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Updated on: March 16, 2016

Congenital Anomaly Coding in ICD-10-CM

Original story posted on: June 13, 2012

According to the Centers for Disease Control (CDC), in the U.S. about one in every 33 babies is born with a congenital anomaly (also called birth defects), and these babies are at a greater risk for illness and long-term disability than babies born without birth defects. Currently, congenital heart defects are the most common type of birth defect.


Congenital anomalies have a variety of causes, such as pregnancy or delivery complications, genetic malformations and infections experienced in utero. Some congenital anomalies have no known cause.

Chapter 17 of ICD-10-CM contains codes for congenital malformation, deformations and chromosomal abnormalities. Specifically, this chapter contains the following categories:

Q00 – Q07          Congenital malformations of the nervous system

Q10 – Q18          Congenital malformations of eye, ear, face and neck

Q20 – Q28          Congenital malformations of the circulatory system

Q30 – Q34          Congenital malformations of the respiratory system

Q35 – Q37          Cleft lip and cleft palate

Q38 – Q45          Other congenital malformations of genital organs

Q50 – Q56          Congenital malformations of genital organs

Q60 – Q64          Congenital malformations of the urinary system

Q65 – Q79          Congenital malformations and deformations of the musculoskeletal system

Q80 – Q89          Other congenital malformations

Q90 – Q99          Chromosomal abnormalities, not elsewhere classified

Although there are numerous types of congenital anomalies, some of them can be classified into one of the following categories:

  • Absence – missing organs or structures
  • Atresia – closure of a normal body opening or tubular structure
  • Double/doubling – duplication of an organ or structure
  • Fusion – merging or coherence of adjacent organs or structures
  • Macro – excessively large or big
  • Micro – very small or microscopic
  • Stenosis – narrowing of an organ or structure



In order to prepare you for ICD-10-CM coding of congenital anomalies, the following exercise has been prepared as an educational tool.

A 2012 ICD-10-CM code book or an electronic file of the code book contents will be needed in order to use this coding manual. To access the 2012 ICD-10-CM code book electronic files, please go online to http://www.cms.gov/ICD10/11b14_2012_ICD10CM_and_GEMs.asp#TopOfPage .

Under the “Downloads” subheading, scroll down and download the folder titled “2012 Code Tables and Index [ZIP, 16.7MB].” Once the folder is open, you will find several files, and you will need the following files when completing these exercises:

“ICD10CM_2012_Full_Tabular.pdf” – this is the 2012 ICD-10-CM Tabular List of Diseases and Injuries.

“ICD10CM_2012_Full_DIndex.pdf” – this is the 2012 ICD-10-CM Index to Diseases and Injuries.



Exercise: Use the ICD-10-CM tabular list to write the code descriptions and identify the type of anomaly for these ICD-10-CM codes. The first row has been completed as an example. Do not code from this chart, as it is to be used for educational purposes only.














Atresia of foramina of Magendie and Luschka
















































































































































Jones, Lolita M. (2012). Chapter 17: Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99). ICD-10-CM Core Concepts: A Chapter-by-Chapter Review of Coding Conventions and Challenges (p. 17-12 and 17-13). Fort Washington, Md.: Lolita M. Jones Consulting Services.

Centers for Disease Control: Facts About Birth Defects, http://www.cdc.gov/ncbddd/birthdefects/facts.html

Disclaimer: Every reasonable effort was made to ensure the accuracy of this information at the time it was published. However, due to the nature of industry changes over time we cannot guarantee its validity after the year it was published.
Lolita M. Jones, MSHS, RHIA, CCS

Lolita M. Jones, MSHS, RHIA, CCS has provided Product Consultant services to a warehousing and analytics start-up that developed and marketed decision support software, health outcomes services, and regulatory compliance toolsets. Her goal is to combine her medical coding expertise with data mining-pattern recognition, to help improve data accuracy and compliance in medical coding and reimbursement (i.e., ICD-10-CM, ICD-10-PCS, CPT, HCPCS Level II, modifiers, DRGs, APCs, and eAPGs). Ms. Jones also provides remote and on-site training/consulting in her newly developed Healthcare Data Mining Clinic educational series. She is currently pursuing a Graduate Certificate in Healthcare Data Analytics from a top university. Ms. Jones is based in New York and can be reached at .

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