Developmental Disorders
Hemifacial Microsomia
aka HFM · Craniofacial Microsomia · First and Second Branchial Arch Syndrome · Goldenhar Syndrome (with vertebral/ocular anomalies)
Second most common craniofacial anomaly; unilateral underdevelopment of mandible, ear, and soft tissues from first and second arch defects.
§ figuresFigures (3)
Fig. 1Bialateral (both sides) hemifacial microsomia in an adolescent
“Bialateral hemifacial microsomia” · Purplepong · Public domain · Wikimedia Commons
Fig. 2Treatment of a Pruzanski type I case of hemifacial microsomia, which has been achieved with distraction osteogenesis of the right side of the mandible, followed by maxillary osteotomy. Photos of the patient: a) before an
“Treatment of a Pruzanski type I hemifacial microsomia” · Καρακάση, Δ.Θ. και Λαζαρίδη, Ν.Θ. · CC BY-SA 4.0 · Wikimedia Commons
Fig. 3A girl suffering from Pruzanksky type III hemifacial microsomia of the right side: a) and b) face and profile view, demonstrate the asymmetry c) intraoral view shows the turn of the lower dental arch to the right, d) the
“Hemifacial microsomia Pruzansky type III” · Dithkar · CC BY 3.0 · Wikimedia Commons
Verified open-access images only, reproduced for education under their stated licences · no AI-generated or illustrative artwork · Hemifacial Microsomia figures pending faculty review.
§ overviewOverview
Congenital asymmetric hypoplasia of structures derived from the first and second branchial arches, most commonly affecting the mandible, ear, and facial soft tissues.
§ icdICD Classification
Q87.0
§ etiologyEtiology
- 01Sporadic vascular disruption of stapedial artery
- 02Possible genetic component (14q32)
- 03Environmental teratogens
§ epidemiologyEpidemiology
1:3,500 to 1:5,600 births. M:F = 3:2. Right side > left.
§ pathogenesisPathogenesis
Vascular disruption or haematoma in 1st/2nd arch territory → tissue necrosis → hypoplasia of mandible, ear, muscles of mastication.
§ clinicalClinical Features
- 01Mandibular hypoplasia (Pruzansky grades)
- 02Microtia/anotia
- 03Facial nerve weakness
- 04Soft tissue deficiency
- 05Macrostomia
- 06Preauricular tags
- 07Vertebral anomalies (Goldenhar)
§ investigationsInvestigations
- 01Clinical + 3D CT
- 02Audiometry
- 03Spine X-ray if Goldenhar suspected
§ classificationClassification
- 01Pruzansky (I-III) — mandible
- 02OMENS classification — comprehensive
- 03Goldenhar = HFM + vertebral + epibulbar dermoids
§ treatmentTreatment
- 01Multidisciplinary craniofacial team
- 02Distraction osteogenesis (mandible)
- 03Costochondral graft for TMJ reconstruction
- 04Orthognathic surgery at skeletal maturity
- 05Ear reconstruction
§ surgicalSurgical Management
- 01Mandibular distraction
- 02Costochondral rib graft
- 03Microvascular free flap for soft tissue
- 04Auricular reconstruction
§ complicationsComplications
- 01Obstructive sleep apnoea
- 02Malocclusion
- 03Hearing loss
§ prognosisPrognosis
Good functional and aesthetic outcomes with staged surgical management.
§ examKey Examination Points
- 01Pruzansky grading predicts surgical approach
- 02Goldenhar = HFM + vertebral + eye anomalies
- 03Distraction useful in growing child
§ revisionQuick Revision Summary
- 011st + 2nd arch derivative hypoplasia
- 02OMENS: Orbit, Mandible, Ear, Nerve, Soft tissue
- 03Stapedial artery haemorrhage theory
§ vivaBDS Viva Questions
- 01What is the Pruzansky classification?
- 02Differentiate HFM from Goldenhar syndrome.
- 03What is the role of distraction in HFM?
§ mcqsMCQs — Assessment (3)
Question 1
HFM results from defects in which branchial arches?
Question 2
Goldenhar syndrome includes HFM plus:
Question 3
Pruzansky Grade III mandible:
§ evidenceReferences & Evidence
Current Classification
WHO Classification of Tumours Online — Head and Neck
IARC / WHO · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Evidence summary
Highest level of evidence linked to this page: WHO / Official Classification.
- L1 · Classification — 1
- L6 · Textbook — 3
1 linked source was published from 2024 onwards. Where guidance is limited or contested, rely on the higher-level sources above rather than this summary. Educational use only — not a substitute for clinical judgement.
Content last reviewed
September 2026
Evidence last reviewed
September 2026
Next scheduled review
September 2027
Review status
EVIDENCE REVIEWED
Search latest PubMed evidence
Last literature search: 7 September 2026
Current Classification
IARC / WHO. IARC · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Histopathology References
Barnes L. CRC Press · 2008
Detailed histopathological reference for head and neck diagnostic criteria.
Textbook References
Neville BW, Damm DD, Allen CM et al.. Elsevier · 2024
Standard reference textbook of oral and maxillofacial pathology used throughout this atlas.
Rajendran R, Sivapathasundharam B. Elsevier · 2020
Undergraduate and postgraduate oral pathology textbook widely used in BDS and FCPS curricula.
Additional sources cited on this page
- Kaban LB. J Oral Maxillofac Surg 1998
- Neville BW. Oral & Maxillofacial Pathology, 4e
Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.