2027 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 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

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 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

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
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 the other POA exempt codes in Other congenital malformations (Q80-Q89).

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
  • 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 atresia of external ear
  • 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 scoliosis deformity of spine
  • 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
  • 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 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 congenital anomalies, neurodevelopmental delay, ocular abnormalities syndrome
  • 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
  • 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
  • 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

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q87.89: its own notes plus those printed at Q87.8, Q87, and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Q87.8 Other specified congenital malformation syndromes, not elsewhere classified applies to 9 codes

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.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From Q87 Other specified congenital malformation syndromes affecting multiple systems applies to 21 codes
  • code(s) to identify all associated manifestations

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q87.89, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming Q87.89: they are not reported together with it, unless the two conditions are unrelated.

  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q75 Other congenital malformations of skull and face bones
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q89.7 Multiple congenital malformations, not elsewhere classified
    congenital malformation syndromes affecting multiple systems (Q87.-) names Q87.-, which includes this code

Index to Diseases and InjuriesGuidance

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

  • Birt-Hogg-Dube Syndrome

    autosomal dominant neoplastic syndrome characterised by genodermatosis, lung cysts, spontaneous and recurrent pneumothorax; and renal cancer. it is associated with mutations in the folliculin protein gene (flcn protein).
  • Zellweger Syndrome

    an autosomal recessive disorder due to defects in peroxisome biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; muscle hypotonia; sensorineural hearing loss; visual compromise; seizures; progressive degeneration of the kidneys and the liver. zellweger-like syndrome refers to phenotypes resembling the neonatal zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.

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 2027 code set by:

  • Q87.A - Loeys-Dietz syndrome
FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentExpanded into more specific codes in the current code setEffective October 1, 2026 through September 30, 2027.

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, 2026 through September 30, 2027.

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, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q87.89 a CC or MCC?

CMS lists Q87.89 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 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. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Can Q87.89 be reported with E71.510?

Not as a rule. The Excludes1 note at Q87.8, which applies to Q87.89, lists "Zellweger syndrome (E71.510)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).