Spine problems in children have the potential to cause considerable disability and must be taken seriously. Whereas the majority of adults have back pain at times, back pain in children is less common and often due to some specific organic disease that requires treatment. Deformity is of greater concern in the child because of the potential for progression with growth. Conversely, minor truncal asymmetry (Fig. 1) is common in children and may cause undue concern, leading to unnecessary apprehension and treatment.

 

 

 

Figure 1.  Mild truncal asymmetry These mild asymmetries are variations of normal, do not require treatment, and cause no disability.

 

 

Normal Development

The axial system develops during the embryonic period.

Embryo

During the fourth week, mesenchymal cells from the sclerotome grow around the notochord to become the vertebral body and around the neural tube to form the vertebral arches (Fig. 2). Cells from adjacent sclerotomes join to form the precursor of the vertebral body, an intersegmental structure. Between these bodies, the notochord develops into the intervertebral disc. Cells surround the neural tube to become the vertebral arches.

 

 

 

Figure 2.   Sclerotome growth Cells from the sclerotome grow around the notochord and neural tube.

 

 

During the sixth fetal week, chondrification centers appear at three sites on each side of the mesenchymal vertebrae. The centrum is formed by the coalition of the two most anterior centers. Chondrification is complete before the ossification centers appear (Fig. 3). The centrum, together with an ossification center of each arch, make a total of three primary ossification centers for each vertebra.

 

 

 

 

 

Figure 3. Vertebral development Vertebrae develop first as mesenchyme, then cartilage, and finally bone. Secondary ossification centers develop during childhood and fuse during adolescence or early adult life. From Moore (1988).

 

Childhood

During early childhood, the centers of each vertebral arch fuse and are joined to the vertebral body by a cartilaginous neurocentral junction. This junction allows growth to accommodate the enlarging spinal cord. Fusion of the neurocentral junction usually occurs between the third and sixth years. Anterior notching of the vertebrae is sometimes seen in the infant’s or child’s vertebrae and shows the site of somite fusion.

Secondary ossification centers develop at the ends of the transverse and spinous processes and around the vertebral end plates at puberty. These fuse by age 25 years. Congenital defects are common in the axial system. Variations in the lumbar spine occur in about one-third of individuals. Spina bifida occulta is common. Hemivertebrae result from a failure of formation or segmentation. Such lesions are frequently associated with genitourinary abnormalities and less frequently with cardiac, anal, and limb defects; tracheoesophageal fistula; and conductive hearing defects if the cervical spine is involved.

Cord level Initially, the neural and bony elements of corresponding somites lie opposite each other. Thus, the caudal end of the spinal cord fills the spinal canal, and the spinal nerves pass through the corresponding intervertebral foramina. By the 24th fetal week, the cord ends at S1; at birth, at L3; and in the adult, at L1 (Fig. 4). This differential growth rate results in the formation of the caudal equina: the accumulation of the nerves traversing the subarachnoid space to the intervertebral foramina. The end of the cord is attached to the periosteum opposite the first coccygeal vertebra by the filum terminale. The filum is the residual of the embryonic spinal cord.

 

 

 

Figure 4. Spinal cord vertebral column relationship During the fetal period, the spinal cord fills the vertebral canal. With growth, the cord ends at a progressively higher level.

 

 

Sagittal configuration In the frontal projection, the spine is relatively straight throughout growth. In the lateral projection, the spine evolves from a single curve at birth to a triple curve pattern in the child (Fig. 5). Although this triple curve pattern is necessary to assume an upright posture, the obliquity imposes an added load on the lumbar spine. This load contributes to development of spondylolysis in the child, intervertebral disc herniation in the adolescent, and degenerative arthritis in the adult (Fig. 6).

 

 

 

Figure 5. Normal spine development (sagittal plane) The spine changes from a single curve at birth to a triple curve during childhood.

 

 

 

 

Figure 6. Vertebral intersegmental development The vertebral bodies form as intersegmental structures. As blood vessels grow between somites, their final position is midvertebral. The site of blood vessel entry and somite fusion is sometimes seen radiographically as an anterior notch in the vertebral body of the child (red arrows).

 

 

Evaluation

The spine is evaluated as part of a screening examination or to assess pain or deformity.

History and Physical Examination

Screening examination Is there some underlying disorder? Marfan syndrome, neurofibromatosis, ostoeochondrodystrophies, or mucopoly-saccharidoses are readily obvious in the older child but may not be so apparent in the infant.

History Inquire about the onset, progression, disability, and duration. Family history is of great importance because scoliosis and hyperkyphosis are often familial. Back pain is also familial.

Posture Note asymmetry of shoulder height, scapular prominence, flank crease, or asymmetry of the pelvis. Note any skin lesions, especially those in the midline. The presence of midline skin lesions, such as dimples, hemangioma or hair patches, cavus feet, or leg atrophy, are often associated with underlying spinal lesions. Café au lait spots are associated with neurofibromatosis, a cause of scoliosis.

Be aware that minor truncal asymmetries occur in about 10% of children. These are benign, cause no disability, and require no treatment. Avoid calling attention to such normal asymmetries because it only worries the patient and family.

Forward bending Perform the forward bending test (Fig. 7). This is best done with the examiner seated in front of the child. Control the child’s forward bend by holding the hands together. Slowly guide the child’s forward bending while observing the symmetry of each level of the spine. Any significant scoliosis will be readily apparent.

 

 

 

Figure 7. Limited forward bending Limited forward bending (red arrow) is seen in a variety of diseases. It is an important sign that suggests the need for additional studies.

 

 

Assess asymmetry with a scoliometer (Fig. 8), which measures inclination. Minor degrees of asymmetry are usually on–ly a variation of normal, but require follow-up examination. If any abnormalities are found, a detailed physical and screening neurological examination is essential to avoid diagnostic errors. Hesitation, a list to one side, or restricted motion is abnormal. Lesions such as spinal cord tumors, spondylolisthesis, disc herniations, or discitis limit the mobility or symmetry on forward bending.

 

 

 

Figure 8. Truncal inclination Asymmetry may be assessed with an inclinometer or scoliometer. Measures above 5°-7° are an indication for radiographic studies.

 

 

Side view As viewed from the side, the back should curve evenly without any sharp angulation. A sharp angular segment of the spine is seen in Scheuermann kyphosis.

Neurological examination should be part of the examination. In addition to the routine assessment, assess abdominal reflexes. Abdominal reflexes are assessed by gently stroking each quadrant of the abdominal wall (Fig. 9). Absence or marked asymmetry suggests a subtle neurological abnormality that may indicate the need for more intensive neurological investigation, such as MRI.

 

 

 

Figure 9. Abdominal reflexes Stroke each quadrant of the abdomen with the base of a reflex hammer to assess the symmetry of the reflex.

 

Imaging Studies

Radiographs and other imaging studies are indicated to measure the vertebral curves and to further assess specific problems identified by the physical examination (Fig. 10).

 

 

 

 

 

Figure 10. Uses of imaging methods for spinal disorders Avoid ordering a battery of studies, as this is expensive and often exposes the child to unnecessary radiation.

 

 

 

Radiographs Make PA and lateral spine films in the upright position on 36-inch film using shielding and techniques that avoid excessive radiation exposure. Order oblique lumbosacral views to assess the pars if spondylolysis is suspected and not seen on lateral view.

SPECT Single-photon emission computed tomography (SPECT) imaging is useful to assess subtle pars reactions.

CT studies are useful to detail bony deformities or lesions.

MR imaging is used to study patients with neurological findings, those with unexplained progression of deformity, and certain types of deformity, as well as preoperatively for children with neurological impairment. These studies are helpful in evaluating tumors, congenital abnormalities such as Chiari malformation (Fig. 11), various cysts, tethered cords, and filum terminale anomalies.

 

 

 

Figure 11. Chiari malformation and syrinx This malformation is a displacement of the cerebellum into the spinal canal (red arrow). These lesions may be associated with a syrinx (blue).

 

 

Multiple studies order only those imaging studies that are definitely necessary during the initial evaluation (Fig. 12).

 

 

 

 

Figure 12. Osteoid osteoma spine. This lesion caused severe night pain. Enlargement of pedicle is seen on radiographs (red arrow). The bone scan showed a focal hot spot (orange arrow) and the CT scan shows the sclerotic lesion (yellow arrow). Excison was curative.

 

 

 

Normal variability Measurements of deformity are made from standing radiographs (Fig. 13). Be aware of the normal range in the sagittal plane (Fig. 14).

 

 

Figure 13. Method of measuring spinal alignment Select the endplate of the upper and lower vertebrae with greatest deviation from the horizontal plane. Construct an endplate and right angle line (red). The enclosed angle is the degree of kyphosis or lordosis.

 

 

 

Figure 14. Normal values of sagittal measures of the spine in children The range includes values from the 10th to the 90th percentiles. The L5–S1 angle includes measures between the inferior surface of L5 and the superior surface of S1 (green). Lordosis is measured using the Cobb method between L1 and L5 (red). Kyphosis is measured using the Cobb method between T5 and T12 (blue). From Propst-Proctor & Bleck. JPO 3:344, 1983.

 

 

Sagittal plane The normal range for dorsal kyphosis falls between about 20° and 45°. Kyphosis between 45° and 55° is marginal. Kyphosis below 20° is referred to as hypokyphosis, and above 55° as hyperkyphosis. Hyperkyphosis is sometimes referred to as a “round back” deformity. Normal levels for lumbar lordosis fall between 20° and 55°. Likewise, reduced lordosis is termed hypolordosis, and increased lordosis is termed hyperlordosis. Hypolordosis is called a “flat back” and hyperlordosis either a “lordotic deformity” or a “swayback.”

Frontal plane Mild curves that cause truncal asymmetry are usually normal variants. These variations are <10° by Cobb and <5° by scoliometer measure. These asymmetries have not been shown to cause any disability in childhood or adult life. Bone scans are usual in assessing back pain when radiographs are negative or equivocal.

 

 

Congenital Deformities

Diastematomyelia

This is a congenital defect with a central cartilagenous-bony projection that divides the spinal cord (Fig. 15).

 

 

 

Figure 15. Spinal dysraphism Diastematomyelia and other congenital spine defects should be considered in children with cavus feet or limb hypoplasia (red arrow). The interpedicular distance is widened (orange arrow) and a midline bony bar bisects the spinal cord, as shown on myelography (yellow arrow).

