A rare event of perioperative spinal subarachnoid hemorrhage in mixed connective tissue disease: a case report

Spinal subarachnoid hemorrhage (SSH) is an extremely rare event and its causes include trauma, vascular malformation, anticoagulant therapy, and autoimmune disease. Although connective disorders are associated with an increased risk of cerebral subarachnoid hemorrhage (SAH), to our knowledge, the occurrence of SSH associated with mixed connective tissue disease (MCTD) have not been addressed in the literature. We report the unique occurrence of SSH in a patient with anti-U1 ribonucleoprotein (U1-RNP) and anti-nuclear antibodies (ANA) positive MCTD triggered by abdominal surgery.

A 69-year-old woman with MCTD was admitted to our hospital because of vomiting and abdominal pain. Surgical treatment of adhesion ileus with small bowel resection was followed on the second postoperative day by sudden back pain radiating to the legs and abdominal belt-shaped lumbar exanthema. A spinal anesthesia was not performed. Neurological examination revealed loss of sensation and muscle strength in both legs. Magnetic resonance imaging (MRI) showed an SSH beyond T6 and the lumbosacral junction with ventral displacement of the spinal cord without myelopathy. Rapid improvement in motor function and sensitivity allowed a conservative management. The patient recovered successfully and was discharged without neurological deficits.

We postulate that vascular rupture causing SSH was triggered by perioperative increased intraluminal abdominal pressure combined with fragility of the spinal arachnoid mater due to MCTD. The decision to undergo a conservative treatment of the SSH was triggered by the rapid improvement of the neurological presentation and the disappearance of symptoms in a few days. We recommend caution in patients with connective tissue diseases who undergo thoracic or abdominal surgery and be aware for neurosurgeons that SSH is possible although rare and that conservative treatment may be considered.