Search Results
Results 301 - 350 of 556
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Aronoff D M - - 1998
The Klippel-Trénaunay-Weber (KTW) syndrome is a congenital disorder of angiogenesis characterized by macular nevus, skeletal and soft tissue hypertrophy, venous varicosities, and arteriovenous fistulas. Disseminated intravascular coagulation (DIC) and the Kasabach-Merritt syndrome, a consumptive coagulopathy with thrombocytopenia, are both associated with the KTW syndrome. We describe a 30-year-old woman with ...
McGaughran J M - - 1998
Klippel-Feil syndrome (KFS) is defined as a short neck with decreased movement and low posterior hairline. Radiologically, there is a failure of cervical segmentation. Deafness is a well known associated feature and may be of sensorineural, conductive, or mixed type. The audiological assessment of 44 patients with KFS is reported. ...
Ahluwalia T P - - 1998
A 15 Year old female patient with Sturge Weber Syndrome is presented. This neurocutaneous syndrome consists of angiomatosis of the skin and mucosa as well as the leptomeninges. This case report describes the classic presentation of the syndrome, emphasizing the oral manifestations. The radiographic and CT scan show the typical ...
Paladini D - - 1998
Klippel-Trenaunay-Weber syndrome is a rare congenital soft-tissue anomaly which is characterized by the presence of multiple hemangiomata, arteriovenous fistulas and limb hypertrophy. We report the ultrasound findings in two cases of early prenatal diagnosis at 17 and 18 weeks of gestation. The pathogenesis of the syndrome and involvement of the ...
Young S A - - 1998
A 44-year-old woman with Klippel-Trenaunay-Weber syndrome (KTWS) was evaluated for an adrenal mass incidentally discovered on CT. An NP-59 scintigraph showed normal symmetric adrenal tracer uptake, and a Tc-99m labeled RBC scan showed no evidence for an adrenal hemangioma but did show characteristic dilated and tortuous vasculature at multiple other ...
Lai N Y - - 1998
This case illustrates the need to recognize a high cervical cord injury as a cause for bi-brachial weakness with intact bi-crural power, a pattern termed aptly by Sage as 'man in the barrel' syndrome. The patient who had an undiagnosed Klippel-Feil deformity had incurred his cervical injury after a collapse.
Niimi Y - - 1998
Summary: We present a rare case of multiple spinal perimedullary arteriovenous fistulae associated with the Parkes-Weber (PW) syndrome. A 31-year-old male known to have the PW syndrome involving the left leg since birth, presented with a 7-month-history of progressive myelopathy of the lower extremities and dysfunction of the bladder and ...
Lorda-Sanchez I - - 1998
The Klippel-Trenaunay-Weber syndrome (KTWS) is generally thought to occur sporadically, following a somatic mutation model. However, in some cases, clinical manifestations of the syndrome have been found in family members, suggesting an autosomal dominant inheritance. Here we present an epidemiological analysis of a consecutive series of cases with KTWS identified ...
Vesoulis Z - - 1998
We studied the nonspecific nature of the histologic findings in the gastric antral vascular ectasia (GAVE) syndrome by using a morphometric comparison with common gastric lesions including hyperplastic polyps and gastritis. Five clinicopathologically confirmed cases of GAVE syndrome and 41 cases of gastric hyperplastic polyps were diagnosed during a 5-year ...
Evidente V G - - 1998
We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7-mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid-laden, foamy histiocytes. The cerebellar ...
Carcao M - - 1998
We describe a child with macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC), cherry red macules, megalencephaly with hemifacial and segmental overgrowth, macrosomia, and cutis marmorata telangiectasia congenita of the trunk, and visceral and subcutaneous cavernous hemangiomas. The megalencephaly is accompanied by MRI findings of CNS dysgenesis with protrusion of the cerebellar tonsils ...
Shih J C - - 1998
Klippel Trenaunay-Weber syndrome is a complex developmental disorder characterized by a triad of cutaneous haemangioma, varicosities of the body, and unilateral limb hypertrophy. We describe the prenatal diagnosis of Klippel-Trenaunay-Weber syndrome at 15 weeks' gestation using the surface rendering technique of three-dimensional ultrasound. The vivid three-dimensional images of the affected ...
