ICD-10-CM Code Q87.0

Congenital malformation syndromes predominantly affecting facial appearance

Version 2020 Billable Code POA Exempt

Valid for Submission

Q87.0 is a billable code used to specify a medical diagnosis of congenital malformation syndromes predominantly affecting facial appearance. The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code Q87.0 might also be used to specify conditions or terms like aase smith type 1 syndrome, abducens nerve palsy, absence of clavicle, acrocallosal syndrome, acrocephalopolysyndactyly, acrocephalopolysyndactyly type ii, etc The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Short Description:Congen malform syndromes predom affecting facial appearance
Long Description:Congenital malformation syndromes predominantly affecting facial appearance

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code Q87.0:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • Acrocephalopolysyndactyly
  • Acrocephalosyndactyly Apert
  • Cryptophthalmos syndrome
  • Cyclopia
  • Goldenhar syndrome
  • Moebius syndrome
  • Oro-facial-digital syndrome
  • Robin syndrome
  • Whistling face

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code Q87.0 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Aase Smith type 1 syndrome
  • Abducens nerve palsy
  • Absence of clavicle
  • Acrocallosal syndrome
  • Acrocephalopolysyndactyly
  • Acrocephalopolysyndactyly type II
  • Acrocephalopolysyndactyly type III
  • Acrocephalopolysyndactyly type IV
  • Acrocephalosyndactyly
  • Acrocephalosyndactyly type I
  • Acrocephalosyndactyly type V
  • Acromegaloid facial appearance syndrome
  • Acromelic frontonasal dysplasia
  • Acro-oto-ocular syndrome
  • Acrorenal mandibular syndrome
  • Aglossia-adactyly syndrome
  • AHDC1-related intellectual disability, obstructive sleep apnea, mild dysmorphism syndrome
  • Ambiguous genitalia
  • Aortic arch anomaly, facial dysmorphism, intellectual disability syndrome
  • Arachnodactyly
  • Arachnodactyly
  • Arachnodactyly and intellectual disability with facial dysmorphism syndrome
  • Asymmetry of mandible
  • Auriculo-condylar syndrome
  • Autism and facial port-wine stain syndrome
  • Axonal neuropathy
  • Bilateral hearing loss
  • Binder syndrome
  • Blepharonasofacial malformation syndrome
  • BNAR syndrome
  • Brachycephaly
  • Brachytelephalangy, facial dysmorphism, Kallmann syndrome
  • Cardio-acral-facial syndrome
  • Cardio-facio-cutaneous syndrome
  • Central obesity
  • Cerebrofacioarticular syndrome
  • Cerebro-facio-thoracic dysplasia
  • Cerebrooculonasal syndrome
  • Char syndrome
  • Charlie M syndrome
  • Chudley Lowry Hoar syndrome
  • Cleft mandible
  • CODAS syndrome
  • Combined immunodeficiency with faciooculoskeletal anomalies syndrome
  • Congenital anomaly of aortic arch AND/OR descending aorta
  • Congenital asymmetry of jaw
  • Congenital atrophy of optic nerve
  • Congenital conductive hearing loss
  • Congenital cubitus valgus
  • Congenital dislocation of hip
  • Congenital disorder of facial nerve
  • Congenital disorder of facial nerve
  • Congenital disorder of facial nerve
  • Congenital enlarged kidney
  • Congenital facial asymmetry
  • Congenital facial nerve palsy
  • Congenital facial nerve palsy
  • Congenital facial nerve palsy
  • Congenital hydronephrosis
  • Congenital hypertrichosis
  • Congenital hypoplasia of ulna
  • Congenital hypotrichia
  • Congenital livedo reticularis
  • Congenital malformation syndromes affecting facial appearance
  • Congenital mandibular asymmetry
  • Congenital nonprogressive myopathy with Moebius and Robin sequences
  • Congenital omphalocele
  • Congenital sixth nerve palsy
  • Congenital stenosis of carotid artery
  • Connective tissue disorder due to lysyl hydroxylase-3 deficiency
  • Contracture with ectodermal dysplasia and orofacial cleft syndrome
  • Cortical blindness
  • Crane Heise syndrome
  • Craniodigital syndrome and intellectual disability syndrome
  • Craniofacial dysplasia osteopenia syndrome
  • Craniofacial dyssynostosis syndrome
  • Craniofaciofrontodigital syndrome
  • Craniofrontonasal dysplasia
  • Craniomicromelic syndrome
  • Cranio-orbito-ocular dysraphia syndrome
  • Cryptophthalmos syndrome
  • Cutaneous capillary malformation
  • Cyclops
  • Cyclops hypognathus
  • Cyprus facial neuromusculoskeletal syndrome
  • Dandy-Walker syndrome
  • Dandy-Walker syndrome
  • Deafness and intellectual disability Martin Probst type syndrome
