Interventional Spine

Lien T Huynh, MD
Felix L Song, MD, Non ASSR Member
Daniel J Donovan, MD, Non ASSR Member

Excerpta Extraordinaire

Excerpta

Spinal arteriovenous malformations (AVM) occur in <0.1% of the population (1). Of those, perimedullary arteriovenous fistulas (AVF) are relatively rare and constitute approximately 8-19% of spinal AVMs and often present in the second to fourth decades of life with no gender predilection (2). We present a case of spinal AVF in a 4 year old female with hereditary hemorrhagic telangiectasia who developed acute onset of bilateral lower extremity paresis and bowel and bladder dysfunction. MR of the lumbar spine revealed a 2 cm hematoma of the conus. Spinal angiography demonstrated a conus fistula which was supplied by the anterior spinal artery that arises from the left T9 intercostal artery. Embolization was performed using liquid adhesive. Post-embolization angiogram demonstrated absence of filling of the fistula and psuedoanuerysm. Two month post-embolization follow-up demonstrated full recovery of bowel and bladder function and strength in the right lower extremity. Residual weakness and parathesias persisted in the left lower extremity. Knowledge and ability to use liquid adhesives may obviate the need for spinal surgery or instrumentation in young patients and prevent spinal column maldevelopment and future limitation of activities.

References

Authors: Lien T Huynh MD, Felix L Song MD, Daniel J Donovan MD

References:
1. Al-Shahi, R, Warlow, C. A systematic review of the frequency and prognosis of arteriovenous malformations of the brain in adults. Brain 124:1900, 2001.

2. Aydin et al. Angiography-induced closure of perimedullary spinal arteriovenous fistula. British Journal of Radiology 77:969-973, 2004.

3. Hida et al. Corpectomy: a direct approach to perimedullary arteriovenous fistulas of the anterior cervical spinal cord. Journal of Neurosurgery (Spine 2) 96:157-161, 2002.

4. Hida et al. Results of the surgical treatment of perimedullary arteriovenous fistulas with special reference to embolization. Neurosurgery Focus 5(4): Article 9, 1998.

5. Oran et al. Treatment of slow-flow (Type 1) perimedullary spinal arteriovenous fistulas with special reference to embolization. American Journal of Neuroradiology 26:2582-2586, 2005.

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General Spine

Peter Schramm, MD
Saida Zoubaa, MD, Non ASSR Member
Paul Kremer, MD, Non ASSR Member

Excerpta Extraordinaire

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A 22-year-old woman developed an increasing distal paraparesis, which resulted in a severe ataxic gait. MRI demonstrated a very large intramedullary tumour mass from D11 to L2, which could not be identified as one of the common neoplastic diseases of the spinal cord. The tumour was removed completely and the diagnosis of subependymoma confirmed. With respect to our patient and to the limited reports in the literature, we describe some typical features that may help in the diagnostic and preoperative assessment of this very rare spinal cord tumour.

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General Spine

Divyesh G Patel, MD
Joseph G Craig, MB ChB, Non ASSR Member

Excerpta Extraordinaire

Excerpta

Tumoral calcinosis of the spine is a rare manifestation of an uncommon condition. To date the authors are only aware of 10 reported cases involving the cervical and cervicothoracic spine.

Purpose

We present a case of tumoral calcinosis of the cervical spine causing myelopathy that was thought to represent a tumor or infection.

Methods & Materials

A 29-year old African American female presented to the Emergency Department with progressively worsening debilitating back pain that was rated as 10 out of 10. The patient denied any radiation or weakness, but did have upper extremity paresthesias. There was no history of trauma or twisting injury.

The patient’s past medical history was significant for end-stage renal disease requiring hemodialysis.

The physical examination demonstrated the patient to be in mild distress, with midline tenderness at the cervicothoracic junction. Mild weakness of both upper extremities was also noted. Otherwise, the physical examination was noncontributory.

