The effects of delayed diagnosis and treatment in patients with an occult spinal dysraphism

From 1987 to 1993, 21 older individuals presented for the first time with signs and symptoms that eventually led to the diagnosis of occult spinal dysraphism. Assessment consisted of a neurological examination, urodynamic studies preoperatively and postoperatively, and spinal cord imaging. Of 21 patients 18 had an abnormal neurological examination, whereas only 15 had an abnormal urodynamic study, as judged by sphincter electromyography. Radiological imaging showed that 9 patients had a tethered cord alone, 4 each had lipomeningocele and lipoma, 2 had a bony spine abnormality and 1 each had thoracic meningocele and diastematomyelia. Of the 21 patients 19 underwent spinal surgery. Postoperatively, the neurological examination improved in 1 case (5%) and remained unchanged in 18 (95%), while urodynamic findings improved in 3 (16%), were unchanged in 11 (68%) and worsened in 5 (26%). Six patients had progressive deterioration and required secondary spinal surgery, which helped only 2 (33%). These observations confirm that older children and adults with occult spinal dysraphism are more likely to present with irreversible urological and neurological findings than younger children, and so it is imperative that a diagnosis be made and treatment be instituted as early as possible.