 

 

Diagnosis Cutaneous lesions occur in most patients with a hairy patch, dimple, hemangioma, subcutaneous mass, or teratoma at or near the level of the diastematomyelia. Other deformites are common. Nearly all have some associated anomaly, such as spinal dysraphism, asymmetry of the lower extremities, clubfoot, or a cavus foot. Two-thirds have congenital scoliosis. Two-thirds are located in the lumbar spine. Half have neurological abnormalities.

Management Resect the spur in a patient with progressive neurological findings. Follow the others and consider resection should neurological findings develop or if correction of spinal deformity is planned.

Sacral Agenesis

Caudal regression or sacral agenesis includes a spectrum of abnormalities (Fig. 16) with hypoplasia or aplasia (Fig. 17) of the sacrum, which is most common in offspring of diabetic mothers.

 

 

 

 

 

Figure 16. Sacral agenesis classification by Renshaw. The sacrum may be hypoplastic or completely absent (red). The spine–pelvic relationship may be stable or unstable. Based on Renshaw (1978).

 

 

 

 

Figure 17. Sacral agenesis Radiographs show a type 3 deficiency (yellow arrow).

 

Clinical features include knee-flexion contractures with popliteal webbing, dislocations and flexion contractures of the hips, scoliosis, equinovarus deformities of the foot, and instability at the spinal-pelvic junction. These deformities vary in severity with the level of the agenesis and the resulting loss of motor power. Neurological features may be most predictive of progression, and MR imaging is helpful in assessment.

Management is often difficult and depends upon the deformity, motor, and sensory status. Knee flexion deformities are difficult to correct, and recurrence is common. The combination of limited operative procedures and orthotic or mobility aids are tailored to the child. Spine–pelvic instability and hip dislocations are often better tolerated than the stiffness caused by surgical stabilization or reduction.

Exstrophy Bladder

A failure of anterior closure of the pelvis results in pelvic diastasis and an open bladder (Fig. 18). In the more severe form, cloacal exstrophy, an omphalocele containing intestinal contents is also, present.

Clinical features include pelvis diastasis, acetabular retroversion, and lateral rotation of limbs with out-toeing gait. This out-toeing tends to improve with age.

Management Orthopedic disabilities are insufficient to require correction. A pelvic osteotomy may be required during bladder reconstruction to facilitate closure. Perform bilateral supra-acetabular osteotomies and stabilize with a spica cast following urological repair.

 

 

 

Figure 18. Bladder exstrophy This is associated with separation of the pubic bones (yellow arrow) and retroversion of the acetabula. Bilateral iliac osteotomies (red arrows) were performed to facilitate bladder reconstruction.

 

 

Back Pain

Back pain in children, compared to back pain in adults, is more likely to be caused by some significant organic disease, and it should be taken seriously. The more common causes of back pain (Fig. 19) are presented in the following pages.

 

 

 

Figure 19. Causes of back pain in children These are the major causes of back pain in children and adolescents.

 

Prevalence

Back pain becomes increasingly common during childhood (Fig. 20). By mid-teens, recurrent or chronic pain occurs in about a quarter of boys and a third of girls.

 

 

 

 

Figure 20. Percentage of children with back pain In a Finnish study, back pain increased from about 1% at 7 years of age to nearly 20% in adolescents and to 56% for adults. From Taimela (1997). For Americans, incidence is about 30% in adolescence and about 75% in adults. From Olson (1992) and Balague (1995).

 

Evaluation

Be concerned about a history of back pain in children. Sometimes the presenting symptoms of serious conditions may be misleadingly mild, and the spectrum of causes and mode of presentation may differ from those of adults.

Worrisome features include onset before age 4 years, symptoms persisting beyond 4 weeks, interference with function, systemic features, increasing discomfort, night pain, neurological findings, and recent onset of scoliosis.

Examine with a focus on mobility, symmetry, tenderness, neurological status, and hamstring tightness.

Image first with conventional radiographs. Supplement with a bone scan as necessary. High-resolution SPECT imaging may be useful in assessing adolescent stress injuries. Add MRI if tumor or infection is suspected.

Idiopathic Back Pain

Adolescent benign back pain Back pain without physical abnormalities accounts for an increasingly large proportion of the category with advancing age. Overall, about half of children’s and adolescents’ back pain falls into this category.

Management may be difficult. Some suggest limiting backpacks to less than 20% of body weight (not evidence based). Encourage activity, a healthy lifestyle, and weight control. Provide reassurance. Consider this as a common backache, requiring no treatment, and best ignored.

Prognosis If back pain is present in adolescence together with a positive family history of back pain, nearly 90% of these adolescents will have back pain in adult life. Psychosocial problems are more significant than structural abnormalities in determining the likelihood that back pain will become chronic (Fig. 21).

 

 

 

 

 

 

Figure 21. Familial back pain The child may learn about back pain from siblings and parents.

 

Conversion reaction Reflex sympathetic dystrophy or conversion hysteria may underlie back pain. The typical patient presents with gross, bizarre, and disabling symptoms. Most are adolescent girls. As this type of back pain is very difficult to manage, consider referring to an adolescent medicine specialist or pediatric rheumatologist who has experience in managing this problem. Management often includes physical therapy, psychotherapy, and supportive measures.

Rheumatoid Spondylitis

Rheumatoid disorders may cause back and pelvic pain. The age of onset is usually between 4 and 16 years. More than 90% of patients are HLA-B27 positive with the absence of RF and ANA. About a third will have a family history. Symptoms include peripheral arthritis, usually pauciarticular and asymmetric, involving big joints of the lower limbs. Many complain of heel, back, or sacroiliac pain. An acute iridocyclitis may occur. Most patients develop radiographic sacroilitis. Refer to a rheumatologist.

Cervical Disc Space Calcification

Cervical disc space calcification is a rare, idiopathic, inflammatory condition with clinical manifestations of fever, neck pain and stiffness, and eventual disc space calcification (Fig. 22). The pain and fever resolve spontaneously; calcification is seen at the end of the inflammatory phase. Often residual narrowing and irregularity of the disc space is seen if radiographs are made. Manage with rest, a cervical collar, and a nonsteroidal antiinflammatory agent. Resolution of the acute symptoms usually occurs within 7–10 days.

 

 

 

Figure 22. Cervical disc space calcification Note the calcium deposits in the disc space (arrow).

 

 

Tumors

Tumors may be metastatic or primary. Primary tumors may arise from the cord or bone (Fig. 23).

 

 

Figure 23. Vertebral (bone) and spinal cord tumors

 

Metastatic Tumors

These tumors are most common in the thoracic, then lumbar, and least common in the cervical spine (Fig. 24). Manage with chemotherapy and radiation. Mortality is high. Those who survive are likely to have deformity. Early stabilization may prevent progression of the deformity.

 

 

Figure 24. Metastatic tumors to spine From Freiberg (1993).

 

Primary Tumors

Primary tumors may occur in the vertebrae or cord. Most vertebral tumors are benign, and most cord tumors are malignant. Either type may cause spinal cord compression (Fig. 25).

 

 

Figure 25. Tumors causing cord compression in children From Conrad (1992).

 

Spinal cord tumors cause diagnostic difficulties. They may pre–sent to the orthopedist with torticollis, scoliosis, gait disturbances, foot deformities, or back pain. Often forward bending is limited and asymmetrical. Perform a careful neurological examination. Study with plain radiographs first. Look for changes in intrapedicular distance. MRI studies are usually diagnostic.

Vertebral tumors are more common. Most are benign. Most present with pain. Duration of symptoms from benign tumors is usually longer than those from malignant tumors. Most may be diagnosed by conventional radiographs.

Osteoid osteoma and osteoblastoma cause classic night pain, usually secondary scoliosis, limited spinal mobility (Fig. 26), often tenderness, and sometimes classic radiographic features. Bone scans are often diagnostic. Excision is ofteecessary. Exactly localize with preoperative imaging. Percutaneous ablation is an option (Fig. 27).

 

 

Figure 26. List on forward bending This boy with an osteoid osteoma shows asymmetrical forward bending. Bending is restricted on the left side (arrow).

 

 

 

Figure 27. Percutaneous ablation of osteoid osteoma The lesion is difficult to image on the radiograph (white arrow), but inflammation is readily apparent on MRI (red arrow), well defined on CT (yellow arrow), and ablated percutaneously, as shown by intraoperative imaging (green arrow).

 

Eosinophilic granuloma causes pain, tenderness, limited mobility, and usually a focal lesion. The classic vertebrae plana (Fig. 28) is often absent. For solitary, uncomplicated lesions, observational management is appropriate. If lesions are multiple or if neurological involvement is present, operative resection may be necessary.

 

 

Figure 28. Disc collapse from eosinophilic granuloma Note the vertebral collapse. The appearance is classic.

 

Aneurysmal bone cysts cause pain, rarely cord or root compression, sometimes deformity, and limited mobility. Radiographs are often diagnostic with expansion and ballooning of the cortex (Fig. 29). Management is often difficult. Manage with preoperative selective arterial embolization, intralesional excision curettage, bone grafting, and fusion of the affected area if instability is present.

 

 

Figure 29. Aneurysmal bone cyst in 15-year-old boy Note the expansile cystic lesion (arrows).

Scheuermann Disease

Scheuermann disease is a familial disorder of the thoracic spine producing vertebral wedging and kyphosis greater than about 45° (Fig. 30) and often causing back pain.

 

 

Figure 30. Differentiating postural round back and Scheuermann disease Note the smooth contour of the back on forward bending in the child with round back as compared with the angular pattern in the child with Scheuermann disease.

 

Clinical Features

A history of heavy physical loading from athletics or work is common. Often the deformity is familial (Fig. 31).

 

 

Figure 31. Familial Scheuermann This father and son have the same fixed deformity.

 

Patients often complain of deformity, fatigue, and sometimes pain. The normal even contour of the spine is lost with an abrupt kyphotic segment at or above the thoracolumbar level. Tenderness over the apex may be present. Radiographs show anterior body wedging. Mild scoliosis is common. The strict definition requires a wedging of at least 5° involving three vertebrae (Fig. 32).

 

 

Figure 32. Painful kyphosis This 16-year-old male has pain and tenderness over the lower thoracic spine. Note the narrow disc spaces, as well as erosion and deformity of the vertebral bodies (arrows).

 

Management

Treat the pain by NSAIDs, rest, and stress reduction. Sometimes a thoracolumbar sacral orthosis (TLSO) will be helpful in controlling the pain. Management of the deformity is discussed.

