Giant Intrabdominal Meningocele in a Patient with Marfan Syndrome: Case Description and Surgical Management

Anterior sacral meningocele is a rare complication associated with Marfan syndrome. There is no consensus regarding management and surgical treatment. Here, we describe the case of a 44-year-old women with Marfan syndrome who presented a progressive abdominal mass with gastrointestinal and urinary symptoms. Abdominopelvic MRI revealed a 3cm S1 dural ectasia and a 19x17x15cm abdominal mass. A posterior sacral transdural approach was performed. Dural friability made ligation unfeasible. Obliteration with a fat flap and fibrin sealant was the treatment alternative. Postoperative MRI imaging performed 4 months after surgery evidenced complete resolution of the intrabdominal collection. The patient remains free of symptoms 2 years later. Here we present one of the largest meningoceles reported to date in the literature, and illustrate the surgical management in a patient with Marfan syndrome, where technical difficulties arose. Solely posterior sacral transdural approach with fat graft may be sufficient for the treatment of these patients even with voluminous pelvic meningoceles.