2027 ICD-10-CM Diagnosis Code Q89.89Other specified congenital malformations
ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q89
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- POA Exempt
- Chronic Condition
Q89.89 is a billable ICD-10-CM diagnosis code for other specified congenital malformations. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 40 closely related codes. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.
Code Identity
Code Classification
Present on Admission (POA)Billing
Q89.89 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations (Q80-Q89).
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Abnormal communication between pericardial sac and peritoneal cavity
- Acardia
- Acephalobrachius
- Acephalogaster
- Aggressive fibromatosis of abdomen
- Aggressive infantile fibromatosis
- Aggressive systemic infantile myofibromatosis
- Amniotic adhesion
- Aplasia cutis congenita due to underlying malformation
- Aplasia of lymphatic vessel
- Asymmetrical conjoined twins
- Borjeson-Forssman-Lehmann syndrome
- Cardiac anomaly and heterotaxy syndrome
- Celosomus
- Cephalodiprosopus
- CHARGE syndrome
- Coffin-Lowry syndrome
- Congenital absence of stomach
- Congenital anomaly of body cavity
- Congenital anomaly of body wall
- Congenital anomaly of lower trunk
- Congenital anomaly of lymphatic structure of trunk
- Congenital anomaly of peritoneum
- Congenital anomaly of trunk
- Congenital anomaly of upper trunk
- Congenital deformity of soft tissue
- Congenital flat back deformity
- Congenital hemihypertrophy
- Congenital malformation of lymphatic system of cervicofacial region
- Congenital malformation of lymphatic vessel of skin
- Congenital pulmonary lymphatic dysplasia syndrome
- Congenital short trunk
- Derencephalus
- Developmental malformation of branchial arch
- Dicheirus
- Diprosopus
- Diprosopus tetrophthalmus
- Disproportionate short trunk short stature
- Duplication of upper limb
- Dysplasia of lung
- Embryological remnant
- Hereditary disorder of lymphatic system
- Hereditary hyperekplexia
- Hereditary vitreoretinopathy
- Holoacardius acephalus
- Holoacardius amorphus
- Hyperekplexia epilepsy syndrome
- Hyperexplexia
- Infantile myofibromatosis
- Lethal congenital disproportionate short trunk short stature
- Lymphatic malformation
- Marfanoid physique
- Mixed cystic lymphatic malformation
- Monocephalus
- Monocephalus tetrapus dibrachius
- Mullerian duct and limb anomalies syndrome
- Mullerian remnant
- Multicentric infantile myofibromatosis
- Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
- Myofibromatosis
- Odontotrichomelic syndrome
- Omphaloangiopagus
- Parasitic twin of asymmetrical conjoined twins
- Peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung disease
- Persistent Müllerian duct syndrome
- PTEN hamartoma tumor syndrome
- Pygomelus
- Segmental outgrowth, lipomatosis, arteriovenous malformation, epidermal nevus syndrome
- Shprintzen Goldberg craniosynostosis syndrome
- Simonart's band
- Situs ambiguus
- Solitary infantile myofibromatosis
- Stickler syndrome
- Stickler syndrome type 1
- Stickler syndrome type 2
- Stickler syndrome type 3
- Stickler syndrome type 4
- Vascular lesion of cord
- Waardenburg Shah syndrome
- Waardenburg syndrome
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to Q89.89: its own notes plus those printed at Q89.8 and Chapter 17. A note printed at a category, block or chapter applies to every code under it.
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
Use Additional Code
Add a second code, after this one, to fully describe the condition.
- code(s) to identify all associated manifestations
Notes
General instructions on how these codes are used.
- Codes from this chapter are not for use on maternal records
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Acardia, acardius Q89.89
Acardiacus amorphus Q89.89
Acephalobrachia monster Q89.89
Acephalochirus monster Q89.89
Acephalogaster Q89.89
Acephalostomus monster Q89.89
Acephalothorax Q89.89
CHARGE association Q89.89
Disease, diseased See Also: Syndrome;
Goldberg syndrome Q89.89
Hyperekplexia Q89.89
Hyperexplexia Q89.89
Malformation (congenital) See Also: Anomaly;
specified NEC Q89.89
infantile Q89.89
Syndrome See Also: Disease;
Borjeson Forssman Lehmann Q89.89
CHARGE Q89.89
Coffin-Lowry Q89.89
Goldberg Q89.89
Stickler Q89.89
stiff baby Q89.89
Teratencephalus Q89.89
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
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.Hyperekplexia
a neurological disorder characterized by an excessive startle reaction with abnormal reflex; myoclonic jerks; and muscle hypertonia.
Code History & ChangesHistory
Replacement Q89.89 replaces the following previously assigned code(s):
- Q89.8 - Other specified congenital malformations
Questions About Q89.89Overview
What is the ICD-10 code for other specified congenital malformations?
The ICD-10-CM code for other specified congenital malformations is Q89.89 (sometimes written as Q8989). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is Q89.89 (Other specified congenital malformations) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report other specified congenital malformations on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does Q89.89 group to?
When other specified congenital malformations is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.
Is Q89.89 a CC or MCC?
CMS lists Q89.89 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 40 closely related codes in its exclusion list.
Is Q89.89 exempt from POA reporting?
Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for other specified congenital malformations on inpatient claims. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.