Fischbein N J - - 1998
Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by a facial nevus flammeus associated with seizures, developmental delay, and, often, with hemiparesis and hemianopia. On MRI, the most characteristic finding has been reported to be leptomeningeal enhancement, believed to represent leakage of contrast medium through the anomalous pial vessels that ...
Srivastava D N - - 1998
Klippel-Trenaunay syndrome (KTS) is a congenital vascular abnormality consisting of a cutaneous naevus, varicose veins and bone and soft-tissue hypertrophy affecting one or more limbs. A case is presented here with some unusual associated findings seen on MR that, to the best of the authors' knowledge, has not been reported ...
Bataller R - - 1998
The case of a patient affected by Klippel-Trenaunay syndrome presenting with esophageal variceal bleeding caused by hypoplasia of the vena porta is reported. Hemostasis was achieved by performing a proximal spleno-renal shunt. We discuss the likely association of this mesodermal development abnormality and vascular disorders of the portal vein.
Kranemann C F - - 1998
PURPOSE: To evaluate Sturge-Weber-associated glaucoma using ultrasound biomicroscopy. METHOD: Case report. Clinical examination combined with ultrasound biomicroscopy was performed in a patient with Sturge-Weber-associated glaucoma. RESULTS: In the patient's left eye, which had Sturge-Weber-associated glaucoma, a 360-degree supraciliary effusion, dilated superficial and intrascleral vessels, and an open angle were detected ...
Vogels A - - 1998
We present the clinical history and findings in 4 children and adolescents with the association of macrocephaly and cutis marmorata telangiectatica congenita (Macrocephaly-CMTC syndrome). This syndrome has recently been delineated within the general group of patients with manifestations of cutis marmorata telangiectatica-Klippel-Trenaunay-Weber syndrome as a clinically recognisable entity.
Ivker R - - 1997
BACKGROUND: The association of multisystem pathologic conditions and epidermal nevi, known as the epidermal nevus syndrome, includes disorders of bone, central nervous system, eye, kidney, vasculature, body symmetry, and skin. Rarely, vitamin D-resistant rickets has been observed in association with this syndrome. Precocious puberty is another rare finding associated with ...
Sener R N - - 1997
An unusual case with Sturge-Weber syndrome is reported. A computed tomography study revealed the presence of gyriform calcifications in the occipital lobe, and discovered a hidden occipital subcutaneous port-wine nevus, instead of the usually expected nevus in the distribution of the first division of the trigeminal nerve. Existence of an ...
Meine J G - - 1997
The Klippel-Trenaunay-Weber syndrome is characterized by the triad of a port-wine stain, varicose veins, and bony and soft-tissue hypertrophy of an extremity. Recognition is usually possible during infancy or early childhood, and evaluation and treatment is important because morbidity may be minimized. We will review the clinical features, etiology, assessment, ...
Shields J A - - 1997
PURPOSE: Retinal capillary hemangioma, often associated with von Hippel-Lindau syndrome, is not characteristically seen in other conditions. We report a patient with Marshall-Stickler syndrome who had a retinal capillary hemangioma. METHOD: A 31-year-old man with Marshall-Stickler syndrome was evaluated for a retinal vascular tumor. RESULTS: Evaluation showed a retinal capillary ...
Thomsen M N - - 1997
STUDY DESIGN: This investigation was aimed at characterizing anomalies and syndromes associated with Klippel-Feil syndrome in a large group of patients. The authors evaluated the clinical and radiographic features, documented the associated anomalies, and registered the type of treatment. OBJECTIVE: The anomalies or syndromes and the development of scoliosis were ...
Suzuki K - - 1997
A 15-month-old female infant with nystagmus, gait disturbance, diminished reflexes, ophthalmoplegia, and facial paresis was diagnosed as having Fisher syndrome. Magnetic resonance imaging revealed a transient, high-signal-intensity lesion on the left side of the cerebellum on T2-weighted images. The ataxia of Fisher syndrome is not compatible with polyneuritis, but can ...
Apesteguía L - - 1997
We report a case of Klippel-Trenaunay syndrome which was suggested by microcalcifications detected on routine mammograms. Based on mammographic findings subcutaneous localization was suspected and a skin with subcutaneous cellular tissue biopsy was performed, confirming the microcalcifications at this level. The anatomopathologic report consisted of increase in vascularization in the ...
