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Pathological Case of the Month
Lynn A. D'Andrea, MD
From the Department of Pediatrics, Section of Respiratory Medicine, University of Virginia Health Sciences Center, Charlottesville.
Arch Pediatr Adolesc Med. 1998;152:93-94.
A 9-DAY-OLD boy presented to the pediatric emergency department with a 1-day history of intermittent stridor and difficulty feeding. The patient, a 2920-g infant, was born at 40-weeks' gestation following an uncomplicated pregnancy, labor, and delivery. He spent 6 hours in the newborn intensive care unit because of jitteriness and mild respiratory distress. The symptoms resolved except for an intermittent "squeaky, high-pitched cry." One day before admission to the hospital, he became increasingly irritable and also had decreased oral intake. Physical examination on the day of admission revealed an infant with soft, intermittent inspiratory stridor occurring during feedings or while quietly lying supine. The stridor resolved when he held his neck in a hyperextended position or when he was placed prone. A 1- to 2-cm, red-purple ulcerated mass over the right posterior soft palate and pharyngeal arch was seen. A radiograph of the lateral aspect of the neck confirmed a large mass in the oropharynx behind the soft palate (Figure 1). It did not appear to originate from the posterior wall as a nasogastric feeding tube could be seen between the polyp and the posterior wall of the oropharynx on the x-ray film. Soon after admission, the infant began choking and coughed out a piece of tissue. The gross specimen demonstrated a 1x1-cm, in greatest diameter, red-tan polypoid mass. The tissue was examined histopathologically (Figure 2).
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Figure 1.
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Figure 2.
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Diagnosis and Discussion: Benign Pharyngeal Hamartomatous Polyp in a Neonate With Stridor
Figure 1. Hamartomatous polyp in the oropharynx behind the soft palate (arrow). A nasogastric feeding tube is seen between the polyp and the posterior wall of the oropharynx.
Figure 2. Small glandular elements are lined by benign simple cuboidal epithelium with contiguous nests of metaplastic keratinizing squamous cells embedded in a rich capillary vascular bed (hematoxylin-eosin, original magnification x100).
This patient presented with a relatively common diagnostic dilemma—stridor in the neonate. Stridor is produced by the turbulent flow of air through an obstructed upper airway during either inspiration or expiration depending on the site of obstruction. Causes of stridor can be divided into those of acute (usually acquired) or chronic (usually congenital) origin. Causes of stridor can also be differentiated by anatomic location such as supraglottic, glottic (laryngeal), or subglottic (intrathoracic). Supraglottic lesions generally produce inspiratory stridor as the poorly supported airways collapse during inspiration. Laryngeal lesions produce biphasic stridor. Expiratory stridor is produced by intrathoracic lesions as intrathoracic airways are narrowed or compressed during expiration.1
Causes of congenital stridor in neonates are numerous. Congenital anomalies account for most cases of stridor in young children. Laryngomalacia is the most common congenital laryngeal anomaly accounting for 60% to 75% of cases of stridor in newborns.1 Very rarely tumors or polyps of the oronasopharyngeal or laryngeal area are reported. These include hemangiomas, cysts, teratomas, or dermoid polyps.2
Benign hamartomatous polyps of the oronasopharynx, as seen in this infant, are very rare congenital malformations composed of tissue indigenous to the area in which they are found. They are nonneoplastic, self-limiting tumors that have been described as an exaggeration of normal physiologic tissue development.3 They are to be distinguished from the more common, although still rare, teratomas that develop from embryonic tissue, are foreign to the area in which they occur, and contain all 3 primitive germ cell layers, and dermoid polyps or hairy polyps that are composed of ectoderm and mesoderm and are believed to be inclusion errors in the fusion of the lateral palatine processes.2, 4-5
These 3 rare malformations are most commonly seen in neonates. Most infants present with respiratory distress, difficulty feeding, or both.6 Symptoms may be intermittent if the mass is pedunculated and sporadically obstructs the laryngeal or esophageal openings. Sexton6 described a 19-day-old infant with similar symptoms who was found to have a hairy polyp of the oronasopharynx. To our knowledge, no cases of auto-amputation of one of these tumorlike polyps have been described. While the infant appeared stable on presentation, the clinical symptoms and rapid progression points to the necessity for direct or x-ray visualization and careful management of airways and their patency.
An oronasopharyngeal mass is a diagnosis that should be considered in a neonate or infant who presents with intermittent stridor and difficulty feeding. These infants may have had difficulties in the nursery.
AUTHOR INFORMATION
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Accepted for publication December 1, 1996.
We thank G. J. Walker Smith, MD, for help in preparing of the histological photographs.
Corresponding author: Lynn A. D'Andrea, MD, University of Virginia Health Sciences Center, Department of Pediatrics, Section of Respiratory Medicine, Box 386, Charlottesville VA 22908 (e-mail: lad2v{at}virginia.edu).
REFERENCES
1. Simon NP. Evaluation and management of stridor in the newborn. Clin Pediatr. 1991;30:211-216.
2. Morgan DW, Evans JNG. Developmental nasal anomalies. J Laryngol Otol. 1990;104:394-403.
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3. Majumder NK, Venkataramaniah NK, Gupta KR, Gopalakrishnan S. Hamartoma of nasopharynx. J Laryngol Otol. 1977;91:723-727.
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4. Felder H. Benign congenital neoplasms: dermoids and teratomas. Arch Otolaryngol. 1975;101:333-334.
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5. Chaudhry AP, Lore JM Jr, Fisher IE, Gambrino AG. So-called hairy polyps or teratoid tumors of the nasopharynx. Arch Otolaryngol. 1978;104:517-525.
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6. Sexton M. Hairy polyp of the oropharynx. Am J Dermatopathol. 1990;12:294-298.
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SECTION EDITOR: ENID GILBERT-BARNESS, MD
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