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McGrory B J - - 1991
Klippel-Trenaunay syndrome is a rare congenital malformation characterized by a large angiomatous nevus; hypertrophy of soft tissue or overgrowth of bone, or both; and venous varicosities. The cases of 108 patients who had a diagnosis of this syndrome between 1956 and 1990 were reviewed. One hundred and twenty-six anomalies were ...
Eleopra R - - 1991
After commenting briefly on the literature on Fahr syndrome, we describe a patient with an unusual association of cerebral calcifications and motoneuron disease. We discuss the possible role of brain calcifications in the clinical picture and stress the importance of the routine use of CT scanning and MRI to show ...
Sato M - - 1991
A case of mucosal prolapse syndrome in the rectum is reported. A 61-year-old female presented complaining of bloody discharge and constipation for 2 months. Colonoscopy revealed an elevated lesion on the left anterior wall of the rectal canal. Biopsy showed glandular proliferation and fibromuscular obliteration of the lamina propria, findings ...
Goodship J - - 1991
We present a male infant with cranial hemi-hypertrophy, a lymphangioma, a lipoma, and epidermal naevi. A diagnosis of Proteus syndrome was made. His father had had a large lymphangioma resected from the right side of the face as a child. We propose that Proteus syndrome has been transmitted from father ...
Dresse M F - - 1991
The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy ...
Taira T - - 1991
The authors report a case of cerebral aneurysm in an 8-year-old boy with Klippel-Trenaunay-Weber syndrome. This syndrome is a type of neurocutaneous disorder characterized by skin hemangiomas and hypertrophy of soft tissues and/or osseous structures in the involved region. Spinal hemangioma is occasionally found in this syndrome and may cause ...
Posteraro L - - 1991
A patient who developed a unilateral opercular syndrome following a cerebrovascular accident is described. Computed tomography showed that the lesion did not affect the opercular cortex, but involved deep white matter and the head of the caudate nucleus of the left hemisphere. Persistent hypophonia and transient aphasia were associated. Comparison ...
Macovei M - - 1991
The present study describes a patient, aged 72 yrs, with pontine crossed syndrome, displaying ipsilateral to the lesion a syndrome of cranial nerves V, VI, VII, VIII, on the right; a right neocerebellar syndrome; anhidrosis in the right hemibody; contralateral to the lesion: thermoalgic anesthesia with hyperhidrosis in the left ...
Spadaro M - - 1991
We describe the case of a patient with a history of trigeminal neuralgia who suddenly developed the "top of the basilar" syndrome. MRI disclosed ischemic lesions in the left paramedian mesencephalic tectum, in the left ventral thalamus, in the left occipital lobe and a megadolichobasilar artery (MDBA). The association of ...
Oliveria-Souza R - - 1991
Weber's syndrome is one of the classically described brainstem syndromes. The mesencephalic artery and the syndromes resulting from occlusion of its branches have been attracting increasing interest in the past few years. We present here a case of Weber's syndrome emphasizing that (1) it is one of the major syndromes ...
Lawrence C A - - 1991
The disturbance of visual perception associated with nystagmus is a rare phenomenon. This is a case of a 61-year-old woman who developed progressive right hemisensory deficit, left facial sensory deficit, vertigo, staggering to the left, left ptosis, vertical diplopia, and ataxia of the left upper extremity. She had rotatory nystagmus ...
Burke J P - - 1991
Klippel-Trenaunay-Weber syndrome is a rare phacomatosis of uncertain aetiology, variable expression, and disputed pathogenesis, whose cardinal signs are cutaneous angiomas, varicosities, tissue hypertrophy, and arteriovenous fistulae. The case reported herein is the first description of an unusual variant with limb and facial hemihypertrophy, congenital nystagmus, progressive ipsilateral anisomyopia, and strabismus. ...
Sooriakumaran S - - 1991
The Klippel-Trenaunay Syndrome is a triad of congenital anomalies characterized by a vascular naevus, varicose veins and hypertrophy of soft tissue and bone. A number of patients affected with this rare syndrome need amputation. In this paper the systemic problems, stump complications and prosthetic difficulties of four amputees with Klippel-Trenaunay ...
