CLINICAL FEATURES

Disc disease (herniation and/or degeneration) may result in several overlapping clinical syndromes: (1) mild herniation without nerve root compression; (2) herniation with nerve root compression; (3) cauda equina syndrome; (4) chronic degenerative disease with or without leg symptoms; and (5) spinal stenosis.

The onset of symptoms from disc herniation is variable. One specific traumatic episode may produce symptoms, but because of the progressive nature of the disease process in the disc, the symptoms usually develop gradually as the disc slowly changes. (How much herniation is required to produce symptoms is unknown, but “bulging” of the disc commonly reported on magnetic resonance imaging [MRI] studies is probably not sufficient.) The most common complaint is low back pain, which is often deep and aching in nature. This pain, which is sometimes referred to as axial, is usually one sided and may be aggravated by activity and relieved by rest. Coughing, sneezing, or other actions that increase the stress on the disc tend to intensify the pain. The back pain is often localized near the disc and may be referred to the iliac crest or buttock. It is may be the result of stretching of the anulus by the expanding, protruding disc. Radicular pain occurs when the disc protrudes far enough to press on the adjacent nerve root. Nerve root pain is usually quite intense. Often, if the disc herniates completely (extrudes), the low back pain may be relieved because the tension on the anulus is gone, but the leg pain intensifies.

Axial low back pain may occur in combination with radiating pain, or the two pain patterns may occur separately. Radicular pain characteristically spreads over the buttock and passes down the posterior or posterolateral aspect of the thigh and calf and may even spread onto the foot. Both types of pain usually improve with bed rest. If little or no relief of pain occurs with rest, inorganic causes should be considered. Relentless pain that is not relieved or may even be aggravated by recumbency suggests a spinal cord tumor.

Paresthesias in the form of numbness and tingling are common and are usually more marked in the distal portion of the extremity. They may follow a specific dermatome pattern (Fig. 8-8).

FIG. 8-8 Dermatomes of the lower extremity.

Examination often reveals restriction of low back motion. Bending toward the affected side frequently exacerbates the pain. Variable degrees of local tenderness and muscle guarding are present. In an attempt to relieve pressure or tension on the nerve root, the patient may list or bend away from the painful side and stand with the affected hip and knee slightly flexed. A characteristic clinical picture may be present, depending on the level of nerve root involvement (Table 8-2). The sensory examination may reveal diminished sensation along the affected dermatome, although the sensory examination is usually not very helpful. The various tests measuring sciatic nerve root tension are frequently positive if herniation is causing nerve compression (Fig. 8-9).

FIG. 8-9 Testing for nerve root tension. The foot is slowly raised, keeping the knee straight, until sciatic pain is produced. A positive test includes the following: (1) pain produced before 70 degrees is reached, (2) aggravation of the pain by dorsiflexion of the ankle, and (3) relief of the pain by flexion of the knee. If the patient can extend the leg to 90 degrees while sitting but the same test is impossible in the supine position, pain magnification should be suspected. If raising the leg on the contralateral side reproduces pain in the affected leg, disc herniation should be strongly suspected.

SPECIAL STUDIES

The diagnosis usually becomes apparent on the basis of the history and physical examination. Early disc herniation may be difficult to differentiate from other causes of back pain, however.

FIG. 8-10 Abnormal lumbar myelogram revealing a large extradural defect (arrow) in the dye column that is consistent with disc herniation.

NOTE: The number of symptomatic conditions discovered by special studies but not suspected clinically is very small. Tests such as MRI and EMG should only be performed as adjuncts to the clinical assessment.

TREATMENT

The initial treatment is always conservative, and the majority of patients respond well. Treatment is based on the symptoms of the patient and not on any imaging study. Extended periods of inactivity are no longer recommended for most low back disorders, but a short period of bed rest (5 to 10 days) may be very helpful in the treatment of acute disc herniation, mainly if radicular leg pain caused by nerve root compression is present. This is followed by a careful exercise program. While the patient is in bed, the hips and knees are kept moderately flexed. Lying on the abdomen, which increases the lumbar lordosis, is avoided. Hip flexion and pelvic tilt exercises are begun within the limits of pain (Fig. 8-11). Nonsteroidal anti-inflammatory drugs (NSAIDs), analgesics, and moist heat are used as necessary. Narcotics may even be required in cases of nerve root compression and intense leg pain. If improvement occurs, which it does in the majority of cases, gradual resumption of activity is allowed, and the exercise program is expanded. Physical therapy is often useful, mainly for the exercise program. Modalities such as deep heat and massage can be added for their “hands-on” appeal. A lumbosacral corset may be temporarily used. (There is no evidence that continued use of any brace promotes back weakness, however, especially if the patient adheres to an exercise program.) Recurrences are prevented by a proper exercise program and the avoidance of stress to the lower part of the back (Fig. 8-12).

FIG. 8-11 Low back exercises. A, Pelvic tilt performed to decrease the lumbar lordosis and raise the anterior aspect of the pelvis. The small of the back is pressed to the floor, and the abdominal and buttock muscles are tightened. B, Hip flexion, performed to stretch the tight posterior spinal musculature and unload the posterior disc. Each knee is drawn up and pulled firmly to the chest several times and held for 10 to 20 seconds. The exercise is then repeated with both knees. After the acute pain has subsided, the remainder of the exercises are performed. C, Hamstring stretching exercises. D, Hip flexor stretching exercises. E, Quadriceps strengthening and heel cord stretching exercise. F, Abdominal strengthening exercise (sit-ups, which may be partial). All exercises are performed on a carpeted floor and should be repeated in sets of 5 to 10 at least three times daily.

FIG. 8-12 General postural instructions. Patients should be instructed to do the following. A, Bend the knees and hips, and keep the back straight when lifting. B, Hold objects close to the body when carrying. C, Place one foot on a stool when standing. D, Keep the knees higher than the hips when sitting, and keep the back straight when standing by “tucking in” the abdomen and tightening the buttocks to decrease swayback. In addition, patients should avoid high-heeled shoes and sleeping on the abdomen, activities that increase lordosis.

