[Intramedullary bronchogenic cyst. Apropos of 1 case. Discussion of the endo-ectodermal adhesion syndrome]

The authors report the case of a 40-year-old man who has presented for many years an intermittent progressive spastic paraplegia. Plain films of the spine show very important dysraphic abnormalities of the inferior dorsal column and the myelography shows a complete block suggesting an intramedullary space-occupying lesion at four levels above the vertebral abnormalities. The operation permits a total removal of an intramedullary "bronchogenic cyst". In the post-operative course, the neurologic deficit improves, six months later a spastic paraparesia remains. The review of the literature shows that this is an exceptional observation. Intramedullary bronchogenic cysts must be regarded as similar to intramedullary enterogenic cysts. They are not teratomas but dysembryoplasiae due to failure of the ento-ectoblastic separation between the second and the third weeks of life. These cysts form a part of the "ento-ectodermal adhesion syndrome" of Prob et al. The preoperative diagnosis is possible on the association of intermittent progressive syndrome of medullary compression and dysraphic spondylotic changes. The total surgical removal of the cyst, by micro-surgical techniques, is able to preserve the neurologic evolution with an excellent result if the operation is performed before definitive neurologic deficits occur.