2026 ICD-10-CM Diagnosis Code Q75.009Craniosynostosis, unspecified

ICD-10-CM Codes›Q00-Q99›Q65-Q79›Q75

ICD-10-CM Q75.009
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

Q75.009 is a billable ICD-10-CM diagnosis code for craniosynostosis, unspecified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 564 through 566. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Musculoskeletal congenital conditions.

Code Identity

ICD-10-CM Code
Q75.009
Billable Status
Yes — Valid for Submission
Code Describes
Craniosynostosis, unspecified
Short Description
Craniosynostosis, unspecified
Same as the full description in the CMS dataset.
Parent Code
Q75.00
Craniosynostosis, unspecified

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ65-Q79Congenital malformations and deformations of the musculoskeletal system
CategoryQ75Other congenital malformations of skull and face bones
This CodeQ75.009Craniosynostosis, unspecified

Present on Admission (POA)Billing

Q75.009 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review other POA exempt codes.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acrocephalosyndactyly
  • Acrocephalosyndactyly type V
  • Acrocephaly
  • Agammaglobulinemia, microcephaly, craniosynostosis, severe dermatitis syndrome
  • Agenesis of fibula
  • Ambiguous genitalia
  • Aplasia of fibula
  • Baller-Gerold syndrome
  • Closure of fontanelle
  • Complex craniosynostosis
  • Congenital dysplasia of radius
  • Congenital retrognathism
  • Craniomicromelic syndrome
  • Craniorhiny
  • Craniosynostosis and dental anomalies syndrome
  • Craniosynostosis and intracranial calcification syndrome
  • Craniosynostosis Boston type
  • Craniosynostosis fibular aplasia syndrome
  • Craniosynostosis Herrmann Opitz type
  • Craniosynostosis Philadelphia type
  • Craniosynostosis syndrome
  • Craniosynostosis with Dandy-Walker malformation and hydrocephalus syndrome
  • Craniosynostosis, anal anomaly, porokeratosis syndrome
  • Craniosynostosis, microretrognathia, severe intellectual disability syndrome
  • Dandy-Walker syndrome
  • Early fontanel closure
  • Fibroblast growth factor receptor 3-related craniosynostosis
  • Frontonasal dysplasia sequence
  • Holoprosencephaly craniosynostosis syndrome
  • Hunter McAlpine craniosynostosis syndrome
  • Imperfect fusion of skull
  • Marfanoid physique
  • Muenke syndrome
  • Osteocraniostenosis
  • Osteosclerosis, developmental delay, craniosynostosis syndrome
  • Parieto-occipital craniosynostosis
  • Pfeiffer syndrome type 1
  • Pfeiffer syndrome type 2
  • Pfeiffer syndrome type 3
  • Recession of bone
  • SCARF syndrome
  • Shprintzen Goldberg craniosynostosis syndrome
  • Simple craniosynostosis
  • Spondyloepiphyseal dysplasia, craniosynostosis, cleft palate, cataract and intellectual disability syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Imperfect fusion of skull

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Acrocephaly
    • Closure
      • cranial sutures, premature
    • Craniostenosis
    • Craniosynostosis
    • Deficiency, deficient
      • craniofacial axis
    • Imperfect
      • closure (congenital)
        • skull
    • Ossification
      • fontanel, premature
    • Oxycephaly, oxycephalic

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR MAL008
Musculoskeletal congenital conditions
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Acrocephalosyndactyly

    a genetic disorder characterized by craniosynostosis and fusion of the fingers and toes.

  • TWIST1 Gene|TWIST1|TWIST1|Twist Homolog 1 (Acrocephalosyndactyly 3; Saethre-Chotzen Syndrome) (Drosophila) Gene

    this gene plays a role in regulation of transcription and the inhibition of apoptosis. it is also involved in the control of morphogenesis during embryonic development.

  • TWIST1 wt Allele|ACS3|BPES2|BPES3|SCS|TWIST|Twist Homolog 1 (Acrocephalosyndactyly 3; Saethre-Chotzen Syndrome) (Drosophila) wt Allele

    human twist1 wild-type allele is located in the vicinity of 17p13.3 and is approximately 16 kb in length. this allele, which encodes twist-related protein 1, plays a role in the regulation of both transcription and cell lineage determination. mutations in the gene are associated with saethre-chotzen, robinow-sorauf, and baller-gerold syndromes.

  • Twist-Related Protein 1|Acrocephalosyndactyly 3 Protein|Class A Basic Helix-Loop-Helix Protein 38|H-Twist|TWIST|TWIST1|TWIST1 Protein|Twist Homolog|Twist Homolog 1|Twist Related Protein 1|bHLHa38

    twist-related protein 1 (202 aa, ~21 kda) is encoded by the human twist1 gene. this protein plays a role in the negative regulation of both transcription and myogenesis.

  • Type I Acrocephalosyndactyly|Acrocephalosyndactyly Type I|Apert Syndrome

    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.

  • Type III Acrocephalosyndactyly|Acrocephalosyndactyly Type III|Saethre-Chotzen Syndrome|Saethre-Chotzen Syndrome

    a rare autosomal dominant syndrome caused by mutations in the twist1 gene. it is characterized by premature closure of skull bones resulting in abnormally shaped head, high forehead, hypertelorism, and facial asymmetry. it may be associated with fusion of certain fingers or toes.

  • Type V Acrocephalosyndactyly|Acrocephalosyndactyly Type V|Noack Syndrome|Pfeiffer Syndrome

    an autosomal dominant inherited type of acrocephalosyndactyly caused by mutations in the fgfr1 or fgfr2 genes. it is characterized by early closure of the sutures between the skull bones, bulging and wide-set eyes, broad thumbs, big toes, and partial syndactyly in the hands and toes.

Code History & ChangesHistory

Revised Code Q75.009 was revised for FY 2026, effective October 1, 2025.

Replacement Q75.009 replaces the following previously assigned code(s):

  • Q75.0 - Craniosynostosis
FY 2024AddedAdded to the ICD-10-CM code setEffective October 1, 2023.
FY 2025No changes
FY 2026CurrentRevised in the current code setEffective October 1, 2025 through September 30, 2026.

Questions About Q75.009Overview

Is Q75.009 (Craniosynostosis, unspecified) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report craniosynostosis, unspecified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does Q75.009 group to?

When craniosynostosis, unspecified is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 565, 566, with relative weights from 0.7493 to 1.5436 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q75.009 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for craniosynostosis, unspecified on inpatient claims.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.