Neonatal Palatal Mass

Epignathus. It is a tumor arising from the jaw, though it is commonly used to describe tumors or teratomas of the mouth in the newborn. {1} In the year 1940, Ewing classified these nasopharyngeal tumors into 3 types: a} dermoids - consisting of epidermal and mesodermal germ cell layers, attached to soft or hard palate and, or pharynx near midline, b} teratomas - consisting of all three germ layers, with an indifferent degree of organization and c} epignathus, consisting of teratomas with high degree of organization and recognizable structures. {2} The overall incidence of teratomas is about 1 in 4000 live births. Among them oropharyngeal lesions account for less than 2 percent. These epignathus tumors arise from the Basisphenoid at the Rathke’s pouch and descend into the oral cavity causing palatal defects. {3} There is a female predisposition. The cleft palate of mechanical origin is the most common associated anomaly. They are a rarity as so far since 1918 there are only totally 117 cases reported worldwide. {1} Maternal polyhydramnios is a classic feature in epignathus, which occurs due to reduced swallowing due to mechanical obstruction. There can be elevated levels of maternal alpha fetoprotein {AFP}. Moreover AFP levels are used as indicators for detecting the post-operative tumor clearance. Differential diagnosis includes encephalomeningocele, hygroma, and oral granular cell myoblastoma. {4} Early sonographic diagnosis is possible with very rare 1st and 2nd trimester reporting of these cases. Confirmation of the diagnosis is done by histopathology which shows – ganglion cells, cartilage, bone, respiratory epithelium, with fibrous stroma. Prognosis is poor in these babies. Surgical excision is the treatment of choice. Prior to surgery, plain X-ray and computed tomography {CT} scan must be performed to rule out intracranial extension of the tumor. {3}

References :

Ang AT, Ho NK, Ong CL. Giant epignathus with intracranial teratoma in a newborn infant. Australas Radiol. 1990; 34:358–360. Ewing's J. Teratology in neoplastic diseases. 4th ed. Philadelphia: Saunders 1940. Prakash A, Parelkar SV, Oak SN, Gupta RK, Sanghvi BV. Giant epignathus with midline mandibular cleft: Insights in embryology and management, Ann Maxillofac Surg. 2012; 2(1): 56–59. Kothari PR, Jiwane A, Kulkarni B. Congenital naso-pharyngeal teratoma with cleft palate. J Indian Assoc Pediatr Surg 2004; 9(1):42-4.