The Spine Journal

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Case Report

Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series Ivelin Iovtchev, MDa, Nurith Hiller, MDb, Yona Ofran, MDa, Isabella Schwartz, MDa, Jose Cohen, MDc, Stuart A. Rubin, MDa, Zeev Meiner, MDa,* a

Department of Physical Medicine and Rehabilitation, PO Box 24035, Hadassah Hebrew-University Medical Center, Jerusalem, Israel b Department of Radiology, PO Box 24035, Hadassah Hebrew-University Medical Center, Jerusalem, Israel c Department of Neurosurgery, PO Box 24035, Hadassah Hebrew-University Medical Center, Jerusalem, Israel Received 5 November 2012; revised 31 July 2013; accepted 22 August 2013

Abstract

BACKGROUND CONTEXT: Spinal dural arteriovenous fistula (SDAVF) is a slow-flow extramedullary vascular lesion affecting primarily the lower thoracic and lumbar spine. The clinical sequela of these vascular changes is progressive myelopathy and severe lower-extremity weakness. Although surgical or embolic treatment of SDAVFs has improved significantly in the last years, the ambiguity of the symptoms may complicate and delay the diagnosis. The influence of the postponed diagnosis on the functional outcome of patients with SDAVF is unknown. PURPOSE: To describe a case series of patients with SDAVF that illustrates that delayed diagnosis leads to grave neurologic and functional prognosis. STUDY DESIGN: A case series. METHODS: We present a series of seven patients, treated in a tertiary university rehabilitation center over 20 years. Clinical, radiologic, and functional outcomes were evaluated by retrospective chart review. Neurologic and functional evaluation at the end of rehabilitation was evaluated with the lower extremities motor score and the Aminoff-Logue scale, respectively. RESULTS: All our patients were men with a mean age of 60.3616 years (30–72 years), mean time until the diagnosis of SDAVF was 302.86239 days (60–730 days), and mean overall length of stay in acute department and rehabilitation unit was 88.6634 days (46–149 days). At the end of rehabilitation period, four patients remained at wheelchair level with an Aminoff-Logue scale grading of five whereas other functional scales showed also low levels of recovery. CONCLUSIONS: Our series showed that the potential for functional ambulation was poor despite prolonged rehabilitation treatment in late diagnosis SDAVF. Awareness of the early symptoms of SDAVF and immediate intervention may help reduce impairment in such patients. Ó 2013 Elsevier Inc. All rights reserved.

Keywords:

Spinal dural arteriovenous fistula; Rehabilitation; Myelopathy; Functional outcomes; Foix-Alajouanine syndrome; Aminoff-Logue scale

Introduction

FDA device/drug status: Not applicable. Author disclosures: II: Nothing to disclose. NH: Nothing to disclose. YO: Nothing to disclose. IS: Nothing to disclose. JC: Nothing to disclose. SAR: Nothing to disclose. ZM: Nothing to disclose. Part of this work was presented in the ISPRM meeting in Istanbul 2009. * Corresponding author. Department of Physical Medicine and Rehabilitation, PO Box 24035, Hadassah University Hospital, Jerusalem, 91240 Israel. Tel.: þ972 2 5844474; fax: þ972 2 5829113. E-mail address: [email protected] (Z. Meiner) 1529-9430/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.spinee.2013.08.029

Spinal dural arteriovenous fistulas (SDAVFs), also known as Foix-Alajouanine syndrome, are slow-flow extramedullary vascular lesions affecting mostly the lower thoracic and lumbar spinal levels [1]. SDAVFs represent approximately 75% to 80% of all spinal vascular malformations, and the majority of affected patients are men older than 50 years of age [2]. Progression to severe myelopathy or paraplegia is slow. Patients initially may present with acute lower-extremity dysesthesias and intermittent radicular pain mimicking peripheral nerve lesions. There may be also bowel or bladder incontinence and impotence. These

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vague symptoms make the diagnosis complicated and delayed [3]. Many of these patients have already suffered from their symptoms for several months and have undergone unnecessary procedures and surgery before diagnosis [4]. The recommended treatment is either endovascular embolization of the fistula or surgical removal [5]. The influence of the postponed diagnosis and therefore delayed interventions on the functional outcome of SDAVF patients is unknown. In this article we describe a case series comprising seven patients treated in a rehabilitation unit in which the diagnosis of SDAVFs was delayed and were left with severe lower extremity weakness.

