2020 ICD-10-CM Diagnosis Code Q87.2

Congenital malformation syndromes predominantly involving limbs

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q87.2 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • Short description: Congenital malformation syndromes predom involving limbs
  • The 2020 edition of ICD-10-CM Q87.2 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q87.2 - other international versions of ICD-10 Q87.2 may differ.
Applicable To
  • Holt-Oram syndrome
  • Klippel-Trenaunay-Weber syndrome
  • Nail patella syndrome
  • Rubinstein-Taybi syndrome
  • Sirenomelia syndrome
  • Thrombocytopenia with absent radius [TAR] syndrome
  • VATER syndrome
The following code(s) above Q87.2 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to Q87.2:
  • Q00-Q99
    2020 ICD-10-CM Range Q00-Q99

    Congenital malformations, deformations and chromosomal abnormalities

    Note
    • Codes from this chapter are not for use on maternal records
    Type 2 Excludes
    • inborn errors of metabolism (E70-E88)
    Congenital malformations, deformations and chromosomal abnormalities
  • Q87
    ICD-10-CM Diagnosis Code Q87

    Other specified congenital malformation syndromes affecting multiple systems

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Use Additional
    • code(s) to identify all associated manifestations
    Other specified congenital malformation syndromes affecting multiple systems
Approximate Synonyms
  • Klippel trenaunay weber syndrome
  • Radial aplasia-thrombocytopenia syndrome
  • Rubinstein taybi syndrome
  • Thrombocytopenia-absent radius (tar) syndrome
  • Vater association
Clinical Information
  • A chromosomal disorder characterized by mental retardation, broad thumbs, webbing of fingers and toes, beaked nose, short upper lip, pouting lower lip, agenesis of corpus callosum, large foramen magnum, keloid formation, pulmonary stenosis, vertebral anomalies, chest wall anomalies, sleep apnea, and megacolon. The disease has an autosomal dominant pattern of inheritance and is associated with deletions of the short arm of chromosome 16 (16p13.3).
  • A congenital disorder that is characterized by a triad of capillary malformations (hemangioma), venous malformations (arteriovenous fistula), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the vg5q gene which encodes a strong angiogenesis stimulator.
  • A rare autosomal dominant syndrome caused by mutations in the lmx1b gene. It is characterized by fingernail deformities, absence or hypoplasia of the patellae, iliac horns, deformities of the radial heads, nephropathy, and glaucoma.
  • A rare genetic syndrome mapped to chromosome 16p13.3 and associated with mutations in the crebbp gene. It is characterized by mental and growth retardation, distinctive facial features (prominent nose, low-set ears, microcephaly, and small mouth), and broad thumbs and great toes. Patients are at an increased risk of developing benign and malignant neoplasms, including nervous system neoplasms and malignant lymphoproliferative disorders.
  • A rare syndrome characterized by the presence of port-wine stain (a vascular malformation), varicose veins, and hypertrophy of the soft tissues and bones in an extremity. It usually affects one extremity, most commonly a leg.
  • A rare syndrome characterized by the presence of thrombocytopenia associated with bilateral absence of the radius bone.
  • A syndrome of broad thumbs and halluces, typical dysmorphic facies, delayed mental and physical development, pulmonary stenosis, large foramen magnum, vertebral and sternal deformities, dermatoglyphic changes, and other anomalies.
  • A syndrome of multiple abnormalities characterized by the absence or hypoplasia of the patella and congenital nail dystrophy. It is a genetically determined autosomal dominant trait.
  • Characterized by malformations of bones of the thumbs, hands and forearms and/or abnormalities of the heart; in many infants, the thumbs and wrists may be absent, underdeveloped or have an extra bone; scapulae, clavicles, and/or other bones may also be abnormal.
  • Hemihypertrophy of the soft and bone tissue, hemangiomata, and varicose veins form the basic triad of this syndrome. Unilateral lesions of the legs with cutaneous and subcutaneous hemangiomas, varicosities, phlebectasis and occasional arteriovenous fistulae were emphasized in earlier reports, but later findings indicate involvement of many other parts of the body. Vascular anomalies may be present at birth or may appear in infancy. Occasional associated abnormalities may include lymphangiomatous anomalies, limb deformities, cutis marmorata, micro- or macrocephaly, eye diseases, and craniofacial deformities. Most patients have normal mentality, except when vascular lesions invade the cerebral tissue. In the absence of arteriovenous fistulae, the syndrome is often referred to as weber syndrome or parkes weber syndrome. Klippel-trenaunay-weber syndrome is associated in some cases with sturge-weber angiomatosis.
Present On Admission
POA Help
"Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are considered POA.
  • Q87.2 is considered exempt from POA reporting.
ICD-10-CM Q87.2 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 564 Other musculoskeletal system and connective tissue diagnoses with mcc
  • 565 Other musculoskeletal system and connective tissue diagnoses with cc
  • 566 Other musculoskeletal system and connective tissue diagnoses without cc/mcc

