2020 ICD-10-CM Diagnosis Code Q43.1

Hirschsprung's disease

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q43.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM Q43.1 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q43.1 - other international versions of ICD-10 Q43.1 may differ.
Applicable To
  • Aganglionosis
  • Congenital (aganglionic) megacolon
The following code(s) above Q43.1 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q43.1:
  • Q00-Q99
    2020 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    Note
    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    Congenital malformations, deformations and chromosomal abnormalities
Approximate Synonyms
  • Congenital megacolon
  • Hirschsprungs disease
  • Megacolon, congenital
Clinical Information
  • A congenital disorder characterized by the absence of myenteric ganglion cells in the distal colon. It results in a functional stenosis of the distal colon and a massive distention of the proximal colon.
  • Abnormally large or dilated colon due to congenital absence of myenteric ganglion cells in a distal segment of the large bowel; resultant loss of motor function in this segment causes massive hypertrophic dilatation of the normal proximal colon; condition appears soon after birth; called also hirschsprung's disease, aganglionic megacolon and pelvirectal achalasia.
  • Congenital megacolon resulting from the absence of ganglion cells (aganglionosis) in a distal segment of the large intestine. The aganglionic segment is permanently contracted thus causing dilatation proximal to it. In most cases, the aganglionic segment is within the rectum and sigmoid colon.
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q43.1 is considered exempt from POA reporting.
ICD-10-CM Q43.1 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 393 Other digestive system diagnoses with mcc
  • 394 Other digestive system diagnoses with cc
  • 395 Other digestive system diagnoses without cc/mcc

Convert Q43.1 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to Q43.1:
  • Type 1 Excludes: K59.3
    , K59.3
    , P76.1
    ICD-10-CM Diagnosis Code K59.3

    Megacolon, not elsewhere classified

      2016 2017 - Converted to Parent Code 2018 2019 2020 Non-Billable/Non-Specific Code
    Applicable To
    • Dilatation of colon
    Code First
    • , if applicable (T51-T65) to identify toxic agent
    Type 1 Excludes
    • congenital megacolon (aganglionic) (Q43.1)
    • megacolon (due to) (in) Chagas' disease (B57.32)
    • megacolon (due to) (in) Clostridium difficile (A04.7-)
    • megacolon (due to) (in) Hirschsprung's disease (Q43.1)
    ICD-10-CM Diagnosis Code K59.3

    Megacolon, not elsewhere classified

      2016 2017 - Converted to Parent Code 2018 2019 2020 Non-Billable/Non-Specific Code
    Applicable To
    • Dilatation of colon
    Code First
    • , if applicable (T51-T65) to identify toxic agent
    Type 1 Excludes
    • congenital megacolon (aganglionic) (Q43.1)
    • megacolon (due to) (in) Chagas' disease (B57.32)
    • megacolon (due to) (in) Clostridium difficile (A04.7-)
    • megacolon (due to) (in) Hirschsprung's disease (Q43.1)
    ICD-10-CM Diagnosis Code P76.1

    Transitory ileus of newborn

      2016 2017 2018 2019 2020 Billable/Specific Code Code on Newborn Record
    Type 1 Excludes
    • Hirschsprung's disease (Q43.1)

Diagnosis Index entries containing back-references to Q43.1:
  • Aganglionosis Q43.1 (bowel) (colon)
  • Dilatation
    • colon K59.39
      ICD-10-CM Diagnosis Code K59.39

      Other megacolon

        2017 - New Code 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Megacolon NOS
      • congenital Q43.1
  • Hirschsprung's disease or megacolon Q43.1
  • Ileus (bowel) (colon) (inhibitory) (intestine) K56.7
    ICD-10-CM Diagnosis Code K56.7

    Ileus, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    Type 1 Excludes
    Type 2 Excludes
    • intestinal obstruction with hernia (K40-K46)
    • neurogenic K56.0
      ICD-10-CM Diagnosis Code K56.0

      Paralytic ileus

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Paralysis of bowel
      • Paralysis of colon
      • Paralysis of intestine
      Type 1 Excludes
      • Hirschsprung's disease or megacolon Q43.1
  • Macrocolon Q43.1 - see also Megacolon
  • Megacolon (acquired) (functional) (not Hirschsprung's disease) (in) K59.39
    ICD-10-CM Diagnosis Code K59.39

    Other megacolon

      2017 - New Code 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Megacolon NOS
    • congenital, congenitum Q43.1 (aganglionic)
    • Hirschsprung's Q43.1 (disease)
  • Obstruction, obstructed, obstructive
    • intestine K56.609
      ICD-10-CM Diagnosis Code K56.609

      Unspecified intestinal obstruction, unspecified as to partial versus complete obstruction

        2018 - New Code 2019 2020 Billable/Specific Code
      Applicable To
      • Intestinal obstruction NOS
      • neurogenic K56.0
        ICD-10-CM Diagnosis Code K56.0

        Paralytic ileus

          2016 2017 2018 2019 2020 Billable/Specific Code
        Applicable To
        • Paralysis of bowel
        • Paralysis of colon
        • Paralysis of intestine
        Type 1 Excludes
        • Hirschsprung's disease or megacolon Q43.1

ICD-10-CM Codes Adjacent To Q43.1
Q41.9 Congenital absence, atresia and stenosis of small intestine, part unspecified
Q42 Congenital absence, atresia and stenosis of large intestine
Q42.0 Congenital absence, atresia and stenosis of rectum with fistula
Q42.1 Congenital absence, atresia and stenosis of rectum without fistula
Q42.2 Congenital absence, atresia and stenosis of anus with fistula
Q42.3 Congenital absence, atresia and stenosis of anus without fistula
Q42.8 Congenital absence, atresia and stenosis of other parts of large intestine
Q42.9 Congenital absence, atresia and stenosis of large intestine, part unspecified
Q43 Other congenital malformations of intestine
Q43.0 Meckel's diverticulum (displaced) (hypertrophic)
Q43.1 Hirschsprung's disease
Q43.2 Other congenital functional disorders of colon
Q43.3 Congenital malformations of intestinal fixation
Q43.4 Duplication of intestine
Q43.5 Ectopic anus
Q43.6 Congenital fistula of rectum and anus
Q43.7 Persistent cloaca
Q43.8 Other specified congenital malformations of intestine
Q43.9 Congenital malformation of intestine, unspecified
Q44 Congenital malformations of gallbladder, bile ducts and liver
Q44.0 Agenesis, aplasia and hypoplasia of gallbladder

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.