Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent.

We describe a 14-year-old girl with Marfan syndrome who presented with severe postural headache. Dural ectasia was demonstrated radiologically. Cerebrospinal fluid (CSF) pressure was immeasurable on formal measurement. Radionucleide cisternography failed to demonstrate a CSF leak. We consider that the underlying fibrillinopathy in Marfan syndrome rendered the dura sufficiently permeable to CSF leakage to cause the low CSF pressure headache. The patient was treated successfully with epidural autologous blood patch.

Barriers to immunization: attitudes of general practitioners to varicella, the disease and its vaccine.

OBJECTIVES: To investigate the attitudes of general practitioners (GPs) to varicella disease and varicella vaccine. METHODS: A cross-sectional questionnaire was mailed to GPs in Fairfield (located in the south-western suburbs of Sydney, NSW, Australia) to identify attitudes about varicella vaccine, previous experience with varicella disease and the likelihood of prescribing varicella vaccine. RESULTS: Of 239 questionnaires issued, 160 were returned (67%). The majority (72%) of respondents agreed that varicella vaccine should become part of the immunization schedule. However 12% of GPs did not support vaccination for varicella as they considered it to be a benign self-limiting disease. Respondents who had experience with varicella complications were significantly more likely to recommend universal vaccination (OR 3.36; 95% confidence interval (CI) 1.38-8.19) whereas those respondents who were concerned about side effects of the vaccine were less likely to recommend universal vaccination (OR 0.31; CI 0.15-0.63). CONCLUSIONS: The majority of GPs are receptive to varicella vaccination becoming part of the immunization schedule. Experience with varicella complications is associated with recommending universal varicella vaccination. General practitioners in this cohort do not consider varicella to be a benign disease, but they are concerned about possible unknown side effects of the vaccine. Public health measures for introducing universal vaccination need to address these concerns.

Severe gingival recession in trisomy 18 primary dentition. A clinicopathologic case report of self-inflicted injury associated with mental retardation.

This clinicopathologic case report documents severe gingival recession in the primary dentition of a trisomy 18 patient. Primary molar and canine teeth exhibited recession extending beyond the midpoint of the buccal aspect of the root, occasionally reaching the root apex. Radiographic examination revealed taurodontism in both primary and permanent teeth. Clinical and histopathologic findings, along with case history, eliminated the possibility of prepubertal periodontitis and suggested a diagnosis of self-inflicted injury associated with mental retardation. Histologic examination of the primary teeth revealed normal cementum and dentin structure. Taurodontism, histologic structure of the dentition, and severe attachment loss in the primary dentition have not been described previously in trisomy 18.