Case series presentation In the last 20 years, seven patients with delayeddiagnosis SDAVF were treated in our inpatient rehabilitation unit. Clinical, demographic, radiologic, and functional outcomes for these seven patients are summarized in Table and presented in Figs. 1–3. All were men with mean age of 60.3616 years, with a range from 30 to 72. The mean time until the diagnosis of SDAVF was 302.86239 days, with a range from 60 to 730. The overall mean length of stay was 88.6634 days (46–149 days), including 26.1 6 9 days (14–36 days) in the acute department and 62.4629 days (32–113 days) in the rehabilitation unit. The diagnosis of SDAVF was delayed in most of the patients in our series; requiring an average of 10 months and reaching almost 2 years in one patient. Many alternative diagnoses were proposed such as spinal stenosis, myeloradiculitis, peripheral neuropathy and more. In two of our patients, axonal motor neuropathy was found by nerve conduction studies. Cerebrospinal studies were performed in four of six patients. The main findings were elevated red blood cells in two and high protein levels in three. Positive oligoclonal bands typical for inflammatory conditions were found in two patients. Some of the patients were treated because of these false diagnoses with surgical procedures, epidural injections, intravenous and oral steroids, and plasmapheresis. Three patients were treated by embolization, two patients were treated by surgery alone, and two patients were treated by combined approach. Because of the small sample, no definite conclusion regarding the correlation between type of therapy and prognosis could be obtained. The neurologic function of patients with SDAVF at the end of rehabilitation were evaluated using the lower extremities motor score according to the International Standards for the Neurological Classification of Spinal Cord Injury [6]. As can be seen in Table, lower extremities motor score remained less than 30 in 5 of 7 patients. This reflected the severe lower extremity weakness of these patients at the end of rehabilitation. The functional level of patients with SDAVF at the end of rehabilitation were evaluated using the Aminoff-Logue scale (ALS) [7]. This scale consists of six grades of gait, between 0 normal and 5 confined to

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a wheelchair, and 4 grades of micturition between 0 normal and 3 total incontinence or persistent retention. At the end of rehabilitation period, four patients remained at wheelchair level with an ALS grading of 5, and two patients had ALS grading of 4, indicating that they required two crutches, or walker. One patient was able to ambulate with a cane (ALS of 3).

Discussion In this article we described a case series of seven patients suffering from SDAVF treated in one rehabilitation facility over 20 years. Despite prolonged rehabilitation treatment, four of seven patients remained at wheelchair level and their level of independence was low. The diagnosis of SDAVF was delayed in all 7 patients contributing to the low level of independence at the end of rehabilitation. SDAVFs are the most prevalent type of spinal vascular lesions and have been classified into four types: type I, SDAVFs located within the dura of the nerve root sleeve, connecting a dural branch with an intradural medullary vein; type II, congenital intramedullary AVM fed by spinal artery branches; type III, juvenile AVM, a very rare lesion comprising an intramedullary nidus with extramedullary/ extraspinal extension; and type IV, intradural extramedullary AVF, also known as perimedullary fistula [8]. Foix-Alajouanine syndrome was first described in two young men (ages 31 and 37 years) in 1926 [9]. Until the middle of the 20th century, this pathologic entity was only made at autopsy. With the advent of high-resolution magnetic resonance imaging (MRI) and modern spinal angiography, it became possible to diagnose the syndrome ante-mortem and to offer an effective treatment. However, even today, the true incidence of SDAVF is unknown, and often there is a significant delay in the diagnosis, as was demonstrated in our cases. In a series of 49 patients with SDAVF admitted to one unit over 15 years, the time interval between the initial symptoms and diagnosis was a median of 10.5 months (range, 1 day to 20.8 years) [10] and in another large series of 66 patients, the average time from symptoms onset to diagnosis was reported to be 27 months [4]. Because of the low incidence of SDAVF and its puzzling symptomatology, an appropriate diagnostic workup is often not performed, and the time to diagnosis is prolonged [11]. Most of the patients complain of leg weakness or paraparesis, sensory numbness or paresthesias, urinary or bowel incontinence or retention, and pain. Pain was a concern in approximately half of the patients, including back pain and/or radiculopathy [4]. At the time of diagnosis, patients with SDAVF have signs of both myelopathy and lower motor neuron findings emanating from lower thoracic spinal cord and conus medularis involvement of anterior horn nuclei. In our patients several alternative diagnoses were made before the right diagnosis of SDAVF was reached. These possibilities included benign hypertrophy of the

