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Neurosarcoid Infiltration of the Ventricular Catheter Causing Shunt Failure: A Case Report
Infection
Neurosarcoid Infiltration of the Ventricular Catheter Causing Shunt Failure: A Case Report Allen H. Maniker, M.D.,* En-Sook Cho, M.D.,† and Michael Schulder, M.D.* University of Medicine and Dentistry of New Jersey, The New Jersey Medical School, *Section of Neurosurgery and †Department of Pathology, Newark, New Jersey
Maniker AH, Cho E-S, Schulder M. Neurosarcoid infiltration of the ventricular catheter causing shunt failure: a case report. Surg Neurol 1997;48:527–9. BACKGROUND
Neurosarcoid is known to develop in 5% of patients with sarcoidosis. A frequent manifestation of this condition is hydrocephalus, which will often require treatment with a ventricular shunt. METHODS
peritoneal shunt placed. She went on to develop multiple shunt failures that on two occasions revealed the noncaseating granulomatous material of the sarcoid occluding the ventricular catheter lumen. Infiltration of sarcoid granulomata into a shunt catheter lumen has not been described before. Presented here is this patient’s history and a discussion of the findings.
Presented here is the case of a patient whose initial manifestation of neurosarcoidosis was hydrocephalus, and who then represented with multiple shunt failures. RESULTS
On two revisions, the proximal catheter, after removal, was found to be occluded with noncaseating granulomatous material that had infiltrated the shunt lumen. CONCLUSIONS
Although shunts may become occluded by inspissated proteinaceous or cellular debris, the occlusion of a shunt catheter by the ingrowth of the noncaseating granulomatous material of neurosarcoid has not been described before. This patient’s course, the histopathologic findings, and neurosarcoidosis in general are discussed here. © 1997 by Elsevier Science Inc. KEY WORDS
Neurosarcoid, shunt failure, hydrocephalus.
arcoidosis is a multisystem granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar lymph adenopathy, pulmonary infiltration, and skin or eye lesions. Neurosarcoidosis is seen in approximately 5% of patients diagnosed with sarcoid. Our patient, who had a known diagnosis of sarcoidosis, subsequently developed hydrocephalus. In standard fashion, the patient had a ventriculo-
S
Address correspondence and reprint requests to: Allen Maniker, M.D., 90 Bergen Street No. 7300, Newark, NJ 07103 Received June 20, 1996; accepted March 24, 1997. © 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
Case Report This 26-year-old black female presented to an outside hospital with a 24-h history of progressive lethargy. A computed tomography (CT) of the head revealed a communicating hydrocephalus of unknown etiology. A ventriculoperitoneal shunt was placed, and the patient initially did well, gradually becoming more active and ambulatory. One week later she became lethargic and after a CT of the head revealed continuing hydrocephalus, a shunt revision was performed. After this procedure she was transferred to University Hospital (Newark, New Jersey) for further management. A magnetic resonance imaging (MRI) did not reveal any mass lesion or enhancement to explain the hydrocephalus. Cerebrospinal fluid (CSF) examination had shown a mildly elevated protein but had an otherwise normal glucose and cell count. Cytology examination of the CSF was negative. Approximately 1 year before her admission she had been diagnosed with sarcoidosis, by bronchial biopsy. On arrival at University Hospital the patient was awake and alert, slightly ataxic of gait, but otherwise intact. A CT scan showed small ventricles, with the shunt in good position. The following morning the patient was extremely lethargic and a repeat CT showed newly enlarged ventricles. The shunt was then revised. This procedure revealed a distal 0090-3019/97/$17.00 PII S0090-3019(97)00305-4
528 Surg Neurol 1997;48:527–9
1
Ventricular catheter occluded with infiltrating sarcoid tissue
shunt catheter malfunction of unknown etiology. The patient did well postoperatively and a CT of the head demonstrated normal ventricular size. The patient presented 4 months later with progressive headaches, lethargy, and vomiting. A CT scan demonstrated ventriculomegaly. A shunt revision was performed. At operation a proximal obstruction was noted and gross inspection of the removed catheter revealed a yellowish-gray fibrinous material that partially filled the catheter tip and attached to the outer wall of the catheter via the holes (Figure 1). The histopathologic examination of the material revealed well developed noncaseating granulomas surrounded by dense chronic inflammation (Figure 2). No organisms were identified on special stains. The material found in the catheter was confirmed as being consistent with sarcoid granulomata. Neurosarcoidosis was diagnosed. The patient was discharged and maintained on a short course (2 weeks) of Prednisone for her sar-
Histomicrograph of material found in ventricular catheter demonstrating noncaseating granulomas of neurosarcoidosis
2
Maniker et al
coidosis. The patient presented again, 15 months later, with sudden onset of headache and neck pain. A CT scan revealed ventriculomegaly. The shunt was revised. Once again, the ventricular catheter was filled with gray-tan fibrinous material that was consistent in appearance with the material removed from the shunt at the previous revision. Histologic preparation revealed well developed noncaseating granulomas with no identifiable organisms, a tissue diagnosis consistent with sarcoid granulomata. The patient’s symptoms resolved and she was discharged on a longer (4 week) course of Prednisone. At 18 months postrevision she remains symptom-free.