Schmorl Nodes

These nodes are vertical herniations of the intervertebral disc through the vertebral endplate, causing narrowing of the disc space (Fig. 33). Sometimes the condition is referred to as lumbar Scheuermann disease. This herniation is most common in adolescents, is often associated with trauma, and may be the cause of back pain. The lesions may be seen on plain radiographs, although MR imaging is most sensitive and may be indicated when the diagnosis is uncertain. Manage by rest, NSAIDs, and sometimes a TLSO.

 

 

Figure 33. Schmorl nodes With vertical loading, the nucleus may herniate into the vertebral body (red arrow) producing pain and atypical radiographic defects (yellow arrows).

 

Disc Herniation

Disc herniations occur rarely in adolescents. Predisposing features include positive family history, recent trauma, facet asymmetry, spinal stenosis, transitional vertebrae, and spondylolisthesis.

Clinical Features

Herniations usually occur at the L4–L5 or L5–S1 levels, often producing radicular pain and secondary spinal deformity. The patient may be seen because of scoliosis or a list. Straight leg raising is limited, and neurological changes are variable. Radiographs are usually normal. Occult spina bifida is more common in these patients. MRI studies or myelography shows the lesion (Fig. 34). Disability is increased if the herniation is associated with spinal stenosis. Be aware that fracture of the lumbar vertebral ring apophysis may be confused with disc herniations.

 

 

Figure 34. MRI in disc herniation The posterior bulging disc at L4-L5 is clearly demonstrated on MRI.

 

Management

Manage first with NSAIDs, rest, limited activities, and a TLSO. Persisting or increasing disability are indications for MRI and operative disc excision. Endoscopic or open discectomies are successful in 90% of cases.

 

Discitis

Discitis is an inflammation (probably an infection) that involves the lower thoracic or upper lumbar disc spaces in infants and children. Unlike other musculoskeletal infections, discitis usually resolves spontaneously.

Clinical Features

The clinical features of discitis are age related. Discitis in the infant is characterized by fever, irritability, and an unwillingness to walk. The child may show constitutional illness with nausea and vomiting. The adolescent may complain of back pain. Because the symptoms are vague and poorly localized, the diagnosis is often delayed. The findings of fever and malaise, a stiff back, unwillingness to walk, and an elevated ESR and CRP are suggestive of discitis.

Imaging Early in the disease, a bone scan may show increased uptake over several vertebral levels (Fig. 35). After 2–3 weeks, narrowing of the disc space is seen on a lateral radiograph of the spine. MRI often shows worrisome features and may lead to overtreatment (Fig. 36).

 

 

35. Discitis L4-L5 The typical features of discitis are shown on different imaging studies. The bone scan shows increased uptake (red arrow), and later the lateral radiograph shows narrowing (yellow arrow) of the disc space.

 

 

36. MRI of discitis The typical intense inflammatory reaction seen on MRI may lead to concern about abscess formation and lead to unnecessary operative draining.

 

Aspiration or biopsy Disc space aspiration is not necessary unless the disease is –atypical.

Management

Manage based on the stage and severity of the disease. If the child is systemically ill, antistaphylococcal antibiotic treatment is appropriate. If the child is acutely ill, an intravenous route is appropriate. Otherwise, oral medication is adequate. Discitis is more severe in the older child (Fig. 37). Continue antibiotics until the ESR returns to normal. For comfort, consider immobilization in a “panty spica” or brace (Fig. 38) for a period of several weeks.

Prognosis

Long-term studies show a variety of abnormalities that include residual narrowing [A, right], block vertebrae, and limited extension, but the likelihood of back pain is not increased.

 

 

37. Discitis in an older child Discitis is more severe in the older child. Note the extensive inflammation of adjacent vertebrae in the MRI (yellow arrow) and the residual narrowing (red arrow).

 

 

38. Immobilization of the back reduces discomfort Most complete immobilization includes the back and one limb to immobilize the lumbosacral spine (left). Adequate immobilization is often achieved with a custom TLSO that extends well down over the pelvis (arrow).

 

Spondylolysis and Spondylolisthesis

Bilateral or unilateral defects of the pars interarticularis cause spondylolysis (Fig. 39). This defect may allow displacement of the vertebrae, which is called spondylolisthesis. These lesions are the most common cause of structural back pain in children and adolescents.

 

 

39. Spondylolysis The fracture through the pars is shown by red arrows. Note the fracture through the pars as shown on the oblique radiograph (upper left) and diagram (upper right). The scotty dog analogy is often used (lower left) to describe the vertebral elements (yellow lines). The neck is the site of fracture. The superior facet of the sacrum (green arrow) normally prevents forward displacement of L5. This restraint is lost when the pars fractures. The model (lower right), shows the site of the fracture.

 

Pathogenesis

In children, these conditions are usually due to a stress fracture through a congenitally dysplastic pars interarticularis (Fig. 40). This inherent weakness occurs more commonly in certain races (such as Inuit peoples), families, or individuals. The defects often are associated with spina bifida occulta. Spondylolisthesis occurs in about 4% of 4-year-old children and increases to about 6% by maturity. Spondylolisthesis occurs in about a third of those with pars defects, especially in those with mechanical instability. These lesions occur more commonly in children with abnormal bone or connective tissue, as occurs in conditions such as Marfan syndrome and osteopetrosis. Lesions are common in children who participate in certain sports that cause hyperextension of the lumbar spine with rotation, such as gymnastics, wrestling, diving, and weightlifting. Progression after adolescence is unusual.

 

 

40. The natural history of pars defects Most defects develop during early childhood and remain mild. Others develop in late childhood, usually due to repetitive trauma from certain sports or less commonly due to acute trauma.

 

Clinical Features

History and physical examination The child usually complains of back pain. Tenderness may be present at the L5-S1 level. If the displacement is severe, a prominence is palpable over the defect. Straight leg raising and forward bending may be limited. The neurological examination is usually normal. If the condition is acute, secondary scoliosis may be present.

Imaging First, order a standing lateral radiograph of the lumbosacral spine. A forward displacement of the body of L5 or L4 establishes the diagnosis. If no displacement is present, order oblique radiographs of the lower lumbar spine to assess the status of the pars. Spina bifida occulta is common in children with the defect. A bone scan may show reaction (Fig. 41) before radiographs show a defect and may be used to determine the activity and healing potential of the lesion (Fig. 42). Even more sensitive is SPECT imaging in demonstrating the stress reaction of spondylolysis.

 

 

41. Unilateral spondylolysis These studies were performed in a 15-year-old girl with a history of back pain for one month. This bone scan shows an active unilateral defect (orange arrow), and the CT scan shows the defect clearly (red arrow).

 

 

42. Activity of spondylolysis Stages of activity include an acute stage following trauma (red), an intermediate stage, and a late stage (blue).

 

Classification Wiltse classifies spondylolisthesis into two types:

Dysplastic is a congenital facet deficiency allowing slippage.

Isthmic allows slippage due to a defect in the pars interarticularis. These lesions may be due to a fatigue fracture, a stress fracture, or elongation without fracture.

Grade the degree of slip in severity and activity (duration).

Severity Grade on the basis of slip angle (Fig. 43) and displacement (Fig. 44). Slip angle changes usually occur with slips greater than 50%.

 

 

43. Slip angle Draw the sacral line (black) along the posterior margin of the sacrum. Construct a right angle line to sacral line (green). Draw the L5 body line along the superior margin of the body of L5 (red line). The slip angle is the angle between the green and red lines.

 

 

44. Slip displacement The severity of the slip is assessed by the degree of displacement of L5 relative to the sacrum (red arrows) and the slip angle (black triangles). Slips are graded into five categories based on degree of displacement. Note that the slip angle increases progressively through grades 3 to 5.

 

Activity Grade on the duration or activity. Recent fractures are active and show increased uptake on bone scan. Cold lesions are chronic, inactive, and less likely to heal.

Progression Pain is most pronounced at the time of onset or fracture. Most isthmic lesions become stable and painless with time. Pain is aggravated by activity, especially competitive sports. Lesions often are symptomatic in adolescence but become painless in adult life when activity levels are reduced. The incidence of back pain is comparable to normal population levels.

Management

Management is based on the patient’s age, degree of deformity, type of lesion, activity, and physical activity level.

Spondylolysis management depends upon the activity of the lesion.

Acute lesions from an acute injury or recent overuse experience are managed by reduction of activity and usually an under-arm brace (Fig. 45). Often these lesions will heal.

Established lesions Manage symptoms with NSAIDS and activity modification. Operative stabilization is seldom necessary.

 

 

45. Brace for spondylolysis The brace from the front and back shows the anterior closure and posterior extension to provide optimal immobilization for an under-arm brace.

 

Spondylolisthesis is managed based on the severity of the slip, considering the displacement and slip angle. If fusion is required, it is often performed without reduction (Fig. 46).

 

 

46. In situ fusion The fusion is based on the severity of the slip.

 

Grade 1–2 slips Manage with NSAIDS, activity modification, and TLSO as necessary to control symptoms. Follow with standing lateral radiographs.

Grade 3 slips Most require operative stabilization in children. Fuse L4–S1 level with posterolateral autogenous grafting. See next page.

Grade 4 slips These slips may require fusion of L4-S1, as the displacement may be significant, making identification of the transverse process of L5 difficult. If slip angle is severe, reduction is sometimes elected (Fig. 47, 48).

 

 

47. Reduction techniques Reduction of the severe slip may be done with hyperextension casting (blue arrow) or with pedicle screws (green arrow). Once reduced, a two-level fusion is usually performed.

 

 

48. Reduction and fixation This grade 3 slip was reduced, fixed with pedicle screws, and fused. Note the 25° slip angle on the original radiograph.

 

Grade 5 (spondyloptosis) management is controversial. In situ fusion provides pain relief and safety, but the deformity remains. Reduction incurs greater risk but improves appearance and posture (Fig. 49).

Special situations require tailoring of management.

L4 spondylolisthesis is less common, more mechanical in etiology, often causes more symptoms, and is more likely to require operative stabilization.

Spondylolysis with persisting symptoms may be managed by repair of the pars defect with grafting and fixation.

 

 

49. Grade 5 spondylolisthesis The severe slip produces a flattening of the back (red arrow) and complete forward displacement of L5 (yellow outline) on the sacrum. Note that the displacement is grade 5 and slip angle is 45°.

 

Spondylolisthesis Fusion

Fusion is indicated for slips of >50% and those that remain painful following nonoperative management. The need for reduction or instrumention is controversial, but may be indicated for high-grade slips. Fusion of L5–S1 is often sufficient. Fuse from L4–sacrum when the transverse process of L5 is hypoplastic or displaced anteriorly, making a solid single-level fusion less certain.

