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Tovar-Moll Fernanda - - 2007
Experimental studies in nonhuman primates have questioned the selectivity of pyramidal tract damage in giving rise to the classical pyramidal syndrome in humans, characterized by permanent spastic hemiplegia (PSH). According to this view, concomitant injury of extrapyramidal pathways is necessary for the development of both hemiplegia and spasticity. In this ...
Fernandes Teresa - - 2007
Klippel-Feil syndrome, or synostosis of the cervical spine, is the result of an abnormal division of somites during embryonic development. This report analyses an adult male (exhumed from a Portuguese graveyard dating from the 13th to the 15th century) with malformations in the cranium and vertebral column. Besides the lesions ...
Zolkipli Z - - 2007
Sturge-Weber syndrome (SWS) is a sporadic disorder characterized by naevus (port wine stain), a pial angioma, and glaucoma. The angioma comprises abnormal tortuous vessels on the leptomeninges with underlying brain gliosis, calcification, and atrophy. The cerebral angioma is commonly unilateral but may be bilateral. Hemiplegia usually follows recurrent hemiconvulsions and ...
Chen Chih-Ping - - 2007
We describe the prenatal sonographic findings in a case of Klippel-Trénaunay-Weber syndrome including fetal ascites and subcutaneous cystic lesions associated with a relatively low level of maternal serum alpha-fetoprotein and a relatively high level of maternal serum beta-human chorionic gonadotrophin. Klippel-Trénaunay-Weber syndrome may present prenatally with fetal ascites and an ...
Moschos Marilita M - - 2007
PURPOSE: To report a case of a tilted disc syndrome associated with choroidal neovascularization. METHODS: A 55-year-old male patient presented with blurred vision and metamorphopsia of the left eye. He underwent complete ophthalmologic examination, fluorescein angiography, optical coherence tomography (OCT) and multifocal-electroretinogram (mf-ERG). RESULTS: All features are consistent with a ...
Ergün R - - 2007
Sturge-Weber syndrome is one of the neurocutaneous syndromes. It is a rare, nonfamiliar disease that is characterized by facial port-wine stain, leptomeningeal angiomatosis, choroidal angioma, buphthalmos, intracranial calcification, cerebral atrophy, mental retardation, glaucoma, seizures and hemiparesis. CT and MR are complementary in the evaluation of this disease. Epilepsy is an ...
Yi S - - 2007
This is the first published report of a patient with Klippel-Feil syndrome treated with cervical arthroplasty. A 36-year-old man presented with posterior neck pain and myelopathic symptoms. A radiograph demonstrated congenital fusion of the vertebral bodies at C2-3, C4-5 and C5-6. On MRI, the spinal cord was compressed by a ...
Brunetti-Pierri Nicola - - 2007
Parkes Weber syndrome is a disorder characterized by cutaneous blush, arteriovenous fistula, and overgrowth of the affected limb. It has been differentiated from Klippel-Trenaunay syndrome on the basis of the presence of arteriovenous fistula that are always absent in the latter. We report a case of Parkes Weber syndrome with ...
Elgin Ufuk - - 2007
A two-stage antiglaucoma operation was planned for the left eye of an 11-year-old boy with bilateral Sturge-Weber syndrome to avoid intraoperative and postoperative choroidal and retinal effusion as with previous trabeculectomy. The Ex-Press miniature glaucoma implant was placed 10 days before trabeculectomy with mitomycin C. There were no complications with ...
Terada Naoki - - 2007
Klippel-Trenaunay-Weber syndrome (KTS) is an unusual congenital anomaly characterized by cutaneous hemangiomas, varicosities and bony hypertrophy of the extremities. Herein the case is reported of a 24-year-old man with urethral bleeding from hemangiomas associated with KTS that were successfully managed by endoscopic sclerotherapy. A 23-G puncture needle was inserted into ...
Mofidi R - - 2007
This report describes the case of a 21-year-old man with a history of Sturge-Weber syndrome and varicose veins. Examination of the left lower limb revealed the presence of significant port-wine staining, and hypertrophy. Duplex examination revealed a severely hypoplastic deep venous system with a tortuous, dilated, long saphenous vein, which ...
Gupta Archana - - 2007
Sturge-Weber syndrome is a rare neurocutaneous disorder characterized by a facial nevus flammeus and extensive angiomatous changes involving the leptomeninges, the dura, and vessels of the gray and white matter. Oculodermal melanocytosis is characterized by hyperpigmentation of the facial skin in the distribution of the ophthalmic, maxillary, and occasionally mandibular ...
