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Letter to the Editor

Cervical stability

Stefan Alexander König

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Aaron A. Cohen-Gadol, Dennis D. Spencer and William E. Krauss

✓ Harvey Cushing's refinement of Halsted's meticulous surgical techniques facilitated safe resection of intradural spinal tumors. Although Cushing focused his attention on brain tumors at the Peter Bent Brigham Hospital, his numerous contributions to the treatment of intradural spinal tumors include the description of these tumors' natural histories and their histological classifications. The application of his experienced intracranial techniques to the resection of spinal tumors improved outcomes. The authors review selected operative notes and sketches to demonstrate his technique in the excision of the spinal cord tumors.

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Federico Roncaroli, Bernd W. Scheithauer and William E. Krauss

Object. Hemangiomas of the spinal nerve roots are rare. Only 12 cases have been reported in the literature, all since 1965. These lesions occurred in adults, primarily in males, and were located in the cauda equina. Of the 12 lesions, eight were cavernous hemangiomas. The authors report here the clinicopathological features of 10 cases of hemangioma of the spinal nerve root, nine of which were of the capillary type.

Methods. The group included six men and four women who ranged in age from 40 to 62 years. The majority of patients presented with pain and weakness. One patient also experienced worsening of pain during menses. In all cases, gadolinium-enhanced magnetic resonance imaging or computerized tomography myelography demonstrated a discrete intraspinal extramedullary mass. Administration of contrast agent resulted in uniform, intense enhancement. A gross-total resection was achieved in all but one case; in most cases, it was necessary to sacrifice the parent nerve root. Preoperative symptoms and signs improved in nine cases. On histological examination, all but one tumor, a spindle-cell hemangioendothelioma, were shown to be ordinary capillary hemangiomas. Two lesions exhibited a cavernous component, and five showed a partly solid growth pattern resembling juvenile capillary hemangioma. In all cases, the relationship with the nerve root was histologically confirmed. The tumor—nerve relation varied. The intact nerve was displaced by the epineurial mass in three cases. The fascicles appeared separated in six cases, and individual nerve fibers were seen throughout one lesion with endoneurial involvement.

Conclusions. Hemangiomas of spinal nerve roots pose a challenging diagnostic problem. Knowledge of their existence is relevant in that they may mimic tumors and are amenable to surgical treatment.

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Aaron A. Cohen-Gadol, William E. Krauss and Robert J. Spinner

Chronic subarachnoid hemorrhage may cause deposition of hemosiderin on the leptomeninges and subpial layers of the neuraxis, leading to superficial siderosis (SS). The symptoms and signs of SS are progressive and fatal. Exploration of potential sites responsible for intrathecal bleeding and subsequent hemosiderin deposition may prevent disease progression. A source of hemorrhage including dural pathological entities, tumors, and vascular lesions has been previously identified in as many as 50% of patients with SS. In this report, the authors present three patients in whom central nervous system SS developed decades after brachial plexus avulsion injury. They believe that the traumatic dural diverticula in these cases may be a potential source of bleeding. A better understanding of the pathophysiology of SS is important to develop more suitable therapies.

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Aaron A. Cohen-Gadol, Patty P. Atkinson and William E. Krauss

✓ Superficial siderosis of the central nervous system is a rare progressive disease associated with hemosiderin deposition on the leptomeninges of the neuraxis. In addition to tumors and vascular lesions, dural sleeve pseudomeningoceles caused by brachial plexus avulsion injury may be the bleeding source in this disease. The authors describe a patient who underwent anterior cervical spine surgery for spinal cord compression due to the ossification of posterior longitudinal ligament. The operation was complicated by a dural tear and subsequent psedomeningocele formation. Nine years later, this patient developed superficial siderosis. The possible mechanisms involved in the development of superficial siderosis in this patient will be discussed.

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Giuseppe Lanzino, Saul F. Morales-Valero and William E. Krauss

Spinal cord hemangioblastomas occur as sporadic lesions or in the setting of Von Hippel-Lindau disease. In this intraoperative video we present a case of sporadic cervical cord hemangioblastoma and illustrate the main surgical steps to achieve safe and complete resection which include: identification and division of the feeding arteries; careful circumferential dissection of the tumor from the surrounding gliotic cord; identification, isolation and division of the main venous drainage and single piece removal of the tumor.

The video can be found here: http://youtu.be/I7DxqRrfTxc.

