Abstract
Objectives: Heminasal proboscis is a rare congenital malformation that presents complex management issues when associated with other craniofacial abnormalities. We present a case associated with microphthalmia and meningoencephalocele.
Methods: A newborn male known to have a facial mass on prenatal ultrasound was delivered by planned induction at 38 weeks of gestation. He was immediately intubated due to anticipated respiratory difficulty. A right-sided proboscis was identified pedicled on the right medial canthal region. Further airway evaluation identified a piriform aperture and choanal stenosis. A hypoplastic orbit with aplastic optic nerve and a nasoorbitoethmoidal encephalocele were confirmed on magnetic resonance imaging studies. A left-sided upper eyelid coloboma was also present. The airway was initially stabilized with a tracheostomy. Beginning from the age of 10 weeks, he underwent several staged procedures to excise the encephalocele and reconstruct the nasal, orbital, and lacrimal system.
Results: The encephalocele was exposed via a bicoronal approach and frontal craniotomy. The dural defect was successfully repaired with a pericranial flap. Nasal reconstruction provided an adequate nasal passage and tip after 3 staged procedures. Using a Gunderson flap, the right eye was prepared for a later insertion of a prosthetic eye. The patient remains successfully decanulated 3 years following his last reconstructive procedure.
Conclusion: Successful reconstruction of a functional nose is possible in heminasal proboscis. The presence of co-existing craniofacial abnormalities requires a multi-specialty approach and coordination of surgical procedures.