Journal of Neuro-Ophthalmology, December 1998, Volume 18, Issue 4

Homonymous Hemianopsia Due to a Dural Cavernous Hemangioma

The clinical and radiographic features of extra-axial cavernous hemangiomas are described, and a case of homonymous visual field loss due to a dural-based occipital cavernous hemangioma is reported. A patient presented with a homonymous hemianopsia due to an enhancing tentorial mass lesion. The preoperative clinical and magnetic resonance imaging features were suggestive of meningioma. The patient underwent gross total resection of the lesion and the final pathologic examination was consistent with cavernous hemangioma. There was complete resolution of the visual field defect after surgery. Extra-axial cavernous hemangiomas differ from intra-axial cavernous hemangiomas in their clinical and radiographic features. The former lesions may mimic meningioma and should be considered in the differential diagnosis of a dural-based mass. Early recognition of the lesion is important because surgical removal of cavernous hemangiomas may be associated with a higher morbidity and mortality rate than meningiomas.

Journal of Neiiro- Ophllwlmnlogy 18( 4): 250- 254, 1998. © 1998 Lippincott Williams & Wilkins, Philadelphia Homonymous Hemianopsia Due to a Dural Cavernous Hemangioma Andrew G. Lee, M. D., Robert G. Parrish, M. D., and J. Clay Goodman, M. D. The clinical and radiographic features of extra- axial cavernous hemangiomas are described, and a case of homonymous visual field loss due to a dural- based occipital cavernous hemangioma is reported. A patient presented with a homonymous hemianop­sia due to an enhancing tentorial mass lesion. The preoperative clinical and magnetic resonance imaging features were sugges­tive of meningioma. The patient underwent gross total resection of the lesion and the final pathologic examination was consis­tent with cavernous hemangioma. There was complete resolu­tion of the visual field defect after surgery. Extra- axial cavern­ous hemangiomas differ from intra- axial cavernous hemangio­mas in their clinical and radiographic features. The former lesions may mimic meningioma and should be considered in the differential diagnosis of a dural- based mass. Early recog­nition of the lesion is important because surgical removal of cavernous hemangiomas may be associated with a higher mor­bidity and mortality rate than meningiomas. Key Words: Dural cavernous hemangioma- Homonymous hemianopsia. Intracranial cavernous hemangiomas ( CH) are vascu­lar hamartomas that usually occur intra- axially within the cerebral hemispheres ( 1- 20). Although they may arise from extra- axial locations, dural- based lesions are rare ( 1- 20). We report a case of a dural- based occipital hem­angioma that presented with a homonymous hemianop­sia. CASE REPORT A 53- year- old white man presented with blurry vision in both eyes. Medical history was significant for removal of a malignant melanoma from his right forearm in De­cember of 1991. Complete metastatic evaluation at that time, including bone scan, liver function tests, and chest Manuscript received November 18, 1997; accepted February 18, 1998. From the Departments of Ophthalmology ( A. G. L.), Neurology ( A. G. I.., J. C. G.), Neurosurgery ( A. G. L., J. C. G., R. G. P.), and Pathology ( J. C. G.), Baylor College of Medicine, and the Division of Neurosur­gery ( A. G. L.), the M. D. Anderson Cancer Center, the University of Texas, Houston, Texas, U. S. A. Address correspondence and reprint requests to Andrew G. Lee, M. D., Department of Ophthalmology, Baylor College of Medicine, 6565 Fannin St. NC- 205, Houston, TX 77030, U. S. A. radiograph, was unremarkable. Over the next 5 years, there was no evidence for metastatic melanoma or local recurrence. He was well until January 9, 1997, when he experienced painless blurry vision in both eyes. Ophthal­mologic examination showed a visual acuity of 20/ 20 in both eyes. The pupils were equal in size, reacted nor­mally to light, and there was no afferent pupillary defect. Automated visual field testing ( Humphrey 30- 2) re­vealed a right homonymous superior quadrantanopsia ( Fig. 1). Slit- lamp biomicroscopy, motility examination, intraocular pressure measurements, and ophthalmoscopic examination were negative in both eyes. Magnetic reso­nance ( MR) scanning of the head demonstrated a 2- cm lobular lesion in the left occipital area with a broad base against the inferior surface of the