Made by DATEXIS (Data Science and Text-based Information Systems) at Beuth University of Applied Sciences Berlin
Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
Funded by The Federal Ministry for Economic Affairs and Energy; Grant: 01MD19013D, Smart-MD Project, Digital Technologies
Treatment for Klippel–Feil syndrome is symptomatic and may include surgery to relieve cervical or craniocervical instability and constriction of the spinal cord, and to correct scoliosis.
Failing non-surgical therapies, spinal surgery may provide relief. Adjacent segment disease and scoliosis are two examples of common symptoms associated with Klippel–Feil syndrome, and they may be treated surgically. The three categories treated for types of spinal cord deficiencies are massive fusion of the cervical spine (Type I), the fusion of 1 or 2 vertebrae (Type II), and the presence of thoracic and lumbar spine anomalies in association with type I or type II Klippel–Feil syndrome (Type III).
Adjacent segment disease can be addressed by performing cervical disc arthroplasty using a device such as the Bryan cervical disc prosthesis.
The option of the surgery is to maintain range of motion and attenuate the rate of adjacent segment disease advancement without fusion.
Another type of arthroplasty that is becoming an alternate choice to spinal fusion is Total Disc Replacement. Total disc replacement objective is to reduce pain or eradicate it.
Spinal fusion is commonly used to correct spinal deformities such as scoliosis. Arthrodesis is the last resort in pain relieving procedures, usually when arthroplasties fail.
Body braces showed benefit in a randomised controlled trial.
The Milwaukee brace is one particular body brace that is often used to treat kyphosis in the US. Modern CAD/CAM braces are used in Europe to treat different types of kyphosis. These are much easier to wear and have better in-brace corrections than reported for the Milwaukee brace. Since there are different curve patterns (thoracic, thoracolumbar and lumbar), different types of brace are in use, with different advantages and disadvantages.
Surgery
Surgical intervention is warranted in patients who present with new onset neurological signs and symptoms or have a history of progressive neurological manifestations which can be related to this abnormality. The surgical procedure required for the effective treatment of diastematomyelia includes decompression (surgery) of neural elements and removal of bony spur. This may be accomplished with or without resection and repair of the duplicated dural sacs. Resection and repair of the duplicated dural sacs is preferred since the dural abnormality may partly contribute to the "tethering" process responsible for the symptoms of this condition.
Post-myelographic CT scanning provides individualized detailed maps that enable surgical treatment of cervical diastematomyelia, first performed in 1983.
Observation
Asymptomatic patients do not require surgical treatment. These patients should have regular neurological examinations since it is known that the condition can deteriorate. If any progression is identified, then a resection should be performed.
In Germany, a standard treatment for both Scheuermann's disease and lumbar kyphosis is the Schroth method, a system of physical therapy for scoliosis and related spinal deformities.
It involves lying supine, placing a pillow under the scapular region and posteriorly stretching the cervical spine.
Treatment is only necessary if the degree of curvature is sufficient to cause disability or if it causes emotional distress. Splinting does not routinely correct the deformity. Surgical treatments are closing wedge osteotomy, opening wedge osteotomy, and reversed wedge osteotomy. Radiographs of the fingers are useful in planning the surgical procedure. Severe clinodactyly may require soft tissue alterations to the digit such as release of skin, extensor tendon relocation, and collateral ligament advancement.
Scheuermann's disease is self-limiting after growth is complete, meaning that it generally runs its course and never presents further complication. Typically, however, once the patient is fully grown, the bones will maintain the deformity. For this reason, there are many treatment methods and options available that aim to correct the kyphosis while the spine is still growing, and especially aim to prevent it from worsening.
While there is no explanation for what causes Scheuermann's Disease, there are ways to treat it. For decades there has been a lot of controversy surrounding treatment options. For less extreme cases, manual medicine, physical therapy and/or back braces can help reverse or stop the kyphosis before it does become severe. Because the disease is often benign, and because back surgery includes many risks, surgery is usually considered a last resort for patients. In severe or extreme cases, patients may be treated through an extensive surgical procedure in an effort to prevent the disease from worsening or harming the body.