Lee S - - 1990
Sturge-Weber syndrome is a rare congenital disease which can impair a child's brain functions and psychosocial development. Its psychiatric aspects have been ignored in the literature. Two male Chinese patients who presented with paranoid disorders and one female Chinese patient who presented with depressive pseudo-dementia are described. Biological and psychosocial ...
Arvidsson J - - 1990
A 12-year-old girl, born full term after severe pre- and peripartal asphyxia, with transient abnormal newborn neurology and slight motor development deviations but no cerebral palsy syndrome, is described. At the age of 5, she developed a slowly progressive dyskinetic hemisyndrome, initially uncoordinated hyperkinetic movements in her left arm, dystonic ...
Sharma P - - 1990
An unusual retinal manifestation of arteriovenous communications is reported in a case showing a combination of Sturge-Weber and Kippel-Trenaunay syndromes. The arteriovenous communications are thought to be similar to those seen in the limbs in Parkes-Weber syndrome. This retinal finding indicates that the three syndromes are intricately related to each ...
Kato K - - 1990
The authors describe a case of choroid plexus hemangioma in a 49-year-old male. Computed tomographic scan showed an isodense mass at the trigone of the right lateral ventricle with homogeneous enhancement. He also displayed a port-wine nevus on the ipsilateral side of the face. At operation, the tumor was found ...
Riveron F A - - 1990
In rare instances, right pneumonectomy can produce progressive exertional dyspnea and reduce ventilatory reserve because of extreme mediastinal shift (right postpneumonectomy syndrome). The diagnosis can be made by bronchoscopy and computed tomography. We report a case of a 43-year-old patient in whom plombage with two Silastic breast implants produced mediastinal ...
Derkay C S - - 1990
A 17-year-old girl with Klippel-Feil vertebral anomalies presented with sudden onset of VPI and hoarseness after a minor neck torsion injury. Her symptoms were felt to be due to compression of the brain stem by the odontoid process (through basilar invagination). The VPI and hoarseness responded to conservative management with ...
Bisail M - - 1990
A case of POEMS syndrome in a 43-year-old male with polyneuropathy, osteolytic lesion of the basin due to solitary IgA-lambda plasmacytoma, cutaneous scleroderma-like changes, diffuse lymphadenopathy and hepatosplenomegaly is described. Liver biopsy showed a regenerative process of the parenchyma without laboratory and histologic evidence of necrosis. A peculiar finding was ...
Stewart G - - 1990
A baby girl born at 33 weeks' gestation weighing 2250 g presented with Sturge-Weber syndrome, features of the Klippel-Trenaunay syndrome, and absence of the inferior vena cava. We suggest that aplasia of the vena cava may be a feature of Klippel-Trenaunay syndrome when the capillary malformation affects the trunk.
Corsello G - - 1990
A 7-year-old female child with phenotype of Cervico-Oculo-Acousticus (Wildervanck's) syndrome is presented. In addition to fusion of multiple cervical vertebrae with short neck, abducens nerve palsy and deafness, the child showed severe growth and bone delay, renal abnormalities and slight mental retardation. The presence of such malformations seems to suggest ...
Tjaden B L - - 1990
Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative ...
Sawhney MPS
A 22 year-old woman presented with multiple soft, compressible, protuberant, bluish cutaneous lesions as well as firm, non-compressible, subcutaneous masses and varicose veins affecting the right upper limb of three years duration. There was atrophy of soft tissue of forearm by 2.5 cm. X-ray showed soft tissue densities, multiple phleboliths ...
Yoshikawa H - - 1990
Crossed cerebellar diaschisis (CCD) is known as a cerebellar hemispheric hypometabolism due to contralateral supratentorial infarction or tumor. We report a case with the Sturge-Weber syndrome (SWS), whose cerebral blood flow was reduced in the anatomically affected lesion of cerebrum and in the contralateral cerebellar hemisphere. Not only acquired diaschisis, ...
