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Intracranial Extradural Hydatid Cysts: Report of Three Cases Ali Canbolat, M.D., (~agatay C)nal, M.D., Umur Kaya, M.D., and Taylan E. ~oban, M.D. Department of Neurosurgery, Istanbul University, School of Medicine, Istanbul, Turkey
Canbolat A, Onal ~, Kaya U, ~oban TE. Intracranial extradural hydatid cysts: report of three cases. Surg Neurol 1994;41:230-4.
Intracranial extradural hydatid cysts are a rare occurrence with unclear pathogenesis. Three different cases are reported and the postoperative courses are discussed. Of the 11 cases found in the literature, all eight cases whose sex and age were reported are men, and seven of the eight are clustered in between the second and fourth decades. KEY WORDS: Computed tomography; Echinococcus; Extradural localization; Intracranial hydatid cyst; Transoral-transpharyngeal-transclival approach
Intracranial hydatid cysts are rare and constitute only 2 % to 3% o f all hydatid cysts reported [1,5,6,10,13,19]. The symptoms o f the disease are similar to those o f brain tumor, and the preoperative diagnosis is of paramount significance as the cyst has to be removed unruptured [ 1 - 1 3 , 1 6 - 1 8 , 2 1 , 2 2 ] . Such cysts can be studied in two major subgroups: extracerebral and intracerebral. Extracerebral hydatidosis, which makes up a very small portion o f the whole group, can be divided into a cranial form, a cranial and/or extradural form, and a combined form with simultaneous intracerebrai, extradural, and cranial lesions [11]. With this classification in mind, the focus o f this report will be the extradural hydatid cysts including the combined form and excluding the pure cranial hydatidosis that is a rare occurrence. In this manuscript three extradural hydatid cyst cases operated in our clinic are reported.
Case 1 A 10-year-old boy was admitted to the clinic in April 1960 with bilateral visual disturbance and symptoms o f increased intracranial pressure o f 2.5 months' duration. Examination revealed anosmia and no response to light on the right side, bilateral optic atrophy, and paralysis
Address reprint requests to: (~a~atay Onal, M.D., Kantara lhza Cad. Ozeng Sok. 4/9 81080 Erenk6y, Istanbul, Tiirkiye. Received March 19, 1993; accepted June 14, 1993.
© 1994 by ElsevierScienceInc.
o f N. Abducens on the left. Slight deficiency o f hearing on the right and fine nystagmoid jerks in the right horizontal position were present. Ventriculogram showed a space-occupying lesion in the middle fossa. Surgical exploration was performed through a right temporal flap and an extradural hydatid cyst with a diameter o f 4 cm situated between the petrous pyramid and superficial sinus petrosus was extracted. Postoperative course was uneventful.
Case 2 A 23-year-old man consulted us for severe headaches o f 1 year's duration, progressive weakness on the left half o f his body, general epileptic attacks that started 5 months previously, slight left facial asymmetry, and mental disturbances. Examination revealed bilateral papilledema, left homonymous hemianopia, left upper motor neuron facial palsy, left-sided hemiparesis, and mental deterioration. Ventriculogram showed a spaceoccupying lesion in the right frontotemporal region. Four days after admission on D e c e m b e r 26, 1971, a right frontotemporal craniotomy was performed. A limited zone o f necrosis o f bone was seen, and dura was pushed upward at the cranial base, with a cyst that measured 12 cm in diameter. The cyst was punctured with a 24-gauge needle, and the contents were aspirated with extreme care. Multiple small hydatid cysts were seen at the inspection o f the cyst cavity and the thinned dura mater next to these cysts had a small defect through which neural tissue protruded. The cysts were removed, the dural gap was repaired, and the cyst cavity was irrigated with formalin solution. The postoperative period was uneventful.