Caudal or epidural cortisone injections are often very helpful. They may help relieve residual radicular pain. They tend not to relieve axial spine pain and may have no effect on acute leg pain if there is persistent nerve root compression from herniated or extruded disc material.

The prognosis for full recovery is excellent, and the majority of patients with disc herniation will improve with conservative treatment. Mild axial spine pain may recur on a sporadic basis indefinitely, however. Most of the time, this can be managed with mild analgesics and physical therapy. Motivation and reassurance are important. Keeping the patient active and comfortable can be difficult and challenging. What happens to the disc material that has herniated is unknown. It is theorized that it may act as a foreign body and be invaded by granulation tissue. Ultimately, the material may be absorbed or transformed into scar tissue. Larger herniations may actually regress the most.

Disc removal is reserved for those patients with major or progressing neurologic deficits or those with intractable leg pain who fail to respond to conservative management for at least 6 weeks. Of those patients with radicular leg pain documented by radiographic studies, fewer than 5% require surgery to relieve their symptoms. The patient must understand that disc removal is performed to relieve leg pain and that it has no effect on axial spine pain.

There are several ways of removing disc tissue, but the results of surgery are influenced more by patient selection that by the manner that the disc is removed. With a positive history, physical findings, myelography (or similar study), and EMG, there is a 90% to 95% success rate for surgery. In the absence of one or more of these, the cure rate for disc removal by any means declines considerably. Patients must also be cautioned that although their leg pain will usually disappear postoperatively, some mild intermittent low back pain may persist.

SUMMARY

Surgery is effective in most cases, but selection is the key. When advising treatment, the following well-known facts should always be kept in mind:

CLINICAL FEATURES

The history is highly suggestive. Neurogenic claudication is a hallmark symptom. Low back pain, motor weakness, leg cramping (pseudoclaudication), and a sensation of “poor circulation” in the extremities are typical. The patient is usually older than 50 years of age. The symptoms are characteristically aggravated by walking and by extension of the lumbar spine. Improvement usually occurs with rest or flexion of the back. While grocery shopping, the patient often leans forward onto the cart to relieve the leg pain. These symptoms are often misinterpreted as being vascular in origin. Vascular symptoms will improve with rest, however, whether the patient is standing or sitting, but the patient with neurogenic claudication usually requires sitting because the forward flexed position increases the cross-sectional area of the bony canal, thereby relieving the symptoms. Sphincter disturbances and muscle atrophy rarely may be present. Physical findings typical of disc herniation are usually absent. Neurologic findings are also frequently minimal, and results of a straight-leg–raising test are usually normal. Pain on extension of the lumbar spine may be severe. The vascular examination is usually normal, but because vascular disease and stenosis occur in similar age groups and can produce similar symptoms, vascular studies may be necessary in some cases to differentiate the two conditions.

Roentgenographic examination of the lumbar spine usually reveals degenerative changes throughout the lower part of the back. EMG and myelography may help localize the disorder, and CT scanning is frequently diagnostic (Fig. 8-14). MRI is also helpful.

FIG. 8-14 Normal (A) and abnormal (B) computed tomographic scans of the lumbar spine. Marked narrowing (arrow) is present in B because of hypertrophic spurring.

TREATMENT

Nonsurgical treatment is the mainstay of care for the patient with degenerative stenosis. Treatment is directed at reducing the lumbar lordosis and promoting lumbosacral flexion. A light corset is sometimes beneficial. Anti-inflammatory medication may be prescribed, but unfortunately, the older patient who develops this conditon often cannot tolerate NSAIDs, and simple analgesics may be more suitable. Hip flexion and abdominal strengthening exercises may also be helpful. Periodic epidural pain blocks can relieve the leg symptoms.

The long-term prognosis is generally good. Serious neurologic sequelae are unusual. Patients who fail to improve with nonsurgical care may need to be referred to a surgeon who specializes in spine disorders.

CLINICAL FEATURES

Spondylolysis may be symptomatic even without spondylolisthesis, and both conditions may be associated with lumbar disc herniation. The disorder is often asymptomatic, however, and is frequently discovered incidentally in adults on roentgenograms taken for other purposes. When it is seen on roentgenograms taken as a part of a routine evaluation for back pain, it is often difficult to determine whether or not it is playing any role in the patient’s complaints.

Symptoms from spondylolisthesis may begin gradually in the second or third decade. Low back pain, sometimes radiating into the buttocks, occurs with activity and is relieved by rest. If the lesion is acute, symptoms of nerve root irritation may also be present, along with radiation of the pain into the extremities. These symptoms often progress in severity, especially in the teenager.

Examination may reveal guarding of the lower part of the back and “spasm” of the paraspinal muscles, especially in the adolescent. Many children have no pain, however, but present with postural deformity or an abnormal gait pattern resulting from hamstring tightness. With moderate forward slippage, the lumbar lordosis appears increased, and the buttocks may appear more prominent. A palpable “step-off” in the spinous processes of the lumbar spine may be present. There may be tenderness in the affected area and hamstring tightness. Neurologic deficits are rare.

Roentgenograms reveal the typical findings of a defect in the pars interarticularis on both sides, which may be accompanied by forward slippage (Fig. 8-17). Unilateral defects are unusual. The classic findings of periosteal new bone present in stress fractures of long bones are rarely seen in the spine. A bone scan may be positive if the lesion is “acute” in the adolescent. MRI may be indicated in cases of negative bone scan to rule out other causes of pain. CT is also helpful to assess healing potential.

FIG. 8-17 Spondylolisthesis of the lumbosacral junction.

