Surgical Strategies Before Auricular Reconstruction: A Treatment Algorithm for Craniofacial Anomalies in Goldenhar...

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.