2024 ICD-10-CM Diagnosis Code Q87.89

Other specified congenital malformation syndromes, not elsewhere classified

ICD-10-CM Code:
Q87.89
ICD-10 Code for:
Oth congenital malformation syndromes, NEC
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Chronic
Code Navigator:

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    (Q00-Q99)
    • Other congenital malformations
      (Q80-Q89)
      • Other specified congenital malformation syndromes affecting multiple systems
        (Q87)

Q87.89 is a billable diagnosis code used to specify a medical diagnosis of other specified congenital malformation syndromes, not elsewhere classified. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis 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
  • 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
  • Biemond syndrome type 2
  • Biemond's syndrome
  • Blepharophimosis, intellectual disability syndrome
  • Blepharophimosis, intellectual disability syndrome
  • Bohring Opitz syndrome
  • Bone age finding
  • Bosley Salih Alorainy syndrome
  • Bowen-Conradi syndrome
  • Braddock syndrome
  • Branchiootic syndrome
  • BSG syndrome
  • Cardiocranial syndrome Pfeiffer type
  • Carney complex
  • Carney complex, trismus, pseudocamptodactyly syndrome
  • 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 blepharophimosis
  • Congenital blepharophimosis
  • Congenital coloboma of iris
  • Congenital conductive hearing loss
  • Congenital corneal dystrophy
  • 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
  • Cutis laxa-corneal clouding-oligophrenia syndrome
  • de Barsy syndrome
  • Delayed bone age
  • Deletion of part of chromosome 11
  • Deletion of part of long arm of chromosome 12
  • Deletion of part of long arm of chromosome 12
  • Developmental malformation of branchial arch
  • Developmental malformation, deafness, dystonia 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
  • FG syndrome
  • FG syndrome type 1
  • Fixed dilatation of pupil
  • Floating-Harbor syndrome
  • Francois syndrome
  • Fried syndrome
  • Generalized dystonia
  • German syndrome
  • Glucose-galactose malabsorption
  • Gomez Lopez Hernandez syndrome
  • Grubben, De Cock, Borghgraef syndrome
  • Haim Munk syndrome
  • Hecht syndrome
  • Hennekam syndrome
  • 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
  • Insulin resistance
  • Intellectual disability Birk-Barel type
  • Intestinal lymphangiectasis
  • Johnson neuroectodermal syndrome
  • Juberg Hayward syndrome
  • Keutel syndrome
  • Kleefstra 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
  • Metabolic disorder of fetus
  • Mixed conductive AND sensorineural hearing loss
  • Multiple malformation syndrome with senile-like appearance
  • Multiple system malformation syndrome
  • Multisystemic smooth muscle dysfunction syndrome
  • Myhre syndrome
  • Myxoma of heart
  • Myxoma of heart
  • N syndrome
  • Nance-Horan syndrome
  • Neu-Laxova syndrome
  • Neuroectodermal melanolysosomal disease
  • Nicolaides-Baraitser syndrome
  • Oculodental syndrome
  • Oculodentodigital syndrome
  • Oculodento-osseous dysplasia
  • 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
  • Photosensitivity with ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature syndrome
  • Polyglandular hyperfunction
  • Popliteal pterygium syndrome
  • Posterior fossa brain malformation, haemaniogma, arterial anomaly, cardiac defect and aortic coarctation, eye abnormality synodrome and sternal anomaly syndrome
  • Posterior fossa brain malformation, hemangioma, arterial anomaly, cardiac defect and aortic coarctation, and eye abnormality 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
  • RAB18 deficiency
  • Renpenning syndrome
  • RERE-related neurodevelopmental syndrome
  • Revesz syndrome
  • Rhombencephalosynapsis
  • Rutland ciliary disorientation syndrome
  • 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
  • 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
  • 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

Clinical Classification

Clinical Information

  • 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.

Replaced Code

This code was replaced in the 2024 ICD-10-CM code set with the code(s) listed below. The National Center for Health Statistics (NCHS) has published an update to the ICD-10-CM diagnosis codes which became effective October 1, 2023. This code was replaced for the FY 2024 (October 1, 2023 - September 30, 2024).


Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Present on Admission (POA)

Q87.89 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA IndicatorReason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q87.89 to ICD-9-CM

  • ICD-9-CM Code: 759.89 - Specfied cong anomal NEC
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.

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.