• Korean Journal of Bronchoesophagology
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• v.5 no.1
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• pp.68-72
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• 1999

Odontogenic keratocyst is a central destructive lesion of the jaws characterized by a thin, fragile layer of orthokeratinizing or parakeratinizing stratified squamous epithelium. Correlation between the histologic type and the recurrence ratio remains a subject of controversy, and multiple cysts are known to be associated with the nevoid basal cell syndrome. We experienced a case of multiple odontogenic keratocyst in a 25 year-old male patient involving bilateral mandible and maxilla. The cystic mass of the right maxilla was removed by Caldwell-Luc's approach and the right mandibular mass was removed by intraoral approach but the teeth that were impacted in the mandibular bone were remained in order to prevent an iatrogenic fracture.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.32 no.1
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• pp.81-85
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• 2010

Multiple jaw cysts are one of the most constant features of the basal cell nevus syndrome. Basal cell nevus syndrome is inherited as an autosomal dominant trait with variable expressiveness. This syndrome comprises a number of abnormalities such as multiple nevoid basal cell carcinomas of the skin, skeletal abnormalities as bifid rib and fusion of vertebrae, central nervous system abnormalities as mental retardation, eye abnormalities with multiple jaw cysts. The odontogenic keratocysts in patients with this syndrome are often associated with the crowns of unerupted teeth and huge size; on radiographs they may mimic dentigerous cysts. The most important feature of the cyst is its extraordinary recurrence rate. Since recurrence may be long delayed in this lesion, follow-up of any case of odontogenic keratocyst with roentgenograms and clinical examination of basal cell carcinoma are essential for at least five years after surgery. We report the result of 7-year follow up after cyst enucleation associated with basal cell nevus syndrome with the literature of review.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.44 no.3
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• pp.107-111
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• 2018

Objectives: A keratocystic odontogenic tumor (KOT) is a type of odontogenic tumor that mainly occurs in the posterior mandible. Most KOTs appear as solitary lesions; however, they sometimes occur as multiple cysts. This study analyzed the clinical features of multiple KOTs. Materials and Methods: The participants were diagnosed with KOT by biopsy with multiple surgical sites, and were patients at the Pusan National University Hospital and the Pusan National University Dental Hospital from January 1, 2005 to March 31, 2016. Charts, records, images and other findings were reviewed. Results: A total of 31 operations were conducted in 17 patients. The mean patient age was $28.4{\pm}20.1years$. Multiple KOTs were found to occur at a young age (P<0.01). The predominant sites were in the posterior mandible (28.6%). Most cases of multiple lesions appeared in both the upper and lower jaw, and 40.3% of lesions were associated with unerupted and impacted teeth. The overall recurrence rate measured by operation site was 10.4% (8/77 sites). No patients were associated with nevoid basal cell carcinoma syndrome. Conclusion: The pure recurrence rate was lower than estimated, but there was a higher possibility of secondary lesions regardless of the previous operation site; therefore, long-term follow-up is necessary.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.32 no.5
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• pp.431-436
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• 2010

Purpose: It is estimated that the odontogenic keratocyst (OKC) makes up 10% to 12% of all developmen-tal odontogenic cysts. The lesion has been of particular interest because of its specific histopathologic fea-ture, high recurrence rate, and aggressive behavior. Materials and Methods: We investigated 266 OKCs of Korean patients for the sex of patient, the age of the patient, the location of OKC, the recurrence rate related to radiographic impression. Results: The male-to-female ratio was 1.47:1, showing a slight male predilection. Odontogenic keratocysts had a peak of occurrence in the third decade of life. The mandibular angle and ascending ramus area (49.6%) is the most frequent site of OKCs in the jaws. Fourteen cases of unilocular (12%) and 5 cases of multilocular (20%) OKCs recurred. Thirteen cases of smooth (12.9%) and 6 cases of lobulated (14.6%) OKCs recurred. Seventeen cases of OKCs without perforation of cortical bone (12.5%) and 2 cases of OKCs with perforation of cortical bone (33.3%) recurred. Fifteen people of patients with single lesion (12.2%) and 4 people of patients with multiple lesions (66.7%) recurred. Conclusion: In this resul, we consider multiple odontogenic keratocysts can recur more easily. So we have to treat them more carefully and need long-time follow-ups.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.12 no.1
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• pp.127-134
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• 1990

NBCCS is a hereditary disorder characterized by multiple defects of ectodermal and mesodermal structure, which is chiefly affected on skin, jaws & skeleton, CNS, eye and sexual organ. It is well associated with multiple odontogenic keratocyst on the jaws. Thus, we intended to investigate other anomalies presenting on NBCCS in cases of multiple odontogenic keratocyst which were treated in our hospital from 1980 to 1989. Here we reported 6 cases of NBCCS.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.21 no.1
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• pp.74-80
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• 1999

A family is presented in which multiple odontogenic keratocysts have been expressed. 3 members of the two generations of family with features of multiple keratocysts, suspected basal cell nevus syndrome have been investigated. The proband, 19-year-old woman and her mother, and her brother suffered from these cysts and two female patients' disease have been recurred multiply though careful treatments. Close attention to the family and routine follow up will alert the clinician to its recurrences and genetic counseling and serial screening for the development of other symptoms, that is malignant skin carcinoma etc.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.2
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• pp.172-177
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• 2008

Basal cell nevus syndrome, also known as Gorlin-Goltz syndrome, is a hereditary condition transmitted as an autosomal dominant trait that exhibits high penetrance and variable expressivity. It is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. Odontogenic keratocysts, palmar and plantar pits, and hypertelorism are the most frequently observed anomalies. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report two cases of multiple odontogenic keratocysts associated with basal cell nevus syndrome.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.31 no.5
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• pp.429-434
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• 2009

Basal cell nevus syndrome is a rare inherited disorder characterized by mulitple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, skeletal abnormalities and ectopic calcifications. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report a case of a 9-year-old boy with the various manifestation of basal cell nevus syndrome, which are multiple odontogenic keratocysts, pits of the soles, bifid ribs, ectopic calcification, macrocephaly, and hypertelorism, etc. Total five odontogenic keratocysts were found. For the reduction of the size of the odon-togenic keratocysts, following preoperative marsupialization, there were surgically enucleated. And the impacted upper right lateral incisor and canine are tracted orthodontically.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.789-793
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• 2007

Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the clinical features of basal cell nevus syndrome, in which a PTCH gene mutation was confirmed by DNA testing.