This practice set contains high-yield board review questions covering key concepts in 6. Spine. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
Question 2981
Topic: 6. Spine
Figures 20a and 20b show lateral and AP radiographs of a 49-year-old man who sustained a gunshot wound through the left shoulder. He reports neck pain and examination reveals weakness in all four extremities. What is the priority of evaluation?
Correct Answer & Explanation
. Airway, breathing, and circulation
Explanation
The projectile entered the left shoulder and traveled to the right neck; therefore, a high incidence of suspicion must be directed to the airway, great vessels of the neck, and contents of the mediastinum. Immediate assessment of airway, breathing, and circulation takes priority, followed by examination of the neurologic status and other systems, as determined by the examination findings. Subcommittee on ATLS of the American College of Surgeons Committee on Trauma 1993-1997, Spine and Spinal Cord Trauma; Advanced Trauma Life Support Student Manual, ed 6, 1997. International Standards for Neurological and Functional Classification of Spinal Cord Injury. American Spinal Injury Association and International Medical Society of Paraplegia (ASIA/IMSOP).
Question 2982
Topic: Thoracolumbar Spine & Deformity
A 12-year-old girl who is Risser stage 3 has had intermittent mild midback pain for the past 4 weeks. The pain is worse after prolonged sitting and after carrying a heavy backpack at school. She occasionally takes acetaminophen, but the pain does not limit sport activities. Examination reveals a mild right rib prominence during forward bending. Neurologic examination is normal. Radiographs show a 20-degree right thoracic scoliosis with no congenital anomalies or lytic lesions. Management should consist of
Correct Answer & Explanation
. back muscle stretching and reduced weight in the backpack.
Explanation
Mild scoliosis is not a painful condition, but it usually presents during adolescence. Intermittent back pain is reported by 25% to 30% of adolescents whether or not scoliosis is present. Such pain is often attributed to muscle strain from tight muscles, poor posture, or heavy school backpacks. The clinician must distinguish typical pain (mild, intermittent, nonlimiting) from atypical pain. The latter requires more careful examination and imaging studies (bone scan or MRI) to determine the source of pain. The patient's age and right thoracic curve pattern are typical for idiopathic scoliosis; therefore, imaging of the neuroaxis is not necessary to look for cord syrinx, tethering, or tumor. Brace treatment is not required for this small curve unless future progression is demonstrated. Ramirez N, Johnston CE, Browne RH: The prevalence of back pain in children who have idiopathic scoliosis. J Bone Joint Surg Am 1997;79:364-368. Hollingworth P: Back pain in children. Br J Rheum 1996;35:1022-1028.
Question 2983
Topic: 6. Spine
A 14-year-old girl with a right thoracic curve from T4 through L2 measuring 78 degrees is scheduled to undergo posterior spinal fusion for scoliosis. The surgical plan is to fuse from T3 through L2, using pedicle screws at L2 and about the apex at T8. What neural monitoring modality is most likely to identify a reversible neurologic deficit during surgery?
Correct Answer & Explanation
. Somatosensory-evoked potentials of the upper extremities
Explanation
Neural monitoring during scoliosis surgery was initially developed to avoid the devastating effects of spinal cord injury, particularly paraplegia. Monitoring in some form has become standard for this type of surgery. Somatosensory-evoked potentials in the lower extremities will detect many but not all neurologic difficulties with the spinal cord. Anterior spinal cord vascular disruption also can be detected by monitoring motor potentials. Electromyography following stimulation of lumbar pedicle screws can prevent nerve root injury that is the result of misplacement of the screws. This is best documented in the lumbar spine and has not been routinely used in the thoracic spine. The most common neural deficits following spinal surgery, however, are in the upper extremities because of the positioning of the patient in the prone position for long periods. In Schwartz and associates series of 500 patients, impending upper extremity neural injury was detected by somatosensory-evoked potentials in 18 (3.6%) patients. In contrast, lower extremity deficits were detected by combined motor- and sensory-evoked potentials in only 2 (0.4%) out of 500 patients in Padberg and associates series. Neural compression in the upper extremity can be easily detected by somatosensory-evoked potentials, and injury can be prevented by repositioning the patient. Padberg AM, Wilson-Holden TJ, Lenke LG, Bridwell KH: Somatosensory- and motor-evoked potential monitoring without wake-up test during idiopathic scoliosis surgery: An accepted standard of care. Spine 1998;23:1392-1400.
