title: Cleft characteristics and treatment outcomes in hemifacial microsomia compared to non-syndromic cleft lip/palate.
abstract: The goal of this study was to describe the clinical characteristics and treatment outcomes of patients with hemifacial microsomia (HFM) and cleft lip/palate (CL/P), and to compare them to a historic cohort of patients with non-syndromic CL/P treated at the same centre. A retrospective review of patients with HFM and CL/P was performed; the main outcome measures assessed were cleft type/side, surgical outcome, midfacial retrusion, and speech. Twenty-six patients (13 male, 13 female; mean age 22.7±14.9, range 1-52 years) with cleft lip with/without cleft palate (CL±P) were identified: three with cleft lip (12%), two with cleft lip and alveolus and an intact secondary palate (8%), and 21 with cleft lip and palate (CLP) (81%; 15 unilateral and six bilateral). Four patients (19%) had a palatal fistula after palatoplasty. Twelve of 22 patients aged >5 years (55%) had midfacial retrusion and two (9%) required a pharyngeal flap for velopharyngeal insufficiency (VPI). Fisher's exact test demonstrated a higher frequency of complete labial clefting (P=0.004), CLP (P=0.009), midfacial retrusion (P=0.0009), and postoperative palatal fistula (P=0.03) in HFM compared to non-syndromic CL±P. There was no difference in VPI prevalence. This study revealed that patients with HFM and CL±P have more severe forms of orofacial clefting than patients with non-syndromic CL±P. Patients with HFM and CL±P have more severe midfacial retrusion and a higher palatal fistula rate compared to patients with non-syndromic CL±P.
authors.affiliation: Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA, USA.
authors.forename: K M
authors.initials: KM
authors.lastname: Dentino
authors.affiliation: Erasmus University, Rotterdam, The Netherlands.
authors.forename: A
authors.initials: A
authors.lastname: Valstar
authors.affiliation: Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA, USA. Electronic address: bonnie.padwa@childrens.harvard.edu.
authors.forename: B L
authors.initials: BL
authors.lastname: Padwa
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002971:Cleft Lip / Q000473:pathology / Q000601:surgery*; D002972:Cleft Palate / Q000473:pathology / Q000601:surgery*; D005260:Female; D006053:Goldenhar Syndrome / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D014681:Velopharyngeal Insufficiency / Q000601:surgery*; D055815:Young Adult
keywords: cleft lip/palate; hemifacial microsomia