occipital lobe abutting the left tentorium near the midline ( Fig. 2). There was heterogenous signal intensity on the double- echo and Tl-weighted MR images, with relatively homogenous en­hancement of the lesion after the administration of gad-olinium- diethylaminetriaminepentaacetic acid ( DTPA). The tentorium adjacent to the lesion was slightly thick­ened and demonstrated mild homogenous enhancement. There was very little surrounding cerebral edema and only slight regional mass effect on the convexity sulci, which were slightly effaced. The radiologic diagnosis based on the MR features was meningioma. The patient was treated with oral steroids. A cerebral arteriogram was performed. The left common carotid artery injection demonstrated a hypervascular, extra- axial, dural- based mass in the region of the left tentorial incisura that was supplied by the artery of Bernasconi and Cassinari and distal branches of the occipital artery. The right common carotid artery injection demonstrated no abnormal vas­cularity. The left vertebral artery injection demonstrated the hypervascular mass with arterial supply from the dis­tal branches of the posterior meningeal artery of the left vertebral artery and distal tentorial branches of the supe­rior cerebellar artery. The hypervascularity was reticular in nature, appeared early in the arterial phase of the ar­teriogram, and persisted in the late venous phase. It was thought that the angiographic characteristics of the hy­pervascular mass were consistent with the clinical diag­nosis of meningioma. The staining pattern, however, was thought to be slightly atypical in that the lesion was slightly more coarse and had a less well defined border 250 HEMIANOPSIA DUE TO DURAL CAVERNOUS HEMANGIOMA 251 JSH :: « fc:"" « J FOV FflSTftt JRIJ 9? FOV ^ iCK - 5 - 8 ? OB FfiSTPK FIG. 1. Automated visual field testing ( Humphrey •.#.-..•;_ 30- 2) showed a right homonymous superior qua- "^"' "•- drantanopsia. • 3.78 DB than that seen in typical meningioma. On January 21, 1997, the patient underwent a left combined supratento-rial and infratentorial approach and gross total excision of the tentorial mass. A vascular tumor was encountered at the time of surgery. The tumor was completely excised from the dura and the margin of the tentorium was ex­tensively coagulated. There were no postoperative com­plications. Pathologic examination showed multiple, thick- walled vascular channels with a thick endothelial lining consistent with the diagnosis of CH ( Fig. 3). There were a few foci of lymphocytic infiltrates, old hemor­rhage, hemosiderin, and extramedullary hematopoiesis. There was no evidence for cellular tumor such as me­ningioma. On April 11, 1997, a repeat MR scan of the head demonstrated postoperative changes and a small area of persistent enhancement near the midline of the left tentorium and adjacent incisura. A repeat automated visual field in April, 1997 ( Humphrey 30- 2) revealed complete resolution of the homonymous hemianopsia. DISCUSSION Cavernous hemangiomas are vascular malformations characterized histopathologically by abnormal collec­tions of thin- walled sinusoidal spaces lined by endothe­lium and connective tissue ( 7). Cavernous hemangiomas usually are intra- axial tumors of the cerebral hemi­spheres. Cavernous hemangiomas arising from the dura are rare, but when they occur usually arise in the middle cranial fossa ( 3,7,8,11,12,13,17,19). Simard et al. re­viewed 126 cases from the literature ( 1960- 1986) and 12 additional cases of histologically confirmed CH ( 19). Of these cases, 13 were extra- axial in the middle cranial fossa, 4 were in the cerebellopontine angle, and only 1 was in association with the tentorium ( 19). Namba in 1983 reviewed the world literature on CH and reported only 23 cases of extracerebral CH of the middle fossa ( 13). In this review, 10 patients ( 44%) demonstrated ocu­lar symptoms or signs, including diplopia, anisocoria, visual acuity loss, visual field defects, or ocular motor cranial neuropathy ( 13). McCormick and Butler re­viewed nearly 500 vascular malformations of the central nervous system and reported 2 asymptomatic cases of CH of the tentorium cerebelli and mentioned 3 other cases from the German and Italian literature ( 10). Other CH have been reported in the cavernous sinus ( 7,8,13, 17), cerebellopontine angle ( 1), internal auditory canal ( 20), Meckel's cave ( 2), cauda equina ( 14), cranial nerves ( 9), peripheral