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The Laryngoscope
Lippincott Williams & Wilkins, Inc.
© 2003 The American Laryngological,
Rhinological and Otological Society, Inc.

Nasal Glioma and Encephalocele: Diagnosis
and Management
Reza Rahbar, DMD, MD; Vicente A. Resto, MD, PhD; Caroline D. Robson, MD; Antonio R. Perez-Atayde, MD;
Liliana C. Goumnerova, MD; Trevor J. McGill, MD; Gerald B. Healy, MD

Objective: To review thebiology of nasal glioma
and encephalocele and to present an algorithm for
preoperative evaluation and surgical management.
Design: Retrospective review and analysis. Setting:
Tertiary care medical center: 1970 to 2002. Patient:
Sixteen patients with glioma (n 10) and encephalocele (n 6). Outcome: Age at the time of presentation,
sex, signs and symptoms, imaging findings, surgical
approach,pathology, complications, rate of recurrence, and follow-up were recorded. Results: Ten patients presented with nasal glioma with a mean age of
9 months. All patients underwent surgical excision.
No complication was encountered with a mean
follow-up of 3.5 years. Six patients presented with
encephaloceles with a mean age of 15.5 months. All
patients underwent surgical excision. Complicationsincluded cerebrospinal fluid leak (n
1) and epiphora (n
1). Follow-up was 1 to 14 years (mean, 4
years). Conclusion: Nasal glioma and encephalocele
are rare, benign, congenital lesions with a potential
for intracranial extension. Evaluation should include
a complete rhinologic and neurologic examination.
Preoperative imaging with a thin-cut axial and coronal computed tomography scan and/ormultiplanar
magnetic resonance imaging is essential. Surgical intervention should be performed soon after diagnosis
to alleviate the increased risk of meningitis. A frontal
craniotomy approach is recommended if intracranial
extension is identified based on preoperative evaluation, followed by an extracranial resection. If there is
no evidence of intracranial extension, a conservativeextracranial approach is recommended. Key Words:
Nasal mass, glioma, encephalocele.
Laryngoscope, 113:2069 –2077, 2003
Presented at the Triological Society Meeting (Eastern Section), Boston, Massachusetts, January 2003.
From the Department of Otolaryngology, Children’s Hospital, and
Department of Otology and Laryngology, Harvard Medial School (R.R.,
T.J.MCG., G.B.H.); Department of Otology andLaryngology, Massachusetts
Eye and Ear Infirmary, Harvard Medical School (V.A.R.); Department of
Pathology, Children’s Hospital, Harvard Medical School (A.R.P.-A.); Department of Radiology, Children’s Hospital, Harvard Medical School (C.D.R.);
and Department of Neurosurgery, Children’s Hospital, Harvard Medical
School (L.C.G.), Boston, Massachusetts, USA.
Editor’s note: This Manuscript was acceptedfor publication June 20,
2003.
Send Correspondence to Reza Rahbar, DMD, MD, Department of
Otolaryngology, Children’s Hospital, 300 Longwood Avenue, Boston, MA
02115, U.S.A. E-mail: reza.rahbar@tch.harvard.edu

Laryngoscope 113: December 2003

INTRODUCTION
Nasal gliomas and encephaloceles are rare congenital
malformations. These lesions are part of a larger group of
benign nasal andanterior skull base masses, which include entities such as dermoids and hemangiomas. Therefore, the presence of anomalies arising in midline nasal
and anterior skull base structures should raise the possibility of glioma or encephalocele in addition to more frequently encountered lesions.
Nasal gliomas and encephaloceles predominantly appear in the pediatric population, causing nasal obstruction,difficulty with feeding, and nasal cosmetic deformities. More importantly, these lesions can be foci of
infection. This is of paramount importance given the
known propensity for intracranial communication in these
lesions, thus putting the patient at risk for intracranial
infectious and associated complications.
In this article, we report our experience with patients
who presented with...
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