Management of Giant Anterior Meningocele in a Young Patient with Marfan Syndrome

Dural ectasia is a common manifestation of Marfan syndrome, characterized by the progressive weakening and dilation of the dural sac, typically occurring in the lumbar or sacral regions. This condition results from the alteration of connective tissue due to fibrillin-1 deficiency, which compromises the structural integrity of the dura mater. Dural ectasia can cause a range of symptoms, including lower back pain, headaches and neurological deficits, as the expanded dural sac compresses adjacent nerve roots. It is often identified through imaging, with MRI being the modality of choice, showing characteristic ballooning of the dural sac. This condition is particularly relevant in Marfan patients as it may increase the risk of complications such as spinal instability or nerve root compression. Reference Vornetti, Vara and Baroni1