Perniola Tommaso - - 2007
The authors describe a girl with Costello syndrome who showed cerebral palsy and neurosensorial deafness. Brain computer tomography and magnetic resonance findings were normal. Multivoxel proton magnetic resonance spectroscopy showed a lowering of the peak of choline with a reduced choline/creatine ratio at the level of the centrum semiovale. These ...
Krings T - - 2007
The concept of segmental vascular syndromes with different, seemingly unrelated, diseases is based on the embryology of the neural crest and the mesoderm migration of cells that share the same metameric origin. Migrating patterns of these cells link the brain, the cranial bones, and the face on the same side. ...
Siddiqui Furqan H - - 2007
Klippel-Trénaunay-Weber syndrome (KTWS) is a rare congenital disease characterized by cutaneous hemangiomata, venous varicosities and osseous soft tissue hypertrophy of the affected limb. We report a case of a patient with KTWS who had developed severe chronic, non-healing cutaneous ulcers resulting from several angiography procedures with embolization by various agents. ...
Finamore Peter S - - 2007
BACKGROUND: Mirror syndrome is associated with both nonimmune and immune hydrops fetalis. The clinical manifestations are quite varied, and the pathophysiology is poorly understood. We describe a case of mirror syndrome associated with afetus that had a rapidly growing sacrococcygeal teratoma (SCT) without overt hydrops. CASE: At 30 weeks' gestational ...
Habek M - - 2007
Avellis syndrome is a rare form of alternating hemiparesis that is usually because of atherosclerosis. We report a 67-year-old man who developed paresthesiae of the left arm, dysphagia and dysphonia. The clinical picture, magnetic resonance imaging and cerebrospinal fluid findings were consistent with Avellis syndrome caused by brain stem arteritis ...
Danarti Retno - - 2007
BACKGROUND: Klippel-Trenaunay syndrome is defined by a coexistence of nevus flammeus and overgrowth of one or more limbs. Remarkably, however, deficient growth of an affected limb may likewise be noted. OBSERVATIONS: We collected from the literature a number of cases of Klippel-Trenaunay syndrome associated with deficient growth such as shortening ...
Comi Anne M - - 2007
Sturge-Weber syndrome (SWS) is defined by the association of a facial capillary malformation (port-wine stain), with a vascular malformation of the eye, and/or vascular malformation of the brain (leptomeningeal angioma). Variants exist where only one of these three structures is involved with the vascular malformation. SWS occurs sporadically and is ...
Horikoshi Toru - - 2007
A 48-year-old slender woman presented with a rare case of spontaneous intracranial hypotension syndrome manifesting as patulous Eustachian tube. The patient complained of sudden onset of ear fullness and nasal voice as well as typical orthostatic headache. Patulous Eustachian tube was identified by observation of synchronous movement of the tympanic ...
Judd C D - - 2007
BACKGROUND AND PURPOSE: Head and neck infantile hemangiomas are common lesions that are rarely observed in an intracranial location. We report 4 patients with orbital infantile hemangiomas and ipsilateral enhancing intracranial lesions, presumed to be infantile hemangiomas. METHODS: Imaging studies and medical records of 4 infants with orbital hemangiomas and ...
Pereda Marín R M - - 2007
Klippel-Trénaunay syndrome is a congenital malformation characterized by the triad of flat red hemangiomas, soft tissue and bony hypertrophy and varicose veins. In addition, venous malformations or hemangiomas may also be found in the lung, gastrointestinal tract, liver, kidney and bladder, which may result in recurrent hemor-rhage and compromise the ...
Paviour Dominic C - - 2006
Holmes tremor has a characteristic rest, intention, and postural component. The syndrome arises as a consequence of a lesion in the upper brainstem and cerebral peduncles, which, it is postulated, interrupts the cerebello-rubrothalamic pathway. Ataxia, ophthalmoplegia, and bradykinesia are associated features. We present a case of Holmes tremor secondary to ...
Dawczynski Jens - - 2006
Parry-Romberg syndrome is a rare pathologic process, characterized by progressive hemifacial atrophy. A case of Parry-Romberg syndrome with ocular involvement is reported. A 27-year-old male patient with Parry-Romberg syndrome was interdisciplinary investigated and treated. For reconstruction of hemifacial soft tissues a free vascular parascapular graft was performed. Opthalmological findings included ...