Radiographs of the cervical and thoracic spine showed a calcified/ossified mass involving the posterior elements of C6 through T1 with some encroachment on the neural foramina at these levels. CT of the cervical spine and thoracic spine showed a calcified mass about the spinous processes from C6-T2. The lamina, pedicles, and spinous process of C7 were expanded and had a mixed sclerotic/lytic appearance. There were some small lytic regions within the T1 laminae, as well as incorporation of the T1 spinous process by the mass. MRI of the cervical spine demonstrated the mass to be heterogeneous with large areas of diminished signal on T2, T2*, and T1 images. There was narrowing of the central spinal canal from C6 to T1, with complete effacement of the cerebrospinal fluid space at C7. Both neural foramina at C7-T1 were narrowed. No abnormal signal was seen with the spinal cord.

Results

The orthopedic spine service was consulted. The patient was taken for decompression for spinal cord compression that was presumed to be related to a tumor. The mass was composed of calcific and inflammatory tissue which seemed pus-like to the surgical team. Intraoperatively there was concern that this was caseous type necrosis related to tuberculosis infection. The intraoperative gram stain and AFB stains were negative. Intraoperative frozen section was inconclusive for tumor versus inflammatory condition versus infection. The surgical team did note there was significant destruction of the posterior elements by the infiltrative mass. Complete excision of this tissue was performed with decompression at C7 and T1 via laminectomy, partial facetectomies, foraminotomies, and partial vertebral resection of C7.

The final histology showed bands of fibrocollagenous tissue in a trabecular pattern encircling extensive deposits of hydroxyapatite. The deposits were surrounded by a cellular infiltrate of lymphocytes and numerous histiocytes with frequent multinucleated giant cells. The epithelial markers failed to demonstrate any cells. There was no bone in the tissues examined.

Conclusion

Tumoral calcinosis is a rare disorder characterized by soft tissue calcification deposition, typically in a periarticular distribution about the hip, shoulder, and elbow. First described by Inclan et al in the American literature in 1943, the disorder was differentiated from dystrophic and metabolic calcifications in that patients had normal calcium levels and elevated phosphate levels. In the mid-1960s a familial predisposition and a higher incidence in the African American population was found to be associated with the disorder.

Over time a laxity developed in defining the disorder based on the metabolic criteria originally proposed by Inclan et al. As a result three different clinical presentations have been described as representing tumoral calcinosis. The familial variant usually manifests before the third decade with painless lesions about the hip and elbow. It can also occur as a result of chronic renal failure as in our patient. Finally, a sporadic form is thought to occur in patients with tissue degeneration or degenerative joint disease. Durant et al have proposed that calcific tendonitis is a common manifestation of this sporadic form. Recently, however, Olsen et al have suggested that the radiology community reexamine the liberal use of the term tumoral calcinosis, and only apply it to those cases of periarticular calcifications that are associated with a hereditary metabolic dysfunction of phosphate regulation.

Histologically, these lesions demonstrate masses of calcium hydroxyapatite crytals associated with neovascularity, histiocytes, and foreign body giant cells. There usually is a fibrous capsule related to a foreign body type reaction. There have been reports of calcium hydroxyapatite and calcium pyrophosphate hydroxyhydrate (CPPD) crystals coexisting within a lesion, as well as cases of isolated CPPD crystals within lesions classified as tumoral calcinosis. There is controversy related to this as some believe that CPPD crystal deposition seen within spinal ligaments is a distinctly different entity from tumoral calcinosis. However, there have been documented cases of calcium hydroxyapatite and CPPD found together within articular chondrocalcinosis and in a subcutaneous tophaceous mass from pseudogout. Kawano and colleagues even showed that linear CPPD crystal deposits within the ligamentum flavum later changed into calcium hydroxyapatite cystals, the more stable final form of calcium phosphate.

The rarity of this entity and its ability to mimic infection and neoplasm makes diagnosis difficult. In the 21 cases of spinal involvement presented by Durant et al, tumoral calcinosis was not considered as a pre-operative diagnosis in any of the patients. Whether we define tumoral calcinosis as a periarticular calcified mass associated with a hereditary defect in phosphate metabolism or as periarticular calcifications associated with an underlying disorder, it is a diagnostic consideration we should keep in mind.

