1. ICD-10-CM Codes
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2. Q00-Q99
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3. Q80-Q89
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4. Q87-
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5. 2023 ICD-10-CM Diagnosis Code Q87.0

2023 ICD-10-CM Diagnosis Code Q87.0

Congenital malformation syndromes predominantly affecting facial appearance

2016 2017 2018 2019 2020 2021 2022 2023 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 2023 edition of ICD-10-CM Q87.0 became effective on October 1, 2022.
• This is the American ICD-10-CM version of Q87.0 - other international versions of ICD-10 Q87.0 may differ.

• 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

• 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.

• Q87.0 is considered exempt from POA reporting.

• 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

• 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
• 2021 (effective 10/1/2020): No change
• 2022 (effective 10/1/2021): No change
• 2023 (effective 10/1/2022): No change

• 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 2021 2022 2023 Non-Billable/Non-Specific Code

  Type 1 Excludes

  • acromegaly (E22.0)
  • Robin's syndrome (Q87.0)
  ICD-10-CM Diagnosis Code Q04

  Other congenital malformations of brain

  2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code

  Type 1 Excludes

  • cyclopia (Q87.0)
  • macrocephaly (Q75.3)
  ICD-10-CM Diagnosis Code Q10

  Congenital malformations of eyelid, lacrimal apparatus and orbit

  2016 2017 2018 2019 2020 2021 2022 2023 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 2021 2022 2023 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 2021 2022 2023 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 2021 2022 2023 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)

• 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 2021 2022 2023 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 2021 2022 2023 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 2021 2022 2023 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

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