Intermittent palpebral oedema following surgery for nasofrontal encephalocele

To report a case of intermittent palpebral oedema secondary to a cerebrospinal fluid (CSF) leakage, following encephalocele surgery.

A 9-year-old girl consulted with a history of intermittent swelling of the left upper and lower eyelid. The symptoms started at the age of one. As a relevant fact in her medical history, a neurosurgical repair of a nasofrontal encephalocele at the age of 9 months was withheld. Elaborate imaging studies demonstrated a leakage of cerebrospinal fluid (CSF) into the preseptal periorbital soft tissues, originating from a bony defect in the medial orbital wall. The patient was treated conservatively.

A CSF leakage in the orbital or periorbital region is a rare finding. In the literature, similar defects have been reported after trauma or associated with neoplasia. To our knowledge, this is the first case of periorbital CSF leakage after encephalocele-surgery reported in the literature.