2026 ICD-10-CM Diagnosis Code Q87.89Other specified congenital malformation syndromes, not elsewhere classified

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q87

ICD-10-CM Q87.89
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q87.89 is a billable ICD-10-CM diagnosis code for other specified congenital malformation syndromes, not elsewhere classified. 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. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified congenital anomalies.

Code Identity

ICD-10-CM Code
Q87.89
Billable Status
Yes — Valid for Submission
Code Describes
Other specified congenital malformation syndromes, not elsewhere classified
Short Description
Oth congenital malformation syndromes, NEC
Parent Code
Q87.8
Other specified congenital malformation syndromes, not elsewhere classified

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ87Other specified congenital malformation syndromes affecting multiple systems
This CodeQ87.89Other specified congenital malformation syndromes, not elsewhere classified

Present on Admission (POA)Billing

Q87.89 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.

  • 11p partial monosomy syndrome
  • 11p15 deletion syndrome
  • 12q15 deletion syndrome
  • 12q24.31-q24.32 deletion syndrome
  • 3-Phosphoglycerate dehydrogenase deficiency
  • Ablepharon
  • Absent eyebrow
  • Acrodysplasia scoliosis
  • Adrenal hyperfunction
  • Alkuraya Kucinskas syndrome
  • Allemann's syndrome
  • Alstrom syndrome
  • Alveolar bone loss
  • Andersen Tawil syndrome
  • Aortic aneurysm due to Loeys-Dietz syndrome
  • Aplasia cutis congenita secondary to malformation syndrome
  • Aplasia cutis in Johanson-Blizzard syndrome
  • Athetoid movement
  • Atypical Timothy syndrome
  • Autosomal dominant dyskeratosis congenita
  • Autosomal dominant popliteal pterygium syndrome
  • Autosomal recessive popliteal pterygium syndrome
  • Axial mesodermal dysplasia spectrum
  • Bamforth Lazarus syndrome
  • Bardet-Biedl syndrome
  • Benign neoplasm of heart
  • Biemond syndrome type 2
  • Biemond's syndrome
  • Birt Hogg Dubé syndrome
  • Blepharophimosis, intellectual disability syndrome
  • Bohring Opitz syndrome
  • Bone age finding
  • Bosley Salih Alorainy syndrome
  • Bowen-Conradi syndrome
  • Braddock syndrome
  • Branchiootic syndrome
  • BSG syndrome
  • Cardiac urogenital syndrome
  • Cardiocranial syndrome Pfeiffer type
  • Carney complex
  • Carney complex, trismus, pseudocamptodactyly syndrome
  • Carney complex-associated-primary pigmented nodular adrenocortical disease
  • CHD4-related neurodevelopmental disorder
  • Choroideremia
  • Christianson syndrome
  • Chudley McCullough syndrome
  • Cohen syndrome
  • Cold-induced sweating syndrome
  • Congenital absence of eyelash
  • Congenital anomaly of lymphatic structure of trunk
  • Congenital atresia of external auditory canal
  • Congenital atrophy of optic nerve
  • Congenital coloboma of iris
  • Congenital conductive hearing loss
  • Congenital corneal dystrophy
  • Congenital intrauterine infection-like syndrome
  • Congenital lymphangiectasia
  • Congenital malformation of the eyebrow
  • Congenital mixed conductive and sensorineural hearing loss
  • Congenital osteodystrophy
  • Congenital peripheral pulmonary artery stenosis
  • Congenital stenosis of pulmonary artery
  • Constitutional aplastic anemia
  • Cooper Jabs syndrome
  • Costello syndrome
  • Craniofacial deafness hand syndrome
  • Craniofacial ulnar renal syndrome
  • Crisponi syndrome
  • Currarino triad
  • Cutis laxa, autosomal recessive
  • de Barsey syndrome
  • Delayed bone age
  • Deletion of part of chromosome 11
  • Deletion of part of long arm of chromosome 12
  • Developmental malformation of branchial arch
  • Developmental malformation, deafness, dystonia syndrome
  • Diabetes mellitus associated with genetic syndrome
  • DICER1 syndrome
  • Disorder of serine metabolism
  • Donnai-Barrow syndrome
  • Dyskeratosis congenita
  • Dystopia canthorum
  • Dystrophy of anterior cornea
  • Ear, patella, short stature syndrome
  • Feingold syndrome
  • Fetal acidosis
  • Fetal anemia
  • Fetal metabolic disorder
  • FG syndrome
  • FG syndrome type 1
  • Fibrofolliculoma
  • Fixed dilatation of pupil
  • Floating-Harbor syndrome
  • Francois syndrome
  • Fried syndrome
  • Generalized dystonia
  • Genetic syndromic childhood obesity
