Duplication anomalies of the nose include polyhinia (double nose) and supernumerary nostril (assessory nostril). These are rare congenital nasal deformities resulting from aberrant embryological development. Differential diagnoses include glioma, encephalocele, nasal dermoid, nasolacrimal duct duplication, mid facial cleft and proboscis lateralis (K. Nakamura, T. Onizuka. Plast. Reconstr. Surg. 80 (3) (1987) 436–441). Our review of the English language literature revealed eight reported cases of duplication anomalies of the nose. Four of these were cases of polyrhinia (double nose). Of the cases remaining, one patient had a supernumerary nostril in association with a cleft lip, leaving only three reported cases of an isolated supernumerary nostril. We present a newborn infant with an isolated right supernumerary nostril. MRI, CT and photographic documentation are provided. Pertinent embryology, anatomy and a thorough review of the literature are included.

Nager's acrofacial dysostosis is a rare congenital anomaly in which mandibulofacial dysostosis is seen in combination with limb deformities, particularly hypoplasia of the radial aspect of the hand. A boy with a severe manifestation of this syndrome is presented. Deformities include severe bilateral mandibular hypoplasia with trismus, malar hypoplasia, downward sloping palpebral fissures, high-arched hard palate, absent soft palate, external auditory and middle ear abnormalities and bilateral hand deformities. The diagnosis and treatment difficulties in this patient are discussed in light of those reported in the literature. An interdisciplinary team approach to the management of patients with Nager syndrome is emphasized.