References

1. Inclan A, Leon P, Camejo MG. Tumoral calcinosis. J Am Med Assoc 1943;121:490-95.
2. Shaffrey CI, Munoz EL, Sutton CL, et al. Tumural Calcium Phyrophosphate Dihydrate Deposition Disease Mimicking a Cervical Spine Neoplasm: Case Report. Neurosurgery 1995;37:335-39.
3. Kokuban S, Ozawa H, Sakurai M, et al. Tumoral Calcinosis in the Upper Cervical Spine: a Case Report. Spine 1996;21:249-52.
4. Ohashi K, Yamada T, Ishikawa T, et al. Idiopathic Tumoral Calcinosis Involving the Cervical Spine. Skeletal Radiol 1996;25:388-90.
5. Mooney JF 3rd, Glazier SS. Tumoral Calcinosis of the Cervical Spine in an Infant. Case Illustration. J Neurosurg 1997;86:162.
6. Durant DM, Riley LH 3rd, Burger PC, et al. Tumoral Calcinosis of the Spine: a Study of 21 Cases. Spine 2001;26:1673-79.
7. Matsukado K, Amano T, Itou O, et al. Tumoral Calcinosis in the Upper Cervical Spine Causing Progressive Radiculomyelopathy - Case Report. Neurol Med Chir (Tokyo) 2001;41:411-14.
8. Teng Al, Robbin MR, Furey CG, et al. Tumoral Calcinosis in the Cervical Spine in a Patient with CREST Syndrome. A Case Report. J Bone Joint Surg Am 2006; 88:193-97.
9. Olsen KM, Chew FS. Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities. Radiographics 2006;26:871-85.

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Interventional Spine

Serge Menkin, MD

Excerpta Extraordinaire

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A 60 year-old female presented to our office with 17 month history of thoracolumbar junction pain following a fall. The pain was present with sitting, standing, walking. Nuclear bone scan which was done 2 months after the fall showed increased uptake in the left lateral aspect of the T12 vertebral body. The pain was refractory to anti-inflammatory medication, pain medication, physical therapy and one lumbar epidural. She was severely debilitated by her pain. She rated the pain as 7/10 on VAS. A new MRI revealed chronic compression fracture of T12 vertebral body.

The patient underwent T12 vertebroplasty via unipedicular approach with preferential PMMA placement on the left aspect of the vertebral body. After the procedure the patient had complete resolution of her symptoms at thoracolumbar junction. However, in 20 weeks following the vertebroplasty the thoracolumbar pain returned. The character of pain was different than prior to vertebroplasty. The pain was less severe and it was absent on sitting, but present with standing and walking. Repeat imaging, including the plain X-rays and MRI did not reveal any adjacent level fractures. The patient underwent right side injections of T11-T12, T12-L1 facets with 80% improvement in pain.

Most of the literature describing biomechanical changes following the vertebroplasty indicates increased stresses around the endplate of the adjacent vertebra. This case illustrates that at the facets may be affected by the increased loads in a setting of vertebral augmentation of the chronic compression fracture. This may have resulted from the correction of the segmental lordosis which had developed as a compensation for vertebral kyphosis.

References

Polikeit A, Nolte LP, Ferguson SJ. The effect of cement augmentation on the load transfer in an osteoporotic functional spinal unit: finite element
analysis. Spine 2003;28:991-6.

Baroud G, Bohner M. Biomechanical impact of vertebroplasty. Joint Bone Spine 2006;73:144-50

General Spine

David Friedman, M.D.
Reginald Denis, M.D., Non ASSR Member
Adam Flanders, M.D., ASSR Member

Excerpta Extraordinaire

Excerpta

Highly mobile intradural spinal neoplasms are rarely identified. The few reported cases have usually occurred in the cauda equina. A 48-year-old woman presented with low back pain. Routine unenhanced MR imaging of the lumbar spine showed an intradural mass at the L2-L3 level. The patient returned nine months later for a contrast-enhanced scan that demonstrated a peripherally enhancing, intradural mass that had migrated caudally, and was now located predominantly behind the L4 vertebral body. A pre-operative localization scan obtained two weeks later showed that the mass had now migrated rostrally by a distance of 7 mm. The mass was resected and the pathologic diagnosis was schwannoma. Mobility of an intradural spinal neoplasm to the extent seen in this case is very rare; the few reported cases have usually been schwannomas of the cauda equina. There is one case report of a filum ependymoma that migrated 7 cm. Migration of cauda equina tumors secondary to positioning on the operating room table has been described, but is also considered rare. The distance of tumor migration in this case clearly exceeds the small amount of normal mobility of the cauda equina with positioning. Possible mechanisms for migration of schwannoma include elongation of the nerve root by tension resulting from tumor weight, and increased length/mobility of the nerve root involved by the schwannoma. The significance of mobility of an intradural spinal neoplasm is clear; migration of a lesion with respect to the pre-operative MRI examination may necessitate additional laminectomy and modification of the site of durotomy. To avoid this complication, in patients with neoplasms of the cauda equina, a pre-operative MRI examination should be obtained in very close proximity to the time of surgery; intraoperative ultrasound may also be of benefit.