nerves, optic nerves and chiasm, within the ventricular system ( 4), anterior cranial fossa ( 3), and tentorium ( 12,16,18). Among the extra- axial sites, the large number of cases reported in the cavernous sinus ( 7,8,3,17) may suggest a predilection for this loca­tion. Extra- axial CH differ clinically and radiographically from intra- axial CH ( 1- 20). Meyer et al. emphasized that despite similar pathologic features, these two entities are clinically distinct ( 11). These authors argued that extra-axial CH demonstrated features suggestive of neoplasm, including mass effect, encasement of neurovascular structures, growth in pregnancy, and radiographic fea­tures suggestive of tumor. In fact, unlike intra- axial CH, the clinical and radiologic features may mimic meningi­oma before surgery ( 7,15,17). Meyer et al. reported eight extra- axial CH, of which six arose from the cavernous sinus, one from the petrosal sinus, and one from within the torcular Herophili. The preoperative diagnosis was meningioma in seven of these eight patients ( 11). Isla et al. reported a large CH of the dura in the ante­rior fossa of a pregnant woman ( 3). The lesion resembled a meningioma and was difficult to remove because of marked vascularity and profuse bleeding after minimal incision of the capsule ( 3). Perry et al. reported a 77- year- old woman with partial seizures due to an enhanc­ing dural- based parietal convexity mass. Although the preoperative appearance on computed tomography ( CT) scan suggested meningioma, the pathologic findings were typical of a dural CH ( 15). Saldana et al. reported a neonatal CH of the dura matter and reviewed the lit­erature on congenital CH ( 18). Quattrocchi et al. reported a CH of the tentorium cerebelli and reviewed six other cases from the literature. Of these seven cases, two pre­sented with headaches, one with congenital hydroceph­alus, and four were asymptomatic ( 16). Our patient pre­sented with a homonymous visual field defect. J Neitro- Ophlhalnml. Vol. IK No. 4. IWfi 252 A. G. LEE ET AL. FIG. 2. A: Axial T1- weighted magnetic resonance ( MR) scan of the head demonstrated an isointense lobular lesion in the left occipital area { arrow). B: On axial T2- weighted MR scan of the head, the lesion had heterogenous isointense and hyperintense signal characteristics { arrow). C: Coronal T1- weighted MR scan of the head showed homogenous enhancement of the lesion after administration of gadolinium- DTPA. The tentorium adjacent to the lesion was slightly thickened and showed mild homogenous en­hancement after administration of gadolinium- DTPA ( arrow). Computed tomography scans of extracerebral CH usu­ally demonstrate isodense or hyperdense lesions with ho­mogenous contrast enhancement ( 16). The CT findings may be variable, however. Moritake et al. demonstrated an unusual pattern of annular enhancement surrounding a high- density mass with calcification in a neonatal tento­rial CH ( 12). The CT pattern of dural CH may be indis­tinguishable from that of meningioma. Unlike meningi- .1 Neiiw- Ophllmlmol. Vol. IX. No. 4. 1998 HEMIANOPSIA DUE TO DURAL CAVERNOUS HEMANGIOMA 253 FIG. 3. Pathologic examination revealed multiple, thick- walled vascular channels with a thick endothelial lining. There were a few foci of lymphocytic infiltrates, old hemorrhage, hemosiderin, and extramedullar hematopoiesis. oma, however, the lesion does not typically show calci­fication, and the surrounding bone may demonstrate erosion or remodeling rather than hyperostosis ( 5,7). Calcification and bone changes were not present in our patient, but our patient did not undergo a CT scan. The MR signal characteristics of extra- axial dural CH differ substantially from the typical MR features of intra-axial CH. The MR features of intra- axial CH include 1) isointense or hypointense signal on Tl- weighted images; 2) isointense or hyperintense signal on T2- weighted im­ages; 3) a prominent hypointense rim on Tl- and T2- weighted images that may represent hemosiderin- laden macrophages; and 4) nonhomogenous enhancement after gadolinium- DTPA administration. Extra- axial CH, on the other hand, often are homogeneously isointense or hyperintense on T2- weighted images and enhance mark­edly and homogeneously after gadolinium- DTPA ( 5,7). This MR appearance may be identical to that of menin­giomas. Quattrocchi et al. reported the MR findings of a tentorial CH ( 16). The lesion was well circumscribed and had low signal