• Imaging Science in Dentistry
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• v.34 no.4
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• pp.203-207
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• 2004

Basal cell nevus syndrome (BCNS) is principally characterized by cutaneous basal cell carcinomas, multiple odontogenic keratocysts and skeletal abnormalities. Our patient represented several characteristics of BCNS, such as, multiple odontogenic keratocysts, facial nevus, calcification of falx cerebri, parietal bossing and mental retardation. The cyst on posterior mandible showed recurrent and newly developing tendency.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.26 no.3
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• pp.305-309
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• 2000

The basal cell nevus syndrome is a well recognized entity, the major symptoms of which are basal cell nevi, multiple jaw cysts, skeletal anomalies, and ectopic calcification. The syndrome follows a hereditary pattern, which is characterized by a highly penetrant, autosomal dominant gene with multiple and variable effects. The patient often has a characteristic face, with frontal and temporoparietal bossing, which results in an increased cranial circumference. The eyes may appear widely separated, and 40 percent of patients have true ocular hypertelorism. Jaw cysts are one of the most constant features of the syndrome and are present in at least 75 percent of the patients. The cysts are odontogenic keratocysts and frequently multiple. Radiographically, the cysts in patients with basal cell nevus syndrome do not differ significantly from isolated keratocysts. The cysts in patients with this syndrome are often associated with the crowns of unerupted teeth; on radiographs they may mimic dentigerous cysts. We report a case of multiple odontogenic keratocysts associated with basal cell nevus syndrome with the literature of review.

• 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.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.48 no.6
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• pp.386-389
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• 2022

Multiple odontogenic keratocysts (OKC) are a distinguishing feature of nevoid basal cell carcinoma syndrome (NBCCS). Owing to the high recurrence rate of syndromes associated OKCs, complete surgical resection is generally recommended as a definitive treatment. Herein, we report the management of multiple OKCs with marsupialization followed by excision with peripheral ostectomy in an NBCCS patient. We then discuss lesion progression over 11 years of annual follow-ups.

• 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.

• 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.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.35 no.4
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• pp.725-730
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• 2008

Nevoid basal cell carcinoma syndrome is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. The syndrome is an autosomal dominant inherited, with a high penetration and visible expression. The syndrome is characterized by a series of associated anomalies such as cutaneous, dentofacial, skeletal, ophthalmologic, neurological, and genital anomalies. Generally, the jaw cysts are multiple odontogenic keratocysts, affecting any area of maxilla and mandible. Multiple odontogenic keratocysts of this syndrome are more recurrent than the keratocysts of non-syndrome, thus they are treated aggressively for complete removal. We report a case of multiple jaw cysts associated with nevoid basal cell carcinoma syndrome. In clinical and radiological examinations, frontal bossing, hypertelorism, mild mental retardation and two odontogenic keratocysts in both the maxilla and mandible were observed. Two cysts were treated by marsupialization. For the management of eruption of unerupted teeth, periodic recall check and orthodontic treatment are required.

• 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.

• Imaging Science in Dentistry
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• v.41 no.4
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• pp.171-175
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• 2011

Gorlin-Goltz syndrome is an infrequent multi-systemic disease which is characterized by multiple keratocysts in the jaws, calcification of falx cerebri, and basal cell carcinomas. We report a case of Gorlin-Goltz syndrome in a 23-year-old man with emphasis on image findings of keratocyctic odontogenic tumors (KCOTs) on panoramic radiograph, computed tomography, magnetic resonance (MR) imaging, and Ultrasonography (US). In this case, pericoronal lesions were mostly orthokeratinized odontogenic cyst (OOC) concerning the MR and US study, which tended to recur less. The aim of this report was to clarify the characteristic imaging features of the syndrome-related keratocysts that can be used to differentiate KCOT from OOC. Also, our findings suggested that the recurrence rate of KCOTs might be predicted based on their association to teeth.