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Bolan Michele - - 2005
A girl (aged 8.5 years) with Klippel-Trénaunay-Weber syndrome received orthodontic treatment that included rapid palatal expansion. The patient had early and complete intraoral dental eruption with a posterior crossbite and an expressive transverse atresic maxilla. No carious lesions were found, but hemangioma-like changes of the mucosa were observed. A Haas ...
Demir Ozgün Ilhan - - 2005
Narrow duplicated internal auditory canal (IAC) is a rare malformation of the temporal bone that is associated with ipsilateral congenital sensorineural hearing loss. This may be an isolated finding or a part of a syndrome. Radiological examination should demonstrate aplasia or hypoplasia of the neural components of the narrow IAC, ...
Kossoff Eric H - - 2005
Sturge-Weber syndrome is associated with leptomeningeal angioma, trigeminal port-wine stain, epilepsy, and glaucoma. Clinically, many patients with Sturge-Weber syndrome are observed to have both seizures and headaches, but this has never been described in the literature. A questionnaire was mailed to 190 patients with reported comorbid epilepsy and headache as ...
Shannon Jack - - 2005
An 83-year-old woman was admitted for investigation of sudden loss of vision. Extensive plum-purple papules and nodules involved her skin and lips but not mucosae. A cutaneous biopsy demonstrated irregular vascular cavernous channels in the dermis and subcutis; deeper vessels displayed smooth muscle. Blue rubber bleb naevus syndrome was diagnosed. ...
Auluck A - - 2005
Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by triad of vascular nevi, venous varicosities and hyperplasia of soft and hard tissues in the affected area. This syndrome usually affects the extremities but occasionally can manifest in the craniofacial region, including the oral cavity. We report a case of KTS ...
Vélez D - - 2005
BACKGROUND: The term 'glomeruloid haemangioma' was coined by Chan et al. for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in patients without this syndrome. METHODS: We report ...
Comi Anne M - - 2005
Sturge-Weber syndrome presents with vascular malformations of the brain, skin, and eye. Fibronectin has potent effects on angiogenesis, vessel remodeling, and vessel innervation density. To determine fibronectin expression in the blood vessels of Sturge-Weber syndrome brain and skin tissue and to quantify the density and circumference of Sturge-Weber syndrome blood ...
Suprabha B S - - 2005
Sturge-Weber syndrome is an uncommon condition characterized by presence of Portwine stains on the face along with ocular disorders, mental retardation, oral involvement and leptomeningeal angiomas. A report of a case with atypical manifestations of this syndrome along with a step-by-step protocol oral rehabilitation of such patients is described.
Kilickesmez Ozgur - - 2005
Pleomorphic xanthoastrocytoma is a rare, cortically based, partially cystic astroglial tumour with a strongly enhancing solid mural nodule. We report an 11-year-old boy with a pleomorphic xanthoastrocytoma in the contralateral hemisphere to that affected by Sturge-Weber syndrome. This unique case supports the association between Sturge-Weber syndrome and astrocytomas. Patients with ...
Yang Jeong In - - 2005
BACKGROUND: Klippel-Trenaunay-Weber syndrome is a rare congenital soft tissue anomaly with sporadic occurrence characterized by the triad of multiple hemangiomas, arteriovenous fistulas and unilateral limb hypertrophy. CASE: A 27-year-old, pregnant woman was referred at 31 2/7 gestational weeks. On prenatal sonography, multiloculated cystic areas involving the left side of the ...
Perez D E C - - 2005
Sturge-Weber syndrome is a congenital disorder characterized by vascular facial birthmarks and neurological abnormalities. Oral cavity involvement may occur, and the extent of the vascular abnormality may vary considerably. The present authors report the case of a 6-year-old girl with Sturge-Weber syndrome, focusing on the clinical and radiographic features. Her ...
Singh Arun D - - 2005
Choroidal hemangioma is an uncommon benign vascular tumor of the choroid that can be circumscribed or diffuse. Circumscribed choroidal hemangiomas are usually diagnosed between the second to fourth decade of life when they cause visual disturbances owing to the development of an exudative retinal detachment. Circumscribed tumors occur sporadically, without ...
