Sacral Preservation in Cauda Equina Syndrome from Inferior Vena Cava Thrombosis

J Neurosurg Spine 10:000–000,257–259, 2009

Sacral preservation in cauda equina syndrome from inferior vena cava thrombosis

Case report

Al i k S. Wi d g e , M.D., Ph.D.,1 Ne s t o r D. To m y c z , M.D.,2 an d Ad am S. Kan t e r , M.D.2 1School of Medicine and 2Department of Neurosurgery, University of Pittsburgh, Pennsylvania

Acute cauda equina syndrome can occur due to a variety of causes. Inferior vena cava (IVC) thrombosis has been reported as the causal source of this phenomenon twice in the relevant literature, both cases of which presented in a form complete with a component of bowel and/or bladder dysfunction. The authors report an atypical case of cauda equina syndrome in a patient in a hypercoagulable state with an extensive IVC thrombosis, resulting in acute paraparesis in the absence of incontinence or perineal anesthesia. An increasing number of prophylactic and/ or therapeutic IVC filters placed in the perioperative period should engender an increased clinical suspicion for IVC thrombosis in patients presenting with acute paraparesis. (DOI: 10.3171/2008.12.SPINE08389)

Ke y Wo r d s • acute paraparesis • cauda equina syndrome • deep venous thrombosis • vena cava thrombosis

a u d a equina syndrome arises from an insult to the bosis in a patient who presented with moderate lower- collection of lumbosacral nerve roots that arise extremity motor and sensory disturbance, without saddle from the caudal spinal cord. In its classic form, anesthesia or bowel/bladder incontinence. CCES presents with symptoms of low-back and leg pain, lower-extremity weakness and sensory loss, perineal anesthesia, bladder/bowel incontinence, and erectile Case Report dysfunction.19 Bowel and bladder dysfunction are often considered essential to the diagnosis of CES,6,15,22,25 History and Presentation. This 55-year-old Cauca- although many cases of CES with complete sparing of sian male presented with a rapidly progressive parapa- sacral dysfunction have been described.12 Patients with resis 16 days after admission to the hospital following CES often exhibit subtle signs such as decreased perineal an assault. His medical history included a warfarin- sensation and mild urinary retention, signs that may be dependent factor V Leiden mutation, DVT, pulmonary concealed in the hospitalized patient.8 Thus, the manifes- embolus, IVC filter placement, and lumbar stenosis. The tation of CES occurs across a broad spectrum, from dra- patient was neurologically intact upon admission. He matic complete forms with profound sacral dysfunction subsequently developed a lower-extremity compartment to more insidious partial forms that often contribute to a syndrome necessitating multiple left lower-extremity long delay before diagnosis.17 fasciotomies. His anticoagulation therapy was switched Lumbar disc herniations remain the primary pathol- from warfarin to low-dose subcutaneous heparin on hos- ogy evoking CES, although hemorrhagic spinal tumors9,26 pital Day 10. and arteriovenous malformations19 have also been de- On the 16th hospital day, the patient awoke with scribed. Other rare causes include ischemia (such as aortic right-leg weakness, which progressed to involve the left dissection21 and abdominal vascular surgery19), osteomy- leg by early afternoon. On examination, he was insensi- elitis/discitis impacting the epidural space,19 inflamma- tive in both lower extremities to light touch and pinprick tory polyneuropathy,13 vasculitis,11 and traumatic injury stimulation. The lower extremities were cold to the touch, from retropulsed vertebral fragments8 or a penetrating cyanotic, and edematous. A motor examination revealed foreign body.14 Acute CES presenting with severe lower- hip flexor antigravity strength (3/5 on the Medical Re- extremity sensorimotor and bowel/bladder dysfunction search Council muscle grading scale) with total plegia of secondary to IVC thrombosis has also been reported. We all muscle groups (0/5) below the knee. Truncal and up- report a unique case of acute CES caused by IVC throm- per-extremity strength and sensation remained normal. There was no perineal anesthesia, or bowel or bladder incontinence. Abbreviations used in this paper: CES = cauda equina syndrome; DVT = deep venous thrombosis; IVC = inferior vena cava; tPA = Operation and Postoperative Course. An emergency tissue plasminogen activator. CT scan revealed severe thrombosis of the IVC with ex-