Question 2984
Topic: 6. Spine
Figures 11a and 11b show the T2-weighted MRI scans of the lumbar spine of a 53-year-old woman who has low back and right lower extremity pain. What structure is the arrow pointing to in Figure 11a?
Correct Answer & Explanation
. Lumbar synovial cyst
Explanation
The arrow is pointing to a cystic-appearing structure with high signal intensity on T2-weighted image sequencing. It appears to be contiguous with the hypertrophied right facet joint, which appears to also have high signal intensity. The mass significantly narrows the right lateral recess. The high signal intensity suggests that this is a fluid-filled mass. In addition, the facet joints are degenerative and there is a very mild degree of anterolisthesis on the sagittal image. These findings make a lumbar synovial cyst the most likely diagnosis. Most lumbar juxtafacet cysts are observed at the L4-5 level, extradurally and adjacent to the degenerative facet joint. They may contain synovial fluid and/or extruded synovium. Presentation is indistinguishable from that of a herniated disk. The etiology of spinal cysts remains unclear, but there appears to be a strong association between their formation and worsening spinal instability. They occasionally regress spontaneously and may respond to aspiration and injection of corticosteroids, though there is a high recurrence rate with nonsurgical management. Synovial cysts resistant to nonsurgical management should be treated surgically. If the patient's symptoms can be attributable to radicular findings, a microsurgical decompression that limits further destabilization should suffice. However, if there is significant low back pain attributable to spinal instability, decompression and fusion remains an appropriate option. Banning CS, Thorell WE, Leibrock LG: Patient outcome after resection of lumbar juxtafacet cysts. Spine 2001;26:969-972. Deinsberger R, Kinn E, Ungersbock K: Microsurgical treatment of juxta facet cysts of the lumbar spine. J Spinal Disord Tech 2006;19:155-160.
Question 2985
Topic: 6. Spine
A 42-year-old woman who has had an 18-month history of severe low back pain is referred to your office for surgical evaluation. She reports that the pain initially began with right lower extremity pain and management consisted of oral analgesics, nonsteroidal anti-inflammatory drugs, and muscle relaxants. She has seen a chiropractor as well as a pain management specialist and she is status-post epidural steroid injections. She has also completed exhaustive physical therapy, as she is a certified athletic trainer and runs a health fitness program at a community hospital. Currently, she denies lower extremity pain and her pain is isolated to her low back and is subjectively graded as 8/10, with 10 being the worst pain she has ever experienced. The pain is interfering with her activities of daily living and she is seeking definitive treatment. Figures 32a through 32c show current MRI scans. Based on the current available medical literature, what is the most appropriate treatment?
Correct Answer & Explanation
. Lumbar spinal fusion at L5-S1
Explanation
The MRI scans reveal advanced degenerative disk disease at L5-S1. Nonsurgical management has failed to provide relief and the patient is quite debilitated as a result of her back pain. Fritzell and associates demonstrated that in a well-informed and selected group of patients with severe low back pain, lumbar fusion can diminish pain and decrease disability more efficiently than commonly used nonsurgical treatments. In a recent updated Cochrane Review of surgery for degenerative lumbar spondylosis, it was noted that while Fritzell and associates appeared to provide strong evidence in favor of fusion, a more recent trial by Brox and associates demonstrated no difference between those patients undergoing lumbar fusion compared to those receiving cognitive intervention and exercise. The Cochrane Review suggests that this may reflect a difference between the control groups. Fritzell and associates compared lumbar fusion to standard 1990s "usual care," whereas Brox and associates compared lumbar fusion to a "modern rehabilitation program." Bear in mind that this patient is a certified athletic trainer and runs a hospital health fitness department; therefore, at least for purposes of this question, it can be assumed that she has participated in a "modern rehabilitation program." The Cochrane Review goes on to state that preliminary results of three small trials of intradiskal electrotherapy suggest that it is ineffective and that preliminary data from three trials of disk arthroplasty do not permit firm conclusions. Gibson JN, Waddell G: Surgery for degenerative lumbar spondylosis: Updated Cochrane Review. Spine 2005;30:2312-2320. Fritzell P, Hagg O, Wessberg P, et al: 2001 Volvo Award Winner in Clinical Studies: Lumbar fusion versus nonsurgical treatment for chronic low back pain: A multicenter randomized controlled trial from the Swedish Lumbar Spine Study Group. Spine 2001;26:2521-2532.
Question 2986
Topic: 6. Spine
A 46-year-old man has incomplete paraplegia after being involved in a motor vehicle accident. The CT scan shown in Figure 5 reveals marked canal compromise. What is the most appropriate management to improve neurologic status?