Discussion Noncaseating granulomas are the characteristic morphologic feature of sarcoidosis. The sarcoid granuloma consists of a tightly packed central follicle of epithelioid cells surrounded by a perimeter of lymphocytes and fibroblasts. Sarcoid granulomata usually involve multiple organs and the nervous system is involved in approximately 5% of the patients with sarcoidosis [2,4,7]. Central nervous system involvement usually occurs in the earlier phases of the disease, while peripheral nervous system involvement is characteristically seen in the chronic stages [2]. Hydrocephalus, both communicating and noncommunicating, is a relatively common [3] and potentially lethal complication of sarcoidosis. Chronic basilar meningitis with obstruction of CSF flow [2], infiltration of the ventricular system by granulomas [6], granulomatous compression of the aqueduct, or outlet obstruction of the fourth ventricle by granulomas have all been described as causes of hydrocephalus. Free-floating sarcoid granulomas in the CSF have not been described. Other than for the treatment of hydrocephalus, there is rarely a surgical indication for this disease. One paper [1] reports the failure of the ventriculoperitoneal shunt of a neurosarcoid patient; however, it gives no indication for the failure. Our patient clearly demonstrated the presence of noncaseating epithelioid granulomas of neurosarcoid in the tissue removed from the occluded catheter tip. To rule out the presence of foreign debris causing a granulomatous reaction, the tissue obtained from the catheter tip was sectioned and polarized. No foreign debris was found. There is nothing to suggest that there is a property of CSF in sarcoidosis that predisposes ventricular catheter occlusion; however, inflammation of the ependyma that
Neurosarcoid Shunt Infiltration
results from the sarcoid process may have facilitated the attachment of the catheter tip to the adjacent ventricular wall so that the sarcoid granulomas present on the ependymal lining of the ventricle were able to infiltrate the inner lumen of the catheter. Another possibility may involve the tracking of granulomatous material from involved areas of the convexity arachnoid into the catheter lumen during catheter insertion. These theories would conclude that over time this build up of granulomatous tissue caused a complete blockage of the catheter lumen, and subsequently a failure of the shunt. There have been no controlled trials for the treatment of neurosarcoidosis. However, systemic sarcoidosis is often responsive to steroid therapy, and steroids have been recommended for treating neurosarcoidosis [5]. Of note is the fact that after our patient was placed on steroids the time between shunt failures lengthened. There is no literature discussing the treatment of neurosarcoid induced hydrocephalus with anything other than standard ventricular shunting. Blockage of a shunt catheter can occur from a variety of well known sources including blood, abscess/inflammatory products, tumors, choroid plexus, etc. The occurrence of noncaseating granulomatous material within the lumen
Surg Neurol 529 1997;48:527–9
of a shunt catheter has not been described previously and, as seen in this case, should be recognized as a possible cause for shunt failure when evaluating the patient with neurosarcoid. This case demonstrates the need for awareness on the part of the physician of sarcoid granulomatous infiltration as a possible cause of shunt failure. The role of steroids in reducing the frequency of shunt failure in the setting of neurosarcoidosis may deserve further study. REFERENCES 1. Chen RCY, McLeod JG. Neurological complications of sarcoidosis. Clin Exp Neurol 1989;26:99 –112. 2. Delaney P. Neurologic manifestations in sarcoidosis: review of the literature with a report of 23 cases. Ann Intern Med 1977;87:336 – 45. 3. Kendal BE, Tatler GLV. Radiological findings in neurosarcoidosis. Br J Radiol 1978;51:81–92. 4. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909 –17. 5. Turial J, Johns CJ, Teirstein AS. The problem of the treatment of sarcoidosis: report of the subcommittee on therapy. Ann NY Acad Sci 1976;278:743–51. 6. Whelan MA, Stern J. Sarcoidosis presenting as a posterior fossa mass. Surg Neurol 1980;15:455–7. 7. Wiederholt WC, Siekert RG: Neurological manifestations of sarcoidosis. Neurology 1965;15:1147–54.
