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Cavernous Hemangioma Optic

Cavernous Hemangioma Optic

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Published by bodeadumitru9261
We include a further patient with a cavernous haemangioma of the
optic chiasma and left optic tract who presented with an acute defect of
the right visual field and severe retro-orbital pain
We include a further patient with a cavernous haemangioma of the
optic chiasma and left optic tract who presented with an acute defect of
the right visual field and severe retro-orbital pain

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Published by: bodeadumitru9261 on May 14, 2010
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Acta Neurochir (Wien) (2006) 148: 571–578DOI 10.1007/s00701-006-0751-3
Short Illustrated Review
Cavernous haemangiomas of the anterior visual pathways.Short review on occasion of an exceptional case
M. Lehner
, F. A. Fellner
G. Wurm
Department of Neurosurgery, Landes-Nervenklinik Wagner-Jauregg, Linz, Austria
Institute of Radiology, Landes-Nervenklinik Wagner-Jauregg, Linz, Austria
Institute of Diagnostic Radiology, Friedrich-Alexander-University Erlangen-N
uurnberg, Erlangen-N
uurnberg, GermanyReceived March 21, 2005; accepted January 5, 2006; published online March 2, 2006
Springer-Verlag 2006
The anterior optic pathways are rarely affected by vascular malforma-tions. In a meticulous literature review, 42 published cases of patientswith vascular malformations within optic nerves, chiasma and
or optictract were found, 30 of them being diagnosed as cavernous haemangio-mas. All of them suffered from visual disturbances; in 38.1% previoussymptoms had occurred. Surgical treatment resulted in major improve-ment in most patients.We include a further patient with a cavernous haemangioma of theoptic chiasma and left optic tract who presented with an acute defect of the right visual field and severe retro-orbital pain. We succeeded in totalexcision of the malformation via a neuronavigationally guided approach.In the postoperative course, vision of our patient improved immediatelyand was found to be completely normal three months after the surgicalintervention. Considering our patient and the published cases in theliterature, we are of the opinion that microsurgical excision is a safeand efficient treatment for these rare pathologies.
Cavernous haemangioma; anterior optic pathways;cranial nerves; microsurgical excision.
Cavernous haemangiomas are angiographically occultvascular malformations which may occur throughout thebody and neuroaxis, and affect 0.3–0.7% of the popula-tion [6, 33]. The most common location of cavernoushaemangiomas of the central nervous system is thesupratentorial subcortical white matter. They may alsooccur in the cerebellum, the brainstem, the spinal cord,or the spinal nerves [33]. The percentage of distributionreflects the volumes of the distinct compartments of thecentral nervous system [6]. Thus, cavernomas of cranialnerves are a rare entity; in extremely rare cases theyhave been reported to affect the anterior optic pathways[1, 3–7, 9, 10, 12, 14–17, 20, 22–25, 28–31]. Even morerarely, other vascular malformations like venous angio-mas [2, 8, 11, 13] and arteriovenous malformations[11, 17, 18] have been found at this location. Vascularmalformations within the anterior visual pathways cancause visual symptoms as well as headache, retro-orbitalpain and nausea. Clinical symptoms are mostly due tointrinsic or extrinsic bleeding.We undertook a thorough review of the literature onthe topic of cavernous haemangiomas and other vascularmalformations affecting the anterior optic pathways,where we focused on clinical presentation, imagingmodalities, localisation, histopathology and therapeuticstrategies (Table 1). The review was initiated by apatient, who presented with acute visual dysfunctionand retro-orbital pain caused by bleeding of a cavernoushaemangioma located in the chiasm and in the left optictract.
Literature review
The pubmed search engine of the national libraryof medicine and national institutes of health (www.pubmed.gov) was used to perform an internet medlineliterature research based on the search items ‘‘chiasma’’,‘cavernoma’, ‘vascular malformation’and ‘cranialnerves’. No limitations for the timeframe, language
