Oral Health Group
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Oral & Maxillofacial Surgery: Multiple Jaw Cysts in a Syndromic Patient

July 1, 2003
by Oral Health


Carolina Ortega, DDS, Jessica Kudlats, DDS, Nick Blanas, DDS, FRCD(C) and Simon Weinberg, DDS, FRCD(C)

Odontogenic and non-odontogenic jaw cysts usually occur as solitary lesions although the presence of multiple lesions in one patient is possible. The presentation of multiple cysts of the maxilla and mandible is more common when they occur as a component of craniofacial syndromes. The report that follows describes the occurrence of multiple jaw cysts in a syndromic patient.

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CASE REPORT

A 19-year-old male was referred by his dentist for assessment and management of multiple cyst-like lesions of the jaws. The patient was asymptomatic and the lesions in question were incidental findings on routine dental radiographic examination. Physical examination showed that the patient was of a small stature, with facial acne and mild facial asymmetry, a retrognathic mandible and with slight fullness of the left side of the face. There appeared to be no frontal bossing or hypertelorism. Intra-orally, there was a full permanent dentition except for the wisdom teeth and the mucosa was grossly normal. There was mild, diffuse fullness in the left mandibular buccal vestibule and tooth #37 was tipped lingually (Fig. 1).

Plain films including panoramic, occlusal, and periapical radiographs showed a large, corticated, scalloped radiolucency in the left ramus, angle, and posterior body of the mandible. The lesion extended superiorly from just beneath the sigmoid notch and the coronoid process to the inferior border which appeared thinned. Anteriorly, the lesion extended from the distal of tooth #36 to the posterior border of the ramus. Tooth #38 was displaced into the posterior border. There was minor buccal expansion of the mandible. A distinct follicular space was noted around tooth #38. In the right maxilla, tooth #18 was displaced superiorly and was associated with a large mixed density lesion with well-defined borders. Tooth #48 appeared to have an enlarged follicular space (Fig. 2).

A panoramic radiograph taken when the patient was 16 years old shows teeth #18 and 37 to be displaced in comparison to their contralateral counterparts (Fig. 3).

Axial and coronal CT images showed calcification of the falx cerebri and confirmed the presence of large radiolucencies in the right maxillary sinus and in the left posterior mandible (Figs. 4-6). A bifid rib was noted on chest x-ray in the left thorax (Fig. 7).

Under general anesthesia and nasoendotracheal intubation, the lesions in the right maxilla and left mandible were accessed via intraoral incisions. Following the elevation of full thickness mucoperiosteal flaps, both were enucleated and the resulting bony cavities aggressively curettaged (Fig.8). The inferior border of the mandible on the left side was intact, although there were extensive perforations of the buccal and lingual cortical plates. The inferior alveolar nerve was found to be intact at the inferior border. Teeth #18, 28, 38, and 48 were extracted at the same time. All excised tissue was submitted for histopathologic assessment. Chemical cauterization of the bony cavity in the left mandible was carried out using Carnoy’s solution, which is composed of Absolute alcohol, glacial acetic acid, chloroform, and Ferric Chloride (Fig. 9).

The histopathologic report described a thin uniform lining of parakeratinized, stratified squamous epithelium with a lumen containing variable amounts of desquamative keratin. There was focal separation of the epithelial lining from the connective tissue with a lack of rete peg formation. In addition, there was formation of dental lamina rests and daughter cyst within the capsule wall (Fig. 10).

Based on the clinical, radiographic and histologic findings, a final diagnosis of odontogenic keratocyst (OKC) was made for the lesions in the left mandible and right maxilla. Given the presence of the bifid rib and calcified falx cerebri, it is quite likely that the jaw cysts in this case are part of the Gorlin-Goltz syndrome.

DISCUSSION

OKC usually present as single lesions with a predilection for the posterior body and ascending ramus of the mandible as well as the posterior segment or cuspid region of the maxilla. Occassionally, they can occur as multiple cysts occupying all four quadrants of the jaws.

On radiographs, they usually present as well-defined radiolucencies with thin corticated margins that are often associated with crowns of unerupted teeth in a relationship which is not necessarily dentigerous. Tooth resorption and bony expansion are possible but not always evident.

The histology of OKC is characteristic. These cysts consist of thin fibrous connective tissue walls lined by a uniform layer of ortho or parakeratinized, stratified, squamous epithelium, which is six to ten cell layers thick. Rete ridges are often absent and the lumen may be filled with keratin. Epithelial cell rests as well as daughter cysts may be present in the cyst wall.

Methods of treating OKC include enucleation, curettage, marsupialization, cryotherapy, chemical cauterization and surgical resection. This case report describes the use of Carnoy’s solution in combination with enucleation and curettage to treat the odontogenic keratocyst in the left posterior mandible.

The OKC recurrence rate has been shown to be between 17-56% if enucleation alone is used. Comparatively, if Carnoy’s solution is added, the recurrence rate is 1.0-8.7%.1 Recurrences can occur as late as five to ten, or more, years following surgery.1,2 Given this propensity to recur and to recur late, patients with OKC must be followed clinically and radiographically, long term.

Multiple OKC in the same patient is one of the features of Gorlin-Goltz Syndrome (Gorlin Syndrome, Nevoid Basal Cell Carcinoma Syndrome). Other findings of this autosomal dominant condition include rib and vertebral anomalies, multiple basal cell carcinomas and epidermoid cysts (milia) of the skin, frontal and temporo-parietal bossing, hypertelorism, palmar and plantar pits, and calcification of the falx cerebri. In rare instances, ovarian fibromas, medulloblastomas and shortened metacarpals may occur.2

Most anomalies of Gorlin-Goltz Syndrome are minor and not life threatening. In most patients, the ultimate prognosis is dependent on the behaviour of the basal cell carcinomas, the size of the jaw cysts, and the treatment modalities used. If it is suspected that a patient may have Gorlin-Goltz Syndrome, referral for genetic testing and counseling, as well as a referral to a dermatologist for regular, long term monitoring is prudent.

Carolina Ortega and Jessica Kudlats are Residents, Department of Dentistry, Sunnybrook and Women’s College Health Sciences Centre.

Nick Blanas is Associate in Dentistry, Faculty of Dentistry, University of Toronto, and Staff Oral and Maxillofacial Surgeon, Department of Dentistry, Sunnybrook and Women’s College Health Sciences Centre.

Simon Weinberg is Professor Emeritus, Faculty of Dentistry, University of Toronto, and Staff Oral and Maxillofacial Surgeon, Toronto General Hospital. Dr. Weinberg is Oral Health’s editorial board member for Oral and Maxillofacial Surgery.

Oral Health welcomes this original article.

REFERENCES

1.Blanas N, Freund B, Schwartz M, Furst IM: Systematic review of the treatment and prognosis of the odontogenic keratocyst. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90:553-8, 2000.

2.Waldron CA: Odontogenic cysts and tumors. In Neville BW, Damm DD, Allen CM, Bouquot JE (eds.): Oral and Maxillofacial Pathology. Toronto, WB Saunders, 1995, pp 493-540.