Technique

Several approaches are effective. Consider tailoring the approach to the slip severity. With increasing slip severity and angle, increase the length of fusion and immobilization following surgery.

Positioning

Place on rolls in the prone position. Prepare the skin and drape to allow visualization from L2–lower sacrum.

Skin incision

Make a midline vertical skin incision. Alternatives include a curved transverse skin incision centered on the L1 spinous process or parallel paraspinal incisions (Fig. 50). Keep in mind the normal transverse anatomy (Fig. 51).

 

 

Figure 50.

 

 

 

Figure 51.

 

Deep exposure

Make through the midline or through two paraspinal transfascial incisions. Make a midline incision through the lumbosacral fascia to expose the spinous process of each vertebral level to be fused. Expose the lamina, facet joints, and transverse processes (Fig. 52).

 

 

Figure 52.

 

Because spina bifida is common, exercise caution in making this deep exposure to avoid accidental entry into the spinal canal.

An alternative is to make two paraspinal fascial incisions, leaving the transverse processes undisturbed and facilitating the lateral exposure (Fig. 53).

 

 

Figure 53.

 

In both approaches, extend the exposure to the tips of the transverse process. Avoid extending the exposure anterior or laterally beyond tips of the processes to avoid injury to the nerve roots and vessels.

Bone graft

Retract the skin and subcutaneous tissues to expose the posterior ilium. Take a substantial bone graft of corticocancellous and cancellous bone.

Fusion

Decorticate the transverse processes of each level to be fused. Create a notch in each sacral ala. Place cancellous bone from the sacral alar notch to L5 [E] or L4 [F] as planned. Place abundant graft in each lateral gutter [G] that extends laterally to the tips of the transverse process. Perform a facetectomy and graft the defect [H]. A posterior fusion is optional [I]. Some consider the posterior fusion to increase the risk of spinal stenosis without improving the rate of fusion.

Postoperative care

After surgery care may include a pantaloon cast, TLSO, or no immobilization. The extremes include a cast and immobilization for 3–4 months or immediate mobilization with no external support. Most manage with a short period of immobilization and a TLSO for 3–4 months.

Illustration

Modified from Pizzutillo et al. (1986).

 

Scoliosis

Scoliosis is often defined as simply a frontal plane deformity of the spine >10°. The deformity is much more complex, however, and includes significant transverse and sagittal plane components. The causes of scoliosis are numerous (Fig. 54). Mild truncal asymmetry occurs in as much as 10% of the population and may be considered as a variation of normal. Curves greater than 10° are abnormal, and in the growing child may progress to cause a significant problem. Scoliosis is the most common back deformity.

 

 

Figure 54. Classification of scoliosis Scoliosis is classified into general categories.

 

Secondary or Functional Scoliosis

This type of scoliosis can also be described as “functional” because it is secondary to some other problem (Fig. 55). The scoliosis usually resolves when the underlying problem is corrected. The scoliosis is usually flexible and nonstructural. There are no bony changes, and the rotational elements are minimal. The common causes of functional scoliosis are leg length inequality and muscle spasm.

 

 

Figure 55. Underlying causes of scoliosis due to muscle spasm These conditions should be ruled out if the scoliosis is atypical, associated with pain, list, stiffness, tenderness, or obvious muscle spasm.

 

Leg length discrepancy Differences in limb length produce a transient functional scoliosis. As discussed this type of scoliosis seldom becomes rigid or structural, presumably because the scoliosis is present only when the child is standing on both feet. Thus with lying, sitting, and walking, the spine is straight. The fear of causing a structural scoliosis or other back problems is not a valid reason for ordering a shoe lift or for performing limb length equalization procedures.

Muscle spasm Scoliosis may be the presenting sign for several inflammatory or neoplastic disorders. The spinal curvature often functions to relieve discomfort. For example, the back is curved to reduce pressure on a nerve root from a herniated disc. Management is directed at the underlying disorder. Scoliosis will disappear once the underlying problem is corrected.

 

Evaluation

The evaluation should establish the diagnosis, determine the severity, and allow an estimation of the potential for progression of the scoliosis.

History Inquire about the age of onset, progression, and previous management. A family history of deformity (Fig. 56) or pain is important as both run in families. Painful scoliosis in the child suggests an inflammatory or neoplastic basis for the scoliosis.

 

 

Figure 56. Familial scoliosis Scoliosis runs in families. Perform a forward bending test on the parents and siblings. This mother (right) was unaware of her scoliosis.

 

Screening examination Start with a screening examination. Look for conditions such as Marfan syndrome or the café au lait spots of neurofibromatosis. Assess the child’s limb lengths and gait, and perform a neurological examination.

School screening The value of school screening is controversial. The advantage is the earlier detection of deformity. The disadvantage is the large numbers of children with schooliosis, those with minimal truncal asymmetry that are referred to physicians, often studied radiographically, and subjected to the anguish of having scoliosis. Proposals to be more efficient have included establishing a threshold of 7° scoliometer reading and biannual screening.

Back examination Note truncal symmetry (Fig. 57). Note differences in shoulder height, scapular prominence, flank crease, and pelvic symmetry. Ask the patient to bend forward. Be concerned about stiffness or a list, as these suggest an underlying neoplastic or inflammatory process.

 

 

Figure 57. Adolescent idiopathic right thoracic left lumbar scoliosis The flank crease (yellow arrow) and thoracic prominence (red arrow) are shown.

 

Perform the forward bending test Visually scan each level of the spine to assess symmetry. If a “rib hump” is present, measure it with a scoliometer. This simple device measures the tilt of the rib hump. Assess the balance of the spine using a plumb line (Fig. 58). Record the displacement of the weight from the buttock crease.

 

 

Figure 58. Balance Assess alignment with a plumb line

 

Radiographs Radiographs are indicated if the scoliometer reading is greater than 7° or if progression is likely. Progression is more likely if the child is under 12 years of age, when others in the family have significant curves, or if any findings suggest that the curve may not be simply idiopathic. Radiographs should be made on 36-inch film and taken standing with shielding. A single PA radiograph is satisfactory for screening or a baseline study.

Cobb angle This method (Fig. 59) measures the level with the greatest tilt. Note the “apical vertebra,” as this defines the level of the curve. Curves greater than 10° are considered significant.

 

 

Figure 59. Cobb measure of curve The degree of scoliosis (red arcs) is the angular difference between right angle lines drawn to the most tilted vertebral bodies. Note the double curve with the thoracic apex at T8 and the lumbar curve at L4.

 

Flexibility Left and right bending studies show the rigidity of the curves (Fig. 60). The value of these studies is controversial.

 

 

Figure 60. Flexibility study The left bending study (arrow) shows full correction of the lumbar curve. In contrast, the thoracic curve shows minimal correction on right bending

 

Level A general classification of the level of the curve is used for general description without regard for management considerations.

Maturity is assessed by clinical evaluation (Chapter 1) or by the status of the triradiate cartilage or the Risser sign (Fig. 61).

 

 

Figure 61. Risser sign The status of the iliac apophysis is a traditional method of assessing maturity. The apohysis may be unossified (0), partially ossified (1–5) or fused to the illium (5).

 

Congenital Scoliosis

Congenital structural defects may cause a variety of spinal curves (Fig. 62). Such curves are often complex and may require special imaging techniques for assessment. Because these malformations are due to an abnormality of the fetal somite formation, associated lesions in the same somite are common. Thus, the finding of congenital scoliosis, especially one involving the thoracolumbar region, should prompt an ultrasound evaluation of the urinary system and consideration about syndromes such as the VACTERL association.

 

 

Figure 62. Congenital scoliosis This child has upper thoracic congenital scoliosis with severe deformity. This type of deformity should be prevented by early surgery.

 

Pathogenesis

Congenital scoliosis is usually caused by a failure of formation or segmentation (Fig. 63). The progression of the curve is related to the type of bony defect. Curves that are most likely to progress are those with unilateral unsegmented bars that restrict growth on one side while the opposite side grows normally.

 

 

Figure 63. Types of congenital scoliosis. The common defects are a failure in formation or segmentation. Complex deformities may show mixed patterns

 

Evaluation

Note the severity, symmetry, and flexibility of the curve. Screen the child for additional disorders of the urinary and cardiovascular systems. Murmurs should be evaluated by a pediatric cardiologist. Order a renal ultrasound, as 10–20% will have congenital urinary abnormalities, some of which are life-threatening.

Imaging Study the pattern of the curve on AP and lateral radiographs of the entire spine and additional imaging methods for special situations (Fig. 64).

 

 

Figure 64. Special imaging in congenital scoliosis. This congenital scoliosis (red arrows) in a newborn was imaged with MRI because of a neurological deficit. Note the hydromyelia (yellow arrow).

 

Categorize the curve pattern to assess the likelihood of progression. If the curve pattern is ambiguous, CT scans of the apical region are sometimes necessary. MR studies are indicated if neurological abnormalities are found. Plan follow-up and repeat the radiographs in 3–6 months.

Management

The management of congenital scoliosis depends upon the pattern and severity of the curve (Fig. 65, 66) and rate of progression.

 

 

Figure 65. Classification of scoliosis Scoliosis is classified into general categories by level.

 

 

Figure 66. Grades of severity The hemivertebrae (white arrow) often produce little deformity. On the other extreme, unilateral fusions of the vertebrae (red arrows) and ribs (yellow arrow) cause progressive severe deformity. This curve was fused in infancy to prevent further progression

 

Observation is appropriate when the potential for progression is uncertain. Evaluate every 3 months during the first 3 years and again during puberty when spinal growth is greatest.

Orthotic treatment of congenital scoliosis is controversial and less effective than for idiopathic curves. Congenital curves that are long and flexible are most likely to respond to brace treatment.

Operative treatment The goal is to obtain a balanced trunk and spine and to prevent any neurological defects with the least disturbance iormal growth. Operative treatment is required in about half of children with congenital scoliosis. Several options are available.

 

In situ fusion is indicated for curves due to unilateral bars or mild to moderate curves demonstrating progression. In children under 10 years of age, both anterior and posterior fusions are necessary to prevent a crankshaft phenomenon.

Hemivertebra resection This procedure may be indicated for severe curves with spinal imbalanace at or below the thoracolumbar junction in young children.

