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Results 451 - 500 of 556
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Ruiz-Maldonado R - - 1987
We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinically similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of ...
Guyuron B - - 1987
An unusual case of frontal bossing due to excess subcutaneous fibroadipose tissue in a 38-year-old woman with Möbius syndrome is presented. A relatively simple transcoronal resection of soft tissue resulted in successful elimination of this type of frontal bossing.
Barnes L M - - 1987
A 31-year-old man with familial dysplastic nevus syndrome presented with numerous histologically confirmed dysplastic nevi. Seven years before this, all of his nevi, numbering over 150, had been "prophylactically" removed. Despite the removal of all visible nevi, many new nevocellular nevi and atypical nevi appeared during the subsequent seven years. ...
Nielsen J R - - 1987
The Klippel-Trénaunay-Weber syndrome is a congenital angiodysplasia most often characterized by a triad of symptoms: varicose veins, port-wine cutaneous hemangiomas, and symmetrical hypertrophy of the affected limb. We report a case in a 37-year-old man and present a review of the literature.
Kanemoto N - - 1987
The case reported here showed a radiological appearance of hypoplasia of the right lung, dextroposition of the heart, and a curved vascular shadow in the right lower lung field known as a scimitar sign. However, a computed tomography of the chest showed this abnormal vascular shadow draining into the left ...
Clark R D - - 1987
We report on 11 new cases of Proteus syndrome to illustrate the broad range of the phenotype in this hamartomatous dysplasia. The cardinal manifestations of this sporadic disorder are hemihypertrophy, macrodactyly, exostoses, scoliosis, cavernous hemangiomas, lipomas, linear sebaceous nevi, and deeply rugated soles of the feet. Intelligence is usually normal. ...
Brown R E - - 1987
Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis ...
Nagib M G - - 1987
During the neurological work-up of a young patient with Klippel-Feil syndrome, the presence of a neuroschisis of the cervical spinal cord was detected. The patient presented with a transient and acute hemisensory loss and a Horner's syndrome of the opposite side. The unusual presentation and radiological findings in a patient ...
Steiner M - - 1987
Klippel-Trenaunay-Weber syndrome exhibits vascular anomalies including hemangiomas and varicose veins that commonly appear in the facial area. Characteristic findings involving the oral cavity include an enlarged maxilla, displacement of teeth, and malocclusions. Two cases are presented, with generalized and oral findings ranging from a mild to a very severe form ...
Pakter R L - - 1987
The Klippel-Trenaunay-Weber syndrome consists of cutaneous port wine hemangiomas, superficial venous varicosities, and soft tissue and bony hypertrophy of an extremity. We describe three patients with this syndrome with hypodense lesions in the spleen which in one case became isodense after bolus contrast CT. In one case ultrasound found numerous ...
Paller A S - - 1987
Cutaneous vascular abnormalities are a feature of many syndromes with multisystemic involvement. The most common associations are hypertrophy of underlying soft tissue and bone, as in the Klippel-Trenaunay-Weber and Sturge-Weber syndromes, visceral vascular lesions with hemorrhage, as in hereditary hemorrhagic telangiectasia and blue rubber bleb nevus syndrome, and neurologic alterations, ...
Sigler E - - 1986
A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity ...
Vine A K - - 1986
A patient with Hunter's Syndrome (systemic mucopolysaccharidosis type II) was evaluated for bilateral uveal effusion syndrome. The right eye showed a circular peripheral choroidal detachment for 360 degrees. The left eye showed a larger circular peripheral choroidal detachment and an exudative retinal detachment. Evaluation of the sclera during combined sclerectomies ...
Machen B C - - 1986
Intracranial convolutional or gyriform calcification simulating Sturge-Weber syndrome has been described in association with bacterial and viral intracranial infection as well as irradiation and intrathecal methotrexate for central nervous system leukemia. Reported is a case of gyriform calcification caused by chemical meningitis secondary to subarachnoid fat from an epidermoid tumor.
