Baylor University Medical Center Proceedings October 2017 - 459

Congenital midline nasal anomalies
Niraj Vora, MD, Ram Kalagiri, MD, Bryan Guillory, MD, Ashita Shetty, MD, Venkata Nakta Raju, MD, R. K. Powitzky, MD,
and Madhava R. Beeram, MD

Congenital midline nasal anomalies are rare,
with a prevalence of 1
in 20,000 to 40,000
births and with 5% to
7% of them being nasal glioma. Differential
diagnoses of nasal
anomalies include nasal
dermoid cysts, gliomas,
encephaloceles, nasal
polyps, and some other
rare anomalies. Due to
current medical technological advancements,
Figure 1. Nasal endoscopy. (a) Case 1: Nasal glioma. (b) Case 2: Nevus lipomatosus cutaneous superficialis.
most of these anomalies
are easily correctable, though delaying management may lead to fatal
hours of life. A nurse reported three consequent desaturations
in the newborn nursery; the attending neonatologist was coneffects. This report describes two cases-one of nasal glioma and one of
sulted, and the infant was transferred to a tertiary neonatal
nevus lipomatosus cutaneous superficialis-that presented as respiratory
intensive
care unit (NICU) for further management. The infant
distress in a newborn. Approximately 10 to 20 cases of these two condiwas placed on nasal bubble continuous positive airway prestions have been described; notably, this is the second documented case
sure at 4 cm of water for respiratory support with a fraction of
of nevus lipomatosus cutaneous superficialis with nasal presentation.

wo cases involving congenital midline anomalies in neonates manifesting as respiratory distress in newborns are
discussed here, one with a diagnosis of nasal glioma and
one with a diagnosis of nevus lipomatosus cutaneous
superﬁcialis (NLCS) in the nasal cavity. Approximately 20 cases
of nasal glioma have been reported in English publications; less
than 10 cases of NLCS, with only one other case involving the
nasal cavity, have been reported (1-6).

inspired oxygen of 21%. A chest x-ray obtained in the NICU
showed no obvious abnormalities. A complete blood count
with diﬀerentials obtained at the time of NICU admission
was within normal limits as well. The pediatric otolaryngology department was consulted for evaluation of respiratory
obstruction. The infant was found to have a "ﬂeshy mass" in the
right anterior nasal cavity via nasal endoscopy (Figure 1a). The
patient underwent computed tomography of the face, which
showed a 1.2 × 1.1 × 0.7 cm anterior nasal mass with possible
tiny central calciﬁcation. Magnetic resonance imaging of the

CASE 1
A 40-week and 1-day-old gestational age female infant born
in a tertiary center to a 27-year-old mother with good prenatal
care and an uncomplicated pregnancy was initially transferred
to the newborn nursery for routine care after receiving Apgar
scores of 9 and 9 at 1 and 5 minutes of life. Subsequently
the infant developed worsening respiratory distress along with
increased diﬃculty breathing and intercostal retractions at 8

From the Division of Neonatology, Department of Pediatrics (Vora, Kalagiri, Shetty,
Raju, Beeram), Department of Pathology (Guillory), and Department of Pediatric
Otolaryngology (Powitzky), Baylor Scott & White Health and Texas A&M Health
Science Center College of Medicine, Temple, Texas.
Corresponding author: Madhava R. Beeram, MD, Chairman and Professor,
Department of Pediatrics, Texas A&M Health Science Center College of Medicine,
Physician-In-Chief, Baylor Scott & White McLane Children's Hospital, 2401 South
31st Street, Temple, TX 76502 (e-mail: madhava.beeram@bswhealth.org).

Proc (Bayl Univ Med Cent) 2017;30(4):459-460

459

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