Instrumentation and fusion Moderate curves in the older child may be managed by limited correction. Limited correction and careful monitoring are necessary to prevent neurological complications.

Osteotomy or resection and instrumentation These aggressive measures may be necessary for severe deformity and imbalance. Preoperative halo traction and staged correction are techniques that may reduce the risk of neurological complications.

Hemifusion on the convex side may be considered for lumbar curves in infants or young children to provide some correction with growth.

Thoracic Insuffiency Syndrome

This syndrome may accompany congenital scoliosis. This syndrome includes rib fusions and the inability of the thorax to support normal respiration or lung growth.

Management

Campbell and associates have developed a technique for correction that includes an opening wedge thoracostomy with use of a chest-wall distractor known as a vertical, expandable prosthetic titanium rib (Fig. 67).

 

 

Figure 67. Hemithorax expansion Vertical expandable prosthetic titanium ribs were used to reduce scoliosis and expand a constricted hemithorax. The preoperative appearance (white arrow) and the result following distraction (yellow arrow) are shown.

 

This lengthens and expands the constricted hemithorax, allowing growth of the thoracic spine and the rib cage. The procedure is usually performed in early childhood with repeat lengthenings of the prosthesis performed at 4–6 month intervals. The procedure may also be used for bilateral insufficiency (Fig. 68).

 

 

Figure 68. Bilateral expansion Titanium ribs were used for bilateral chest wall expansion to improve pulmonary function.

 

Results

The procedure corrects most components of chest-wall deformity and indirectly corrects congenital scoliosis without the need for spine fusion. Scolisis is reduced and vital capacity is increasaed.

Complications

The most common complication is asymptomatic proximal migration of the rib fixation devices through the ribs.

 

Idiopathic Scoliosis

Prevalence

Mild truncal asymmetry occurs in about 10% of the population and is a normal variant. The diagnosis of scoliosis is reserved for curves >10°, and this occurs in 2–3% of children, with boys and girls equally affected. Progressive curves are more common in girls by 4––7:1, with a prevalence of 0.2% with >30° and 0.1% >40°. About 10% of children identified with scoliosis require treatment.

Etiology

The cause of idiopathic scoliosis is uncertain. The deformity has a genetic component, as the concurrence among twins is greater than 50% and about a quarter of the daughters of mothers with significant scoliosis also have the deformity. Several theories have been proposed (Fig. 69).

 

 

Figure 69. Possible causes of idiopathic scoliosis These are suggested possibilities

 

Natural History

Progression The potential for progression depends upon the age of onset (Fig. 70), curve severity (Fig. 71) and level of skeletal maturity, Risser sign (Fig. 72), and status of the triradiate cartilage (Fig. 73).

 

 

Figure 70. Natural history of idiopathic scoliosis Progression is related to the age of onset of the scoliosis.

 

 

Figure 71. Probability of progression Progression of > 5° is based on the magnitude of the curve at the age of initial detection. From data of Nachemson, Lonstein, and Weinstein (1982).

 

 

Figure 72. Risser sign Likelihood of progression is based on the Risser sign and curve magnitude. From data of Lonstein and Carlson (1984).

 

 

Figure 73. Triradiate cartilage An open triradiate cartilage (arrow) indicates skeletal immaturity and an increased risk of curve progression.

 

Progression is greatest during the adolescent growth spurt, which occurs just prior to menarche.

In adults, curves <30° progress little, and curves 30–50° progress about 10–15° over a lifetime. Curves 50–76° progress about 1° a year. Curves above T12 are more likely to progress.

Morbidity Patients with untreated adolescent idiopathic scoliosis of the lumbar and thoracolumbar spine have been shown to have radiographic degenerative changes of the spine at 50-year follow-up, but have not been shown to have an increased level of disability compared to the general population.

Pulmonary function Restrictive lung disease can be detected in patients with Cobb angles >100 degrees. Increased early mortality has been demonstrated only for severe early onset scoliosis. Ventilation perfusion scans show that the concave lung is the most affected in the majority of cases. Correction of adolescent idiopathic scoliosis has been demonstrated to increase vital capacity an average of 15% in short-term follow-up studies. Individuals with scoliosis have normal mortality rates.

Pain Back pain occurs in about 80% of the cases, which is comparable to the general population. Curves at the lumbar and thoracolumbar regions are most likely to be painful.

Classification

Idiopathic scoliosis is the most common spinal deformity. Idiopathic scoliosis is often divided into categories based on age of onset and curve pattern.

Age of onset Onset may be described simply as early or late. Traditionally, three categories have been used.

Infantile Onset occurs in the first 3 years

Juvenile Onset at age 3 to 10 years.

Adolescent Onset at age 10 years to maturity.

Curve pattern The patterns may be described simply by location. This classification is useful for all types, independent of cause. For idiopathic scoliosis, curves are classified to facilitate management and communication.

King-Moe This classification includes five categories (Fig. 74) and clarifies which thoracic curves require fusion and the importance of the stable vertebra as the endpoint for instrumentation and fusion. Because this classification has been demonstrated to show low inter- and intra–observe reliability and limitations when utilizing modern methods of instrumentation, Lenke and associates developed a new classification.

 

 

Figure 74. King-Moe classification This classification is sometimes still used for classifying curves. Curves may be single or double, with the curve apex (shown by red dots) being thoracic, lumbar, or combined. The extent of fusion (arrows) varies with the curve pattern. Based on King et al. (1983).

 

Lenke This classification includes six curve types (Fig. 75), a lumbar spine modified (A, B, or C) and a sagittal thoracic modified (-, N, or +), creating a total of 42 different curve types. The lumbar modified more precisely defines the position of the lumbar curve. The thoracic modified defines the sagittal alignment as being hypokyphotic, normal (curve 10°–40°) or hyperkyphotic. This classification system has been shown to be quite reliable, and its use is increasing.

 

 

Figure 75. Lenke classification Curves are described as proximal thoracic, main thoracic, and thoracolumbar/lumbar in location. Structural curves (red dots) and nonstructural curves (green dots) are differentiated by flexibility. The major curve has the largest Cobb measurement (red arrow).

 

Management Principles

Manage scoliosis by observation, bracing, or surgery. Exercises, electrical stimulation techniques, and manipulation are ineffective and should be avoided. Ninety percent of curves are mild and require only observation. The objectives of treatment are to avoid unnecessary treatment, to minimize the morbidity of required treatment, and to successfully arrest progressive curves or correct curves that cause or will likely cause disability.

Reassurance is an important part of management. Avoid the term scoliosis for mild curves, and simply refer to the deformity as a “mild truncal asymmetry.” This reduces the apprehension that is associated with the diagnosis of scoliosis. This diagnosis often causes apprehension, as scoliosis is usually equated with treatment either by bracing or surgery.

Indications for treatment should be individualized; however, some generalizations can be made (Fig. 76).

 

 

Figure 76. Mild to moderate curve management Curves less than about 45° are managed based on curve severity (Cobb angle) and maturation.

 

Observation only is indicated for patients with curves of less than 25°. Mature patients may be discharged or advised only if they become symptomatic. Follow immature adolescents with a radiograph every 6 months until maturity.

Brace treatment is indicated for immature patients (Risser 0 or 1) with curves of 25°–40°. Boys may be treated with Risser 2–3 if the curve exceeds 30° and is progressive. Observe smaller curves for progression. Progression is defined as a documented increase of 5 or more degrees.

Operative treatment is usually indicated for immature patients with curves >40° and mature patients with curves >50°.

 

Infantile Scoliosis

Infantile idiopathic scoliosis occurs in infants and children under 3 years of age. Because the deformity often is associated with plageocephaly and hip dysplasia, it is thought to be a positional deformity. Like other position deformities, spontaneous resolution usually occurs. In some cases, the scoliosis is secondary to an underlying spinal abnormality. These cases progress to become severe. Infantile scoliosis is rare in North America.

 

Evaluation

Boys with left thoracic curves are the most common group to have infantile sciolosis. Study by radiographs and measure the apical-rib-vertebral angle difference, or RVAD (Fig. 77). If the RVAD exceeds 20°, study with an MRI, as about a quarter will show a significant neuroanatomical abnormality such as Chiari-1 malformations.

 

 

Figure 77. Rib-vertebral angle difference This is the angle between the axis of the ribs (red lines) and a right angle to the body of the vertebrae (black line). Note on the left side here that the lines are parallel, giving a RVAD of 0°.

 

Management

Curves with angles of <20° resolve and require only observation. Follow closely curves >20°. If curves progress and exceed 30°, manage with a brace. Curves uncontrolled by bracing that exceed 40° may require operative correction. Several operative options are available.

Rib distractors Titanium rib distraction with lumbar laminar fixation may be serially expanded for gradual reduction in curve severity (Fig. 78).

 

 

Figure 78. Progressive correction by rib expanders This young child with scoliosis was managed by expandable fixation (yellow arrows), utilizing rib fixation at three sites and a single lumbar hook. Note the progressive reduction in curve magnitude.

 

Spinal instrumentation Instrumentation without fusion preserves growth (Fig. 79).

Fusion Consider anterior and posterior fusion to arrest progression and prevent crankshaft deformity. Be aware that, following fusion, trunk height will be lost at about 0.07 cm per level fused times the years of remaining growth.

 

Figure 79. Instrumentation without fusion This distraction rod was placed to prevent progression while allowing the spine to continue to grow.

 

Juvenile Scoliosis

This form of scoliosis is identified between 3 and 10 years of age (Fig. 80). Gender ratios are about equal for the younger patients, but girls become predominant toward puberty. About two-thirds of the curves are progressive. Most require bracing.

 

 

Figure 80. Juvenile scoliosis This girl shows an elevated right shoulder and thoracic asymmetry.

 

Evaluation

Measure the Cobb angles. For children with a Cobb angle >20°, study with a full spine MRI, as 20–25% will show a significant spinal abnormality such as Chiari I malformations or tumor. Hypokyphosis with values <20° suggests a poorer prognosis and complicates orthotic management.

Management

Follow for progression. A few curves resolve spontaneously. Institute orthotic management for progressive curves that exceed 20° [A].

Bracing Manage curves with an apex below T7 with a TLSO. A Milwaukee brace is necessary for more proximal curves. Considering the long duration of bracing necessary, balance brace time with tolerance. Avoid bracing for too many years, as the child must endure many years of brace treatment as well as the final surgical correction.

Operative correction is indicated for curves exceeding 40°–50°. Anterior and posterior fusions are necessary for young children to prevent the crankshaft deformity. Be certain to correct or maintaiormal sagittal alignment. Instrumentation without fusion may be considered in young children, as described for the infantile form.