In Germany, a standard treatment for both Scheuermann's disease and lumbar kyphosis is the Schroth method, a system of specialized physical therapy for scoliosis and related spinal deformities. The method has been shown to reduce pain and decrease kyphotic angle significantly during an inpatient treatment program.
Treatment is usually conservative in nature. Patient education on lifestyle modifications, chiropractic, nonsteroidal anti-inflammatory drugs (NSAIDs), physical therapy, and osteopathic care are common forms of manual care that help manage such conditions. Other alternative therapies such as massage, trigger-point therapy, yoga and acupuncture may be of limited benefit. Surgery is occasionally performed.
Many of the treatments for cervical spondylosis have not been subjected to rigorous, controlled trials. Surgery is advocated for cervical radiculopathy in patients who have intractable pain, progressive symptoms, or weakness that fails to improve with conservative therapy. Surgical indications for cervical spondylosis with myelopathy (CSM) remain somewhat controversial, but "most clinicians recommend operative therapy over conservative therapy for moderate-to-severe myelopathy" (Baron, M.E.).
Physical therapy may be effective for restoring range of motion, flexibility and core strengthening. Decompressive therapies (i.e. manual mobilization, mechanical traction) may also help alleviate pain. However, physical therapy and osteopathy cannot "cure" the degeneration, and some people view that strong compliance with postural modification is necessary to realize maximum benefit from decompression, adjustments and flexibility rehabilitation.
It has been argued, however, that the cause of spondylosis is simply old age, and that posture modification treatment is often practiced by those who have a financial interest (such as Worker's Compensation) in proving that it is caused by work conditions and poor physical habits. Understanding anatomy is the key to conservative management of spondylosis.
Often, degenerative disc disease can be successfully treated without surgery. One or a combination of treatments such as physical therapy, anti-inflammatory medications such as nonsteroidal anti-inflammatory drugs, traction, or epidural steroid injection often provide adequate relief of troubling symptoms.
Surgery may be recommended if the conservative treatment options do not provide relief within two to three months. If leg or back pain limits normal activity, if there is weakness or numbness in the legs, if it is difficult to walk or stand, or if medication or physical therapy are ineffective, surgery may be necessary, most often spinal fusion. There are many surgical options for the treatment of degenerative disc disease, including anterior and posterior approaches. The most common surgical treatments include:
New treatments are emerging that are still in the beginning clinical trial phases. Glucosamine injections may offer pain relief for some without precluding the use of more aggressive treatment options . In the US, artificial disc replacement is viewed cautiously as a possible alternative to fusion in carefully selected patients, yet it is widely used in a broader range of cases in Europe, where multi-level disc replacement of the cervical and lumbar spine is common . Adult stem cell therapies for disc regeneration are in their infancy. Investigation into mesenchymal stem cell therapy knife-less fusion of vertebrae in the United States began in 2006.
Non surgical treatments include steroid injections in the lower back or radiofrequency sensory ablation. Physical therapy interventions are also helpful in early cases and are focused around mobilization, neural stretching, and core strengthening exercises. Surgical intervention is usually a last resort if all conservative methods fail. It can be treated surgically with posterolateral fusion or resection of the transitional articulation.
A study measured outcome from surgery of 49 cases of scoliosis and kyphoscoliosis. Of this sample, 36 patients were monitored for a period of 8 years.
- 23% - excellent condition
- 29% - good condition
- 34% - satisfactory
- 14% - bad
Bad refers to cases where the surgery failed to address the disease and the patient either had to undergo a revision surgery or continues to suffer from a poor quality of life as before surgery.
It should be noted that typically post-surgery complications range up to 5% involving all major and minor complications when measured within one year of surgery. However, there may be a progressive decline in patient’s condition after a few years.
