1/180. Sturge--Weber syndrome: report of an unusual cutaneous distribution. This is an 11 month old girl who has been referred to our institute for a seizure disorder. Her physical examination showed angiomas on the left side of her face, and more extensive and prominent ones on her palms and soles. Her right face and arm were smaller than the left, associated with hypotonia and moderate weakness in the right forearm. Computerized tomography of head showed intracranial calcification in the left fronto-parietal region, and brain MRI, with gadolinium, revealed an extensive leptomeningeal angioma over the entire left hemisphere. This case was diagnosed as sturge-weber syndrome with unusual cutaneous manifestations. ( info) |
| The sturge-weber syndrome consists of unilateral port-wine haemangioma of the face which may be associated with an ipsilateral intracranial haemangioma and choroidal angioma. The common derivation of the meningeal, choroidal and facial vessels may explain a congenital malformation of all three areas. I report the case of a child with typical sturge-weber syndrome who had a previously undescribed association with bilateral congenital syndactyly. ( info) |
| 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 after treatment with anticonvulsants. This treatment was also contributing to the development of profound megaloblastic anemia. The unspecificity of the Sturge-Weber calcification is stressed and the hypothesis is put forward that the calcification may be secondary to folic acid deficiency interfering with the matabolism in the central nervous system. ( info) |
| Two patients with adolescent onset glaucoma associated with sturge-weber syndrome have been presented. Both patients were successfully treated with ab externo trabeculectomy. This procedure is suggested as an additional means for controlling the late onset glaucoma associated with the syndrome without provoking additional complications or deterioration. Early treatment is emphasized before the irreversible changes of chronic glaucoma become manifest. ( info) |
5/180. Clinical experience with presumed hemangioma of the choroid: radioactive phosphorus uptake studies as an aid in differential diagnosis. A total of 27 cases of choroidal hemangioma was evaluated with radioactive phosphorus uptake studies. In each case the diagnosis of malignant melanoma was initially entertained, but the 32P test was unequivocally negative in every instance. follow-up studies in every case have supported the clinical diagnosis. In 22 patients, the lesion was treated with photocoagulation, and in every treated case the serous macular detachment disappeared. The importance of early and correct clinical diagnosis is stressed, since these are salvageable and potentially useful seeing eyes. ( info) |
| A 6-month-old boy with sturge-weber syndrome was scheduled for congenital glaucoma and left buphthalmus surgery. physical examination revealed haemangioma throughout the right trigeminal nerve, congenital glaucoma, left megalocornea and bilateral buphthalmus. Examination of the eye was performed under general anaesthesia, was followed 2 days later by trabeculotomy. No premedication was given to the patient. After induction of anaesthesia with halothane, O2 and N2O muscle relaxation was achieved with atracurium and he was intubated gently. No difference was observed in vital signs during surgery. At the end of the operation he was given oxygen 100% and extubated, muscle relaxant reversal was with atropine and neostigmine. No complication was observed in the post-operative period. ( info) |
| A case of sturge-weber syndrome is described in which all paranasal sinuses were grossly enlarged. The mastoid, petrous and squamous portions of the temporal bone on both sides were fully pneumatized and the right orbit was distorted. There was resistant infection in the left maxillary sinus. These unusual features were well demonstrated by computed tomographic scans. ( info) |
| ISSUES AND PURPOSE: Chronic illness is a way of life for parents of children with sturge-weber syndrome (SWS), a rare progressive congenital disease that has as its defining feature a port wine stain. This case study describes the experience of one family living with a child with SWS. CONCLUSIONS: This family's struggle with a devastating syndrome and the ways in which they coped and maintained hope inform all those who care for families living with a chronically ill, disabled child. PRACTICE IMPLICATIONS: social support is critical for families facing overwhelming care needs. Families also need anticipatory guidance about child rearing, developmental milestones, decision making, and coping strategies. Additionally, families may need help in mobilizing professional and family resources and in effectively using available services. ( info) |
9/180. Sturge Weber syndrome with intraoral manifestations. A case report. 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 "tram line" intracranial calcifications. This case report presents a typical case of Sturge Weber Syndrome. It gives the radiological and CT scan findings and the important role played by them in the diagnosis of this syndrome. Emphasis is given to the differentiation of diphenylhydantoin induced gingival hyperplasia from the angiomatous enlargement of the gingiva before any treatment is planned. ( info) |
10/180. Double phakomatosis. 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. ( info) |