Padilla W - - 2005
We describe the first case of Weber's Syndrome to present as a manifestation of decompression illness in a recreational scuba diver. Weber's Syndrome is characterized by the presence of an oculomotor nerve palsy and contralateral hemiparesis. The patient was a 55 year-old male with a past medical history of a ...
Huiskamp E A - - 2005
PURPOSE: To report the treatment outcome of photodynamic therapy with verteporfin (PDT) for exudative retinal detachment associated with diffuse choroidal haemangioma in Sturge-Weber syndrome. METHODS: An interventional case report of a 12-year-old girl with Sturge-Weber syndrome who developed an exudative retinal detachment (visual acuity 20/400) that was treated with PDT ...
Taddeucci Grazia - - 2005
The Sturge-Weber syndrome was recently subdivided into type I (facial and leptomeningeal angioma, possible glaucoma), type II (facial angioma, without evident endocranial involvement), and type III (exclusive leptomeningeal angioma). Thus far in the literature only 24 cases of Sturge-Weber syndrome type III have been reported. This study presents a case ...
Nyberg R H - - 2005
A new case of macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) syndrome is described. The patient presented typical congenital findings in utero, although the syndrome was diagnosed postnatally. The M-CMTC syndrome should be considered when there is a marked fetal overgrowth and progressive macrocephaly with no indications of maternal hyperglycemia or fetal ...
Di Iorio G - - 2005
Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation of unknown etiology characterized by cutaneous hemangiomas, venous varicosities and bony and soft tissues hypertrophy usually affecting one limb. Several complex anomalies involving various organs and systems have been described, whereas involvement of the peripheral nervous system has rarely been reported in ...
Ahmed Irfan - - 2005
We present our experience of two cases with genitourinary manifestations of Klippel-Trenaunay syndrome. A MEDLINE search for the period 2001-2004 was done using the keywords "Klippel-Trenaunay syndrome", "vascular malformation" and "genitourinary". Only three cases with genitourinary manifestations of this syndrome were reported during this period. Genitourinary problems are rare in ...
Bhalala Utpal S - - 2004
Rendu Osler Weber syndrome is a rare vascular disorder and presents with diagnostic problems because of varied and sometimes misleading clinical presentations and radiological findings. A case report of Rendu Osler Weber syndrome in which the diagnosis was suggested in view of presence of pulmonary Arterio-Venous malformations is described. The ...
Al Robaee Ahmad - - 2004
We describe a 23-month-old child with phakomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome. Phakomatosis pigmentovascularis type IIb is a rare cutaneous malformation characterized by the simultaneous occurrence of nevus flammeus and melanocytic nevi. A brief review of the literature is presented and classification of this rare disease is discussed.
Miyama Sahoko - - 2004
We describe a 7-month-old female with leptomeningeal angiomatosis who developed infantile spasms. She did not manifest facial nevus or ocular choroidal angioma. Leptomeningeal angiomatosis is characterized by venous angiomas of leptomeninges and usually accompanied by facial nevus, a condition known as Sturge-Weber syndrome. In Sturge-Weber syndrome, leptomeningeal angiomas can cause ...
Lagravère Manuel O - - 2004
Short neck and fusion of cervical vertebrae are observed in several genetic conditions and well-defined syndromes. An 8-year-old boy with a short neck, low-set posterior hairline, deafness and limited neck motion was suspected of having such a condition. Clinical and radiographic examination led to the diagnosis of Klippel-Feil syndrome.
Kumar Sarita - - 2004
Phakomatoses or neurocutaneous syndromes are an important cause of seizures in the pediatric age group. The Sturge-Weber syndrome may affect the eye, skin and brain at different times. The skin lesions need not always manifest. We report a case of isolated affection of the central nervous system in a case ...
Dorothy A - - 2004
A variant of hemangioma and an uncommon congenital condition, the Sturge Weber syndrome (SWS), also called encephalo trigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the skin of the face (cutaneous angioma) and pia arachnoid (leptomeningeal angioma). It occurs typically in the ophthalmic and maxillary distributions of the trigeminal ...
Kumar V - - 2004
Sturge - Weber syndrome (SWS) is a rare disorder that occur with a frequency of approximately 1 per 50,0001. It isa neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. The hallmark is intracranial vascular angioma, most often involving the occipital and posterior parietal lobes, but it can ...
