• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.38 no.1
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• pp.29-37
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• 2012

Introduction: Fibrous-osseous lesions of the jaws are difficult to diagnose precisely until excised biopsy results are found, so they might be confused with malignant lesions. This clinical study focused on the diagnostic aids of lesions that demonstrate different clinical, radiologic, and histological findings. Materials and Methods: A total of 16 patients with benign fibrous-osseous lesions on the jaws (6 fibrous dysplasias, 6 ossifying fibromas, 3 cemental dysplasias, and one osteoblastoma) were reviewed. Nine patients with malignant fibrous-osseous lesions (8 osteosarcomas and one Ewing's sarcoma) were also retrospectively reviewed. Results: Osteosarcoma patients complained of facial swelling and tooth mobility. The radiographic findings showed the irregular resorption of cortical bone and periosteal reactions. Histological features included cellular pleomorphism and atypical mitosis. An Ewing's sarcoma patient complained of tooth mobility and facial swelling. Onion-skin appearance and irregular expansile marginal bony radiolucency were seen in the radiography. Fibrous dysplasia patients complained of facial swelling and asymmetry. The radiographic features were mostly ground-glass radiopacity. Histological findings showed a bony trabeculae pattern surrounded by fibrous ground substances. Ossifying fibroma patients complained of buccal swelling and jaw pains, showing expanded cortical radiolucent lesions with a radiopaque margin. Histological findings were revealed as cellular fibrous stroma with immature woven bones. In cemental dysplasia, most of their lesions were found in a routine dental exam. Well-circumscribed radiopaque lesions were observed in the radiography, and cementum-like ossicles with fibrous stroma were seen in the microscopy. An osteoblastoma patient complained of jaw pain and facial swelling. Radiographic findings were mottled, dense radiopacity with osteolytic margin. Trabeculae of the osteoid with a vascular network and numerous osteoblasts with woven bone were predominantly found in the microscopy. Conclusion: Our study showed similar results as other studies. We suggest the clinical parameters of diagnosis and treatment for malignant and benign fibrous-osseous lesions of the jaws.

• Journal of the korean academy of Pediatric Dentistry
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• v.37 no.2
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• pp.240-245
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• 2010

Neurofibromatosis type 1, an autosomal dominant disorder with prevalence of 1 per 3000 people, has clinical features of cafe au lait spots in skin, multiple neurofibroma and dysplasia in skeletal, endocrinal, and blood vessel systems. Actual intraoral neurofibromas are known to occur in 25% of patients. A 9 year-old girl diagnosed with type 1 neurofibromatosis visited our hospital with chief complaint of gingival swelling. Gingival enlargement in lower anterior region existed and cafe au lait spots were confirmed in patient' skin. Enlarged gingival tissue were excised under local anesthesia. Neurofibroma was confirmed with biopsy. Clinical examination after months showed fine recovery without any evidence of recurrence. Due to its possibility of recurrence, periodic follow-up will be needed.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.606-609
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• 2003

Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida occulta may be present, also ocular and dental abnormalities are reported. Radiologic findings are the osteopathy and striation of the long bone. We experienced a case of Goltz syndrome in a 9-year old female who was presented with right side hypotrophy, focal dermal hypoplasia, ocular(anidria, microcornea), dental(oligodontia, amelogenesis) and skeletal(syndactyly) abnormalities. Skin biopsy was performed and showed decreased expression of type I collagen gene with Northern blotting.

• Journal of Yeungnam Medical Science
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• v.33 no.1
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• pp.44-47
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• 2016

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.32 no.1
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• pp.19-26
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• 2006

Objective: In order to elucidate the retrogressive degeneration of orofacial cleft, the fissured tissues of prenatal and postnatal cleft lip and palate were examined by histological and immunohistochemical methods. Design: Totally 42 cases of prenatal (n=17) and postnatal (n=25) cleft lip and/or palate were examined in comparison with 10 cases of normal lip and oral mucosa using immunohistochemical stainings of MMP-3, MMP-9, MMP-10, cathepsin G, PCNA, E-cadherin, TGase 2, HSP-70, vWF, and VEGF. Main Outcome Measures: In the fissured tissue the sebaceous glands were strongly positive for PCNA and grew into the underlying fibromuscular tissue (24/42). Some hyperplastic sebaceous glands of prenatal cleft lip produced infundibular follicular cyst (9/17). The skin and mucosal epithelia from the postnatal cleft lip and palate (10/25) showed severe basal hyperplasia (11/25) and melanocyte infiltration (7/25). Results: The immunostaining of MMP-3 and HSP-70 were strongly positive in the hyperplastic sebaceous glands and nearby atrophying muscle bundles of the fissured tissue, while MMP-9, MMP-10, and cathepsin G were almost negative. The immunoreactions of the other antibodies used in this study were similar between in the fissured tissues and in the normal controls. Conclusions: These data suggest that the over-expression of MMP-3 is closely related to the sebaceous gland hyperplasia, epithelial dysplasia, and the muscle degeneration, and that the over-expression of MMP-3 in the fissured tissue may continuously aggravate the cleft condition in the later life.