• 대한기관식도과학회지
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• 제5권1호
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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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• 제32권1호
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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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• 제44권3호
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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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• 제32권5호
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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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• 제12권1호
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• pp.127-134
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• 1990

본인은 본교실에서 치험한 악골내 발생된 각화성 낭종들중 다발성인 경우에 이상의 증례에서 동반된 이상소견을 의도적으로 조사해 봄으로써, 악골계를 담당하는 의사로서 악골내에서 다발성 낭종이 발견될 때 NBCCS을 간과해서는 안됨을 느끼게 된다. 또한 이상의 증례에서는 확연한 가족력을 찾아볼 수는 없었으나 이 증후군 자체가 상염색체 우성유전으로 인자전달이 잘 되는 것이므로 세심한 주의와 계속적인 관찰이 요하리라 생각된다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제21권1호
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• pp.74-80
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• 1999

본 교실에서는 가족력을 가지며, 하악에 다발성으로 재발된 치성각화낭종의 증례가 있어 보고하는 바이다. 환자들은 한 가족의 어머니와 두 자녀였으며 그중 어머니와 딸의 경우 낭종적출후 수차례의 재발을 보여 딸은 앞으로도 수술을 예정하고 있다. 낭종이외의 다른 기저세포모반증후군의 증거는 보이지 않았다. 처치로서 어머니와 딸의 경우 낭종적출술을 시행하였고 아들의 경우 낭종적출후 결손부의 크기가 커서 동종골이식을 시행하였다. 현재 어머니와 아들의 경우 재발은 보이지 않고 있으나 향후 지속적인 구강검사를 통해 관리예정이며 유전학적인 검사도 고려하고 있다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제30권2호
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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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• 제31권5호
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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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• 제50권8호
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• pp.789-793
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• 2007

기저세포 모반 증후군은 피부의 기저세포 암종, 악골의 치성 각화낭종, 손, 발바닥의 소와, 이소성 석회화, 기저세포 모반 증후군의 가족력 등을 주요 특징으로 하는 상염색체 우성 유전 질환이다. 저자들은 갑자기 커지는 좌측 안면부 종물을 주소로 내원한 9세 남자 환아에서 외관상 특징적인 소견 관찰되고 분자유전학적 검사에서 PTCH 유전자의 결손 확인되어 기저세포 모반 증후군 확진된 1례를 경험하였기에 보고하는 바이다.