Pfeiffer R A - - 1990
A family is reported in which at least the propositus, his mother and his grandfather suffer from proximal symphalangism, conductive hearing loss due to stapes fixation, Klippel-Feil anomaly and abnormality of the nose with lack of alar flare. It is noteworthy that the first metacarpal bone is not abnormal. This ...
Kulenkampff H A - - 1990
Massive osteolysis is a very rare tumour-like lesion usually affecting young adults. There are 132 reported cases which we have reviewed, including 31 single cases of pelvic involvement. The term "massive osteolysis" is based on the typical radiological findings, such as increasing translucency and loss of bone density. The diagnosis ...
Levine C - - 1990
Localized soft tissue and bone overgrowth may be the result of many different causes and will vary in severity, from involvement of a single digit to one half of the body. There are a variety of causes, including chronic lymphedema; lymphangiomatous malformations; neurofibromatosis; vascular malformations including the Klippel-Trenaunay-Weber syndrome; macrodystrophia ...
Good W V - - 1989
A 10-year-old girl with Klippel-Trenaunay-Weber syndrome (KTWS) showed neuroradiologic signs of enlarged optic nerve and medial rectus shadows. In 3 years of follow-up, her vision has remained normal. This is the first report of optic nerve enlargement in this syndrome. The case demonstrates that enlargement of the optic nerve shadows ...
Diekmann-Guiroy B - - 1989
Intracranial tumors associated with Klippel-Feil syndrome usually occur in children, with spinal tumors being more common in adults affected by the syndrome. A rare case of a dermoid cyst at the craniocervical junction presenting as aseptic meningitis in an adult with Klippel-Feil syndrome is described. A review of the literature ...
Kumar A - - 1989
We describe a patient with soft-tissue and bony hypertrophy of all four limbs. The upper limbs were erythematous, with associated finger deformity. The soft-tissue irregular hypertrophy and engorgement subsided when the limbs were elevated. Retinal examination showed large, dilated, tortuous veins, with no dye leakage on fluorescein angiography. We feel ...
West P D - - 1989
We present a case of the Wildervanck (cervico-oculo-acoustic) syndrome exhibiting congenital deafness. Klippel-Feil anomaly and lateral rectus palsy with enophthalmos. Audiometry indicated a predominantly conductive loss which, because of masking difficulties, was assumed to be bilateral: an erroneous assumption supported by results of conventional petrous bone tomography which failed to ...
Ocić G - - 1989
The authors presented a case of relatively pure syndrome of alexia with agraphia in a patient speaking Serbo-Croatian language. Reading disturbances showed some characteristics of a deep dyslexia. Besides severely impaired reading and writing abilities, some components of Gerstamnn's syndrome were also present. Infarction in the posterior half of the ...
Bansal S K - - 1989
We report a case in whom clinical entity of ataxic hemiparesis (AH) is associated with one and half syndrome. The later finding favours a lesion in the dorsal pons while a lesion in the ventral pons will cause AH. Computerised axial tomography (CT) showed a pontine infarction over right side. ...
Ito M - - 1989
It is well known that when Sturge-Weber syndrome manifests with seizures in early infancy, hemiparesis develops early, seizures become intractable, and motor weakness and mental retardation are progressive. In North America and Europe, early surgical intervention is recommended in such cases. However, neurosurgical management of Sturge-Weber syndrome has not been ...
Lie J T - - 1988
Klippel-Trenaunay syndrome is a congenital angiodysplasia consisting of the triad of angiomas, osteohypertrophy and venous varicosities. Visceral involvement is not uncommon and may cause life-threatening complications. Presented here is a detailed study of the pathology of angiodysplasia in Klippel-Trenaunay syndrome. Venous fibromuscular dysplasia is the most prominent and consistent vascular ...
Klopfer J - - 1988
We examined a young patient with sickle cell anemia and a history of spinal meningitis who presented with signs of Weber's syndrome. Weber's syndrome is manifested by a homolateral third nerve palsy and contralateral hemiplegia. Evaluation and management of this case is presented, with a discussion on the probable association ...
