Intraorbital ganglioglioma of optic nerve in a patient with neurofibromatosis type 1.

We report the case of an orbital optic nerve gangliogoma in a 55-year-old woman with neurofibromatosis type 1 (NF1). Clinical course neuroimaging findings, pathology, and treatment options of gangliogloma are discussed and contrasted with pilocytic astrocytomas of the optic nerve, a much more frequent visual pathway neoplasm in NF1 patients.

Tolosa-Hunt syndrome--diagnostic problem of painful ophthalmoplegia.

BACKGROUND: Tolosa-Hunt syndrome (THS) is an uncommon disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. The disease is characterized by periorbital pain, paresis of the bulbomotor and quick response to steroid treatment. The orbital process may lead to optic nerve atrophy. According to the International Headache Society Classification of 2004, the diagnostic protocol includes magnetic resonance imaging (MRI) and biopsy. CASE REPORTS: We presented 46-year old male patient, with THS. The patient had unilateral periorbital pain, inflammatory process in the cavernous sinus, the apex of the orbit and the paranasal sinuses. Inflammatory process had spread into the fascia of the bulbomotor and performed compression to the optic nerve, causing paresis of the bulbomotor, protrusion of the eyeball and atrophy of the optic nerve. Pulse doses of corticosteroids were effective. Regarding the presented patient, diagnostic dilemmas arose from nonspecific sinusitis. The initial ophthalmological diagnosis, based on periorbital pain, drop in visual acuity and the narrow chamber angle was angular glaucoma, which resulted in a delayed diagnosis of THS and the beginning of the treatment. MRI and positive response to the treatment with corticosteroids were relevant for making the diagnosis. CONCLUSION: According to the International Headache Society Classification of 2004, THS is an entity that occurs rarely, its etiopathogenesis is unknown, it is manifested clinically by unilateral orbital pain associated with simple or multiple oculomotor paralyses, which resolves spontaneously but may recur. MRI orbital phlebography and biopsy are the recommended methods for making diagniosis. In our patient MRI findings and positive response to the corticosteroide treatment were relevant for making the diagnosis.

[Primitive trigeminal artery associated (corrected) with arteriovenous malformation of cerebellum].

INTRODUCTION: Persistence of fetal cerebral carotid basilar anastomoses is rare. When it occurs, it is often associated with other vascular malformations, and other pathological conditions. Trigeminal primitive artery persistence is the most often among them. Coincidence of primitive trigeminal artery with cerebellar arteriovenous malformation is extremely rare. CASE REPORT: We reported a case of a 31-year-old woman with subarachnoid hemorrhage and cerebellar hematoma admitted in serious condition. Angiography demonstrated cerebellar arteriovenous malformation, primitive trigeminal artery and other malformation of Willis circle. After a complete surgical removal of arteriovenous malformation the patient was discharged without neurological or any other deficit. CONCLUSION: We reported an extremely rare condition, which had been reported very few times in the literature. The importance of primitive artery persistence is in changed anatomical and physiological condition of cerebral circulation, that is especially important in surgical procedures.

Transvenous embolization of dural carotid cavernous fistula through the facial and ophthalmic vein.

INTRODUCTION: Dural carotid cavernous fistula is acquired, relatively rare, condition comprising of numerous small-caliber meningeal arterial branches, draining directly into cavernous sinus. Endovascular therapy is the treatment of choice, preferably by a transvenous approach. In the case of inaccessible inferior petrosal sinus, other alternative routes are considered. We presented a case of dural carotid cavernous fistula completely occluded with Guglielmi detachable coils, using a transvenous approach through facial and superior ophthalmic vein. CASE REPORT: A 62-year-old man was referred with a gradual worsening proptosis, red eye, and decreased visual acuity, on the right side. Digital subtraction an giography revealed the presence of a right dural carotid cavernous fistula, predominantly supplied from dural branches of the right internal carotid artery siphon, with minimal contribution from the right middle meningeal artery and contra lateral dural branches of the left internal carotid artery siphon. The fistula was drainaged through the dilated superior ophthalmic vein, and via the facial to the internal jugular vein. There was neither pacification of pterygoid and petrous sinuses, nor cortical venous reflux. Endovascular treatment was performed by a transvenous approach. A guiding catheter was placed in the right facial vein. A microcatheter was advanced through the dilated angular and superior ophthalmic vein, and its tip positioned into the right cavernous sinus. Coils were deployed, until a complete angiographic occlusion of the fistula had been achieved. The patient experienced rapid improvement in the symptoms, with complete normalization of his condition one month after the treatment. CONCLUSION: Coil embolization of dural carotid cavernous fistula by transvenous catheterization, through the facial and superior ophthalmic vein, can be considered as safe and effective treatment option in the presence of marked anterior drainage.

