Endoscopic-Assisted Resection of Nasopharyngeal Choristoma with Features of Both Congenital Hairy Polyp and Compound Odontoma Arising from the Skull Base in a Premature Infant

Congenital hairy polyps are rare benign lesions of ectodermal and mesodermal origin that most commonly arise as pedunculated masses in the oropharynx and nasopharynx. These can be associated with other abnormalities, including cleft palate. Hairy cell polyps that include features consistent with compound odontoma have been reported in animals. However, no such lesions in humans have been previously reported in human literature.

We report a case of nasopharyngeal choristoma with features consistent with hairy cell polyp and compound odontoma arising from the skull base in a premature newborn. A fetal cleft palate was diagnosed prenatally and a fetal lip mass was suspected based on ultrasound limited by maternal body habitus. The pregnancy was also complicated by type-II diabetes mellitus, polyhydramnios, and severe preeclampsia leading to delivery by Cesarean at 33 weeks gestation. At birth, the lesion presented as a mass protruding protruded from the oral cavity through a cleft palate defect ([Fig. 1]). The patient was intubated for central cyanosis and surfactant administration after Cesarean delivery at 33 weeks of gestation, but was able to be immediately extubated to continuous positive airway pressure (CPAP) after surfactant administration. Caffeine therapy was initiated for intermittent apnea. He maintained adequate oxygen saturations oxygenation and ventilation with CPAP and then intermittent CPAP and high-flow nasal cannula (HFNC) prior to surgical intervention. Karyotype analysis demonstrated standard male karyotype (46, XY). Preoperative imaging, including MRI with and without contrast and CT without contrast, demonstrated a soft tissue mass arising from the nasopharynx and extending through a cleft palate. The mass was found to contained bony/tooth-like structures ([Fig. 2]), raising suspicion for a congenital teratoma. Agenesis of the corpus callosum was also noted.

The mass was stable in size and was excised without complication at 20 days of age using a combined oral and endoscopic endonasal approach. The mass arose from the skull base in the midline nasopharynx. After removal of the 5 cm × 4 cm × 1.5 cm pedunculated mass, the base of the stalk was cauterized with electrocautery. Direct laryngoscopy and rigid bronchoscopy were performed at the time of surgery, and no other airway anomalies were noted. Surgical pathology found a choristomatous lesion with features of congenital hairy polyp but with the addition of well-developed tooth structures consistent with a compound odontoma. The absence of endodermal elements distinguished this mass as a hairy polyp.

Caffeine therapy was discontinued and the patient was transitioned to room air within two days postoperatively. He was able to take adequate oral feeds using a specialty cleft palate bottle/feeder. After gaining weight well on oral feeds, he was discharged home on postoperative day 12 (32 days of age) with a suction machine for secretion management.

Nasopharyngeal tumors are rare in neonates, and when they do occur typically affect term, female infants. We report a very rare case of a nasopharyngeal choristoma with features of both congenital hairy polyp and compound odontoma arising from the midline skull base in a preterm male. This is the first reported case in humans.

Fig. 1 Nasopharyngeal choristoma (black arrow) protruding through a cleft palate defect in a premature newborn. Hard palate: double white arrow. Oral tongue: white arrow.

Fig. 2 Preoperative CT scan demonstrated mass (black arrow) containing numerous areas of bone/teeth. Mandible: white arrow. OG tube: double white arrow. CT, computed tomography.

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Artikel online veröffentlicht:
12. Februar 2021