Convert Q87.2 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to Q87.2:
  • Code First: D69.42
    ICD-10-CM Diagnosis Code D69.42

    Congenital and hereditary thrombocytopenia purpura

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Congenital thrombocytopenia
    • Hereditary thrombocytopenia
    Code First
    • congential or hereditary disorder, such as:
    • thrombocytopenia with absent radius (TAR syndrome) (Q87.2)
  • Type 1 Excludes: Q74.1
    , Q84.3
    ICD-10-CM Diagnosis Code Q74.1

    Congenital malformation of knee

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital absence of patella
    • Congenital dislocation of patella
    • Congenital genu valgum
    • Congenital genu varum
    • Rudimentary patella
    Type 1 Excludes
    • congenital dislocation of knee (Q68.2)
    • congenital genu recurvatum (Q68.2)
    • nail patella syndrome (Q87.2)
    ICD-10-CM Diagnosis Code Q84.3

    Anonychia

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Type 1 Excludes
    • nail patella syndrome (Q87.2)
  • Type 2 Excludes: N07
    ICD-10-CM Diagnosis Code N07

    Hereditary nephropathy, not elsewhere classified

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 2 Excludes
    • Alport's syndrome (Q87.81-)
    • hereditary amyloid nephropathy (E85.-)
    • nail patella syndrome (Q87.2)
    • non-neuropathic heredofamilial amyloidosis (E85.-)

Diagnosis Index entries containing back-references to Q87.2:
  • Fong's syndrome Q87.2 (hereditary osteo-onychodysplasia)
  • Holt-Oram syndrome Q87.2
  • Klippel-Trenaunay Q87.2 (-Weber)
  • Mietens' syndrome Q87.2
  • Nail - see also condition
    • patella syndrome Q87.2
  • Onycho-osteodysplasia Q87.2
  • Osteo-onycho-arthro-dysplasia Q87.2
  • Osteo-onychodysplasia, hereditary Q87.2
  • Österreicher-Turner syndrome Q87.2
  • Rubinstein-Taybi syndrome Q87.2
  • Sirenomelia Q87.2 (syndrome)
  • Syndrome - see also Disease
    • Fong's Q87.2
    • nail patella Q87.2
    • Osterreicher-Turner Q87.2
    • sirenomelia Q87.2
    • TAR Q87.2 (thrombocytopenia with absent radius)
    • thrombocytopenia with absent radius Q87.2 (TAR)
    • VATER Q87.2
  • TAR Q87.2 (thrombocytopenia with absent radius)
  • Taybi's syndrome Q87.2
  • Thrombocytopenia, thrombocytopenic D69.6
    ICD-10-CM Diagnosis Code D69.6

    Thrombocytopenia, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • with absent radius Q87.2 (TAR)
  • Turner-Kieser syndrome Q87.2
  • VATER syndrome Q87.2

ICD-10-CM Codes Adjacent To Q87.2
Q86 Congenital malformation syndromes due to known exogenous causes, not elsewhere classified
Q86.0 Fetal alcohol syndrome (dysmorphic)
Q86.1 Fetal hydantoin syndrome
Q86.2 Dysmorphism due to warfarin
Q86.8 Other congenital malformation syndromes due to known exogenous causes
Q87 Other specified congenital malformation syndromes affecting multiple systems
Q87.0 Congenital malformation syndromes predominantly affecting facial appearance
Q87.1 Congenital malformation syndromes predominantly associated with short stature
Q87.11 Prader-Willi syndrome
Q87.19 Other congenital malformation syndromes predominantly associated with short stature
Q87.2 Congenital malformation syndromes predominantly involving limbs
Q87.3 Congenital malformation syndromes involving early overgrowth
Q87.4 Marfan's syndrome
Q87.40 …… unspecified
Q87.41 Marfan's syndrome with cardiovascular manifestations
Q87.410 Marfan's syndrome with aortic dilation
Q87.418 Marfan's syndrome with other cardiovascular manifestations
Q87.42 …… with ocular manifestations
Q87.43 …… with skeletal manifestation
Q87.5 Other congenital malformation syndromes with other skeletal changes
Q87.8 Other specified congenital malformation syndromes, not elsewhere classified

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.