Schofield D - - 1986
We describe a 3 1/2-year-old boy with the Klippel-Trenaunay and Sturge-Weber syndromes. The child had congenital superficial capillary hemangiomas, congenital glaucoma and mild hydrocephalus. During the first year of life he experienced intermittent hematuria. When he was 3 years old he presented with seizures and left hemihypertrophy first was noted. ...
Sorensen R U - - 1986
A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of ...
Rosenbaum D M - - 1986
Atlantooccipital instability is an uncommon and usually lethal result of major trauma. Nontraumatic cases of instability at this site are rare. We report the findings in two children with Down syndrome who have striking atlantooccipital subluxation demonstrated on flexion and extension radiographs. Since there is increasing radiologic evaluation of cervical ...
Jaksch H - - 1986
The computed tomography, magnetic resonance imaging and angiographic findings are described in a patient with Klippel-Trenaunay syndrome, who also had a cerebral haemorrhage from an arteriovenous malformation. The resulting aphasia disappeared completely after resorption of the haemorrhage. In this syndrome, the occurrence of a cerebral angioma has not previously been ...
Naguib M - - 1986
Anaesthesia for the repair of a large occipito cervical encephalomyelocele in a neonate with Kippel-Feil syndrome is described. The fusion of the cervical spines, a short neck, low posterior hair line and Sprengel's deformity, which were present in this patient, collectively indicated Klippel-Feil syndrome. In addition to the usual stigmata ...
Schmitt B - - 1986
A child with Klippel-Trenaunay syndrome (KTS) and severe anemia caused by bleeding from diffuse intestinal hemangiomatosis is presented. Hemangiomas of the bowel should be considered in any child with unexplained anemia and coexisting cutaneous hemangiomas. The diagnostic workup of patients with KTS and symptomatic hemangiomatosis is outlined with respect to ...
Regenbogen L - - 1985
A 12-year-old boy with the cervico-oculo-acoustic syndrome, which comprises Duane retraction syndrome, Klippel-Feil anomaly (fused cervical vertebrae) and congenital hearing loss, is described. To this classical triad an optic nerve head coloboma was associated. To the authors' knowledge this is the first case in which such an association has been ...
Mohr G - - 1985
A 30-year-old obese patient with a rapidly progressing chiasmal syndrome presented with acute bitemporal hemianopia, severe bilateral amblyopia, mental confusion, and lethargy. X-ray films of the skull showed a normal sella turcica and computed tomography scan as well as angiography revealed a vascular mass within the chiasmatic cistern. At operation, ...
Ohtani I - - 1985
Klippel-Feil syndrome, the triad of short neck, low occipital hairline, and limitation of movement of the head, is often associated with other serious congenital anomalies of the nervous, urogenital, and cardiovascular, and respiratory systems. Hearing loss is the second most common associated anomaly following neurologic defects. Histologic studies of the ...
Enjolras O - - 1985
A retrospective study was made of 106 cases of facial port-wine stains. It was concluded that only patients with lesions located in the ophthalmic (or V1 trigeminal) cutaneous area are at risk for associated neuro-ocular symptoms. It is proposed that Sturge-Weber syndrome results from a dysmorphogenesis of cephalic neuroectoderm.
Mücke J - - 1985
In 1983 the Proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses ...
Nagib M G - - 1985
A retrospective analysis of 11 children with the diagnosis of Klippel-Feil syndrome treated at the University of Minnesota Hospital over a period of 20 years is presented. The salient features of the syndrome and its associated anomalies are reviewed. Emphasis is placed on its neurological aspects, particularly the potential risks ...
Weiner S N - - 1985
The Klippel-Trenaunay syndrome is composed of the triad of unilateral limb hypertrophy, abnormalities of the deep venous system, and port-wine hemangiomas. An interesting case is presented in which there were multiple abnormalities of the renal veins and intrarenal hemangiomas resulting in renal failure in addition to the usual peripheral abnormalities.
