The basics of spinal cord tethering syndrome

Spinal cord tethering syndrome is a congenital disease that belongs to the category of neural tube developmental malformations. It may be related to the deficiency of folic acid and other nutrients during the mother’s pregnancy, and is highly prevalent in Asian populations, with a higher concentration of cases in North China, and a slightly higher number of cases in females than in males. The so-called tethering refers to the pulling and straining of the spinal cord due to various reasons, resulting in ischemia and hypoxia of the spinal cord, thus causing various clinical symptoms. Tethering of the spinal cord is often combined with other malformations, such as spinal bulge, longitudinal fissure of the spinal cord (type I and II), lumbar intra- and extradural lipomas, congenital tumors, spinal cord cavities, and scoliosis, etc. If there are no other malformations in combination, it is called simple tethering. Children may not have obvious symptoms after birth, but most of them will have localized skin abnormalities in the lumbosacral and sacrococcygeal regions, including: masses, dermatoglyphic sinus (localized abnormal skin hair), skin depressions, and dermatoglyphics (small tails), etc. It is recommended that MRI of the lumbosacral spine be performed as early as possible to rule out spinal tethering in the case of such malformations. If the spinal cord tethering cannot be released in time, the pulling of the spinal cord will become heavier and heavier as the child grows and develops, and gradually there will be abnormalities of urination and defecation, including urinary incontinence, retention, urinary weakness, incomplete urination, dryness of stools, and decrease in the number of times, etc. Some of the patients may have horseshoe foot deformity, which is specifically manifested as: heightened arches of the feet, inversion of the feet, flexion of the toes, and inability to move, etc. The spinal cord tethering leads to abnormalities in the lower limbs. The pulling of the spinal cord leads to the loss of nutrition of the lower limb nerves, which may result in the weakening of sensation in the lower limbs and difficulty in wound healing. Because of the pull on the spinal cord, patients with spinal cord tethering may experience a sudden worsening of symptoms in the event of spinal trauma, and may experience short-term urinary and fecal incontinence or even paraplegia. In a few cases of simple tethered cord, symptoms may not be apparent during adolescence, but gradually appear in adulthood, such as back and leg pain and discomfort. Patients with spinal cord tethering need to be operated on as soon as possible after detection. Clinical studies have shown that patients with spinal cord tethering who are operated on before the age of 3 years have a better outcome. Surgery is performed to remove the tethering, and in patients with lipomas and congenital tumors, it is necessary to treat them together in order to relieve the pressure of the lesion on the spinal cord. The purpose of surgery is to abort the natural course of the disease and prevent further aggravation of symptoms. For patients with existing symptoms, some of them can be relieved, but most of them need to be treated with other treatments. In general, the more severe the symptoms and the more complex the deformity, the less obvious the postoperative symptomatic relief.