2026 ICD-10-CM Diagnosis Code Q87.2Congenital malformation syndromes predominantly involving limbs

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q87

ICD-10-CM Q87.2
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q87.2 is a billable ICD-10-CM diagnosis code for congenital malformation syndromes predominantly involving limbs. 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
Q87.2
Billable Status
Yes — Valid for Submission
Code Describes
Congenital malformation syndromes predominantly involving limbs
Short Description
Congenital malformation syndromes predom involving limbs
Parent Code
Q87
Other specified congenital malformation syndromes affecting multiple systems

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ87Other specified congenital malformation syndromes affecting multiple systems
This CodeQ87.2Congenital malformation syndromes predominantly involving limbs

Present on Admission (POA)Billing

Q87.2 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.

  • 4q partial monosomy syndrome
  • 4q25 proximal deletion syndrome
  • Aase syndrome
  • Acrocardiofacial syndrome
  • Acrocephalopolysyndactyly
  • Acrocephalopolysyndactyly type III
  • Acrocephalosyndactyly
  • Acrocephalosyndactyly type V
  • Acrofrontofacionasal dysostosis type 2
  • Acropectoral syndrome
  • Acrorenal syndrome
  • Acrorenoocular syndrome
  • Adams-Oliver syndrome
  • ADULT syndrome
  • Amegakaryocytic thrombocytopenia
  • Anal atresia
  • Antecubital pterygium syndrome
  • Aplasia of bone of radius and/or ulna
  • Aplasia of radius
  • Ballard syndrome
  • Banki syndrome
  • Bent bone dysplasia group
  • Bilateral hearing loss
  • Brachydactyly of toes
  • Brachymesophalangia
  • Campomelia Cumming type
  • Camptodactyly and tall stature with scoliosis and hearing loss syndrome
  • Camptodactyly with fibrous tissue hyperplasia and skeletal dysplasia syndrome
  • Capra DeMarco syndrome
  • Catel Manzke syndrome
  • Caudal appendage deafness syndrome
  • Chiari malformation
  • Chiari malformation type I
  • CHILD syndrome
  • Conductive hearing loss of left ear
  • Conductive hearing loss of right ear
  • Conductive hearing loss, bilateral
  • Congenital abnormal shape of fibula
  • Congenital abnormal shape of tibia
  • Congenital absence of radius
  • Congenital anomaly of caudal vertebra
  • Congenital anomaly of patella
  • Congenital cleft hand
  • Congenital complete absence of bilateral lower limbs
  • Congenital complete absence of bilateral upper limbs
  • Congenital complete absence of left lower limb
  • Congenital complete absence of left upper limb
  • Congenital complete absence of lower limb
  • Congenital complete absence of right lower limb
  • Congenital complete absence of right upper limb
  • Congenital complete absence of upper limb
  • Congenital hypoplasia of bone of pelvis
  • Congenital hypoplasia of femur
  • Congenital hypoplastic anemia
  • Congenital ichthyosiform erythroderma
  • Congenital microgastria
  • Congenital microgastria with limb reduction defect syndrome
  • Congenital mixed conductive and sensorineural hearing loss
  • Congenital radioulnar synostosis
  • Constitutional aplastic anemia
  • Constriction ring syndrome
  • Cooks syndrome
  • Cryptomicrotia brachydactyly syndrome
  • Curry Jones syndrome
  • Deletion of part of chromosome 4
  • Disorder characterized by multiple exostoses
  • Duplication of fibula
  • Duplication of lower limb bone
  • Dystopia canthorum
  • EN1-related dorsoventral syndrome
  • Epileptic encephalopathy
  • Escobar syndrome
  • Femoral hypoplasia - unusual facies syndrome
  • Fetal sirenomelia
  • Fibular dimelia diplopodia syndrome
  • Fuhrmann syndrome
  • Grebe syndrome
  • Guttmacher syndrome
  • Hand-foot-genital syndrome
  • Holt-Oram syndrome
  • Hypoplastic anemia
  • Ichthyosiform erythroderma
  • Jackson-Weiss syndrome
  • Karsch Neugebauer syndrome
  • Langer-Giedion syndrome
  • Levy-Hollister syndrome
  • Limb body wall complex
  • Limb reduction-ichthyosis syndrome
  • Long thumb brachydactyly syndrome
  • Mesomelic dysplasia of lower limb
  • Mesomelic dysplasia, digital anomalies, intellectual disability syndrome
  • Mietens syndrome
  • Mirror polydactyly, vertebral segmentation and limb defect syndrome
  • Mixed conductive AND sensorineural hearing loss
  • Mixed conductive and sensorineural hearing loss of left ear
  • Mixed conductive and sensorineural hearing loss of right ear
  • Mixed conductive and sensorineural hearing loss, bilateral
  • Multiple malformation syndrome with facial-limb defects as major feature
  • Multiple malformation syndrome with limb defect as major feature
  • Multiple malformation syndrome, small stature, without skeletal dysplasia
  • Nager syndrome
  • Nail-patella syndrome
  • Nievergelt's syndrome
  • Oculootoradial syndrome
  • Patella dysplasia
  • PDE4D haploinsufficiency syndrome
  • Pelviscapular dysplasia
  • Pfeiffer syndrome type 1
  • Pfeiffer syndrome type 2
  • Pfeiffer syndrome type 3
  • PHAVER syndrome
  • Port-wine stain in Rubinstein-Taybi syndrome
  • Proximal deletion of long arm of chromosome 4
  • Radial aplasia-thrombocytopenia syndrome
  • RAPADILINO syndrome
  • Rubinstein-Taybi syndrome
  • Ruvalcaba syndrome
  • Saethre-Chotzen syndrome
  • Sagittal craniosynostosis
  • Sensorineural hearing loss of bilateral ears
  • Severe intellectual disability, hypotonia, strabismus, coarse face, planovalgus syndrome
  • Short rib dysplasia
  • Sirenomelus
  • Split foot
  • Spondylocamptodactyly syndrome
  • STAR syndrome
  • Tel Hashomer camptodactyly syndrome
  • Telecanthus
  • Temple Baraitser syndrome
  • Temtamy preaxial brachydactyly syndrome
  • Tetraamelia with multiple malformation syndrome
  • Thoracomelic dysplasia
  • Townes syndrome
  • Trichorhinophalangeal dysplasia type I
  • Trichorhinophalangeal dysplasia type III
  • Trichorhinophalangeal syndrome
  • Trichorhinophalangeal syndrome type 1 and 3
  • VATER association
  • Waardenburg syndrome
  • Waardenburg syndrome type 3
  • WT limb blood syndrome
  • X-linked intellectual disability Stevenson type

