EMERGI-QUIZ CASE PRESENTATION
A Teenage Girl with Acute Back Pain Jennifer R. Marin, MD
A
14-year-old previously healthy Hispanic female presents to the emergency department with a weeklong history of back pain. Her symptoms began 4 days before arrival when she complained to her mother of lower back pain. She reported that her pain was sudden in onset and not precipitated by trauma. The pain was described as midline between her hips, and it wakes her up from sleep. She had decreased mobility and difficulty walking. She had not tried any over-the-counter analgesics for the pain. She also began having abdominal pain and fevers to 100.38F (37.98C) for 2 days before arrival. The abdominal pain was diffuse and constant. She had no nausea or vomiting, and although she had been eating less than usual, she maintained her usual urine output. She did not have a history of constipation but could not recall when she had her last bowel movement. She had no dysuria. She denied sexual activity, and her last menstrual period was 2 weeks prior. She had no weight loss, no night sweats, and no travel or tuberculosis exposures. She had no bowel or bladder incontinence, no numbness or tingling of the lower extremities, and felt her mobility was limited by her pain and not by weakness. On examination, her temperature was 36.88C, with a heart rate of 80 beats/min, respiratory rate of 20, and blood pressure of 118/74. She was lying flat on the examination table, quiet, and reluctant to move. Her head examination was negative for oropharyngeal lesions, her neck was supple, and there was no point tenderness of her cervical spine region. Her breath sounds were symmetric and clear bilaterally. Her heart rate was regular, and there was no evidence of a murmur or extra heart sounds. Her abdominal examination revealed bowel
Presented at the Section on Emergency Medicine Emergi-Quiz competition at the American Academy of Pediatrics National Convention and Exposition, Atlanta, GA, October 7, 2006. Department of Pediatrics, Division of Emergency Medicine, Children’s Hospital of Philadelphia, Philadelphia, PA. Reprint requests and correspondence: Jennifer R. Marin, MD, Department of Pediatrics, Division of Emergency Medicine, Children’s Hospital of Philadelphia, 34th and Civic center Blvd, Philadelphia, PA 19104. (E-mail:
[email protected]) 1522-8401/$ - see front matter ª 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.cpem.2007.02.007
sounds, and a soft but diffusely tender abdomen. There were no masses, and her liver edge was palpable just below the costal margin. The spleen was nonpalpable. She had full range of motion of her hips, and straight leg raise test was negative. There was no costovertebral angle tenderness, and on examination of her spine, there was no erythema, edema, bruising, or point tenderness of the spinous processes or paraspinous muscles. She was unable to flex, extend, or rotate her back. Neurologically, she was alert and oriented, her cranial nerves (II-XII) were symmetric and intact, she had 5/5 upper and lower extremity strength, and 2+ deep tendon reflexes of the upper and lower extremities. Her toes were down-going bilaterally, and sensation was grossly intact. She had good rectal tone, with no evidence of stool in the rectal vault. She was reluctant to walk but was able to with a slightly antalgic gait. Initial laboratory results revealed a white blood cell count of 4100, with a differential of 53% neutrophils, 43% lymphocytes, and 0% bands. Hemoglobin was 12.9 g/dL, and platelet count was 117,000. C-reactive protein was 1.3 mg/dL, and erythrocyte sedimentation rate was 43 mm/h. Urinalysis was unremarkable. Plain films of her lumbosacral spine were negative, and an abdominal x-ray showed air throughout the intestines. Blood and urine cultures were sent. The patient was admitted to the hospital, and the following morning, another test was performed, which revealed a potential diagnosis.
Differential Diagnosis The differential diagnosis of a child with back pain is broad but deserves careful scrutiny, as 60% of back pain in children is due to an organic etiology, in stark contrast to adults [1]. Younger children are more likely to have organic pathology. Arriving at the diagnosis may be difficult because children are less able to describe their pain and, in younger children, even to localize it. Hallmarks of organic disease in children are acute back pain, especially with lack of trauma, and chronic, progressive pain that limits a child’s mobility. Any of these findings deserve a thorough evaluation. 65
J. Marin
66 Table 1
Differential diagnosis.
Traumatic Spondylolysis Spondylolisthesis Disk degeneration Vertebral compression fracture Muscular strain Structural Scoliosis Scheuermann’s disease Neoplastic Osteoid osteoma Leukemia Lymphoma Metastatic tumor Neurofibroma Osteoblastoma Aneurysmal bone cyst Referred pain Pyelonephritis Pneumonia Pancreatitis Cholecystitis Pelvic inflammatory disease Infectious Diskitis Vertebral osteomyelitis Epidural abscess Paraspinous abscess Appendicitis (retrocecal) Psoas abscess Rheumatologic Ankylosing spondylitis Reiter disease Juvenile rheumatoid arthritis Data from Pediatr Clin North Am 1996;43:899-918 and UpToDate.com August 2006.
