• Journal of Periodontal and Implant Science
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• v.24 no.2
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• pp.255-260
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• 1994

We experienced a case of Sturge-Weber Syndrome in a 32 years old female. The diagnosis was established by clinical features of Sturge-Weber Syndrome including unilateral facial portwine nevus, vascular hyperplasia of oral mucosa(espectially 1st & 2nd division of trigeminal nerve). But, plain radiographys of the skull revealed no evidence of calcification. A brief review of related literatures was made. Common Clinical findings in Sturge-Weber Syndrome and specific signs & symptoms manifested by this patient were discussed.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.11 no.2
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• pp.67-73
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• 1989

Sturge - Weber Syndrome is a congenital disorder and characterized by facial hemangioma following one or more divisions of the trigeminal nerve, epilepsy, mental retardation, contralateral hemiplegia, occlular involvement, gingival involvement. A 34 year old Korean man, a 25 year old Korean woman and a 48 year old Korean woman were found to have red - purple colored pigmentation on the hemifacial area and upper oral mucosal area.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.909-912
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• 2003

Klippel-Trenaunay syndrome is a rare mesodermal phakomatosis characterized by cutaneous haemangiomata( usually unilateral and involving an extremity), venous varicosities and osseous and soft tissue hypertrophy, of the affected limb. Sturge-Weber syndrome, also a mesodermal pharkomatosis, is characterized by a port-wine nevus, which is present from birth and covers the face and cranium in the territory of the first division of the trigeminal nerve. Homolateral to the skin lesion, there is atrophy and calcification of the cerebral hemisphere. We experienced an unusual 26-months-old female, who had features of both Klippel-Trenaunay syndrome and Sturge-Weber syndrome. She had an extensive nevus flammeus which extended primarily over both sides of her face and the right side of the trunk and extremities, hypertrophy of the right extremity without evidence of arterovenous fistula, right glaucoma, choroidal hemangioma and leptomeningeal hemangioma, which combined Klippel-Trenaunay syndrome and Sturge-Weber syndrome. We reported this rare case with a brief review of some related literatures.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.145-149
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• 2009

Sturge-Weber Syndrome is a rare congenital disorder and is characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement(eg, glaucoma) and neurologic involvement(eg, epilepsy, mental retardation). Oral menifestations include unilateral blood vessel expansion of the oral mucosa and gingiva, gingival hyperplasia, giant tooth, ipsilateral large tongue, blood vessel anomaly of maxilla or mandible and abnormal eruption sequence. This case report is about 8-year-old Sturge-Weber Syndrome patient presented violet discoloration on upper gingiva and buccal mucosa, gingival hyperplasia and abnormal eruption sequence. In this case, we performed lingual frenectomy and periodic oral hygiene management, and obtained satisfactory result.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.15-18
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• 2010

Sturge-Weber syndrome is a rare nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation involving the tissue of brain and face. The clinical features are characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement and neurologic involvement such as epilepsy, mental retardation, and contralateral hemiplegia. Oral manifestations include unilateral blood vessel expansion of the oral mucosa, vascular hyperplasia of gingiva, pyogenic granuloma-like massive hemangiomatous proliferation of oral mucosa, macrodontia, ipsilateral macroglossia, blood vessel anomaly of maxilla or mandible and abnormal tooth eruption sequence. This case report is about 11-year-old Sturge-Weber syndrome patient presented port wine nevus on the face, venous malformation on soft plate and buccal mucosa. In this case we performed simple extraction of several deciduous teeth and periodic oral hygiene management. If a patient with Sturge-Weber syndrome has to undergo dental surgery in affected areas of the mouth, great care must be taken to prevent severe hemorrhage.