TREATMENT

In the adolescent, the goal of treatment is to achieve healing of the stress fracture and if that is not possible, to have a painless nonunion. It is helpful to determine if the lesion is simply a stress reaction that has not yet progressed to a complete fracture. It is also important to assess healing and the status of the lesion, whether acute or chronic. If the lesion is acute and no slip is present, the patient may benefit from prolonged bracing and restricted activities for several months in an attempt to encourage bony healing before nonunion can develop, which could allow slippage to occur. If the scan is cold and chronic with several millimeters of separation, then the lesion probably will never heal, especially if healing has not occurred by 6 months. In this case, such extreme limitations on the patient’s activities would not be helpful. Then, the treatment is similar to that for an adult.

Management in the adult is usually conservative and consists of rest, a careful exercise program, hamstring stretching, weight loss, anti-inflammatory medication, and the intermittent use of a lumbosacral orthosis. A change in occupation or work habits is sometimes indicated. The spondylolisthesis may increase in degree, but this is a gradual process and usually ceases by maturity. Further slippage in adulthood is rare. Progressive symptoms or intractable pain occasionally warrants surgical intervention. Decompression with spinal fusion is usually performed.

EVALUATION

The treating physician is often required to give an opinion regarding the etiology of musculoskeletal conditions involved in workers’ compensation, so obtaining an accurate physical examination and history is especially important to determine whether an injury actually occurred or if the pain simply developed during or after an activity. If pain began some time later, it should be recorded how much time passed between the event or work activity and the onset of symptoms. The pain may be referred to one or both legs, but it is usually not truly radicular in nature. Frequently, a description of the location and nature of the pain changes slightly from visit to visit.

Physical findings are often nonspecific. There is usually generalized low back tenderness and diminished range of motion. Sometimes, the physical findings are “nonanatomic” in nature. Waddell signs are often cited when nonorganic physical findings are present (Table 8-3). There is usually no neurologic deficit, and sciatic tension tests are generally normal but may be difficult to assess.

Table 8-3 Waddell Tests

SPECIAL STUDIES

All of the anatomic structures of the lower part of the back (discs, ligaments, facet joints, bone, and muscle) can be the source of pain. The intervertebral disc is thought by many to be the source of most low back pain. Unfortunately, as many as 35% of asymptomatic adults will have abnormal findings on myelography, CT, or MRI that are usually related to the disc. This makes evaluation of this problem difficult, and it is felt by many that in the majority of cases, the exact underlying pathology probably cannot be determined and the condition is simply called “idiopathic.” Initially, a routine roentgenographic examination should be performed in 2 to 4 weeks if the patient does not improve, mainly to rule out any serious disorder. Further studies (EMG, CT, MRI, bone scan, myelography) should be performed only as adjuncts to the physical examination and history. The yield of clinically useful information from these studies is often poor. Subjecting patients to further extensive testing in a search for the exact etiology of their pain is likely to be futile, sometimes painful, and always costly. Diagnosing a spinal disorder solely on the basis of any of these tests should be avoided. It is rare that these special tests clearly demonstrate a source for the pain when it is not suspected clinically. In addition, any treatment (e.g., surgery) based solely on a special study will often fail. In general, myelography and other special studies should be used only under the following circumstances: (1) if surgical disc removal is contemplated for intractable leg pain or a serious neurologic deficit, or (2) if other serious spinal abnormality is suspected.

TREATMENT AND PROGNOSIS

Considerable controversy exists regarding the treatment of this problem. Historically, the disorder has always been resistant to traditional medical care. Thus, there has been a proliferation of rehabilitation services, pain centers, work-hardening programs, surgical procedures, chiropractic care, and physical therapy services. Patients often prefer this type of “hands-on” treatment, but no scientific proof exists that any of these treatments is any better than nature’s own. And although orthopedic and neurologic surgeons are commonly called on to assess and treat this condition, in fewer than 1% of these cases is surgery ever indicated (usually for leg pain associated with severe disc herniation), and not for at least 6 to 8 weeks. Surgery may even become a negative factor for returning the patient to work in that it gives validity to the injury.

Fortunately, the natural history of most back problems is that they improve and the end result of conservative management is usually good. Unfortunately, the longer the patient is away from work with benign low back pain, the less likely it is that the patient will ever return to gainful employment, and after 6 months of not working, the chance that the patient will return to any work is minimal. Thus, for a number of reasons, the trend for managing this condition has changed. There has been a de-emphasis on the initial rest period and more encouragement to early exercise and returning to work on a limited basis. This trend has developed largely because of the low success rate with prolonged bed rest and the belief that it causes a deterioration of the musculoskeletal and cardiopulmonary systems (deconditioning). It has also been shown that patients who begin exercise programs quickly after minor low back injuries have less pain and are disabled for shorter periods of time. (Only the patient with nerve root impingement because of disc herniation may require more rest.) The following approach may be helpful:

It is important for the treating physician to remain the patient’s advocate but to be objective at the same time, a situation that often results in conflict. Patients should be made to understand that the pain may recur if they resume light work, but it is usually not as severe as it was initially and does not mean any damage is occurring. It should not require further time off from work. The patient should continue the exercise program and practice good body mechanics. (A formal education program may be necessary.) Active participation by the patient and compliance are extremely important.

If no improvement has occurred after 4 to 6 weeks, referral to a specialist is indicated. Psychosocial factors and work-related conditions may also need investigation. The attitude of the patient to the job and involvement of the employer also play important roles in the length of disability. Workers who like their jobs and employers who promote a good working environment are more likely to have a mutually satisfactory relationship.

CLINICAL FEATURES

Only axial dorsal spine pain occurs in most cases. With central herniation and myelopathy, however, gradually increasing motor weakness in the lower extremities becomes apparent. Sphincter control may be lost, and diffuse numbness is common. Sensory testing may help determine the level of involvement. Examination usually reveals limited motion in the dorsal spine. Major neurologic deficits may be present in the lower extremities (spasticity, clonus). Roentgenographic examination often reveals calcification, narrowing, and spondylosis in the dorsal spine. Myelography may reveal a complete block in central lesions. MRI is often needed.

