and publication type were imposed on the search pro-cess. The ﬁrst relevant article was published in 1978 byFermaglich . 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 , no remarkson therapeutic strategy could be found in the other case. 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 . In 10% (4 patients), symptoms remainedunchanged. Only one patient experienced deteriorationafter total excision of the malformation . 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).
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