Makaryus Amgad N - - 2006
Eisenmenger's syndrome occurs when a large congenital or surgically created shunt between the left and right sides of the heart causes an increase in pulmonary vascular resistance that equals or surpasses systemic resistance, resulting in a reversal of the shunt from a left-to-right shunt to a right-to-left or bi-directional shunt. ...
Cohen M Michael MM - - 2006
This vascular review is organized under the following headings: vasculogenesis and angiogenesis; vascular endothelial growth factors, their receptors, TIE receptors, and angiopoietins; other factors in blood vessel formation; parallel patterning in blood vessels and nerves; physiological and pathological neovascularization; the role of VEGF receptors in metastasis; anti-angiogenic therapy for tumors; ...
Kihiczak George G - - 2006
Klippel-Trenaunay syndrome is characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and soft tissue. Appropriate evaluation and treatment of children displaying features of the disease may minimize morbidity. The clinical appearance, etiology, genetics, diagnostics, and treatment of Klippel-Trenaunay syndrome are herein explored.
Gruson Lisa M - - 2006
Phacomatosis pigmentokeratotica (PPK) represents a specific "twin nevus" syndrome in which a speckled lentiginous nevus (SLN) is associated with an organoid nevus with sebaceous differentiation. A boy with a large nevus sebaceus on the left face and upper part of the trunk, a giant segmental SLN extending from the abdomen ...
Nathan Nirmal - - 2006
Sturge-Weber syndrome (SWS), Klippel-Trenaunay syndrome (KTS), and Parkes-Weber syndrome (PWS) represent a wide range of congenital vascular abnormality syndromes. Although many of the diagnostic criteria overlap for these syndromes, there are important differences among these diseases that carry important prognostic and therapeutic implications. This review attempts to clearly distinguish among ...
Dalal Ashwin B - - 2006
OBJECTIVE: Hemihyperplasia is a heterogenous group of disorders characterized by asymmetric limb growth. There is considerable confusion regarding their classification and ascertainment into various syndromes. We tried to look into the various aspects of hemihyperplasia syndromes. METHODS: Records of 17 consecutive cases of hemihyperplasia were reviewed and were ascertained into ...
Krasnianski Michael - - 2006
In comparison with the lateral (Wallenberg), medial (Dejerine) and hemimedullary (Reinhold) medulla oblongata syndromes, the Babinski-Nageotte and Cestan-Chenais syndromes are much less familiar cerebrovascular disorders. While the Babinski-Nageotte syndrome is usually confused with the hemimedullary syndrome, reports of the extremely rare Cestan-Chenais syndrome are missing from the modern neurological literature. ...
Akagi Daisuke - - 2006
A 35-year-old woman with Klippel-Trénaunay syndrome developed a popliteal artery aneurysm in the affected extremity. The aneurysm was successfully treated by aneurysmectomy and bypass grafting with autologous saphenous vein. In Klippel-Trénaunay syndrome, angiodysplasia in the venous system is common. However, reports of an arterial aneurysm in a patient with Klippel-Trénaunay ...
Scop Jason - - 2006
A middle-aged woman was admitted to hospital after a motor vehicle accident. A large acute intracranial haematoma was initially thought to be traumatic in origin, but its unusual position and the lack of external features of head trauma prompted investigation for other causes of intracerebral haemorrhage. Moyamoya intracranial vascular abnormalities ...
Atzeni Fabiola - - 2006
Extracellular matrix mineralization or calcification occurs in many pathologic conditions, including atherosclerosis, medial wall calcification, diffuse idiopathic skeletal hyperostosis, and chondrocalcinosis. Vascular wall calcification is the most common and involves two mechanisms: passive calcification resulting from breakdown of the protection system and active calcification resulting from transdifferentiation of mesenchymal cells ...
Jindal Ravul - - 2006
A 22-year-old woman with a diagnosis of Klippel-Trenaunay syndrome presented with pain in the left hypochondrium and vomiting. Investigations revealed a huge splenomegaly with multiple hypodense lesions and irregular, linearly enhanced areas. She underwent splenectomy and had good postoperative recovery. Histopathology showed hemangioma/lymphangioma of the spleen.
Welty Linda D - - 2006
Sturge-Weber syndrome (SWS) is a rare, sporadic, progressive, congenital syndrome. In its complete trisymptomatic form, SWS is physically characterized by port-wine stains over the trigeminal area, leptomeningeal angiomas usually over the parieto-occipital region, and eye abnormalities. Clinical manifestation for infants with SWS depends on the affected organs, but can include ...
Yallapragada Anil V - - 2006
Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical ...