Mariën P - - 1996
A 73-year-old right-handed man with ischemic infarction in the vascular territory of the right arteria cerebellaris superior is described. In the acute phase he presented with cerebellar and brainstem symptoms, followed within a few days by a paresis of the right arm and unexpected language disturbances of aphasic origin. The ...
Griffiths P D - - 1996
In this article I review the typical clinical and neuroradiological features of Sturge-Weber syndrome and introduce some of the recently described findings demonstrated by magnetic resonance imaging and perfusion-based single photon emission computed tomography. The article is concluded by a discussion into the most efficient way of imaging children with ...
Lyle W G - - 1996
Acroangiodermatitis is an unusual cutaneous sequela of chronic venous insufficiency and congenital vascular malformations of the lower extremities. It typically manifests as purple-colored plaques on the extensor surfaces of the toes of the affected limb. Histologically, it closely resembles Kaposi's sarcoma and had thus been named "pseudo-Kaposi's sarcoma." This is ...
Fait G - - 1996
Klippel-Trénaunay-Weber syndrome is a rare congenital deep-vein malformation. Pregnancy in patients with this syndrome is rare and only a few cases have been reported. Known obstetrical risks in pregnant patients with this syndrome include bleeding from angiomata in the genitalia, and coagulation disturbances. We present a 31 year old woman ...
Pareja J A - - 1996
Four SUNCT patients with a clinical "SUNCT status" witnessed by the investigators are reported. Such an extreme aggregation of typical attacks lasting for the better part of the day, 1 to 3 days in a row has not previously been observed. Appropriate supplementary examinations ruled out an intracranial lesion in ...
Gates P E - - 1996
Klippel-Trenaunay syndrome is a triad of cutaneous hemangiomas, varicose veins, and hypertrophy of soft tissue and bone; when combined with arteriovenous fistulas, the syndrome is known as Klippel-Trenaunay-Weber syndrome. Orthopaedic surgical management of localized limb-length discrepancy or hypertrophy in these conditions is frequently indicated, especially in the lower limb. Forty ...
de Cock H - - 1996
A Border collie was presented at the age of 9 weeks with several lesions of the right forelimb, including a reddish-blue haemangiomatous macula in the medio-dorsal part of the elbow, multiple, scattered small cavernous haemangioma-like lesions at the plantar part of the foot and a general hypertrophy of the limb. ...
Singh D - - 1996
Two patients with Type I Klippel-Feil syndrome presented at the antenatal clinic. The first patient, who suffered from sleep apnoea, was delivered of a healthy infant by vacuum extraction. The second, who was profoundly deaf and had marked kyphoscoliosis, developed pregnancy-induced hypertension and urinary tract infection and was delivered at ...
Fodstad H - - 1996
The terms moyamoya disease, moyamoya syndrome and moyamoya phenomenon can be found dispersed throughout the literature. The diagnostic criteria for moyamoya disease are: (1) stenosis or occlusion of the anterior cerebral, middle cerebral and internal carotid arteries, (2) an abnormal vascular network near these arteries and (3) bilateral findings. When ...
Bhandari S - - 1996
A 72-year old man presented with clinical signs of cervical myelopathy , a short neck, low posterior hairline, and limited neck movements. He also had a solitary kidney and anomaly of the third rib. Biochemical hypoparathyroidism with low parathyroid hormone level was discovered and secondary causes excluded. Treatment with alfacalcidol, ...
Spallone A - - 1996
Klippel-Trenaunay syndrome is a rare neurocutaneous disorder in which skeletal hypertrophy, vascular nevi, and vessel anomalies coexist. Involvement of the cranial bone is rare, and intracranial anomalies associated with features of this syndrome are exceptional. We report a case of an adult Klippel-Trenaunay woman with a huge cranial hypertrophy harboring ...
Chen P C - - 1996
A full-term female neonate presented with facial port-wine stain, cutaneous telangiectasia, left facial hemihypertrophy, and left hemimegalencephaly at birth and subsequently developed hypertrophic change of left limb. She fit the diagnostic criteria of Klippel-Trenaunay-Weber syndrome. However, it was an unusual variant of this syndrome because the patient had left facial ...
Griffiths P D - - 1996
PURPOSE: To assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome. METHODS: Subjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal ...