In another study that evaluated surgical treatment of kyphoscoliosis and scoliosis due to congenital reasons, 91% of surgeries were found to be successful and met their intended objectives for the two-year follow-up period after surgery. The sample consisted of 23 patients of whom 17 were male and 6 were female, with an average age of 27 years, ranging from 13 to 61 years. The most popular type of surgeries for spinal correction includes pedicle subtraction osteotomy (PSO) and posterior vertebral column resection (pVCR).
Another study which focused on elderly patients found that the rate of complications was much higher for a sample population of 72 cases with mean age of 60.7 years. The rate of complications was as high as 22% in the entire sample. The study points that in the case of elderly patients, surgery should only be considered when there is no other option left; the disease is in progression stage, and the quality of life has degraded to an extent where conservative treatments can no longer help with pain.
While there are many surgical approaches for spinal deformity correction including anterior only, posterior only, anterior-posterior, the techniques that are most popular nowadays include the posterior only VCR or pVCR. One of the studies which analyze pVCR technique also noted the benefit of using a technique called NMEP monitoring in assisting the surgeon avoid any neurological complications while performing a spine surgery.
In conclusion, the decision to undergo a corrective spine surgery is a complex one but sometimes becomes necessary when the quality of life has degraded to such an extent that potential benefits outweigh the risks. No surgery is devoid of risks but by carefully assessing factors such as the skills and experience of the surgical team, previous record or history of outcomes, and the techniques that are used for spine surgery, a patient along with his or her doctor can certainly help in achieving a successful outcome.
As studies are repeatedly pointing out, the success rates for spinal surgeries have improved so much so that the risks rates can now be comparable to other types of surgeries. These success rates also tend to be higher at a younger age when compared to the elderly age.
Current surgical procedures used to treat spondylosis aim to alleviate the signs and symptoms of the disease by decreasing pressure in the spinal canal (decompression surgery) and/or by controlling spine movement (fusion surgery).
Decompression surgery: The vertebral column can be operated on from both an anterior and posterior approach. The approach varies depending on the site and cause of root compression. Commonly, osteophytes and portions of intervertebral disc are removed.
Fusion surgery: Performed when there is evidence of spinal instability or mal-alignment. Use of instrumentation (such as pedicle screws) in fusion surgeries varies across studies.
Scheuermann's disease can be successfully corrected with surgical procedures, almost all of which include spinal fusion and hardware instrumentation, i.e., rods, pedicle screws, etc. While many patients are typically interested in getting surgery for their correction, it is important to realize the surgery aims to reduce pain, and not cosmetic defect. As always, surgical intervention should be used as a last resort once conservative treatment fails or the patient's health is in imminent danger as any surgical procedure is not without risk; however, the chances of complication are relatively low and the surgeries are often successful.
One of the largest debates surrounding Scheuermann's disease correction is the use of very different correction procedures. There are different techniques to correct kyphosis; usually the differences being posterior/anterior entry or posterior entry (rear) only. The classic surgical procedure partially entails entering two titanium rods, each roughly one and a half feet long (depending on the size of the khyphosis), into the back on either side of the spine. Eight titanium screws and hardware are drilled through the bone and secure the rods onto either side of the spine. On the internal-facing side of the spine, ligaments (which can be too short, pulling the spine into the general shape of kyphosis) must be surgically cut or released, not only stopping part of the cause of the kyphosis, but also allowing the titanium rods to pull the spine into a more natural position. Normally, the damaged discs between the troubled vertebrae (wedged vertebrae) are removed and replaced with bone grafting from the hip or other parts of the vertebrae, which once healed or 'fused' will solidify. The titanium instrumentation holds everything in place during healing and is not necessary once fusion completes. Recovery begins in the hospital and depending on whether the operation is one- or two-stage the patient can expect to be in hospital for minimum of a week, possibly longer depending on recovery.
