From the Departments of Clinical Surgery and Pathology, Government Medical College, Rohtak (Haryana), India

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Samson Logan
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1 EPIGNTHUS-- CSE REPORT By R. K. KESWNI, M.S., F.R.C.S.(C), D..B., T. D. CHUGH, M.D., J. C. DHLL, M.B., B.S., and G. C. MEHROTR, M.D. From the Departments of Clinical Surgery and Pathology, Government Medical College, Rohtak (Haryana), India TERTOMTOUS lesions occurring in the mouth in the newborn are generally known as epignathi, though strictly speaking this term implies a derivation from the jaws. Malignant changes in these tumours have not yet been described, but these infants seldom survive the neonatal period. These tumours vary in structural complexity. The simpler types are compatible with life. Smaller growths may not be immediately evident as they arise farther back in the oral cavity. These consist mainly of adipose tissue with a central area of skeletal muscle or cartilage or bone and covered by stratified squamous epithelium. Tumours of any greater degree of complexity than those just mentioned are practically always fatal and are seen therefore in the foetus or stillborn infants or, as mentioned above, cause death in the neonatal period. Larger growths may be attached to the roof of the pharynx, palate or alveolus. The base of the skull is not always affected and the tumour protrudes out of the oral cavity. Their size may be as big as that of a grapefruit. Sometimes these tumours are hour-glass shape with an intracranial extension (Willis, 1962 ; Lucas, 1964). Case Report.-- newly born male child was brought to the hospital four hours after birth, with the chief presenting symptom of a large polypoid tumour protruding from the oral cavity. The extra-oral part of the tumour measured about 8 cm. 5 cm. There was no evidence of difficulty in breathing. The child appeared quite healthy and weighed about 6"5 lb. (Fig. I, ). The following associated deformities were noted : the left ear was slightly short and deformed ; preputial phimosis ; cleft soft palate. Local Examination.--The tumour arose from the left alveolar margin and adjoining anterior surface of the maxilla and hard palate. The pedicle appeared narrow and measured 3 cm. in diameter. The turnout had a constriction about its centre outside the oral cavity. The oral fissure was widely open and filled tightly with the protruding mass, more so on the left side. The mandible was correspondingly deformed with caudal arching of the mandibular body, also more so on the left side. The prominence of the cheek was pushed upwards. Temporomandibular joints were felt to be in their normal position. The infant was given a few drops of water to drink and the swallowing reflex appeared normal and without any evidence of obstruction. During bouts of crying no increase in tension was felt in the tumour, thus reducing the possibility of intracranial extension. Externally, the colour of the tumour was variegated with areas of reddish brown colour intermingling with white membranous areas. In one place, it was covered with a knob of skin under which there was felt to be a small cyst. The consistency was variable from firm to hard. s the patient was not in distress, operation was delayed to the next morning. Operation.--Intubation was done through the nose without anmsthesia and the patient was then anmsthetised with N20 and 02. The tumour was easily excised from the left alveolar arch, maxilla and palate. remnant of the pedicle containing hard particles was also excised from the region of the anterior surface of the maxilla. Bleeding was minimal and easily controlled. The raw area was covered with adjoining mucosa. t the end of the operation, it was seen that the patient could not close the mouth because of deformity of the mandible. The mouth was covered with wet gauze to prevent drying of oral tissues. 355

FIG. I, Pre-operative photograph of the baby showing a polypoid tumour filling the

B, Immediately postoperative in maximum close-mouth position.

C, Five months post-operative, shortly before death.

mandibular deformity. C FIG. 2, X-ray skull, P.

2 FIG. I, Pre-operative photograph of the baby showing a polypoid tumour filling the oral fissure with a constriction in the lower part. B, Immediately postoperative in maximum close-mouth position. The left mandibular arch is grossly deformed. C, Five months post-operative, shortly before death. There is cystic recurrence in the left alveolar arch with no improvement in mandibular deformity. C FIG. 2, X-ray skull, P.. view, two weeks after surgical excision of the tumour. Shows opacity in the left maxillary area, obviously a remnant of the tumour. B, X-ray of the specimen, following excision of the turnout. Reveals osteoid tissue resembling limb structures. B

EPIGNTHUS-- CSE REPORT 357 Examination of the Specimen.--The specimen measured I2 x 5 cm. T h e gross appearance o f the t u m o u r was polypoid with a constriction in the lower part.

Reveals polypoid structure with cystic spaces, and variegated colour. 13, Shows well-keratinised strips of stratified squamous epithelium and epithelial pearls.