References

1. Friedman JA, Wetjen NM, Atkinson JLD. Utility of intraoperative ultrasound for tumors of the cauda equina. Spine 2003;28:288-290 2. Varughese G, Mazagri R. Mobile tumours in the lumbar spinal canal: a diagnostic problem. Can J Surg 1997;40:59-63 3. Isu T, Iwasaki Y, Akino M, Nagashima M, Abe H. Mobile schwannoma of the cauda equina diagnosed by magnetic resonance imaging. Neurosurgery 1989:;25:968-971 No disclosures.

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General Spine

Christine P Chao, MD
Leo F. Czervionke, MD, ASSR Member
Daniel F. Broderick, MD, ASSR Member

Excerpta Extraordinaire

Excerpta

A 27 year-old man presented with a 1 year history of progressive bilateral lower extremity pain and numbness radiating from his lower back. He denied upper extremity symptoms, disequilibrium, or any bowel or bladder dysfunction. The patient had been evaluated at an outside facility and told that he had a developmental disorder due to his short stature and that he needed a 10 level decompressive laminectomy. Past medical history was significant for a heart murmur at birth which required no treatment or prophylaxis. Physical examination was unremarkable except for a short stature with a height of 158 cm. The patient had a normal gait and normal upper and lower extremity strength and sensation.

Radiographs of his cervical, thoracic and lumbar spine demonstrated universal platyspondyly of the vertebrae with marked extension of the lateral margins of the vertebral bodies beyond the pedicles on the AP view and a rectangular and elongated configuration on the lateral view. Magnetic resonance (MR) imaging further revealed diffuse irregular, narrowed intervertebral spaces, broad-based disc bulging and shortened pedicles. The constellation of abnormal findings resulted in multilevel spinal and neural foraminal stenoses.

The main differential diagnoses included brachyolmia, mucopolysaccharidosis, spondyloepiphyseal dysplasia, and severe Scheuermann disease. The patient carried a presumptive diagnosis of brachyolmia, a word derived from the Greek word meaning "short trunk", as he exhibited the typical radiographic and MR imaging appearances of this rare bone dysplasia and no stigmata of the other differential diagnoses.

Brachyolmia is characterized clinically by childhood onset of a short trunk resulting from universal platyspondyly, without significant involvement of epiphyses, metaphyses, or diaphyses of the long bones. The four main types of brachyolmia include 3 autosomal recessive types (Hobaek, Toledo and Maroteaux) and an unnamed autosomal dominant type. Additionally, there are individual cases which elude classification. Prognosis varies amongst the subtypes, with spinal stenosis previously reported as a complication of the Hobaek type, felt to be the subtype that our patient most likely exhibits. Although there is no specific treatment for any of the brachyolmias, our patient is considering decompressive spinal surgery for palliation of his symptoms.

This case of a 27 year-old man with presumed brachyolmia complicated by spinal stenosis provides an excellent opportunity to review the characteristic radiographic and MR findings in this rare disease.

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General Spine

Richard Silbergleit, M.D.
Ay-Ming Wang, M.D., Non ASSR Member
Ahmad Haidary, M.D., Non ASSR Member

Excerpta Extraordinaire

Excerpta

Pigmented Villonodular Synovitis (PVNS) is a destructive non-nepolastic process that typically involves large synovial joints such as the hips and knees. Involvement of smaller synovial joints occurs but involvement of the spine is unusual. An aggressive case involving a synovial facet joint in the cervical spine with bone destruction is demonstrated with plain film, CT, MRI, and histopathogy. Clues to the non-neoplastic nature of the lesion are the involvement of both sides of the joint.

