71 Facial Asymmetry in Children Facial symmetry can be a good indicator of an underlying syndromic diagnosis. Asymmetry may be created by clefting of the face, lip, or cranial vault; by early suture line closure; by eye/lid anomalies; by ear anomalies; and by facial growth restrictions. The most common anomalies of the lip, nose, and face are those resulting from cleft lip and palate. Cleft lip (Fig. 71.1A,B) with or without cleft palate (Fig. 71.2) occurs in ~1/1000 births, and as lesions and defects are more severe, the proportion of male patients increases. Cleft lip and palate are associated with many other syndromes, including Pierre Robin sequence (see below), ectrodactylyectodermal dysplasia-clefting (EEC) syndrome, ectrodactyly-cleft palate (ECP) syndrome, Bowen-Armstrong syndrome, Larsen syndrome, Van der Woude syndrome, velocardiofacial (Shprintzen) syndrome, among many other more rare, and some lethal, syndromes. Fig. 71.1 (A,B) Unilateral cleft lip. Fig. 71.2 Bilateral cleft lip (and palate). Nasal asymmetry, the classic “cleft lip nose,” results from a cleft lip and often persists despite efforts at repair of the lip, requiring reconstructive rhinoplasty for correction. Primary nasal cleft is much less common. Midface growth restriction with or without cleft palate may occur in the setting of autosomal dominant achondroplastic dwarfism; with associated proptosis in Crouzon syndrome; or with syndactyly and proptosis in Apert syndrome (acrocephalosyndactyly). Other rare syndromes causing midface hypoplasia are Pfeiffer syndrome (also has hypertelorism and downsloping palpebral fissures), and Jackson-Weiss syndrome (also has acrocephaly, hypertelorism, and proptosis). Primary cranial vault clefts can be simple, resulting in hypertelorism, or quite complex (Fig. 71.3), resulting in facial duplications associated with hydrocephalus. Craniosynostosis, or premature closure of the cranial suture lines, may occur spontaneously (idiopathic) or in conjunction with a syndromic diagnosis such as Crouzon syndrome or Apert syndrome.
Lip/Nose/Face
Cranial Vault
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