Heminasal proboscis is a rare congenital malformation that presents complex management issues when associated with other
craniofacial abnormalities. A newborn male, known to have a facial mass on prenatal ultrasonography, was delivered by planned induction at 37 weeks' gestation. He was intubated immediately because of anticipated respiratory difficulty. He had a right-sided proboscis, pedicled on the right medial canthal region. Additional airway evaluation identified a piriform aperture and choanal
stenosis. Magnetic resonance imaging confirmed a hypoplastic orbit with aplastic optic nerve and a naso-orbito-ethmoidal
encephalocele. Right upper and lower eyelid
colobomas were present. The airway was initially stabilized with a
tracheostomy. Beginning at the age of 10 weeks, he underwent several staged procedures to excise the
encephalocele and to reconstruct the nose, orbit, eyelid, and lacrimal drainage system. The
encephalocele was exposed via a bicoronal approach and frontal
craniotomy. The dural defect was repaired with a pericranial flap. Nasal reconstruction provided an adequate nasal passage and tip after three staged procedures. Creation of a conjunctival flap prepared the right eye for later insertion of a prosthetic shell. The patient remained successfully decannulated 3 years after his last reconstructive procedure. Identification of all associated anomalies is important before initiating surgical repair. The presence of coexisting
craniofacial abnormalities requires a multispecialty approach and coordination of
surgical procedures.