  • Developmental hereditary disorder
  • Developmental malformation of branchial arch
  • Dextrotransposition of aorta
  • Diencephalic mesencephalic junction dysplasia
  • Dislocation of hip and facial dysmorphism syndrome
  • Ear, face and neck congenital anomalies
  • Early-onset epileptic encephalopathy, cortical blindness, intellectual disability, facial dysmorphism syndrome
  • Edinburgh malformation syndrome
  • Emery Nelson syndrome
  • Epiphyseal dysplasia, hearing loss, dysmorphism syndrome
  • Facial asymmetry
  • Facial dysmorphism, cleft palate, loose skin syndrome
  • Facial dysmorphism, conductive hearing loss, heart defect syndrome
  • Facial dysmorphism, immunodeficiency, livedo, short stature syndrome
  • Facial dysmorphism, lens dislocation, anterior segment abnormalities, spontaneous filtering bleb syndrome
  • Facial dysmorphism, macrocephaly, myopia, Dandy-Walker malformation syndrome
  • Facial milia, lobate tongue, lingual and labial frenula syndrome
  • Facio-auriculo-vertebral spectrum
  • Familial aplasia of the vermis
  • Familial omphalocele syndrome with facial dysmorphism
  • Filippi syndrome
  • Fine Lubinsky syndrome
  • First and second branchial arch syndrome
  • First arch syndrome
  • Flat face, microstomia, ear anomaly syndrome
  • Frank-Ter Haar syndrome
  • Freeman-Sheldon syndrome
  • Frontonasal dysplasia sequence
  • Frontonasal dysplasia, severe microphthalmia, severe facial clefting syndrome
  • Fryns macrocephaly
  • Fryns syndrome
  • Goldberg Shprintzen megacolon syndrome
  • Goldenhar syndrome
  • Gollop syndrome
  • Gorlin-Chaudhry-Moss syndrome
  • Greig cephalopolysyndactyly syndrome
  • Grob's syndrome
  • Growth retardation
  • Hadziselimovic syndrome
  • Hall Riggs syndrome
  • Hallermann-Streiff syndrome
  • Hanhart's syndrome
  • Harrod syndrome
  • Holmes Gang syndrome
  • Hypertelorism
  • Hypertrichosis and acromegaloid facial appearance syndrome
  • Hypogonadotropic hypogonadism, severe microcephaly, sensorineural hearing loss, dysmorphism syndrome
  • Illum syndrome
  • Immuno-osseous dysplasia
  • Intellectual disability Wolff type
  • Intellectual disability, brachydactyly, Pierre Robin syndrome
  • Intellectual disability, craniofacial dysmorphism, cryptorchidism syndrome
  • Intellectual disability, craniofacial dysmorphism, hypogonadism, diabetes mellitus syndrome
  • Intellectual disability, facial dysmorphism, hand anomalies syndrome
  • Intellectual disability, obesity, brain malformation, facial dysmorphism syndrome
  • Intellectual disability, severe speech delay, mild dysmorphism syndrome
  • Internal carotid artery stenosis
  • Isolated hereditary congenital facial paralysis
  • Isotretinoin-like syndrome
  • Jawad syndrome
  • Joubert syndrome
  • Joubert syndrome with orofaciodigital defect
  • Juberg Marsidi syndrome
  • Kagami Ogata syndrome
  • Kapur Toriello syndrome
  • Keipert syndrome
  • King Denborough syndrome
  • Koolen De Vries syndrome
  • Larsen-like syndrome B3GAT3 type
  • Lentiglobus
  • Lethal faciocardiomelic dysplasia
  • Lethal polymalformative syndrome Boissel type
  • Livedo reticularis
  • Lymphedema of lower extremity
  • Lymphedema, atrial septal defect, facial changes syndrome
  • Malformation defect spectrum
  • McDonough syndrome
  • Megalocornea
  • Mehes syndrome
  • Melnick-Fraser syndrome
  • Microcephalic primordial dwarfism Montreal type
  • Microcephaly, short stature, intellectual disability, facial dysmorphism syndrome
  • Microstomia
  • Microtia
  • Mietens syndrome
  • Miller syndrome
  • Moebius syndrome
  • Moebius syndrome, axonal neuropathy, hypogonadotropic hypogonadism syndrome
  • Mohr syndrome
  • Mowat-Wilson syndrome
  • Moyamoya angiopathy, short stature, facial dysmorphism, hypergonadotropic hypogonadism syndrome
  • Moyamoya disease
  • Multicentric osteolysis nodulosis arthropathy spectrum
  • Multiple malformation syndrome with facial defects as major feature
  • Multiple malformation syndrome with facial-limb defects as major feature
  • Multiple malformation syndrome, moderate short stature, facial
  • Multiple malformation syndrome, small stature, without skeletal dysplasia
  • Nager syndrome
  • Nijmegen breakage syndrome-like disorder
  • Obstructive sleep apnea syndrome
  • Occipital encephalocele
  • Oculocerebrofacial syndrome Kaufman type
  • Oculodento-osseous dysplasia
  • Oculomaxillofacial dysostosis