  • German syndrome
  • Gomez Lopez Hernandez syndrome
  • Grubben, De Cock, Borghgraef syndrome
  • Haim Munk syndrome
  • Hecht syndrome
  • Hennekam syndrome
  • Hereditary choroidal dystrophy
  • Hereditary disorder of lymphatic system
  • Heritable pulmonary arterial hypertension
  • Hydrops fetalis
  • Hydrops, lactic acidosis, sideroblastic anemia, multisystemic failure syndrome
  • Hyperreflexia
  • Immotile cilia syndrome
  • Immotile cilia syndrome due to defective radial spokes
  • Infantile hemangioma
  • Insulin resistance
  • Intellectual disability Birk-Barel type
  • Intestinal lymphangiectasis
  • Jansen-de Vries syndrome
  • Johnson neuroectodermal syndrome
  • Juberg Hayward syndrome
  • Keutel syndrome
  • KLHL7-related Bohring Opitz-like syndrome
  • KLHL7-related Bohring-Opitz-like and Crisponi/cold-induced sweating-like overlap syndrome
  • KLHL7-related Crisponi/cold-induced sweating-like syndrome
  • LAMA5-related multisystemic syndrome
  • Laurence-Moon syndrome
  • LIG4 syndrome
  • Loeys-Dietz syndrome
  • Lowry MacLean syndrome
  • Malabsorption of glucose
  • Marden Walker syndrome
  • Marfanoid physique
  • Marfanoid syndrome De Silva type
  • Matthew Wood syndrome
  • McKusick Kaufman syndrome
  • Meacham syndrome
  • Micronodular adrenal hyperplasia
  • Mixed conductive AND sensorineural hearing loss
  • Multiple fibrofolliculomas
  • Multiple malformation syndrome with senile-like appearance
  • Multiple system malformation syndrome
  • Multisystemic smooth muscle dysfunction syndrome
  • Myhre syndrome
  • Myxoma of heart
  • N syndrome
  • Nance-Horan syndrome
  • Neu-Laxova syndrome
  • Neuroectodermal melanolysosomal disease
  • Nicolaides-Baraitser syndrome
  • Nodular hyperplasia of adrenal cortex
  • Nodule of adrenal cortex
  • Oculodental syndrome
  • Oculodentodigital syndrome
  • Oculodento-osseous dysplasia
  • Oculodento-osseous dysplasia - mild type
  • Oculodento-osseous dysplasia - severe type
  • Oculofaciocardiodental syndrome
  • Oculotrichodysplasia
  • Ohdo syndrome, Maat-Kievit-Brunner type
  • Ohdo syndrome, Say-Barber-Biesecker-Young-Simpson variant
  • Oto-onycho-peroneal syndrome
  • Pallister-Hall syndrome
  • Papillon-Lefèvre syndrome
  • Partial ablepharon
  • Patent ductus arteriosus
  • Pena-Shokeir syndrome type I
  • Periodic paralysis
  • Periodontitis due to Papillon-Lefèvre syndrome
  • Periodontitis exacerbated by Cohen syndrome
  • Peripheral pulmonary artery stenosis
  • PHACE syndrome
  • PHACES syndrome
  • Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome
  • Polyglandular hyperfunction
  • Popliteal pterygium syndrome
  • Primary ciliary dyskinesia and retinitis pigmentosa syndrome
  • Primary ciliary dyskinesia due to transposition of ciliary microtubules
  • Pseudoleprechaunism syndrome Patterson type
  • Pseudoprogeria syndrome
  • PURA syndrome
  • Pure gonadal dysgenesis
  • Pure gonadal dysgenesis 46,XY
  • RAB18 deficiency
  • Renpenning syndrome
  • RERE-related neurodevelopmental syndrome
  • Revesz syndrome
  • Rhombencephalosynapsis
  • Rutland ciliary disorientation syndrome
  • Sagittal craniosynostosis
  • Scholte syndrome
  • Schöpf-Schulz-Passarge syndrome
  • Sensory hearing loss
  • Severe combined immunodeficiency with low T- and B-cell numbers
  • Severe combined immunodeficiency, microcephaly, growth retardation, sensitivity to ionizing radiation syndrome
  • Siegler Brewer Carey syndrome
  • Singleton-Merten syndrome
  • Snyder-Robinson syndrome
  • Speech delay
  • Stern Lubinsky Durrie syndrome
  • Stimmler syndrome
  • Stromme syndrome
  • Symptomatic form of Coffin-Lowry syndrome in female carrier
  • Timothy syndrome
  • Timothy syndrome type 1
  • Trichothiodystrophy
  • VACTEL syndrome
  • Van den Bosch syndrome
  • Vertebral abnormalities, anal atresia, cardiac abnormalities, tracheo-esophageal fistula, renal anomalies, limb defects syndrome
  • Vici syndrome
  • Waardenburg syndrome
  • Waardenburg syndrome type 1
  • Waardenburg syndrome type 2
  • Warburg micro syndrome
  • Weaver Williams syndrome
  • Wolfram-like syndrome
  • X-linked intellectual disability Armfield type
  • X-linked intellectual disability Pai type
  • X-linked retinitis pigmentosa
  • XY type gonadal dysgenesis with associated anomalies syndrome
  • Young's syndrome
  • Zimmermann-Laband syndrome

