Dermoid of the Nasopharynx Causing Neonatal Respiratory Distress

Abstract

The dermoid is a rare and benign malformation of bigerminal origin. It is seen frequently in the neonatal period, and the clinical presentation depends on the site and on the size of the lesion, and the classical clinical picture is of a tumor derived from the naso- or oropharynx, leading to respiratory distress and/or feeding disorders. A female newborn was born cyanotic requiring intubation. The oroscopy revealed a large smooth mass. Transoral surgery was performed with successful extubation. Seven months after, there are no signs of recurrence of the lesion. This report adds another case of this rare pathology that can be life-threatening condition in the neonate. It emphasizes the importance of dermoid in the differential diagnosis of the naso- or oropharyngeal lesions, especially in neonates.

Oropharyngeal Hairy Polyp: A Case of Respiratory Failure in a Newborn

“…These findings were consistent with oropharyngeal dermoid polyp (DP); in particular, a DP does not show restricted diffusion, contrary to an epidermoid cyst [ 1 ]. DPs have to be differentiated by other lesions within the region of the skull base or pharynx, which may cause children’s respiratory distress, such as meningoencephalocele, neuroglial heterotopias, and gliomas [ 1 , 2 , 3 ]. In particular, a DP differs from a meningoencephalocele, which is hypointense on T1-weighted and FLAIR images (similar to brain tissue), showing a direct communication through a skull base defect with the cranial vault; or from neuroglial heterotopy, which shows T1 and T2 signal characteristics of brain gray and white matter with small enclosed cysts; instead of gliomas, which usually show a hypointense signal on T1-weighted images [ 1 ].…”

“…Even if the use of ultrasound is useful for the first-line investigation of neck masses in neonates [ 4 ], considering the severity of the respiratory failure, our patient directly underwent MRI examination for optimal lesion characterization and surgical planning. Moreover, MRI allowed us to assess the relationship of the lesion with the adjacent soft tissues, to depict adjacent bony changes [ 3 , 5 ], and to exclude intracranial extension, thus providing essential information for the surgical planning [ 2 ]. This approach is essential in terms of surgical management and outcome.…”

“…The histologic assessment confirmed MRI suspicion, revealing a stratified keratinized epithelium mass, containing dermal skin appendages as sebaceous and sudoriferous glands, mixed to adipose tissue ( a ) with a small cartilaginous fragment (as shown in detail in b ), consistent with hairy polyp (also known as dermoid polyps (DPs)). It is a benign epithelial inclusion cystic lesion [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ] resulting from abnormal separation of ectoderm and neuroectoderm along the lines of embryonic fusion, during the first steps of embryogenesis, between the third–fifth gestational week [ 6 , 7 ]. Dermoids may occur in the head and neck region, in particular, settled in the orbital region, nasal dorsum, mouth floor, infratemporal fossa, nasopharynx, oropharynx, and anterior and lateral part of the neck, also arising from the eustachian tube [ 3 ], although they may be found anywhere on the body [ 5 ].…”

“…Dermoids may occur in the head and neck region, in particular, settled in the orbital region, nasal dorsum, mouth floor, infratemporal fossa, nasopharynx, oropharynx, and anterior and lateral part of the neck, also arising from the eustachian tube [ 3 ], although they may be found anywhere on the body [ 5 ]. DPs are frequently found in the neonatal period, but occasionally they can be seen in older children, since a DP’s clinical presentation depends on the site of origin and on its size [ 2 ]. When in the naso-oropharyngeal cavity, as in our case, DPs can represent a rare cause of upper airway obstruction, ranging from intermittent symptoms or even respiratory distress [ 1 , 2 ].…”

“…DPs are frequently found in the neonatal period, but occasionally they can be seen in older children, since a DP’s clinical presentation depends on the site of origin and on its size [ 2 ]. When in the naso-oropharyngeal cavity, as in our case, DPs can represent a rare cause of upper airway obstruction, ranging from intermittent symptoms or even respiratory distress [ 1 , 2 ]. Other clinical presentations described are feeding disorders with vomits, hemoptysis, hearing loss, otorrhagia, snoring, and recurrent ear infections [ 1 , 8 ].…”