Congenital epulis also known as congenital granular cell tumor, congenital granular epulis, congenital granular cell myoblastoma and congenital granular cell fibroblastoma is a very rare condition among newborn and is predominant in females. The tumor is benign with no clear etiology but is reported to be hormone-related although other theories do exist. The female predominance argues in favor of the endocrine theory. Histological and electron microscope studies suggest a reactive theory in which the tumor results from stromal gingival cells such as fibroblasts or histiocytes. The cases of spontaneous regression in the literature and the absence of recurrence after incomplete tumor resection support this theory.

They are mostly recognized at birth or just after birth except in cases where the size is very small and, therefore, absence of symptoms. Prenatal diagnosis remains difficult because of the absence of specific signs and also as the tumor generally develops beyond the 22nd week of gestation. Fetal three-dimensional ultrasound and magnetic resonance imaging (MRI) can provide the diagnosis by the 36th week of gestation, thereby helping to decide and plan in advance an early multidisciplinary management of the newborn with participation from the neonatologist, the maxillofacial or head and neck surgeon or the pediatric surgeon and the anesthesiologist.

Classically, there is a single firm tumor with a quite regular surface, sometimes multilobed, sessile or pedenculated, pink or red in color and nonpainful on palpation. The tumor size varies from a few millimeters to around 10 cm at its widest diameter. The maxillary location opposite the future canines or incisors is the most frequent, but the mandibular region can also be involved. Multiple lesions or maxillary and mandibular locations have been described in 5–16% of the cases.

Although benign, immediate surgical intervention is required because of risk to patient’s life due to asphyxia. Some other common clinical manifestations include dyspnea, cyanosis, cough, difficulty in sucking and swallowing and occasionally, vomiting.

Perinatal MRI of the oral cavity helps in differentiating congenital epulis from other masses of oral cavity and in determining characteristics of the mass. It is, therefore, a recommended procedure for surgical planning. Literature shows no evidence of surgical excision affecting the growth of the bone or the eruption of teeth. Surgery can be performed under local or general anesthesia and the choice between the two depends on the size, number and location. Authors have shown preference for general anesthesia when tumor size is large or is present at multiple locations. With single and sessile tumor local anesthesia is preferred.

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