No abstract available.
Sturge-Weber Syndrome*

No abstract available.
Sturge-Weber Syndrome*

No abstract available.
Sturge-Weber Syndrome*

Humans ; Infant, Newborn ; Sturge-Weber Syndrome

Adolescent ; Diagnosis, Differential ; Humans ; Male ; Sturge-Weber Syndrome ; pathology

PURPOSE: To observe MR findings of vascular abnormality in Sturge-Weber syndrome and to determine the value of MRI in diagnosis. MATERIALS AND METHODS: Ten patients with Sturge-Weber syndrome (age : 3 months-32 years)were evaluated by MR imaging ; in six and four cases, respectively, the results were correlated with those of CT and angiography. We retrospectively analysed changes in the cortical vein and deep venous system, including the medullary and subependymal vein, as well as an largement of the choroid plexus, leptomeningeal enhancement, and changes in diploic space. RESULTS: In all cases except one, in which non-contrast enhanced study had been performed, varying degrees of leptomeningeal enhancement were seen. In nine cases the cortical vein became smaller; enlargement of the choroid plexus was seen in eight cases, change in the diploic space in seven (including three in which there was angiomatous involvement), and enlargement of the deep venous system in seven. In younger patients, collateral pathways were less developed and leptomeningeal angiomatous changes were more pronounced than in those who were older. CONCLUSION: MRI is a useful modality for the evaluationn of vascular changes in Sturge-Weber syndrome. These changes vary according to a patient's age and the duration of the disease.
Angiography ; Choroid Plexus ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Sturge-Weber Syndrome* ; Veins

Sturge Weber syndrome (SWS) is a rare, congenital neurocutaneous syndrome. We rarely experienced neurocutaneous disorders in forensic practice but there are unexpected sudden deaths of patients with SWS due to epilepsy, intracranial hemorrhage, thromboses and secondary accident. We introduce a case of drowning death probably due to an epileptic attack of a person diagnosed as a patient of SWS by an autopsy. We review some of the neurocutaneous syndromes and a discussion of autopsy approach is presented.
Autopsy ; Death, Sudden ; Drowning ; Epilepsy ; Humans ; Intracranial Hemorrhages ; Neurocutaneous Syndromes ; Sturge-Weber Syndrome ; Thrombosis

Sturge-Weber syndrome was rare. But reported from 1860. We present a typical case of Sturge-Weber syndrome in a child and discussed the symptoms, signs, and pathological finding of various examinations in neurosurgical field such as plain x-ray, 4-vessel angiography, CT scan, EEG, IQ test, exophthamometry, opthalmometry, and fundoscopy. We find marked abnormality and asymmetry in that examination.
Angiography ; Child ; Electroencephalography ; Humans ; Sturge-Weber Syndrome* ; Tomography, X-Ray Computed

We present a case of nevus flammeus associated with angiokeratoma of Fordyce and lymphangioma circumscriptum in a 26 year-old Korean man. We have known that the congenital hemangiomas may be associated with many syndromes such as Kasabacb-Merritt syndrome, Sturge-Weber syndrome and etc. Recently the congenital hemangiomas associated with angiokeratoma or lymphangioma were reported in several literatures, Watson and McCarthy reported that it seemed to be established that three types of benign vascular tumors had a common origin and grew in an identical fashion by projecting buds of endothelial tissue. So the congenital hemangiomas may have a close association with angiokeratoma or lymphangioma.
Adult ; Angiokeratoma* ; Hemangioma ; Humans ; Lymphangioma* ; Nevus* ; Port-Wine Stain* ; Sturge-Weber Syndrome

Sturge-Weber syndrome is a rare congenital anomaly which includes facial port-wine stains with ipsilateral intracranial, hemangioma, ipsilateral choroidal hemangioma, and congenital glaucoma. The syndrome is thought to result from dysmorphogenesis of cephalic neuroectoderm. We report a case of Sturge-Weber syndrome combined with congenital ocular anomaly such as phthisis bulbi, corneal opacity, and iris anomaly.
Choroid ; Corneal Opacity ; Glaucoma ; Hemangioma ; Iris ; Neural Plate ; Port-Wine Stain ; Sturge-Weber Syndrome*