intensity on Tl- weighted images, a high signal intensity on T2- weighted images, and homog­enous enhancement with gadolinium- DTPA. Similar MR signal characteristics have been demonstrated for CH in other extra- axial locations ( e. g., cavernous sinus) ( 5,7,8). In our patient, the preoperative diagnosis based on the MR features was meningioma. Cerebral angiography may or may not be useful in distinguishing CH from meningioma. Cerebral angiog­raphy in tentorial CH may demonstrate no abnormalities, an avascular mass, or a fine arterial, capillary, and ve­nous tumor blush ( 5,7,8). In our patient, the angiographic findings were thought to be consistent with a meningio­ma. In most cases, the diagnosis of CH cannot be made until the time of surgery. The correct diagnosis is impor­tant because of the higher risk of morbidity and mortality after removal of these vascular lesions ( 3,13). Although surgical therapy has been the mainstay of therapy for CH, surgical resection of vascular, extra- axial, dural CH may be difficult because of intraoperative bleeding. Namba et al. reported a 38% mortality rate with only three complete resections for extra- axial CH ( 13). Nev­ertheless, complete excisions of dural CH have been re­ported ( 15). Our patient underwent successful resection without intraoperative or postoperative complications and with complete resolution of the preoperative hom­onymous hemianopic visual field loss. Significant blood loss was not encountered in our patient, and no blood transfusion was required. Preoperative endovascular em­bolization or radiation therapy have been suggested by some authors, but remain controversial ( 3,5,7,8). Our pa­tient did not undergo embolization or radiation therapy. Unfortunately, the terminology regarding vascular le­sions of the central nervous system ( CNS) in the litera­ture is confusing because of inconsistent and imprecise usage. The current case draws particular attention to the terms " angioma" and " hemangioma." Intraparenchy-mal CNS vascular malformations composed of vascular channels that are devoid of intervening neuroglial tissue are correctly termed " cavernous angiomas." Central nervous system cavernous angiomas are usually low-flow lesions that may be safely removed surgically. These lesions also lack intervening mesenchymal tissue, in contrast to the benign vascular tumor known as CH. Cavernous hemangiomas consist of capacious vascular channels separated by variable amounts of fibrous or myxoid connective tissue. The CH bears a close relation­ship in age and anatomic distribution to the more com­mon capillary hemangioma. Cavernous hemangiomas may include superficially located components of capil­lary hemangioma, but unlike the pure capillary heman­gioma, which tends to regress by sclerosis with age, CH may spontaneously slowly enlarge and may exhibit ac­celerated growth during puberty or pregnancy. Surgical excision of CH may result in catastrophic hemorrhage. The cavernous angioma and CH also possess distinguish­ing genetic and syndromic associations. The familial form of cavernous angioma in Hispanic Americans has been mapped to a locus on chromosome 7 and traced to a common Mexican ancestor. Cavernous hemangiomas, on the other hand, may be associated with the Maffucci syndrome, blue rubber bleb nevus syndrome, or Kasa-bach- Merritt syndrome ( 21- 23). The lesion that we describe in this report is a CH of the leptomeninges. Meyer et al. believed that the term " cav­ernous hemangioma" was a misnomer for extra- axial CH and suggested that the term " sinus cavernoma" was more appropriate ( 11). We believe that these new terms do not contribute to diagnostic clarity. To our knowledge, this is the first case of extra- axial tentorial CH involving the occipital lobe and presenting as a homonymous hemianopsia. Cavernous hemangio­mas should be considered in the differential diagnosis of dural- based mass lesions. The clinical and radiographic features of dural CH may mimic meningioma, and accu­rate diagnosis is crucial to avoid unnecessary surgical morbidity and mortality. Extra- axial CH are clinically and radiographically distinct from intra- axial CH, and may represent a distinct clinical entity. J Neiiro- Oplillidliiiol, Vol. IK. No. 4. I99S 254 A. G. LEE ET AL, Acknowledgment: This work was supported in part by a grant from Research to Prevent Blindness, Inc., New York, New York. References 1. 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