Case 3 An 11-year-old boy was referred to neurosurgery on D e c e m b e r 3, 1985, with complaints o f nausea and occasional vomiting, impairment o f speech, mental dullness, right-sided hearing loss, ataxic gait, and moderate weakness on the left side o f 10 months' duration. H e was alert and oriented. Examination revealed diminished visual acuity, bilateral papilledema, conjugate 0090-3019/94/$7.00
Intracranial Extradural Hydatid Cysts
gaze paralysis, remote motor weakness in mastication, slight facial weakness, and hearing loss all on the right side. He displayed various degrees of paralysis of lower cranial nerves on the right, mild motor weakness, truncal ataxia, and disturbance in coordination on the left, dysarthria, and mental deterioration. Babinski's sign was present bilaterally. Plain roentgenograms of the skull showed an enlarged posterior fossa. Audiogram displayed hypoacusia on the right. Computed tomography (CT) performed on the axial, sagittal, and coronal planes revealed a nonenhancing bilobulated cystic lesion of cerebrospinal fluid density (9 H.U.) located anteriorly to the right of the pons and extending superiorly to tentorium and inferiorly to anterior part of the foramen magnum with a hyperdense capsule in the retroclival region. The fourth ventricle was compressed and the left posterior displacement of the brain stem had caused a triventricular hydrocephalus (Figure 1 A,B,C). A right-sided suboccipital craniectomy was performed on December 9, 1985. After dural opening exploration revealed that the entire brain stem was displaced to the opposite side by an extradural fluctuant mass. The lesion was punctured by a 22-gauge needle, and waterlike cyst fluid was obtained. The puncture site was sealed by bipolar coagulation after removal of 40 cc cyst fluid. Cytologic examination of the fluid showed nothing except for some unidentified round crystalloids. Biochemical examinations showed its composition to be similar to that of blood serum. Follow-up CT scan just after the surgical intervention revealed bifrontal and intraventricular air in frontal horns and an air-fluid level in the punctured lesion. The patient tolerated the operation well on the early postoperative period, but developed tetraparesis on the second day. Bifrontal subdural air was evacuated with subdural puncture, and the CT scan on the second day showed no abnormalities. Tetraparesis with slow resolution was attributed to ischemia due to acute displacement of the brainstem. CT scan performed on the fourteenth postoperative day showed the reaccumulation of the cyst fluid. The neurologic state of the patient remained unchanged during the following 20 days. CT scan performed 35 days after the operation showed the persistent reaccumulation within the cyst. A transoral-transpharyngeal-transclival approach with the preoperative diagnosis of hydatid cyst was performed on January 14, 1986. The pearly white extradural cyst membrane was totally removed. No invasion of bone, and no liquor outflow were noted. Pathologic examination confirmed that the surgical specimen was a parasitic cyst with brood capsules and that the fluid contained the scoleces and hooks of the echinococcus organism. On the third postoperative day fever and signs of meningeal irritation appeared, and purulent cerebrospinal fluid (CSF) was obtained by lum-
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bar puncture. CSF and sputum cultures yielded Klebsiella pneumoniae. Appropriate therapy for meningeal infection and pneumonia was begun. CT scan on the fourth postoperative day showed a small hypodense lesion in front of the clivus, some intrasellar air and no hydrocephalus. Despite extensive therapy, the patient's neurologic state did not show any improvement, and he developed progressive respiratory distress. CT on the sixth postoperative day showed progressive hydrocephalus with transependymal CSF diffusion that required external drainage. Deterioration in clinical state continued and the patient died of septicemia on the ninth postoperative day in a state of decerebrate rigidity. No postmortem examination was permitted.