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Holt-Oram syndrome
  • Klippel-Trenaunay-Weber syndrome
  • Nail patella syndrome
  • Rubinstein-Taybi syndrome
  • Sirenomelia syndrome
  • Thrombocytopenia with absent radius TAR syndrome
  • VATER 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.

    • Fong's syndrome(hereditary osteo-onychodysplasia)
    • Holt-Oram syndrome
    • Klippel-Trenaunay(-Weber) syndrome
    • Mietens' syndrome
    • Nail
      • patella syndrome
    • Onycho-osteodysplasia
    • Osteo-onycho-arthro-dysplasia
    • Osteo-onychodysplasia, hereditary
    • Österreicher-Turner syndrome
    • Rubinstein-Taybi syndrome
    • Sirenomelia(syndrome)
    • Syndrome
      • Fong's
    • Syndrome
      • nail patella
    • Syndrome
      • Osterreicher-Turner
    • Syndrome
      • sirenomelia
    • Syndrome
      • TAR (thrombocytopenia with absent radius)
    • Syndrome
      • thrombocytopenia with absent radius (TAR)
    • Syndrome
      • VATER
    • TAR(thrombocytopenia with absent radius) syndrome
    • Taybi's syndrome
    • Thrombocytopenia, thrombocytopenic
      • with absent radius (TAR)
    • Turner-Kieser syndrome
    • VATER 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

  • 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.

  • Acrocephalosyndactyly

    a genetic disorder characterized by craniosynostosis and fusion of the fingers and toes.

  • TWIST1 Gene|TWIST1|TWIST1|Twist Homolog 1 (Acrocephalosyndactyly 3; Saethre-Chotzen Syndrome) (Drosophila) Gene

    this gene plays a role in regulation of transcription and the inhibition of apoptosis. it is also involved in the control of morphogenesis during embryonic development.

  • TWIST1 wt Allele|ACS3|BPES2|BPES3|SCS|TWIST|Twist Homolog 1 (Acrocephalosyndactyly 3; Saethre-Chotzen Syndrome) (Drosophila) wt Allele

    human twist1 wild-type allele is located in the vicinity of 17p13.3 and is approximately 16 kb in length. this allele, which encodes twist-related protein 1, plays a role in the regulation of both transcription and cell lineage determination. mutations in the gene are associated with saethre-chotzen, robinow-sorauf, and baller-gerold syndromes.

  • Twist-Related Protein 1|Acrocephalosyndactyly 3 Protein|Class A Basic Helix-Loop-Helix Protein 38|H-Twist|TWIST|TWIST1|TWIST1 Protein|Twist Homolog|Twist Homolog 1|Twist Related Protein 1|bHLHa38

    twist-related protein 1 (202 aa, ~21 kda) is encoded by the human twist1 gene. this protein plays a role in the negative regulation of both transcription and myogenesis.

  • Type I Acrocephalosyndactyly|Acrocephalosyndactyly Type I|Apert Syndrome

    an autosomal dominant inherited type of acrocephalosyndactyly caused by mutations in the fgfr2 gene. it is characterized by early closure of the sutures between the skull bones, bulging eyes, low-set ears, fusion of the second, third, and forth fingers, and fusion of the toes.

  • Type III Acrocephalosyndactyly|Acrocephalosyndactyly Type III|Saethre-Chotzen Syndrome|Saethre-Chotzen Syndrome

    a rare autosomal dominant syndrome caused by mutations in the twist1 gene. it is characterized by premature closure of skull bones resulting in abnormally shaped head, high forehead, hypertelorism, and facial asymmetry. it may be associated with fusion of certain fingers or toes.

  • Type V Acrocephalosyndactyly|Acrocephalosyndactyly Type V|Noack Syndrome|Pfeiffer Syndrome

    an autosomal dominant inherited type of acrocephalosyndactyly caused by mutations in the fgfr1 or fgfr2 genes. it is characterized by early closure of the sutures between the skull bones, bulging and wide-set eyes, broad thumbs, big toes, and partial syndactyly in the hands and toes.

  • Escobar Syndrome

    a rare congenital disorder, this is the non-lethal variant of multiple pterygium syndrome, characterized by orthopedic and craniofacial abnormalities, pterygium and akinethesia. the majority of cases are autosomal dominant.

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 Q87.2 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 HistoryHistory

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 Q87.2Overview

Is Q87.2 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report congenital malformation syndromes predominantly involving limbs on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q87.2 group to?

When congenital malformation syndromes predominantly involving limbs 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 Q87.2 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 congenital malformation syndromes predominantly involving limbs on inpatient claims.

What is the ICD-9 equivalent of Q87.2?

Under the General Equivalence Mappings, congenital malformation syndromes predominantly involving limbs 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.