• Maxillofacial Plastic and Reconstructive Surgery
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• v.36 no.1
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• pp.7-12
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• 2014

Nevoid basal cell carcinoma syndrome (NBCCS) is inherited as an autosomal dominant trait with variable conditions, including multiple basal cell carcinoma, numerous keratocystic odontogenic tumors (KOTs) in the jaws, ectopic calcification of the falx cerebri, bifid ribs, macrocephaly, kyphoscoliosis, cleft palate, frontal and temporal bossing, mild ocular hypertelorism, mild mandibular prognathism, vertebral fusion, and so on. A 16-year-old boy visited the Dong-A University Medical Center, requiring diagnosis and treatment of multiple cystic lesions. He presented with many conditions related to NBCCS, including multiple KOTs, bifid rib, cleft lip, frontal bossing, mild ocular hypertelorism, and mild mandibular prognathism. No characteristic cutaneous manifestations (nevoid basal cell carcinoma) were observed in this patient. We report on a case of multiple KOTs associated with NBCCS with a review of the literature.

• 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.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.3
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• pp.489-497
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• 2009

Occurrence of multiple cysts in jaw bone is rare compared to solitary cysts. numerous cysts occurring in jaw bone which not accompany any syndromes are defined as multiple jaw cysts, and most of these cases in children are keratocystic odontogenic tumor (KCOT) Multiple KCOT occurring in children are often associated with basal cell nevus syndrome(BCNS), so if multiple cysts are found on the radiograph, we suspect this syndrome and pursue clinical and pathological tests. In this case, a pediatric patient, reporting with multiple cysts in the jaw was suspected of BCNS, but hasn't shown any other symptoms of this syndrome up to date, and has kept repeating surgical operation and recurrence of the tumor. Although no symptoms besides multiple jaw cysts is present, it is often reported that other symptoms appear late in the patient's age. Therefore, in cases where multiple odontogenic tumors are found in children, continuous radiographic and clinical follow-ups in order to check the progress of the syndrome is considered important.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.41 no.1
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• pp.37-42
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• 2015

Aggressive benign odontogenic neoplasms have substantial potential to grow to an enormous size with resulting bone deformities, and they often invade adjacent tissues and spread beyond their normal clinical and radiographic margins; as such, they have a high rate of recurrence. Historically, management (conservative versus aggressive) on the basis of clinical, radiographic and/or histopathologic characteristics has been controversial. However, recent advances in the understanding of the biological features of these lesions may provide greater evidence of the benefits of conservative management. Three patients with different complaints and final histopathologic diagnoses were enrolled in the study. All three cases were treated by a single operator with similar conservative surgical procedures. During follow-up, the patients had uneventful secondary healing and bone regeneration, less packing time than previously reported, no clinical or radiographic evidence of recurrence and no apparent deformity. The aggressive behavior of these lesions requires long clinical and radiographic follow-up. Conservative surgical management may be an option to reduce recurrence and morbidity and increase the probability of uneventful secondary healing and bone regeneration.

• Journal of the korean academy of Pediatric Dentistry
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• v.29 no.2
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• pp.196-203
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• 2002

Most dentigerous cysts are solitary. Bilateral and multiple dentigerous cysts are rare and occur typically in association with a number of syndromes such as Maroteaux-Lamy syndrome, Hunter's syndrome, Basal cell nevus syndrome, Marfan syndrome, cleidocranial dysplasia. The presented case is of bilateral nonsyndromic, dentigerous cysts associated with mandibular right and left first premolars. A marsupialization procedure may be a choice of treatment for a large sized dentigerous cyst rather than an enucleation. The marsupialization procedure is recommended during the age when the erupting force of the teeth is still strong. We can expect the unerupted tooth to erupt normally. Although most of bilateral or multiple dentigerous cysts which are not associated with syndromes are rare, a bilateral dentigerous cyst without syndrome is seen. Therefore, it is wise to explain a possibility of development of new one to patient / parents in advance.

• 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.