Baylor University Medical Center Proceedings October 2017 - 460

face was also performed. The patient was temporarily treated
with phenylephrine for signiﬁcant nasal congestion and was
weaned oﬀ oxygen by the third day of life. At the time, she
was tolerating appropriate feedings and gaining weight. The
patient underwent endoscopic surgical excision of the nasal
mass by pediatric otolaryngology without complication. Surgical pathology examination conﬁrmed heterotopic glial tissue
(nasal glioma). The patient recovered well with no postoperative feeding or breathing problems.
CASE 2
A 39-week-old gestational age infant was admitted to the
NICU immediately after delivery for respiratory distress on
continuous airway pressure at 5 cm of water. The NICU team
was called to delivery for variable and late decelerations secondary to meconium-stained amniotic ﬂuid. The pregnancy was
complicated by maternal obesity, rubella nonimmune status,
and oligohydramnios. Physical examination was pertinent for
a right upper lid coloboma, preauricular appendage, and large
nasal appendage from medial right nares. The infant was gradually weaned oﬀ oxygen support and transitioned to all oral feeds.
Magnetic resonance imaging of the face demonstrated a 0.9 ×
0.7 × 0.8 cm exophytic mass arising from the right nasal fossa
along with fat signal. The infant was discharged from the NICU
due to stable breathing and feeding and was closely followed
as an outpatient by the pediatric otolaryngology department.
At 4 months of age, the patient underwent endoscopic surgical
excision (Figure 1b) by pediatric otolaryngology without complication. Surgical pathology examination conﬁrmed NLCS.
The patient recovered well, with no further breathing or feeding issues.
DISCUSSION
Nasal gliomas account for approximately 5% of all congenital nasal anomalies. The diﬀerential diagnoses of midline nasal
anomalies that result from atypical embryologic development
include nasal gliomas, dermoid cysts, and encephaloceles (7).
The word "nasal glioma" is something of a misnomer, as it implies a neoplastic condition (7, 8). In fact, approximately 60%
of these nasal gliomas are extranasal; 30% of them are intranasal
lying within the nasal cavity, mouth, or pterygopalatine fossa;
and 10% are mixed (1, 2). In 20% of cases, gliomas connect to
the intracranial space via ﬁbrous stalk (8). Histologically, these
tumors are a product of astrocytic neuroglial cells intertwined
with ﬁbrous and vascular connective tissue explicitly enclosed
with skin or nasal respiratory mucosa.

460

Midline nasal anomalies should be considered when assessing infants with respiratory distress. Intranasal lesions often
present with nasal obstruction, which can sometimes cause lifethreatening airway obstruction in infants due to their obligate
nasal breathing. When signiﬁcant nasal obstruction occurs in
infants, feeding is also aﬀected; some infants may even require
temporary feeding tube support until treatment can take place.
A comprehensive physical examination is crucial for diagnosis, and pediatric otolaryngology consultation and appropriate
neuroimaging studies should be promptly considered when
suspicion exists in order to diagnose and treat the condition
eﬀectively. Prompt intervention may be required due to obstructive symptoms.
The treatment of choice is surgical excision, which has a 4%
to 10% recurrence rate. A detailed preoperative assessment is
essential to demarcate the precise position and extension of the
tumor, rule out cranial involvement, and plan an appropriate
surgical approach. The traditional approach for an intracranial
connection is a frontal craniotomy; other potential approaches
may include a transfacial lateral rhinotomy (7). Intranasal endoscopic approaches are increasingly utilized and may be considered alone for intranasal lesions and in combination with
other approaches for more complex lesions.
Acknowledgments
We thank Mr. Adam Stephens, medical editor, Department
of Academic Research Operations, Baylor Scott & White Medical
Center, Temple, Texas, for assistance in editing this manuscript.
1.

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