Neurosarcoid Infiltration of the Ventricular Catheter Causing Shunt Failure: A Case Report Allen H. Maniker, M.D.,* En-Sook Cho, M.D.,† and Michael Schulder, M.D.* University of Medicine and Dentistry of New Jersey, The New Jersey Medical School, *Section of Neurosurgery and †Department of Pathology, Newark, New Jersey
Maniker AH, Cho E-S, Schulder M. Neurosarcoid infiltration of the ventricular catheter causing shunt failure: a case report. Surg Neurol 1997;48:527–9. BACKGROUND
Neurosarcoid is known to develop in 5% of patients with sarcoidosis. A frequent manifestation of this condition is hydrocephalus, which will often require treatment with a ventricular shunt. METHODS
peritoneal shunt placed. She went on to develop multiple shunt failures that on two occasions revealed the noncaseating granulomatous material of the sarcoid occluding the ventricular catheter lumen. Infiltration of sarcoid granulomata into a shunt catheter lumen has not been described before. Presented here is this patient’s history and a discussion of the findings.
Presented here is the case of a patient whose initial manifestation of neurosarcoidosis was hydrocephalus, and who then represented with multiple shunt failures. RESULTS
On two revisions, the proximal catheter, after removal, was found to be occluded with noncaseating granulomatous material that had infiltrated the shunt lumen. CONCLUSIONS
Although shunts may become occluded by inspissated proteinaceous or cellular debris, the occlusion of a shunt catheter by the ingrowth of the noncaseating granulomatous material of neurosarcoid has not been described before. This patient’s course, the histopathologic findings, and neurosarcoidosis in general are discussed here. © 1997 by Elsevier Science Inc. KEY WORDS
Neurosarcoid, shunt failure, hydrocephalus.
arcoidosis is a multisystem granulomatous disorder of unknown etiology, most commonly affecting young adults and presenting most frequently with bilateral hilar lymph adenopathy, pulmonary infiltration, and skin or eye lesions. Neurosarcoidosis is seen in approximately 5% of patients diagnosed with sarcoid. Our patient, who had a known diagnosis of sarcoidosis, subsequently developed hydrocephalus. In standard fashion, the patient had a ventriculo-
S
Address correspondence and reprint requests to: Allen Maniker, M.D., 90 Bergen Street No. 7300, Newark, NJ 07103 Received June 20, 1996; accepted March 24, 1997. © 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
Case Report This 26-year-old black female presented to an outside hospital with a 24-h history of progressive lethargy. A computed tomography (CT) of the head revealed a communicating hydrocephalus of unknown etiology. A ventriculoperitoneal shunt was placed, and the patient initially did well, gradually becoming more active and ambulatory. One week later she became lethargic and after a CT of the head revealed continuing hydrocephalus, a shunt revision was performed. After this procedure she was transferred to University Hospital (Newark, New Jersey) for further management. A magnetic resonance imaging (MRI) did not reveal any mass lesion or enhancement to explain the hydrocephalus. Cerebrospinal fluid (CSF) examination had shown a mildly elevated protein but had an otherwise normal glucose and cell count. Cytology examination of the CSF was negative. Approximately 1 year before her admission she had been diagnosed with sarcoidosis, by bronchial biopsy. On arrival at University Hospital the patient was awake and alert, slightly ataxic of gait, but otherwise intact. A CT scan showed small ventricles, with the shunt in good position. The following morning the patient was extremely lethargic and a repeat CT showed newly enlarged ventricles. The shunt was then revised. This procedure revealed a distal 0090-3019/97/$17.00 PII S0090-3019(97)00305-4
528 Surg Neurol 1997;48:527–9
1
Ventricular catheter occluded with infiltrating sarcoid tissue
shunt catheter malfunction of unknown etiology. The patient did well postoperatively and a CT of the head demonstrated normal ventricular size. The patient presented 4 months later with progressive headaches, lethargy, and vomiting. A CT scan demonstrated ventriculomegaly. A shunt revision was performed. At operation a proximal obstruction was noted and gross inspection of the removed catheter revealed a yellowish-gray fibrinous material that partially filled the catheter tip and attached to the outer wall of the catheter via the holes (Figure 1). The histopathologic examination of the material revealed well developed noncaseating granulomas surrounded by dense chronic inflammation (Figure 2). No organisms were identified on special stains. The material found in the catheter was confirmed as being consistent with sarcoid granulomata. Neurosarcoidosis was diagnosed. The patient was discharged and maintained on a short course (2 weeks) of Prednisone for her sar-
Histomicrograph of material found in ventricular catheter demonstrating noncaseating granulomas of neurosarcoidosis
2
Maniker et al
coidosis. The patient presented again, 15 months later, with sudden onset of headache and neck pain. A CT scan revealed ventriculomegaly. The shunt was revised. Once again, the ventricular catheter was filled with gray-tan fibrinous material that was consistent in appearance with the material removed from the shunt at the previous revision. Histologic preparation revealed well developed noncaseating granulomas with no identifiable organisms, a tissue diagnosis consistent with sarcoid granulomata. The patient’s symptoms resolved and she was discharged on a longer (4 week) course of Prednisone. At 18 months postrevision she remains symptom-free.
