2020 ICD-10-CM Diagnosis Code Q87.0

Congenital malformation syndromes predominantly affecting facial appearance

    2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
  • Q87.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • Short description: Congen malform syndromes predom affecting facial appearance
  • The 2020 edition of ICD-10-CM Q87.0 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of Q87.0 - other international versions of ICD-10 Q87.0 may differ.
Applicable To
  • Acrocephalopolysyndactyly
  • Acrocephalosyndactyly [Apert]
  • Cryptophthalmos syndrome
  • Cyclopia
  • Goldenhar syndrome
  • Moebius syndrome
  • Oro-facial-digital syndrome
  • Robin syndrome
  • Whistling face
The following code(s) above Q87.0 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 Q87.0:
  • 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
  • Acrocephalosyndactyly type i
  • Acrocephalosyndactyly type iii
  • Acrocephalosyndactyly type v
  • Aperts syndrome
  • Branchio oto renal syndrome
  • Carpenter's syndrome
  • Carpenters syndrome
  • Cryptophthalmos syndrome
  • Cryptophthalmos syndrome (eye condition)
  • Freeman sheldon syndrome
  • Goldenhar syndrome
  • Gorlin-chaudhry-moss syndrome
  • Melnick-fraser syndrome
  • Mobius syndrome
  • Oromandibular-limb hypogenesis spectrum
  • Oto palato digital syndrome, type 1
  • Oto palato digital syndrome, type 2
  • Oto-palato-digital syndrome, type i
  • Oto-palato-digital syndrome, type ii
  • Pfeiffer syndrome
  • Pierre robin syndrome
  • Robin sequence
  • Robin syndrome
  • Saethre chotzen syndrome
Clinical Information
  • A complex syndrome characterized mainly by aural, oral, and mandibular developmental anomalies which may vary from mild to severe and frequently involve one side of the body. Vertebral anomalies, epibulbar epidermoids, mental retardation, and numerous other anomalies are frequently associated. Orofacial anomalies differ from those in mandibulofacial dysostosis which are usually symmetrical. The presence of hypertelorism may indicate aneuploidy.
  • A congenital birth defect characterized by incomplete development or absence of face structures, usually affecting one side of the face. The defects include partially formed or absent ear, nose, lip, mandible, and/or soft palate.
  • A rare congenital malformation characterized by micrognathia, posterior retraction of the tongue, and cleft palate.
  • A rare syndrome that is inherited in an autosomal dominant or recessive pattern and caused by mutations in the myh3 gene. It is a severe form of arthrogryposis. It is characterized by the presence of distinctive facial features (small mouth, midface hypoplasia, short nose, drooping of the eyelids, deep folds in the area between the nose and the lips, and strabismus), joint deformities that lead to permanently bent fingers and toes, club foot, scoliosis, and walking difficulties.
  • A rare, genetically heterogenous syndrome, characterized by cryptophthalmos, craniofacial abnormalities, urogenital abnormalities, and syndactyly.
  • A syndrome of congenital facial paralysis, frequently associated with abducens palsy and other congenital abnormalities including lingual palsy, clubfeet, brachial disorders, cognitive deficits, and pectoral muscle defects. Pathologic findings are variable and include brain stem nuclear aplasia, facial nerve aplasia, and facial muscle aplasia, consistent with a multifactorial etiology. (Adams et al., Principles of Neurology, 6th ed, p1020)
  • A very rare congenital syndrome affecting the sixth and seventh cranial nerves. It is characterized by facial paralysis leading to lack of facial expression and the inability to move the eyes laterally.
  • Abnormalities of the orodental, facial, renal, and digital structures occurring in various combinations and frequently associated with retarded mental development. Patients in the original report were all females, except for the case with klinefelter syndrome
  • An association of cryptophthalmos with a wide range of abnormalities, consisting mostly of orofacial defects, laryngeal stenosis, syndactyly, decreased number of digits, renal dysplasia, and hypogenitalism. A small number of cases consist of only acrofacial and urogenital malformations without cryptophthalmos.
  • 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.
  • Congenital malformation characterized by micrognathia, glossoptosis and cleft palate. The mandibular abnormalities often result in difficulties in sucking and swallowing. The syndrome may be isolated or associated with other syndromes (e.g., andersen syndrome; campomelic dysplasia). Developmental mis-expression of sox9 transcription factor gene on chromosome 17q and its surrounding region is associated with the syndrome.
  • Rare autosomal recessive congenital malformation syndrome characterized by cryptophthalmos, syndactyly and urogenital abnormalities. Other anomalies of bone, ear, lung, and nose are common. Mutations on fras1 and frem2 are associated with the syndrome.
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.
  • Q87.0 is considered exempt from POA reporting.
ICD-10-CM Q87.0 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 Q87.0 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 Q87.0:
  • Type 1 Excludes: M26.0
    , Q04
    , Q10
    , Q11.2
    , Q18
    , Q18
    ICD-10-CM Diagnosis Code M26.0