Adolescent Scoliosis

Idiopathic scoliosis with an onset after age 10 years is the most common and classic form.

Brace Principles

Bracing usually slows or arrests progression of most spinal curvatures in immature patients with progressive curves between 25° and 40°. Brace curves with documented progression above 25° or curves above 30° when first seen.

Bracing options Select the orthosis based on the type and level of curve and the anticipated tolerance of the patient (Fig. 81).

 

 

Figure 81. Types of braces These are common braces and generalizations about their use.

 

The most effective bracing types and protocols are also the most restrictive and cause the greatest psychosocial disability. Select a balance that is best for the patient.

Nighttime braces are best tolerated, but effectiveness is contoversial. The Charleston bending brace is most widely used. The brace is worn only at night, allowing the child freedom during the day.

TLSO brace This is the most commonly used orthosis. It is appropriate for curves with an apex in the midthorax and below. The Boston brace (Fig. 82) is prefabricated with custom pads applied by the orthotist. Most include a 15° lordosis correction. The brace may be worn on a 16- to 23-hour-per-day protocol.

 

 

Figure 82. Boston brace These underarm braces are useful for low thoracic and lumbar curves. They can be hidden by clothing for both genders.

 

Milwaukee brace For upper thoracic curves, the Milwaukee brace may be necessary. This brace is the most restrictive and is compatible with limited activity.

Introduce the brace over a period of several weeks. Encourage acceptance as quickly as possible. Discomfort in the brace should be corrected by making necessary modifications early. Continued discomfort reduces compliance, increasing the risk of bracing treatment failure. Modifications in the brace will correct this problem. Encourage normal activities while being braced.

Improving acceptance Several methods can be used to reduce the adverse effects of brace treatment of scoliosis (Fig. 83). The bracing schedule may be tailored to the patient. Some patients are already at or beyond their tolerance limits. It may be best to maintain a relationship with the patient and family and to follow the patient without treatment. If the curve is advanced, it may be best to elect an operative option earlier than is normally appropriate. Make certain the patient and family are aware that the time in the brace and the control of the curve are proportional.

 

 

Figure 83. Improving acceptance of brace treatment These are techniques that may be used to keep the management within the tolerance limit of the patient.

 

Initial response should show a reduction of the curve by >50%.

Follow-up Schedule follow-up visits every 4 to 6 months to assess fit, size, compliance, and curve progression. Obtain a standing PA radiograph out of the brace to assess progress.

Dealing with compliance Bracing is uncomfortable, often adversely affects self-image, and imposes some difficulties with social and athletic activities. The patient should participate in most prebracing activities (Fig. 84).

 

4

 

Figure 84. Physical activity This girl remains physically active even while wearing a Milwaukee brace.

 

 All of these problems further complicate an already difficult time in life. The physician must not exceed the “tolerance limit” of psychological stress on the patient. If this tolerance limit is exceeded, the patient will become noncompliant and may not return for follow-up. He or she may simply ignore the problem or seek nonconventional methods of treatment that are less demanding. Make the patient and family aware that control or correction of the curve is related to the time in the brace.

Discontinue bracing about 2 years post-menarcheal or Risser 4 for girls and Risser 5 for boys. Progression while bracing may indicate the need for operative stabilization.

Operative Treatment Principles

Indications Operative management is the most definitive and effective method of management of scoliosis. It is appropriate for curves that exceed 40°–50°.

Approaches Select the approach based on the curve characteristics and the experience of the surgeon.

Posterior fusion This standard approach allows correction and instrumentation of the majority of curves and levels.

Anterior fusion The advantages of anterior fusion include a reduction in the number of vertebra requiring fusion (Fig. 85), less dissection, and correction of hypokyphosis. Anterior fixation provides excellent stability when extended to or just beyond the neutral vertebrae.

 

 

Figure 85. Anterior fixation This lumbar curve was instrumented and fused, involving only five vertebrae.

 

Fusion levels It is important to establish fusion levels thoughtfully. Too short a fusion may result in progression; fusion too long increases the risk of pain and degenerative changes. Inappropriate fusion levels may cause spinal malalignment, changes in posture, and postfusion back pain. Definitions are useful in planning instrumentation and fusions (Fig. 86). Note the rotation of the apical vertebra (Fig. 87).

 

 

Figure 86. Curve definitions These are definitions by Lenke and associates for assessing and defining curve configuration in planning instrumentation and fusion.

 

 

Figure 87. Apical vertebral rotation The degree of rotation is graded based on the

 

Curves Fuse the major curve or largest curve, regardless of its flexibility, and minor or structural curves.

Upper and lower end vertebrae may extend to the neutral vertebra, which is defined as the lowest vertebra bisected by the CSVL.

Combined anterior and posterior fusion This procedure is indicated to prevent crankshaft phenonemon in children, for correction of severe curves, or to reduce the risk of recurrence in patients with constitutional disorders such as Marfan syndrome.

 

Operative Technique

Instrument to reduce the scoliosis and maintain or improve sagittal alignment. Avoid excessive distraction, and incorporate solid fixation. Decorticate carefully, excise facet joints when feasible, and add supplemental bone. This supplemental bone may be autogenous, bank bone, or agents that induce osteogenesis.

Harrington instrumentation was the initial standard that incorporated distraction and compression of the ends of the curves. This technique provided little control of sagittal alignment and has been largely replaced.

Luque fixation utilizes sublaminar wires fixed to posterior rods.

Drummond fixation employs spinous processes to posterior rod fixation.

Cotrel and Dubousse introduced a universal system that provides translation and rotation in addition to distraction, which permits a solid three-dimensional correction. Many modifications of this form, such as the Isola and TRSH systems, have been developed.

Hybrid fixation utilizes options such as dual rods, laminar wires, pedicle screws, and cross links to achieve maximum stability (Fig. 88).

 

 

Figure 88. Hybrid instrumentation Correction and fixation of this curve utilized dual rods, pedicle screws (yellow arrows), laminar wires (white arrows), and cross links (red arrows).

 

Video-assisted thoracoscopy These procedures allow closed anterior releases, rib resection and harvesting, and insertion of correctional implants to reduce operative morbidity (Fig. 89). Such procedures require special skills and instrumentation, and carry a steep learning curve. Due to the increased complication rate, the procedure is controversial.

 

 

Figure 89. Thoroscopic instrumentation This curve was instrumented percutaneously with video assistance.

 

Spinal Monitoring

Monitoring is utilized to reduce risk of neurological injury during spinal exposure and instrumentation.

Wake-up test Intraoperative wake-up neurological testing is effective and inexpensive but difficult to use, and has largely been replaced by continuous monitoring methods.

Intraoperative neurophysiological monitoring includes transcranial motor-evoked potentials (TcMEP) and neurogenic motor-evoked potentials (NMEP).

Complications

Operative complications are not uncommon because of the magnitude of the procedure and also the vulnerability of the cord and nerve routes (Fig. 90).These complications are described as early, such as neurological injuries, and late, such as pseudarthrosis.

 

 

Figure 90. Complications following scoliosis surgery These complications may follow surgical correction of idiopathic scoliosis.

 

Sagittal Deformity

Sagittal alignment (Fig. 91) is affected by our upright posture and significantly affects appearance, cardiopulmonary function, and potential for degenerative arthritis of the spine. Because the spine has greater mobility in flexion and extension than side bending, sagittal deformities are not complicated by a rotational component, as occurs with scoliosis. The spine has three curves: cervical lordosis, thoracic kyphosis, and lumbar lordosis. Upright posture requires that these curves be balanced; they are interrelated. Furthermore, lower extremity alignment affects the spine. For example, excessive lumbar lordosis is usually compensated by hip flexion.

 

 

Figure 91. Patterns of sagittal deformity Normal (green), Scheuermann kyphosis (red); hyperlordosis secondary to hip flexion contracture (blue); flat back (yellow) and associated neuromuscular disorders; and thoracic lordosis (brown) with pulmonary compromise, as seen in muscular dystrophy.

 

Kyphosis

Kyphosis is a posterior convex angulation of the spine. Kyphosis is normal for the thoracic spine with normal range from about 20°–50°.

Postural round-back This is a normal variation. The major problem is cosmetic. It is flexible, as the posture can be improved by asking the child to straighten up, and it does not cause a permanent deformity.

Congenital kyphosis Congenital kyphosis may be due to a failure of formation, segmentation, or mixed types (Fig. 92) and (Fig. 93). The apex of the curve is most common between T10 and L1. Deformities secondary to a failure of formation are usually progressive and may lead to paraplegia. Assess the apex with high-quality radiographs and a CT study if necessary. Classify the type of deformity. For progressive deformities under about 55°–60°, fuse posteriorly. More severe deformities may require anterior and posterior fusions.

 

 

Figure 92. Congenital kyphosis Vertebral hypoplasia may lead to paraplegia (red arrow). Kyphosis in spina bifida (yellow arrow) often shown best by CT scans (white arrow). This severe kyposis may cause skin breakdown over the apex and difficulty in positioning

 

 

Figure 93. Classification of congenital kyphosis and kyphoscoliosis Based on McMaster and Singh (1999).

 

Scheuermann Kyphosis

This disease often causes both pain and deformity (Fig. 94). The deformity may present with back pain, as discussed on page 205, or as a deformity.

 

Figure 94. Scheuermann kyphosis Note the round back deformity and anterior wedging of vertebrae (red arrows).

 

Deformity Management of the deformity is controversial, as long-term disability is mild and effective treatment is difficult.

Moderate curves <60° Manage with observation and encourage physical activity. Curves >60° in skeletally immature children (Risser sign <3) may be improved by brace treatment. Consider applying a preliminary hyperextension plaster cast to improve flexibility. For curves above T7, use a Milwaukee brace (Fig. 95). For lower curves, use an underarm brace. Brace initially for 20 hours daily. Once the curve is controlled, taper the brace to nighttime use.

 

 

Figure 95. Milwaukee brace management of juvenile kyphosis The Milwaukee brace is effective in managing kyphosis. The outcome is related to the severity of the curve at the beginning of treatment. Based on Sachs et al. (1987).

 

Curves >80° uncontrolled by bracing may require operative correction with posterior instrumentation and fusion.

Natural history of this condition is usually benign, except in individuals with kyphosis that was upper thoracic and >100° who were likely to have restrictive lung disease.