They will then often be required to wear a brace for several months to ensure the spine heals correctly leaving the patient with the correct posture. The titanium instrumentation can stay in the body permanently, or be removed years later. Patients undergoing surgery for Scheuermann's disease often need physical therapy to manage pain and mobility, however their range of motion is generally not limited very much. Recovery from kyphosis correction surgery can be very long; typically patients are not allowed to lift anything above 5 or 10 pounds for 6 months to a year. Many are out of work for at least 6 months. However, once the fusion is solidified, most patients can return to their usual lifestyle within one to two years.
If a contracture is less than 30 degrees, it may not interfere with normal functioning. The common treatment is splinting and occupational therapy. Surgery is the last option for most cases as the result may not be satisfactory.
Manipulative physiotherapy, therapeutic exercises and chiropractic manipulative therapy shows beneficial results for decreasing pain and increasing spinal range of motion. As areas of the spine and tendons can become inflamed NSAIDs such as ibuprofen and Naproxen can be helpful in both relieving pain and inflammation associated with DISH. It is hoped that by minimizing inflammation in these areas, further calcification of tendons and ligaments of the spine leading to bony outgrowths (enthesophytes) will be prevented, although causative factors are still unknown.
The heterogeneity of the Klippel–Feil syndrome has made it difficult to outline the diagnosis as well as the prognosis classes for this disease. Because of this, it has complicated the exact explanation of the genetic cause of the syndrome.
The prognosis for most individuals with KFS is good if the disorder is treated early on and appropriately. Activities that can injure the neck should be avoided, as it may contribute to further damage. Other diseases associated with the syndrome can be fatal if not treated, or if found too late to be treatable.
The Boston brace is a plastic exterior that can be made with a small amount of lordosis to minimize stresses on discs that have experienced herniated discs.
In the case where Ehlers Danlos syndrome (EDS) is responsible, being properly fitted with a customized brace may be a solution to avoid strain and limit the frequency of instability.
Since lumbar hyperlordosis is usually caused by habitual poor posture, rather than by an inherent physical defect like scoliosis or hyperkyphosis, it can be reversed. This can be accomplished by stretching the lower back, hip-flexors, hamstring muscles, and strengthening abdominal muscles.Dancers should ensure that they don't strain themselves during dance rehearsals and performances. To help with lifts, the concept of isometric contraction, during which the length of muscle remains the same during contraction, is important for stability and posture.
Lumbar hyperlordosis may be treated by strengthening the hip extensors on the back of the thighs, and by stretching the hip flexors on the front of the thighs.
Only the muscles on the front and on the back of the thighs can rotate the pelvis forward or backward while in a standing position because they can discharge the force on the ground through the legs and feet. Abdominal muscles and erector spinae can't discharge force on an anchor point while standing, unless one is holding his hands somewhere, hence their function will be to flex or extend the torso, not the hip.
Back hyper-extensions on a Roman chair or inflatable ball will strengthen all the posterior chain and will treat hyperlordosis. So too will stiff legged deadlifts and supine hip lifts and any other similar movement strengthening the posterior chain "without involving the hip flexors" in the front of the thighs. Abdominal exercises could be avoided altogether if they stimulate too much the psoas and the other hip flexors.
Controversy regarding the degree to which manipulative therapy can help a patient still exists. If therapeutic measures reduce symptoms, but not the measurable degree of lordotic curvature, this could be viewed as a successful outcome of treatment, though based solely on subjective data. The presence of measurable abnormality does not automatically equate with a level of reported symptoms.
The management of true cauda equina syndrome frequently involves surgical decompression. When cauda equina syndrome is caused by a herniated disk early surgical decompression is recommended.
Cauda equina syndrome of sudden onset is regarded as a medical/surgical emergency. Surgical decompression by means of laminectomy or other approaches may be undertaken within 6, 24 or 48 hours of symptoms developing if a compressive lesion, e.g., ruptured disc, epidural abscess, tumour or haematoma is demonstrated. Early treatment may significantly improve the chance that long-term neurological damage will be avoided.
