Spinal intramedullary neuroepithelial (ependymal) cyst. A rare cause of treatable acute para paresis
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《美国医学杂志》
1 Department of Pediatrics, St John's National Academy of Health Sciences, Bangalore, India
2 Department of Pathology, St John's National Academy of Health Sciences, Bangalore, India
3 Department of Radiology, St John's National Academy of Health Sciences, Bangalore, India
Abstract
An 8-yr-old female child presented with acute onset of severe pain in the lower limbs and difficulty in walking. Spine MRI showed hyperintense signals on T2 weighted images at T2-T3 level, which was intramedullary in location. The patient was operated and histopathology reported as neuroepithelial cyst. Spinal intramedullary neuroepithelial cysts are rare. Spinal cord compression due to the cyst is very uncommon and because of its rarity the present case is being reported. The clinical feetures, embryogenesis and literature were reviewed briefly.
Keywords: Neuroepithelial cyst; Spine; Intramedullary
Neuroepithelial cyst are uncommon congenital lesion, majority of which are confined to central nervous system and are commonly located in the paraventricular white matter of the frontal and parietal lobes.[1] Spinal intramedullary neuroepithelial cyst are rare.[2] Only a few pathologically proven cases have been reported in the literature[2],[3],[4],[5],[6],[7],[8],[9],[10].Neuroepithelial cysts are derived from the displaced ependymal cells during embryogenesis.[9],[12] They differ from other congenital enterogenous cysts which result from displaced elements of alimentary canal and do not exhibit goblet cell differentiation.
Case Report
An 8-yr-old female child was admitted to the Hospital with the history of severe pain in the lower limbs and difficulty in walking for 7 days. There was no history of fever, backache or paraesthesia. There was no history of trauma to the back. No family history of tuberculosis was found and there was no history of bladder or bowel involvement. Her past history was not significant and her immunization was complete. On clinical examination the patient was conscious and vitals were normal. Neurological examination revealed no abnormality of higher function & cranial nerves were normal. Upper limbs deep tendon reflexes were marginally exaggerated. In lower limbs tone was increased, deep tendon reflexes were exaggerated with upgoing plantars. There was no sensory loss but hyperesthesia was present in lower limbs. On examination of spine there was no tenderness or deformity present There was no weakness of respiratory muscles and bladder was not palpable. Other systemic examination was within normal limits. The investigations (hemogram & urine analysis) were normal. X-ray of thoracic and lumbosacral spine were normal. MRI at Cervicodorsal Spine revealed a T2 Hyperintense lesion at T2- T3 level, intramedullary in location along the anterior aspect. The cord was expanded with reduction at the anterior and posterior subarachnoid space with diffuse edema. Figure1.The patient was subjected to surgery.
T1- T3 laminectomy was performed and it revealed an intramedullary cystic lesion with well defined capsule. The lesion with the capsule was removed totally and sent for histopathological examination. Intraoperative diagnosis was cystic glioma. Postoperative course was uneventful. Her weakness in the lower limbs improved and she was able to walk without support. Patient was discharged after one week.
The histological examination revealed the following- The wall of the cyst consisted of a thin layer of astrocystic glial tissue with some supporting mesenchyma(delicate connective tissue) . It was lined by single layer of ciliated epithelium like ependyma and partly by cuboidal to flattened epithelium .The epithelium had no basement membrane. No glandular components and smooth muscle fibres could be identified .The histological diagnosis of neuroepithelial cyst was given Figure2.
Discussion
Neuroepithelial cysts are rare cystic lesion outside the central nervous system. In the CNS they are usually seen in relation to the ventricular system, sometimes over the lateral convexities, in the subarachnoid space, intracerebrally in the mesenchephallic region.[10] The first case of intradural ependymal cyst was reported by Hyman et al in 1938.[4] The first two cases of intramedullary ependymal cysts were reported by Fortuna A et al in 1978.[3] Iwahashi (1999) has reported a case of intramedullary ependymal cysts in the dorsal cord in an 18 month old female.[11] These lesions are thought to be of developmental origin. These cysts have been considered to originate from extruded ependymal cells which are located close to the anterior cord substance at the time of closure of the neural tube and these cells have lost continuity and become isolated from the floor plate of the neural tube.[8],[12] The ependymal nature of the epithelium can be identified on the basis of intracellular junctions, the neucleoplasm and the intracellular inclusions.[12] Other ultra structural and immunocytochemical staining features have recently been described to differentiate ependymal from other cysts.[8],[9],[12] There is no evidence of neoplastic transformation of the cyst wall. Rather the expansion of the cyst appears to be from active secretion of the fluid into cystic cavity. MRI depicted the cyst as a well defined area of low density without contrast enhancement. It is usually difficult to differentiate these cysts pre-operatively from other cystic lesions including dermoid, epidermoid, arachnoid cyst or cystic glioma. Since the neuro radiological findings and clinical features are nonspecific, surgery is the treatment of choice for intramedullary spinal neuroepithelial cyst before it causes irreversible damage.
