ICD-10-CM Code Q75.4

Mandibulofacial dysostosis

Version 2021 Billable Code POA Exempt

Valid for Submission

Q75.4 is a billable code used to specify a medical diagnosis of mandibulofacial dysostosis. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code Q75.4 might also be used to specify conditions or terms like first arch syndrome, hypertelorism, intermandibular dysostosis, mandibulofacial dysostosis with microcephaly, mandibulofacial dysostosis, macroblepharon, macrostomia syndrome, otomandibular dysostosis, etc The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Short Description:Mandibulofacial dysostosis
Long Description:Mandibulofacial dysostosis

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 Q75.4:

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.
  • Franceschetti syndrome
  • Treacher Collins syndrome

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 Q75.4 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:

  • First arch syndrome
  • Hypertelorism
  • Intermandibular dysostosis
  • Mandibulofacial dysostosis with microcephaly
  • Mandibulofacial dysostosis, macroblepharon, macrostomia syndrome
  • Otomandibular dysostosis
  • Patterson Stevenson Fontaine syndrome
  • Sphenoidal dysostosis
  • Temporo-auro-mandibular dysostosis
  • Treacher Collins syndrome
  • X-linked mandibulofacial dysostosis

Clinical Information

  • MANDIBULOFACIAL DYSOSTOSIS-. a hereditary disorder occurring in two forms: the complete form franceschetti's syndrome is characterized by antimongoloid slant of the palpebral fissures coloboma of the lower lid micrognathia and hypoplasia of the zygomatic arches and congenital microtia. it is transmitted as an autosomal trait. the incomplete form treacher collins syndrome is characterized by the same anomalies in less pronounced degree. it occurs sporadically but an autosomal dominant mode of transmission is suspected. dorland 27th ed

Diagnostic Related Groups

The ICD-10 code Q75.4 is grouped in the following groups for version MS-DRG V38.0 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 10/01/2020 through 09/30/2021.


Present on Admission (POA)

Q75.4 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 Q75.4 to ICD-9

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

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • Congenital malformations and deformations of the musculoskeletal system (Q65-Q79)
      • Other congenital malformations of skull and face bones (Q75)

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
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

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.

  • Apert syndrome (Medical Encyclopedia)
  • Cleidocranial dysostosis (Medical Encyclopedia)
  • Craniosynostosis (Medical Encyclopedia)
  • Craniosynostosis repair (Medical Encyclopedia)
  • Head and face reconstruction (Medical Encyclopedia)
  • Pierre Robin syndrome (Medical Encyclopedia)

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Treacher Collins syndrome Treacher Collins syndrome is a condition that affects the development of bones and other tissues of the face. The signs and symptoms of this disorder vary greatly, ranging from almost unnoticeable to severe. Most affected individuals have underdeveloped facial bones, particularly the cheek bones, and a very small jaw and chin (micrognathia). Some people with this condition are also born with an opening in the roof of the mouth called a cleft palate. In severe cases, underdevelopment of the facial bones may restrict an affected infant's airway, causing potentially life-threatening respiratory problems.People with Treacher Collins syndrome often have eyes that slant downward, sparse eyelashes, and a notch in the lower eyelids called an eyelid coloboma. Some affected individuals have additional eye abnormalities that can lead to vision loss. This condition is also characterized by absent, small, or unusually formed ears. Hearing loss occurs in about half of all affected individuals; hearing loss is caused by defects of the three small bones in the middle ear, which transmit sound, or by underdevelopment of the ear canal. People with Treacher Collins syndrome usually have normal intelligence.
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Mandibulofacial dysostosis with microcephaly Mandibulofacial dysostosis with microcephaly (MFDM) is a disorder that causes abnormalities of the head and face. People with this disorder often have an unusually small head at birth, and the head does not grow at the same rate as the rest of the body, so it appears that the head is getting smaller as the body grows (progressive microcephaly). Affected individuals have developmental delay and intellectual disability that can range from mild to severe. Speech and language problems are also common in this disorder.Facial abnormalities that occur in MFDM include underdevelopment of the middle of the face and the cheekbones (midface and malar hypoplasia) and an unusually small lower jaw (mandibular hypoplasia, also called micrognathia). The external ears are small and abnormally shaped, and they may have skin growths in front of them called preauricular tags. There may also be abnormalities of the ear canal, the tiny bones in the ears (ossicles), or a part of the inner ear called the semicircular canals. These ear abnormalities lead to hearing loss in most affected individuals. Some people with MFDM have an opening in the roof of the mouth (cleft palate), which may also contribute to hearing loss by increasing the risk of ear infections. Affected individuals can also have a blockage of the nasal passages (choanal atresia) that can cause respiratory problems.Heart problems, abnormalities of the thumbs, and short stature are other features that can occur in MFDM. Some people with this disorder also have blockage of the esophagus (esophageal atresia). In esophageal atresia, the upper esophagus does not connect to the lower esophagus and stomach. Most babies born with esophageal atresia (EA) also have a tracheoesophageal fistula (TEF), in which the esophagus and the trachea are abnormally connected, allowing fluids from the esophagus to get into the airways and interfere with breathing. Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a life-threatening condition; without treatment, it prevents normal feeding and can cause lung damage from repeated exposure to esophageal fluids.
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