Successful treatment of intractable pseudomeningocele with FXIII deficiency by surgery and FXIII replacement therapy: A case report

Abstract

Pseudomeningocele is an extradural cystic collection of cerebrospinal fluid (CSF) and is rare and typically asymptomatic. However, pseudomeningocele is sometimes associated with symptoms. Whether symptomatic pseudomeningocele is best treated conservatively or surgically remains controversial. Factor XIII (FXIII) is a blood coagulation factor that also promotes fibroblast proliferation during wound healing. Although treatment of postsurgical CSF leakage with FXIII has been reported, there have been no reports on surgical treatment and FXIII replacement therapy of pseudomeningocele with FXIII deficiency. We report a case of pseudomeningocele with FXIII deficiency that was successfully treated by surgery and FXIII replacement therapy. The patient presented with symptoms of intracranial hypotension syndrome that had started a few months after laminectomy for thoracic ossification of the ligamentum flavum 2 years earlier. Magnetic resonance imaging and delayed computed tomography myelography confirmed a diagnosis of pseudomeningocele. Epidural blood patch treatment was performed twice but did not result in improvement. Furthermore, the FXIII level decreased to 56%, so the patient was also diagnosed as having acquired FXIII deficiency. We elected to treat the patient by surgery with FXIII replacement therapy. The dural injury was repaired using an artificial dura mater patch, fibrin glue, and polyglycolic acid sheets. The FXIII level was 74%–135% during the perioperative period. The patient had a good postoperative course. Postoperative magnetic resonance images showed resolution of the pseudomeningocele. There was no recurrence during 6 months of follow-up. Perioperative FXIII replacement may be a useful treatment for pseudomeningocele with FXIII deficiency.