Giant Epignathus with Intracranial Teratoma in a Newborn Infant

Abstract

A male newborn infant with a giant epignathus associated with intracranial teratoma and obstructive hydrocephalus detected antenatally is reported. Only 4 similar cases have been reported in the literature. This disorder is uniformly lethal. We believe this is the first reported case of epignathus with congenital intracranial teratoma diagnosed antenatally by ultrasonography.

Neonatal epignathus in the oral and pharyngeal regions: A case report

“…Surgical resection is the only effective way to treat teratomas . The published report of clinical findings in patients with giant epignathus is shown in Table . In the present case, although partial resection of the tumor by pediatric surgeons was made as a first step, this was unavoidable because the tumor was oppressing her face.…”

“…8 The published report of clinical findings in patients with giant epignathus is shown in Table 1. 2,3,6,11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] In the present case, although partial resection of the tumor by pediatric surgeons was made as a first step, this was unavoidable because the tumor was oppressing her face. It had been difficult to approach the tumor in the pharyngeal region before resection of the tumor protruding from the left nostril.…”

Oral teratoma (epignathus) with intracranial extension: A report of two cases

“…These very rare lesions show no evidence of a familial tendency (Levine et al, 1990), but there is a predilection for females. Most pregnancies resulting in an affected infant are complicated by polyhydramnios (Duckett and Claireaux, 1963;Hatzihaberis et al, 1978;Kang et al, 1978;Kaplan et al, 1980;Ang et al, 1990), ascribed to the development of obstruction to fetal swallowing. Placental oedema is seen in some cases and may reflect cardiac decompensation due to the presence of a comparatively large and vascular tumour (Kaplan et al, 1980).…”

“…The tumor has the histological features of a mature, benign teratoma, and is attached to an intraoral surface, most often palatal or pharyngeal (Ang et al, 1990). Occasional cases have been described with associated intracerebral teratoma, but this association is exceptional (Ang et al, 1990;Duckett and Claireaux, 1963;Ehrich, 1945). We describe two such cases, with ultrasonic and post-mortem descriptions.…”

“…Congenital oral teratoma, also referred to as epignathus, is an unusual lesion estimated to affect between 1 in 35 000 and 1 in 200 000 live-births (Levine et al, 1990;Holt et al, 1979). The tumor has the histological features of a mature, benign teratoma, and is attached to an intraoral surface, most often palatal or pharyngeal (Ang et al, 1990). Occasional cases have been described with associated intracerebral teratoma, but this association is exceptional (Ang et al, 1990;Duckett and Claireaux, 1963;Ehrich, 1945).…”