Benhaiem-Sigaux N - - 1985
An intraspinal vascular malformation associated with the Klippel-Trenaunay-Weber (KTW) syndrome initially was thought to be intramedullary on angiographic findings. Postmortem examination revealed an entirely posterior extramedullary arteriovenous fistula (AVF) fed by the anterior spinal artery. The association of the KTW syndrome with a so-called intramedullary AVF has been described in ...
Herbai G - - 1985
Integrated thermographic measurements were made in a patient suffering from a complex syndrome which included scleroderma, Osler-Weber-Rendu disease and a marked atherosclerotic circulatory insufficiency. A new anti-estrogenic drug, cyclofenil, elicited a rapid and prolonged curative effect against the entire syndrome. The circulatory amelioration was registered periodically by integrated thermography of ...
McCue C M - - 1984
Interest in Rendu-Osler-Weber (ROW) syndrome has been renewed because of new treatment for the pulmonary artery fistulae that occur in approximately one-half of the patients. Pulmonary arteriovenous malformations (AVM) can be occluded safely by the transvenous placement of a silicone balloon thus avoiding the many potential complications of thoracotomy. Thirty-three ...
Hoskins H D HD - - 1984
Goniotomy is a safe effective procedure in isolated trabeculodysgenesis occurring in children under the age of two years, and is preferred by the authors in all such cases when visibility allows. It may also be effective for varying periods of time in Sturge-Weber syndrome, Aniridia, Axenfeld's syndrome, and Lowe's syndrome ...
Epstein N E - - 1984
A 17-year-old white male patient sustained a cervical hyperextension injury while body surfing. Plain cervical radiographs, tomography, and CAT scan showed neither fracture nor subluxation, but congenital narrowing of the spinal canal and fusion of C2-C3 (Klippel-Feil). Clinically, he had a central cord syndrome, characterized by a motor dominant myelopathy. ...
Krebs H B - - 1984
Congenital dysplastic angiopathy (Klippel-Trenaunay-Weber Syndrome) consists of angiomata and congenital varicosities with trophic changes of soft tissues and various parts of the skeletal system. Involvement of female genitalia is rare and has been reported to occur in only four patients. The authors treated a patient with a lymphangioma of the ...
O'Brien J P - - 1984
Destruction (elastolysis) of the internal elastic lamina is frequently observed near early atherosclerotic plaques. Elastolysis and plaque formation are also found together in the temporal arteritis/polymyalgia rheumatica syndrome. Could it be that atherosclerosis and the syndrome are more closely akin than usually thought, with elastolysis acting as the pathogenetic link ...
Peh W C - - 1984
This is a case report of the unusual condition of Klippel-Feil Syndrome. Association with rectovestibular fistula has yet to be described. The rare association with bilateral sixth nerve palsy and renal agenesis is also present. The literature is reviewed with regards to the relevant anomalies as well as the etiology.
Cremers C W - - 1984
A 22-year-old woman with mixed hearing loss, Klippel-Feil anomalad (fused cervical vertebrae), and bilateral abducens palsy with retracted bulb (Duane's syndrome) is classified as having the cervico-oculo-acoustic syndrome or Wildervanck syndrome. In this syndrome, the unilateral or bilateral congenital hearing loss is usually severe and of neurosensory origin. To our ...
Ward J B - - 1983
A patient exhibiting ocular and systemic manifestations of both Sturge-Weber and Wyburn-Mason syndrome is described. The ocular, dermatologic, and neurological findings are detailed. The simultaneous occurrence of more than one phakomatosis is rare, but several combinations have been described in the literature. This appears to be the first report of ...
Whittle I R - - 1983
A young girl with Klippel-Feil syndrome presented with the onset of mirror movements in early childhood. Computerized tomography studies of her cervical spine and brain revealed fibrous diastematomyelia with duplication of the cervical spinal cord and an extra-axial midline posterior fossa cyst, together with the multiple cervical vertebral anomalies. Exploration ...
