Spina bifida

Definition

Spina bifida (external retromedullary hydrorachis) is a congenital defect of the neural tube, the primordium of the central nervous system in the embryo.

The defect is the result of an incomplete closure of the neural tube during embryonic development which prevents the complete formation of the spinal cord. In addition, the spinal cord is sometimes exposed because of incomplete closure of vertebral bodies. One out of every 1000 newborn babies in the United States suffers from Spina bifida. The mother can reduce this risk by up to 70% by consuming sufficient amounts of folic acid prior to conception. A Spina bifida opening may be closed surgically by strips of back muscle and skin after birth or even during the pregnancy. However, surgery cannot restore normal spinal cord functions. Because in 80% of all Spina bifida cases, the opening is located at the level of the lower lumbar spine or the sacrum, most of the affected children present the same problems as paraplegics, such as permanent paralyses and intestinal and vesical dysfunctions. Generally, they must undergo urological intervention because of a diminished bladder-filling and kidney urinary flow capacity. This condition, if untreated, may cause kidney failure and premature death. Classical surgical techniques, such as sectioning bladder nerves in order to decrease intravesical pressure and increase bladder capacity, or the implantation of a neurostimulator normally rely on a dorsal approach. They cannot, however, be used in children suffering from Spina bifida because their backs have been surgically closed before or after birth.

Treatment

Pudendal pain is typically treated by decompression of the pudendal nerve. Treatment options include transgluteal or transperineal surgery or abdominal laparoscopy. While the pudendal canal is most easily accessed through the perineum, if surgery must be performed deep in the small pelvis, complete access is not possible. The same limitation exists with transgluteal surgery. In such cases, for example when the complete resection of deep endometriosis or adhesions is required, laparoscopy offers an optimal approach. Laparoscopy can also be used to treat different causes of pain associated with surgical lesions of the pudendal nerve or sacral root (such as the removal of pins, sutures or scar tissue).

Neuromodulation is another therapeutic option to treat pudendal pain. The LION procedure developed by Prof. Possover allows neuromodulation of the sacral nerve roots which contain pudendal nerve fibres. Sacral neuromodulation is the therapeutic option of choice in the following clinical cases:

  • Failure of pudendal nerve neurolysis
  • Neurological conditions, such as multiple sclerosis, Parkinson’s syndrome or diabetic neuropathy (neurological disease caused by diabetes).
  • As a follow-up procedure to pelvic surgery, when pain cannot be explained by any other cause, such as the presence of pins or sutures.

Benefits and Risks

In Spina bifida patients, laparoscopic visualization is the only surgical method to implant neuroprotheses on the pelvic nerves. Thanks to such implants, it is possible to control a hyperactive bladder, trigger miction and defecation, and provoke erections and ejaculations.