• Clinical and Experimental Pediatrics
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• v.55 no.1
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• pp.29-33
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• 2012

PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.

• Journal of Korean Neurosurgical Society
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• v.40 no.4
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• pp.273-276
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• 2006

Although cavernous angioma itself is not rare, the epidural spinal localization is uncommon and makes preoperative differential diagnosis difficult. An extraordinary case of a thoracic epidural cavernous angioma in very young age, causing sudden paraplegia is presented. Only 79 cases have been reported in the literatures and among them, this kid was the youngest. A 23-month-old boy was referred to us with a 2-day history of sudden both lower limb weakness. Two days before admission, he got up at morning and was unable to stand and even to move the legs. MRI revealed an epidural mass surrounding spinal cord associated with cord compression at the level of the C5 through T3. Through posterior approach with exposure of C6 to T3 level, the hematomatous mass was removed subtotally due to intraoperative bleeding and its ventral location. After the first operation, the weakness of bilateral lower extremities was improved so as to move gainst the gravity. But the next day, the limb weakness was aggravated as same as preoperative status due to mass effect of new hematoma. The second operation was performed to remove the hematoma and to control the bleeding focus. Several weeks later, the limb weakness was improved and he was able to walk. The literatures about spinal cavernous angioma are reviewed.

• Archives of Plastic Surgery
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• v.35 no.6
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• pp.735-738
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• 2008

Purpose: Intraosseous hemangiomas are rare and account for fewer than 1% of all bone tumors. The site that is most commonly involved are the vertebral column and the skull. Within the facial skeleton, hemangiomas can occur in the mandible, maxilla, the nasal bones, and rarely the zygoma. Methods: We report a case of an intraosseous hemangioma of the zygoma in a 49-year-old male. The patient had a slow growing hard mass in the left zygoma, which had been present for 8 years. Other than the cosmetic deformity, the patient experienced no pain and did not have any problem. He had no history of trauma in that area and no ocular symptoms. Preoperative computed tomography showed a trabeculated mass arising from the body of the left zygoma. The mass was surgically removed without having to reconstruct the bone defect by spairing the inner cortex. Results: Histopatholgical examination indicated a cavernous hemangioma. After 4 months of follow up, no functional and cosmetic impairment was identified. The patient was satisfied with the result. Conclusion: An intraosseous hemangioma of the zygoma can be treated with total surgical excision with preservation of the inner cortex, thus eliminating the need for reconstruction of bone defect.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.680-684
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• 2004

The hemangioma, a benign proliferation of blood vessel, is the most common tumor of infancy and childhood. In many instances, the lesion probably represents a hamartoma or malformation rather than a true neoplasm. In the oral cavity, common sites are lips, followed by tongue, buccal mucosa and palate. Clinical characteristics appear as a flat or raised reddish-blue lesion and are generally solitary. They are classified on the basis of their histological appearance into capillary, mixed, cavernous or a sclerosing variety. A 6-year-old male of this case was referred to the Department of Pediatric Dentistry, Chonnam National University with a chief complaint of swelling lesion on gingiva. The strawberry appearance mass was detected by clinical examination on attached gingiva at the upper left primary lateral incisor and canine. Surgical excision and biopsy were carried out for histological examination and the lesion was diagnosed with a capillary hemangioma. The risk of recurrence after this therapy is rare, and there is no malignant transformation. Despite their benign origins and behaviour, hemangiomas in the region of oral cavity are always clinically important to the dental profession because of bleeding tendency.

• The Korean Journal of Nuclear Medicine
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• v.13 no.1_2
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• pp.73-76
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• 1979

A case of hepatic hemangioma is presented in which blood pool imaging by $^{99m}Tc$-HSA provided a definite diagnostic clue. Cavernous hemangiomas are the most common benign tumor of the liver. But the majority of them are asymptomatic and are found incidentally at laparotomy or autopsy. Selective hepatic arteriogrophy has been the most helpful technique for the preoperative diagnosis. Recently radioisotope scan and ultrasonogram proved to be a new, definite and non-invasive diagnostic procedure.

• Journal of Korean Neurosurgical Society
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• v.65 no.3
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• pp.415-421
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• 2022

Objective : Seizure recurrence after the first-ever seizure in patients with a supratentorial cerebral cavernous malformation (CCM) is almost certain, so the diagnosis and treatment of epilepsy is justified. The optimal method of management of these patients is still a matter of debate. The aim of our study was to identify factors associated with postoperative seizure control and assess the surgical morbidity rate. Methods : We retrospectively analysed 45 consecutive patients with a supratentorial CCM and symptomatic epilepsy in a single centre. Pre- and postoperative epidemiological data, seizure-related patient histories, neuroimaging results, surgery details and outcomes were obtained from hospital medical records. Seizure outcomes were assessed at least 12 months after surgery. Results : Thirty-five patients (77.8%) were seizure free at the long-term follow-up (Engel class I); six (13,3%) had rare, nocturnal seizures (Engel class II); and four (8.9%) showed meaningful improvement (Engel class III). In 15 patients (33%) in the Engel I group; it was possible to discontinue antiepileptic medication. Although there was not statistical significance, our results suggest that patients can benefit from early surgery. No deaths occurred in our study, and mild postoperative neurologic deficits were observed in two patients (4%) at the long-term follow-up. Conclusion : Surgical resection of CCMs should be considered in all patients with a supratentorial malformation and epilepsy due to the favourable surgical results in terms of the epileptic seizure control rate and low postoperative morbidity risk, despite the use of different predictors for the seizure outcome.

