Other congenital malformations, not elsewhere classified (Q89) ICD-10-CM
The Q89 code range covers other congenital malformations, not elsewhere classified with 13 ICD-10-CM diagnosis codes. 10 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Codes in the Q89 Range 13 codes · 10 billable
- Q89 Other congenital malformations, not elsewhere classifiedNon-billable
- Q89.0 Congenital absence and malformations of spleenNon-billable
- Q89.01 Asplenia (congenital)
- Q89.09 Congenital malformations of spleen
- Q89.1 Congenital malformations of adrenal gland
- Q89.2 Congenital malformations of other endocrine glands
- Q89.3 Situs inversus
- Q89.4 Conjoined twins
- Q89.7 Multiple congenital malformations, not elsewhere classified
- Q89.8 Other specified congenital malformationsNon-billable
- Q89.81 Kabuki syndrome
- Q89.89 Other specified congenital malformations
- Q89.9 Congenital malformation, unspecified
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the Q89 range.
CHARGE Syndrome
Rare disease characterized by COLOBOMA; CHOANAL ATRESIA; and abnormal SEMICIRCULAR CANALS. Mutations in CHD7 protein resulting in disturbed neural crest development are associated with CHARGE Syndrome.
Congenital Abnormalities
Malformations of organs or body parts during development in utero.
Hyperekplexia
A neurological disorder characterized by an excessive startle reaction with ABNORMAL REFLEX; MYOCLONIC JERKS; and MUSCLE HYPERTONIA.
Kabuki Syndrome
A rare, autosomal dominant or X-linked dominant inherited syndrome caused by mutations in the KMT2D gene (also known as MLL2) or the KDM6A gene. It is characterized by distinctive facial features including arched eyebrows, long eyelashes, long palpebral fissures with the lower lids turned out at the outside edges, a flat nose, and large protruding earlobes, developmental delay and intellectual disability.
Situs Inversus
A congenital abnormality in which organs in the THORAX and the ABDOMEN are opposite to their normal positions (situs solitus) due to lateral transposition. Normally the STOMACH and SPLEEN are on the left, LIVER on the right, the three-lobed right lung is on the right, and the two-lobed left lung on the left. Situs inversus has a familial pattern and has been associated with a number of genes related to microtubule-associated proteins.
Twins, Conjoined
MONOZYGOTIC TWINS who are joined in utero. They may be well developed and share only a superficial connection, often in the frontal, transverse or sagittal body plane, or they may share a partial duplication of a body structure. Alternatively, there may be a small and incompletely developed twin conjoined to a larger, more fully developed twin.
About the Q89 Code Range
These conditions are present at birth and involve the spleen, glands, or other body structures. They do not fit elsewhere in the classification.
The spleen subdivisions distinguish congenital absence, called asplenia, from other spleen malformations. Separate subdivisions identify malformations of the adrenal gland and other endocrine glands, as well as conjoined twins.
Other subdivisions distinguish multiple malformations, other specified malformations, and an unspecified malformation. Q89.81 identifies Kabuki syndrome within the specified malformations.
FY 2027 changes: The FY 2027 ICD-10-CM update, effective October 1, 2026, deleted Q89.8.
Questions About This Page
How many billable codes are in the Q89 range?
Of the 13 codes in this range, 10 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the Q89 range classify?
The range classifies other congenital malformations, not elsewhere classified. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
