Goldenhar syndrome (GS) presents complex craniofacial malformations and challenging timing for auricular reconstruction. This study characterizes the craniofacial phenotype and staged surgical management preceding ear reconstruction. Single-center retrospective cohort of 30 patients (2019–2024). Craniofacial anomalies and all procedures performed before auricular reconstruction were recorded. Categorical data were summarized as n (%), and ages as median. Auricular anomalies were universal; microtia occurred in 27/30 (90.0%) and external auditory canal atresia in 43.3%. Hemifacial microsomia with mandibular hypoplasia affected 100%, predominantly right-sided, with Pruzansky–Kaban types I 10.0%, IIA 40.0%, IIB 10.0%, and III 33.3%. Epibulbar dermoids were present in 93.3%, macrostomia in 43.3%, and congenital facial nerve palsy in 26.7%. Key procedures followed an age-adapted sequence: primary palatoplasty in 36.7% at 1.2 years 1.0–3.1; macrostomia repair in 40.0% at 1.0 year 0.8–2.6; primary mandibular distraction in 50.0% at 7.0 years 4.0–7.0; secondary distraction in 23.3% at 11.0 years 7.0–14.0; and microvascular free fibula flap in 40.0% at 14.0 years 10.0–14.5. Orthognathic surgery was reserved for skeletal maturity (Le Fort I, BSSO, genioplasty at 18.0–20.5 y), while auricular reconstruction stages I to III were completed at 10.0 9.0–14.8, 13.0 10.8–15.3, and 18.0 16.5–19.5 years, respectively. GS in this cohort showed near-universal syndromic microtia with frequent mandibular hypoplasia, macrostomia, and epibulbar dermoids. An age-based sequence, from early cleft/macrostomia repair, through childhood distraction and adolescent fibula/orthognathic surgery, to delayed costochondral auricular reconstruction, provides a structured framework for correction before ear reconstruction.
Fukumoto-Inukai et al. (Mon,) studied this question.