Q40-Q45
Medium Complexity

Other congenital malformations of the digestive system

Primary Specialty: Pediatrics
Last Updated: 2025-09-10

ICD-10 Codes (43)

40 billable
0 category headers
Q41
Congenital absence, atresia and stenosis of small intestine
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Q41.0
Billable
Congenital absence, atresia and stenosis of duodenum
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Q41.1
Billable
Congenital absence, atresia and stenosis of jejunum
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Q41.2
Billable
Congenital absence, atresia and stenosis of ileum
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Q41.8
Billable
Congenital absence, atresia and stenosis of other specified parts of small intestine
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Q41.9
Billable
Congenital absence, atresia and stenosis of small intestine, part unspecified
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Q42
Billable
Congenital absence, atresia and stenosis of large intestine
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Q42.0
Billable
Congenital absence, atresia and stenosis of rectum with fistula
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Q42.1
Billable
Congenital absence, atresia and stenosis of rectum without fistula
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Q42.2
Billable
Congenital absence, atresia and stenosis of anus with fistula
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Q42.3
Billable
Congenital absence, atresia and stenosis of anus without fistula
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Q42.8
Billable
Congenital absence, atresia and stenosis of other parts of large intestine
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Q42.9
Billable
Congenital absence, atresia and stenosis of large intestine, part unspecified
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Q43
Billable
Other congenital malformations of intestine
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Q43.0
Billable
Meckel's diverticulum (displaced) (hypertrophic)
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Q43.1
Billable
Hirschsprung's disease
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Q43.2
Billable
Other congenital functional disorders of colon
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Q43.3
Billable
Congenital malformations of intestinal fixation
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Q43.4
Billable
Duplication of intestine
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Q43.5
Billable
Ectopic anus
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Q43.6
Billable
Congenital fistula of rectum and anus
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Q43.7
Billable
Persistent cloaca
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Q43.8
Billable
Other specified congenital malformations of intestine
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Q43.9
Billable
Congenital malformation of intestine, unspecified
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Q44
Congenital malformations of gallbladder, bile ducts and liver
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Q44.0
Billable
Agenesis, aplasia and hypoplasia of gallbladder
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Q44.1
Billable
Other congenital malformations of gallbladder
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Q44.2
Billable
Atresia of bile ducts
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Q44.3
Billable
Congenital stenosis and stricture of bile ducts
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Q44.4
Billable
Choledochal cyst
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Q44.5
Billable
Other congenital malformations of bile ducts
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Q44.6
Billable
Cystic disease of liver
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Q44.7
Billable
Other congenital malformations of liver
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Q44.70
Billable
Other congenital malformation of liver, unspecified
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Q44.71
Billable
Alagille syndrome
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Q44.79
Billable
Other congenital malformations of liver
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Q45
Other congenital malformations of digestive system
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Q45.0
Billable
Agenesis, aplasia and hypoplasia of pancreas
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Q45.1
Billable
Annular pancreas
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Q45.2
Billable
Congenital pancreatic cyst
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Q45.3
Billable
Other congenital malformations of pancreas and pancreatic duct
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Q45.8
Billable
Other specified congenital malformations of digestive system
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Q45.9
Billable
Congenital malformation of digestive system, unspecified
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Updates & Changes

FY 2026 Updates

Current Year

New Codes (1)

Q23.4
Hypoplastic left heart syndrome with mitral atresia

Revised Codes (1)

Q21.0
Ventricular septal defect - updated to include hemodynamic significance when known

Deleted Codes

No codes deleted in this range for FY 2026

Historical Changes

  • •FY 2025: Routine maintenance updates with minor terminology clarifications
  • •FY 2024: Enhanced specificity requirements for certain code ranges
  • •FY 2023: Updated documentation guidelines for improved clarity

Upcoming Changes

  • •Proposed updates pending review by Coordination and Maintenance Committee
  • •Under consideration: Enhanced digital health integration codes

Implementation Guidance

  • •Review all FY 2026 updates for Q40-Q45 codes before implementation
  • •Always verify the most current codes in the ICD-10-CM manual
  • •Ensure clinical documentation supports the selected diagnosis codes
  • +3 more guidance items...