It is helpful to consider the causes of back pain by system or category, so as not to overlook an important process. The most common causes in children include traumatic, infectious, oncologic, and diseases causing referred pain (Table 1). One should be able to obtain a history of a traumatic injury in children who present with back pain secondary to compression fractures, as may be seen in an axial load injury. This injury presents with localized pain over the affected vertebrae. Radicular pain may develop and may occasionally be so severe as to cause a sympathetic ileus and vomiting. The signs of muscular back strain and lumbar disk herniation are similar to those in adults, but in adolescence, these diagnoses should be made with caution, and in childhood, they are distinctly rare [2]. Spondylolysis is a common injury in adolescent athletes and is essentially a stress fracture of the posterior arch of the vertebra. It represents a defect in the pars interarticularis, and if accompanied by forward translation of one vertebra relative to another is called spondylolisthesis.
With these 2 conditions, back pain is most often felt in the lower lumbar area. Infectious causes of back pain often require a timesensitive diagnosis because delay may cause dissemination of the infection, or local, irreversible damage. An epidural abscess represents a neurosurgical emergency and usually presents with back pain, fever, and tenderness to percussion of the vertebra over the site of involvement. Signs of spinal cord compression may or may not be present. There is usually a preceding history of trauma to that area in otherwise healthy children. Diskitis represents inflammation of the intervertebral disk and is a relatively rare disease of childhood. It occurs in children less than 3 years old; however, cases may occur through adolescence [3]. Diskitis usually presents gradually with children being irritable and complaining of back pain or refusal to walk. Occasionally, a fever is present, but children are generally not toxic appearing. In some patients, abdominal pain may be the only complaint. In contrast to diskitis, children with vertebral osteomyelitis are older and more likely to be febrile and ill-appearing at the time of presentation [4]. Back pain from an oncologic process may result from a primary musculoskeletal or spinal cord tumor, including Ewing sarcoma, neuroblastoma, meningioma, and ependymoma; from metastatic disease; or from a nonsolid organ malignancy, such as leukemia or lymphoma. The most common neoplasm that presents with back pain in children is osteoid osteoma, a benign bone tumor characterized by nocturnal pain and prompt relief with nonsteroidal anti-inflammatory drugs. Other types of benign bone tumors and tumor-like lesions of the spine include osteochondroma, osteoblastoma, aneurysmal bone cyst, eosinophilic granuloma, hemangioma, and giant cell tumor [5]. It is important to remember that back pain may be the result of referred pain from a nearby site of pathology. Pneumonia, appendicitis, and pyelonephritis are a few common causes of referred back pain in children. In the emergency department, the physical examination of a child with back pain should focus on the neurologic examination. Any concerns for spinal cord involvement should prompt a neurosurgical evaluation. Initial evaluation may consist of a complete blood count, inflammatory markers, urinalysis, plain films of the spine, and, depending on the index of suspicion, magnetic resonance imaging (MRI), and/or bone scan.
Case Progression and Diagnosis The morning after admission, an MRI of the spine was obtained and revealed abnormal enhancement of the L3 and vertebral body with mild enhancement of the paraspinous soft tissues (Figure 1). Orthopedics was consulted, and in concert with radiology, vertebral osteomyelitis was felt to be the most likely diagnosis.
A teenage girl with acute back pain
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Figure 1 MRI of the lumbar spine (A) sagittal and (B) axial images demonstrating abnormal enhancement of the L3 vertebral body.
The patient was started on intravenous cefazolin to cover the most likely cause of osteomyelitis in this age group, Staphylococcus aureus. The diagnosis of Pott’s disease was entertained given the location of enhancement on MRI, and her purified protein derivative, chest x-ray, and blood for acid fast bacilli were negative. In an attempt to isolate an organism, she underwent computed tomography–guided biopsy of the L4 vertebra, and bacterial, fungal, and mycobacterial cultures were all negative. Throughout her initial hospital course, daily laboratory studies were drawn, which showed an interesting trend. Despite antibiotic therapy, her C-reactive protein continued to rise to 6.5 mg/dL. Her white blood cell count dropped from 4000 to 3000, her hemoglobin fell from 12.9 to 11.6 g/dL, and her platelets reached a nadir of 88,000. At this point, she was evaluated for malignancy, and a bone marrow biopsy revealed a hypercellular bone marrow with blasts. She was ultimately diagnosed with acute lymphoblastic leukemia (ALL).
