The Wildervanck syndrome consists of the Klippel-Feil deformity of the spine, eyeball retraction, lateral gaze weakness, and hearing loss. Conductive hearing loss, as well as a more frequently occurring sensorineural hearing loss, is caused by dysplasia of the inner ear. Typically, polytomograms demonstrate a bulbous vestibule and dilated lateral semicircular ...

A case of Sturge-Weber syndrome, uncommon in its intraoral extent, is presented. The pathologic process involved both the soft and osseous tissues of both the maxilla and mandible of the affected side. The first known histopathologic description of alveolar bone involvement is presented. The patients was treated by means of ...

In the phakomatoses--von Recklinghausen's disease, the Sturge-Weber syndrome, tuberous sclerosis, and von Hippel-Lindau disease--pathologic changes in the eye are often evident. Most cases are described as a single entity. The following is a case report of concomitant Sturge-Weber syndrome and von Recklinghausen's disease in which both conditions involved the eye.

An 8-year-old boy with a variant of the Klippel-Trenaunay-Weber syndrome (KTW syndrome) is described. The hemangiomatous tissue located to the right half of his trunk and extremities was hypotrophic. On the same side, on his face and gingivae the tissue appeared hypertrophic and dental abnormalities were present. Moreover, the patient ...

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine hemangiomas, deep venous system abnormalities, superficial varicosities, and bony and soft-tissue hypertrophy. When associated with an arteriovenous fistula, it has been termed Klippel-Trenaunay-Parkes-Weber syndrome. It is imperative that both the radiologist and surgeon be aware of this entity, as incomplete evaluation and inappropriate ...

The stapes gusher which is the most dramatic complication of stapedectomy arises from an abnormal communication between the subarchnoid and perilymphatic spaces. This congenital defect may be associated with other anomalies such as the Klippel-Feil syndrome. Two cases of stapes gusher in patients with congenital fixation of the footplate are ...

A patient with a rare case of Klippel-Trenaunay-Weber syndrome presented with paraplegia due to compression by a vertebral and epidural cavernous hemangioma. The metameric distribution of the large cutaneous vascular nevus provided the clinical clue to the nature of the spinal lesion. The association of the two lesions is explained ...

A review of Weber-Christian syndrome in infancy is presented along with the report of two cases. Both infants had low serum properdin levels, and one patient showed remissions of his disease when he was T-lymphocytopenic. This suggests that the T-lymphocyte may have an important role in the pathogenesis of Weber--Christian ...

Three cases of Klippel--Trenaunay--Weber's syndrome are presented. The syndrome is characterized by cutaneous hemangioma, hypertrophy of an extremity and varicose veins. It is combined with angiodysplasias, either in the form of aplasia/hypoplasia of deep veins and/or in the form of arteriovenous shunts. The varicose veins will frequently be the dominant ...

Two cases of interhemispheric subdural haematoma are reported. The first patient presented with a falx syndrome of contralateral hemiparesis, most marked in the lower extremity. The second patient had vertex headaches and was diagnosed with computerised tomography. Serial studies suggested that the interhemispheric haematoma may have migrated to a lateral ...

A case of recurrent meningitis due to a stapes footplate fistula in a child with Klippel-Fiel syndrome is reported. The relationship of deafness to this latter syndrome is noted and theories for the aetiology of the fistula discussed. A stapedectomy was performed and the oval window was obliterated using a ...

Cobb syndrome consists of a vascular skin nevus associated with an angioma in the spinal cord. We describe a young man with this condition. To our knowledge, this is the 28th case reported. Cobb syndrome must be differentiated from other syndromes characterized by vascular cutaneous lesions associated with central nervous ...

Intervertebral disk calcification and spine fusion were observed in patients with Klippel-Feil syndrome, myositis ossificans progressive, juvenile rheumatoid arthritis, ankylosing spondylitis, and surgical spine fusion. The fusion appears to come first, leading to calcification. This suggests that fusion prevents the normal mechanical stresses within the disk, leading to premature degenerative ...

Five cases in which the Klippel-Trenaunay-Weber syndrome was associated with a spinal cord arteriovenous malformation are reported: they formed part of a series of 150 spinal arteriovenous malformations. Hypertrophic lesions, dilated arteries and varicose veins were present in every case, but cutaneous angiomatosis was found in only two. In each ...

A syndrome characterized by hypoxemia aggravated by exercise, orthodeoxia, hypocapnia, and evidence of hyperdynamic circulation, but otherwise normal indices of pulmonary air flow, volume, and distribution of ventilation has been observed as an infrequent complication of hepatic cirrhosis. An illustrative case is described, the features of which support the presence ...