J. Neurosurg.: Spine / Volume 10 / March 2009 257 A. S. Widge, N. D. Tomycz, and A. S. Kanter tension into both iliac veins. The patient was immediately Local inflammation may also contribute to acute CES taken to the operating room for a pharmacomechanical in the setting of IVC thrombosis. Thrombi provoke an in- endovascular thrombectomy (Fig. 1). Postoperatively, travascular inflammatory reaction with immune cell infil- he received a continuous femoral catheter tPA infusion tration and resultant mass effect.1 Even in the absence of and intravenous heparin. The patient’s neurological ex- frank compression, venous inflammation and/or dilation amination results improved significantly within 24 hours. may irritate cauda equina nerve roots akin to the neuro- Warfarin was restarted on postoperative Day 2. At that vascular conflict observed in trigeminal neuralgia.7,20 time, he experienced full return of lower-extremity sensa- The 2 prior reports of acute CES from IVC thrombo- tion and improvement in his distal lower-extremity motor sis described a complete form of CES involving low-back strength to 4/5. He was subsequently transferred on post- pain, lower-extremity weakness and pain, perineal numb- operative Day 8 to a rehabilitation service with full (5/5) ness, bowel/bladder dysfunction, and lower-extremity hy- lower-extremity strength and normal sensation. poreflexia.4,16 The symptomatology noted in our patient has only been previously described in more chronic pre- 5,10,24 Discussion sentations. The lack of sacral root symptoms in this case may be explained by the anatomical arrangement of Inferior vena cava thrombosis is a rare cause of acute the cauda equina nerve roots. The lumbar roots lie ven- CES. The anatomical link between the IVC and the cauda trally, particularly at higher lumbar levels, placing them equina is the venous connection, via the iliolumbar veins, closer to the anterior epidural and paravertebral venous between the IVC and the valveless Batson plexus sur- plexus. Necropsy models using Young’s modulus have rounding the spinal cord and nerve roots. Branches of the demonstrated that the linear strain on stretched nerve Batson plexus run within the spinal epidural space and roots within the cauda equina may be least on the sacral neural foramina along the nerve roots. In addition to the roots, which may explain cases of CES such as this one in connection to the IVC, lumbar veins also ascend above which sacral dysfunction was not evident upon examina- L-2 and connect to the azygous venous system. Thus, tion. thrombosis of the IVC necessitates that the lumbar plexus The underlying cause of the IVC thrombosis leading drain the lower extremities, leading to reversal of flow and to CES also remains inconsistent, but patients generally venous dilation.1 This dilation can result in lumbosacral have a thrombotic predisposition. Reported causes in- nerve root dysfunction via both ischemic and inflamma- clude a factor V Leiden mutation,5 protein C deficiency,20 tory mechanisms. an antiplatelet antibody,24 mechanical compression of the The cauda equina nerve roots have only a thin con- IVC, 20 and Behçet disease affecting the spinal vascula- nective tissue sheath and limited vascularity, making ture.20 In addition to hereditary thrombophilia, the pres- them particularly sensitive to compressive forces.19 A ence of an IVC filter may have been an independent risk vascular watershed zone exists within these roots; it has factor for IVC thrombosis in this patient. In a retrospec- been shown that as little as 10 mm Hg of increased pres- tive review, Corriere et al.3 found that retrievable filters, sure can produce microischemic zones.15,19 This vulner- especially those of a biconical design, increased the inci- ability is exacerbated by the venous stasis inherent in IVC dence of IVC thrombosis 200-fold. In the pediatric popu- thrombosis.4 In the patient presented here, preexisting lation, abnormalities of the vena cava, such as stenosis, lumbar stenosis may have additionally contributed to the must be considered as causative agents as well.16,18,23 CES by “precompressing” the cauda equina, thus reduc- Symptoms and signs of DVT, including limb swelling its functional reserve. ing,3 painful cyanosis (phlegmasia cerulea dolens),3,4 ab-

Fig. 1. Computed tomography scan through the abdomen with intravenous contrast administration (left) shows a large thrombus occluding the IVC, with infiltrative changes in the vessel wall (arrowhead). After 2 consecutive attempts at thrombectomy with intervening tPA infusion, postoperative venography (right) demonstrated only a residual clot attached to the IVC filter (arrowhead).

258 J. Neurosurg.: Spine / Volume 10 / March 2009 Sacral preservation in CES from IVC thrombosis dominal compartment syndrome,3 or simple paleness of a spine simulating prolapse of an intervertebral disc. Spine 28:E5– single extremity16 occur with most cases of IVC thrombo- E12, 2003 sis. In one patient series, massive limb swelling was the 8. Harrop JS, Hunt GE, Vaccaro AR: Conus medullaris and cauda 20 equina syndrome as a result of traumatic injuries: management most common sign, occurring in 7 of 10 patients. How- principles. Neurosurg Focus 16(6):E4, 2004 ever, neurological signs of IVC thrombosis can develop 9. Heuer GG, Stiefel MF, Bailey RL, Schuster JM: Acute parapare- 16 24 over weeks or months, and may be unaccompanied by sis from hemorrhagic spinal ependymoma: diagnostic dilemma other signs or symptoms of acute thrombosis. and surgical management. Report of two cases and review of the Cauda equina syndrome caused by IVC thrombosis literature. 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