TREATMENT

Treatment is symptomatic for the typical case, with thoracic pain caused by degenerative disc disease. Symptoms in most cases with no cord compression resolve without sequelae. The treatment is surgical in those with cord compression caused by disc rupture, and immediate referral is indicated. Residual neurologic deficits are not unusual, in spite of surgical decompression.

CLINICAL FEATURES OF IDIOPATHIC SCOLIOSIS

Genetic or idiopathic scoliosis usually appears clinically between the ages of 10 to 13 years, but may be seen at any age. By definition, the curve must be greater than 10 degrees. (This has been used historically because 10 degrees is the limit that can be detected by clinical examination.) It is six times more common in females, and serious curvatures are also more frequent in females. Right thoracic curves are the most common. In young people, the disease is usually asymptomatic, and subjective complaints are absent. (Pain should suggest tumor or other disorder.) Many cases are diagnosed by nurses in screening clinics or by other family members. Idiopathic scoliosis is sometimes classified according to age of onset (infantile, juvenile, adolescent) or by curve pattern. The adolescent type is most common. Prevalence is 1% to 3% of the general population.

The diagnosis is usually made on routine physical examination. Attention should be focused on the problem in all children, but especially in those between the ages of 10 to 14 years, when spinal growth is most rapid. For the examination, the patient should be undressed to the waist or wear a bathing suit, and a routine should be followed. The shoulders and iliac crests are inspected to determine whether they are level. The scapulae, rib cage, and flanks are then observed for symmetry. The spinous processes are palpated to determine their alignment. The patient is then asked to bend symmetrically forward at the waist with the arms hanging free (Fig. 8-19). Observation from the back or front will detect the spinal rotation in the form of a rib hump or abnormal paraspinal muscular prominence. Height measurements are taken initially and at all follow-up visits to gauge the growth rate of the patient and assess the risk of rapid progression of the curve. Additional data should be obtained regarding skeletal and sexual maturity (onset of menses, etc.).

FIG. 8-19 A, Scoliosis with rib prominence resulting from vertebral rotation is best exhibited on forward bending (the Adams position). B, Cross-section of the chest showing rib distortion caused by vertebral rotation.

The diagnosis is confirmed, and the degree of curvature is measured by a standing roentgenogram of the spine (Fig. 8-20). There is no other method of determining the severity of the curve, and a patient should never leave the office without an accurate roentgenographic measurement of the curvature. The roentgenogram may have to be repeated at intervals to determine whether or not the curve is progressive. Breasts and gonads should be shielded when films are done. The degree of skeletal maturity can be determined by assessing the status of the iliac apophysis using the Risser sign (Fig. 8-21).

FIG. 8-20 Cobb method of measuring the severity of a curve. The upper and lower end vertebrae are identified. The upper end vertebra is the highest one whose superior border converges toward the concavity of the curve, and the lower end vertebra is the one whose inferior border converges toward the concavity. Lines are drawn along these borders, and the curve is measured directly (a) or geometrically (b).

FIG. 8-21 The Risser sign. Ossification of the iliac crest begins at the anterior superior iliac spine and progresses to the sacroiliac joint. Risser’s classification divides the crest into quadrants, and maturity is graded according to the number of ossified quadrants. A Risser sign I is 25%, for example. Full maturation starting at one takes about 2 years.

MRI is usually not indicated unless there is some evidence for a secondary cause of the curve: (1) significant pain, (2) abnormal neurologic symptoms or findings, (3) a left thoracic curve (which is often associated with an underlying spinal disorder), or (4) rapid progression.

NATURAL HISTORY

Although it is impossible to accurately predict the outcome of most curves, the following facts are known about scoliosis:

TREATMENT

The most important aspect in the treatment of scoliosis is early detection. Although the cosmetic appearance is the patient’s greatest concern, cardiopulmonary dysfunction has the most serious physical impact. A curve that is obviously visible when the patient is standing is often already approaching 30 to 40 degrees. Detecting a curve before it reaches 20 degrees is important because curves greater than 20 degrees tend to progress in the young. Prompt referral of all scoliosis patients to a specialist is mandatory. Frequent reexaminations are essential while the child is at an age where progression is common. The failure to diagnose or treat this problem early may result in progressive deformity, pain, cardiopulmonary compromise, and disability. Early discovery and treatment can prevent this progression.

The treatment depends on the age of the patient and the severity of the curve (Fig. 8-22). In the immature patient, frequent observation is necessary until the curve reaches 20 degrees. Curvatures more than 20 to 25 degrees may require treatment, depending on age and maturity. (The older child whose growth has slowed might only be observed if the curve is not excessive.) The curve can be stabilized and, in some cases, improved by spinal bracing, but full, permanent correction is not possible or necessary. The Milwaukee brace or a thoracolumbosacral orthotic (TLSO) is commonly used for this purpose (Fig. 8-23). The newer underarm orthotics are more easily accepted by the young patient because of their low-profile design. Treatment in the brace is continuous for 23 hours a day, and the brace may have to be worn for 2 years or longer. Exercises are performed in the brace to improve the cosmetic appearance and decrease the curvature, although the family needs to understand that the treatment will not eliminate the curve. Exercises alone will produce no change in the curvature, nor will they prevent any progression without the brace. Excellent results are obtained with proper use of the brace in curvatures between 20 and 40 degrees. It will not fully correct the curve, but it will usually prevent the curve from progressing to the stage where spinal surgery is necessary. Curves greater than 45 degrees cannot be effectively braced. Thus, early detection and treatment are important. Successful nonsurgical management maintains the spinal flexibility that spinal fusion eliminates.

FIG. 8-22 Algorithm for decision-making for genetic scoliosis in the growing child.

