Management of recurrent cerebrospinal fluid leak, current practices and open challenges. A systematic literature review.

SUMMARY Cerebrospinal fluid leak (CSFL) is a rare pathological condition which requires prompt and adequate management due to the high rate of associated major complications. At present, CSFL can be successfully treated through endoscopic endonasal approaches with success rates higher than 90%. Despite this, CSFL recurrence may occur, and its management is critical and still represents a matter of debate. A systematic review of the literature on the management of recurrent CSFL was conducted. It included a cohort of 1,083 cases of CSFL treated with surgical or conservative approaches; 112 cases of recurrence were reported for an overall recurrence rate of 10.3%. Most of the leaks were localised in the anterior skull base (68.3%) and identification of recurrent CSFL may be troublesome. Therefore, the entire skull base must be meticulously examined and long-term follow-up is mandatory. The risk for CSFL recurrence is higher in patients affected by idiopathic intracranial hypertension (IIH), but its role in CSFL recurrence is yet to be understood. Recurrent CSFL must be repaired as soon as possible in order to reduce the risk of intracranial complications. The use of early post-operative CSF diversion by lumbar drain (LD) is currently a matter of debate even in case of CSFL recurrences.