11 Years Old Female with Synergistic Presentation of Cranial and Spinal Pathologies: Myxopapillary Ependymoma with Hydrocephalus - A Rare Entity
Keywords:Cerebrospinal fluid, entriculoperitoneal Shunt, Myxopapillary Ependymoma, rimary Intra Dural Spinal Cord Tumor
The correlation between hydrocephalus and spinal cord tumor was first described by Kyrieleis et. al. 1% concomitant presence was documented by Mirroni et.al. Moreover, the understanding of the pathophysiological association between these two conditions is still lacking. 11 years old girl presented in the outpatient department of Jinnah hospital Lahore with complaints of difficulty in walking for the last 2 years, numbness of right leg for the last one year, and severe bilateral sciatica for 2 months. On further inquiry, she told that she is also suffering from headaches and blurred vision for the last 6 months. Her neuroimaging revealed hydrocephalus and mixed density heterogeneously enhancing intradural lesion at the conus. She underwent ventriculoperitoneal shunting for hydrocephalus and laminoplasty followed by excision of spinal tumor reported being myxopapillary ependymoma grade II. The presence of headache along with signs & symptoms of raised intracranial pressure in these patients warrant cranial imaging. CSF diversion for hydrocephalus and gross total excision of the spinal lesion is the standard acceptable treatment.
Die KW Augenveranderungenbei den entzundlichen, Erkrankungen des Zentralnervensystem. In: Schieck F, Bruckner A (eds) Kurzes Handbuch der Ophtalmologie. 1931; Vol. 2: 779–80.
Huisman T. Pediatric tumors of the spine. Cancer Imaging. 2009; 9 (A): S45–S48.
Mirone G, Cinalli G, Spennato P, Ruggiero C, Aliberti F. Hydrocephalus and spinal cord tumors: a review. Childs Nerv Syst. 2011; 27 (10): 1741-9.
Russell N. Infantile spinal cord tumor presenting as hydrocephalus. Ann Saudi Med. 2000; 20 (4): 244-5.
Caviness J, Tucker M, Pia S, Tam D. Hydrocephalus as a possible early symptom in a child with a spinal cord tumor. Pediatr Neurol. 1998; 18 (2): 169-71.
Rangwala D, Oh G, Barks L, Nikas C. Intramedullary thoracic spine astrocytoma presenting as hydrocephalus in an infant: a case report. Pediatr Neurosurg. 2017; 52: 327-30.
Schijman E, Zúccaro G, Monges JA. Spinal tumors and hydrocephalus. Childs Brain, 1981; 8 (6): 401-5.
Lazzara W, Ragland L, Smith W, Knorr R, Weiner A, Kamath V et al. Basimeningeal/spinal axis primitive neuroectodermal tumor without intraaxial lesion: radiological features. J Neuroimaging. 1998; 8 (1): 49-54.
Gardner WJ, Spitler DK, Whitten C. Increased intracranial pressure caused by increased protein content in the cerebrospinal fluid. An explanation of papilledema in certain cases of small intracranial and intraspinal tumors, and in the Guillain-Barre syndrome. N Engl J Med. 1954; 250: 932-6.
Bamford CR, & Labadie EL. Reversal of dementia in normotensive hydrocephalus after removal of a cauda equina tumor. Case report. J Neurosurg. 1976; 45: 104-7.
Børgesen SE, Sørensen SC, & Olesen J, et al. Spinal tumors associated with increased intracranial pressure. Report of two cases and a discussion on the pathophysiology. Acta Neurol Scand. 1977; 56: 263-8.
Nishida K, Ueda S, & Matsumoto K, et al. Cauda equinaneurinoma associated with normal pressure hydrocephalus: case report. Neurol Med Chir. 1990; 30: 258-62.
Rifkinson-Mann S, Wisoff JH, & Epstein F. The association of hydrocephalus with intramedullary spinal cord tumors: a series of 25 patients. Neurosurgery. 1990; 27: 749-54.
Maurice-Williams RS, & Lucey JJ. Raised intracranial pressure due to spinal tumours: 3 rare cases with a probable common mechanism. Br J Surg. 1975; 62: 92-5.
Dardenne G. Hypertension intracrânienne et contaminations chimiques et cytologiques du liquidecéphalo-rachidien. Acta Neurochir. 1967; 17: 46-70.
Eade OE, & Urich H. Metastasising gliomas in young subjects. J Pathol. 1971; 103: 245-56.
Eden KC. Dissemination of a glioma of the spinal cord in the leptomeninges. Brain. 1938; 61: 298-310.
O'Connell JEA. The subarachnoid dissemination of spinal tumors. J Neurol Neurosurg Psychiatry. 1946; 9: 55-62.
Perese DM, Slepian A, & Nigogosyan G. Postoperative dissemination of astrocytoma of the spinal cord along the ventricles of the brain. A case report. J Neurosurg. 1959; 16: 114-9.
Sloof JL, Kernohan JW, & McCarty CS. Primary Intramedullary Tumors of the Spinal Cord and Filum Terminale. Philadelphia: WB Saunders, 1964; 255.
Woltman HW, Kernohan JW, & Adson AW, et al. Intramedullary tumors of spinal cord and gliomas of intradural portion of filum terminale. Fate of patients who have these tumors. Arch Neurol Psychiatry. 1951; 65: 378-95.
Russell DS & Rubinstein LJ. Pathology of Tumors of the Nervous System, ed 4. London: Edward Arnold, 1989: 431.
Rivierez M, Oueslati S, Philippon J, Pradat P, Foncin JF, Muckensturm B, Dorwling-Carter D, Cornu P. Ependymoma of the intradural filum terminale in adults; 20 cases. Neurochirurgie. 1990; 36: 96-107.
Guyotat J, Fischer G, Remond J, Rizk T, Laham J. Giant ependymoma of the cauda equina. Long-term development apropos of 7 cases. Neurochirurgie. 1993; 39: 85-91.
Gagliardi FM, Cervoni L, Domenicucci J, Celli P, Salvati M. Ependymomas of the filum terminale in childhood: report of four cases and review of the literature. Childs Nerv Syst. 1993; 9: 3-6.
Buge A, Philippon J, Poisson M, Rancurel G, Camenad’Almeida MC. Hydrocephalus during an ependymoma of the cauda equina with meningeal hemorrhage. Possible deductions about the physiopathology of normal-pressure hydrocephalus. Nouv Presse Med. 1974; 3: 1083-6.
Kalff R. Hydrocephalus aresorptivus and parkinsonism caused by a cauda ependymoma. Fortschr Neurol Psychiatr. 1985; 53: 85-7.
Roualdes G, Bataille B, Boissonnot L, Muckensturm B, Salles M, Bontoux D, Gil R, Lefevre JP. Intracranial hypertension and hydrocephalus caused by spinal cord tumors. Apropos of 3 cases. Neurochirurgie. 1987; 33: 216-9.
Phan TG, Krauss WE, Fealey RD. Recurrent lumbar ependymoma presenting as headache and communicating hydrocephalus. Mayo Clin Proc. 2000; 75: 850-2.
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