Ocular manifestations of sphenoid mucoceles: clinical features and neurosurgical management of three cases and review of the literature.

BACKGROUND: Sphenoid mucoceles (SMCs) are unusual lesions, with about 130 cases reported in the literature. Although benign, they may involve the orbit and cause acute restrictive ophthalmoplegia, proptosis, and reduced visual acuity. METHODS AND RESULTS: We present three cases (1 male, 2 females, aged 35, 36, and 46 years) of orbital involvement with acute decreased visual acuity by SMC. The lesions were promptly excised via a transnasal approach to decompress the optic nerve. After the decompression, the patients recovered completely. CONCLUSION: We believe that immediate surgical drainage of the SMC and prolonged antibiotic therapy are necessary and resulted in recovery of visual acuity. Prompt microsurgical transsphenoidal decompression is simple, effective, safe, and is necessary for avoiding persistent visual loss. Antibiotic therapy alone usually results in a poor outcome. We discuss the etiology, clinical manifestations, and management of this rare condition in the light of 130 other cases in the literature.

Gliomatosis cerebri: intra vitam stereotactic determination in two cases and review of the literature.

Gliomatosis cerebri (GC) is an infiltrative neoplastic process that is diffusely distributed through neural structures, whose anatomical configuration remains intact. Most of the cases with GC reported in the literature were diagnosed at autopsy (101/110, 92%). We report two cases of GC in a 42-year-old woman and a 65-year-old man. The diagnosis was confirmed neuropathologically after MRI-guided stereoetactic biopsy. External radiation therapy was performed and led to a complete recovery of all clinical signs. Follow-up examinations 17 and 21 months after diagnosis showed permanent reversal of all clinical and radiological signs. MRI should be used as a primary imaging study in the evaluation of GC and for the determination of the target points for stereotactic biopsy. We discuss the aetiology, clinical manifestation, difficulties of intra vitam diagnosis, and management of this rare condition in the light of other 110 (101 postmortem and nine intra vitam) reported cases of GC in the literature.

Posterior cervical foraminotomy. A follow-up study of 67 surgically treated patients with compressive radiculopathy.

A retrospective study was conducted on 67 patients undergoing posterior cervical foraminotomy (PCF) for unilateral intraforaminal soft and hard disc disease. Neurologic impairment, employment, and severity of associated signs were assessed preoperatively and at a 3.1-year average follow-up (range, 1.5-7 years). Diminution or complete disappearance of radicular symptoms was observed in 62 cases (93%), 3 months to 3 years after surgery. Minimal neurologic deficits persisted in 5 cases (7%). Neck pain improved in 62 cases, remained unchanged in three, and progressed in two cases with severe preoperative deficits. Fifty-three patients (79%) returned to their previous occupation; only seven (10%) retired prematurely on the basis of disc disease alone. Based on Prolo's functional economic outcome rating scale, 60 patients (90%) showed excellent economic outcome. Posterior cervical foraminotomy is an efficient means of decompressing lateral spinal roots compromised by soft disk herniations or osteophytic spurs, without the risk of an anterior approach with or without fusion. Careful patient selection and microsurgical technique are essential in obtaining consistent, excellent results. Additionally, the recent trend toward minimally invasive techniques and key-hole operations in neurosurgery and other specialisations favours the posterior approach.

Orbital and cerebral cavernomas: comparison of clinical, neuroimaging, and neuropathological features.

Our results with 15 orbital cavernomas showed that there are important differences in comparison with cerebral cavernomas: in contrast all orbital cavernomas were embedded by a lilac hard and compact capsule. Clinical symptoms were characterized by the growth of the orbital cavernomas. There were no signs of hemorrhage, which is typical for cerebral cavernomas. The latter showed in contrast to orbital cavernomas a degenerated collagenous tissue forming the vessel walls. The capsule of the orbital cavernomas can be proved by magnetic resonance imaging (MRI). Because of its tendency to lead to irreversible loss of visual acuity, we recommend early surgery after the onset of symptoms.

Microsurgical treatment of intramedullary spinal cord tumors.