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Birt-Hogg-Dube syndrome
    • Cantrell's syndrome
    • Cerebro-hepato-renal syndrome
    • Syndrome
      • basal cell nevus
    • Syndrome
      • Birt-Hogg-Dube syndrome
    • Syndrome
      • cardiofaciocutaneous
    • Syndrome
      • congenital
        • affecting multiple systems NEC
    • Syndrome
      • congenital
        • muscular hypertrophy-cerebral
    • Syndrome
      • Glass
    • Syndrome
      • Gorlin's
    • Syndrome
      • Oliver-McFarlane
    • Syndrome
      • popliteal
        • web
    • Syndrome
      • SATB2-associated
    • Syndrome
      • Snyder-Robinson
    • Web, webbed(congenital)
      • popliteal syndrome

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL010
Other specified and unspecified congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Alveolar Bone Loss

    resorption or wasting of the tooth-supporting bone (alveolar process) in the maxilla or mandible.

  • Hydrops Fetalis

    abnormal accumulation of serous fluid in two or more fetal compartments, such as skin; pleura; pericardium; placenta; peritoneum; amniotic fluid. general fetal edema may be of non-immunologic origin, or of immunologic origin as in the case of erythroblastosis fetalis.

  • Costello Syndrome

    rare congenital disorder with multiple anomalies including: characteristic dysmorphic craniofacial features, musculoskeletal abnormalities, neurocognitive delay, and high prevalence of cancer. germline mutations in h-ras protein can cause costello syndrome. costello syndrome shows early phenotypic overlap with other disorders that involve map kinase signaling system (e.g., noonan syndrome and cardiofaciocutaneous syndrome).

  • 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.

  • Alstrom Syndrome

    rare autosomal recessive disease characterized by multiple organ dysfunction. the key clinical features include retinal degeneration (nystagmus, pathologic; retinitis pigmentosa; and eventual blindness), childhood obesity, sensorineural hearing loss, and normal mental development. endocrinologic complications include type 2 diabetes mellitus; hyperinsulinemia; acanthosis nigricans; hypothyroidism; and progressive renal and hepatic failures. the disease is caused by mutations in the alms1 gene.

  • Carney Complex

    autosomal dominant syndrome characterized by cardiac and cutaneous myxomas; lentiginosis (spotty pigmentation of the skin), and endocrinopathy and its associated endocrine tumors. the cardiac myxomas may lead to sudden cardiac death and other complications in carney complex patients. the gene coding for the prkar1a protein is one of the causative genetic loci (type 1). a second locus is at chromosome 2p16 (type 2).

  • Dyskeratosis Congenita

    a predominantly x-linked recessive syndrome characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. oral and dental abnormalities may also be present. complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. (from int j paediatr dent 2000 dec;10(4):328-34) the x-linked form is also known as zinsser-cole-engman syndrome and involves the gene which encodes a highly conserved protein called dyskerin.

  • Choroideremia

    an x chromosome-linked abnormality characterized by atrophy of the choroid and degeneration of the retinal pigment epithelium causing night blindness.

  • Insulin Resistance

    diminished effectiveness of insulin in lowering blood sugar levels: requiring the use of 200 units or more of insulin per day to prevent hyperglycemia or ketosis.

  • Metabolic Syndrome

    a cluster of symptoms that are risk factors for cardiovascular diseases and type 2 diabetes mellitus. the major components of metabolic syndrome include abdominal obesity; atherogenic dyslipidemia; hypertension; hyperglycemia; insulin resistance; a proinflammatory state; and a prothrombotic (thrombosis) state.

  • Hydrops Fetalis

    a condition characterized by fluid accumulation in two or more anatomic compartments in the fetus.

  • Immune Hydrops Fetalis

    fluid accumulation in multiple fetal anatomic cavities attributable to a maternal immune response against fetal blood cell antigens.

  • Non-Immune Hydrops Fetalis

    fluid accumulation in multiple fetal anatomic cavities that is of non-immune origin.

  • Costello Syndrome

    a genetic syndrome caused by mutations in the hras gene. it is characterized by developmental delay, mental retardation, loose skin folds, cardiomyopathy, tachycardia, and structural heart defects. patients are at an increased risk of developing benign or malignant neoplasms.

