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| Year : 2001 | Volume
: 49
| Issue : 3 | Page : 302-4 |
Intramedullary neurenteric cyst in mid thoracic spine in an adult : a case report.
Singhal BS, Parekh HN, Ursekar M, Deopujari CE, Manghani DK
Department of Neurology, Bombay Hospital Institute of Medical Sciences, 12, Marine Lines, Mumbai-400 020, India.
Correspondence Address:
Department of Neurology, Bombay Hospital Institute of Medical Sciences, 12, Marine Lines, Mumbai-400 020, India.
[email protected]
Neurenteric cysts are very rare, particularly in adults. These are congenital intraspinal cysts of endodermal origin. A 67 years old man, presenting with backache and paraesthesiae of one and half years' duration, followed by subacute flaccid paraplegia, developing in a week is described. MRI revealed intramedullary cyst at T7. He underwent emergency thoracic laminectomy and complete excision of the cyst. Histopathology confirmed a neurenteric cyst. In view of their rarity, peculiarity in terms of age, location and presentation, we report this case.
How to cite this article:
Singhal B S, Parekh H N, Ursekar M, Deopujari C E, Manghani D K. Intramedullary neurenteric cyst in mid thoracic spine in an adult : a case report. Neurol India 2001;49:302 |
How to cite this URL:
Singhal B S, Parekh H N, Ursekar M, Deopujari C E, Manghani D K. Intramedullary neurenteric cyst in mid thoracic spine in an adult : a case report. Neurol India [serial online] 2001 [cited 2023 Dec 1];49:302. Available from: https://www.neurologyindia.com/text.asp?2001/49/3/302/1234 |
Enterogenous cysts of the central nervous system are rare cystic lesions characterised by a single epithelial lining resembling that of the cells of the alimentary canal. These cysts occur most frequently in the spinal canal, especially in the lower cervical and upper thoracic regions.[1] There are also rare case reports of cerebellopontine angle, medulla oblongata,[2],[3] lateral ventricles and fourth ventricle location. The malefemale ratio is 2:1 with the average age of 25.8 years with a range from 8 days to 54 years.[3] The rare clinical presentation in an elderly person, histological findings and limited literature on intramedullary spinal cord cyst are discussed in this report.
A 67 year old man presented with progressive tingling and numbness of the right leg that had progressed over one and half years. It started distally in the little toe and then extended progressively upwards towards the hip over a period of one year. He had similar problems in the left leg, which started in the toes and progressed rapidly up to the hip joint within 15 days. He developed acute onset weakness of the both lower limbs, which evolved over three days. He also had urinary retention. There was no history of fever, tuberculosis, neurocutaneous stigmata or trauma. He is a known diabetic on regular oral hypoglycaemic agents. Neurological examination revealed power of grade 0/5 in both lower limbs, with a sensory level at T10. The lower limb reflexes were absent. Routine blood investigations were normal. Magnetic resonance imaging (MRI) of the thoracic spine revealed a sharply marginated intramedullary cyst at the T7 vertebral level [Figure:1a] causing mild focal expansion of the cord. Post contrast T1WI showed faint enhancement of its posterior margin. T2WI showed the cyst fluid to be hyperintense [Figure:1b]. The diagnostic considerations on imaging included arachnoid, epidermoid and neurenteric cysts.
Surgical Findings : Thoracic 6-8 level laminectomies were performed with removal of the intramedullary cyst, which was found at the T7 level. After localising with fluoroscopy, localised microsurgical exploration of T6-8 area was done. On opening the dura, a diffuse cord bulge was seen. No other abnormality was seen on the surface. Midline split revealed a translucent cyst wall. This was very thin and separated easily from the cord tissue, leading into the anterior subarachnoid space. Cyst contained milky fluid and was completely excised.
Histopathology showed a folded cyst wall composed of fibro-collagenous tissue lined mainly by cuboidal cells [Figure:2a] and at places by short columnar cells. The latter, at places, showed a prominent brush border (arrow, [Figure:2b]. Focal squamous metaplasia of lining cells was seen at places.
Cysts lined by columnar epithelium of presumed endodermal derivation have been described using various names, including enterogenic cyst, enterogenous cyst, neurenteric cyst, gastrocytoma, (foregut, respiratory, bronchogenic, epithelial) epithelial lined, archenteric cyst.[1],[4] Embryologically, the notochord plate is interposed between the foregut and the neuroectoderm during the third week of embryonic life. If there are adhesions between the neuroectoderm and the endoderm after their previously closed contact or if there is an accessory neurenteric canal, the notochord may split. Others regard this process as a primary splitting of the notochord resulting from incomplete or faulty excalation.[5],[6],[7],[8],[9],[10] The frequent association with anterior vertebral defects, together with their usual distribution in the lower cervical and thoracic regions, strongly suggests that the formation of enteric cyst is attributable to the failure of separation of the notochord and foregut during formation of the alimentary canal.[1]
The review by Agnoli et al[5] of 33 histologically verified enterogenous intraspinal cysts showed that 18 were located in the cervico-dorsal spine, within the boundaries of C3 and T7; 80% were intradural extramedullary and 12 % were intramedullary lesions. Among 119 patients with intraspinal cysts that were reviewed previously, 76 were males and 43 were females.[11],[12] The diagnosis was established during the first decade of life in 41 patients (34%) and in second decade of life in 27 patients (23%). Only seven patients were more than 50 years of age when their lesions were discovered. Of these 119 cysts, 53 were totally or partially in the thoracic spinal canal. The most common location was in the cervical or thoracic spinal canal, anterior or anterolateral to the spinal cord, with occasional intramedullary involvement as well.[1] Neurenteric cysts may also be associated with anterior or posterior spina bifida, widened vertebral bodies, fused vertebrae, hemivertebrae and diastematomyelia which can be detected by plain roentgenograms and tomography.[13]
The case reported here serves to illustrate that intraspinal neurenteric cysts may occur in isolation, without vertebral bony abnormalities even in adults and older people. This diagnosis, although rare, should be kept in mind for early diagnosis and prompt treatment of this benign lesion.
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| 10. | Palma L, Di Lorenzo N : Spinal endodermal cysts without associated vertebral or other congenital abnormalities. Acta Neurochir (Wien) 1976; 33 : 283-300. |
| 11. | Wilkins RH, Odom GL : Spinal intradural cysts. In : Handbook of Clinical Neurology: Tumours of the Spine and Spinal Cord. Viken PJ, Bruyn GW (eds). Part2, North-Holland Publishing Co, Amsterdam. 1976; Vol 20 : 55-102. |
| 12. | Wilkins Rossitch E Jr : Intraspinal cysts. In : Disorders of the Pediatric Spine, Raven Press, New York. 1995; 445-466. |
| 13. | Wilkins H, Rengachary SS : Neurosurgery 2nd Edition vol. 3 McGraw-Hill, New York. 1996; 3515. |
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