Molteni N - - 1988
We describe two young adult patients with seizures and cerebral calcifications since childhood, diagnosed as Sturge Weber syndrome, who also had gluten enteropathy. Although the calcifications were located in regions similar to calcifications of Sturge Weber cases, many of the features of the syndrome were absent, and this diagnosis seems ...
Burns A M - - 1988
A mother with the Klippel-Feil syndrome, congenital hydrocephalus and increased intracranial pressure presented for delivery by Caesarean section at 33 weeks because of pre-eclampsia. Anaesthetic management comprised awake intubation using the fibreoptic bronchoscope, followed by induction and maintenance of general anaesthesia for the delivery of a live male infant. This ...
Rappaport Z H - - 1988
The Sturge-Weber syndrome includes unilateral cerebral cortical angiomatosis, which often leads to progressive cerebral dysfunction and epileptic seizures that are medically difficult to control. Cerebral resections and hemispherectomy have been successfully performed in the past in intractable epileptic cases. Two children with medically unresponsive generalized seizure activity secondary to the ...
van Rijn P M - - 1988
A wide variety of anomalies of the middle and inner ear has been reported in association with congenital deafness in Klippel-Feil syndrome. Findings of six new exploratory tympanotomies are added to a review of 14 previously reported; for this entire group, better hearing was achieved in eight ears. When the ...
Mahmoud S F - - 1988
An unusual and extreme case of Klippel-Trenaunay syndrome is presented. The patient has an extensive capillary hemangioma involving the right side of the body, increases in the length and girth of the right lower limb, gross varicosities of the superficial venous system, and complete absence of the deep venous system ...
Oyesiku N M - - 1988
A 12-week-old girl presented with cutaneous haemangiomata and hemihypertrophy of the chest and right upper extremity--stigmata of the Klippel-Trenaunay-Weber syndrome. Screening cranial CT-scan followed by cerebral angiogram revealed what is believed to be the first reported occurrence of a cerebral arteriovenous fistula in a patient with this syndrome. Craniotomy and ...
Burke J P - - 1988
Proteus syndrome is a recently recognized hamartoneoplastic malformation syndrome of uncertain etiology and variable expression, whose cardinal manifestations are pigmented nevi, hemihypertrophy, macrodactly, lipomata, and cerebroid-gyriform configuration of the skin on the soles of the feet. The characteristic features may be present at birth but become more apparent with time. ...
Daum R E - - 1988
A patient with Klippel-Feil syndrome who underwent abdominal surgery is presented and the anomaly reviewed. The anatomical abnormality and potentially unstable neck provide a potentially difficult tracheal intubation which was undertaken using an awake fibreoptic technique. The role of the fiberscope and the advantage of pre-operative assessment of the difficult ...
Cohen M M MM - - 1988
Four patients with Proteus syndrome are reported; 3 of the 4 died of unusual causes. Current findings of the syndrome are reviewed, and the present state of its delineation is assessed with emphasis on what is known about natural history. Differential diagnosis includes neurofibromatosis, Klippel-Trénaunay-Weber syndrome, Maffucci syndrome, epidermal nevus ...
Barkhof F - - 1988
Among 100 patients with an infarction of the brain reported on MR and clinically confirmed there were 4 with widespread lesions of the temporal and occipital lobes, thalamus, midbrain, pons and cerebellum, all supplied by arteries originating around the top of the basilar artery. Clinically these patients presented the "top ...
Moor J T - - 1988
The various combinations of varicose veins, limb hypertrophy, and hemangiomas necessitate diagnostic consideration of the Klippel-Trenaunay syndrome versus Weber's syndrome and cavernous hemangioma. The wide spectrum of this disease has generated diverse approaches, and treatment must be individualized. Whereas the Klippel-Trenaunay syndrome is rarely a surgical disease, the gigantic proportions ...
Paller A S - - 1987
The Sturge-Weber syndrome is characterized by angiomas of the leptomeninges overlying the cerebral cortex in association with a facial nevus flammeus. Although frequently included with other neurocutaneous genodermatoses, the syndrome is almost always sporadic in occurrence. The most common associated neurologic abnormality is seizures, which are controlled in more than ...
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 ...
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