Table Clinical and laboratory characteristics of seven SDAVF patients Patient 1 Age (yr), sex Initial symptoms

Initial signs on admission

Treatment

Patient 5

Thoracic MRI: a Spinal MRI: anomalous hyperintense T2 signal dural vessels at L1–L2 extending from T5 and spinal stenosis at through the inferior L1–L3 (Fig. 2, Left aspect of the cord with and Right) enhancement from T8 to T11 (Fig. 1, Left). Spinal angiogram: AV fistula at the T9-T11 level (Fig. 1, Right) ND CSF: Pro- 837 mg/L Cells: 2750 RBC Oligo: Neg NCS: motor neuropathy Embolization 2 Decompressive laminectomy L1–L3

Patient 6

Patient 7

30, Male 72, Male Progressive weakness in Lower extremities weakness for one and left leg for 4 mo. The a half years patient became paraparetic and lost deteriorated to severe control of his paraparesis with lost sphincters. control of his sphincters. Spastic paraparesis, Proximal paraparesis proximal weakness in more on the right, no both legs, bilateral reflexes, reduced hyper- reflexes with sensation without positive pyramidal sensory level. signs and clonus. Sensory level at T6. Operation was Underwent T4–T6 unsuccessful in laminectomy with resecting the complete resection of malformation. After the malformation. surgery, the patient Improved slightly in became paraplegic and rehabilitation after totally bed ridden. surgery.

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485

Thoracic MRI: Thoracic MRI: an Thoracic MRI: vascular Lumbar MRI: spinal suspicious for lesion at T6 stenosis. elongated spinal AVM. hyperintense T2 signal Spinal angiogram: dural Thoracic MRI: AV fistula Spinal angiogram: a T7 AV fistula at the level at the level of T9 on at T8–T9 (Fig. 3, AV fistula with the right. Left). of T6 extension from Spinal angiogram: AV T5 to T8. fistula at T8–T9 level (Fig. 3, Right).

Thoracic MRI: space occupying level at T11–T12 level. Spinal angiogram: suspected vascular malformation.

CSF: Pro- 1200 mg/L Cells: 2 RBC, 6 Ly Oligo: Pos

CSF: Pro- 609 mg/L Cells: 10 RBC 1 Ly Oligo: ND NCS: motor neuropathy Laminectomyþbopsy

Embolization

CSF: Pro- 535 mg/L Cells: 130 RBC 4 Ly Oligo: Pos

CSF: Pro-745 mg/L ND Cells: 0. Oligo: Neg NCS: motor and sensory neuropathy Posterior decompression Embolization Laminectomyþresection at L2–L5 levels and embolization

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Other laboratory results

Patient 4

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Time to diagnosis (d) Imaging results

