Related Experiment Video
Updated: May 19, 2026

08:03
Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
Published on: November 4, 2025
Evaluation of ICD-9-CM codes for craniofacial microsomia
Daniela V Luquetti1, Babette S Saltzman, Daniela Vivaldi
1Seattle Children's Hospital, Craniofacial Center, Seattle, Washington 98101, USA. daniel.luquetti@seattlechildrens.org
Summary
A specific code for craniofacial microsomia (CFM) is needed for accurate surveillance and research. Current International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) codes are insufficient for identifying CFM cases effectively.
Area of Science:
- Medical informatics
- Genetics and genomics
- Public health
Background:
- Craniofacial microsomia (CFM) is a congenital condition involving microtia and mandibular underdevelopment.
- Accurate identification of CFM cases is crucial for surveillance and research.
- The absence of a specific International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) code hinders standardized data collection for CFM.
Purpose of the Study:
- To evaluate the validity of existing ICD-9-CM codes for identifying individuals with craniofacial microsomia.
- To assess the effectiveness of current coding practices in capturing CFM cases within healthcare databases.
Main Methods:
- Developed eligibility criteria for CFM cases by an expert panel.
- Queried hospital discharge data from two craniofacial centers using 11 potential ICD-9-CM codes.
- Reviewed medical records of identified individuals to classify them as CFM cases or non-cases.
Main Results:
- Only a small percentage of individuals identified by existing codes met CFM eligibility criteria (6% at SCH, 12% at UNC).
- The ICD-9-CM code 744.23 (microtia) demonstrated the highest positive predictive value (>80%) and sensitivity (>70%) among evaluated codes.
- Nonspecific codes (754.0, 756.0) showed low sensitivity and low positive predictive values for identifying CFM cases.
Conclusions:
- Existing ICD-9-CM codes are inadequate for precise craniofacial microsomia surveillance.
- A dedicated CFM code is necessary to improve data accuracy for research and public health initiatives.
- Enhanced coding systems are essential for better understanding and managing craniofacial microsomia.