Postoperative Hyperkyphosis

This serious deformity is common following laminectomy in children for conditions such as tumors or trauma. This deformity is best prevented by decompression or exposures that save posterior elements or early posterior fusion in wide excisions in growing children.

Normal lordosis Lordosis is the anterior convex angulation of the lumbar spine. The normal range of lordosis is from about 30° to 50°.

Developmental lordosis This developmental variation is common in the prepubescent child (Fig. 96). Parents are concerned. The deformity is flexible, and the screening examination is normal. Radiographs are not necessary. Resolution occurs with growth.

 

 

Figure 96. Physiologic lordosis of puberty This form of lordosis (red arrow) is seen during late childhood just prior to puberty. The spine is flexible and the lordosis disappears on forward bending (white arrow).

 

Functional hyperlordosis This deformity is functional, a compensation for a fixed deformity above or below the lumbosacral level.

Hyperkyphosis is the primary deformity, and the hyperlordosis is compensatory. This compensatory deformity remains flexible, and this flexibility is demonstrated by correction of the lordosis on forward bending.

Hip flexion contracture causes a functional increase in lordosis, usually >60°. This deformity is very common in cerebral palsy. Assess with the prone extension test (Fig. 97). Lordosis is also common in children with bilateral developmental hip dislocations or coxa vara.

 

 

Figure 97. Prone extension test for assessing hip flexion contracture The thigh is gradually lifted until the pelvis starts to extend. This indicates the limit of hip extension. The contracture is the angle between the thigh (red line) and the horizontal (yellow line).

 

Structural Hyperlordosis or Hypolordosis

Operative procedures that arrest growth of the posterior lumbar vertebrae, such as shunting or rhizotomy, may result in increasing lordosis with growth.

Spondyloptosis causes a secondary hypolordosis with flattening of the buttocks.

Neuromuscular disorders such as muscular dystrophy may cause hypolordosis.

Fractures with malunion may cause an increase or decrease in lordosis.

 

Cervical Spine

Cervical spine problems that often present with neck complaints is covered in Chapter 9.

Radiographs

Conventional radiographs remain the most valuable method of imaging the neck and shoulder.

Pseudosubluxation at C2–C3 and less commonly at C3–C4 is common in children under the age of 9 years (Fig. 98).

 

 

Figure 98. Pseudosubluxation The normal alignment of the cervical spine is usually well demonstrated by a lateral radiograph. Pseudosubluxation is common in younger children with C2 displaced forward on C3 (yellow arrow).

 

ADI The atlanto-dens interval (ADI) is the distance between the odontoid and anterior arch of axis (Fig. 99). This measure is most important in children. This distance is <4–5mm in children. When the ADI >10–12 mm, all ligaments have failed. Flexion-extension lateral radiographs (Fig. 100) demonstrate instability most graphically.

 

 

Figure 99. Cervical measures These lines and measures are commonly used. The SAC, or space available for the cord (yellow line), and ADI, or atlanto-dens interval (red line), are expressed in mm.

 

 

Figure 100. Neutral and flexion views of cervical spine These studies show the relationship between arch of atlas (red ring) and the front of the odontoid (yellow line). The distance between is the ADI (red line). This relationship changes with neck flexion (right), demonstrating C1-C2 instability, with the ADI increasing from 2 to 10 mm due to rupture of the transverse atlantal ligament.

 

SAC The space available for the cord (SAC) is between the odontoid and the posterior arch of the axis.

Occiput–C1 relationship is often assessed by McRae and McGregor lines (Fig. 99).

Special Studies

Additional imaging studies may be appropriate, depending upon the evaluation. Look for associated defects. For example, order a renal ultrasound evaluation if the diagnosis of Klippel-Feil syndrome is made. In children with disproportionate dwarfism, prior to any surgical procedure requiring anesthesia, order a screening flexion–extension lateral radiograph of the cervical spine. If instability is demonstrated, special intubation techniques will prevent injury to the cervical spinal cord.

Basilar Impression

Basilar impression is a congenital or acquired deformity in which the cervical spine extends into the foramen magnum. The deformity may be congenital or secondary to osteopenia due to conditions such as rickets or osteogenesis imperfecta. This deformity may cause symptoms during adolescence.

Occipital-Atlantal Instability

Instability at the occiput-C1 level is rare and usually due to a congenital bony defect or marked ligamentous laxity, as seen in Down syndrome. Seldom is operative stabilization by fusioecessary.

 

Atlantoaxial Instability

Instability at the C1–C2 level is relatively common. Instability is due to abnormalities of the odontoid (Fig. 101, 102) or to ligamentous laxity.

 

 

Figure 101. Odontoid hypoplasia Note the hypoplastic odontoid and the instability, as demonstrated by an ADI of 8 mm.

 

 

Figure 102. Odontoid types These varied types contribute to varying degrees of instability. Based on Copley and Dormans (1998).

 

Instability results from rupture or attenuation of the transverse atlantal or alar ligaments (Fig. 103).

 

 

Figure 103. Constraining ligaments These multiple ligaments usually prevent the odontoid from compressing the cord.

 

Such ligamentous deficiencies are common in Down syndrome and in rheumatoid arthritis. Instability is also common in disproportionate dwarfism. Children with these problems should avoid activities that cause cervical spine stress and should have an evaluation prior to being administered a general anesthetic.

 

Polyarticular Juvenile Rheumatoid Arthritis

Clinical stiffness and radiographic changes in the cervical spine occur commonly in polyarticular-onset and systemic-onset disease. Neck problems are rare in pauciarticular-onset disease. Although stiffness and radiographic changes are common, children seldom complain of neck pain.

 

Klippel–Feil Syndrome

The Klippel-Feil syndrome includes clinical (Fig. 104) and radiographic (Fig. 105) features. The syndrome is now known to be much more generalized.

 

 

Figure 104. Clinical features of Klippel-Feil syndrome This syndrome includes shortening of the neck, a low hairline and neck stiffness.

 

 

Figure 105. Radiographic features of Klippel-Feil syndrome This syndrome cervical fusions (red arrow), and various other abnormalities, such as scoliosis (yellow arrow).

Clinical features About half have the classic findings of fusions, low hairline, and stiffness. Classify the condition by the levels of fusions. Other clinical associations include congenital scoliosis, renal anomalies, Sprengel deformity, synkinesia, congenital heart disease, and impaired hearing (Fig. 106). Other deformities include odontoid abnormalities, occipito-cervical fusion, and basilar impression.

 

 

Figure 106. Associations Disorders about the neck are often associated with other congenital defects. Renal and cervical instability problems may not be diagnosed unless special studies are ordered.

 

Evaluate carefully with full spine examination, neurological, cardiac, renal, and hearing screening. Make radiographs of the entire spine. Order a renal ultrasound. If neurological findings are present, study with MR imaging.

Management includes advising the family of the risks and avoiding activities such as diving, football, and gymnastics, which place excessive loads on the cervical spine. Arthrodesis of unstable segments may be required if excessive instability and neurological abnormalities are present.

Natural history Affected individuals have instability problems above and degenerative problems below the levels of fusion. Adults have disability from this syndrome.

 

Spine in Generalized Disorders

Many constitutional disorders, such as the osteochondrodystrophies and metabolic and chromosomal abnormalities, are associated with scoliosis. In these children, during each clinic visit, screen for spinal deformilty.

Achondroplasia

This is a rhizomelic short-limb dwarfism, which is usually readily recognized at birth. Major and disabling spine deformities occur often in these children (Fig. 107).

 

 

Figure 107. Achondroplasia Note the kyphosis in the infant (red arrow) and the narrow lumbar canals in the adolescent (yellow arrows).

 

Stenosis of the foramen magnum causes increased hypotonia, sleep apnea, and sudden infant death syndrome. Foramen magnum decompression, duroplasty, and cervical laminectomy may be necessary if symptoms are severe.

Thoracolumbar kyphosis is common in most infants. The deformity is usually flexible. Treat rigid curves >30° with an orthosis. If deformity exceeds 40° after age 5, anterior and posterior fusion may be required.

Spinal stenosis is common and often becomes symptomatic in early adult life. The stenosis may be aggravated by thoracolumbar kyphosis. This deformity is usually treated in adulthood.

Pseudoachondroplasia

This autosomal dominant short-limb dwarfism causes several spinal problems.

Atlantoaxial instability from odontoid deficiencies and generalized laxity is demonstrated by flexion–extension radiographs and MRI if unstable. Decompression and fusion may be required.

Thoracolumbar deformities include kyphosis and scoliosis.

Hyperlordosis may result from hip flexion contracture.

Osteogenesis Imperfecta

Deformity is due to osteopenia (Fig. 108), and scoliosis and basiler invagination are serious problems. Bracing is inappropriate, as it may cause chest and rib deformity and is unlikely to arrest progression of the curve. Operative stabilization and fusion are indicated for curves exceeding 35°–45°. Instrument with posterior sublaminar segmental fixation and fusion. Add anterior fusion if the deformity is severe and/or associated with kyphosis.

 

 

Figure 108. Osteogenesis imperfecta Note the vertebral deformity (red arrow) and accentuated lumbar lordosis (orange arrow).

 

Spondyloepiphyseal Dysplasia

This is a group of short-trunk dwarfism with dysplasia of the spine and long bones.

Atlantoaxial instability occurs in about 40% from odontoid deficiencies, and generalized laxity is demonstrated by flexion–extension radiographs and MRI if unstable. Decompression and fusion may be required.

Thoracolumbar scoliosis and kyphosis are common and may cause back pain in adults. Manage as with idiopathic scoliosis.

Diastrophic Dysplasia

This is an autosomal recessive disorder with short-limb dwarfism. Spine deformities include generalized cervical spina bifida, cervical spine kyphosis, and thoracolumbar kyphoscoliosis. These deformities may be severe and require instrumentation and fusion.

Marfan Syndrome

This is an autosomal dominant disorder of connective tissue.

Scoliosis develops in most patients (Fig. 109). Curve patterns are often double, major structural, right thoracic, left lumbar. Some curves are triple. Curves usually start earlier and are more progressive, refractory, and rigid.

 

 

Figure 109. Marfan syndrome Note the severe right thoroacolumbar curve. This curve is not improved by bracing, making instrumention and fusioecessary.

 

Brace management is less effective than for idiopathic scoliosis but is used with similar indications and protocols.

Operative management is indicated for curves >50° with segmental fixation using sublaminar wires. Be certain to balance the spine and restore normal sagittal alignment.

Spinal deformities have included atlantoaxial instability and spondyloptosis, among others.

