Late spinal dislocation after treatment of spinal arteriovenous malformation

© Masson, Paris, 2004

Neurochirurgie, 2004, 50, n° 6, 647-651

Cas clinique

LATE SPINAL DISLOCATION AFTER TREATMENT OF SPINAL ARTERIOVENOUS MALFORMATION A case of Charcot spinal arthropathy R. VIALLE (1), F. PARKER (1), J.-F. LEPEINTRE (1), G. RODESCH (2), J.-L. TASSIN (3), M. TADIÉ (1) (1) Service de Neurochirurgie, CHU de Bicêtre, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre. (2) Service de Neuroradiologie, Hôpital Foch, 40, rue Worth, BP 36, 92151 Suresnes Cedex. (3) Hôpital Belle-Isle, 2, rue Belle-Isle, 57000 Metz.

SUMMARY: Late spinal dislocation after treatment of spinal arteriovenous malformation. A case of Charcot spinal arthropathy

R. VIALLE, F. PARKER , J.-F. LEPEINTRE , G. RODESCH, J.-L. TASSIN, M. TADIÉ (Neurochirurgie, 2004, 50, 647651) Neuropathic arthropathy of the spine is a destructive condition of the spine which is secondary to a loss of the protective proprioceptive reflexes. In the majority of cases, it occurs in patients who have suffered from traumatic medullary lesions and is responsible for destruction of the vertebral bodies and considerable spinal deformity. We report a case of neurogenic lumbar arthropathy in a patient with a spinal arteriovenous malformation. This vascular lesion caused considerable disturbances of proprioception. The course was favorable with regard to the deformity after correction and fusion by posterior approach.

RÉSUMÉ : Malformation artério-veineuse médullaire responsable d’une déformation rachidienne tardive : un cas d’arthropathie rachidienne de Charcot L’arthropathie neuropathique du rachis est généralement secondaire à la perte des réflexes proprioceptifs de la colonne vertébrale. Cette situation est responsable de phénomènes dégénératifs exacerbés, responsables de lésions osseuses parfois spectaculaires. Dans la majorité des cas, cette pathologie survient chez des patients ayant présenté une lésion médullaire traumatique et est responsable d’une déformation rachidienne considérable. Nous rapportons un cas d’arthropathie neuropathique lombaire chez un patient présentant une malformation artério-veineuse médullaire responsable de perturbations de la proprioception. L’évolution de la déformation rachidienne était favorable après correction et fusion par voie postérieure.

Key-words: Charcot spinal arthropathy, medullary arteriovenous malformation, spinal arteriovenous malformation, proprioception.

Charcot spine, also known as spinal neuropathic or neurogenic arthropathy, is a destructive condition, which affects the intervertebral disc and the adjacent vertebral bodies. As a result of a loss of joint protection mechanisms, which is generally secondary to a condition affecting the deep sensation pathways and causes a rapidly degenerative dislocation. The majority of cases reported in the literature involve tabetic spines, post-traumatic paraplegia and congenital insensitivity to pain. We report a case of spinal neurogenic arthropathy which occurred many years after surgical treatment of a spinal arteriovenous malformation. We

describe the diagnostic method and the therapeutic management. We discuss the differential diagnosis and the therapeutic strategies which could be devised in similar circumstances. CASE HISTORY A 27-years-old man was treated for an intramedullary arteriovenous malformation. He had been presented lumbar pain and sciatica at the posterior aspect of both thighs for about ten years. Superficial sensitivity was reduced at the anterior aspect of the left thigh. There were no disturbances of deep sensation.

Article reçu le 28 février 2004. Accepté le 13 septembre 2004. Reprint Request: R. VIALLE, 105, avenue André-Morizet, 92100 Boulogne-Billancourt. e-mail: [email protected]

