Nasal septal lipoma in a child: Pai syndrome or not?

Abstract Lipoma is the most common soft tissue mass in adults but it is uncommon in children. Nasal septal lipoma is an even more rare entity. In this report, a 12-year-old girl was presented with congenital nasal septal lipoma. She had permanent partial nasal obstruction on her right side and disfi...

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Bibliographic Details
Published in:International journal of pediatric otorhinolaryngology 2014-04, Vol.78 (4), p.697-700
Main Authors: AbdollahiFakhim, Shahin, Bayazian, Gholamreza, Notash, Rezvan
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - diagnosis
Abnormalities, Multiple - surgery
Agenesis of Corpus Callosum - diagnosis
Agenesis of Corpus Callosum - surgery
Brain Neoplasms - diagnosis
Brain Neoplasms - surgery
Child
Cleft Lip - diagnosis
Cleft Lip - surgery
Coloboma - diagnosis
Coloboma - surgery
Corpus callosal agenesis
Diagnosis, Differential
Female
Follow-Up Studies
Humans
Interhemispheric lipoma
Lipoma - diagnosis
Lipoma - surgery
Magnetic Resonance Imaging - methods
Nasal mass
Nasal Obstruction - diagnosis
Nasal Obstruction - etiology
Nasal Polyps - diagnosis
Nasal Polyps - surgery
Nasal septal lipoma
Nasal Septum - parasitology
Nasal Septum - surgery
Nose Neoplasms
Otolaryngology
Pediatrics
Risk Assessment
Skin Diseases - diagnosis
Skin Diseases - surgery
Tomography, X-Ray Computed - methods
Treatment Outcome
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Description
Summary:Abstract Lipoma is the most common soft tissue mass in adults but it is uncommon in children. Nasal septal lipoma is an even more rare entity. In this report, a 12-year-old girl was presented with congenital nasal septal lipoma. She had permanent partial nasal obstruction on her right side and disfigurement of her right upper lip. CT scan revealed a lipoma-like mass in the septum plus an additional incidental finding of an intracranial lipoma. Further workup with MRI demonstrated corpus callosum agenesis with interhemispheric lipoma. To remove the mass, septoplasty, nasal lipoma excision and nasal vestibule V-Y advancement flap were performed. To close the lip a rotational advancement flap was required. Our case seemed to be a variety of Pai syndrome. We recommend imaging of the central nervous system in newborns with congenital lipomas located in the craniofacial midline to rule out the Pai syndrome.
ISSN:0165-5876
1872-8464
DOI:10.1016/j.ijporl.2014.01.027