Gass J D - - 1996
Sturge-Weber syndrome is a disorder characterized by ipsilateral cavernous hemangioma of the face, uvea, and brain in patients who may present with an enlarged eye, exudative retinal detachment, glaucoma, and seizures. This report presents the clinicopathologic findings of an otherwise healthy infant with ipsilateral arteriovenous and capillary hemangiomas of the ...
Whelan A J - - 1995
We report on a case of Klippel-Trenaunay Weber syndrome (KTWS) associated with a reciprocal translocation [46,XX,t (5;11) (q13.3;p15.1)]. The patient has developmental delay and minor anomalies in addition to classic findings of KTWS. These data support the notion that Klippel-Trenaunay-Weber syndrome may be due to a single gene defect and ...
Goyal M - - 1995
Lemierre's syndrome is an uncommon clinical entity characterized by oropharyngeal infection followed by septic thrombophlebitis of the jugular vein with embolization to the lungs and other organs. The organism is a gram-negative anaerobic bacterium, Fusobacterium necrophorum. We report a case of Lemierre's syndrome in an 8-year-old child who presented with ...
Bartels C - - 1995
Klippel-Trenaunay syndrome is characterized by the triad of unilateral port-wine hemangiomas, varicose veins, and hypertrophy of bone and soft tissue affecting one or more limbs. The rare F.P. Weber syndrome describes the mentioned entity and additional arteriovenous malformations. The association of an arterial aneurysm with the F.P. Weber syndrome has ...
Kim J S - - 1995
BACKGROUND: Classically, patients with unilateral lateral medullary infarction exhibit sensory abnormalities over the ipsilateral face and contralateral hemibody. As a variant, bilateral or contralateral facial sensory changes can be seen. However, sensory changes in the ipsilateral extremities are extremely rare. CASE DESCRIPTIONS: We describe three patients with lateral medullary syndrome ...
Patel P R - - 1995
Maurice Klippel (1858-1942) was a turn-of-the-century French internist with a special interest in neurology and psychiatry. He authored numerous medical papers in these disciplines, but is best known to spine surgeons for his description, co-authored with Andre Feil, of a patient with congenital fusion of the cervical vertebrae, a condition ...
Dolkart L A - - 1995
Sturge-Weber syndrome is a rare disease involving a port-wine facial nevus and an associated intracranial venous malformation. There are no reports of this disorder in association with pregnancy in the English literature, and the effects of pregnancy on this syndrome remain unknown. We present a case of Sturge-Weber syndrome diagnosed ...
Vargas L M - - 1995
Francisco Goya, the Spanish painter, suffered from a disease of uncertain etiology which affected his vision, hearing, and balance. The nature of his illness probably was a curious syndrome known today as Vogt-Koyanagi=Harada or uveomeningoencephalytic syndrome. This is an autoimmune disorder which involves the visual and cochlear pigment derived from ...
Akagi S - - 1995
STUDY DESIGN: This is a case report of a patient with Maffucci's syndrome, with hemangioma located in the cervical spine causing paraplegia. OBJECTIVE: To present a rare case of Maffucci's syndrome with hemangioma located in the cervical spine, and to discuss the incidence and treatment of hemangioma located in the ...
Neubert A G - - 1995
BACKGROUND: Klippel-Trenaunay-Weber syndrome is a sporadic genetic syndrome characterized by localized hemangiomas, venous varicosities, and asymmetric osseous hypertrophy of the ipsilateral extremities. Most commonly seen in association with hemangiomas, Kasabach-Merritt syndrome is defined by the presence of thrombocytopenia and a consumptive coagulopathy. CASE: A 22-year-old primigravida with a prior diagnosis ...
Reich D S - - 1995
Sturge-Weber syndrome is a rare congenital angiomatosis of unknown cause that is defined by the following triad: facial port-wine stain, leptomeningeal vascular anomalies, and choroidal vascular lesions associated with glaucoma. Klippel-Trenaunay-Weber syndrome is a related disease with questionable hereditary factors diagnosed by the following triad: superficial nevus of the lower ...
Chung M H - - 1995
Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman ...
Hallett K B - - 1995
A case of a young male with the Klippel-Trénaunay-Weber syndrome is described. Typical features of hemihypertrophy, hemangiomata, macrodactyly, and macrocephaly were present. The most striking oral feature was generalized severe gingival hypertrophy confirmed histologically, ultrastructurally, and by collagen analysis. In the absence of other known systemic causes of gingival enlargement, ...
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