General Spine

Ryan D Murtagh, MD, MBA,
Judith Post, MD, ASSR Member
Joycelyn Bruce, MD, Non ASSR Member
Kathryn K Post, , Non ASSR Member

Excerpta Extraordinaire

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Severe cord compression from invasive, long-standing pulmonary aspergillosis in a well-controlled HIV positive patient.

Purpose

To describe the serial magnetic resonance imaging (MRI) findings in a rare case of severe cord compression due to epidural extension of invasive aspergillus of the right upper lobe in a patient with well-controlled HIV.

Methods & Materials

This is a 41 year-old male who has been HIV positive for 10 years. He presented to Jackson Memorial Hospital in 2003 with symptoms of cough and right arm pain. He was well controlled on his anti-retroviral medications, with undetectable viral load and CD4 count of 535. At the time of presentation he was found to have right upper lobe opacity on chest x-ray. Computed tomography (CT) of the chest was performed, showing a right upper lobe mass without definite chest wall or mediastinal invasion. At that time, this was felt to most likely represent neoplasm. Two CT-guided biopsies were performed in December, 2003, both of which revealed only chronic inflammation without malignancy or infectious agent. Bronchoscopy and video-assisted thoracic surgery (VATS) were performed in January of 2004, both of which were inconclusive. He was followed over a two year period, at which time he presented with increasing right shoulder and arm pain. MRI was performed in February of 2006, showing an invasive mass in the right lung apex with involvement of the T1 and T2 vertebral bodies and minimal extension into the ventral epidural space. There was no cord compression at that time. He again underwent bronchoscopy and VATS with inconclusive results. He was lost to follow up for several months, but returned in September of 2006 with severe back pain and myelopathic symptoms. MRI was performed at that time, showing extension into the epidural space with severe cord compression. He underwent emergent T1-3 laminectomy. Pathology demonstrated large amounts of dense connective tissue with marked acute and chronic inflammation, as well as fungal organisms with pseudohyphae. Special staining of the specimens showed that the fungal elements were consistent with aspergillosis. Culture of the specimen grew A. fumigatus species.

Results

Initial non-contrast CT demonstrates a large right apical mass without definite invasion of the chest wall and no spinal canal involvement. MRI performed two years later shows a large right apical mass with invasion of T1 and T2 bodies, the posterior elements, and minimal extension into the epidural space. The mass was isointense on both T1 and T2 weighted images and enhanced avidly. There was no cord compression at that time. Patient returned seven months later due to increasing pain and myelopathic symptoms. Repeat MRI showed progression, now with invasion of T3, widening of the right neural foramina at T1-2 through T3-4, and extension into the epidural space with severe cord compression from T1-3.

Conclusion

Pulmonary aspergillosis, both invasive and semi-invasive, is being seen with increasing frequency due to rising numbers of immunocompromised patients. We present a case of long-standing pulmonary aspergillosis in an HIV positive patient causing direct epidural cord compression. Of particular interest is the presence of isointense T2 signal intensity in the epidural mass. We postulate that this might be explained by the large amount of dense connective tissue seen on pathologic examination. This is likely a result of this HIV positive patient being able to mount a sufficient inflammatory response because of his well controlled HIV infection. We conclude that fungal disease should be included in the differential diagnosis of a spinal epidural lesion in a well controlled HIV positive patient.

References

Go BM, Ziring DJ, Kountz DS.
Spinal epidural abscess due to Aspergillus sp in a patient with acquired immunodeficiency syndrome.
South Med J. 1993 Aug;86(8):957-60.

Wagner DK, Varkey B, Sheth NK, DaMert GJ.
Epidural abscess, vertebral destruction, and paraplegia caused by extending infection from an aspergilloma.
Am J Med. 1985 Mar;78(3):518-22.

Dietz R, Huber G, Thetter O, Volkmer I. Aspergillosis of the lung with osteoclasis and paraplegia. Neuroradiology. 1982;23(4):219-21

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General Spine

Ryan D Murtagh, MD, MBA,
Judith Post, MD, ASSR Member
Sklar Evelyn, MD, ASSR Member

Excerpta Extraordinaire

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Spinal cord, spinal intradural space, vertebral body, and brain metastasis in a patient with testicular cancer.

Purpose

To describe the magnetic resonance imaging (MRI) features of an unusual case of metastasis to the spinal cord, spinal intradural space, vertebral body, and brain from a testicular neoplasm.

