TY - JOUR
T1 - Heminasal proboscis with associated microphthalmos and encephalocele
AU - Boahene, Derek Kofi O.
AU - Bartley, George B.
AU - Clay, Ricky P.
AU - Thompson, Dana M.
PY - 2005/3
Y1 - 2005/3
N2 - 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.
AB - 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.
KW - Craniofacial anomaly
KW - Heminasal proboscis
KW - Nasal reconstruction
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U2 - 10.1097/00001665-200503000-00018
DO - 10.1097/00001665-200503000-00018
M3 - Article
C2 - 15750429
AN - SCOPUS:17844380814
SN - 1049-2275
VL - 16
SP - 300
EP - 306
JO - Journal of Craniofacial Surgery
JF - Journal of Craniofacial Surgery
IS - 2
ER -