A Rare Case of an Oropharyngeal Mass Causing Respiratory Distress

A full-term female infant was born via normal vaginal delivery with a birthweight of 3340 g to a primigravida with an unremarkable perinatal period. The baby developed cough and coryza on day 15 of life, progressively increasing over 3 days, with feeding difficulty and an experience of 1 episode of abnormal movement with cyanosis. At the time of presentation, the neonate was started on humidified high-flow oxygen and antibiotics. However, the baby had respiratory distress, which progressively increased, and she needed intubation and ionotropic support. A membranous mass protruding and obstructing the oropharynx was identified at the time of intubation (Figure 1). The clinical examination findings were normal. Magnetic resonance imaging of the face and neck suggested a well-defined fat-attenuating lesion in the retropharyngeal space causing moderate narrowing of the oropharynx and vallecula with a mass effect on the airway (Figure 2). On transfer to our center, the mass was mobilized from the oropharynx and brought out into the oral cavity. Sutures were affixed, and the mass was held under tension with gauze and an umbilical cord clamp to avoid obstruction of the airway (Figure 3). Once lateralized, the mass ceased impinging on the airway, and the baby maintained saturation on humidified high-flow nasal cannula without a need for endotracheal intubation. Video laryngoscopy was planned by the pediatric surgery team under general anesthesia. The soft tissue mass was arising from the left tonsillar fossa and posterior pharyngeal wall. It was determined to be a pedunculated 5-by-2–cm mass obstructing the airway. The mass was excised with bipolar cautery. After the operation, the child was extubated and shifted to the neonatal intensive care unit. The infant tolerated the procedure well, breastfeeding was started on the second postoperative day, and the baby was discharged the next day.Differentials for an oropharyngeal mass in a newborn include the following: hemangiomas, lymphangiomas, Langerhans cell histiocytosis, teratomas, oral choristoma, and malignant tumors (Table 1).1The hematoxylin and eosin–stained section revealed a polypoidal mass lined by hyperplastic squamous epithelium with focal ulceration and underlying fibroadipose tissue infiltrated by acute and chronic inflammatory cells (Figure 4). Thus, this was classified as a hamartoma with superadded infection. The child is on regular follow-up with normal development and no complications.The term hamartoma is derived from the Greek word “hamartia,” referring to a defect or an error. It was originally coined by Albrecht in 1904 to denote a developmental tumor-like malformation.2 They rarely occur in the head and neck region.3The incidence of isolated hamartomas in children is not well known, probably due to variations in terminology used across different sources. The etiology of these tumors is unknown, as several mechanisms have been proposed, including the failure of regression of the nasopharyngeal membrane to misdirected pluripotent tissue.4 Hamartomas are usually congenital in origin and encapsulated. The incidence is usually isolated or syndromic, with a rare malignant transformation. Hamartomas in the oral cavity include odontogenic and nonodontogenic derivatives arising at the dorsum of the tongue, enamel, dens evaginatus, buccal bone (exostosis), palate (leiomyomatous hamartomas), buccal mucosa, and gingivae.Hamartoma is a nonneoplastic, unifocal, or multifocal developmental malformation compromising a mixture of cytologically normal mature cells and tissues that are indigenous to the anatomic location, showing a disorganized architectural pattern with a predominance of one of its components: the tumor-like proliferation of mature tissue that is native to the organ from which it developed.5 Despite an extensive literature search, we could not find a case report of a hamartoma with an origin in the tonsillar fossa.The presentation of oropharyngeal masses in neonates is nonspecific, making the diagnosis challenging. The usual presentations include swelling, cervical lymphadenopathy, epistaxis, pain, nasal breathing, dysphagia, facial paralysis, otorrhea, obstruction of the oropharynx, or asymptomatic masses. Hamartomas usually have a limited growth potential after adolescence and undergo spontaneous regression.The baby presented with cough and difficulty feeding because of the progressively increasing size of the mass in the oropharynx. Respiratory distress was secondary to the mass, and profuse secretions were causing cyanosis and difficulty in feeding. Investigations were within normal limits. Symptoms of cough, coryza, and cyanotic spells subsided after the mass was lateralized, and humidified oxygen therapy was given. After excision of the mass, the baby did not have any symptoms.We are reporting this mass as presenting with respiratory distress and possessing a great challenge during intubation as well as breastfeeding, as the mass causes obstruction of the oropharynx with a gradual increase in size.We initially treated this patient by mobilizing the mass sideways to relieve airway obstruction, which allowed the child to be weaned off the ventilator and improved her general condition prior to excision.This is a rare neoplasm, rarely causing respiratory distress in the neonatal period. The mass, if left untreated, can lead to secondary superimposed infections. The mass was innovatively managed, and outcomes were excellent, with a short hospital stay.These cases should be managed with a multidisciplinary approach, including a setup with a neonatologist, otolaryngologist, or pediatric surgeon and pediatric oncologist. Surgical excision is the definitive treatment for nonneoplastic hamartomas. The prognosis is excellent, with no recurrence after complete excision.6The presentation of oropharyngeal hamartomas depends on the size, shape, and location of the lesion, with possibilities ranging from an acute respiratory failure with difficulty feeding to an incidental finding on inspection.Syndromic associations should also be assessed and managed accordingly.Prognosis is excellent after resection.These cases should be managed with a multidisciplinary approach, including a setup with a neonatologist, otolaryngologist, or pediatric surgeon and pediatric oncologist to generate a high index of suspicion for diagnosis and management.American Board of Pediatrics Neonatal-Perinatal Content SpecificationKnow the clinical features of an infant with airway obstructionPlan appropriate diagnostic evaluation and management for an infant with airway obstructionRecognize the pathologic features of respiratory distress syndromeRecognize normal development of the nose, mouth, throat, and neck