References
1. Lantos PL, Vandenberg SR, Kleihues Paul. Cysts and tumour-like conditions. In: David I Graham, Peter L Lantos, eds. Greenfield's Neuropathology, 6th edn. London, Newyork, 1997; 2 : 780-786.
2. Fortuna A, Mercuri S. Intradural spinal cysts. Acta Neurochir (Wien) 1983; 68 : 289-314.
3. Dharker SR, Kanhere S, Dharker RS, Intramedullary epithelial cyst of spinal cord. Surg Neuro l979; 12 : 443-444.
4. Fortuna A, Palma L, Mercuri S. Spinal neuroepithelial cysts. Report of two cases and review of the literature. Acta Neurochir(Wien) 1978; 45 : 177-185.
5. Hyman I, Hamby WB, Sanes S. Ependymal cysts of the cervicodorsal region of the spinal cord. Arch Neurol Psychiat 1938; 40 : 1005-1012.
6. Mosso JA, Verity MA. Ependymal cysts of the spinal cord:Case report. J Neurosurg 1975; 43 : 757-760.
7. Osenbach RK, Godersky JC, Traynelis VC et al. Intradural extramedullary cysts of spinal canal: Clinical presentation, Radiographic diagnosis and surgical management. Neurosurgery 1992; 30 : 35-42.
8. Pagni CA, Canavero S. Vinattieri A et al. Intramedullary spinal ependymal cyst: Case report. Surg Neurol 1991; 35 : 325-328.
9. Robertson DP, Kirkpatrick JB, Harper RL et al. Spinal intramedullary ependymal cyst. Report of three cases. J Neurosurg 1991; 75 : 312-316.
10. Sharma BS, Banerjee AK, Khosla VK et al. Congenital intramedullary spinal ependymal cyst. Surg Neurol 1987; 27 : 476-480.
11. Iwahashi H, Kawai S, Watabe Y et al. Spinal intramedullary ependymal cyst: A case report. Surg Neurol 1999; 52 : 357-361.
12. Hirano A, Ghatak NR, Wisoff HW et al. An epithelial cyst of the spinal cord. An electron microscopic study. Acta Neuropathologica 1971; 18 : 214-223.(Lalitha AV, Rout Pritilat)
2 Department of Pathology, St John's National Academy of Health Sciences, Bangalore, India
3 Department of Radiology, St John's National Academy of Health Sciences, Bangalore, India
Abstract
An 8-yr-old female child presented with acute onset of severe pain in the lower limbs and difficulty in walking. Spine MRI showed hyperintense signals on T2 weighted images at T2-T3 level, which was intramedullary in location. The patient was operated and histopathology reported as neuroepithelial cyst. Spinal intramedullary neuroepithelial cysts are rare. Spinal cord compression due to the cyst is very uncommon and because of its rarity the present case is being reported. The clinical feetures, embryogenesis and literature were reviewed briefly.
Keywords: Neuroepithelial cyst; Spine; Intramedullary
Neuroepithelial cyst are uncommon congenital lesion, majority of which are confined to central nervous system and are commonly located in the paraventricular white matter of the frontal and parietal lobes.[1] Spinal intramedullary neuroepithelial cyst are rare.[2] Only a few pathologically proven cases have been reported in the literature[2],[3],[4],[5],[6],[7],[8],[9],[10].Neuroepithelial cysts are derived from the displaced ependymal cells during embryogenesis.[9],[12] They differ from other congenital enterogenous cysts which result from displaced elements of alimentary canal and do not exhibit goblet cell differentiation.
Case Report
An 8-yr-old female child was admitted to the Hospital with the history of severe pain in the lower limbs and difficulty in walking for 7 days. There was no history of fever, backache or paraesthesia. There was no history of trauma to the back. No family history of tuberculosis was found and there was no history of bladder or bowel involvement. Her past history was not significant and her immunization was complete. On clinical examination the patient was conscious and vitals were normal. Neurological examination revealed no abnormality of higher function & cranial nerves were normal. Upper limbs deep tendon reflexes were marginally exaggerated. In lower limbs tone was increased, deep tendon reflexes were exaggerated with upgoing plantars. There was no sensory loss but hyperesthesia was present in lower limbs. On examination of spine there was no tenderness or deformity present There was no weakness of respiratory muscles and bladder was not palpable. Other systemic examination was within normal limits. The investigations (hemogram & urine analysis) were normal. X-ray of thoracic and lumbosacral spine were normal. MRI at Cervicodorsal Spine revealed a T2 Hyperintense lesion at T2- T3 level, intramedullary in location along the anterior aspect. The cord was expanded with reduction at the anterior and posterior subarachnoid space with diffuse edema. Figure1.The patient was subjected to surgery.
