A Large Congenital Epulis With Associated Incomplete Cleft Lip
Congenital Epulis (congenital granular cell tumor) is a relatively uncommon benign mass which, when occurs, is always present at birth. The lesion is commonly small and may involute with no intervention. Occasionally, the size of the lesion may necessitate surgical intervention secondary to airway concerns or difficulty feeding. The following is a case of an unusually large congenital epulis with a closely associated incomplete cleft lip.
CASE: The Oral and Maxillofacial Surgery service at Westchester Medical Center was consulted by the Neonatal ICU to evaluate and treat a one day old female with a large lesion protruding from her oral cavity. The otherwise healthy female was born one day prior at an outside facility. The lesion measured approximately 6x8cm at birth and was not detected on any prenatal sonograms per the mother. The APGAR scores were 9 and 9 at 1 and 5 minutes. The birth weight and length were in the 50th and 90th percentile respectively. The patient was transferred to Westchester Medical Center for further management. There was no reported airway depression as birth and the patient was transported without airway protection. At the time of consultation the patient had been intubated in preparation for an MRI. A lobulated lesion measuring approximately 5cm diameter was noted to be protruding from the right side of the patient’s anterior maxilla. The lesion was compressible with a doughy consistency, and was pedunculated on anterior maxillary alveolar ridge right of midline. Although superficial vasculature was visible on the surface of the lesion, no bruit or thrill was detected and the lesion did not blanch upon compression. visible on the surface of the lesion, no bruit or thrill was detected and the lesion did not blanch upon compression.
The remainder of the physical examination and laboratory studies was not remarkable. T1 and T2 weighted pre and post contrast MRI as well as MRA studies were obtained. The lesion in the T1 non contrast enhanced images was iso-intense to the surrounding tissue. T2 non contrast enhanced and T1 contrast enhanced images demonstrated splotchy areas of enhancement, mostly in the periphery of the lesion. A single prominent right external carotid artery branch possibly of the internal maxillary artery was noted on the MRA supplying the lesion.
The clinical preoperative diagnosis was congenital epulis. It was thus decided to remove the lesion in its entirety without incisional biopsy. The patient was taken to the OR and placed on the operating room table. As discussed the patient was already intubated. The area surrounding the lesion was painted with 10% providone iodine. The stalk was then isolated and double clamped using Kelly forceps. The stalk was then sharply divided between the clamps using a #15 scalpel blade. The proximal stump was then ligated with 0 silk and the proximal clamp was then removed. This technique resulted in zero blood loss. The patient was extubated later that day and was tolerating PO by that evening. Pt was discharged the next day. At follow up, on post op day ten, the stump and silk suture had fallen off and the surgical site was clean and hemostatic. The post op course was uneventful and the patient was developing appropriately. The final diagnosis of the lesion was Congenital Epulis. Upon removal of the lesion an incomplete right sided cleft lip was noted. The location of the cleft corresponded exactly to the stalk of the lesion. The primary cleft repair was performed 2 months later.
Discussion: Congenital epulis are rare benign solid tumors of unclear origin. They have been described as hamartomas. Most are 2cm or less in diameter. Smaller lesions have been known to involute with no intervention. There is a large female predilection. In 10% of cases the patient will present with multiple lesions. It is more commonly found in the maxilla and lateral to the midline. In those cases where the lesion interferes with feeding or there is concern for airway patency excision is indicated. Excision, even incomplete, is considered curative.
Histologically, the lesion is similar to the adult granular cell tumor or myoblastoma. They contain syncytial sheets of round to polygonal cells with eosinophilic granular cytoplasm. The congenital epulis is noticeably more vascular than the adult version. Ultrastructurally, congenital epulis demonstrates intermediate filaments with fusiform electron densities suggests that some of the granular cells have morphologic attributes of myofibroblasts. The current consensus on the adult tumor etiology is the Schwann cell. This theory behind separate etiologies is bolstered by the fact that CE stain uniformly negative for S100 in contrast with the adult version.
An argument can be made in this case for the presence of the tumor interfering with fusion of the right maxillary process with the median nasal process thereby directly causing the formation of the incomplete cleft. Hamartomas grow at the same rate as the surrounding tissues. Judging by the size of the tumor it can by argued that it was present in early gestation at the point of fusion of the maxillary processes. Several articles have postulated mechanical obstruction etiologies for palatal clefts but an exhaustive literature search revealed no articles suggesting a mechanical etiology for cleft lip.