Of the venous abnormalities in the Sturge-Weber syndrome, deep venous occlusion is an uncommon finding. As far as can be determined it has been described in only two previously recorded cases. Another such case is presented to illustrate the usefulness of this finding, in conjunction with other venous anomalies, in ...

A 53-year-old white man was noted to have bilateral retinitis pigmentosa associated with a left nevus flammeus, a left choroidal hemangioma and unilateral glaucoma. No central nervous system manifestations of Sturge-Weber syndrome were noted. The association of retinitis pigmentosa and the nevus flammeus is probably a chance occurrence.

Two cases of the Klippel-Trenaunay-Weber syndrome are presented. In one of the cases postsurgical complications arose in association with the vascular component of the syndrome complex. Oral surgeons and dentists must remain aware of the potentially serious complications of dental and/or surgical procedures in such cases. Other stigmata of the ...

Cerebral cortical calcification identical to that of the Sturge-Weber syndrome was observed in two children. In one child the calcification appeared after intrathecal administration of methotrexate and skull irradiation because of leukemia involving the central nervous system. In the other child, who had coeliac disease and epilepsy, the calcification appeared ...

A case of Behcet's syndrome with vascular complications is presented. This case had a familial occurrence of Behcet's syndrome. His vascular complications were deep vein thrombophlebitis of both legs, aneurysms of the left common iliac artery and the left femoral artery and the occlusion of the left subclavian artery. He ...

The most consistent electroencephalographic finding in 16 cases of Sturge-Weber syndrome was a unilateral reduction of background amplitude in the waking record. Comparable asymmetries were noted in those patients in whom sleep recording also was done. Physiologic responses (to hyperventilation and photic driving) usually were decreased on the involved side. ...

The combination of Klippel-Trénaunay-Weber syndrome (KTWS) with intracranial vascular malformations is documented by angiography in a 25 year old man with the classical features of KTWS: systematized naevi, hypertrophy of the right side, and varicosis. In addition, the syndrome was associated with "cuts laxa", a coincidence of somar rarity. The ...

We describe a female infant with the cervical vertebral fusion (Klippel-Feil) syndrome whom we recognized at birth because of her short neck, restriction of cervical movement, and low posterior hairline. X-ray examination showed anomalies of C1, and between C2-3 and C3-4; thus, we classified her as type II, with variable ...

Extensive retinal vascular disease was noted in three patients from two families with Favre-Goldmann syndrome. In addition to classical features they had pronounced leakage from some retinal vessels. Vessels were either opaque ('sclerotic') or non-perfused. Cystoid macular oedema was a contributing cause of decreased vision. Two of the three patients ...

Four children affected by Sturge-Weber syndrome and demonstrating bilateral intracranial calcification are described, bringing up to 21 the number of similar reported cases. The frequency of bilateral hemisphere involvement in this syndrome is not known, but it might be as high as 15%. If present, neurosurgical intervention is, in our ...

The Klippel-Trenaunay syndrome presents with a vascular nevus extending over an entire extremity, ipsilateral varicose veins dating from infancy and hypertrophy of all the tissues of the affected limb. This paper illustrates the problems that can be encountered in the management of this disorder. Congenital angiodysplasias should be suspected in ...

Large, infiltrative cavernous hemangiomas of the distal colon were present in 3 children with the Klippel-Trenaunay syndrome, 2 of whom are the subject of this report. The lesion is manifested clinically as intermittent rectal bleeding starting in the first 5 years of life. The radiographic features include (a) varicose lesions ...

A 22-year-old man was seen with the ocular histoplasmosis syndrome. This case demonstrates that spontaneous or "de novo" neovascularization without prior pigmentary changes can occasionally, although rarely, give rise to the disciform macular disease process and the potential for loss of central vision. The lesion initially responded favorably to treatment ...

Hitherto unidentifiable and therefore untreatable congenital microfistulas were detected with the aid of continuous-wave directional Doppler and a fine-beam pencil probe. The microfistulas formed part of the Klippel-Trenaunay (K-T) syndrome. The management of one case is reported in detail and in three others salient features are touched upon. Careful clinical ...

Genitourinary hemangiomas in 2 children with Klippel-Trenaunay syndrome are reported. The presence of cutaneous hemangiomas should alert urologists to the possibility of urinary tract hemangiomas inpatients with gross hematuria. Transurethral manipulation of bladder hemangiomas should be avoided because of the danger of excessive hemorrhage, the treatment of choice being partial ...

An infant with the linear nevus sebaceous syndrome also had new findings of porencephaly and nonfunctioning major cerebral venous sinuses. This infant is the first described with the syndrome to undergo angiographic study. Similar nonfunctioning major cerebral venous sinuses are seen in the Sturge-Weber syndrome. A leptomeningeal angioma recently was ...