3 EPIGNTHUS-- CSE REPORT 357 Examination of the Specimen.--The specimen measured I2 x 5 cm. T h e gross appearance o f the t u m o u r was polypoid with a constriction in the lower part. T h e consistency was firm to hard. T h e cut surface was also firm to hard and revealed a few small cystic spaces. B C D FIG. 3, Photograph of the specimen cut in the vertical plane. Reveals polypoid structure with cystic spaces, and variegated colour. 13, Shows well-keratinised strips of stratified squamous epithelium and epithelial pearls. C, Shows cartilaginous tissue and a single glandular structure with intervening connective tissue. D, Shows multiple small glandular structures, lymphoid, adipose and connective tissue. X-ray Examination (Fig. 2, B ). - - L i n e a r and irregularly shaped calcified structures were seen, some of which resembled the terminal parts o f the extremities. However, no definite resemblance could be seen between the structures and the known shape of bones. Microscopic Examination (Fig. 3).--Tissues from all the three germinal layers could be seen. Predominant tissues were well-keratinised strips o f stratified squamous epithelium (Fig. 3, B), cartilaginous tissue (Fig. 3, c), at places heavily calcified, well-developed osteoid

358 BRITISH JOURNL OF PLSTIC SURGERY tissue, adipose tissue, fibro-muscular and lymphoid tissue. few glandular structures (Fig. 3, c and D) could also be seen.

4 358 BRITISH JOURNL OF PLSTIC SURGERY tissue, adipose tissue, fibro-muscular and lymphoid tissue. few glandular structures (Fig. 3, c and D) could also be seen. ll these tissues were seen lying jumbled up without forming ally definite organ. Follow-up.--In the post-operative period, the patient presented a difficult feeding problem and had to be assisted with tube feeding. Despite this, he was gradually losing weight. n attempt at gradual moulding of the mandible was made with the help of a head cap and elastic nylon band passing below the chin but with no immediate improvement. X-ray (P.. view) (Fig. 2, ) of the face after the operation revealed a radio-opaque shadow in the left maxillary area, i.e. the site of attachment of the pedicle. lateral view of the skull, however, did not reveal any intracranial extension of the same pathology. The patient was examined at two-monthly intervals and found to be losing weight. t five months, he was found to be very merasmic and weighed 3 lb. The tumour had recurred and presented as a large cystic swelling (about 7 cm. in diameter) in the left gingivo-buccal sulcus. The mandible deformity had not improved. few days after the last examination, the patient died. Comments.--The interesting aspects of epignathus are (I) its rarity; (2) the complexity of its internal structure making it incompatible with life ; (3) anmsthetic management ; (4) the cause of death during and after the neonatal period ; (5) management of the mandibular deformity. The anmsthetic management in this case was adequately handled by nasal intubation. Oral intubation was impossible as the tumour filled the oral fissure. Tracheostomy might have caused difficulties in the post-operative management of the child. However, tracheostomy has to be done when there is pharyngeal extension of this tumour (Ochsner and yan, I95I ; Sollee, I965). In the literature available, it has not been clearly mentioned how these children progress following surgical extirpation of the lesion. similar lesion was reported by Wynn et al. (I956), but surgical excision could not be done because of the poor condition of the child who died IO hours following birth. In our patient there were two causes of deterioration in his condition : (I) recurrence of the tumour leading to inanition ; (2) inadequate feeding due to mandibular deformity. The problem of management of this type of mandibular deformity raised quite a few doubts as to the correct line of treatment. One suggestion was to do a reverse Esmarch's type of procedure, but osteotomy in a newborn child might be difficult to tolerate. Besides, it probably would not have allowed complete closure of the left mandibular body because of its deformed (concave) shape. The only alternative was to assist the feeding and apply gradual traction on the mandible to help to mould it back into position. In this process, nature would help because of various muscular arrangements, e.g. masseter posteriorly and orbicularis oris anteriorly. In the case reported by Ochsner and yan (I95I), the tumour was obviously completely excised along with the portion of tongue, from where it arose. This child had a long-term survival and apparently the mandibular deformity, if it existed, had corrected itself spontaneously with the growth of the child. SUMMRY case with a rare oral teratoma (epignathus) is presented. The infant survived for five months following surgical excision of the tumour. nmsthetic, surgical and post-operative problems are discussed.

5 EPIGNTHUS-- CSE REPORT 359 I am grateful to Dr G. S. Sekhon, head of the Department of Clinical Surgery for giving me permission to publish this case; to Dr Waryam Singh, Medical Superintendent, for allowing the use of the hospital records ; to Dr L J. Dewan, Principal of the Medical College, for providing photographic and other research facilties ; and to Mr M. L. Sharma, photographer, who printed these photographs. REFERENCES LUCS, R. B. (I964). " Pathology of Tumours of the Oral Tissues." London : Churchill. OCHSNER,., and YN~ W. B. (I95I). Surgery, 30, 560. SOLLEE,. N. (I965). rchs Otolar, 82, 49. WILLIS, R.. (I962). " The Pathology of the Tumours of Children." Edinburgh and London : Oliver & Boyd. WYNN, S. K., WXMN, S., RITCmE, B.o and SKOTZK% M. (I956). m. J. Dis. Child., 91, 495.

Infratemporal fossa: Tikrit University college of Dentistry Dr.Ban I.S. head & neck Anatomy 2 nd y.

Infratemporal fossa: Tikrit University college of Dentistry Dr.Ban I.S. head & neck Anatomy 2 nd y. Infratemporal fossa: This is a space lying beneath the base of the skull between the lateral wall of the pharynx and the ramus of the mandible. It is also referred to as the parapharyngeal or lateral pharyngeal