Correct Answer & Explanation
. Anterior L2 corpectomy and iliac crest strut grafting, with or without posterior instrumentation and fusion from L1 to L3
Explanation
According to a study by the Scoliosis Research Society, the use of anterior decompression is most predictable for improving neurologic status. This is particularly true of bowel and bladder functional loss. Laminectomy is contraindicated because it further destabilizes the spine. Posterior instrumentation and indirect reduction through distraction and ligamentotaxis only incompletely decompress the compromised canal and are successful only if performed within 48 hours of injury. While some improvement may occur with closed management, the amount of recovery is less than that achieved with surgical decompression. A posterior approach and instrumentation may be added to the anterior decompression based on the characteristics of associated injuries to the posterior element. Gertzbein SD: Scoliosis Research Society multicenter spine fracture study. Spine 1992;17:528-540. Garfin SR, Vaccaro AR (eds): Orthopaedic Knowledge Update: Spine. Rosemont, IL, American Academy of Orthopaedic Surgeons, 1997, pp 197-215.
Question 2987
Topic: 6. Spine
A 21-year-old woman who was wearing a seat belt sustained an injury of the thoracolumbar junction in a motor vehicle accident. The AP radiograph shows widening between the L1 and L2 spinous processes, and the CT scan shows the empty facet sign at this level. The initial evaluation should include
Correct Answer & Explanation
. CT of the abdomen.
Explanation
The patient has a flexion-distraction injury of the thoracolumbar spine that is often associated with wearing a seat belt. The fracture has a high risk of associated intra-abdominal injury; therefore, the initial evaluation should include a CT of the abdomen. The most common visceral injury is to the bowel. Smith WS, Kaufer H: Patterns and mechanisms of lumbar injuries associated with lap seat belts. J Bone Joint Surg Am 1969;51:239-254.
Question 2988
Topic: 6. Spine
Examination of a 34-year-old man who has had left leg pain for the past 6 weeks reveals minimal weakness of the left extensor hallucis longus and normal ankle jerk and patellar reflexes. Figure 33 shows an axial MRI scan of the L4-5 disk. Based on these findings, the MRI scan results are consistent with compression of the
Correct Answer & Explanation
. traversing L5 nerve root and the patient's history and examination.
Explanation
The patient has an L5 radiculopathy secondary to an L4-5 disk herniation that is compressing the traversing L5 nerve root.
Question 2989
Topic: 6. Spine
A 14-year-old football player has had thigh pain and weakness following a full-contact scrimmage 24 hours ago. He recalls that he felt a sharp pain in his back after colliding with a much heavier player. Examination reveals that the spine is minimally tender to palpation in the upper lumbar region. Motor testing reveals quadriceps weakness bilaterally, and a reverse straight leg raising test is positive. Plain radiographs of the thoracolumbar spine are normal. A myelogram, a CT scan with contrast, and an MRI scan are shown in Figures 41a through 41c. What is the most likely diagnosis?
Correct Answer & Explanation
. Vertebral end plate fracture
Explanation
Fracture of the vertebral end plate is a relatively uncommon injury that is most often seen in adolescent boys. The injury is characterized by traumatic displacement of the vertebral ring-apophysis into the spinal canal and associated disk herniation. Over one third of these injuries are seen in children with lumbar Scheuermann disease. The injury most frequently involves the midlumbar vertebra, and symptoms are often indistinguishable from those associated with a herniated disk. The injury is usually not visible on plain radiographs. The diagnosis is typically made after obtaining MRI or contrast CT scans. Treatment consists of laminotomy and excision of the osteochondral fragments. Beaty JH (ed): Orthopaedic Knowledge Update 6. Rosemont, Ill, American Academy of Orthopaedic Surgeons, 1999, pp 635-651.
Question 2990
Topic: 6. Spine
A 53-year-old man reports acute, severe left shoulder pain after undergoing abdominal surgery 10 days ago. Initial management, consisting of anti-inflammatory drugs, physical therapy, and a subacromial injection of corticosteroid, fails to provide relief. Reexamination of the shoulder 2 months after the onset of symptoms reveals atrophy of the infraspinous and supraspinous fossa and profound weakness of active abduction and external rotation. His neck is supple with a full range of motion. Plain radiographs and an MRI scan of the shoulder are normal. What diagnostic study should be performed next in the evaluation of this patient?