Multiple cavernous hemangiomas of the orbit: separate occurrence within a 24-year period.

BACKGROUND: Cavernous hemangioma is a frequent and the most common, primary, benign tumor of the orbit in adults. It is typically single and unilateral, considered not to recur after having been completely excised. Multiple orbital cavernous hemangiomas without signs of hemangiomatosis are rare. Multiple cavernous hemangiomas may recur after a complete excision and may exist with concurrent systemic tumors. Tumor recurrence is supposed to develop from vasculature that is present already in response to a proliferate stimulus. CASE REPORT: A 39-year-old female with painless proptosis of the right orbit was found to have four orbital tumors. The first orbitotomy was performed in 1984 by excising four cavernous hemangiomas. Six years later, another, the fifth one cavernous hemangioma was totally excised from the same orbit. Nine years after the first operation, reorbitotomy was performed because of positive radiological and clinical signs of de novo tumor in the orbit. The operation did not confirm the tumorous tissue. The fourth orbitotomy was performed 24 years after the first operation and two cavernous hemangiomas were totally excised. CONCLUSION: This case show the possibility of cavernous hemangioma recurrence after a previously totally excised tumor, separated more than two decades. A very long follow-up of the patients operated for these benign tumor lesions is recommended.

[Vasculature of the distal part of optic nerve].

BACKGROUND/AIM: Vascularisation of the distal, namely intracranial and intracanalicular parts of the optic nerve have not been explained in conventional textbooks of anatomy, while there have been explanations of proximal, that is intraorbital segment. The aim of this research was to study the pattern of arterial supply of the intracranial and intracanalicular part (the distal part) of human optic nerve. METHODS: The optic nerve and the ophthalmic artery (OA), predominately in their intracranial and intracanalicular parts, were investigated in 25 human specimens by three different methods: macroscopic, stereomicroscopic, and histological observations. Mixture with 10% of India ink and gelatin was injected through the intracranial part of the internal carotid artery, and the most proximal part of the OA. Each optic canal specimen was fixed in formaldehyde and finally paraffin embedded, sectioned, and stained with Masson trichrome, Azan, Toluidin blue, and Van Gieson methods. RESULTS: OA passed through the optic canal within the dural sheath of the optic nerve. In 44% of our specimens the OA was on the inferomedial side of the optic nerve at the entrance point to the optic canal. OA left the optic canal at its lateral border in the apex of the orbit in 72% of our specimens. The intracanalicular portion of the optic nerve receives arterial blood principally from the intracanalicular part of OA. OA gives one (72% of the specimens) to two branches that supply the intracanalicular part of the optic nerve. Each branch pierces the dura mater from below and then supplies the nerve through the pia mater. These arteries then terminate in a pial vascular network of continuous transverse centripetal arterioles and capillaries that surround each optic nerve. The rich anastomoses with branches of superior hypophyseal artery, from the cranial cavity, which take part in the optic nerve vascularization in its hole length, was observed. There were no intraaxial vessels in the intracranial and intracanalicular parts of the nerve in our specimens. CONCLUSION: These anatomical data offer important informations for understanding the variety of the pathology in the region of optic canal and orbito-cranial junction, and is also useful for designing operative strategies. This report indicates the delicacy and vulnerability of the intracranial and intracanalicular capillary network to traumatic disruption.

A retrospective analysis of anesthesiologic complications in pediatric neurosurgery.

BACKGROUND AND OBJECTIVE: Different clinical and surgical factors can influence the occurrence of anesthesiologic complications in pediatric neurosurgery. Preoperative knowledge of these factors is of great importance in the application of safe anesthetics and a favorable surgical outcome. The objective was to establish the importance of clinical and surgical risk factors on the frequency of anesthesia complications in pediatric neurosurgery. DATA AND METHOD: The research, from 1996 to 2000, involved 705 children, aged from <1 year to 15 years, who underwent surgery for elective neurosurgical pathology and severe head injuries. We analysed the influence that: age, the preoperative neurologic diagnosis, the urgency of the operation, additional disorders, the surgical position, and the duration of anesthesia had on the frequency of anesthesia complications. To test the statistical relevance and to confirm the hypothesis, the Pearson's chi-square test, Mann-Whitney U-test, and univariate and multivariate logistic regressions were used. RESULTS: Anesthesia complications (cardiovascular, respiratory, air embolism, allergic reactions) were present in 68/705 (9.6%) patients. Their frequency was statistically greater in children for whom the surgery was >240 min, who were in the sitting position and when comorbidity was evident. Neither age nor the urgency of the operation or reoperation had any significant influence on the occurrence of anesthetic complications. CONCLUSION: The duration of anesthesia, the sitting position of the patient, and the presence of comorbidities significantly increase the risk of anesthesia complications in pediatric neurosurgery.

