonic aganglionosis253780003
- Aganglionosis of Auerbach plexus360434004
- Aganglionosis of Auerbach's plexus360434004
- Aganglionosis of colon360441005
- CCHS - Congenital central hypoventilation399040002
- Congenital central hypoventilation399040002
- Congenital pulmonary hypoventilation399040002
- Ondine curse399040002
- Hirschsprung disease-intellectual disability syndrome703535000
- Hirschsprung disease-mental retardation syndrome703535000
- Mowat-Wilson syndrome703535000
- Shah Waardenburg syndrome715952000
- Waardenburg Hirschsprung syndrome715952000
- Waardenburg Shah syndrome715952000
- Waardenburg syndrome co-occurrent with Hirschsprung disease715952000
- Waardenburg syndrome type 4715952000
- Goldberg Shprintzen megacolon syndrome717822006
- Megacolon microcephaly syndrome717822006
- Congenital central alveolar hypoventilation with Hirschsprung disease syndrome719972004
- Haddad syndrome719972004
- Ondine Hirschsprung disease719972004
- Ondine Hirschsprung syndrome719972004
- Hirschsprung disease with deafness and polydactyly syndrome721221000
- Santos Mateus Leal syndrome721221000
- Hirschsprung disease with type D brachydactyly syndrome721222007
- Al Gazali Donnai Muller syndrome721223002
- Hirschsprung disease with nail hypoplasia and dysmorphism721223002
- Hirschsprung disease of rectosigmoid region721608001
- Rectosigmoid Hirschsprung disease721608001
- Extensive aganglionosis Hirschsprung disease721609009
- Aganglionosis of large intestine723183004
- Neurologic Waardenburg Shah syndrome765325002
- PCWH - peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease765325002
- Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease765325002
- Hirschsprung disease, ganglioneuroblastoma syndrome783737007
- Hirschsprung disease and intellectual disability due to 2q22 microdeletion890118006
- Hirschsprung disease and intellectual disability due to del(2)(q22)890118006
- Hirschsprung disease and intellectual disability due to monosomy 2q22890118006
- Mowat-Wilson syndrome due to 2q22 microdeletion890118006
- Mowat-Wilson syndrome due to del(2)q(22)890118006
- Mowat-Wilson syndrome due to monosomy 2q22890118006
- Aganglionosis of small intestine1142282000
- Congenital aganglionosis of small intestine1142282000
- Congenital hypoplasia of nail unit1144442009
UMLS
- Aganglionic MegacolonC0019569
- Aganglionic megacolonC0019569
- AganglionosisC0019569
- CONGEN MEGACOLONC0019569
- Congenital (aganglionic) megacolonC0019569
- Congenital MegacolonC0019569
- Congenital aganglionic megacolonC0019569
- Congenital aganglionic megacolon (disorder)C0019569
- Congenital intestinal aganglionosisC0019569
- Congenital megacolonC0019569
- Disease, HirschsprungC0019569
- Disease, Hirschsprung'sC0019569
- Enlarged colon lacking nerve cellsC0019569
- HD - Hirschsprung's diseaseC0019569
- HIRSCHSPRUNG DISC0019569
- HIRSCHSPRUNGS DISC0019569
- HSCRC0019569
- Hirschsprung DiseaseC0019569
- Hirschsprung diseaseC0019569
- Hirschsprung megacolonC0019569
- Hirschsprung's DiseaseC0019569
- Hirschsprung's diseaseC0019569
- Hirschsprungs DiseaseC0019569
- MEGACOLON CONGENC0019569
- Megacolon congenitalC0019569
- Megacolon, AganglionicC0019569
- Megacolon, CongenitalC0019569
- Pelvirectal achalasiaC0019569
- aganglionic megacolonC0019569
- aganglionosisC0019569
- congenital megacolonC0019569
- disease hirschsprungC0019569
- disease hirschsprung'sC0019569
- disease hirschsprungsC0019569
- diseases hirschsprungC0019569
- hirschsprung diseaseC0019569
- hirschsprung's diseaseC0019569
- hirschsprungs diseaseC0019569
- megacolon congenitalC0019569
- pelvirectal achalasiaC0019569
Clinical Terms
- pelvirectal achalasia
- Congenital intestinal aganglionosis
- HIRSCHSPRUNGS DIS
- Congenital aganglionosis of small intestine
- Shah Waardenburg syndrome
- Total colonic aganglionosis
- Congenital central hypoventilation
- Central alveolar hypoventilation syndrome
- Ondine Hirschsprung syndrome