and publication type were imposed on the search pro-cess. The first relevant article was published in 1978 byFermaglich [8]. Full papers of all hits, dealing withthe correct localisation and pathology, were ordered.Articles were available in the English, French and Italianlanguages. The references of the full articles were com-pared with the positive responses of our search in orderto complete the literature table. We found 28 relevantcase reports and short reports dealing with the topic of vascular malformations of the anterior visual pathways[1–20, 22–25, 28–31]. Double publications were ex-cluded. Most authors reported on a single case [2–5,7–10, 13–16, 18, 20, 22–25, 30–31], eight reported on2 to 4 cases [1, 6, 11–12, 17, 19, 28–29]; five publi-cations enclosed a literature search and review on‘chiasmal apoplexy’’, ‘cryptic chiasmal malforma-tions’’, or ‘‘cavernous haemangiomas of cranial nerves’’[6, 17, 18, 28, 31].In total, 42 cases of vascular malformations involv-ing the anterior optic pathways were found, 30 of thembeing histologically diagnosed as cavernous haeman-giomas (Table 1). Published data were reviewed accord-ing to age, sex, symptoms, acuteness, lesion localisation,imaging, therapeutic strategies, histopathology, andoutcome.
Analysis of literature review
The mean age at clinical presentation of the wholepatient series was 32.4 years with an age range of 4 to63 years. The ratio between males and females was 14 to25; in three cases there was no comment on sex and age.The most common clinical symptoms and signs of the patients reviewed were visual disturbances (100%,42 patients), headache and
or retro-orbital pain (69%,29 patients). Sudden onset of complaints (59.5%, 25patients) was observed more frequently than subacute,chronic or slow progressive development of symptoms.In 16 of 42 reviewed patients (38.1%), previous episodesof typical symptoms had occurred before a correct diag-nosis was made. The optic chiasma was involved in38 patients (90.5%), whereas sole location in the opticnerve or in the optic tract was found in only onecase, respectively. Bleeding was detected in 78.6%(33 patients) (Table 1).Surgical treatment was performed in 40 of 42 reportedpatients (95.2%), clinical observance without surgicalintervention was chosen in one case [1], no remarkson therapeutic strategy could be found in the other case[4]. The surgical techniques used varied from total exci-sion in 22 patients (55%), partial resection in two cases(5%), decompression with biopsy in 13 patients (32.5%),simple biopsy in two cases (5%), biopsy and additionalradiotherapy in one case (2.5%).80% (32 patients) of the surgically treated patientsexperienced improvement of symptoms, including onepatient with total recovery after decompressive resec-tion [19]. In 10% (4 patients), symptoms remainedunchanged. Only one patient experienced deteriorationafter total excision of the malformation [12]. There wereno remarks on outcome in three patients (Table 1).Histological examination of the specimen of patientsreviewed proved the lesions to be cavernous haemangio-mas in 30 patients (71.5%), venous angiomas in fourpatients (9.5%) [2, 8, 10, 12] and arteriovenous malfor-mations in four patients (9.5%) [10, 16, 17]. In fourcases, no information about histopathology was avail-able [1, 19] (Table 1).
Illustrative case
A 39-year-old female patient presented with acutedefects of the right visual field and severe retro-orbitalpain. The patient had a long history of headache; onclinical examination no other neurological deficit couldbe found. Computed perimetry showed partial hemia-nopsia of the right visual field. Preoperative visuallyevoked potentials demonstrated loss of usual configura-tion of the potential for the stimulated left eye withdeformed and delayed triple peak of diminished ampli-tude. No major abnormality was found for the stimulatedright eye.The CT-scan showed multiple hyperdense struc-tures without contrast enhancement in the left frontallobe, in the pons and within the optic chiasm. Pre-and postoperative MR examinations were done using a1.5 T whole body scanner with a standard head coil(Magnetom Symphony, Siemens). MR protocol includedT2-weighted turbo spin-echo (TSE), T2
-weightedgradient-echo (GRE), T1-weighted conventional spin-echo sequences before and after i.v. application of gadolinium (Omniscan, Amersham Health), dosage0.1mmol
kg body weight, in the transverse orientation(slice thickness 6mm, gap 1.2mm) as well as a contrastenhanced T1-weighted 3D MP-RAGE data set with aneffective slice thickness of 1.0mm. MRI demonstratedthe supra- and infratentorial lesions to be cavernoushaemangiomas, suggesting a familial pattern. The lesioncorresponding to the neurological deficit was the one inthe chiasm reaching into the left optic tract which
Cavernous haemangiomas of the anterior visual pathways

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