Discussion Noncaseating granulomas are the characteristic morphologic feature of sarcoidosis. The sarcoid granuloma consists of a tightly packed central follicle of epithelioid cells surrounded by a perimeter of lymphocytes and fibroblasts. Sarcoid granulomata usually involve multiple organs and the nervous system is involved in approximately 5% of the patients with sarcoidosis [2,4,7]. Central nervous system involvement usually occurs in the earlier phases of the disease, while peripheral nervous system involvement is characteristically seen in the chronic stages [2]. Hydrocephalus, both communicating and noncommunicating, is a relatively common [3] and potentially lethal complication of sarcoidosis. Chronic basilar meningitis with obstruction of CSF flow [2], infiltration of the ventricular system by granulomas [6], granulomatous compression of the aqueduct, or outlet obstruction of the fourth ventricle by granulomas have all been described as causes of hydrocephalus. Free-floating sarcoid granulomas in the CSF have not been described. Other than for the treatment of hydrocephalus, there is rarely a surgical indication for this disease. One paper [1] reports the failure of the ventriculoperitoneal shunt of a neurosarcoid patient; however, it gives no indication for the failure. Our patient clearly demonstrated the presence of noncaseating epithelioid granulomas of neurosarcoid in the tissue removed from the occluded catheter tip. To rule out the presence of foreign debris causing a granulomatous reaction, the tissue obtained from the catheter tip was sectioned and polarized. No foreign debris was found. There is nothing to suggest that there is a property of CSF in sarcoidosis that predisposes ventricular catheter occlusion; however, inflammation of the ependyma that
Neurosarcoid Shunt Infiltration
results from the sarcoid process may have facilitated the attachment of the catheter tip to the adjacent ventricular wall so that the sarcoid granulomas present on the ependymal lining of the ventricle were able to infiltrate the inner lumen of the catheter. Another possibility may involve the tracking of granulomatous material from involved areas of the convexity arachnoid into the catheter lumen during catheter insertion. These theories would conclude that over time this build up of granulomatous tissue caused a complete blockage of the catheter lumen, and subsequently a failure of the shunt. There have been no controlled trials for the treatment of neurosarcoidosis. However, systemic sarcoidosis is often responsive to steroid therapy, and steroids have been recommended for treating neurosarcoidosis [5]. Of note is the fact that after our patient was placed on steroids the time between shunt failures lengthened. There is no literature discussing the treatment of neurosarcoid induced hydrocephalus with anything other than standard ventricular shunting. Blockage of a shunt catheter can occur from a variety of well known sources including blood, abscess/inflammatory products, tumors, choroid plexus, etc. The occurrence of noncaseating granulomatous material within the lumen
Surg Neurol 529 1997;48:527–9
of a shunt catheter has not been described previously and, as seen in this case, should be recognized as a possible cause for shunt failure when evaluating the patient with neurosarcoid. This case demonstrates the need for awareness on the part of the physician of sarcoid granulomatous infiltration as a possible cause of shunt failure. The role of steroids in reducing the frequency of shunt failure in the setting of neurosarcoidosis may deserve further study. REFERENCES 1. Chen RCY, McLeod JG. Neurological complications of sarcoidosis. Clin Exp Neurol 1989;26:99 –112. 2. Delaney P. Neurologic manifestations in sarcoidosis: review of the literature with a report of 23 cases. Ann Intern Med 1977;87:336 – 45. 3. Kendal BE, Tatler GLV. Radiological findings in neurosarcoidosis. Br J Radiol 1978;51:81–92. 4. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and its neurological manifestations. Arch Neurol 1985;42:909 –17. 5. Turial J, Johns CJ, Teirstein AS. The problem of the treatment of sarcoidosis: report of the subcommittee on therapy. Ann NY Acad Sci 1976;278:743–51. 6. Whelan MA, Stern J. Sarcoidosis presenting as a posterior fossa mass. Surg Neurol 1980;15:455–7. 7. Wiederholt WC, Siekert RG: Neurological manifestations of sarcoidosis. Neurology 1965;15:1147–54.