Acute Lymphoblastic Leukemia Acute lymphoblastic leukemia is the most common malignancy in children and is responsible for about 25% of childhood cancers in those younger than 15 years [6]. The peak incidence is between 2 and 5 years of age [7]. Age is an important prognostic factor in the outcome of treatment for ALL. Current therapies have resulted in 80% event-free survival for children 1 to 10 years of age. Adolescents have not uniformly benefited from the improved survival seen in childhood ALL, with a 59% 6-year event-free survival reported in 1993 [8].
Most patients with ALL will present with abnormal peripheral blood counts; however, 10% of patients will present with a normal complete blood count. The complete blood count will often demonstrate a leukocytosis, with anemia, and/or thrombocytopenia. However, about half of patients will present with a white blood cell count less than 10,000, and about 20% will present with a platelet count of more than 100,000 [7]. Blasts are generally not present when the white count is not elevated. Patients who present with ALL will often have organomegaly, fever, and lymphadenopathy. Abdominal pain can be seen in approximately 10% of patients presenting with ALL [7]. Bone or joint pain is seen in about 30% of patients [7] but is rarely the only presenting feature. Characteristically, the pain will not respond to anti-inflammatory medications. In children, long bones are more often affected than the axial skeleton [6]. Skeletal lesions occur more often in children with leukemia than in adults because a child’s small reserve can be rapidly replaced by leukemic cells. It is not uncommon for patients with ALL who present with bone pain to be misdiagnosed as having osteomyelitis, septic arthritis, diskitis, and arthritis. In fact, many of these patients have a delay in diagnosis for up to 7 weeks [9]. Jonsson et al [10], in a retrospective review of 300 cases of ALL in children, found that patients with prominent bone pain preceding the diagnosis frequently had nearly normal hematologic values, and that this feature may contribute to a delay in diagnosis. There are only a few published case series of patients with ALL who presented with back pain as their chief
68 complaint. Pandya et al [11] described 3 children with ALL, 7 to 10 years old, who presented with back pain and kyphosis. A 2005 case series published by Kobayashi et al [12] presented 16 patients with leukemia or lymphoma who had bone pain; 4 patients had back pain, and 12 had a normal complete blood count.
Summary Initially, when presented with a child with back pain, it is important to consider a broad and complete differential diagnosis because back pain in children is often the sign of a pathological process. The red flags to be aware of when evaluating a child with back pain are acute or progressive back pain, limitation of activity, any neurologic signs or symptoms, and pain unresponsive to anti-inflammatory drugs. Malignancy is an important consideration in children with back pain, and evidence of at least 2 abnormal cell lines on a complete blood count requires evaluation for an oncologic process. Although rare, back pain may be the initial and only manifestation of leukemia.
References 1. Carriere B, Cummins-Mcmanus B. Vertebral fractures as initial signs for acute lymphoblastic leukemia. Pediatr Emerg Care 2001;17:258261.
J. Marin 2. Corneli H Pain: back. In Fleischer G, Ludwig S, editors. Textbook of pediatric emergency medicine. 5th ed. Philadelphia (PA): Lippincott and Williams; 2006. p. 477281. 3. Brown R, Hussain M, McHugh K, Novelli V. Discitis in young children. J Bone Joint Surg Br 2001;83:106211. 4. Fernandez M, Carrol CL, Baker CJ. Discitis and vertebral osteomyelitis in children: an 18-year review. Pediatrics 2000;105:12992304. 5. Knoeller SM, Uhl M, Adler CP. Differential diagnosis of benign tumors and tumor-like lesions in the spine. Own cases and review of the literature. Neoplasma 2004;51:117226. 6. Shapiro FD, de Leval L. A boy with vertebral compression fractures. N Engl J Med 2000;343:1168276. 7. Pearce J, Sills RH. Childhood leukemia. Pediatr Rev 2005;26:962104. 8. Ramanujachar R, Richards S, Hann I, et al. Adolescents with acute lymphoblastic leukaemia: emerging from the shadow of paediatric and adult treatment protocols. Pediatr Blood Cancer 2005;47: 748275. 9. Samuda GM, Cheng MY, Yeung CY. Back pain and vertebral compression: an uncommon presentation of childhood acute lymphoblastic leukemia. J Pediatr Orthop 1987;7:17528. 10. Jonsson OG, Sartain P, Ducore JM, et al. Bone pain as an initial symptom of childhood acute lymphoblastic leukemia: association with nearly normal hematologic indexes. J Pediatr 1990; 117:23327. 11. Pandya NA, Meller ST, MacVicar D, et al. Vertebral compression fractures in acute lymphoblastic leukaemia and remodelling after treatment. Arch Dis Child 2001;85:49223. 12. Kobayashi D, Satsuma S, Kamegaya M, et al. Musculoskeletal conditions of acute leukemia and malignant lymphoma in children. J Pediatr Orthop B 2005;14:156261.