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November 5, 2012

Coding for Congenital Anomalies
For The Record
Vol. 24 No. 20 P. 26

Congenital anomalies, also known as birth defects, may result from pregnancy or birth complications, genetic malformation, or a viral infection in utero. Congenital is defined as a condition that is present at birth but may not show manifestations until later in life. Anomaly is a developmental deformity. An anomaly documented as acquired is a condition not present at birth.

Congenital Anomaly Guidelines
The ICD-9-CM Official Guidelines for Coding and Reporting have the following guidelines related to congenital anomalies:

• If any congenital anomaly is documented, assign the appropriate code from categories 740 to 759. It is also appropriate to assign codes from other chapters to specify conditions associated with the anomaly, if applicable.

• The congenital anomaly may be sequenced as the principal or secondary diagnosis depending on the circumstances of admission.

• If there is no unique code assignment for the congenital anomaly, assign codes for any documented manifestations that are present.

• When there is a code that specifically identifies the anomaly, it is still appropriate to assign codes for manifestations that are not inherent to the anomaly. However, do not assign codes for manifestations that are inherent to the congenital anomaly.

• Congenital anomaly codes may be used throughout a patient’s life when documented by the physician. If the congenital anomaly has been corrected, assign a personal history code for the anomaly.

There are more than 50 congenital anomaly syndromes that may not have a specific ICD-9-CM code assignment available. Coding Clinic has advised to assign code 759.89, Other specified anomalies, on several occasions for congenital syndromes that are not indexed to a specific code. Also in several instances, the ICD-9-CM index will direct the coder to code 759.89 for some congenital anomalies. Examples of congenital anomalies assigned to code 759.89 by either the AHA Coding Clinic for ICD-9-CM or the ICD-9-CM alphabetic index include the following: Alport, Angelman, Bardet-Biedl (or Biedl-Bardet), Börjeson-Forssman-Lehmann, branchio-oto-renal (1999, third quarter, pages 17-18), cardiofaciocutaneous, Carpenter, Costello (1999, third quarter, pages 18-19), Fraser, Freeman-Sheldon, Gillespie, Gorlin-Chaudhry-Moss, inverted Marfan’s, Joubert, Kabuki, Laurence-Moon, Marchesani-Weill (or Weill-Marchesani), Menkes, Noonan, Russell-Silver, Smith-Lemli-Opitz, startle disease (2004, second quarter, page 12), Taybi (otopalatodigital syndrome), Ullrich-Feichtiger, and Williams syndrome (1998, third quarter, page 8).

As stated above, along with 759.89, additional codes may be assigned for any associated manifestations of the condition.

Categories of Congenital Abnormalities
The following are different categories of congenital abnormalities:

• chromosome abnormalities (eg, Down syndrome);

• single-gene abnormalities (eg, autosomal dominant inheritance; autosomal recessive inheritance, such as cystic fibrosis; sex linked, such as hemophilia);

• conditions occurring during pregnancy that may cause abnormalities at birth (eg, diabetes);

• genetic problem combined with environmental problems (eg, spina bifida); and

• unknown cause.

Schatzki Ring
A common problem is what code to assign for Schatzki ring when it is an initial diagnosis in an adult patient. Schatzki ring defaults to code 750.3, Congenital tracheoesophageal fistula, esophageal atresia/stenosis, when the physician has not documented whether it is congenital or acquired. Documentation of acquired Schatzki ring will change the code to 530.3, Stricture/stenosis of esophagus.

According to Coding Clinic, the physician should be queried for clarification when Schatzki ring is newly diagnosed in an adult patient and there is no additional documentation stating whether it is acquired or congenital. However, often the presence of Schatzki ring identified during an upper gastroscopy is an incidental finding that does not require further care or treatment. Therefore, even if the physician documents the condition, it may not be possible to code it unless the physician documents the clinical significance of the condition in terms of requiring evaluation, monitoring, treatment, increased nursing care, or increased length of stay (AHA Coding Clinic for ICD-9-CM, 2012, first quarter, pages 15-16).

Coding and sequencing for congenital anomalies are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding.

— This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.


ICD-10-CM Coding for Congenital Anomalies
Some congenital anomaly sections in ICD-10-CM have been greatly expanded to allow for more precision in reporting these conditions. For example, in the circulatory system, there are nine congenital anomaly categories compared with three in ICD-9-CM. These nine categories are as follows:

• Q20, Congenital malformations of cardiac chambers and connections;

• Q21, Congenital malformations of cardiac septa;

• Q22, Congenital malformations of pulmonary and tricuspid valves;

• Q23, Congenital malformations of aortic and mitral valves;

• Q24, Other congenital malformations of heart;

• Q25, Congenital malformations of great arteries;

• Q26, Congenital malformations of great veins;

• Q27, Other congenital malformations of peripheral vascular system; and

• Q28, Other congenital malformations of circulatory system.

The coding guidelines pertaining to congenital anomalies remain unchanged from ICD-9-CM to ICD-10-CM.

— AH