FIG. 8-23 A, The Milwaukee brace. B, The thoracolumbosacral orthosis (TLSO). The newer underarm braces have largely replaced other orthoses when the outrigger is not required because they are as effective and cosmetically more acceptable.

Surgery is generally reserved for those cases in which the curvature is more than 45 to 50 degrees. Correction of the entire curvature by intraoperative instrumentation and maintenance of the correction by spinal fusion along the entire length of the curve are usually performed. This may be preceded by corrective casts or traction. Surgical success rates are high, but the loss of spine motion can create limitations for the patient.

In the adult, the spinal deformity may progress and eventually become painful, although the rate of back pain is similar to the general population. The assessment and treatment are essentially the same as it is for the common idiopathic nonspecific low back pain seen in conjunction with degenerative disc disease. Occasionally, instrumentation and spinal fusion may be indicated in these cases, but surgical intervention in the adult with benign back pain is controversial, and conservative treatment remains the mainstay of treatment.

Surgery for the cosmetic deformity alone involves major rib reconstruction. It is usually not recommended, although a few centers have had experience with the procedure.

Female patients with curves greater than 25 degrees may experience a few degrees of increase in their curve with each pregnancy. In most patients, however, neither pregnancy nor delivery is complicated by the presence of scoliosis.

CLINICAL FEATURES

Poor posture is a common complaint, and fatigue and pain often accompany the deformity. The family history may be positive. Examination reveals an increase in the normal kyphosis of the dorsal spine that is usually associated with local tenderness. There may be tightness of the hamstring, pectoral, and iliopsoas muscles. In contrast to postural round back, this kyphosis is usually fixed. That is, the kyphosis does not flatten with spine extension as it does with postural round back. The abdomen may be protuberant, and there is usually a compensatory increase in the lumbar lordosis. A mild scoliosis may also be present. The results of neurologic examination are usually normal.

A positive diagnosis is possible only with a roentgenographic examination. Wedging of the vertebrae, irregularity of the end plate, and typical Schmorl’s nodules are seen on the lateral view, usually between T2 and T12 (Fig. 8-26). Synostoses and osteophyte formation are not uncommon in the adult patient.

FIG. 8-26 Roentgenogram of Scheuermann’s disease. End-plate irregularities and mild wedging deformities are present. There is also increased anteroposterior diameter.

TREATMENT

If the patient is immature with growth remaining, conservative treatment consisting of a brace and postural exercises usually results in a cure. The brace is worn full time for approximately 1 year and is used at night for an additional year. Hamstring stretching and pelvic tilt exercises are initiated. Pain often resolves when growth ceases; however, severe deformity with pain or neurologic symptoms are indications for surgery at any age. The surgery is similar to that performed for scoliosis.

Long-term results of conservative treatment are generally favorable when the disease is confined to the dorsal spine. There is an increased incidence of back pain with curves that are low in the dorsal spine or upper lumbar spine. The working capacity for the patient is usually not affected, however.

CLINICAL FEATURES

Presentation varies with age, and symptoms may be difficult to localize. The child does not usually appear to be acutely ill but may complain of low back pain that often radiates into the abdomen or lower extremities. The child has difficulty walking and standing and may even refuse to walk or sit at all. A slight limp may be present. (All limping children should have their spine examined.) Examination reveals restriction of motion in the lumbar spine in association with hamstring spasm and a flattening of the normal lumbar lordosis. Local tenderness in the midlumbar region is usually present. The child is often irritable and may run a low-grade fever. Nausea and vomiting occasionally occur. Young children may even perceive their pain as abdominal in origin.

There is a positive blood culture in approximately 50% of patients, and the organism is usually Staphylococcus. Needle aspiration of the affected disc sometimes reveals the same organism. There is usually an increase in the sedimentation rate and white blood cell (WBC) count. The bone scan is usually abnormal, and plain roentgenograms frequently reveal single disc space narrowing with irregularity of the adjacent end plates. MRI may be needed to rule out other conditions. Eventually, fusion may even occur between the involved vertebrae.

TREATMENT

Treatment consists of antibiotics (directed against S. aureus on an empiric basis), bed rest, and sometimes the application of a brace. Treatment is continued until the systemic signs of infection, such as the sedimentation rate, creatine phosphokinase [CPK], temperature, and WBC count, are normal. The prognosis is usually good. Surgery is rarely necessary.

CLINICAL FEATURES

Clinical signs include bilateral sacroiliac tenderness and limited motion of the lumbar spine. An important physical finding is the loss of chest expansion to less than 2.5 cm because of costosternal involvement, and this can be easily checked by measuring chest expansion at the nipple line before and after deep inspiration. Virtually any of the other joints of the skeleton may be involved, but most commonly the hips, knees, and shoulders are the secondary joints affected. When there is involvement of the hips and shoulders, permanent damage may develop, but in the other joints the process often resolves without any residual disability or deformity, and this is similar to the arthritis that may be seen with inflammatory bowel disease. There may be tenderness at tendon insertion sites (enthesitis), especially the attachments of the plantar fascia, heel cord, and greater trochanter.

Usually, no skin lesions occur with ankylosing spondylitis, but an anterior uveitis occurs in approximately 25% of the patients. There is usually no urethritis, which helps distinguish it from Reiter’s syndrome. Aortitis develops in 5% of cases.

ROENTGENOGRAPHIC FINDINGS

The early roentgenographic features are usually those of bilateral sacroiliitis. Initially, there are erosions of these joints, and they lose their clear-cut demarcation because of cystic changes and subchondral sclerosis. (MRI is the most sensitive study for detecting early ankylosing spondylitis and should be considered whenever the history is suggestive, but plain films are normal.) The vertebral bodies may also become demineralized, and a typical squaring of the anterior vertebral bodies develops. Apophyseal joint irregularities and paraspinal ligamentous calcifications develop later, and eventually there may be complete fusion of the sacroiliac and hip joints as healing takes place following the inflammation. As the disease progresses, calcifications of the anulus fibrosis and ossification of the anterior longitudinal ligament develop, which give rise to the so-called bamboo-spine appearance characteristic of ankylosing spondylitis (Fig. 8-28).