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The spinal arteriogram showed an intramedullary arteriovenous malformation supplied by a left L2 radiculopial artery and a coronary branch of the anterior spinal artery issuing from a left T10 radiculomedullary artery (figure 1). Within the malformation, numerous vascular ectasias could be distinguished. The malformation drained through a radicular vein connecting with epidural plexuses at left L2 level. Treatment initially comprised in 1982 bilateral embolization of the lumbar arteries L1, L2 and L3, then surgical removal of the main part of the vascular malformation. The operation was performed via a posterior approach after laminectomy from T10 to T12 and opening of the dura. The malformation was totally removed except a minimal portion of residual nidus left in place, encrusted on the left lateral aspect of the spinal cord at the site of emergence of the second lumbar root. The postoperative course was uneventful. The pain decreased but the patient continued to present brief episodes of pain in the left leg. Hypoesthesia remained unchanged. One year after the initial treat-

ment, the neurological status was unchanged. The patient presented deafferentation pain in the area extending from T10 to L4 on the left side. There was a “scoliotic” outline of deformity on clinical examination and X-ray of the area of the laminectomy (figure 2), which was already visible on the first preoperative plates. The patient was afterwards followed-up for several years without any clinical change. Twelve years after the first operation, symptoms progressed again with the onset of a posterior funicular syndrome of the left leg. Plain X-rays showed an accentuation of spinal deformity and vertebral dislocation at L1-L2 level (figure 3). CT scan showed destruction of the vertebral bodies of L1 and L2. Transpedicular bone biopsy showed no proliferation resulting from a tumor. MRI confirmed the destruction of the vertebral bodies of L1 and L2 without involvement of the adjacent soft tissues. Due to the rapid progression of the spinal deformity, corrective fusion was performed via posterior approach (figure 4). Posterior fusion was performed by the use

FIG. 1. — Spinal arteriogram showing spinal arteriovenous malformation (AVm) supplied by a coronary branch of the anterior spinal artery (SAa) issuing from a T10 left radiculomedullary artery (RMa). FIG. 1. — Artériographie médullaire montrant la malformation artério-veineuse (AVm) dont l’afférence principale est une branche coronaire de l’artère spinale antérieure (SAa) issue d’une artère radiculo-médullaire T10 gauche (RMa).

FIG. 2. — Frontal radiograph of thoracolumbar junction, 6 months after surgical treatment of the vascular malformation. Note the moderate right lumbar scoliotic curve. FIG. 2. — Radiographie de face de la charnière thoracolombaire, 6 mois après le traitement chirurgical de la malformation vasculaire médullaire. On note une courbure scoliotique lombaire droite.

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FIG. 3. — Frontal (A) and lateral (B) radiographs of the thoracolumbar junction 12 years after surgical treatment of the vascular malformation. Spinal deformity increased with a progressive thoracolumbar dislocation. FIG. 3. — Radiographie de face (A) et de profil (B) de la charnière thoraco-lombaire, 12 ans après le traitement chirurgical de la malformation vasculaire médullaire. La déformation rachidienne a évolué considérablement, avec constitution progressive d’une dislocation de la charnière thoraco-lombaire.

FIG. 4. — Frontal (A) and lateral (B) radiographs after surgical correction and fusion by posterior approach. FIG. 4. — Radiographie de face (A) et de profil (B) après correction chirurgicale et arthrodèse de la déformation par voie postérieure.

of autogenous bone graft from the posterior iliac crest. Angiography showed a latero-vertebral hypervascularization within the concavity of the deformity (figure 5). The postoperative course was favorable. Fusion was obtained within 6 months without addi-

FIG. 5. — Spinal arteriogram showing hypervascularization within the concavity of residual deformity, three months after surgical correction and fusion. FIG. 5. — Artériographie vertébrale montrant l’hypervascularisation persistant au contact de la concavité de la déformation, trois mois après correction chirurgicale et arthrodèse.

tional anterior arthrodesis (figure 6). Seven years after this last operation, clinical status and radiographs remained unchanged. The patient still complained of some deafferentation pains and proprioceptive disturbances in both legs. Angiography showed local hyperhemia

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FIG. 6. — Sagittal magnetic resonance imaging of the thoracolumbar junction. T2-weighted image, 3 months after surgical treatment (A) of the dislocation showed incomplete anterior fusion. On T1weighted image, 6 months after surgery (B), the fusion was complete. FIG. 6. — Imagerie par résonance magnétique de la charnière thoraco-lombaire. La séquence pondérée en T2 met en évidence, 3 mois après la correction chirurgicale par voie postérieure, une arthrodèse antérieure encore incomplète (A). L’arthrodèse antérieure est complète sur l’imagerie effectuée en séquence pondérée en T1, 6 mois après le traitement chirurgical.

surrounding the residual malformation without circulating shunt. No additional treatment is currently planned for this residual malformation.

