Spinal dysraphism. An orthopaedic syndrome in children accompanying occult forms.

Much interest has been shown in the pathology of the more gross developmental anomalies of the spinal cord and its coverings and in their clinical manifestations, most of which are not amenable to treatment. It has not been appreciated that lesser anomalies can also produce disabilities, that they can be diagnosed in life and that they can frequently be treated by surgery before the secondary effects have become severe and irreversible. Clinical experience over a number of years of the many children sent to orthopaedic clinics with various types of foot or lower limb defect led us to suspect the presence in some children of a spinal cord lesion responsible for a particular pattern of lower limb abnormalities. Such children were watched carefully and the development of their abnormalities and subsequent radiological and neurological investigations confirmed us in our belief that in a number of those children presenting at first with relatively trivial foot defects, there was an underlying spinal anomaly causing their troubles which might well be dealt with at an early stage, thus preventing further disability and perhaps improving such disability as had already occurred. We therefore decided to operate, at first upon those children whose abnormalities were beginning to increase seriously, and later, in the light of experience gained, upon all those in whom we had good reason to suspect the presence of a spinal lesion. We now believe that we can identify with certainty a syndrome associated with and diagnostic of spinal dysraphism when there is an occult lesion affecting the spinal cord. By diagnosing this condition early we can undertake spinal surgery before severe deformity and disability of the lower limbs has occurred confident that surgery will be beneficial. The clinical manifestations are for the most part orthopaedic in that it is the lower limbs and feet which usually suffer. The treatment in the first place is principally in the field of neurosurgery since the removal of the primary cause, when it is possible, demands laminectomy and exploration around the spinal cord within the dura mater. Subsequent orthopaedic care will be needed only to correct established deformity if the diagnosis has been made late. Spinal dysraphism is a term which has been applied to failure of complete development in the midline of the dorsal aspect of the embryo. The extent of this failure may be of mild, moderate or severe degree. The best known clinical form is meningomyelocoele which is an example of an overt lesion, but the lesion may not be visible on the surface nor make itself evident clinically. Spina bifida occulta is such a condition and is so commonly noted in routine x-ray investigations that it is regarded as a curiosity rather than as an entity with possible clinical implications. Ingraham and Lowrey (1943) have given an indication of the types of lesion to be found clinically, and other writers have described the particular lesions which interested them. None appears to have regarded these lesions as being of the same origin except Lichtenstein (1940), who revived the term spinal dysraphism and under this heading grouped disorders according to whether they manifested themselves as arising from cutaneous, mesodermal or neural derivatives of the dorsal median region of the developing embryo. In this communication we are concerned only with those forms of spinal dysraphism which directly or indirectly affect the spinal cord and spinal nerves and our aims are to describe these lesions, to indicate the general pattern of the associated orthopaedic syndrome and to draw attention to the value of spinal surgery as a preventive measure when undertaken early.