Finley Thomas A - - 2004
Progressive hemifacial atrophy in Parry-Romberg syndrome is associated with a wide variety of ophthalmologic findings. Progressive visual loss, especially in the first decade, is rare in this condition. We present a young girl originally felt to have linear scleroderma and anisometropic amblyopia, who was eventually diagnosed with Parry-Romberg syndrome as ...
Muñiz Antonio E - - 2004
Young infants may present to an emergency department after an acute life-threatening event. The differential diagnosis for an acute life-threatening event is quite extensive. Fortunately, most will have benign or treatable causes. A case of Sturge-Weber syndrome is described in a 2-day old who presented to the emergency department after ...
Kawada Jun-Ichi - - 2004
We report a case of hemiconvulsion-hemiplegia (HH) syndrome. An 18-month-old female infant had a hemiconvulsion followed by left hemiplegia. Magnetic resonance imaging immediately after the onset of hemiplegia showed high intensity in the right hemisphere in diffusion-weighted images (DWI), while T1- and T2-weighted images were normal. Single photon emission computed ...
Chen Changzheng - - 2004
PURPOSE:To report indocyanine green angiography (ICGA) findings in one patient of diffuse choroidal hemangioma associated with Struge-Weber syndrome. METHODS: Color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with Sturge-Weber syndrome. RESULTS: Three findings were unveiled by ICGA: rapid filling of ...
Suver Daniel W - - 2004
Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of (1) capillary malformations, (2) venous malformations, and (3) soft tissue or bony hypertrophy. There exists one report of a patient with KTS and an associated sensorineural hearing loss. We describe an adolescent girl with KTS and AV fistulas ...
Tracy M R - - 2004
Klippel-Feil syndrome occurs in a heterogeneous group of patients unified only by the presence of a congenital defect in the formation or segmentation of the cervical spine. Numerous associated abnormalities of other organ systems may be present. This heterogeneity requires comprehensive evaluation of all patients and treatment regimes that can ...
Baselga Eulalia - - 2004
Sturge-Weber syndrome is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal angioma. Although the precise pathogenesis is unknown, available data regarding genetics, embryogenesis, and pathologic features are briefly reviewed. Clinical features vary from mild incomplete forms to full-blown disease with facial ...
Paksoy Yahya - - 2004
STUDY DESIGN: In the present report, radiologic findings of the patient with the Klippel-Feil syndrome associated with persistent trigeminal artery are reviewed. In same case, there was also spina bifida occulta, cervical spondylosis, atlanto-occipital assimilation, bilateral cervical ribs, and lack of flow signal in the C4 segment of contralateral vertebral ...
Thomas-Sohl Kristin A - - 2004
Sturge-Weber syndrome is a rare disorder that occurs with a frequency of approximately 1 per 50,000. The disease is characterized by an intracranial vascular anomaly, leptomeningeal angiomatosis, most often involving the occipital and posterior parietal lobes. Facial cutaneous vascular malformations, seizures, and glaucoma are among the most common symptoms and ...
Konstantinou Dimitris T - - 2004
STUDY DESIGN: This report aims, in the light of the recent literature, to describe the clinical features of bilateral thoracic outlet syndrome in a case of Klippel-Feil syndrome, the results of electrophysiologic evaluation, and the outcome after surgical intervention. OBJECTIVES: Cervical ribs in the context of Klippel-Feil syndrome as the ...
Tubbs R Shane - - 2004
Mirror movements usually seen in the Klippel-Feil syndrome are most commonly appreciated in the upper extremities. Lower extremity involvement is seen rarely and when observed, is found in conjunction with upper extremity mirror movements. We report what we believe to be the first case of mirror movements found exclusively in ...
Scope Alon - - 2004
Parry-Romberg syndrome is a clinical entity consisting of progressive hemifacial atrophy appearing at a young age. Animal studies indicate that sympathectomy can produce hemifacial atrophy. To our knowledge, this is the first report of a patient with a possible association between Parry-Romberg syndrome and thoracoscopic sympathectomy.
Ziyeh S - - 2004
Klippel-Trenaunay and Parkes Weber (Klippel-Trenaunay-Weber) syndromes consist of vascular malformations of the capillary, venous and lymphatic systems combined with soft tissue and bone hypertrophy of the affected extremity. Klippel-Trenaunay syndrome is a pure low-flow condition, while Parkes Weber syndrome is characterized by significant arteriovenous fistulas. The distinction of both entities ...
