Specific code icon 2014 ICD-10-CM Diagnosis Code Q43.1

Hirschsprung's disease

  • Q43.1 is a billable ICD-10-CM code that can be used to specify a diagnosis.
  • On October 1, 2014 ICD-10-CM will replace ICD-9-CM in the United States, therefore, Q43.1 and all other ICD-10-CM codes should only be used for training or planning purposes until then.
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.
Applicable To
  • Aganglionosis
  • Congenital (aganglionic) megacolon
Description Synonyms
  • Congenital megacolon
  • Hirschsprungs disease
  • Megacolon, congenital
Present On Admission
  • Q43.1 is considered exempt from POA reporting.
Mortality Data
  • Between 1999-2007 there were 183 deaths in the United States where ICD-10 Q43.1 was indicated as the underlying cause of death [source: cdc.govlink to cdc.gov]
  • ICD-10 Q43.1 as underlying cause of death data broken down by: gender, age, race, year
ICD-10-CM Q43.1 is grouped within Diagnostic Related Group(s) (MS-DRG v30.0):
  • 393 Other digestive system diagnoses with mcc
  • 394 Other digestive system diagnoses with cc
  • 395 Other digestive system diagnoses without cc/mcc
Convert ICD-10-CM Q43.1 to ICD-9-CM
The following ICD-10-CM Index entries contain back-references to ICD-10-CM Q43.1: