• 대한치과교정학회지
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• 제30권6호
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• pp.739-745
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• 2000

외배엽 이형성증(ectodermal dysplasia)는 외배엽에서 분화되는 조직의 일부에 선천적인 이형성이 나타나는 유전적 질환으로 모낭 및 땀샘의 결여로 인한 성긴 모발과 눈썹, 눈과 입주위 피부의 착색 및 각화와 치아형성의 이상 등이 특징적으로 나타나는 질환이다. 구강내 소견으로 무치증 혹은 부분 무치증을 보이며 부분 무치증의 경우 치관이 원추형으로 나타나 결과적으로 전반적인 치아간극 및 수직고경의 감소로 저작 및 발음기능의 저하 뿐 아니라 심미적이지 못한 안모로 사회적 및 정서적 우울감에 빠지기 쉬우므로 조기치료가 요구된다. 바람직한 보철물 제작 및 안모의 개선을 위해서는 교정과 및 타 전문과와의 협진이 요구되며, 본 증례에서는 ectopic dysplasia로 진단된 10세 남아를 교정의사와 보철의사의 긴밀한 협진으로 저작 및 발음기능의 회복과 안면고경의 증가로 심미성이 개선된 좋은 결과를 얻었기에 보고하고자 한다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제42권
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• pp.31.1-31.7
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• 2020

Background: Fibrous dysplasia (FD) is a rare, sporadic, and benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. FD localized in the cranial and facial bones is called craniofacial fibrous dysplasia (CFD). Cystic degeneration in CFD cases is rare; cystic degeneration appearing in both the maxilla and the mandible FD lesion is even rarer. The aim of this article was to report a case of fibrous dysplasia of the mandible and maxilla complicated by nonspecific cystic degeneration. Case presentation: A 30-year-old woman presented with a rare case of non-specific cystic degeneration in a mandible and maxilla FD lesion that occurred 11 years after surgery. She was diagnosed with polyostotic CFD and underwent maxillary and mandibular bone contouring. Cyst enucleation under general anesthesia was performed in the mandibular region due to pain and discomfort. Conclusions: In cases involving non-aggressive and non-invasive FD cystic degeneration in focal areas, conservative treatment is recommended. However, if cystic degeneration of FD develops rapidly and causes discomfort, pain, or dysfunction, surgical treatment should be considered.

• Journal of Korean Neurosurgical Society
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• 제30권11호
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• pp.1300-1307
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• 2001

Purposes : This study reports the possible causes of seizure recurrence in patients underwent previous epilepsy surgery, and surgical strategy for resection of the additional epileptogenic zone locating at the distant area to the site of first resection. Methods : A total of 10 patients with previous surgery due to intractable epilepsy were studied. Five of these underwent standard temporal lobectomy, four extratemporal resection, and one corticoamygdalectomy. Seizure outcome of these were class III-IV. Evaluation methods for reoperation included MRI, 3D-surface rendering of MRI, PET, prologned video-EEG recording with surface electrodes and subdural grid electrodes. Additional resection was done in the frontal lobe in two, in the temporal lobe in three, in the parietal lobe in two, and in the supplementary sensori-motor area in two. Tumor in the superior frontal gyrus in the left hemisphere was removed in one patient. Extent of resection was decided based on the results of ictal subdural grid EEGs and MRI findings. Awake anesthesia and electrocortical stimulation were performed in the two patients for defining the eloquent area. Results : Histopathologic findings revealed extratemporal cortical dysplasia in six, hippocampal sclerosis and cortical dysplasia of the temporal neocortex in one, neuronal gliosis in two, and meningioma in one. Previous pathology of the five patients with cortical dysplasia in the second operation was hippocampal sclerosis plus cortical dysplasia of the temporal neocortex. After reoperation, seizure outcomes were class I in six, class II in three, class III in one at the mean follow-up period of 17.5 months. Characteristically, patients in class II-III after reoperation showed histopathologic findings of hippocampal sclerosis plus temporal neocortical cortical dysplasia plus extratemporal cortical dysplasia. Conclusions : Seizure recurrence after epilepsy surgery was related with the presence of an additional epileptogenic zone distant to the site of first operation, and the majority of the histopathology of the surgical specimens was cortical dysplasia. In particular, hippocampal sclerosis plus temporal neocortical cortical dysplasia was highly related with seizure recurrence in patients with previous operation. In these patients, multimodal evaluation methods were necessary in defining the additional epileptogenic zone.

• 대한소아치과학회지
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• 제28권1호
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• pp.180-184
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• 2001

쇄골두개 이형성증(Cleidocranial Dysplasia)은 autosomal dominant skeletal dysplasia로 쇄골의 부재 두개골 봉합지연 및 치아이상의 특징을 갖는 질환이다. 치아이상 중에 유치열 발달은 정상인데 반해 영구치 맹출 실패가 가장 특징적인 소견이며 다수의 과잉치의 존재, 치아형태이상과 치근형태이상 등이 존재한다. 영구치의 지연 혹은 맹출 부전의 원인으로 (1) 다수의 과잉치 존재, (2) cellular cementum이 없는 기형적 치근형태, (3) 악골의 높은 골밀도, (4) 유치와 골의 비정상적 흡수를 들 수 있으며 저하된 골대사가주원인이며 두번째로 다수의 과잉치의 존재를 들 수 있다. 이의 치료방법은 더 많은 치아 맹출의 환경조성을 위한 교정치료, 외과적 처치 및 보철적 수복이 필요하다. 본 증례는 쇄골두개 이형성증으로 진단받은 7세 10개월 여 환아로 영구치 미맹출을 주소로 본원에 내원하였다. 임상구강 검사에서 유치의 만기잔존, 악궁의 협착, 전치부 반대교합과 다수의 치아우식증이 관찰되었으며 악골의 방사선사진에서 유치의 만기잔존 및 상하악에 다수의 과잉치가 관찰되었고 두부방사선사진에서 미폐쇄된 봉합과 봉간골(wormian bone)이 관찰되었으며 흥부방사선사진에서 쇄골의 부재가 보였다. 이 환아의 성장에 따른 치열 발달을 이해하고 시기에 따른 적절한 치과적 처치에 대해서 본 증례에서 보고하는 바이다.