DISCUSSION Neurogenic arthropathy was first described at the 19th century, mainly in patients suffering from tertiary syphilis [3, 15]. This is a destructive joint disease which can affect not only the peripheral joints, but also the spine. Neurogenic arthropathy has been described in other diseases such as hemiplegia [11], syringomyelia [11], peripheral neuropathies [16] and diabetes [11]. Eloesser advanced the first etiological hypotheses in 1917 after animal studies [7]. A neurogenic arthropathy appears when a joint is deprived of its means of protective sensation, particularly deep pain sensation. Thus, the joint destruction could result from the exacerbation of the physiological degeneration process, due to stress conditions, in the absence of sensory protective mechanisms of the joint, such as withdrawal reflexes in response to pain and ligamental retraction reflexes. The lack of ligamental retraction reflex could explain the tendency to dislocation of the involved joints. Neurogenic arthropathy of the spine is rare and has been reported chiefly in patients suffering from tabes dorsalis. Spinal involvement occurs in 6 to 21% of patients presenting other neurogenic joint conditions [5, 17, 20]. The thoracolumbar junction is usually involved be-

cause this spinal segment is subject to particular demands from tiny and repeated movements, which keep the trunk balanced at all times in the sitting and upright position. In contrast, cervical, thoracic or sacral segments are exceptionally involved. Many cases have been reported in patients with traumatic paraplegias [2, 9, 15, 17], congenital insensitivity to pain [8, 13] and infections or tumors of the spinal cord [20]. We report the first case of spinal neuropathic arthropathy secondary to a spinal arteriovenous malformation. The loss of deep pain sensation secondary to the arteriovenous medullary lesion could explain the onset of the destructive condition of the thoracolumbar junction. The necessity to perform an extensive laminectomy probably played a role in the development of the spinal deformity. Neurogenic arthropathy can be favored by the impairment of the posterior stabilizing components secondary to laminectomy. This connection has been reported in many cases of spinal neurogenic arthropathy which occurred in paraplegic patients [2, 12, 16]. Nevertheless, the combination of stabilization and fusion by posterior approach does not eliminate the risk of onset of spinal dislocation above or below the site of fusion. In fact, the intervertebral strains are increased at the limits of the fusion site and can favor the occurrence of neurogenic arthropathy, especially when deep sensation is impaired. Thus, a second site neurogenic arthropathy could be observed at the end of a fusion site following

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the initial treatment of a neurogenic arthropathy of the spine [2]. The differential diagnosis is sometimes difficult. Progressive involvement of the spine combining destruction of the vertebral bodies with a soft-tissue “pseudo inflammatory” reaction, forming a veritable “cocoon” around the lesion, often leads to initially suspect an infection [10] or tumor. In the case reported here, hypervascularization within the concavity of the spinal deformity led to suspect a tumor as the cause. Histology obtained by transpedicular biopsy showed only signs of non-specific bone necrosis. The treatment of neuropathic spinal arthropathy, often causing vertebral destruction and considerable instability, is usually surgical. It is necessary to obtain fusion of the affected vertebrae in order to suppress the movements responsible for the progressive destruction of the spine. Initially, conservative treatment was the only method available since osteosynthesis techniques were not sufficiently reliable to treat these major deformities [11, 18]. Bone graft without instrumentation permitted stabilization in many cases [1, 4, 17]. Currently the most appropriate solution to treat these dislocations is anterior and posterior arthrodesis with osteosynthesis, usually posterior [19]. The anterior and posterior procedure could be performed during one or two separate surgical procedures. This heavy therapeutic program is quite often necessary in the case of major destruction of the bone of the vertebral bodies which prevents achievement of sufficient anterior stability [2, 6, 13, 14, 16]. In our case, the major deformity in the frontal and sagittal planes was not associated with major destruction of the vertebral bodies and spontaneous inter-somatic fusion was possible after stabilization and isolated posterior arthrodesis with bone graft and instrumentation. To conclude, neurogenic arthropathy of the spine remains a rare and poorly understood cause of spinal deformity. It occurs most often after traumatic paraplegia, when intervertebral proprioception is impaired. Disturbances of spinal posture may occur in many other neurological contexts, such as spinal cord arteriovenous malformations. In the absence of infectious or neoplastic disease, the occurrence of a spinal deformity with bony destruction and vertebral dislocation should suggest the diagnosis of neuropathic spinal arthropathy and lead to surgical treatment of the affected spinal segments. Further monitoring by clinical examination and X-ray should be maintained, since multifocal vertebral lesions can occur in cases of extensive proprioceptive deficit.