Discussion Intracranial extradural hydatid cysts are rare cases with still undefined ethiopathology with respect to their anatomic localization. Extracerebral hydatidosis can be divided into a cranial form with solitary or multiple cysts, a cranial and/or extradural form, and a combined form with simultaneous intracerebral, extradural, and cranial lesions [ 15]. Extradural vessels that may carry hexacanth embryos to the extradural space are few or nonexistent, and the existence of primary extradural hydatidosis, therefore, is still a matter of question. In one of Samiy and Zadeh's cases, reported in 1965, with normal skull films circumscribed erosions of the internal table were found during craniotomy, and these were interpreted as primary lesions leading to the development of numerous extradural cysts [ 15]. On the other hand, the extradural space may be infested by intracerebral cysts through a healthy dura mater [12,15]. Tarcan and Griponissiotis attribute this kind of infestation to a skull injury that causes a rupture of the primary cyst attached to the dura mater, letting secondary or daughter cysts out to the extradural space [9,20]. Neurologic symptoms that are caused by pure extradural hydatid cysts are generally less striking than the ones caused by intracerebral cysts. This is probably due to the fact that a great deal of pressure is transmitted extracranially through numerous defects in the bone [15]. A total of nine cases of intracranial extradural hydatid cyst, one of which has been reported by Tarcan from our clinic in 1961 [20], is shown in Table 1. Including the two new cases recorded in this paper, the most astonishing fact is that all of the eight cases whose sex have been reported were men. Another important point is--with the 10-year-old boy reported by Tarcan as an exception--seven of the eight cases whose ages have been documented were around the second and the fourth decades of their lives. This is an unexpected result
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Canbolat et al
A
Figure 1. CT appearance of the cystic lesion in case 3 in the axial (A), sagittal (B), and coronal (C) planes.
considering the predominance in the first decade in previous series of intracranial hydatid cysts. An additional significant point that should be emphasized is the CT appearance of the last case of the report. Computed tomography is of help in preoperative diagnosis and determination of the size and location of cerebral hydatid cysts. The appearance is typical showing a cystic lesion, spherical in shape, with sharply defined border and absorption value similar to that ofcerebrospinal fluid [1,11,13,14,16-19,21,22]. In Turkey, where hydatid disease is endemic, diagnosis with CT presents
no problems. Even though CT appearance of an intracranial hydatid cyst is typical, the lobulated image and the location of the lesion in this specific case were unusual. The unconventional appearance on sagittal and coronal planes as well as its uncommon localization led us away from a confident preoperatory diagnosis ofhydatid cyst. Serologic tests of the disease in case of isolated infestations of the central nervous system gave no clues [1,5]. The last but not the least important fact that should be underlined is about the surgical procedure used in
Intracranial Extradural H y d a t i d Cysts
T a b l e 1.
Surg N e u r o l 1994;41:230-4
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Summary of Nine Previously Reported Intracranial Extradural Hydatid Cyst Cases
Case no.
Date
Age
Sex
Form
1 2
1932 1934
? 28
? Male
Sella turcica Disseminated
3
1950
?
~
4
1957
?
?
5
1960
10
Male
Extradural? Cranial? Multiple extradural with bone invasion + multiple intracerebral = combined form (verified in autopsy) Multiple extradural with bone invasion + intracerebral = combined form Intracerebral + extradural = combined form Solitary extradural
6
1964
23
Male
Cranial + extradural
7
1965
32
Male
Cranial + extradural
Parietal + occipital Frontal
8
1965
17
Male
9
1979
15
Male
Multiple extradural with bone invasion Solitary extradural
Localization
Symptoms ? Increased intracranial pressure + paralysis of lower cranial nerves + retroauricular bulging
Temporal
Postoperative course ) not operated
Griponissiotis
Temporal
Frontal Temporoparietal
6. 7.
8. 9. 10. 11.
12.
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Carrau et al Krebs et al
Goinard et al
~
?
this very specific case. A posterior approach was chosen in the first operation because the diagnostic procedures had not revealed an extradural localization and the decision to puncture the cyst exposed brain to the hazards of spillage of the hydatid cyst fluid with subsequent development of the daughter cysts. It was only appreciated during the second operation that the cyst could have been removed anteriorly with transoraltranspharyngeal-transclival approach without the risk of disseminating the cyst contents. To avoid such a decision, magnetic resonance imaging (MRI), which was not available at that time, could have been a helpful diagnostic examination in a case with such a rare cyst localization.
Authors
14. 15.
16. 17. 18. •
Increased intracranial pressure Increased intracranial pressure Painful bulging on the head Increased intracranial pressure Increased intracranial pressure
Uneventful
Tarcan
Fistula formation after 1 year Uneventful
Samiy-Zadeh
Uneventful
Samiy-Zadeh
Uneventful
Ozgen et al
Samiy-Zadeh
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Canbolat et al
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