Methods & Materials

This is a 24 year old male who was initially diagnosed with testicular cancer in 2003 at an outside hospital. Orchiectomy was performed at that time, with findings of a stage I pure seminoma. He underwent prophylactic radiation treatment and was subsequently lost to follow up.
In September of 2004, he returned to his oncologist with a cough and back pain. Workup at that time revealed a large right lower lobe mass. He did not have insurance and was therefore referred to Jackson Memorial Hospital. Here, several cycles of chemotherapy were administered in an effort to reduce the size of the right lower lobe mass. An attempted resection was performed in November of 2005, but it was felt that the mass was too large to be safely removed. Further attempts to reduce the size of the mass with chemotherapy and stem cell transplant were successful, with repeat thoracotomy performed in March of 2006. Pathology revealed no residual viable tumor cells in the lung mass. He was discharged to home shortly thereafter.
He returned to the emergency deparment approximately three weeks later with complaints of nausea and vomiting. MRI of the brain revealed a large enhancing mass in the right occipital lobe. He was taken to the operating room for craniotomy and resection of the mass. Pathology revealed dense cellularity with embryonal cell carcinoma with elements of seminoma and yolk sac tumor. After surgery, he was discharged to home without complications. Follow-up MRI performed one month after the surgery revealed a large enhancing mass in the operative bed consistent with tumor recurrence. He was again taken to the operating room with similar results and again discharged to home.
He was seen in clinic shortly after discharge with complaints of severe back pain and right sided weakness. MRI of the spine was ordered. This study showed widespread metastatic disease with numerous intramedullary and intradural spinal lesions throughout the cervical, thoracic, and lumbar spine. Given the extent of his disease, he was given palliative radiation treatment to the entire spine and discharged to home with Hospice care.

Results

MRI of the brain shows a large right parieto-occipital mass. The lesion is isointense to slightly hyperintense on FLAIR images and enhances heterogeneously following contrast administration.
MRI of the entire spine showed several large intramedullary lesions in the cervical and thoracic cord, as well as multiple intradural, extramedullary lesions through the cervical, thoracic, and lumbar spine. The lesions are isointense to cord on pre-contrast T1 images and enhance avidly following administration of contrast (Magnevist). The lesions are predominately isointense on T2 weighted images. Vertebral body involvement was noted as well. One month follow-up MRI of the lumbar spine shows marked interval progression of intradural disease.

Conclusion

Germ cell tumors of the testis most frequently metastasize to the lungs, liver, brain and bone (1). Several case reports have documented cord compression as a result of epidural metastasis (2, 3). However, the MRI characteristics of intramedullary spinal cord metastasis of testicular tumors has not been well described in the literature. Of particular interest is the relative isointensity of the lesions on T2 weighted images which may be related to the dense cellularity of the tumor.

References

1. Bredael JJ, Vugrin D, Whitmore WF. Autopsy findings in 154 patients with germ cell tumors of the testis. Cancer. 1982 Aug 1; 50(3): 548-51
2. Lee JK, Kim SH, Kim JH. Metastatic spinal cord compression of testicular yolk sac tumor. Childs Nerv Syst. 2002 Apr;18(3-4):171-4. Epub 2002 Mar 2.
3. Colak A, Benli K, Berker M, Onol B. Epidural metastasis of testicular yolk sac tumor: an unusual cause of spinal cord compression. Case report. Pediatr Neurosurg 1991-1992;17(3):139-41.

Images

Jonathan Morris, MD
Peter Kalina, MD, Non ASSR Member
Corey Raffel, MD, PhD, Non ASSR Member

Excerpta Extraordinaire

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A 7 month old boy presented with irritability, refusal to crawl and loss of neck control. MRI revealed a C2 to L4 partially enhancing spinal epidural hematoma (SEH) with cord compression. There was no trauma history. A PTT of 79 precluded a CT guided biopsy (to help exclude other etiologies of spinal epidural lesions in children). Factor VIII was found to be less than 0.01 U/ml. As a result of the MRI and laboratory data, the boy was able to be diagnosed with severe hemophilia A. He improved following aggresive Factor VIII replacement therapy.
Non-traumatic SEH is a rare complication of hemophilia. Complete neurological recovery is possible after replacement therapy, without the need for an extensive surgical decompression.

References

Jonathan Morris, MD