Range Overview

high priority

The ICD-10 code range Q40-Q45 pertains to Other Congenital Malformations of the Digestive System. This includes a variety of congenital anomalies, deformities, and chromosomal abnormalities that affect the digestive system. These codes are used to document conditions such as congenital malformations of the esophagus, stomach, intestines, and other parts of the digestive tract present at birth.

Key Usage Points:

  • •These codes are used to document congenital malformations of the digestive system.
  • •Each code within the range specifies a different part of the digestive system.
  • •The codes can be used in any healthcare setting where the patient's condition is being documented.
  • •The codes are typically used by pediatricians, gastroenterologists, and general practitioners.
  • •Always code to the highest level of specificity.

Coding Guidelines

When to Use:

  • ✓When a patient is diagnosed with a congenital malformation of the digestive system.
  • ✓When a patient's congenital digestive system malformation is relevant to their current medical care.
  • ✓When a patient's congenital digestive system malformation impacts their treatment plan.
  • ✓When a patient is being referred for further evaluation of a suspected congenital digestive system malformation.

When NOT to Use:

  • ✗When the patient has a digestive system disorder, but it is not congenital.
  • ✗When the patient's digestive system condition is acquired, not congenital.
  • ✗When the patient's condition is related to the digestive system but is not a malformation.
  • ✗When the patient's condition has been previously documented and is not relevant to the current care.

Code Exclusions

Always verify exclusions with the patient's medical record and the latest ICD-10 coding guidelines.

Documentation Requirements

Proper documentation for these codes requires detailed medical records that specify the type of congenital malformation, the part of the digestive system affected, and how the condition impacts the patient's health and treatment plan.

Clinical Information:

  • •Specific type of congenital malformation
  • •Part of the digestive system affected
  • •Impact on patient's health
  • •Relevant treatment plan
  • •Any related symptoms or complications

Supporting Evidence:

  • •Medical history
  • •Physical examination findings
  • •Imaging results
  • •Surgical reports
Good Documentation Example:

Patient diagnosed with congenital malformation of the esophagus (Q40.0). The condition causes difficulty swallowing and has necessitated a modified diet.

Poor Documentation Example:

Patient has a congenital condition.

Common Documentation Errors:

  • âš Not specifying the type of malformation
  • âš Not indicating the part of the digestive system affected
  • âš Not documenting the impact on the patient's health
  • âš Not including supporting evidence

Range Statistics

8
Total Codes
40
Billable
Complexity:
Medium
Primary Use:Clinical Documentation
Chapter:17

Coding Complexity

Medium
Complexity Rating

Coding for congenital malformations of the digestive system requires a good understanding of the anatomy and a careful review of the patient's medical records. The complexity is considered medium because while the codes are specific, they require a detailed understanding of the condition and its impact on the patient's health.

Key Factors:
  • â–¸Understanding the anatomy of the digestive system
  • â–¸Differentiating between congenital and acquired conditions
  • â–¸Identifying the specific type of malformation
  • â–¸Coding to the highest level of specificity
  • â–¸Keeping up-to-date with changes in coding guidelines

Specialty Focus

These codes are primarily used in pediatrics, gastroenterology, and general practice. They are used to document congenital malformations of the digestive system in patients of all ages.

Primary Specialties:
Pediatrics
40%
Gastroenterology
35%
General Practice
25%
Clinical Scenarios:
  • • A newborn diagnosed with a congenital malformation of the stomach.
  • • A toddler with a congenital malformation of the intestines causing chronic constipation.
  • • A teenager with a congenital malformation of the esophagus causing difficulty swallowing.
  • • An adult with a congenital malformation of the digestive system causing recurring abdominal pain.

Resources & References

There are several resources available for coding congenital malformations of the digestive system. These include the official ICD-10 guidelines, clinical reference materials, and educational resources.

Official Guidelines:

  • ICD-10-CM Official Guidelines for Coding and Reporting
  • American Health Information Management Association (AHIMA) guidelines
  • American Academy of Professional Coders (AAPC) guidelines

Clinical References:

  • American Academy of Pediatrics (AAP) clinical practice guidelines
  • American Gastroenterological Association (AGA) clinical practice guidelines

Educational Materials:

  • AAPC ICD-10 training materials
  • AHIMA ICD-10 educational resources

Frequently Asked Questions

Can I use a Q40-Q45 code for an acquired malformation of the digestive system?

No, these codes are specifically for congenital malformations. Acquired malformations should be coded using the appropriate code from the K00-K95 range.