Eighty patients with intramedullary spinal cord tumors were treated by microsurgical methods between 1988 and 1996. Twenty-nine patients were diagnosed with astrocytoma, 36 with ependymoma, five with metastasis, four with lipoma, three with dermoid or epidermoid tumor, two with neurofibroma, and one with neuroma. Vascular and infectious lesions (such as abscesses and hemangioblastomas) were excluded. After laminectomy, total removal of the lesion was achieved in 68 of 80 patients and subtotal removal in 12. Postoperative radiation therapy was performed in 13 of 80 patients. The follow-up period ranged from 12 to 92 months (mean 42.2 months). All patients underwent preoperative and postoperative magnetic resonance imaging at intervals ranging from 3 months to 5 years postoperatively. Four patients showed clinical and radiological evidence of local tumor recurrence during the follow-up period. Four patients died 5 months to 15 months postoperatively from the re-expansion of their primary metastatic disease. The operative results at long term (after the 6th postoperative month) were better than the results at short term (before the 6th postoperative month) and revealed clinical improvement in 63, no change in 10, and deterioration in seven patients. We recommend early radical surgery, whenever possible, to be performed when the patient's neurological status is still good. Subtotal removal and irradiation are better for malignant or metastatic tumors. Partial decompressive removal is best for large intramedullary lipomas. Plastic laminotomy with preservation of the intervertebral joints is especially recommended in young or middle-aged patients.

Complications of angioma surgery--personal experience in 191 patients with cerebral angiomas.

In the last years, treatment decisions of arteriovenous malformations (AVMs) were influenced by the improvement of stereotactic radiosurgery and were revolutionized by development of embolization techniques. The aim of this report was to examine the results, effectiveness, and complications associated with angioma surgery. 191 patients with AVMs were operated by the first author between 1981 and 1996. Angioma localization was distributed as follows: frontal 51 (26.7%), temporal 44 (23%), parietal 45 (23.6%), and occipital 24 (12.6%). Twelve (6.3%) AVMs were located in the cerebellum and 15 (7.9%) in other deep regions. Twenty-nine (15.2%) AVMs were associated with single or multiple aneurysms. The preoperative symptoms were hemorrhage (50.3%), seizure (33.5%), headache (23.0%), focal neurological deficits (12.6%), and other minor symptoms. In 9.9%, the disease remains preoperatively asymptomatic. Based on the Spetzler/Martin scale (S/M), 38 patients were grade I, 39 grade II, 52 grade III, 39 grade IV, and 23 grade V. The following severe complications were observed: postoperative hemorrhage in 13 (6.8%), infection in six (3.1%), infarction in two (1.0%), and death in three (1.6%). The risk for postoperative complications was related to the preoperative S/M grade of the AVM. Severe complications only occurred in AVM grades IV and V. In 62 patients with grade IV and V AVM, three patients died (4.8%) and 12 showed neurological deterioration (19.4%). Only 3/129 (2.3%) patients with grade I-III AVM deteriorated postoperatively. No severe complications were observed in preembolized and recently operated patients. Microsurgical management of cerebral AVMs seems to be a reasonably safe procedure especially in grade I-III AVMs, with a mortality of less than 2%. With enough experience and exact attention to detail, the experienced neurosurgeon can remove many of these AVMs with a minimum of risk to the affected patient. Although hemorrhage from an AVM can be disabling or deadly, the course in many nonoperated high-grade AVMs (S/M grades IV and V) can be quite benign, if compared with their surgical risk. This may justify conservative treatment or treatment with radiosurgery in some high-grade (S/M grades IV and V) angiomas, especially in elderly patients.

The transconjunctival microsurgical approach to the orbit: recent experience in 22 cases.

We report a 5-year follow-up of 22 consecutive patients, who underwent transconjunctival management of their orbital lesions without muscle dissection. The presentation, natural history, management, surgical appraisal of the transconjunctival approach and its indications are discussed. At follow-up, 21 of the patients showed excellent cosmetic and functional results. Our experience suggests that this approach remains a useful modality, with good intraoperative visibility and minimal postoperative scar formation, for managing selected patients with space-occupying lesions located in the inferior medial and basal compartment of the orbit. Not suitable for the transconjunctival approach are deep intraconal lesions (in the orbital apex) and extraconal superior lesions. Rare complications of transconjunctival approach have included temporary eye muscle injury with ophthalmoplegia postoperatively. More recently, the use of the transconjunctival approach has allowed surgeons to reduce cosmetic failures, functional deficits and deformities of the orbit. Because of its low risks, the absence of postoperative bleeding, and the limited hospitalization and immobilization of the patients, the transconjunctival approach is a successful procedure especially in elderly patients with intercurrent disease.

Multiple intracranial tuberculomas with atypical response to tuberculostatic chemotherapy: literature review and a case report.

