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May 29, 2006
A cleft is an opening or a split in the upper lip (cleft lip), the roof of the mouth (cleft palate), or both because there is not enough tissue in the mouth or lip area. The tissue that is available does not join together properly. These conditions are some of the most common birth defects, affecting one of every 700 babies. It is normal for a fetus in the womb to have an opening in the lip and mouth area when the mouth is forming, but these structures fuse together in the first three months. Cleft lip and cleft palate may occur as isolated birth defects, but they are also commonly associated with other known medical conditions. According to the ICD-9-CM Official Guidelines for Coding and Reporting effective December 1, 2005, “When a congenital anomaly does not have a unique code assignment, assign additional code(s) for any manifestations that may be present.” For example, multiple pterygium syndrome is not specifically indexed in ICD-9-CM. The appropriate code assignment for this syndrome is 759.89 (AHA Coding Clinic for ICD-9-CM, 1987, September-October, page 9). Because it does not have a unique code assignment, all other documented manifestations, such as cleft lip and/or cleft palate, are coded and sequenced as secondary diagnoses. The ICD-9-CM Official Guidelines for Coding and Reporting goes on to state, “When the code assignment specifically identifies the congenital anomaly, manifestations that are an inherent component of the anomaly should not be coded separately. Additional codes should be assigned for manifestations that are not an inherent component.” Notice that Stickler syndrome is indexed to code 759.89 in ICD-9-CM; however, this is not a unique code assignment for the syndrome. Code 759.89 includes a wide range of other specified anomalies. Therefore, it would be appropriate to code all documented manifestations associated with the congenital anomaly, such as cleft palate. According to AHA Coding Clinic for ICD-9-CM, “Any additional features that are not captured in the code should be coded separately” (1996, third quarter, page 15). For example, Nager syndrome is indexed to code 756.0. However, there are other manifestations associated with this condition that are not captured in the code assignment. Therefore, AHA Coding Clinic for ICD-9-CM’s advice is to code separately any additional conditions not captured in the code assignment. Cleft Lip • 749.10, Cleft lip, unspecified; • 749.11, Cleft lip, unilateral, complete; • 749.12, Cleft lip, unilateral, incomplete; • 749.13, Cleft lip, bilateral, complete; and • 749.14, Cleft lip, bilateral, incomplete. Cleft Palate • 749.00, Cleft palate, unspecified; • 749.01, Cleft palate, unilateral, complete; • 749.02, Cleft palate, unilateral, incomplete; • 749.03, Cleft palate, bilateral, complete; and • 749.04, Cleft palate, bilateral, incomplete. Since cleft lip and cleft palate develop independently of each other, they can occur together or separately. The code assignments for both cleft lip and cleft palate occurring together include the following: • 749.20, Cleft palate with cleft lip, unspecified; • 749.21, Cleft palate with cleft lip, unilateral, complete; • 749.22, Cleft palate with cleft lip, unilateral, incomplete; • 749.23, Cleft palate with cleft lip, bilateral, complete; • 749.24, Cleft palate with cleft lip, bilateral, incomplete; and • 749.25, Cleft palate with cleft lip, other combinations. The appropriate code assignment will depend on the physician’s documentation available in the medical record. Treatment In utero surgery may soon be the standard treatment for repair of cleft lip and cleft palate. If that is the case, do not assign a diagnosis code from Chapter 15 (Perinatal Codes). Instead, assign a code from category 655 in Chapter 11 to identify the fetal condition affecting the obstetric encounter. Also, assign the procedure code 75.36 to identify the in utero surgery performed. Coding and sequencing for cleft lip and cleft palate 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 nearly 5,000 healthcare providers. The company
and its representatives do not assume any responsibility for reimbursement
decisions or claims denials made by providers or payors 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.
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