FIG. 8-28 Ankylosing spondylitis. A, On the anteroposterior view the sacroiliac joints and the lumbar spine are fused. B, A lateral view shows the typical bamboo-spine appearance.

LABORATORY FINDINGS

The current availability of the HLA-B27 system is important in the diagnosis of ankylosing spondylitis. This antigen occurs in more than 90% patients with ankylosing spondylitis (compared with 7% prevalence in the overall general population), and the test may provide a useful method to screen those patients with either a familial history of the disease or low back pain suggestive of the disorder. Other laboratory findings suggestive of the disorder include an elevated sedimentation rate and CPK and a mild anemia.

EXTRASKELETAL MANIFESTATIONS

The extraskeletal manifestations are those of uveitis, aortic insufficiency, and restrictive pulmonary disease. Because this is a chronic disease, the development of secondary amyloidosis is not uncommon and should be considered in those patients in whom chronic renal disease develops. On rare occasions, severe spinal deformity may lead to such kyphosis that the patient is unable to see forward.

DIFFERENTIAL DIAGNOSIS

The differential diagnosis of ankylosing spondylitis includes the other spondyloarthopathies and rheumatoid arthritis. Psoriatic arthropathy, although similar, usually has late involvement of the sacroiliac joints; involves both the small and the large joints, particularly of the upper extremity; and has very specific skin lesions associated with it. Although aortitis may occur in psoriatic arthropathy, urethritis is absent, as is pulmonary fibrosis.

Reiter’s Syndrome is similar to psoriatic arthropathy in that sacroiliac joint involvement usually occurs late. The small and large joints, particularly of the lower extremity, are affected, and skin lesions are usually specific. Uveitis may occur, and urethritis is present at least at some point in the illness. Aortitis may occur, but pulmonary fibrosis has not been reported.

The arthritis associated with inflammatory bowel disease usually involves the large joints, particularly hips and shoulders, and sacroiliitis may be present early in the illness. Skin manifestations may be nonspecific or pathognomonic. In the patient with chronic ulcerative colitis, the skin lesion of pyodermic gangrenosum is at least very suggestive of the chronic underlying illness. In chronic inflammatory bowel disease, uveitis may occur but is uncommon. Urethritis is usually not present, and aortitis is rare.

Rheumatoid arthritis usually affects the peripheral joints and is more common in females. The tenosynovium and bursae are also frequently involved with the inflammatory process. It rarely affects the axial skeleton.

TREATMENT

PROGNOSIS

Early diagnosis, good long-term management, frequent follow-up examinations (including helping the patient understand the disease), exercise, and the appropriate medications usually provide the patient with a normal life span. Death may occur as a result of the development of aortic insufficiency or secondary amyloidosis with chronic renal disease. If valvular disease develops, the patient should be educated regarding the use of prophylactic antibiotics to prevent subacute bacterial endocarditis. The usual course of the disease is not life threatening, however, and a relatively normal lifestyle and work load are generally possible.

CLINICAL FEATURES

Many patients have had minor symptoms of DISH for years before the condition is diagnosed. The primary complaints are those of low-grade spinal stiffness and pain, although the symptoms and signs are often relatively minor despite extensive hypertrophic spur formation. The thoracic spine is most often affected, most commonly on the right side. The presence of large anterior cervical osteophytes may lead to dysphagia (Fig. 8-29). Cervical myelopathy may result from ossification of the posterior longitudinal ligament. Other potential sequelae include lumbar stenosis and serious cervical spinal cord injuries, often associated with minor trauma. Peripheral joint symptoms are usually mild, although heel and elbow pain are often the presenting complaints. Extraspinal manifestations also include an increased risk of heterotopic ossification There are no diagnostic laboratory tests.

FIG. 8-29 Diffuse idiopathic skeletal hyperostosis involving the cervical spine producing only minimal symptoms. Roentgenographic findings usually look worse than the disease.

The roentgenographic findings are those of bridging anterior osteophytes fusing at least four vertebral bodies while preserving disc space. The condition is differentiated from ankylosing spondylitis by lack of involvement of the SI and apophyseal joints and negative HLA testing. Elbow and heel radiographs often reveal osteophytes (enthesophytes). Bone scanning may show increased uptake in areas of involvement, sometimes resembling metastatic disease.

Treatment consists of NSAIDs and an exercise program (walking and stretching). Symptoms are usually mild. The rare case of myelopathy or dysphagia may require surgical intervention.

CLINICAL FEATURES

The early manifestations of the disorder are weakness, anorexia, weight loss, and bone pain, especially involving the spine. The majority of patients present initially with back pain. This often leads to the detection of a destructive skeletal lesion. As the disease progresses, other organ systems become involved and result in more bone pain, anemia, renal insufficiency, and/or bacterial infections. The infections, often recurrent bouts of pneumonia or urinary tract infections, are usually due to the dysproteinemia. They are often pneumococcal in origin and are apparently related to the patient’s inability to synthesize normal amounts of specific antibody in response to the bacterial challenge. Secondary amyloidosis is also sometimes associated with the disorder. Amyloid deposition may be manifested in several patterns. Involvement of the heart or kidney may lead to cardiac failure or nephrotic syndrome.

ROENTGENOGRAPHIC FEATURES

The classic finding is the “punched-out” lesion with sharply demarcated edges (Fig. 8-30). Usually, multiple lesions are found, but in 10%, only a single skeletal defect may be present. Diffuse osteoporosis because of generalized demineralization is the only finding in about 25% of cases. The typical lesions may occur in any part of the skeleton but are most common in the spine, skull, ribs, and pelvis. Pathologic fractures are common. Intervertebral body collapse may result in nerve root or cord compression. Diffuse bony involvement may even result in hypercalcemia. Using the bone scan to assess the disorder is helpful, but a negative scan can be misleading. This tumor is often “cold” on a bone scan because of its rapid destruction. Plain films are often better for evaluation. CT and MRI are also helpful.