Surgery may be required to remove blood, bone fragments, a tumor or tumors, a herniated disc or an abnormal bone growth. If the tumor cannot be removed surgically and it is malignant then radiotherapy may be used as an alternative to relieve pressure, with spinal neoplasms chemotherapy can also be used. If the syndrome is due to an inflammatory condition e.g., ankylosing spondylitis, anti-inflammatory, including steroids can be used as an effective treatment. If a bacterial infection is the cause then an appropriate course of antibiotics can be used to treat it.
Cauda equina syndrome can occur during pregnancy due to lumbar disc herniation; age of mother increases the risk. Surgery can still be performed and the pregnancy does not adversely affect treatment. Treatment for those with cauda equina can and should be carried out at any time during pregnancy.
Lifestyle issues may need to be addressed post - treatment. Issues could include the patients need for physiotherapy and occupational therapy due to lower limb dysfunction. Obesity might also need to be tackled.
Pregnant mothers are advised to take folic acid supplements to reduce risk of iniencephaly by up to 70%. Pregnant mothers are also advised not to take antiepileptic drugs, diuretics, antihistamines, and sulfa drugs, all of which have been associated with increased risk for neural tube defects.
Physical therapy can be somewhat useful for patient’s recovery from surgery. The main focus of rehabilitation is centered on controlling the bladder and bowel functions and decreasing muscle weakness in the lower extremities.
The procedure of spine shortening via vertebral osteotomy (SSVO) for TCS is a surgical technique that avoids the complication with revision tethering. In this research a lateral retropleural approach was used for SSVO in recurrent TCS in a 21-year-old female. The patient presented with progressive lower extremity weakness, bowel and bladder incontinence, and back pain in the setting of childhood repair of mylomeningocele and two previous detethering procedures. The research on performing SSVO in this patient allowed the scientists to conclude that SSVO via lateral retropleural approach is a good treatment for the recurrence of TCS. This procedure is minimally invasive compared to the posterior approach which gives the advantages of having direct access to the vertebral body and disc while avoiding the need to have an operation near the spinal cord but further research is still needed.
Because neurological deficits are generally irreversible, early surgery is recommended when symptoms begin to worsen. In children, early surgery is recommended to prevent further neurological deterioration, including but not limited to chronic urinary incontinence.
In adults, surgery to detether (free) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms. Although detethering is the common surgical approach to TCS, another surgical option for adults is a spine-shortening vertebral osteotomy. A vertebral osteotomy aims to indirectly relieve the excess tension on the spinal cord by removing a portion of the spine, shortening it. This procedure offers a unique benefit in that the spinal cord remains fixated to the spine, preventing retethering and spinal cord injury as possible surgical complications. However, its complexity and limited “track record” presently keeps vertebral osteotomies reserved as an option for patients who have failed in preventing retethering after detethering procedure(s).
Other treatment is symptomatic and supportive. Medications such as NSAIDs, opiates, synthetic opiates, COX-2 inhibitors, and off-label applications of tricyclic antidepressants combined with anti-seizure compounds have yet to prove they are of value in treatment of this affliction's pain manifestations. There is anecdotal evidence that TENS units may benefit some patients.
Treatment may be needed in adults who, while previously asymptomatic, begin to experience pain, lower back degeneration, scoliosis, neck and upper back problems and bladder control issues. Surgery on adults with minimal symptoms is somewhat controversial. For example, a website from the Columbia University Department of Neurosurgery says, "For the child that has reached adult height with minimal if any symptoms, some neurosurgeons would advocate careful observation only." However, surgery for those who have worsening symptoms is less controversial. If the only abnormality is a thickened, shortened filum, then a limited lumbosacral laminectomy with division of the filum may be sufficient to relieve the symptoms.
