• 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.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.270-274
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• 2009

Dentigerous cysts generally encompass the crown of an unerupted tooth. These cysts are usually solitary. They are the second most common odontogenic type of cysts following radicular cysts, and are frequently associated with impacted mandibular third molars or maxillary canines. Most multiple cysts found in the jaw are odontogenic keratocysts associated with the nevoid basal cell carcinoma syndrome, mucopolysaccharidoses and cleidocranial dysplasia. Although a single dentigerous cyst is well documented in the medical literature, including the prevalence, treatment and prognosis, multiple dentigerous cysts without any systemic symptoms is unusual. Furthermore, cases involving both the maxilla and mandible are especially rare. We present the case of an 11-year-old boy with nonsyndromic multiple dentigerous cysts associated with a mandibular second premolar and a maxillary canine. The treatment was conservative and included marsupialization and eruption guidance. Further follow up is planned to rule out additional problems and the possible identification of a syndrome.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.1
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• pp.34-39
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• 2014

Nevoid basal cell carcinoma syndrome(NBCCS) is a autosomal dominant disorder, and its major manifestations are multiple basal cell carcinoma, keratocystic odontogenic tumor, rib anomalies, palmer and plantar pits, calcification of the falx cerebri. Keratocystic odontogenic tumor(KCOT) is defined as intraosseous tumor of odontogenic origin with a characteristic lining of parakeratinized stratified squamous epithelium and potential aggressive behavior. We report a case of a 3-year-old patient with nevoid basal cell carcinoma syndrome who initially presented with unilocular keratocystic odontogenic tumor in maxillary canine region. Keratocystic odontogenic tumor was treated by enucleation, and periodic follow-up check will be required for early diagnosis of additional diseases related with this syndrome.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.21 no.4
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• pp.360-365
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• 1999

This study was aimed to suggest to better treatment method of jaw cyst that the maximum diameter was wider than 3cm, using different treatment and clinical and radiographic result. We divided the 60 patients into three groups, group A(20 patients) were treated with cyst enucleation and Decalcified Freeze-Dried Allogeneic Bone(DFDB) graft, group B(20 patients) were treated with cyst enucleation and autogenous bone graft, group C(20 patients) were treated with only cyst enucleation. Each group was evaluated with panoramic radiograph and clinical sign & symptom at pre-op and post-op(immediate, 6, 12, 24, 36 month). Bone density was evaluated with disital densitometer. The result was as follows : 1. Post-Op infection was higher in group C(4 pts.) than in group A(1 pt.) and B(1 pt.) 2. Post-Op gingival recession was higher in group C(3 Pts.) than in group A(1 pt.) and B(1 pt.) 3. Anatomic distortion was higher in group C(3 Pt.) than in group A(1 Pt.), and B(1 pt.) 4. Reoperation was done in two patients who were in group C 5. There were donor site morbidity in two patients 6. There was no significant difference between group A and B in their bony density in their follow up period(p>0.05). 7. There were significant differences between group A, B and group C in their bony density until post-op 24 months but a little differences at post-op 36 months(P<0.01)

• 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.