Medicine; pathology

Epignathus

S. C a l d e r o n ,

Case report of long-term survival with no surgery

I. K a p l a n ~, M. G o r n i s h 2

Department of Oral and Maxillofacial Surgery and 2Section of Neuroradiology, Department of Radiology, Beilinson Medical Center, Petah Tiqva, and 1Section of Oral Pathology and Oral Medicine, Sackler School of Dental Medicine, Tel Aviv University Israel

S. Calderon, I. Kaplan, M. Gornish: Epignathus: Case report o f long-term survival with no surgery. Int. J. Oral Maxillofac. Surg. 1991; 20." 322-324. Abstract. Clinical and radiological features of an epignathus of the soft palate and oropharynx are presented. The case was conservatively treated and followed for 8 years.

Key words: epignathus; oropharynx, teratoma. Accepted for publication 18 July 1991

Epignathus is a term used for teratomas arising in the oral cavity, and attached to the jaw. The definition may differ among different authors 2, 3, 6, 7, 14. Epignathi are benign in most cases. They may be attached to either maxilla, mandible, palate or base of the skull and the lesion may invade the cranium and, nasal or oral cavities. The location and size of these tumors are often incompatible with life, causing prenatal or perinatal death 6, ~3,15. Very few cases of successful surgical removal of the tumor have been reported I' 3, 8, 16. In some cases epignathi are associated with other malformations, such as cleft palate 1, 14, abnormal mandibular structure 3, 6, 14 and separation of nasal bone 1. Macroscopically, tissues such as teeth, bone, hair and skin can occasionally be identified in these tumors 9, 10. Histologically they show disorganized neoplastic tissue of various types. W h e n small, some of these tumors are usually simple in structure, containing adipose tissue with a center of muscle, cartilage or bone and stratified squamous epithelium. Tumors of greater structural complexity are usually fatal and thus seen mostly in stillborns7. A 4½-month-old female child with

Fig. 1. Protruding multilobulated mass in the soft palate.

Fig. Fig. Fig. Fig.

2a. 2b. 2c. 2d.

Focus of fat density (small arrow). Note focal calcification (white nodule). Fluid density in mass (arrowhead). Note incomplete hard palate (open arrow). Upper extent of lesion. Subtle soft tissue fullness on patient's right.

Epignathus

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6, 10, 11, 13 16. Only a few o f these h a d been successfully r e m o v e d 1, 3, s, 16. M o s t o f these children died shortly after b i r t h 3. E p i g n a t h u s occurs m o r e frequently in females, at a 1:3 ratio. N o e x p l a n a t i o n has yet been f o u n d for the female predilection a n d for the relative rarity o f e p i g n a t h u s c o m p a r e d to t e r a t o m a s in o t h e r locations 3. A n associated defect in the h a r d palate structure exists in this case as in o t h e r r e p o r t e d cases 1, 3, 6, 14. M a l i g n a n t t r a n s f o r m a t i o n of t e r a t o m a s m a y occur, particularly in those lesions with p o o r l y differentiated tissues, t h o u g h this is rare 2, 3, s.

Fig. 3a, b. Semi-coronal CT scans through the oro-nasopharynx. Lobulated soft tissue mass indicated by arrow.

e p i g n a t h u s in the soft palate is presented. She has since been r e x a m i n e d a n d followed for 8 years, w i t h o u t perf o r m i n g surgery.

Case report A female infant was first examined at 4½ months of age because of a protruding multilobulated mass in the soft palate. The mass was 3 to 4 cm in size and hard on palpation. It displaced the uvula to the left and resulted in a marked distortion and narrowing of the oropharynx. Incomplete closure of the hard palate was noted but the uvula was not cleft (Fig. 1). When the patient was 4 years of age, a computed tomographic scan was performed of the oropharyngeal area in the axial and coronal planes (Figs. 2 and 3). Figs. 2a-d are a sequence of axial scans from the level of the anterior maxilla, below the palate at the C3 level (Fig. 2a), through the nasopharynx at the level of the coronoid process of the mandible at the C1 level (Fig. 2d). Figs. 3a, b are semi-coronal scans. The plane of Fig. 3a passes from the anterior clinoid process above, through the pterygoid process and the mentum below. The plane of Fig. 3b passes from the basisphenoid above through the angle of the mandible. Distortion of the usually symmetric oropharyngeal air coh~mn is seen in black on all .the scans. On Fig. 2c the hard palate is seen to be incomplete to the left of the midline. On Figs. 2a, 2b and 3b a lobulated soft tissue mass is present on the right side. The lesion has mixed soft tissue densities, including fat (Fig. 2a, arrow), focal calcification (white nodule on Figs. 2a and 3b) and fluid density areas (Fig. 2b, arrowhead).