Morquio Syndrome

This mucopolysaccharidosis type IV is one of a spectrum of lysosomal storage diseases. The spine is normal at birth, but deformities develop with growth (Fig. 110).

 

Figure 110. Morquio syndrome Vertebral body changes (yellow arrows) are useful in evaluation. Odontoid hypoplasia (red arrow) is a serious defect.

 

Odontoid dysplasia is common and life-threatening. Odontoid aplasia, hypoplasia, or os odontoideum may cause instability. This instability, combined with accumulation of mucopolysaccharides within the spinal canal, may compromise the cord, causing sudden death or quadraplegia. Manage instability with neurological compromise first with evaluation by dynamic MRI studies. Fuse occiput to C3 or more proximal if posterior elements are adequate. Consider prophylactic stabilization if instability is severe.

Neurofibromatosis

Spine involvement ieurofibromatosis is common (Fig. 111). Look for bony dysplasia associated with the scoliosis. If dysplastic features are present, consider MRI or CT studies. Follow carefully, as rapid progression may occur with growth.

 

 

Figure 111. Neurofibromatosis Curves tend to be sharp and progressive (red arrow).

 

Nondystrophic scoliosis Manage like idiopathic scoliosis.

Dystrophic scoliosis is often characterized by short angular progressive curves. Brace treatment is ineffective. Correct by combined anterior and posterior spinal fusion. Include the entire structural levels in both the fusion masses.

Rett Syndrome

Rett syndrome is a progressive encephalopathy observed only in girls, who are apparently normal until 6 to 12 months of age. It is characterized by autism, dementia, ataxia, stereotypic hand movements, hyperreflexia, spasticity, seizures, and scoliosis (Fig. 112). Scoliosis is usually progressive and seldom responds to brace management. Most require posterior fusion with segmental instrumentation.

 

 

Figure 112. Rett syndrome Deformity is severe and progressive, often requiring long fusion.

 

Down Syndrome

Trisomy-21 syndrome includes characteristic faces, congenital heart disease, mental retardation, and excessive joint laxity. Upper cervical instability involving the occipito-cervical and the atlantoaxial levels develop in many children. This instability results from joint and ligamentous laxity.

Clinical manifestations of cord compromise from instability include disturbances in gait, exercise intolerance, and neck pain. Mild weakness and hyperreflexia may be found. Screen with flexion-extension radiographs by ages 5–6 years.

Management Be concerned if ADI >5 mm. Follow yearly with examination and every several years with radiographs. Some recommend fusion with ADI >10 mm.

 

Torticollis

Torticollis, or wryneck, includes a variety of conditions (Fig. 113) that require different management.

 

 

Figure 113. Causes of torticollis The causes are many, but the vast majority of torticollis cases are due to disorders listed in the top three categories.

 

Acute Torticollis

Acute torticollis is relatively common. It may occur spontaneously, follow minimal trauma, or occur after an upper respiratory infection (Fig. 114). Why the head tilts is uncertain. The tilt may be due to muscle spasm secondary to cervical lymphadenitis or possibly due to a minor subluxation of the cervical vertebrae.

 

 

Figure 114. Acute torticollis This form of torticollis develops suddenly in a previously normal child. Usually the deformity resolves spontaneously in a day or two.

 

Clinical features Acute torticollis causes the head to tilt, rotate to one side and become fixed. Radiographs of the cervical spine are difficult to assess because of the lateral flexion and rotation. Laboratory studies are normal.

Manage by immobilizing the neck with a folded towel and encourage rest. Early management is usually provided by the primary care physician. In most children, acute torticollis resolves within 24 hours. If the deformity persists longer than 24–48 hours, be more concerned and manage as rotatory displacement.

Rotatory Displacement

The more severe form of acute torticollis is called rotatory displacement or rotatory subluxation. This may be associated with severe pharangitis or otitis media, or follow head and neck surgery or trauma; in some cases, it occurs spontaneously. Treat rotatory displacement early to avoid permanent fixation and residual deformity (Fig. 115).

 

 

Figure 115. Management of rotatory subluxation

 

Evaluate Determine the duration of the deformity and any associated history such as trauma or infections. Sometimes torticollis follows head or neck surgery. Patients with Marfan syndrome are susceptible. Examine the child for localized tenderness and neck range of motion. Perform a careful neurological examination. Be aware that spinal cord tumors may present with torticollis. Perform appropriate laboratory studies if infection is suspected. The value and reliability of conventional and dynamic CT scans are controversial and probably have little value in planning management.

Manage First apply traction. If early, head-halter traction is appropriate. In most children, the torticollis resolves with traction. If the deformity has persisted more than a week before resolution, consider extending the period of immobilization for 2 or 3 months using a Minerva cast (Fig. 116). For persisting deformity, halo traction or manipulation under anesthesia may be necessary. Should all these measures fail, operative repositioning and C1–C2 fusion may be necessary.

 

 

Figure 116. Minerva cast This form of immobilization may be useful in children with delayed resolution of rotatory subluxation or requiring reduction. This type of cast is sometimes useful to provide immobilization following operative correction. The Minerva cast is better tolerated by the child than the parents. It is most easily applied with the child sitting or standing.

 

Chronic Nonmuscular Torticollis

In about 20% of children with chronic torticollis, it is due to nonmuscular causes. Radiographs may show conditions such as Klippel-Feil anomaly or hemivertebrae. If radiographs are negative and the sternocleidomastoid muscle is not contracted, consider an ocular etiology. Refer to an ophthalmologist for evaluation. Consider the other conditions that may cause torticollis, such as neonatal brachial plexus palsies and spinal cord tumors, before starting treatment.

Muscular Torticollis

Muscular torticollis is relatively common and presents in two age groups.

Infantile muscular torticollis The infant (Fig. 117) is first seen because of a head tilt.

 

 

Figure 117. Muscular Torticollis This is the most commoeck problem in childhood. Torticollis is usually seen first in the infant (left). Some advocate treatment by stretching (right), but its value is uncertain.

 

Sometimes a history of a breech delivery is given and a firm tumor of the sternocleidomastoid muscle is palpated. Usually only a head tilt and limited neck motion due to a contracture of the muscle are found. Plagiocephaly (asymmetrical head) may be present (Fig. 118, 119).

 

 

 

Figure 118. Muscular torticollis and plagiocephaly The mass (red arrow) develops in early infancy and disappears spontaneously over a period of several months. The plagiocephaly (blue arrows) may persist longer.

 

 

Figure 119.  Plagiocephaly torticollis Cranial deformity is readily shown by CT scans. 3-D reconstructions provide graphic documentation of the extent of the deformity.

 

Be certain to rule out developmental hip dysplasia. Even if the hip examination is negative, evaluate the hip by either ultrasound if the infant is seen in the neonatal period or by a single AP radiograph of the pelvis if the infant is older than about 10 weeks of age.

Infantile torticollis resolves spontaneously in about 90% of cases. The value of physical therapy by stretching is uncertain [A, right]. Of those that persist, operative correction may be necessary. Delay correction until about 3 years of age. Plagiocephaly rarely persists and is a cosmetic problem.

Juvenile muscular torticollis Sometimes muscular torticollis appears to develop during childhood (Fig. 120). In this juvenile type, usually both heads of the muscles are contracted, causing the head tilt and limiting neck motion. This type of torticollis usually is permanent and often requires operative correction.

 

 

Figure 120. Sternocleidomastoid contracture Both the clavicular origin (red arrow) and sternal origin (blue arrow) are contracted.

 

Operative correction Bipolar release is the most effective procedure for correction of both infantile and juvenile forms of muscular torticollis.

 

Bipolar Release for Muscular Torticollis

Most cases of muscular torticollis resolve during infancy. If the deformity persists into childhood and poses a cosmetic disability, release is indicated. Bipolar release of the contracted sternocleidomastoid muscle is usually appropriate, as double-level release provides better correction with less risk of recurrence. Endoscopic releases have been recommended; however, the open release can be performed through small skin line incisions, which provide equivalent cosmetic results.

Preoperative Planning Make certain the diagnosis is muscular torticollis. Note the location of the contractures (Fig. 121). This boy shows the typical deformity with the clavicular head (red arrow) more contracted than the sternal head (blue arrow). If the deformity is severe, both distal heads should be released. If the deformity is moderate and if leaving the contour is considered important, the sternal head may be left intact.

 

Figure 121.

 

Technique Position the child with a towel under the upper chest to allow extension of the head to make the contracture prominent during the release. Avoid nerves and vessels during the release procedure (Fig. 122 – В). Although the array of these structures may seem worrisome, they can easily be avoided. The facial nerve lies well anterior, and the auricular artery and nerve can be avoided by careful isolation of each segment of scar before excision. The accessory nerve lies distant, midway between the incisions. Distally, the vein and nerve lie deep to the fascia. The strands of fibrotic scar are distributed randomly within the muscle (Fig. 122 – С).

 

 

Figure 122.

 

 Perform the proximal release first (Fig. 123). Make a small transverse incision over the proximal portion of the muscle.

 

 

Figure 123.

 

With the muscle under tension, identify the fibrotic strands and divide each. Make certain that the release is complete. Make the second short transverse skin line incision in the skin crease well above the clavicle (Fig. 124). The mobile skin allows an extensive excision through a small incision.

 

 

Figure 124.

 

Release the muscle, fibrous tissue, and investing fascia. Close only the skin with subcuticular sutures. Reinforce the closure with paper tape. Apply a bulky dressing. Once the child is awake, a slightly compressive overdressing may be applied.

After Care The child may be discharged the next day. Gentle stretching may be started within a few days. If the deformity is severe, apply a fiberglass jacket that includes the head (Minerva cast). Position the head in slight overcorrection in the cast. Cut windows for the face and ears. This cast may be applied in the clinic several days following discharge and should be worn for about 6 weeks. At cast removal, the incisions will be well healed (Fig. 125).

 

 

Figure 125.

 

At 3 months after surgery, the scars (Fig. 126) and the head tilt (Fig. 127) are acceptable.

 

 

 

Figure 126.

 

 

Figure 127.

 

This position is much better than the preoperative head tilt (Fig. 128).

 

 

Figure 128.

 

Complications are uncommon.

Residual head tilt is usually due to incomplete release or lack of postoperative range of motion or immobilization.

Bad scars may be due to excessive length, non-skin-line orientation, noncosmetic closure, or keloid formation.

Neurovascular damage can be avoided by careful technique.

Recurrence is uncommon and its cause may be uncertain.