- Hirschsprung disease with type D brachydactyly syndrome
- Waardenburg syndrome
- Hirschsprung megacolon
- Secondary congenital megacolon
- Aganglionosis of colon
- Disease, Hirschsprung's
- CCHS - Congenital central hypoventilation
- Hirschsprungs Disease
- MEGACOLON CONGEN
- aganglionic megacolon
- Aganglionosis of Auerbach plexus
- Waardenburg syndrome type 4
- hirschsprung disease
- Ondine curse
- Megacolon congenital
- Hirschsprung disease with nail hypoplasia and dysmorphism
- Haddad syndrome
- Short segment Hirschsprung's disease
- Al Gazali Donnai Muller syndrome
- CONGEN MEGACOLON
- HD - Hirschsprung's disease
- disease hirschsprung's
- Goldberg Shprintzen megacolon syndrome
- Congenital megacolon
- disease hirschsprungs
- Congenital (aganglionic) megacolon
- Long segment Hirschsprung disease
- Neurologic Waardenburg Shah syndrome
- TCA - Total colonic aganglionosis
- Hirschsprung disease and intellectual disability due to del(2)(q22)
- Hirschsprung disease and intellectual disability due to 2q22 microdeletion
- Megacolon, Congenital
- disease hirschsprung
- Hirschsprung disease and intellectual disability due to monosomy 2q22
- Congenital hypoplasia of nail unit
- Congenital aganglionic megacolon
- diseases hirschsprung
- Enlarged colon lacking nerve cells
- Aganglionosis of Auerbach's plexus
- Rectosigmoid Hirschsprung disease
- Waardenburg syndrome co-occurrent with Hirschsprung disease
- HIRSCHSPRUNG DIS
- Aganglionosis of small intestine
- Hirschsprung disease-mental retardation syndrome
- Waardenburg's syndrome
- Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease
- aganglionosis
- Waardenburg Hirschsprung syndrome
- Ondine Hirschsprung disease
- Disease, Hirschsprung
- Congenital central alveolar hypoventilation with Hirschsprung disease syndrome
- Extensive aganglionosis Hirschsprung disease
- Congenital pulmonary hypoventilation
- Secondary megacolon - congenital
- Mowat-Wilson syndrome due to 2q22 microdeletion
- Aganglionosis of large intestine
- Mowat-Wilson syndrome due to del(2)q(22)
- HSCR
- Hirschsprung disease of rectosigmoid region
- Megacolon microcephaly syndrome
- Mowat-Wilson syndrome due to monosomy 2q22
- Santos Mateus Leal syndrome
- Megacolon, Aganglionic
- Hirschsprung disease, ganglioneuroblastoma syndrome
- Short segment Hirschsprung disease
- Congenital aganglionic megacolon (disorder)
- PCWH - peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease
- Total intestinal aganglionosis
- Hirschsprung disease-intellectual disability syndrome
- Waardenburg Shah syndrome
- Long segment Hirschsprung's disease
- Mowat-Wilson syndrome
- Hirschsprung disease with deafness and polydactyly syndrome
- Congenital dilatation of colon
Frequently Asked Questions
What is the ICD-10 code for hirschsprung's disease?
The ICD-10-CM code for hirschsprung's disease is Q43.1. The full clinical description is "Hirschsprung's disease". Q43.1 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code Q43.1 mean?
ICD-10-CM code Q43.1 represents “Hirschsprung's disease”. 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 Q43.1 a billable code?
Yes, Q43.1 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is Q43.1 in?
Q43.1 is in Chapter 17: Congenital Malformations, Deformations and Chromosomal Abnormalities (codes Q00-Q99).
What SNOMED CT codes does Q43.1 map to?
Q43.1 maps to 27 SNOMED CT concepts: 204739008, 360434004, 360441005, 723183004, 1142282000, and 22 more. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for Q43.1?
Q43.1 is linked to 1 UMLS Concept Unique Identifier: C0019569. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does Q43.1 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like hirschsprung's disease 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 Q43.1?
Q43.1 maps to the ICD-11 code: LB16.1 (Hirschsprung disease).
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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.