• 대한임상검사과학회지
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• 제36권2호
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• pp.178-184
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• 2004

This research focuses on the overall evaluation of tumor protein (p53) and cell growth marker (Ki-67) in their functions as carcinogenic factors in both a HPV-infected group and in a HPV-noninfected group with the precancerous dysplasia of uterine cervix. Histological grades were determined by the H&E staining and the expression level of p53 and Ki-67 were tested by the immunohistochemistry method. The results were as follows. Among the total of 66 cases, p53 (+) was observed in 19 cases (29.0%) from the mild grade group, 22 cases (33.0%) from the moderate grade group, and 19 cases (29.0%) from the severe grade group. The values correlate with the increase of dysplasia intensity in HPV-noninfected group and showed significant correlation (p<0.05), but there were no significant difference from the HPV-infected group. Among a total of 66 cases, the mitotic index of Ki-67 (+) were observed in 19 cases (29.0%) from the mild grade group, 22 cases (33.0%) from the moderate grade group, and 19 cases (29.0%) from the severe grade group. The values were significantly different against dysplasia intensity (p<0.05), but showed no significant difference from HPV infection. After cross comparing the statistical parameters of p53 and ki-67 in their significance, p53 was shown to be statistically significant with Ki-67 while there was no statistically significant difference with Ki-67 (p<0.05). Taken together, tumor protein (p53) and an index of Ki-67 observed in cervical dysplasia and in HPV related dysplasia of cervix uterine did not have any notable significance with HPV infection. The incidence rate of p53, however, had some significant correlation with dysplasia while Ki-67 had no particular statistical significance. As a result, p53 and Ki-67 can be considered as effective diagnostic markers in predicting the disease progression of dysplasia to cervical cancer.

• Journal of Gastric Cancer
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• 제1권2호
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• pp.83-91
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• 2001

Purpose: This study was to observe whether the apoptotic function of tumor-infiltrating lymphocytes (TIL) is induced in human gastric epithelial dysplasia and gastric adenocarcinoma according to the role of FasL expression. Materials and Methods: A total of 56 gastric epithelial dysplasia and gastric adenocarcinoma patients were enrolled in this study: 9 cases of gastric epithelial dysplasia, 18 cases of early gastric carcinomas (EGC) and 29 cases of advanced gastric carcinomas (AGC). Immunohistochemical staining was performed for FasL and CD45, and the terminal deoxynucleotidyl transferase mediated dUTP nick end labelling (TUNEL) method was used to detect cell death in tumor-infiltrating lymphocytes. Results: 1) Positive reactions of FasL to neoplastic cells were $88.9\%$ (8/9) in gastric epithelial dysplasia, $83.3\%$ (15/18) in EGC, and $75.9\%$ (22/29) in AGC. 2) Expression of TIL was decreased in the FasL positive region and was increased in the FasL negative region, and significant expression of TIL was observed in the AGC group (P=0.001). 3) Expression of apoptotic TIL was very similar to the FasL expression, and $100\%$ expression was observed in gastric epithelial dysplasia group. 4) Expression of apoptotic TIL was increased in the FasL positive region and decreased in the FasL negative region, and significant apoptotic expression was observed in the gastric epithelial dysplasia and EGC groups (P=0.0420, P=0.0263, respectively). Conclusion: These results suggest that FasL is a prevalent mediator of immune privilege in epithelial dysplasia and cancer of the stomach.

• Imaging Science in Dentistry
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• 제30권2호
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• pp.149-154
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• 2000

Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제12권1호
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• pp.193-201
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• 1990

본 증례는 약 3~4년 전부터 우측 하악 골체부에 골성종창 및 간헐적 둔통이 있어 타병원에서 부분절제술을 시행받았으나 재발의 증세를 보여 내원한 60세 여자환자에 대해 병소부위를 포함하여 partial mandibulectomy와 동시에 metal plate와 iliac bone을 이용한 즉시 재건술을 시행하였으며 조직 병리학적 검사결과 florid osseous dysplasia로 진단 하였고, 술후 6개월이 경과한 현재까지 재발이나 별다른 합병증없이 양호한 치유경과를 보여주고 있다.

• 대한두경부종양학회지
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• 제20권1호
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• pp.29-32
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• 2004

Fibrous dysplasia of the mandible is an unusual manifestation of the disease that is usually benign, occurs in young individuals, and is managed by conservative curettage or debridement. We present a case of persistent fibrous dysplasia complicated by pain and abscess formation that was successfully managed by radical resection and reconstruction with a free fibular flap. Although mandibular fibrous dysplasia is preferentially managed conservatively, treatment of this disease has evolved to a point where total excision and immediate reconstruction may be the treatment of choice and offer the best outcome.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제35권5호
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• pp.337-341
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• 2013

Fibrous dysplasia is a chronic developmental disease of the skeleton involving formation of immature bone. Involvement of facial bones can result in deformation of facial contour. Prominent cheek area is often treated with malarplasty, which has a variety of modifications, depending on the surgeon's preference. The authors report on a case of polyostotic fibrous dysplasia in which the patient's right cheek had a prominent appearance. The prominence was corrected with malarplasty without internal fixation. The soft nature of bone involved in fibrous dysplasia could provide greater flexibility for modification of the traditional surgery.