Correct Answer & Explanation
. Electromyography and nerve conduction velocity studies
Explanation
Suprascapular nerve palsy is a fairly uncommon yet well-known cause of shoulder pain and weakness. A variety of causes have been described, including compression by a ganglion cyst, an anomalous or thickened superior transverse scapular ligament, a humeral and scapular fracture, and traction or kinking of the nerve in the suprascapular notch. In this patient, the injury is most likely caused by traction or compression of the nerve in the suprascapular notch as the result of positioning during abdominal surgery; therefore, the studies of choice are electromyography and nerve conduction velocity studies. While MRI of the cervical spine may be of some value in ruling out a radiculopathy, the clinical history does not support such a cause for this condition. Rengachary SS, Neff JP, Singer PA, Brackett CE: Suprascapular entrapment neuropathy: A clinical, anatomical, and comparative study. Part 1: Clinical study. Neurosurgery 1979;5:441-446. Rengachary SS, Burr D, Lucas S, Hassanein KM, Mohn MP, Matzke H: Suprascapular entrapment neuropathy: A clinical, anatomical and comparative study. Part 2: Anatomical study. Neurosurgery 1979;5:447-451.
Question 2991
Topic: 6. Spine
A 21-year-old woman with Marfan syndrome is seeking evaluation of her scoliosis. She reports no back or leg pain, and the neurologic examination is normal. Lateral and bending radiographs are shown in Figures 7a through 7e. Management should consist of
Correct Answer & Explanation
. anterior spinal fusion from T10 to L4.
Explanation
Because the patient's thoracolumbar scoliosis is of a large enough magnitude, observation or bracing is not recommended. The thoracolumbar curve is flexible enough and L4 corrects well enough to the pelvis to consider anterior spinal fusion from T10 to L4. Garfin SR, Vaccaro AR (eds): Orthopaedic Knowledge Update: Spine. Rosemont, IL, American Academy of Orthopaedic Surgeons, 1997, pp 161-171. Turi M, Johnston CE II, Richards BS: Anterior correction of idiopathic scoliosis using TSRH instrumentation. Spine 1993;18:417-422.
Question 2992
Topic: 6. Spine
A 10-year-old girl has been referred for evaluation of a prominence at the lower cervical spine. The patient is asymptomatic, and the examination reveals no evidence of neurologic abnormality. A radiograph and CT scans are shown in Figures 12a through 12c. What is the most likely diagnosis?
Correct Answer & Explanation
. Tuberculosis
Explanation
Tuberculosis is uncommon in the cervical spine but has a relatively greater incidence in young children. In a review of 40 patients with lower cervical spine involvement (C2 to C7), 24 were younger than age 10 years at presentation. In children, the disease is characterized by more extensive involvement with the formation of large abscesses. In older patients with lower cervical tuberculosis, the disease is more localized but is more likely to cause paraplegia. Four-drug antituberculosis therapy should be used. For patients with pain or neurologic dysfunction, anterior excision of diseased bone and grafting are indicated. Whether vertebral body excision and grafting should be done in an asymptomatic 10-year-old child is debatable. The CT scan shows a large "cold" abscess that is partially calcified. Hsu LC, Leong JC: Tuberculosis of the lower cervical spine (C2 to C7): A report on 40 cases. J Bone Joint Surg Br 1984;66:1-5.
Question 2993
Topic: 6. Spine
After making a tackle, a football player is found prone and unconscious without spontaneous respirations. Initial management should consist of
Correct Answer & Explanation
. head and neck stabilization, log roll to a supine position, face mask removal, and initiation of assisted breathing.
Explanation
The on-field evaluation and management of a seriously injured athlete requires that health care teams have a game plan in place and proper equipment that is readily available. The initial step, which consists of stabilizing the head and neck by manually holding them in a neutral position, is then followed by assessment of breathing, pulses, and level of consciousness. If the athlete is breathing, management should consist of mouth guard removal and airway maintenance. If the athlete is not breathing, the face mask should be removed, with the chin strap left in place. The airway must be established, followed by initiation of assisted breathing. CPR is instituted only when breathing and circulation are compromised. In the unconscious athlete or if a cervical spine injury is suspected, the helmet must not be removed until the athlete has been transported to an appropriate facility and the cervical spine has been completely evaluated. McSwain NE, Garnelli, RL: Helmet removal from injured patients. Bull of Am Coll Surg 1997;82:42-44.
Question 2994
Topic: 6. Spine
A 53-year-old woman has severe neck and left shoulder pain after a rollover motor vehicle accident. Radiographs and a CT scan of the cervical spine are shown in Figures 34a through 34c. Management should consist of
Correct Answer & Explanation
. lateral mass plate fixation at C4-C6.