Madaan Vishal - - 2006
Sturge-Weber syndrome is a neurocutaneous syndrome that manifests with vascular malformations involving the brain, eye, and skin. We report the case of an elderly patient suffering from Sturge-Weber syndrome who presented with episodic "angry" slapping symptoms to a psychiatric facility. A detailed history, physical and mental-state examination, and elaborate neuropsychological, ...
Baldwin C J - - 2006
Klippel Trenauney syndrome is a rare congenital abnormality that includes extremity hypertrophy. We report an unusual case of breast asymmetry due to unilateral chest and limb hypertrophy and describe successful unilateral breast reduction. We highlight the potential problems of surgery within this group and a management approach to minimise complications.
Duman Levent - - 2006
Klippel-Trenaunay syndrome is a rare congenital disorder characterized by combined vascular malformations, varicosities, and limb enlargement. A child case of Klippel-Trenaunay syndrome with a large cervicomediastinal cystic lymphatic malformation and a vascular mass on the chest wall has been presented to demonstrate combined use of conservative and surgical approaches in ...
Rubenwolf Peter - - 2006
The Klippel-Trenaunay Syndrome (KTS) is defined as a triad of cutaneous capillary malformations, venous varicosities, bone and soft tissue hemi-hypertrophy. The urinary tract is involved in up to 10%. We report the clinical presentation and surgical management of a 9-year-old boy with extensive lympho-venous malformations of the bladder which led ...
Kumar Atul - - 2005
We document a previously unreported association of Vogt-Koyanagi-Harada (VKH) syndrome with intracranial meningioma. A female patient with diminished vision, exudative retinal detachment, and headache was diagnosed with VKH syndrome, more precisely a Harada form of disease with intracranial meningioma, on the basis of exudative retinal detachment, typical fundus fluorescein findings, ...
Lee Chang-Woo - - 2005
Sturge-Weber syndrome can be associated with facial port-wine stains and intracranial calcification, and concurrent Klippel-Trenaunay-Weber syndrome has been reported. Klippel-Trenaunay-Weber syndrome is a rare congenital mesodermal phakomatosis characterized by cutaneous hemangiomas, venous varicosities and soft tissue or bone hypertrophy of the affected extremities. This report is presented a rare case ...
Haktanir Alpay - - 2005
A patient with Klippel-Feil syndrome reported with a pneumatocyst of the proximal epiphysis of a right cervical rib. There was no additional skeletal abnormality except for fusion of the C3 and C4 vertebral bodies. To the best of our knowledge, this is the first reported instance of this particular association.
Verhelst Helene - - 2005
The patient presented here has extensive hemangiomata plana, especially on the right forehead, right upper eyelid, and right leg, as well as right leg hypertrophy and macrocephaly. Cerebral magnetic resonance imaging (MRI) showed abnormalities in only one occipital lobe consisting of focal cortical atrophy, leptomeningeal enhancement, and ipsilateral choroid plexus ...
Yuksel Murvet - - 2005
We present here the case of a 12-year-old boy who had Klippel-Feil syndrome with renal, cardiac and multiple skeletal anomalies, and we show the relevent three-dimensional computed tomography images. Our patient had a triple renal pelvis, mitral valve prolapsus, multiple cervical vertebrae fusions, cervical ribs, hypoplasia of the right thumb, ...
Sharma D - - 2005
A 9-year-old cyanosed child suffering from Osler-Weber-Rendu syndrome with bilateral pulmonary arteriovenous malformations (PAVMs) was posted for cerebral angiography under general anaesthesia. Careful preanaesthetic evaluation led to the diagnosis of coexisting congenital methaemoglobinaemia. There is no previous report of Osler-Weber-Rendu syndrome coexisting with congenital methaemoglobinaemia. This report emphasizes that a ...
Huiras Erin E - - 2005
Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, ...
Sehata H - - 2005
In two patients, one with Klippel-Feil syndrome and one with fibrous dysplasia of the maxilla, no part of the larynx could be visualized by direct laryngoscopy. In both cases, the use of an intubation device equipped with a charge-coupled device camera, the endotracheal intubation device (EID), allowed smooth tracheal intubation.
Lin Hung Sheng - - 2005
Cortical lesion might elicit restricted acral sensory deficit but a disparity of topographies in cheiro-oral-pedal syndrome is very rare. We report the first case of cheiro-oral-pedal syndrome due to a contralateral parasagittal hemangioma involving the supplement sensory area in parietal lobe. This unusual link between neuroanatomy and neurological feature is ...
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