2024 ICD-10-CM Diagnosis Code Q43.1

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Aganglionosis of Auerbach's plexus
• Aganglionosis of colon
• Aganglionosis of large intestine
• Central alveolar hypoventilation syndrome
• Congenital aganglionic megacolon
• Congenital central hypoventilation
• Congenital dilatation of colon
• Congenital dilatation of colon
• Congenital dilatation of colon
• Congenital dilatation of colon
• Congenital dilatation of colon
• Extensive aganglionosis Hirschsprung disease
• Goldberg Shprintzen megacolon syndrome
• Haddad syndrome
• Hirschsprung disease of rectosigmoid region
• Hirschsprung disease with deafness and polydactyly syndrome
• Hirschsprung disease with nail hypoplasia and dysmorphism
• Hirschsprung disease with type D brachydactyly syndrome
• Hirschsprung disease, ganglioneuroblastoma syndrome
• Long segment Hirschsprung's disease
• Mowat-Wilson syndrome
• Mowat-Wilson syndrome
• Mowat-Wilson syndrome due to monosomy 2q22
• Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease
• Secondary megacolon - congenital
• Short segment Hirschsprung's disease
• Total colonic aganglionosis
• Total intestinal aganglionosis
• Waardenburg Shah syndrome
• Waardenburg syndrome
• Waardenburg syndrome

Clinical Information

• Waardenburg Syndrome

  rare, autosomal dominant disease with variable penetrance and several known clinical types. characteristics may include depigmentation of the hair and skin, congenital deafness, heterochromia iridis, medial eyebrow hyperplasia, hypertrophy of the nasal root, and especially dystopia canthorum. the underlying cause may be defective development of the neural crest (neurocristopathy). waardenburg's syndrome may be closely related to piebaldism. klein-waardenburg syndrome refers to a disorder that also includes upper limb abnormalities.

• Total Intestinal Aganglionosis

  a complete lack of ganglia in the intestine. this is an extremely severe form of hirschsprung disease.

Q43.1 is grouped with Diagnostic Related Groups - MS-DRG Mapping

Diagnostic Related Groups (DRGs) are a classification system used to group together diagnosis codes for the purpose of reimbursement and healthcare management. DRGs are used to standardize the way hospitals and other providers are paid for inpatient services. In this system patients are grouped together based on their principal diagnosis in areas referred to as Major Diagnostic Categories (MDC). Once a patient is assigned a particular diagnostic category, providers receive a predetermined payment rate for the services rendered. This reimbursement rate is often fixed and is meant to cover the cost of care for that patient. Reimbursement is also affected by the relative weight of a diagnostic related group based on the severity of a patient's illness and the associated cost of care during hospitalization.

Diagnostic related groups encourage healthcare providers to focus on quality and efficiency in patient care while managing costs effectively. The diagnosis code is present in the following groups for version MS-DRG V41.0 applicable from 10/01/2023 through 09/30/2024.

MS-DRG	MS-DRG Title	MCD	Relative Weight
393	OTHER DIGESTIVE SYSTEM DIAGNOSES WITH MCC	06	1.6196
394	OTHER DIGESTIVE SYSTEM DIAGNOSES WITH CC	06	0.9369
395	OTHER DIGESTIVE SYSTEM DIAGNOSES WITHOUT CC/MCC	06	0.6475

The relative weight of a diagnostic related group determines the reimbursement rate based on the severity of a patient's illness and the associated cost of care during hospitalization.

Present on Admission (POA)

Q43.1 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA Indicator	Reason for Code	CMS will pay the CC/MCC DRG?
Y	Diagnosis was present at time of inpatient admission.	YES
N	Diagnosis was not present at time of inpatient admission.	NO
U	Documentation insufficient to determine if the condition was present at the time of inpatient admission.	NO
W	Clinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.	YES
1	Unreported/Not used - Exempt from POA reporting.	NO

Convert Q43.1 to ICD-9-CM

• ICD-9-CM Code: 751.3 - Hirschsprung's disease
  Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Colonic Diseases

Your colon, also known as the large intestine, is part of your digestive system. It's a long, hollow tube at the end of your digestive tract where your body makes and stores stool. Many disorders affect the colon's ability to work properly. Some of these include:

• Colorectal cancer
• Colonic polyps - extra tissue growing in the colon that can become cancerous
• Ulcerative colitis - ulcers of the colon and rectum
• Diverticulitis - inflammation or infection of pouches in the colon
• Irritable bowel syndrome - an uncomfortable condition causing abdominal cramping and other symptoms

Treatment for colonic diseases varies greatly depending on the disease and its severity. Treatment may involve diet, medicines and in some cases, surgery.

NIH: National Institute of Diabetes and Digestive and Kidney Diseases

[Learn More in MedlinePlus]

Hirschsprung disease

Hirschsprung disease is an intestinal disorder characterized by the absence of nerves in parts of the intestine. This condition occurs when the nerves in the intestine (enteric nerves) do not form properly during development before birth (embryonic development). This condition is usually identified in the first two months of life, although less severe cases may be diagnosed later in childhood.

Enteric nerves trigger the muscle contractions that move stool through the intestine. Without these nerves in parts of the intestine, the material cannot be pushed through, causing severe constipation or complete blockage of the intestine in people with Hirschsprung disease. Other signs and symptoms of this condition include vomiting, abdominal pain or swelling, diarrhea, poor feeding, malnutrition, and slow growth. People with this disorder are at risk of developing more serious conditions such as inflammation of the intestine (enterocolitis) or a hole in the wall of the intestine (intestinal perforation), which can cause serious infection and may be fatal.

There are two main types of Hirschsprung disease, known as short-segment disease and long-segment disease, which are defined by the region of the intestine lacking nerve cells. In short-segment disease, nerve cells are missing from only the last segment of the large intestine (colon). This type is most common, occurring in approximately 80 percent of people with Hirschsprung disease. For unknown reasons, short-segment disease is four times more common in men than in women. Long-segment disease occurs when nerve cells are missing from most of the large intestine and is the more severe type. Long-segment disease is found in approximately 20 percent of people with Hirschsprung disease and affects men and women equally. Very rarely, nerve cells are missing from the entire large intestine and sometimes part of the small intestine (total colonic aganglionosis) or from all of the large and small intestine (total intestinal aganglionosis).

Hirschsprung disease can occur in combination with other conditions, such as Waardenburg syndrome, type IV; Mowat-Wilson syndrome; or congenital central hypoventilation syndrome. These cases are described as syndromic. Hirschsprung disease can also occur without other conditions, and these cases are referred to as isolated or nonsyndromic.

[Learn More in MedlinePlus]

Hirschsprung Disease

Overview of Hirschsprung disease, a birth defect in which some nerve cells are missing in the large intestine. Discusses symptoms, diagnosis, and treatment.
[Learn More in MedlinePlus]

Code History

• FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.