Closed spinal dysraphism reveals itself through skin abnormalities in 50% to more than 80% of cases . In the context of ARM, vascular nevus, lumbo-sacral subcutaneous mass, skin dimple and deviation of the natal cleft should be searched for. The manifestations of tethered cord have rightfully been called protean by Hoffman and coworkers in 1976 . Presenting symptoms may include motor and sensory deficits in the lower extremities, gluteal and genital region, leg or back pain (especially in older children and adults), foot deformities, leg length discrepancies and scoliosis (neuro-orthopaedic syndrome) or bladder and bowel dysfunction (Fig. 18.1). The majority of our patients had abnormal findings in one or several of these aspects (27/35), but only a minority (6/35) presented with clear-cut progressive symptoms. Most authors agree, that faecal incontinence and urinary abnormalities are more common in patients with tethered cord [2,5,20], whereas constipation is more common in ARM without tethered cord . When the type of ARM is accounted for, faecal incontinence
C Club feet. D Pressure sore due to sensory loss and trophic problems on deformed foot
Fig. 18.1 Signs and symptoms of tethered cord. A Subcutaneous mass and reddish vascular naevi point to a lumbosacral lipoma of the spinal cord. B Neuro-orthopaedic syndrome.
C Club feet. D Pressure sore due to sensory loss and trophic problems on deformed foot and constipation are similar; only urinary dysfunction remains slightly more common . This relationship and the commonly found dysplasia of the sacral bone point to maldevelopment of the respective innervation rather than secondary factors.
Abnormalities of the sacral bone and lumbar spine point to the presence of intraspinal pathology, but plain x-rays are less sensitive than ultrasound and MRI and are therefore no longer advocated as a screening procedure for intraspinal pathology. All neonates with ARM should be subjected to an ultrasound examination of the spine and spinal canal, with emphasis on conus shape and position as well as exclusion of irregular intraspinal tissue and abnormal fluid collections (syrinx, arachnoid cyst, meningocele, myelocystocele). An MRI is indicated at 3 months of age if either ultrasound or clinical signs (skin lesion, neurological or neuro-orthopaedic abnormalities of the lower extremities) are positive. In the neonatal period, the image quality of MRI is significantly reduced. From a neurosurgical standpoint, early MRI is indicated only in cases requiring early intervention, such as large myelocystoceles precluding supine positioning of the infant. Whether all infants with negative ultrasound and without clinical signs and symptoms should still be screened with MRI at about 1 year of age is not entirely clear. The sensitivity of MRI is higher than that of ultrasound, estimated at 95.6% and 86.5%, respectively. Specificity is similar, at 90.9% and 92.9%, respectively [21,22]. It appears prudent, therefore, to screen all patients using MRI, at least those patients with higher risk (e.g. cloacal and complex malformations).
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