Patient 3

76, Male 48, Male 67, Male 68, Male Low back pain for many Low back pain, gait and Urinary disturbances for Low back pain and weakness of the right sensory disturbances 5 mo. Low back pain, years, difficulties in leg for 2 mo followed for 6 mo. sacral hypoesthesia, walking, and spinal by acute urinary claudication for and inability to urinate several mo for 5 wk. retention and hypoesthesia of lower extremities. Weakness of L5 muscles Proximal muscles Mild proximal weakness, Proximal weakness in Proximal weakness on on the left, loss of weakness with reduced reflexes and legs, reduced reflexes, legs, hyporeflexes with reflexes, decrease reduced reflexes and loss of sensation on positive pyramidal positive pyramidal sensation on L5–S1 on normal sensory the right leg. signs, sacral and signs. Loss of the left. examination perianal anesthesia, vibratory and position sensory level at T10. sensation on the left with sensory level at T8. Underwent a multilevel Diagnosed as Diagnosed with After decompressive Progressive spastic myeloradiculitis and posterior myeloradiculitis and laminectomy surgery, paraparesis over 12 was treated with decompression at patient became a treated with mo. After, the intravenous steroids L2–L5 without intravenous steroids complete paraplegic embolization became and plasmapharesis improvement. After and total lost control without improvement. paraplegic with total without improvement. embolization Became paraplegic of his sphincters loss control of his Improved after developed dense with complete urinary sphincters. embolization. paraplegia and loss of retention. sensation of the lower extremities. 730 180 365 150 60

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Clinical course

Patient 2

61, Male Gait disorder for 6 mo, acute urinary retention, pain in the lower back and legs.

ADL, activities of daily living; ALS, Aminoff-Logue scale [7]; AV, arteriovenous; CSF, cerebrospinal fluid; LEMS, lower extremities motor score [6]; LOS, length of stay; Ly, lymphocytes; MRI, magnetic resonance imaging; NCS, nerve conduction study; ND, not done; Oligo, oligoclonal bands; Pro, protein; RBC, red blood cells; SDAVF, spinal dural arteriovenous fistula.

LEMS at discharge ALS grading at discharge

3 5

14 5

30 4

26 5

46 3

Regained partial Independent at independence in wheelchair level and wheelchair mobility able to walk for short but remained distances using a dependent in all ADL rolling walker. functions. Independent in all ADL functions and regained partial control of sphincters. 28 18 4 5 Independent in transfers Able to ambulate with forearm crutches for and able to ambulate 100 ms and to climb with a walker for household distances. stairs. Regained full control of his Minimal assistance sphincters and was level in basic ADL independent in all functions ADL functions. Succeeded to walk with Independent in forearm crutches for wheelchair propulsion 100 m, regained but not in transfers. Partial independent in partial control of his sphincters, and ADL functions and became independent had permanent in basic ADLs. indwelling catheter.

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Patient 6 Patient 5

65 149

Patient 4 Patient 3

100 87

Patient 2 Patient 1

LOS acute 106 careþRehab (d) Functional level at Independent in all wheelchair-level discharge from activities, including rehabilitation chair-to-bed transfers and all wheelchairlevel ADLs.

Table (Continued )

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Patient 7

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prostate in patients 1 and 4; myeloradiculitis in patients 2 and 5; peripheral neuropathy in patients 1, 5, and 7 ;and spinal stenosis in patient 4. Our patients also received several unnecessary treatments as the result of the false diagnoses, including plasmapheresis, steroids, and surgery. Our patient number 2 underwent lumber microdisectomy surgery before the SDAVF was diagnosed, similar to another case in the literature described by Stevens et al. [12]. These findings emphasis the need for a high index of suspicion for SDAVF in a middle-aged man with the combination of urinary disturbances, motor or sensory symptoms in the legs and low back, and neuropathic pain in the lower limbs. SDAVF are well depicted on MRI, and the vascular origin of the symptoms can therefore be made early. It might however be difficult, or impossible, to precise the shunt involved (type and localization). Recent papers have been published on the interest of MR angiography in the diagnosis of SDAVFs [13]. Compared with routine spin echo sequences, contrast enhanced MR angiography offers improved characterization of the spinal vasculature and the possibility of visualization of large arteries and veins. However, the role of MR angiography in intradural spinal cord arteriovenous shunts remains debatable. Angiography, therefore, remains the gold standard for optimal analysis of the anatomical, morphologic, and angio-architectural features necessary for therapeutic decisions of SDAVFs [14]. In a study of patients with undiagnosed myelopathy, 28% had AVM, most of them SDAVFs, demonstrated by spinal angiography [15]. It was suggested that all adult patients with unexplained myelopathy after neurologic evaluation and MRI demonstrating increased T2 signal or flow voids should undergo spinal angiography. The decision to pursue angiography in patients with myelopathy without these two MRI findings should take into consideration the presence of clinical factors associated with spinal AVMs, including sex (male), age (older than 35), urinary incontinence, and perineal sensory changes [15]. Treatment options for SDAVF include microsurgery and endovascular embolization. There is still controversy regarding the relative advantages of each one. Although recently many authors favor the endovascular approach some still believe that surgery is superior [16] A metaanalysis of 16 surgical studies and 10 endovascular therapy studies [17] demonstrated that embolization resulted in initial occlusion of the fistula in 46% of cases with a morbidity of less than 4% and an absence of mortality whereas surgery resulted in successful occlusion of the SDAVF in 98% of the cases with a morbidity of less than 2% without mortality. In the surgical cases, clinical improvement was found in 55% and stabilization of the clinical condition in 34% of the patients, respectively. In our series we did not observe any difference in rehabilitation outcomes between either methods of treatment for SDAVF. Larger studies are needed to determine the influence of treatment on long term rehabilitation outcomes.

