Extradural spinal arachnoid cysts associated with spina bifida occulta

Spinal extradural arachnoid cysts are an uncommon cause of neural compression in children. Even more uncommon is the association of such cysts with spina bifida occulta.

Two girls, 12 and 8-years-old, presented with left leg pain, deteriorating gait, clinical signs of left L5 and S1 root compression, without bladder or bowel symptoms. The first patient had left foot drop. The second patient had muscle wasting and smaller left foot with pes cavus. Radiographs showed spina bifida occulta of S1 in both. MRI revealed an extradural cyst at the S1 level, indenting the thecal sac and the L5 and S1 roots. At operation in both patients a large arachnoid cyst arising from a small dural defect in the axilla of the left S1 root was compressing and displacing it and the dural sac. It was removed and the defect was repaired. The first patient improved with complete recovery of the foot drop. An MRI at 12 months showed no cyst recurrence. The second patient made good recovery initially, but at 10 months developed recurrent symptoms. An MRI scan showed recurrence of the cyst with root compression. On repeat exploration a different dural defect was identified in a more anterior position and was repaired.

The coexistence of extradural arachnoid cyst and corresponding bifid spinal segment has not been described previously. It raises the suspicion that the dural defect giving rise to the arachnoid cyst may be due to segmental dural dysgenesis in the context of the dysrhaphic neuroectodermal malformation.