  • Oculo-palato-digital syndrome
  • Ogden syndrome
  • Okamoto syndrome
  • Ophthalmomandibulomelic dysplasia
  • Opocephalus
  • Oral-facial-digital syndrome
  • Oro-facial digital syndrome type 1
  • Oro-facial digital syndrome type 10
  • Oro-facial digital syndrome type 11
  • Oro-facial digital syndrome type 12
  • Oro-facial digital syndrome type 13
  • Oro-facial digital syndrome type 14
  • Oro-facial digital syndrome type 5
  • Oro-facial digital syndrome type 8
  • Orofacial-digital syndrome III
  • Orofacial-digital syndrome IV
  • Oromandibular-limb hypogenesis spectrum
  • Osteopenia, myopia, hearing loss, intellectual disability, facial dysmorphism syndrome
  • Otocephalic syndrome
  • Otofaciocervical syndrome
  • Oto-palato-digital syndrome, type I
  • Oto-palato-digital syndrome, type II
  • Pallister W syndrome
  • Pelviscapular dysplasia
  • Peripheral axonal neuropathy
  • Perlman syndrome
  • Pierre Robin sequence faciodigital anomaly syndrome
  • Pilotto syndrome
  • Pitt Hopkins-like syndrome
  • Poliosis
  • Port-wine stain of skin
  • Postaxial polydactyly, anterior pituitary anomalies, facial dysmorphism syndrome
  • Prieto Badia Mulas syndrome
  • Pseudoaminopterin syndrome
  • Pseudopapilledema
  • Ramos Arroyo syndrome
  • Renier Gabreels Jasper syndrome
  • Right aortic arch
  • Robin sequence
  • Robin sequence
  • Robin sequence
  • Robin sequence and oligodactyly syndrome
  • Roifman syndrome
  • Rozin Hertz Goodman syndrome
  • Ruvalcaba syndrome
  • SCARF syndrome
  • Schilbach Rott syndrome
  • Seaver Cassidy syndrome
  • Severe intellectual disability, poor language, strabismus, grimacing face, long fingers syndrome
  • Severe intellectual disability, short stature, behavioral abnormalities, facial dysmorphism syndrome
  • Short stature, onychodysplasia, facial dysmorphism, hypotrichosis syndrome
  • Short stature, Pierre Robin sequence, cleft mandible, hand anomalies, clubfoot syndrome
  • Short stature, unique facies, enamel hypoplasia, progressive joint stiffness, high-pitched voice syndrome
  • Short ulna, dysmorphism, hypotonia, intellectual disability syndrome
  • Sixth cranial nerve finding
  • Smith Fineman Myers syndrome
  • Sonoda syndrome
  • Speech delay
  • Spherophakia
  • Steinfeld syndrome
  • Summitt syndrome
  • Syndromic multisystem autoimmune disease due to ITCH deficiency
  • TARP syndrome
  • Teebi Shaltout syndrome
  • Temple syndrome
  • Temtamy syndrome
  • Thakker Donnai syndrome
  • THOC6-related developmental delay-microcephaly-facial dysmorphism syndrome
  • Tongue absent
  • Toriello Carey syndrome
  • Townes syndrome
  • Van den Ende-Gupta syndrome
  • Velofacioskeletal syndrome
  • Ventricular extrasystoles with syncope, perodactyly and Robin sequence syndrome
  • Ventricular premature beats
  • Weill-Marchesani syndrome
  • White forelock with malformations syndrome
  • Wildervanck's syndrome
  • Wilson Turner syndrome
  • X-linked intellectual disability and epilepsy with progressive joint contracture and facial dysmorphism syndrome
  • X-linked intellectual disability and hypotonia with facial dysmorphism and aggressive behavior syndrome
  • X-linked intellectual disability with cubitus valgus and dysmorphism syndrome
  • Zechi Ceide syndrome

Diagnostic Related Groups

The ICD-10 code Q87.0 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.


Present on Admission (POA)

Q87.0 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here .

CMS POA Indicator Options and Definitions
POA Indicator CodePOA Reason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q87.0 to ICD-9

  • 755.55 - Acrocephalosyndactyly (Approximate Flag)
  • 756.0 - Anomal skull/face bones (Approximate Flag)

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • Other congenital malformations (Q80-Q89)
      • Oth congenital malform syndromes affecting multiple systems (Q87)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

Craniofacial Abnormalities

Craniofacial is a medical term that relates to the bones of the skull and face. Craniofacial abnormalities are birth defects of the face or head. Some, like cleft lip and palate, are among the most common of all birth defects. Others are very rare. Most of them affect how a person's face or head looks. These conditions may also affect other parts of the body.

Treatment depends on the type of problem. Plastic and reconstructive surgery may help the person's appearance.

[Learn More]