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Extensive Arachnoid Ossification with Associated Syringomyelia Presenting As Thoracic Myelopathy Case Report and Review of the Literature

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Extensive Arachnoid Ossification with Associated Syringomyelia Presenting As Thoracic Myelopathy Case Report and Review of the Literature Neurosurg Focus 6 (5):Article 9, 1999 Extensive arachnoid ossification with associated syringomyelia presenting as thoracic myelopathy Case report and review of the literature Konstantin V. Slavin, M.D., Randall R. Nixon, M.D., Ph.D., Gary M. Nesbit, M.D., and Kim J. Burchiel, M.D., F.A.C.S. Departments of Neurological Surgery, Pathology, and Radiology, Oregon Health Sciences University, Portland, Oregon The authors present the case of a patient in whom progressive thoracic myelopathy was caused by the extensive ossification of the arachnoid membrane and associated intramedullary syrinx. Based on their findings and the results of a literature search, they describe a pathological basis of this rare condition, discuss its incidence and symptomatology, and suggest a simple classification of various types of the arachnoid ossification. They also discuss magnetic resonance imaging features of arachnoid ossification and associated spinal cord changes. Emphasis is placed on the particular value of plain computerized tomography, which is highly sensitive for detecting intraspinal calcifications and ossifications, in the diagnostic evaluation of patients whose clinical picture indicates progressive myelopathy. Key Words * arachnoiditis * ossification * myelopathy * syringomyelia * thoracic spine Of the various causes of spinal cord compression, calcification and ossification of the arachnoid membrane are relatively rare. It appears that there are three distinct types of calcification, of which two carry some clinical significance due to the production of symptoms. The first type, with microscopic calcifications, is frequently encountered in asymptomatic patients during spine surgeries and in autopsy series of the general population. The second type involves formation of large isolated calcified and ossified plaques within, and adjacent to, the arachnoid membrane. This entity has been repeatedly described in patients presenting with signs of spinal cord compression, some of whom improved after undergoing surgical removal of the plaque(s). The third type is the least common, consisting of diffuse ossification of the arachnoid membrane at one or several segments of the spinal cord and may be associated with larger ossified masses such as those encountered in the second type. Because of the relative rarity of these conditions, it is not known whether all three types are the consequent stages of the same progressive disease. The development of modern imaging techniques raises the possibility that patients with this disease may be followed to determine the entity's natural history. Unauthenticated | Downloaded 10/02/21 07:26 AM UTC In this report we describe a patient in whom diffuse arachnoid membrane ossification at the thoracic level was pathologically and radiologically verified, and we review the current literature on this subject. CASE REPORT This 54-year-old woman presented with a 9-year history of progressive spastic lower paraparesis and bilateral sensory loss in her legs that extended to the T-8 level. History. Without apparent reason, the patient began to experience symptoms of pain, tightness, and discomfort in the left side of her chest. She had not sustained any major injuries, meningitis, systemic infection, or subarachnoid hemorrhage, and she was otherwise healthy. At the time of her initial neurological evaluation in 1991, she was found to have decreased left-sided sensation to pin-prick stimulation and temperature from the level of T-8 to the knee. Mild impairment of the superficial sensation on the right side of the chest wall was also noted. The patient was found to have a subtle decrease in strength and coordination in the left foot as compared with the right, with a positive Babinski sign on the left. At that time, magnetic resonance (MR) imaging of the thoracic spine revealed a large multiseptated syrinx that extended from T-7 to the conus medullaris at the L2­3 level (Fig. 1). No mass lesions, herniated discs, or other abnormalities were demonstrated, and no abnormal enhancement was observed anywhere within the spinal cord. There was no tonsillar herniation. Fig. 1. Axial T1-weighted MR images. Left: Image obtained at the T-11 level revealing a hypointense intramedullary cavity (wide arrow) and bright hyperintense mass adjacent to the dorsolateral surface of the spinal cord (short arrow). Right: Image obtained at the T-8 level demonstrating large intramedullary syrinx and irregular hypointense rim (arrows) around the spinal cord suggestive of arachnoid ossification. (Study performed in June of 1992.) The patient gradually deteriorated over the next several years despite undergoing conservative treatment. Her sensory loss became more apparent on the right side, and the spastic paraparesis worsened to the point that she could not walk without holding onto something. The left foot drop was supported by external orthosis. She lost most of her lower-extremity proprioception and began