Defraia Efisio - - 2004
Klippel-Trénaunay-Weber syndrome is characterized by cutaneous hemangioma, arteriovenous fistulas or varicosities (or both), and unilateral hypertrophy of hard and soft tissues with different localization. Klippel-Trénaunay-Weber syndrome represents a syndrome of interest for those in the fields of oral and maxillofacial radiology and pathology because of the high incidence of compromise ...
Hojo Nobumasa - - 2004
We report the case of a 73-year-old man with myelodysplastic syndrome (MDS) who developed Weber-Christian disease (WCD). Bone marrow aspirates showed refractory anemia with abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy 8, 9, and 14. The patient had intermittent fever associated with multiple tender erythematous ...
Vente Claudia - - 2004
A 42-year-old man had a large speckled lentiginous nevus on the left side of his trunk. The involved area was painful when touched and paresthetic. Moreover, the ipsilateral half of his body showed a pronounced hyperhidrosis. This case can be categorized as a typical example of speckled lentiginous nevus syndrome, ...
Mukhtar Iftikhar A - - 2004
The Kasabach-Merritt syndrome of consumptive coagulopathy associated with massive hemangiomas is a potentially life-threatening problem in patients with a pathologic fracture of the osseous lesion. This can result in massive bleeding even after minor trauma. In such patients, operative management of long bone hemangiomatous lesions, including percutaneous needle aspirations, may ...
Singh N N - - 2003
This case report describes a rare, non-epileptic manifestation of neurocysticercosis where a 22-year-old male presented with acute onset right 3rd nerve palsy with left hemiplegia (Weber syndrome). Computerized tomography and magnetic resonance imaging revealed cysticercus granuloma. The patient improved and became asymptomatic with steroid treatment. Recognizing this clinical entity would ...
Lewerenz J - - 2003
POEMS/Crow-Fukase syndrome is a rare multisystem disorder associated with elevated vascular endothelial growth factor (VEGF), which clinically presents with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B(12) deficiency, highly elevated VEGF and in ...
Poblete-Gutiérrez Pamela - - 2003
An 11-year-old boy revealed multiple telangiectases confined to the left cheek and the left side of the lips. Additionally, unilateral epistaxis was present. Radiological examinations of the brain, lungs and abdomen were normal and bleeding of the gastrointestinal tract was excluded. In this case, we propose a probable segmental type ...
Tan Hüseyin - - 2003
A case with pulmonary atresia/ventricular septal defect associated with port-wine stain and retinal vascular abnormality is reported. Clinical findings were similar to both PHACE syndrome and Sturge-Weber syndrome (SWS). But, the most frequent and well-known features of both syndromes were absent. So, it could not be concluded whether this is ...
Anand Rajiv - - 2003
PURPOSE: To report successful treatment of a diffuse choroidal hemangioma associated with chronic retinal detachment. DESIGN: Interventional case report. METHODS: A patient with bilateral Sturge Weber syndrome and associated retinal detachments failed external beam radiation. He was then treated with single session, multispot photodynamic therapy with verteporfin. There was prompt ...
Hopsu Erkki - - 2003
Beckwith-Wiedemann syndrome is a genetic fetal overgrowth disturbance characterized by organomegaly, abdominal wall defects, postnatal hypoglycemia, and increased frequency of embryonic and postnatal tumors. Hearing loss in connection with this syndrome is rare. We describe a patient with Beckwith-Wiedemann syndrome having a progressive conductive hearing loss caused by a stapedial ...
Feller L - - 2003
We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the Sturge-Weber syndrome, after Sturge and Weber who first described this affliction ...
Sella Enzo J EJ Foot and Ankle Service, and Department of Orthopaedics and Rehabilitation, Yale University, New Haven, CT, USA. - - 2003
A case of a patient with Klippel-Trenaunay-Weber syndrome (KTW) with Charcot osteoarthropathy is presented. A medline literature review was performed using the words Klippel-Trenaunay, orthopaedic, ankle, foot, and Charcot joint. Seven articles reported orthopaedic manifestations of KTW. They included limb hypertrophy and atrophy, limb-length discrepancies, digital anomalies, ulcerations, and spine ...
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