Ten patients are presented who demonstrate a newly recognized association of macrocephaly with unusual angiomatosis and limb asymmetry in three somewhat similar cutaneous vascular disorders: Klippel-Trenaunay-Weber syndrome, the combination of Sturge-Weber anomaly with Klippel-Trenaunay-Weber syndrome, and cutis marmorata telangiectatica congenita. The etiology of the macrocephaly in patients with these conditions ...

Tuberous sclerosis and Klippel-Trenaunay-Weber (KTW) syndromes are phakomatoses which are believed to be inherited separately. A 41 year old woman presented with the classic features of tuberous sclerosis: adenoma sebaceum, mental retardation, and seizures. In addition, the diagnostic triad of KTW involved the left lower limb: cutaneous naevi, a vascular ...

Of thirty-nine patients with Klippel-Feil syndrome, twenty-five (64 per cent) had significant genitourinary-tract anomalies demonstrated by intravenous urogram and physical examination. The incidence of these anomalies in Feil's three types of the syndrome was essentially the same, unilateral renal agenesis being the most common. A routine intravenous urogram is indicated ...

Clinical and electrophysiological observations are described in 7 patients with clinically well-identified vascular lesions of the brain-stem or diencephalon. In the patients of Group A with a thalamic syndrome, the somatosensory cerebral evoked potentials had a reduced voltage and increased latency on the affected side. No significant anomalies were recorded ...

Electron microscopy of hepatic tissue obtained by percutaneous needle biopsy from nine patients with Gilbert's syndrome has revealed in every case gross hypertrophy of hepatocyte agranular endoplasmic reticulum but no other important abnormality. While this may have relevance to impairment of microsomal enzyme activity controlling bilirubin conjugation within liver cells, ...

Post-inflammatory elastolysis and cutis laxa (Marshall, Heyl & Weber, 1966) is a skin disease in African infants which appears to be comparatively common in at least two countries. Destruction of elastic tissue and atrophy are preceded by urticarial or by annular erythematous-popular lesions and result in severe disfigurement. The clinical ...

Chronic liver disease typical of chronic active 'lupoid' hepatitis together with cyanosis, clubbing and hypertrophic osteoarthropathy in a 42-year-old female is described. In addition she had severe nose bleeds, gastro-intestinal haemorrhages, syncopal attacks with generalised convulsive seizures, pulmonary arterio-venous fistulae as manifestations of Rendu-Osler-Weber syndrome. A study of the literature ...

An extreme case of Klippel-Trenaunay-Parkes Weber syndrome is presented. The patient had extensive cutaneous naevus involving the left side of the body and consisting of naevus flammeus, hemangioma cavernosum, and naevus verrucosus. The left extremities were longer and there were multiple arteriovenous connections between a and v subclavia and a ...

The association of soft tissue and bony overgrowth, varicose veins and port-wine nevus is known as Klippel-Trenaunay-Weber syndrome. An unusual case of a young woman with this syndrome is presented. In addition to the classic features she had face swelling, bilateral limb involvement and an infected cutaneous lymphangioma. No evidence ...

Electroencephalographic (E.E.G.), polyelectromyographic (poly-E.M.G.), and electro-oculographic (E.O.G.) studies were made of 16 young children and babies referred because of prolonged episodes of rapid irregular movements of eyes and limbs (R.I.M.E.L.) sometimes involving also the trunk and head. This unusual syndrome of unknown aetiology developed over a period of a few ...

Two cases of denervation hyperpathia that responded well to treatment with carbamazepine are presented. A theoretical relation of this disorder to trigeminal neuralgia and focal seizures is emphasized in the light of experimental data and clinical characteristics.

A case with signs of a lower cervical anterior spinal artery syndrome incident to vertebral angiography by the transfemoral approach is described. The literature is reviewed and a probable pathogenesis of angiography-related spinal cord injury is discussed.

Founded in 1981, Vein Clinics of Ameica is based in Schaumburg, Illinois. The site provides illustrated descriptions of varicose veins, spider veins, venous leg ulcers, facial veins, and hand veins, describes restless leg syndrome, and Klippel-Trenaunay Syndrome. Treatments described include endovenous laser treatment and sclerotherapy. Some frequently asked questions and ...

Endocrine function was evaluated in a 38-year-old man who had patchy asymmetrical acral hypertrophy and giantism. The history and clinical manifestations were consistent with previously described cases of the Klippel-Trenaunay syndrome. Pituitary and peripheral hormone concentrations were generally elevated, but his endocrine status appeared normal by clinical evaluation. Of particular ...