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Heterotopic Neuroglial Tissue Causing Airway Obstruction in the Newborn
Philomena Mufalli Behar, MD;
Susan Muller, DMD;
Mark E. Gerber, MD;
N. Wendell Todd, MD
Arch Otolaryngol Head Neck Surg. 2001;127:997-1002.
Background Heterotopic neuroglial (brain) tissue is a rare cause of airway obstruction
in newborns. Fewer than 30 cases have been reported in the English literature.
Brain heterotopias can mimic more common congenital anomalies of the head
and neck.
Objective To review our experience in the diagnosis and treatment of children
with heterotopic pharyngeal neuroglial tissue.
Design Case series.
Setting Tertiary care children's hospital.
Patients Four newborns with airway obstruction caused by heterotopic neuroglial
tissue.
Results All patients were infants (3 full-term girls and a 32 weeks' gestation
boy) who had airway obstruction in the newborn period. All patients underwent
preoperative computed tomography and magnetic resonance imaging, which revealed
a heterogeneous mass involving the pharynx, neck, and parapharyngeal space.
Bony deformities of the skull base and mandible were present in all patients,
although intracranial connection was absent. Multiple surgical procedures
were performed in all 4 patients. Tracheotomy was performed in 2 patients,
gastrostomy tube placement was required in 3, and a nasopharyngeal tube was
used in 1. Combined cervicofacial and transoral approaches were used for resection,
preserving vital structures. Histopathologic evaluation revealed mature glial
tissue and choroid plexus–like structures.
Conclusions Heterotopic neuroglial tissue must be considered in the differential
diagnosis of airway obstruction in the newborn. Management is surgical resection,
with attention to vital structures and function—analogous to surgery
for lymphangioma. Multiple surgical procedures might be necessary in the treatment
of these patients.
From the Department of Pediatric Otolaryngology, The Children's Hospital
of Buffalo, Buffalo, NY (Dr Behar); the Departments of Otolaryngology (Drs
Muller and Todd), Pathology (Dr Muller), and Pediatrics (Dr Todd), Emory University,
Atlanta, Ga; and the Department of Otolaryngology, Children's Memorial Hospital,
Chicago, Ill (Dr Gerber).
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