Warhit J M - - 1983
We were able to detect hemihypertrophy of a fetus in a routine prenatal ultrasound examination. The presence of hemangiomas and varicose veins in the hypertrophied left lower extremity at birth enabled us to diagnose the Klippel-Trenaunay-Weber syndrome. The possibility of a congenital arteriovenous fistula causing the constellation of findings was ...
Miyamoto R T - - 1983
The Klippel-Feil syndrome is characterized by a short, thick neck with restricted head mobility and a low occipital hairline. The head appears to rest directly on the thorax and the trapezius muscles flare out on either side of the neck, producing a pterygium-like effect. Cervical roentgenograms demonstrate fusion of some ...
Milner L S - - 1983
Hearing is impaired in 30% of patients with Klippel-Feil syndrome, owing to a variety of anatomical deformities of the inner and middle ear cavities. Recurrent meningitis due to a stapedial footplate fistula associated with this syndrome is rare. A case of Klippel-Feil syndrome, deafness and recurrent meningitis due to a ...
You C K - - 1983
The Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by a vascular nevus, varicose veins and bony and soft-tissue hypertrophy. Although the syndrome is uncommon, initially the condition appears as a skin blemish or varicose veins. To avoid errors in management, it is important to recognize the syndrome. In ...
Kostianen S - - 1983
Three cases of the antebrachial-palmar hammer syndrome are described in volley ball players. They all suffered from a decreased blood perfusion of their right hand and fingers due to the repeated ball and floor impacts during the volley ball training and playing. The vascular lesions of the radial and ulnar ...
Jampala V C - - 1983
The authors report on two fully right-handed men without prior psychiatric history who developed mania secondary to vascular lesions of the left and right hemispheres, respectively. One patient, whose left hemisphere lesion produced a transient posterior aphasia, is believed to be the first patient reported in the literature whose secondary ...
Jafri S Z - - 1983
Computed tomography (CT) and ultrasound were used to evaluate two patients with Klippel-Trenaunay (K-T) syndrome. Bowel and bladder hemangiomas were demonstrated in these cases. Computed tomography demonstrated multiple low attenuation areas in the liver and spleen in one case. Computed tomography is capable of noninvasively demonstrating bowel and bladder wall ...
Sakai M - - 1983
The Klippel-Feil syndrome is usually associated with sensorineural hearing impairment, but rarely is it associated with conductive or mixed deafness. A 22-year-old female presented with fusion of the cervical vertebrae, torticollis, scoliosis, pterygium colli, the Sprengel deformity with an omovertebral bone, concavity of the thorax and conductive hearing impairment of ...
Ambrosetto P - - 1983
2 cases of Sturge-Weber syndrome without facial nevus are reported. The patients presented different forms of epilepsy. The diagnosis was made by computed tomography (CT) which showed typical intracranial calcifications in both occipital regions. The problems concerning the atypical and incomplete forms of the syndrome are briefly discussed. The importance ...
Kessler H B - - 1983
A wide spectrum of osseous abnormalities has been reported in association with vascular lesions. Enchondromas, bone hypertrophy, bone lysis, and sclerotic bone lesions have all been reported in association with hemangiomas, lymphangiomas, varicosities, and arteriovenous fistulae. These associations can be grouped into four major syndromes: Maffucci syndrome, mixed sclerosing bone ...
Uram M - - 1982
The Sturge-Weber syndrome is characterized by the presence of a leptomeningeal hemangioma variably associated with a facial nevus flammeus, seizure disorder, mental retardation, hemiparesis, homonymous hemianopsia, glaucoma, and other signs and symptoms. The relationship between the cutaneous manifestations and the other features of this disorder was studied in 35 patients. ...
Niechajev I A - - 1982
Based on a clinical and radiological study of seven patients, a syndrome consisting of congenital hemangiomas with arteriovenous fistulae engaging the soft tissues and the bones, and associated with shortening of the involved extremity, for which we suggest the name angiomatosis osteohypotrophica, is reported. Angioma, varicose veins and/or arteriovenous fistulae ...
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