2026 ICD-10-CM Diagnosis Code Q89.89Other specified congenital malformations
Q89.89 is a billable ICD-10-CM diagnosis code for other specified congenital malformations. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 564 through 566. The code is exempt from POA reporting.
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 other POA exempt codes.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Abnormal communication between pericardial sac and peritoneal cavity
- Abnormal fetal duplication
- 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
- 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 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
- Thoracodidymus
- Waardenburg Shah syndrome
- Waardenburg syndrome
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
- Acardia, acardius - Q89.89
- Acardiacus amorphus - Q89.89
- genitourinary organs NEC - Q89.89
- Acephalobrachia monster - Q89.89
- Acephalochirus monster - Q89.89
- Acephalogaster - Q89.89
- Acephalostomus monster - Q89.89
- Acephalothorax - Q89.89
- organ or site NEC - 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
- Nephrosis, nephrotic (Epstein's) (syndrome) (congenital) - N04.9
- Finnish type (congenital) - 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
External Cause of Injuries IndexGuidance
References for this code in the External Cause of Injuries Index.
- Absence(of) (organ or part) (complete or partial)
- organ
- or site, congenital NEC
- Acardia, acardius
- Acardiacus amorphus
- Accessory(congenital)
- genitourinary organs NEC
- Acephalobrachia monster
- Acephalochirus monster
- Acephalogaster
- Acephalostomus monster
- Acephalothorax
- Anomaly, anomalous(congenital) (unspecified type)
- specified organ or site NEC
- Atresia, atretic
- organ or site NEC
- CHARGE association
- Cyst(colloid) (mucous) (simple) (retention)
- congenital NEC
- Disease, diseased
- Kok
- Disease, diseased
- Startle
- Goldberg syndrome
- Hyperekplexia
- Hyperexplexia
- Malformation(congenital)
- specified NEC
- Myofibromatosis
- infantile
- Nephrosis, nephrotic(Epstein's) (syndrome) (congenital)
- Finnish type (congenital)
- Syndrome
- Borjeson Forssman Lehmann
- Syndrome
- CHARGE
- Syndrome
- Coffin-Lowry
- Syndrome
- Goldberg
- Syndrome
- Stickler
- Syndrome
- stiff baby
- Teratencephalus
Clinical InformationClinical
Myofibromatosis
a condition characterized by multiple formations of myofibromas (leiomyoma).Waardenburg Syndrome
rare, autosomal dominant disease with variable penetrance and several known clinical types. characteristics may include depigmentation of the hair and skin, congenital deafness, heterochromia iridis, medial eyebrow hyperplasia, hypertrophy of the nasal root, and especially dystopia canthorum. the underlying cause may be defective development of the neural crest (neurocristopathy). waardenburg's syndrome may be closely related to piebaldism. klein-waardenburg syndrome refers to a disorder that also includes upper limb abnormalities.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.Leiomyoma
a benign tumor derived from smooth muscle tissue, also known as a fibroid tumor. they rarely occur outside of the uterus and the gastrointestinal tract but can occur in the skin and subcutaneous tissue, probably arising from the smooth muscle of small blood vessels in these tissues.Thoracodidymus
conjoined twins united at the thorax.Stickler Syndrome
a rare autosomal dominant syndrome caused by mutations in the col11a1, col11a2, and col2a1 genes which affect the production of type ii and xi collagen. it is characterized by a range of signs and symptoms including cleft palate, large tongue, small lower jaw, hearing loss, myopia, glaucoma, retinal detachment, skeletal, and joint abnormalities.Stickler Syndrome Type 1|STL1
stickler syndrome inherited in an autosomal dominant pattern, caused by mutation(s) in the col2a1 gene, encoding collagen alpha-1(ii) chain.Stickler Syndrome Type 2|Stickler Syndrome Type II
a rare autosomal dominant syndrome caused by mutations in the col11a1 gene. it is characterized by an abnormal ocular vitreous architecture (beaded vitreous phenotype). other signs and symptoms include retinal detachment, joint hypermobility, hearing loss, and midline clefting.
Code History & ChangesHistory
New Code Q89.89 was added to the ICD-10-CM code set for FY 2026, effective October 1, 2025.
Replacement Q89.89 replaces the following previously assigned code(s):
- Q89.8 - Other specified congenital malformations
Questions About Q89.89Overview
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, 2025 through September 30, 2026.
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, 565, 566, with relative weights from 0.7493 to 1.5436 depending on complications. Higher weights mean higher Medicare reimbursement.
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.