Approximately 34 cases of intracranial tuberculomas with paradoxical response to antituberculous chemotherapy have been documented worldwide. In most of the previously reported cases an associated tuberculous meningitis was reported. The majority of these patients were children or young adults, who had inoperable intracranial tuberculomas located in high risk regions that developed a few weeks or months after the start of an appropriate chemotherapy. Fifty-three percent of the patients recovered completely, 37% improved with mild neurological defects and 10% died. It is interesting that these intracranial tuberculomas developed or enlarged at a stage when systemic tuberculosis was being treated successfully. A recent experience with these potentially curable tumors of the central nervous system is reported. The literature is reviewed, and diagnostic and therapeutic considerations are discussed. The possible immunological mechanisms of this phenomenon are analyzed. In conclusion, patients who are suspected to have a CNS-tuberculosis should receive a prolonged (12-30 months) course of effective antituberculous therapy. The evidence of new intracranial tuberculomas or the expansion of older existing lesions does not indicate the need to change the antituberculous drug program. In such cases systemic dexamethasone as adjuvant therapy for 4 to 8 weeks is worthwhile and effective. Surgical intervention may be necessary in situations with acute complications of CNS tuberculosis, such as shunting procedures for the treatment of hydrocephalus. When the diagnosis is not ensured and there is no response to therapy within 8 weeks, a stereotactic biopsy on a suspected tuberculoma could be performed. If the largest lesion is not located in high risk deep regions of the brain, it could be totally removed surgically. With this combined management, a satisfactory outcome can be obtained in the majority of cases.

Multiple intracranial tuberculomas with atypical response to tuberculostatic chemotherapy: literature review and a case report.

Approximately 34 cases of intracranial tuberculomas with paradoxical response to antituberculous chemotherapy have been documented worldwide. In most of the previously reported cases of this entity an associated tuberculous meningitis has been reported. The majority of these patients were children or young adults, who had inoperably located intracranial tuberculomas in high risk regions developing a few weeks or months after the start of appropriate chemotherapy. 53% of them recovered completely, 37% improved with mild neurological deficits and 10% died. It is interesting that these intracranial tuberculomas developed or enlarged at a stage when systemic tuberculosis was being treated successfully. We report our recent experience with these potentially curable tumours of the central nervous system. The literature is reviewed and diagnostic and therapeutic considerations are discussed. The possible immunological mechanisms of this phenomenon are analysed. In conclusion, patients, who are suspected to be suffering from CNS-tuberculosis should receive a prolonged (12-30 months) course of effective antituberculous therapy. Evidence of new intracranial tuberculomas or the expansion of older existing lesions require no change in the antituberculous drug programme. In such cases systemic dexamethasone as adjuvant therapy for 4 to 8 weeks is worthwhile and effective. Surgical intervention may be necessary in situations with acute complications of CNS tuberculosis such as shunting procedures for the treatment of hydrocephalus. When the diagnosis is not firm and there is no response to therapy within 8 weeks, a stereotactic biopsy of a suspected tuberculoma should be performed. If the largest lesion is not located in high risk deep regions of the brain, it should be total removed surgically. With this combined management, a satisfactory outcome can be obtained in the majority of cases.

[Microsurgical transconjunctival approach to the orbits. Initial retrospective analysis of experiences with the transconjunctival approach in 15 patients. Clinical and surgical aspects]. / Mikrochirurgischer transkonjunktivaler Zugang zur Orbita. Erste retrospektive Erfahrungsanalyse mit dem transkonjunktivalen Zugang bei 15 Patienten. Klinische und chirurgische Aspekte.

The authors report 4-year follow-up results for their first 15 consecutive tumor patients, who underwent transconjunctival surgery of their orbital lesions without muscle dissection. Presentation, natural history, management, surgical appraisal of the transconjunctival approach and its indications are discussed. At follow-up, 14 of the patients showed excellent cosmetic and functional results. Our experience with the transconjunctival approach in the past 4 years suggests that this approach remains a useful modality, with good intraoperative visibility and minimal postoperative scar formation, for managing selected patients with space-occupying lesions located in the inferior medial and basal compartment of the orbit. Not suitable for the transconjunctival approach are deep intraconal lesions (orbital apex) and extraconal superior lesions. The rare problems with the transconjunctival approach have included temporary eye muscle injury with ophthalmoplegia postoperatively. More recently, the use of the transconjunctival approach has allowed surgeons to reduce cosmetic failures, functional deficits and deformities of the orbit. Because of its low risks, the absence of postoperative bleeding, and the absence of prolongation of hospitalization or immobilization of the patients, the transconjunctival approach is a successful procedure, especially in elderly multimorbid patients.

Torsion of an accessory lobe of the liver in an infant.

This report concerns a 6-month-old boy who had omphalocele repair as a newborn and then had torsion of an accessory hepatic lobe, resulting in necrosis. A successful outcome was achieved by resecting the torsed accessory lobe.

Umbilical polyp: a report of two cases.

Two cases with umbilical polyp are reported. This rare malformation arises from remnants of the omphalomesenteric duct and may be clinically mistaken for a granuloma pyogenicum or other benign or malignant umbilical neoplasms. Histologically umbilical polyp shows branching glandular structures lined by intestinal mucosa in connection with the surface of the skin. When the umbilical polyp is not associated with any other types of underlying abnormalities, it may be treated by a simple surgical excision.