ACKNOWLEDGEMENTS: We are indebted to Prof. Michel Hurth for his help in performing this work. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

REFERENCES [1] BRIGGS JR, FREEHAFER AA. Fusion of the Charcot spine: report of three cases. Clin Orthop 1967 ; 53 : 83. [2] BROWN CW, JONES B, DONALDSON DH, AKMAKJIAN J, BRUGMAN JL. Neuropathic (Charcot) arthropathy of the spine after traumatic spinal paraplegia. Spine 1992 ; 17 : 103-108. [3] CHARCOT JM. Sur quelques arthropathies qui paraissent dépendre d’une lésion du cerveau ou de la moelle épinière. Arch Physiol Norm Pathol 1868 ; 1 : 161-178. [4] CLEVELAND M, WILSON HJ. Charcot disease of the spine. J Bone Joint Surg 1959 ; 41A : 336-340. [5] CULLING J. Charcot’s disease of the spine. Proc R Soc Med 1974 ; 67 : 1026-1027. [6] DEVLIN JV, OGILVIE JW, TRANSFELDT EE, BOACHIEADJEI O, BRADFORD DS. Surgical treatment of neuropathic spinal arthropathy. J Spinal Disord 1991 ; 4 : 319-328. [7] ELOESSER L. On the nature of neuropathic affections of the joints. Ann Surg 1917 ; 56 : 201-207. [8] HEGGENESS MH. Charcot arthropathy of the spine with resulting paraparesis developing during pregnancy in a patient with congenital insensivity to pain. Spine 1994 ; 19 : 95-98. [9] KALEN V, ISONO SS, CHO CS, PERKASH I. Charcot arthropathy of the spine in long-standing paraplegia. Spine 1987 ; 12 : 42-47. [10] KAPILA A, LINES M. Neuropathic spinal arthropathy: CT and MR findings. J Comput Ass Tomogr 1987 ; 11 : 736-739. [11] KEY JA. Clinical observations of tabetic arthropathies (Charcot joints). Am J Syph 1932 ; 16 : 429. [12] LUKE DL, BRIDWELL KH. “Silent” spinal dislocation in a Charcot spine occuring postlaminectomy: case report and review of literature. J Spinal Disord 1990 ; 3 : 87-92. [13] PIAZZA MR, BASSET GS, BUNNELL WP. Neuropathic spinal arthropathy in congenital insensivity to pain. Clin Orthop 1988 ; 236 : 175-179. [14] SCHWARTZ HS. Traumatic Charcot spine. J Spinal Disord 1987 ; 8 : 269-275. [15] SLABAUGH PB, SMITH TK. Neuropathic spine after spinal cord injury: A case report. J Bone Joint Surg 1978 ; 60A : 1005-1006. [16] SOBEL JW, BOHLMAN HH, FREEHAFER AA. Charcot’s arthropathy of the spine following spinal cord injury. J Bone Joint Surg 1985 ; 67A : 771-776. [17] STEINDLER A, WILLIAMS LA, PUIG-GURI J. Tabetic arthropathies. Uro Cutan Rev 1942 ; 46 : 633. [18] THOMAS DF. Vertebral osteoarthropathy or Charcot’s disease of the spine: review of the literature and a report of two cases. J Bone Joint Surg 1952 ; 34B : 248-255. [19] VACCARO AR, SILBER JS. Post-traumatic spinal deformity. Spine 2001 ; 26 : 111-118. [20] WIRTH CR, JACOBS RL, ROLANDER SD. Neuropathic spinal arthropathy: A review of the Charcot spine. Spine 1980 ; 6 : 558-567.