FIG. 8-30 Multiple myeloma (arrows). (This patient had a normal bone scan.)

LABORATORY FINDINGS

In approximately half of the cases, the urine contains the Bence Jones protein, which has the unique property of precipitating in an acid pH when the temperature is between 4.4°C and 15.6°C and redissolving when the temperature is 32.2°C. Anemia is often present, and the sedimentation rate is usually elevated. Serum protein electrophoresis usually displays the characteristic dysproteinemia. The serum calcium concentration is occasionally elevated, but the alkaline phosphatase level is usually normal.

TREATMENT AND PROGNOSIS

Because patients with multiple myeloma may have hypercalcemia, hypercalciuria, and hyperuricemia, the importance of ambulation and adequate hydration in their treatment cannot be overstressed. Every effort should be made not to immobilize the patient. Because plasma cell tumors are characteristically radiosensitive, radiographic therapy has been shown to be of extreme help in the control of localized symptomatic lesions. An oncologist should be consulted. Response to chemotherapy is measured by objective signs of improvement, including decrease in concentration of abnormal M-type serum globulins, decreased Bence Jones proteinuria, hematologic improvement of the anemia, and cessation of further skeletal destruction.

The prognosis remains poor, even with newer therapies. Median survival time is about 10 years in patients without bone lesions.

CLINICAL FEATURES

The disease is insidious in onset, with the clinical disorder preceded by asymptomatic silent bone changes over many years. Spontaneous vertebral fractures, which are the most common skeletal injuries resulting from osteoporosis, may develop early after menopause but osteoporosis is often discovered as an incidental roentgenographic finding. Approximately 50% of women older than 65 years of age have spinal compression fractures; about two thirds of the fractures are clinically unrecognized. These fractures develop first because the vertebral body is composed of cancellous bone, which undergoes loss earlier than cortical bone. Osteoporosis leads to fragility fracture from trauma or force equal to or less than that resulting from a fall from standard height that would not cause a normal bone to fracture. Sometimes sneezing or vigorous coughing precipitates a fracture in osteoporotic bone. Acute or chronic back pain, made worse by standing erect or occasionally even by lying flat, should raise suspicion of vertebral fractures in postmenopausal women. The acute pain with a fracture subsides over 2 to 3 months, but could linger as chronic low back pain due to increasing lumbar lordosis. This chronic pain subsides within 6 months unless multiple fractures produce a picture of constant pain. The pain is typically reproduced by deep pressure over the spinous process at the involved level. Neurological deficits are rare.

The midback vertebrae T12 to L3 are the most common fracture sites, followed by T6 to T9 (Fig. 8-31). The rare osteoporotic fracture in the upper thoracic spine may be indicative of an underlying malignant tumor and may necessitate MRI studies. There is little correlation between the degree of collapse of the vertebral body and level of pain. Overall, 20% of women experience another vertebral fracture after their first fracture. They are also at increased risk for nonvertebral fracture. Eventually, vertebral collapse may cause the characteristic loss of body height, protuberant abdomen, and dorsal thoracic kyphosis called “dowager’s” hump. After achieving maximum height, most midlife women and men will lose 1 to 1½ inches of height as part of their normal aging process, primarily as a result of shrinkage of interverbral disks. In otherwise asymptomatic women, a height loss greater than 1½ inches may be associated with compression fractures of vertebra(e). Excessive kyphosis may even allow the rib cage to shift downward leading to restrictive lung disease. This sagging also prevents comfortable expansion of the stomach after eating and causes early satiety, which further limits nutritional intake. A rib–pelvis distance of less than 2 fingers’ width and inability to touch the occiput to wall when standing against the wall suggests the presence of compression fracture.

FIG. 8-31 Osteoporosis. Multiple anterior wedge compression fractures are present. The discs bulge into the adjacent body causing a biconcave deformity, called the “codfish vertebra.”

In the early postmenopausal state, the incidence of fracture of the distal radius is also high. Because the femur is composed of both cortical and cancellous bone, hip fractures usually develop years later, after age 65. Most (80%) of all hip fractures are associated with osteoporosis. Oral alveolar bone loss (which can lead to tooth loss) is strongly correlated with osteoporosis. Even in women without osteoporosis, there is a correlation between spinal bone density and number of teeth. Overall tooth counts are easy to do, and a tooth count less than 20 should suggest the need for further screening for osteoporosis.

Osteoporosis may also alter the psychological state of the individual with changes in self-esteem, body image, and mood. The patient may become more apprehensive and fearful of activity as the potential of fracture lingers in her mind. Many patients experience severe depression.

EVALUATION

The following patients should undergo assessment for postmenopausal osteoporosis: (1) all women 65 years old or older; (2) all adult women aged 40 years and older with a history of fracture or fractures not caused by severe trauma (such as motor vehicle accident); and (3) younger postmenopausal women who have clinical risk factors for fracture. The evaluation should include a comprehensive medical examination, including a complete history and physical examination. Risk factors should be evaluated, and there should be an assessment of the patient’s reliability, understanding, and willingness to accept available intervention.

BONE DENSITY MEASUREMENT

Currently, there is no accurate measure of overall bone strength. BMD is frequently used as a proxy measure and accounts for approximately 70% of bone strength. There is a 50% to 100% increase in fracture risk for each SD decline in bone density (approximately 0.1 g/cm bone mass). Although low bone density reliably predicts the risk of fracture, increase in bone density in response to treatment does not demonstrate a direct correlation with a reduction in fractures. Therefore, a few percentage point differences achieved by various treatments have little clinical meaning. Keep in mind that because the rate of bone loss after menopause contributes equally to the risk of fracture as the total bone mass present at the time of the menopause, a normal bone density measurement at the time of menopause does not mean that the patient will not be at risk of fracture later in life.