T1- T3 laminectomy was performed and it revealed an intramedullary cystic lesion with well defined capsule. The lesion with the capsule was removed totally and sent for histopathological examination. Intraoperative diagnosis was cystic glioma. Postoperative course was uneventful. Her weakness in the lower limbs improved and she was able to walk without support. Patient was discharged after one week.
The histological examination revealed the following- The wall of the cyst consisted of a thin layer of astrocystic glial tissue with some supporting mesenchyma(delicate connective tissue) . It was lined by single layer of ciliated epithelium like ependyma and partly by cuboidal to flattened epithelium .The epithelium had no basement membrane. No glandular components and smooth muscle fibres could be identified .The histological diagnosis of neuroepithelial cyst was given Figure2.
Discussion
Neuroepithelial cysts are rare cystic lesion outside the central nervous system. In the CNS they are usually seen in relation to the ventricular system, sometimes over the lateral convexities, in the subarachnoid space, intracerebrally in the mesenchephallic region.[10] The first case of intradural ependymal cyst was reported by Hyman et al in 1938.[4] The first two cases of intramedullary ependymal cysts were reported by Fortuna A et al in 1978.[3] Iwahashi (1999) has reported a case of intramedullary ependymal cysts in the dorsal cord in an 18 month old female.[11] These lesions are thought to be of developmental origin. These cysts have been considered to originate from extruded ependymal cells which are located close to the anterior cord substance at the time of closure of the neural tube and these cells have lost continuity and become isolated from the floor plate of the neural tube.[8],[12] The ependymal nature of the epithelium can be identified on the basis of intracellular junctions, the neucleoplasm and the intracellular inclusions.[12] Other ultra structural and immunocytochemical staining features have recently been described to differentiate ependymal from other cysts.[8],[9],[12] There is no evidence of neoplastic transformation of the cyst wall. Rather the expansion of the cyst appears to be from active secretion of the fluid into cystic cavity. MRI depicted the cyst as a well defined area of low density without contrast enhancement. It is usually difficult to differentiate these cysts pre-operatively from other cystic lesions including dermoid, epidermoid, arachnoid cyst or cystic glioma. Since the neuro radiological findings and clinical features are nonspecific, surgery is the treatment of choice for intramedullary spinal neuroepithelial cyst before it causes irreversible damage.
References
1. Lantos PL, Vandenberg SR, Kleihues Paul. Cysts and tumour-like conditions. In: David I Graham, Peter L Lantos, eds. Greenfield's Neuropathology, 6th edn. London, Newyork, 1997; 2 : 780-786.
2. Fortuna A, Mercuri S. Intradural spinal cysts. Acta Neurochir (Wien) 1983; 68 : 289-314.
3. Dharker SR, Kanhere S, Dharker RS, Intramedullary epithelial cyst of spinal cord. Surg Neuro l979; 12 : 443-444.
4. Fortuna A, Palma L, Mercuri S. Spinal neuroepithelial cysts. Report of two cases and review of the literature. Acta Neurochir(Wien) 1978; 45 : 177-185.
5. Hyman I, Hamby WB, Sanes S. Ependymal cysts of the cervicodorsal region of the spinal cord. Arch Neurol Psychiat 1938; 40 : 1005-1012.
6. Mosso JA, Verity MA. Ependymal cysts of the spinal cord:Case report. J Neurosurg 1975; 43 : 757-760.
7. Osenbach RK, Godersky JC, Traynelis VC et al. Intradural extramedullary cysts of spinal canal: Clinical presentation, Radiographic diagnosis and surgical management. Neurosurgery 1992; 30 : 35-42.
8. Pagni CA, Canavero S. Vinattieri A et al. Intramedullary spinal ependymal cyst: Case report. Surg Neurol 1991; 35 : 325-328.
9. Robertson DP, Kirkpatrick JB, Harper RL et al. Spinal intramedullary ependymal cyst. Report of three cases. J Neurosurg 1991; 75 : 312-316.
10. Sharma BS, Banerjee AK, Khosla VK et al. Congenital intramedullary spinal ependymal cyst. Surg Neurol 1987; 27 : 476-480.
11. Iwahashi H, Kawai S, Watabe Y et al. Spinal intramedullary ependymal cyst: A case report. Surg Neurol 1999; 52 : 357-361.
12. Hirano A, Ghatak NR, Wisoff HW et al. An epithelial cyst of the spinal cord. An electron microscopic study. Acta Neuropathologica 1971; 18 : 214-223.(Lalitha AV, Rout Pritilat)