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Tomohiro Inoue, Aaron A. Cohen-Gadol and William E. Krauss

✓ Almost 40 cases of spontaneous transdural spinal cord herniation have been reported in the literature. These patients often present with gait disturbance and sensory changes, and their condition is diagnosed as Brown—Séquard syndrome. The pathogenesis of this condition has remained poorly understood. In particular, there is no agreement whether the dural defect is acquired or congenital. In the reported case, a 21-year-old man presented with a 3-year history of intermittent low-pressure headaches consistent with intracranial hypotension. Eventually, the headaches resolved but he developed myelopathy due to a spinal cord herniation. In this case, the authors hypothesize that the progressive spinal cord herniation through a spontaneous dural tear sealed the site of the cerebrospinal fluid leak, causing the resolution of headaches.

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Federico Roncaroli, Bernd W. Scheithauer and William E. Krauss

✓ The authors describe the clinicopathological features of four cases of capillary hemangioma of the spinal cord. All occurred in adult patients. The presenting symptoms were similar to those of more common intramedullary tumors. Radiologically, they resemble other vascular spinal cord tumors. All patients underwent surgery, and the outcomes varied. Histologically, the lesions resembled capillary hemangioma of skin or of soft tissue that is composed of lobules of small capillaries with associated feeding vessels, all enveloped by a delicate fibrous capsule. Capillary hemangiomas of the central and peripheral nervous system are extremely rare. Although examples of these lesions have been described as occurring in the dura mater and in peripheral nerve, including spinal nerve roots, none has occurred within the spinal cord. Knowledge of their existence may help practitioners to avoid misdiagnosis of tumor and resultant overtreatment of these benign lesions.

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Cervical pneumatocyst

Case report

Jason M. Hoover, Doris E. Wenger, Laurence J. Eckel and William E. Krauss

The authors present the case of a 56-year-old right hand–dominant woman who was referred for chronic neck pain and a second opinion regarding a cervical lesion. The patient's pain was localized to the subaxial spine in the midline. She reported a subjective sense of intermittent left arm weakness manifesting as difficulty manipulating small objects with her hands and fingers. She also reported paresthesias and numbness in the left hand. Physical and neurological examinations demonstrated no abnormal findings except for a positive Tinel sign over the left median nerve at the wrist. Electromyography demonstrated bilateral carpal tunnel syndrome with no cervical radiculopathy. Cervical spine imaging demonstrated multilevel degenerative disc disease and a pneumatocyst of the C-5 vertebral body. The alignment of the cervical spine was normal. A review of the patient's cervical imaging studies obtained in 1995, 2007, 2008, and 2010 demonstrated that the pneumatocyst was not present in 1995 but was present in 2007. The lesion had not changed in appearance since 2007. At an outside institution, multilevel fusion of the cervical spine was recommended to treat the pneumatocyst prior to evaluation at the authors' institution. The authors, however, did not think that the pneumatocyst was the cause of the patient's neck pain, and cervical pneumatocysts typically have a benign course. As such, the authors recommended conservative management and repeated MR imaging in 6 months. Splinting was used to treat the patient's carpal tunnel syndrome.

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J. Bradley White, Gary M. Miller, Kennith F. Layton and William E. Krauss

Object

Enhancement of pathological entities in the central nervous system is a common finding when the blood–brain barrier has been compromised. In the brain, the presence or absence of gadolinium enhancement is often an indicator of tumor invasiveness and/or grade. In the spinal cord, however, contrast enhancement has been shown in all tumor types, regardless of grade. In this study the authors explore the incidence of nonenhancing tumors of the spinal cord and the clinical course of patients with these lesions.

Methods

A retrospective analysis was conducted in which investigators examined the patterns of enhancement of histologically proven intramedullary spinal cord tumors that had been evaluated at the Mayo Clinic between 1998 and 2002. The tumors that did not enhance were the subject of this report.

Results

A total of 130 patients with intramedullary tumors were evaluated. Of those, 11 patients (9%) had tumors that did not enhance. Histologically, a majority of tumors were astrocytomas (eight low-grade and two high-grade lesions); one tumor was a subependymoma. Morphologically, most of the tumors were diffuse and none had associated cysts. Tumors spanned from two to seven levels and were located throughout the spinal cord (four cervical, three cervicothoracic, one thoracic, and three thoracolumbar). Biopsy procedures were performed in eight patients, subtotal resection was performed in two, and gross-total resection in one. After a mean follow-up period of 19 months, tumors remained stable in eight patients but progressed in three, two of whom died.

Conclusions

A number of intramedullary spinal cord tumors will not enhance after addition of contrast agents. The absence of enhancement does not imply the absence of tumor.