• The Korean Journal of Nuclear Medicine
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• v.21 no.2
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• pp.191-197
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• 1987

39 patients with focal hepatic lesions were evaluated by $^{99m}Tc-RBC$ liver scan. The diagnosis of focal hepatic lesions were made by percutaneous needle biopsy, angiography, surgery, or clinical courses. Thses diagnoses included 24 cases of hemangioma, 7 hepatomas, 6 metastatic disease, 1 abscess, and 1 cyst. 19 hemangiomas showed focal hot activity on delayed static planar images. 3 small deep seated hemangiomas were diagnosed by SPECT that would have been missed by planar images alone. 2 large hemangiomas had no radioisotope uptake within the lesions on delayed images and at surgery cavernous hemangioma with thrombosis, calcification, and fibrosis were found. For hepatic hemangiomas in our series, the sensitivity was 91.7% and the specificity was 100%. The remaining 15 patients including hepatomas, metastatic lesions, cyst and abscess showed cold defect on delayed blood pool images. It is concluded that $^{99m}Tc-RBC$ liver scan should be the choice of primary diagnostic procecure for clinically suspected hepatic hemangioma since it's inexpensive, non-invasive, and readly available.

• Korean Journal of Head & Neck Oncology
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• v.31 no.1
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• pp.27-30
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• 2015

근육내 혈관종은 주로 몸통과 사지에 발생하며, 두경부 영역에서의 발생은 드문 것으로 알려졌다. 저자들은 교근에서 발생한 2예의 근육내 혈관종을 치험하였기에 문헌 고찰과 함께 보고하는 바이다. 임상의사들은 특별한 원인 없이 진행되는 이하선 부위의 종창을 주소로 내원하였을 때, 교근에서 발생한 근육내 혈관종의 가능성도 염두 해두어야 한다.

• BMB Reports
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• v.39 no.6
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• pp.677-685
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• 2006

Proteins that are unfolded or misfolded in the endoplasmic reticulum (ER) must be targeted for refolding or degradation to maintain the homeostasis of the ER. Derlin-1 was reportedly implicated in the retro-translocation of misfolded proteins from the ER to the cytosol for degradation. In this report, we showed that Derlin-1 was down-regulated in the endothelial cells derived from human hepatic cavernous hemangioma (CHEC) compared with other tested cells. Electron microscopy analysis showed that ER was aberrantly enlarged in CHEC cells, but not in other tested cells. When overexpressed, Derlin-1 induced the dilated ER to return normal size. This ER dynamic was associated with the activation of unfolded protein response (UPR). In CHEC cells where Derlin-1 was down-regulated, increased expression of the immunoglobulin heavy chain-binding protein (Bip) and UPR-specific splicing of X-box DNA-binding protein 1 (XBP1) mRNA were detected, as compared with that in other tested cells, indicating that UPR was activated. After Derlin-1 overexpression, the extent of UPR activation diminished, as evidenced by decreased expression of Bip, reduced amount of the spliced form of XBP1 ($XBP1_S$), and elevated expression of the unspliced form of XBP1 ($XBP1_U$). Taken together, these findings provide another example of a single protein being able to affect ER dynamic in mammalian cells, and an insight into the possible molecular mechanism(s).

• The Korean Journal of Nuclear Medicine
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• v.36 no.6
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• pp.344-356
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• 2002

Purpose: RBC blood pool SPECT has been used to diagnose focal liver lesion such as hemangioma owing to its high specificity. However, low spatial resolution is a major limitation of this modality. Recently, ordered subset expectation maximization (OSEM) has been introduced to obtain tomographic images for clinical application. We compared this new modified iterative reconstruction method, OSEM with conventional filtered back projection (FBP) in imaging of liver hemangioma. Materials and Methods: Sixty four projection data were acquired using dual head gamma camera in 28 lesions of 24 patients with cavernous hemangioma of liver and these raw data were transferred to LINUX based personal computer. After the replacement of header file as interfile, OSEM was performed under various conditions of subsets (1,2,4,8,16, and 32) and iteration numbers (1,2,4,8, and 16) to obtain the best setting for liver imaging. The best condition for imaging in our investigation was considered to be 4 iterations and 16 subsets. After then, all the images were processed by both FBP and OSEM. Three experts reviewed these images without any information. Results: According to blind review of 28 lesions, OSEM images revealed at least same or better image quality than those of FBP in nearly all cases. Although there showed no significant difference in detection of large lesions more than 3 cm, 5 lesions with 1.5 to 3 cm in diameter were detected by OSEM only. However, both techniques failed to depict 4 cases of small lesions less than 1.5 cm. Conclusion: OSEM revealed better contrast and define in depiction of liver hemangioma as well as higher sensitivity in detection of small lesions. Furthermore this reconstruction method dose not require high performance computer system or long reconstruction time, therefore OSEM is supposed to be good method that can be applied to RBC blood pool SPECT for the diagnosis of liver hemangioma.