using visual reference Unauthenticated | Downloaded 10/02/21 07:26 AM UTC to maintain balance. Numbness in both legs was associated with a tingling sensation and feeling of tightness. She developed chronic constipation and difficulties initiating urination as well as episodes of urinary incontinence. Repeated MR images obtained on several occasions demonstrated no changes in the syrinx (Fig. 2). Fig. 2. Sagittal T1 (left) and T2-weighted (right) MR images of the lower thoracic spine revealing a multiloculated intramedullary cavity extending from T8­12. The ossified lesion at T-11 posteriorly shown at Fig. 1 left appears hyperintense on T1 and hypointense on T2-weighted images (arrows). There is an abrupt lack of normal CSF signal at T7­8 posterior to the spinal cord (curved arrow). (Study performed in July of 1998.) In 1997, the patient developed a herpetic rash on the right side of her chest in the area corresponding to the upper edge of her sensory loss. Subsequently, she developed a postherpetic neuralgia with associated hyperesthesia and pain that partially responded to oral carbamazepine therapy. Presentation. The patient continued to work as a school teacher. In need of pain management, she was referred to us for consideration of surgical treatment of her intramedullary syrinx. She was presented with the available therapeutic options and chose to proceed with surgery. Operation. During surgical exploration, a T9­11 laminectomy was performed. After opening the dura, the spinal cord was found to be covered with a light gray hard shell, which appeared to be calcified arachnoid membrane. Using the microscope, we opened it and then carefully peeled it away from the substance of the spinal cord. This shell tracked ventrally on both sides and had to be sharply transected on the left and superiorly, and then pulled out in two irregularly shaped pieces that measured 14 X 8 mm and 6 X 8 mm, respectively. It appeared to be circumferentially encasing the spinal cord like an armor but did not follow the exiting nerve roots. In the superior (cephalad) aspect of the dural opening it continued under the dura, but in the caudal direction the spinal cord was visually free of the abnormal arachnoid. On gross inspection of the shell pieces a smooth outer surface facing the dura and an irregular Unauthenticated | Downloaded 10/02/21 07:26 AM UTC rough surface facing the spinal cord were observed. An additional thicker ossified piece (3 X 3 X 5 mm), removed from the left dorsal surface of the spinal cord, left a deep groove on the surface of the cord. Immediately superior to that, a large vascular structure that resembled a venous malformation was visualized; it was coagulated and left in place. After that, a longitudinal midline myelotomy was performed, and the intramedullary cyst was entered. The inner surface of the cyst was smooth and white, with no septations. The cyst continued caudally as a single compartment. Once the cyst was opened, the spinal cord collapsed, and the edges of the myelotomy were reapproximated. A K-tube was inserted into the cyst and tunneled under the skin into subcutaneous pocket. A duraplasty was performed with a dural substitute, and the incision closed by standard techniques. One week later, the syringoperitoneal shunt was completed. Histological Findings. Histological examination of the removed plaques revealed metaplastic woven and lamellar bone enclosed by dense fibrous tissue that was consistent with the arachnoidal location (Fig. 3). The bone fragments contained normal bone marrow constituents. No inflammation was present. A small fragment of spinal cord white dura mater was also examined and found to have no diagnostic abnormalities. Fig. 3. Photomicrographs of the resected ossified sample of the thoracic arachnoid membrane. Upper: Polarized light. The plaque has the appearance of the normal bone with woven and lamellar elements and is surrounded by dense fibrous tissue representing the Unauthenticated | Downloaded 10/02/21 07:26 AM UTC arachnoid. Normal bone marrow elements are seen inside the plaque. H&E; original magnification X 20. Postoperative Course. In the immediate postoperative period the patient developed worsening of her paraparesis, no change in sensation, and a slight improvement in the dysesthetic pain. She required intermittent bladder catheterization, but due to the effects of oral laxatives and stool softeners, her constipation improved. She was discharged to the rehabilitation facility, where the treatment goals of an interdisciplinary team were to improve her mobility, regain bladder control, and to reduce her postoperative pain. Postoperative computerized tomography (CT) scanning of the thoracic spine revealed an extensive ossification surrounding the spinal cord that extended from the uppermost slice at T-6 where it was predominantly posterolateral on the left. At the level of T7­8, the ossification became circumferential (Fig. 4 left) and then disappeared at the upper border of T-9, which corresponded to the upper end of the surgical exploration (Fig. 4 right). Fig. 4. Axial CT scans at the levels of T-8 (left) and T-11 (right) after the patient underwent a T9­11 laminectomy. A circumferential ossification can be seen encasing the spinal cord at midthoracic level (arrows).
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