Explanation
The plain radiographs show a horizontal orientation of the C5 facet joint. The CT scan through C5 reveals an ipsilateral pedicle and lamina fracture (floating facet). This injury involves two adjacent motion segments and is extremely unstable. Lateral mass plates, with or without the purchase of the "floating facet," provide the best means of stabilization and should include the facet above (C4) and below (C6) the level of injury. Orthotic immobilization is insufficient for this particular injury. Halo vest treatment does not control the subaxial spine well and is of limited value. While simple midline (Rogers) wiring provides some tension band restoration, it is not optimal for rotational control. The use of lateral mass plates provides rotational stability. Another option would be anterior fusion and plating, which would save cervical segments. Levine AM, Mazel C, Roy-Camille R: Management of fracture separations of the articular mass using posterior cervical plating. Spine 1992;17:S447-S454. Levine AM: Facet fractures and dislocations, in Levine AM, Eismont FJ, Garfin S, Zigler JE (eds): Spine Trauma. Philadelphia, PA, WB Saunders, 1998, pp 360-362. Whitehill R, Richman JA, Glaser JA: Failure of immobilization of the cervical spine by the halo vest: A report of five cases. J Bone Joint Surg Am 1986;68:326-332.
Question 2995
Topic: 6. Spine
A 65-year-old man with ankylosing spondylitis has neck pain after falling back over his lawnmower, striking his thoracic spine, and forcing his neck into extension. Examination reveals subtle weakness of the intrinsics and finger flexors at approximately 4+/5. Initial management consists of immobilization in a rigid collar, and placing his head in the anatomic position. Radiographs reveal a subtle extension fracture of the lower cervical spine. Approximately 6 hours after the injury, he reports increasing paresthesias in his upper and lower extremities, and examination now shows his intrinsics are 2/5, finger flexors are 3/5, and his triceps are now weak at 4/5 on manual motor testing. In addition, his lower extremities now show weakness in both dorsal and plantar flexion of the ankle in the range of 4/5. Repeat radiographs appear unchanged. An MRI scan is shown in Figure 2. Management should now consist of
Correct Answer & Explanation
. posterior laminectomy and spinal fusion.
Explanation
It is not uncommon for patients with ankylosing spondylitis to sustain extension-type fractures, most typically of the cervicothoracic junction. These fractures can appear nondisplaced or minimally displaced initially, making them difficult to diagnose. Because there is no mobility between vertebrae, fractures tend to occur more like those of a transverse fracture of a long bone. In addition, the vertebral bodies are vascular and their canals are relatively enclosed, making them vulnerable to epidural bleeding. The MRI scan reveals an epidural hematoma located posteriorly on the cord; therefore, the treatment of choice is surgical evacuation and a posterior laminectomy. Because of the intrinsic instability of such fractures at the time of the laminectomy, internal fixation and stabilization with a posterior fusion is warranted. A simple laminectomy will only increase instability, and control is unlikely with halo vest immobilization. An anterior procedure will not effectively treat the problem given the location of the hematoma. Consideration can be given to methylprednisolone and observation; however, this will not eradicate the problem. Bohlman HH: Acute fractures and dislocations of the cervical spine. J Bone Joint Surg Am 1979;61:1119-1142.
Question 2996
Topic: 6. Spine
A patient with myelopathy underwent a one-level corpectomy 1 day ago and is now home. In the middle of the night he calls to report markedly increased difficulty in swallowing, diaphoresis, a change in his voice, and difficulty lying flat. What is the best course of action?
Correct Answer & Explanation
. Advise immediate transport to the emergency department for evaluation of the airway, possible intubation, and possible cricothyroidotomy.
Explanation
The patient has respiratory distress as manifested by his difficulty in lying flat. In addition, the diaphoresis and the change in his voice indicate retropharyngeal edema or hematoma that is compressing his larynx. The only appropriate treatment is hospital admission and elective intubation. During intubation it is possible to cause laryngospasm in a patient with a hyperacute airway; therefore, the surgeon should be prepared to perform a cricothyroidotomy. Often a fiberoptically guided intubation is the only way to find the airway in the presence of retropharyngeal edema or hematoma. Emery SE, Smith MD, Bohlman HH: Upper-airway obstruction after multilevel cervical corpectomy for myelopathy. J Bone Joint Surg Am 1991;73:544-551.