Intrapontine malignant nerve sheath tumor: MRI and MRS features.

The primary source of malignant intracerebral nerve sheath tumors is still unclear We report the imaging and MR spectroscopic findings in a 39-year-old man with a very rare brain stem tumor MR examination revealed the presence of intraaxial brain stem tumor with a partial exophytic growth. On pathological examination, the neoplasm appeared to be an intrapontine nerve sheath tumor originating most likely from the intrapontine segment of one of the cranial nerve fibres. The tumor showed exophytic growth, with consequent spread to adjacent subaracnoid space. MR spectroscopy revealed the presence of very high concentration of choline, associated with no creatine and N-acetyl aspartate resonance, suggesting non-glial origin of the intraaxial neoplastic infiltration. MR spectroscopy seems to be a helpful diagnostic modality not only in the estimation of the grade of astrocytomas that has been already confirmed in literature, but also in the diferentiation between glial and non-glial origin of primary intracranial neoplasms when MR imaging is not conclusive.

[Intracranial menigioma manifested after delivery in a patient with Hodgkin's disease].

BACKGROUND: Intracranial meningioma is rerely reported in the patients treated for Hodgkin's disease (HD), known to mainly occur in the area of radiation therapy. CASE REPORT: A 26-year-old woman with HD, and intracranial meningioma following the delivery, was presented. As we knew, a similar case had not been reported in the literature before. Three years prior the surgery for intracranial tumor, the patient had been started to be treated for HD of neoplasm stage I (NS I) type, by the use of the standard (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone) BEA-COPP protocol. The remission of HD, achieved after 7 cycles, persisted over a 27-month-period up to the delivery. Within this period, the patient was without neurologic disorder, but with the obvious psychotic behavior, for which the patient was treated with haloperidol. Two days following the normal delivery, during the acute disorder of the consciousness, intracranial tumor was found. A complete bilateral meningioma (11.7 x 8.3 x 8.1 cm) of the frontal parietal zone was removed. CONCLUSION: there were no reliable signs of the use of an intensive hemotherapy in the reported case (alkylating cytostatics and topoisomerases inhibitors) which might have caused the proliferation of a benign solid tumor. The pregnancy was supposed to be the possible second risk factor for causing the growth of a meningioma. On the basis of the significant psychic disorders before the pregnancy, as well as upon the size of the operated on tumor, we concluded that the occurrence of intracranial meningioma could be regarded the parallel neoplastic disease or the second primary tumor.

[Significance of the spatial reconstruction based on mathematical modeling in the surgical treatment of giant intracranial aneurysms].

BACKGROUND: The use of computer models for the 3-dimensional reconstruction could be a reliable method to overcome technical imperfections of diagnostic procedures for the microsurgical operation of giant intracranial aneurysms. CASE REPORT: We presented a case of successfully operated 52-year-old woman with giant intracranial aneurysm, in which the computer 3-dimensional reconstruction of blood vessels and the aneurysmal neck had been decisive for making the diagnosis. The model for 3-dimensional reconstruction of blood vessels was based on the two 2-dimensional projections of the conventional angiography. Standard neuroradiologic diagnostic procedures showed a giant aneurysm on the left middle cerebral artery, but the conventional subtraction and CT angiography did not reveal enough information. By the use of a personal computer, we performed a 3-dimensional spatial reconstruction of the left carotid artery to visualize the neck of aneurysm and its supplying blood vessels. CONCLUSION: The 3-dimensional spatial reconstruction of the cerebral vessels of a giant aneurysm based on the conventional angiography could be useful for planning the surgical procedure.

[Intraorbital wooden foreign body, diagnosed by NMR and ultrasonography, and extirpation by cranio-orbital approach]. / Intraorbitno drveno strano telo dijagnostikovano pregledom pomocu NMR i ultrazvuka i ekstirpovano kranio-orbitnim pristupom.

AIM: The case of the phlegmon of the left orbit associated with ophtalmoplegia and blindness in a 16-years old boy was presented. It was caused by the penetrafion of the wooden foreign body under unknown circumstances. CASE REPORT: Repeated CT scans did not reveal the presence of the foreign body before MR imaging and ultrasonography. The removal (43 x 8 mm) was done one month after the injury by osteoplastic orbitotomy, using microsurgical technique. Three months later the signs of inflammation and eye protrusion withdrew. Motility of the left upper lid and eye were normal. CONCLUSION: MR imaging and ultrasonography were decisive in the diagnose of organic foreign body in the orbit.