    Major anomalies of jaw size

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    ICD-10-CM Diagnosis Code Q04

    Other congenital malformations of brain

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    ICD-10-CM Diagnosis Code Q10

    Congenital malformations of eyelid, lacrimal apparatus and orbit

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • cryptophthalmos NOS (Q11.2)
    • cryptophthalmos syndrome (Q87.0)
    ICD-10-CM Diagnosis Code Q11.2

    Microphthalmos

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Cryptophthalmos NOS
    • Dysplasia of eye
    • Hypoplasia of eye
    • Rudimentary eye
    Type 1 Excludes
    • cryptophthalmos syndrome (Q87.0)
    ICD-10-CM Diagnosis Code Q18

    Other congenital malformations of face and neck

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • cleft lip and cleft palate (Q35-Q37)
    • conditions classified to Q67.0-Q67.4
    • congenital malformations of skull and face bones (Q75.-)
    • cyclopia (Q87.0)
    • dentofacial anomalies [including malocclusion] (M26.-)
    • malformation syndromes affecting facial appearance (Q87.0)
    • persistent thyroglossal duct (Q89.2)
    ICD-10-CM Diagnosis Code Q18

    Other congenital malformations of face and neck

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • cleft lip and cleft palate (Q35-Q37)
    • conditions classified to Q67.0-Q67.4
    • congenital malformations of skull and face bones (Q75.-)
    • cyclopia (Q87.0)
    • dentofacial anomalies [including malocclusion] (M26.-)
    • malformation syndromes affecting facial appearance (Q87.0)
    • persistent thyroglossal duct (Q89.2)

Diagnosis Index entries containing back-references to Q87.0:
  • Acrocephalopolysyndactyly Q87.0
  • Acrocephalosyndactyly Q87.0
  • Aglossia-adactylia syndrome Q87.0
  • Apert's syndrome Q87.0
  • Carpenter's syndrome Q87.0
  • Cryptophthalmos Q11.2
    ICD-10-CM Diagnosis Code Q11.2

    Microphthalmos

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Cryptophthalmos NOS
    • Dysplasia of eye
    • Hypoplasia of eye
    • Rudimentary eye
    Type 1 Excludes
    • cryptophthalmos syndrome (Q87.0)
    • syndrome Q87.0
  • Cyclopia, cyclops Q87.0
  • Cyclopism Q87.0
  • Dyscranio-pygo-phalangy Q87.0
  • Dysplasia - see also Anomaly
    • oculodentodigital Q87.0
  • Fraser's syndrome Q87.0
  • Freeman Sheldon syndrome Q87.0
  • Goldenhar Q87.0 (-Gorlin)
  • Gorlin-Chaudry-Moss syndrome Q87.0
  • Hallerman-Streiff syndrome Q87.0
  • Hanhart's syndrome Q87.0
  • Marchesani Q87.0 (-Weill)
  • Meyer-Schwickerath and Weyers syndrome Q87.0
  • Moebius, Möbius
    • syndrome Q87.0
      • congenital oculofacial paralysis Q87.0 (with other anomalies)
  • Mohr's syndrome Q87.0 (Types I and II)
  • Papillon-Léage and Psaume syndrome Q87.0
  • Paralysis, paralytic (complete) (incomplete) G83.9
    ICD-10-CM Diagnosis Code G83.9

    Paralytic syndrome, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • oculofacial, congenital Q87.0 (Moebius)
  • Pierre Robin deformity or syndrome Q87.0
  • Robin Q87.0 (-Pierre)
  • Syndrome - see also Disease
    • congenital
      • facial diplegia Q87.0
      • oculo-auriculovertebral Q87.0
      • oculofacial diplegia Q87.0 (Moebius)
    • cryptophthalmos Q87.0
    • eyelid-malar-mandible Q87.0
    • first arch Q87.0
    • micrognathia-glossoptosis Q87.0
    • oral-facial-digital Q87.0
    • oro-facial-digital Q87.0
    • oto-palatal-digital Q87.0
    • whistling face Q87.0
  • Tower skull Q75.0
    ICD-10-CM Diagnosis Code Q75.0

    Craniosynostosis

      2016 2017 2018 2019 2020 Billable/Specific Code POA Exempt
    Applicable To
    • Acrocephaly
    • Imperfect fusion of skull
    • Oxycephaly
    • Trigonocephaly
    • with exophthalmos Q87.0
  • Ullrich-Feichtiger syndrome Q87.0
  • Whistling face Q87.0

ICD-10-CM Codes Adjacent To Q87.0
Q85.09 Other neurofibromatosis
Q85.1 Tuberous sclerosis
Q85.8 Other phakomatoses, not elsewhere classified
Q85.9 Phakomatosis, unspecified
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

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