Question 2997
Topic: 6. Spine
A 38-year-old man reports a 2-week history of acute lower back pain with radiation into the left lower extremity. There is no history of trauma and no systemic signs are noted. Examination reveals a positive straight leg test at 35 degrees on the left side and a contralateral straight leg raise on the right side. Motor testing demonstrates mild weakness of the gluteus medius and weakness of the extensor hallucis longus of 3+/5. Sensory examination demonstrates decreased sensation along the lateral aspect of the calf and top of the foot. Knee and ankle reflexes are intact and symmetrical. Radiographs demonstrate no obvious abnormality. MRI scans show a posterolateral disk hernation. The diagnosis at this time is consistent with a herniated nucleus pulposus at
Correct Answer & Explanation
. L4-5.
Explanation
The patient's history and physical examination findings are consistent with a lumbar disk herniation at the L4-5 level. Weakness of the extensor hallucis longus and gluteus medius are consistent with an L5 lumbar radiculopathy. Nerve root tension signs are also consistent with sciatica from a lumbar disk herniation. The MRI scans confirm a posterolateral disk herniation at L4-5, which typically affects the exiting L5 nerve root. Hoppenfeld S: Orthopedic Neurology. Philadelphia, PA, JB Lippincott, 1977, pp 45-74.
Question 2998
Topic: 6. Spine
Figures 22a and 22b show the radiograph and sagittal MRI scan of the upper cervical spine of a 62-year-old woman who has had a long history of rheumatoid arthritis. Following hospitalization and skeletal traction, her symptoms improve significantly, her neurologic examination returns to normal, and repeat radiographs show a normal occiput and C1-C2 relationship. Treatment should now include
Correct Answer & Explanation
. occipitocervical stabilization.
Explanation
Although opinions differ on whether a decompression is indicated in a patient with symptomatic basilar invagination, it is generally agreed that occipitocervical stabilization is indicated. This has been done with and without concomitant arthrodesis. Crockard HA, Grob D: Rheumatoid arthritis upper cervical involvement, in Clark CR (ed): The Cervical Spine, ed 3. Philadelphia, PA, Lippincott Raven, 1998, p 701.
Question 2999
Topic: 6. Spine
A 39-year-old man reports low back pain, lower extremity numbness, and urinary retention after being injured in a motor vehicle accident 1 day ago. He is able to walk but is in pain. A straight leg raise results in increased back pain, and examination reveals that perianal sensation is decreased. Placement of a urinary catheter results in 500 mL of urine. What is the next most appropriate step in management?
Correct Answer & Explanation
. Emergent MRI
Explanation
Acute cauda equina syndrome, including saddle hypesthesia and bowel/bladder incontinence, is a red flag that demands emergent evaluation with MRI and urgent surgery if compression is confirmed. Results appear to be improved if surgery is performed within 48 hours. The other treatment approaches listed are not indicated if a cauda equina syndrome is present. Ahn UM, Ahn NU, Buchowski JM, et al: Cauda equina syndrome secondary to lumbar disc herniation: A meta-analysis of surgical outcomes. Spine 2000;25:1515-1522. Shapiro S: Medical realities of cauda equina syndrome secondary to lumbar disc herniation. Spine 2000;25:348-351.
Question 3000
Topic: 6. Spine
A 12-year-old girl has scoliosis at T5-T10 that measures 62 degrees. A clinical photograph of the axilla is shown in Figure 56. Management should consist of
Correct Answer & Explanation
. anterior and posterior spinal fusion with posterior segmental instrumentation.
Explanation
Neurofibromatosis type 1 (NF-1) is an autosomal-dominant disorder affecting about 1 in 4,000 people. NF-1 causes tumors to grow along various types of nerves and affects the development of non-nervous tissues, such as bone and skin. The gene for NF-1 is located on the long arm of chromosome 17 and codes the protein neurofibromin. Research indicates that NF-1 acts as a tumor-suppressor gene and, as such, plays an important role in the control of cell growth and differentiation. Axillary and inguinal freckling is considered a good diagnostic marker for NF-1. The hyperpigmented spots that measure from 2 mm to 4 mm may be congenital, but these typically appear and increase later in life. Scoliosis is the most common musculoskeletal disorder of NF-1. The curves are frequently dystrophic, kyphotic, and have a high risk of pseudarthrosis following spinal fusion. Anterior and posterior spinal fusion with rigid posterior segmental instrumentation is the treatment of choice. Goldberg Y, Dibbern K, Klein J, Riccardi VM, Graham JM Jr: Neurofibromatosis type 1: An update and review for the primary pediatrician. Clin Pediatr 1996;35:545-561.
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