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Fig. 1. (Left) MR image of SDAVF in patient 1. T2-weighted MR image of the thoracic spine in the sagittal plane demonstrating diffuse swelling and increased T2 signal of the cord representing cord edema. Tortuous pathologic intradural vessels at the lower thoracic level are seen, consistent with SDAVF (white arrows). (Right) Selective spinal angiogram in patient 1 through injections of the left T9 radicular artery (long arrow) demonstrating the connection between the artery and a large, long serpiginous draining vein (short arrows). MR, magnetic resonance; SDAVF, spinal dural arteriovenous fistulas.

The prognosis of SDAVF is unpredictable. Evaluation of long-term outcome showed an enormous heterogeneity with clinical improvement ranging between 25% and

100% of the cases after treatment [18]. The eventual outcome may depend on several factors, including the duration of symptoms, the degree of disability before treatment, and

Fig. 2. (Left) Sagittal T2-weighted MR image of the lumbar spine of patient 2 demonstrating SDAVF at the level of L1 vertebra (white arrow). Note severe L2–L3 disc herniation and a scar from previous lumbar surgery. (Right) Axial T1-weighted MR image showing the tortuous pathologic intradural vessels (white arrow). MR, magnetic resonance; SDAVF, spinal dural arteriovenous fistulas.

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emphasizes the important of early diagnosis and early intervention in SDAVF. References

Fig. 3. (Left) Sagittal T2-weighted MR image of the thoracic spine of patient 5 demonstrating tortuous pathologic intradural vessels consistent with SDAVF (white arrows). Diffuse intramedullary high intensity is seen along the thoracic cord consistent with cord edema. (Right) Selective spinal angiogram in patient 6 through injections of the left T9 radicular artery demonstrating the connection between the artery and a large, long serpiginous draining vein (white arrows). MR, magnetic resonance; SDAVF, spinal dural arteriovenous fistulas.

the success of the initial procedure to close the fistula. It is generally thought that a better prognosis will be obtained in younger patients with less severe symptoms. In addition, a few studies showed a correlation between a poor preoperative neurologic status and a lack of improvement [19]. Another important factor that determined the final outcome is the time to diagnosis. It was shown that a better outcome has been related to shorter delay between symptom onset and initiation of endovascular or surgical treatment [20]. This finding was also found in most of our patients, whereas shorter time to intervention resulted in better prognosis. These findings emphasized the utmost importance of early diagnosis of SDAVF and the initiation of specific intervention, surgical or embolization, as well as rehabilitation treatment, as soon as possible.

Conclusions Although all our patients achieved independence at wheelchair level, most of them were unable to ambulate and required assistance in activities of daily living. The main finding of this case series is that the potential for functional ambulation in paraplegic patients with SDAVF is poor despite prolonged rehabilitation treatment, when endovascular or surgical treatment is postponed. This finding

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Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series.

Spinal dural arteriovenous fistula (SDAVF) is a slow-flow extramedullary vascular lesion affecting primarily the lower thoracic and lumbar spine. The ...
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