Communicating hydrocephalus and raised intracranial pressure in association with multi-systemic smooth muscle dysfunction syndrome (MSMDS).

Multi-systemic smooth muscle dysfunction syndrome (MSMDS) is extremely rare and can manifest in multiple ways. Associated hydrocephalus has not yet been reported. Here, we report a three-year-old girl with communicating hydrocephalus and raised intracranial pressure secondary to MSMDS. Pathological mechanisms are proposed, as is the need to investigate patients diagnosed with MSMDS for ventriculomegaly and raised pressure.

On day admissions in neurosurgery: a comparative cohort study.

Day of admission neurosurgery is a viable method to reduce health care associated costs, complications and length of stay. Within the national health service in England the picture is very mixed with some neurosurgery centres universally admitting patients the day before and others admitting on the day of surgery.We altered our admissions policy during a 4 month time period from 'day-before' surgery to 'day-of' surgery for elective neurosurgery. A number of patients still continued to be admitted the day before surgery due to consultant choice. We conducted a comparative cohort study of these two patient groups to see if there were any differences in surgical cancellation rates, the reasons for these cancellations and the implied cost savings.In total 199 patients underwent neurosurgery during this time period, 87 patients were admitted on the 'day-of' and 112 patients on the 'day-before' surgery. The overall cancellation rate was 18%. The cancellation rate in patients admitted on the 'day-of' surgery was 12.6% (11/87). The rate of cancellation in patients admitted the 'day-before' surgery was 22.3% (25/112). This difference was not significant (p = 0.1). Day of surgery admission resulted in a cost saving of almost £30,000 in this group of patients over a 4 month period. If extrapolated for all patients over the course of a year it would result in cost savings in the region of £150,000.In summary, admitting elective neurosurgery patients on the day of surgery does not affect cancellation rates, prevents unnecessary overnight hospital admission and results in significant cost saving.

FANCD2 re-expression is associated with glioma grade and chemical inhibition of the Fanconi Anaemia pathway sensitises gliomas to chemotherapeutic agents.

Brain tumours kill more children and adults under 40 than any other cancer. Around half of primary brain tumours are glioblastoma multiforme (GBMs) where treatment remains a significant challenge, where survival rates have improved little over the last 40 years, thus highlighting an unmet need for the identification/development of novel therapeutic targets and agents to improve GBM treatment. Using archived and fresh glioma tissue, we show that in contrast to normal brain or benign schwannomas GBMs exhibit re-expression of FANCD2, a key protein of the Fanconi Anaemia (FA) DNA repair pathway, and possess an active FA pathway. Importantly, FANCD2 expression levels are strongly associated with tumour grade, revealing a potential exploitable therapeutic window to allow inhibition of the FA pathway in tumour cells, whilst sparing normal brain tissue. Using several small molecule inhibitors of the FA pathway in combination with isogenic FA-proficient/deficient glioma cell lines as well as primary GBM cultures, we demonstrate that inhibition of the FA pathway sensitises gliomas to the chemotherapeutic agents Temozolomide and Carmustine. Our findings therefore provide a strong rationale for the development of novel and potent inhibitors of the FA pathway to improve the treatment of GBMs, which may ultimately impact on patient outcome.

Aorto-enteric fistula development secondary to mycotic abdominal aortic aneurysm following intravesical bacillus Calmette-Guerin (BCG) treatment for transitional cell carcinoma of the bladder.

INTRODUCTION: Intravesical BCG-instillation for bladder cancer is considered safe but is not without risk. While most side-effects are localised and self-limiting, the development of secondary vascular pathology is a rare but significant complication. PRESENTATION OF CASE: A 77-year-old male presented with a mycotic abdominal aortic aneurysm and associated aorto-enteric fistula 18 months after receiving intravesical BCG-instillations for early stage transitional cell carcinoma. DISCUSSION: Response rates to intravesical BCG for early stage transitional cell carcinoma are high. The procedure produces a localised inflammatory response in the bladder but the exact mechanism of action is unclear. The treatment is generally well tolerated but BCG-sepsis and secondary vascular complications have been documented. Mycotic abdominal aortic aneurysm with associated aorto-enteric fistula secondary to BCG is very rare. Few examples have been documented internationally and the extent of corresponding research and associated management proposals is limited. Surgical options include in situ repair with prosthetic graft, debridement with extra-anatomical bypass and, occasionally, endovascular stent grafting. Recommended medical therapy for systemic BCG infection is Isoniazid, Rifampicin and Ethambutol. CONCLUSION: Current screening methods must be updated with clarification regarding duration of anti-tuberculous therapy and impact of concomitant anti-tuberculous medication on the therapeutic action of intravesical BCG. Long-term outcomes for patients post graft repair for mycotic aneurysm are unknown and more research is required regarding the susceptibility of vascular grafts to mycobacterial infection. Recognition of the risks associated with BCG-instillations, even in immunocompetent subjects, is paramount and must be considered even several months or years after receiving the therapy.