The clinical relevance of a bone density measurement in a postmenopausal woman is estimated by using the T-score. For younger women, interpretation uses the Z-score. The T-score is the standard deviation between patient and average peak young adult bone mass. The more negative, the greater the risk of fracture. The Z-score is the SD between the patient and the average bone mass for same age and weight. A Z-score lower than −2.0 requires diagnostic evaluation for secondary causes other than post menopausal bone loss.

Dual-energy X-ray absorptiometry (DEXA) is the technical standard for measuring BMD. It provides good precision for all sites of osteoporotic fractures, it is faster to perform, and the radiation dose is much less than for the standard chest radiograph film. For high precision, the DEXA technique measures mostly at three sites of greatest interest: the radius, the hip, and the spine. However, for practical clinical use and screening, measurements are made at the spine and hip. A normal value at one site does not preclude low bone density at another site. Serial measurements are best made at least 1 year apart. Portable DEXA machines have been developed that measure the heel, forearms, and finger. The hip is the preferred site of measurement in most individuals, because it predicts the risk of hip fracture, the most important consequence of osteoporosis. In younger individuals, such as perimenopausal or early postmenopausal women, spine measurements may be the most sensitive indicator of bone loss. Bone spurs, which are frequent in osteoarthritis, tend to falsely increase bone density of the spine in older individuals.

PREVENTION AND TREATMENT

The prevention and treatment of osteoporosis begins at youth and continues throughout life. Effective preventive strategies that can be implemented during skeletal development (infancy and childhood) and in later life are needed to minimize the physical, social, and economic consequences of osteoporosis. The goals of prevention programs are to (1) optimize skeletal development in the young and maximize the acquisition of peak bone mass that occurs at skeletal maturity; (2) prevent age related and secondary causes of bone loss; (3) preserve the structural integrity of skeleton; and (4) prevent fractures. These goals are met by promoting a diet with adequate calcium content, encouraging good general nutrition, promoting adequate vitamin D intake, regular weight-bearing exercise, and avoiding tobacco and caffeine. Exercise should always include lifting weights with the arms to improve the strength of those bones that commonly fracture in osteoporotic patients (radius and upper humerus). Young women should be discouraged from becoming overly concerned with the thin body image. A certain amount of extra weight may actually be healthy for the skeleton in both the young and old. As part of an annual gynecologic examination, all women should be counseled on the risks of osteoporosis and related fractures.

Additional measures include the following:

The goals of treatment of osteoporosis include preventing fractures, stabilizing or achieving an increase in bone mass, relieving symptoms of fractures and skeletal deformity, and maximizing physical function (e.g., halting progressive deformity). Candidates for treatment include (1) women with T-scores of −1.5 or below with at least one risk factor, (2) women in whom nonpharmacological preventive measures are ineffective (bone loss continues or low-trauma fractures occur), and (3) women with postmenopausal osteoporosis (i.e., women with low-trauma fractures and low BMD, and women with BMD T-scores of −2.0 and below). All patients being considered for drug treatment of osteoporosis should also be counseled on risk factor reduction and adherence to universal recommendations about calcium, vitamin D, and exercise. Before initiating treatment, patients should be evaluated for secondary causes of osteoporosis and have central DEXA measurements, when available.

MANAGEMENT OF OSTEOPOROTIC FRACTURES

Hip fractures almost always require surgical repair if the patient is to become ambulatory again. Depending on the location and severity of the fracture, condition of the neighboring joint, and general status of the patient, procedures may include open reduction and internal fixation with pins and plates, hemiarthroplasties, and total arthroplasties. These surgical procedures are followed by intense rehabilitation in an attempt to return patients to their prefracture functional level. Long-bone fractures often require either external or internal fixation. Other fractures (e.g., vertebral, rib, and pelvic fractures) are usually managed with only supportive care, requiring no specific orthopedic treatment.

For acutely symptomatic fractures, treatment with analgesics is required. A few small, randomized clinical trials suggest that calcitonin may reduce pain related to acute vertebral compression fracture. Short periods of bed rest may be helpful for pain management, but in general, early mobilization is recommended as it helps prevent further bone loss. Occasionally, use of a soft elastic-style brace may facilitate earlier mobilization. Muscle spasms often occur with acute compression fractures and can be treated with muscle relaxants and heat treatments. Various physical modalities, such as ultrasound and transcutaneous nerve stimulation, may be beneficial in some patients.

In some cases, compression fractures may also be treated by percutaneous injection of artificial cement (polymethylmethacrylate) into the vertebral body (vertebroplasty or kyphoplasty); this has been reported to offer significant immediate pain relief in the majority of patients. Long-term effects are unknown, and conclusions are based on observational studies in patients with severe persistent back pain from acute or subacute vertebral fractures. Kyphoplasty—unlike vertebroplasty—has the advantage of restoring lost height by inflating a balloon tamp within and between fracture fragments before injection of the cement. Inclusion criteria include the following: pain localized to fracture, pain refractory to medical management, or a fracture of less than 12 months. Exclusion criteria include the following: fracture extending into the posterior vertebral cortex, retropulsed fragment, cord compression, radiculopathy, coagulopathy, or discitis with osteomyelitis.

Management during the perifracture period must also consider blood clot prevention in patients who will have delayed ambulation, the avoidance of substances that may inhibit fracture repair (NSAIDs, nicotine, corticosteroids), and the frequent need for supplemental caloric intake. Physical medicine and rehabilitation can also improve function and lower disability and the risk of falls. Finally, since fewer than 5% of patients with osteoporotic fractures are referred for medical evaluation and treatment, more aggressive diagnostic and therapeutic intervention of this population will help to prevent subsequent fractures.