• The Korean Journal of Nuclear Medicine
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• v.30 no.1
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• pp.56-64
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• 1996

The purpose of this study was to evaluate the characteristic perfusion changes in patients with Sturge-Weber syndrome by comparison of the findings of brain MR images and perfusion SPECT images. $^{99m}Tc$-HMPAO or $^{99m}Tc$-ECD interictal brain SPECTs were performed on 5 pediatric patients with Sturge-Weber syndrome within 2 weeks after MR imaging. Brain SPECTs of three patients without calcification showed diminished perfusion in the affected area on MR image. A 3 month-old patient without brain atrophy or calcification demonstrated paradoxical hyperperfusion in the affected hemisphere, and follow-up perfusion SPECT revealed decreased perfusion in the same area. The other patient with advanced calcified lesion and atrophy on MR image showed diffusely decreased perfusion in the affected hemisphere, but a focal area of increased perfusion was also noted in the ipsilateral temporal lobe on SPECT. In conclusion, brain perfusion of the affected area of Sturge-Weber syndrome patients was usually diminished, but early or advanced patients may show paradoxical diffuse or focal hyperperfusion in the affected hemisphere. Further studies are needed for better understanding of these perfusion changes and pathophysiology of Sturge-Weber syndrome.

• Journal of Dental Anesthesia and Pain Medicine
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• v.15 no.3
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• pp.173-179
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• 2015

Issues related to the control of seizures and bleeding, as well as behavioral management due to mental retardation, render dental treatment less accessible or impossible for patients with Sturge-Weber syndrome (SWS). A 41-year-old man with SWS visited a dental clinic for rehabilitation of missing dentition. A bilateral port-wine facial nevus and intraoral hemangiomatous swollen lesion of the left maxillary and mandibular gingivae, mucosa, and lips were noted. The patient exhibited extreme anxiety immediately after injection of a local anesthetic and required various dental treatments to be performed over multiple visits. Therefore, full-mouth rehabilitation over two visits with general anesthesia and two visits with target-controlled intravenous infusion of a sedative anesthesia were planned. Despite concerns regarding seizure control, bleeding control, and airway management, no specific complications occurred during the treatments, and the patient was satisfied with the results.

• The Journal of the Korean dental association
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• v.27 no.6 s.241
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• pp.539-543
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• 1989

• Journal of Korean Neurosurgical Society
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• v.67 no.3
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• pp.265-269
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• 2024

Pediatric central nervous system (CNS) vascular malformations are a group of abnormal blood vessel formations within the brain or spinal cord in children. The most crucial point of pediatric CNS vascular malformation is that no golden standard classifications exist. In addition, there is a big gap in knowledge and the viewpoint of clinicians, radiologists, and pathologists. In addition, many genes associated with pediatric CNS vascular malformation, such as Sturge-Weber-Dimitri syndrome with guanine nucleotide-binding protein G(q) subunit alpha (GNAQ) gene mutation, and cavernous malformations with cerebral cavernous malformations 1 (CCM1), CCM2, and CCM3 gene mutation, were recently revealed. For proper therapeutic approaches, we must understand the lesions' characterizations in anatomical, morphological, and functional views. In this review, the author would like to provide basic pediatric CNS vascular malformation concepts with understandable diagrams. Thus, the author hopes that it might be helpful for the proper diagnosis and treatment of CNS pediatric vascular malformations.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.766-771
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• 1988

Von Recklinghausen`s neurofibromatosis, tuberous sclerosis and encephalotrigeminal angiomatosis[Sturge-Kalischer-Weber syndrome] are frequently classified under the heading of organic neurocutaneous syndromes. Both neurofibromatosis and tuberous sclerosis are believed to represent instances of simple autosomal dominant heredity. Multiple neurofibroma and cafe*-au-lait spots are the hallmarks of the van-Recklinghausen`s disease. The characteristic features of the fully developed syndrome are [1] pigmentation of the skin, including cafe*-au-lait spots, pigmented freckles and males, and occasionally a generalized darkening of the skin; [2] subcutaneous nodules and deep neurofibromatous tumors and diffuse plexiform growths of neural tissue; [3] skeletal anomalies, especially scoliosis; and [4] predilection to malignancy. In recent years cystic lung disease, usually of the so-called honeycomb lung variety, has been reported on several occasions in patients with tuberous sclerosis. This association has been shown to our sporadically as well as in members of a single family. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochromocytoma, meningocele or, less commonly, parenchymal pulmonary neurofibromatosis. Author have experienced a case of von Recklinghausen`s disease. This case developed a huge neurofibroma in the both side thorax and invaded to the Lt. 7th rib.