This syndrome was first noticed in the late 19th century. While information has been available for years, little widespread blind research has been done. More research has been called for, and doctors have conducted many studies with good results. There is a low morbidity rate, and no complications have been documented other than those typical of any type of back surgery. The association of this condition with others has been noticed, and needs further research to understand such relationships. TCS is causally linked to Chiari malformation and any affirmative diagnosis of TCS must be followed by screening for Chiari's several degrees. TCS may also be related to Ehlers-Danlos syndrome, or Klippel-Feil syndrome, which should also be screened for upon a positive TCS diagnosis. Spinal compression and the resulting relief is a known issue with this disorder. Like with the early-onset form, this disease form is linked to the Arnold-Chiari malformation, in which the brain is pulled or lowers into the top of the spine.
Treatment for spondylolysis ranges from bracing, activity restriction, extension exercises, flexion exercises and deep abdominal strengthening, that is administered through physical therapy. The duration of physical therapy a patient receives varies upon the severity of spondylolysis, however typically ranges from three to six months. The goal of physical therapy is to minimize movement at the unstable defect of the pars interarticularis. Once a patient completes physical therapy, and displays no symptoms or inflammation in the lower back, they are cleared to continue with daily or athletic activities. However, a patient may need to maintain a variety of rehabilitation techniques after physical therapy to prevent the recurrence of spondylolysis.
Activity restriction of spondylolysis is advised for a short period of time once the patient becomes symptomatic, followed by a guided physical therapy program. Once spondylolysis has been diagnosed, treatment often consists of a short rest period of two to three days, followed by a physical therapy program. There should be restriction of heavy lifting, excessive bending, twisting and avoidance of any work, recreational activities or participation in sport that causes stress to the lumbar spine. Activity restriction can help eliminate and control a patient's symptoms so they are able to resume their normal activities. Activity restriction is most commonly used in conjunction with other rehabilitation techniques including bracing.
Because of the unclear pathogenesis and pathophysiology of Tarlov cysts, there is no consensus on the optimal treatment of symptomatic sacral perineural cysts. Patients often choose to pursue treatment when the progression of neurological deficits seriously impacts their quality of life.
Since cysts are innervated, microfenestration and surgical sleeving of the cysts to diminish the amount of accumulated cerebrospinal fluid and decrease compression of the spine and spinal nerves has been successful in a number of patients. The cysts are carefully separated enough from surrounding tissue to be wrapped with fatty tissue or pericardial biomaterial to excise the fluid from the cyst. If the cyst does not drain spontaneously, then it is drained and patched using a biosynthetic dural patch.
The use of this technique is done in the U.S. and is spreading in Europe but recovery is generally extensive. Microfenestration alone has been done with some success in Asia.
A biopolymer plate is also being used experimentally to strengthen a sacrum thinned by cystic erosion by Dr. Frank Feigenbaum.
The risks of CSF leakage are higher on patients that have bilateral cysts on the same spinal level or clusters of cysts along multiple vertebrae, but immediate recognition of the leakage and repair can mitigate that risk.
Various treatment methods have been tried in the past, including the extraction of cerebrospinal fluids from the cyst, fibrin glue injection and the complete or partial removal of cyst. Epidurals can provide temporary relief but are not generally recommended as they can cause cysts to enlarge. Extraction of fluid can provide limited or no relief depending on rate the cysts refill and the need to repeat the procedure. Removal of the cyst results in irreversible damage to the intersecting spinal nerve.
Although fibrin-glue therapy initially had been thought to be a promising therapy in the treatment of these cysts, there have been multiple problems associated with the fibrin glue therapy including seepage of fibrin. It is no longer recommended for use at present by the Health Department in some countries and neurosurgeons previously performing the procedures.
Nevertheless, all types of surgical treatment pose common risks, including neurological deficits, infection and inflammation, spinal headache, urinary disturbances, and leakage of cerebrospinal fluids.
Here is an article for treatment of meningeal diverticulum. Feigenbaum F1, Henderson FC. Giant sacral meningeal diverticula: surgical implications of the "thecal tip" sign. Report of two cases. J Neurosurg Spine. 2006 Nov;5(5):443-6.