In the case reported, the epignathus, first n o t e d at age 4½ m o n t h s , appears c o m p a t i b l e with life a n d does n o t seriously affect the child's n o r m a l function. Since a well-developed t o o t h was f o u n d in the lesion it is p r o b a b l y a well-differentiated t u m o r with a very small chance of m a l i g n a n t t r a n s f o r m a t i o n . She is being closely observed for a n y changes in t u m o r size, in which case complete removal will be performed. This case appears to be u n i q u e with respect to the relatively large size o f the t u m o r a n d the p a t i e n t ' s survival w i t h o u t surgery.

References

Fig. 4. Patient at 8 years. Note growth of teeth. Right-sided lesions remains with deviation of uvula.

A biopsy was performed and the material proved to be a tooth of apparently normal structure. The location was obviously ectopic, which led to the diagnosis of teratoma. The child showed no signs of dyspnea or dysphagia and, since complete excision of the tumor would have necessitated immediate reconstruction of the soft palate by pharyngeal flaps, the parents refused to authorize complete removal of the lesion. The child is being followed periodically without evidence of any change in the size of the tumor. At 7 years of age, she is functioning normally (Fig. 4) except for a minor nasal speech defect due to velopharyngeal insufficiency (Fig. 4).

Discussion E p i g n a t h u s is a t o p o g r a p h i c term attrib u t e d to t e r a t o m a s arising f r o m the oral cavity. A review o f the literature revealed n u m e r o u s cases of epignathi 1, 3, 4,

1. BENNETT JP. A case of epignathus with long term survival. Br J Plast Surg 1970: 23: 3604. 2. GROSFELD JL, BALLANTINETV, LOWE D, BAEHNERRL. Benign and malignant teratoma in children: analysis of 85 patients. Surgery 1976: 80: 297-305. 3. HATZIHABERISF, NTAMATISD, STAURINOS D. Giant epignathus. J Pediatr Surg 1978: 13:517 8. 4. HmABAYASmS, UEDA K. Nasopharyngeal teratoma attached to the lower jaw. Plast Reconstr Surg 1985: 76: 93941. 5. HUDSON JW, JAFFREY B, CHASE DC, GRAY J. Malignant teratoma of the mandible. J Oral Maxillofac Surg 1983: 41: 540-3. 6. KESWAN~RK, CHUGH TD, DHALL JC, et al. Epignathus: a case report. Br J Plast Surg 1968: 21: 355-9. 7. LUCAS RB. Pathology of tumours of the oral tissues. London: Churchill Livingstone, 1976: 288. 8. MAEDA K, YAMAMOTOT, YOSHIMURAH, ITOH H. Epignathus: a report of two neonatal cases. J Pediatr Surg 1989: 24: 139-44. 9. MILLER AP, OWENS JB. Teratoma of the tongue. Cancer 1966: 19: 1583-6. 10. OCHSNER A, AYERS WB. Case of epignathus. Survival of the host after its excision. Surgery 1951: 30: 560-4.

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11. RINXALaA, RANTA R. Separate epignathi of the mandible and the nasopharynx with palate: case report. Br J Plast Surg 1979: 27: 1072-5. 12. ROSE PE, HOWARD ER. Congenital teratoma of the submandibular gland. J Pediatr Surg 1982: 17: 414-6. 13. STRAUSSBERG R, SIROTA L, BAR-ZIv J, LANDMANJ, DULITZKI F. Teratoma of the head and neck in infancy. Israel J Med Sci 1989: 25: 654-6.

14. TUSON KW. Epignathus: basicranial teratoma. Br J Surg 1971: 58: 935-8. 15. VINTNERS HV, MURPHY J, WITTMANN B, NORMAN MG. Intracranial teratoma: antenatal diagnosis at 31 weeks by ultrasound. Acta Neuropathol (Berl) 1982: 58: 233-6. 16. ZAKARIA MAK. Epignathus (congenital teratoma of the hard palate): a case report. Br J Oral Maxillofac Surg 1986: 24: 272-6.

Address: S. Calderon, DMD Department of Oral and Maxillofacial Surgery Beilinson Medical Center Petah Tiqva 49 100 Israel

Epignathus. Case report of long-term survival with no surgery.

Clinical and radiological features of an epignathus of the soft palate and oropharynx are presented. The case was conservatively treated and followed ...
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