  • Myhre Syndrome

    a rare, autosomal dominant inherited disorder caused by mutations in the smad4 gene. it is characterized by developmental abnormalities, mild to moderate intellectual disability, hearing loss, skin stiffness, skeletal abnormalities, and typical facial features (short palpebral fissures, shortened distance between the nose and upper lip, midface hypoplasia, prognathism, cleft palate, and/or cleft lip).

  • DICER1 Syndrome

    a rare, autosomal dominant inherited syndrome caused by mutations in the dicer1 gene. people with this syndrome are at an increased risk of developing pleuropulmonary blastoma, cystic nephroma, sertoli-leydig cell tumor of the ovary, and multinodular goiter.

  • Feingold Syndrome

    a rare autosomal dominant syndrome caused by mutations in the mycn oncogene. it is characterized by microcephaly, limb abnormalities, esophageal and/or duodenal atresia.

  • Autosomal Dominant Popliteal Pterygium Syndrome

    a rare, autosomal dominant inherited syndrome caused by mutations in the irf6 gene. it is characterized by the presence of cleft palate, cleft lip, pits in the lower lip, web behind the knee (popliteal pterygium), syndactyly, cryptorchidism, scrotal malformation, and hypoplasia of the labia majora.

  • LIG4 Syndrome

    a very rare genetic disorder caused by mutation in the lig4 gene. it is characterized by unusual facial features, microcephaly, growth and developmental delay, severe immunodeficiency, and skin abnormalities.

  • Homeostatic Model Assessment of Insulin Resistance

    an assessment of beta-cell function and insulin resistance based on fasting blood glucose and insulin concentrations.

  • Hyperandrogenism, Insulin Resistance, Acanthosis Nigricans Syndrome|HAIR-AN Syndrome

    a condition characterized by hyperandrogenism, insulin resistance, and acanthosis nigricans, typically associated with obesity in teenage girls. it is considered to be a subtype of polycystic ovarian syndrome, but may occur in male individuals. etiology is unclear, but some cases may be associated with mutations affecting the tyrosine kinase domain of the insulin receptor.

  • Insulin Receptor Mutation - Associated Insulin Resistance Syndromes

    insulin resistance caused by inactivating mutation(s) in the insr gene encoding the insulin receptor.

  • Insulin Resistance

    decreased sensitivity to circulating insulin which may result in acanthosis nigicrans, elevated insulin level or hyperglycemia.

  • Insulin Resistance Measurement|INSULINR|Insulin Resistance|Insulin Resistance

    the determination of the insulin resistance (cells inability to respond to insulin) in a biological specimen.

  • Insulin Resistance Syndrome

    a cluster of closely related metabolic abnormalities associated with insulin resistance that confer an increased risk of the development of type 2 diabetes and cardiovascular disease. these abnormalities may include obesity, high blood pressure, abnormal cholesterol levels, proteinuria, and/or polycystic ovary syndrome.

  • Insulin Resistant Diabetes Mellitus with Acanthosis Nigricans and Hyperandrogenism|Type A Insulin Resistance Syndrome

    a syndrome of insulin resistance caused by mutation(s) in the insr gene, encoding the insulin receptor. this condition is characterized by a clinical triad of hyperinsulinemia, acanthosis nigricans, and hyperandrogenism without lipodystrophy. this is the least severe of a spectrum of disorders; the other two conditions are rabson-mendenhall syndrome and donohoe syndrome.

  • Obesity-Associated Insulin Resistance

    insulin resistance associated with obesity, which may be attributed in part to impaired insulin signaling in target tissues, or impaired insulin-stimulated glucose transport due to reduced expression of the glucose transporter protein 4.

  • Sensory Hearing Loss

    hearing loss caused by damage to the cochlea in the inner ear.

Patient EducationClinical

Birth Defects

A birth defect is a problem that happens while a baby is developing in the mother's body. Most birth defects happen during the first 3 months of pregnancy. One out of every 33 babies in the United States is born with a birth defect.

The full article covers:

  • What are birth defects?
  • What causes birth defects?
  • Who is at risk of having a baby with birth defects?
  • How are birth defects diagnosed?
  • What are the treatments for birth defects?
  • Can birth defects be prevented?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q87.89 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
759.89 Specfied cong anomal NEC
Approximate The match is approximate rather than exact.

Code History & ChangesHistory

Replaced This code was replaced in the FY 2026 code set by:

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About Q87.89Overview

Is Q87.89 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other specified congenital malformation syndromes, not elsewhere classified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q87.89 group to?

When other specified congenital malformation syndromes, not elsewhere classified 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 Q87.89 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 malformation syndromes, not elsewhere classified on inpatient claims.

What is the ICD-9 equivalent of Q87.89?

Under the General Equivalence Mappings, other specified congenital malformation syndromes, not elsewhere classified converts to ICD-9-CM 759.89 (specfied cong anomal NEC). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.