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    v1.0.28
    OpenPayerBilling CodesOther congenital malformations of the digestive systemICD-10-CM q42-congenital-large-intestine-malformations
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    ICD-10-CM Code For Congenital Absence, Atresia And Stenosis Of Large Intestine: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q42 includes congenital absence, atresia, and stenosis of the large intestine, covering diagnoses where segments of the colon or rectum are missing, occluded, or narrowed. These ICD-10-CM diagnosis codes are used to document neonatal intestinal obstruction, surgical repairs, and associated anatomic involvement such as specific colon segments or rectal anomalies. Accurate coding within the ICD-10-CM Q42 group supports proper claim adjudication and complete clinical documentation for appropriate reimbursement.

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    ICD-10-CM Q42: Congenital Absence, Atresia And Stenosis Of Large Intestine Overview

    This group includes congenital malformations of the large intestine such as absence, atresia, and stenosis that impair bowel continuity or lumen caliber. These conditions affect the digestive system and typically present in neonates or infants with bowel obstruction or failure to pass meconium. Accurate coding is important for appropriate clinical documentation and supports reimbursement processes tied to surgical and inpatient care.

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    When to Use

    Use a code from this group when a congenital absence, atresia, or stenosis of the large intestine is diagnosed clinically or confirmed by imaging or surgery. Codes are applied to document the specific anatomic site and to support coding for surgical repair, neonatal care, and related complications.

    ICD-10-CM Codes

    6 rows

    ICD-10-CM CodeDescription
    Q42.0Congenital absence, atresia and stenosis of rectum with fistula
    Q42.1Congenital absence, atresia and stenosis of rectum without fistula
    Q42.2Congenital absence, atresia and stenosis of anus with fistula
    Q42.3Congenital absence, atresia and stenosis of anus without fistula
    Q42.8Congenital absence, atresia and stenosis of other parts of large intestine
    Q42.9Congenital absence, atresia and stenosis of large intestine, part unspecified

    DRG Codes

    3 rows

    DRG CodeDescription
    393Other Digestive System Diagnoses with MCC
    394Other Digestive System Diagnoses with CC
    395Other Digestive System Diagnoses without CC/MCC

    Typical Clinical Scenarios

    • A full-term neonate born with failure to pass meconium in the first 24–48 hours, progressive abdominal distension, bilious vomiting, and inability to tolerate feeds; radiographs or contrast studies suggest distal large bowel obstruction due to absence of a segment of colon identified on imaging or at surgery. A congenital agenesis or aplasia of a portion of the colon is documented intraoperatively, and a code from this group is assigned for the congenital absence. Typical codes: Q42.0, Q42.1
    • An infant with chronic constipation since birth, poor weight gain, and recurrent enterocolitis episodes who undergoes diagnostic evaluation including contrast enema and rectal biopsy showing a short segment congenital atresia or stenosis of the large intestine causing partial obstruction; surgical repair or resection is planned. The congenital atresia/stenosis of the colon explains the longstanding obstructive symptoms and is coded from this group. Typical codes: Q42.2, Q42.3
    • An adolescent or adult with a history of previously repaired congenital large bowel malformation presenting with new symptoms of bowel obstruction or chronic abdominal pain due to residual or unrecognized congenital anomaly (for example, a congenital colonic stenosis discovered during workup for chronic obstructive symptoms or found incidentally during surgery for complications). The encounter documents a congenital malformation of the large intestine as the underlying cause or comorbidity, and an appropriate code from this group is used. Typical codes: Q42.8, Q42.9

    Related Codes

    Q38-OTHER-CONGENITAL-MALFORMATIONS-TONGUE-MOUTH-PHARYNX

    ICD-10-CM Code For Other Congenital Malformations Of Tongue Mouth And Pharynx: Group Overview

    Q39-CONGENITAL-MALFORMATIONS-OF-ESOPHAGUS

    ICD-10-CM Code For Congenital Malformations Of Esophagus: Group Overview

    Q40-OTHER-CONGENITAL-MALFORMATIONS-UPPER-ALIMENTARY-TRACT

    ICD-10-CM Code For Other Congenital Malformations Of Upper Alimentary Tract: Group Overview

    Q41-CONGENITAL-ABSENCE-ATRESIA-STENOSIS-SMALL-INTESTINE

    ICD-10-CM Code For Congenital Absence, Atresia And Stenosis Of Small Intestine: Group Overview

    Q43-OTHER-CONGENITAL-MALFORMATIONS-OF-INTESTINE

    ICD-10-CM Code For Other Congenital Malformations Of Intestine: Group Overview

    Q44-CONGENITAL-MALFORMATIONS-OF-GALLBLADDER

    ICD-10-CM Code For Congenital Malformations Of Gallbladder, Bile Ducts And Liver: Group Overview

    Q45-OTHER-CONGENITAL-MALFORMATIONS-DIGESTIVE

    ICD-10-CM Code For Other Congenital Malformations Of Digestive System: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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