Y. Arkha

HEARING PRESERVATION AFTER GAMMA KNIFE RADIOSURGERY FOR VESTIBULAR SCHWANNOMAS PRESENTING WITH HIGH-LEVEL HEARING

OBJECTIVEThe aim of this study was to evaluate long-term hearing preservation after gamma knife radiosurgery (GKS) for vestibular schwannomas in patients with initially normal or subnormal hearing (Gardner-Robertson Class 1) and to determine the predictive factors for functional hearing preservation. METHODSSince July 1992, more than 2053 vestibular schwannomas have been treated by GKS and followed at the Timone University Hospital, Marseille. A minimum of 3 years of follow-up (range, 3–11 years; median, 48 months) is available for 74 patients (without neurofibromatosis Type 2 or previous surgery) with Gardner-Robertson Class 1 hearing. RESULTSThe average age of the patients was 47.5 years (range, 17–76 years). The number of tumors in Koos Stage I was 8, the average number in Stage II was 21, the average number in Stage III was 43, and the average number in Stage IV was 2. The median number of isocenters was 8 (range, 2–45), and the median marginal dose was 12 Gy (range, 9–13 Gy). At the time of the last follow-up evaluation, 78.4% of the patients had preserved functional hearing. Tumor control was achieved in 93% of the cases. The probability of preserving functional hearing was higher in patients who had an initial symptom other than hearing decrease (91.1%), in patients younger than 50 years (83.7%), and in those treated with a dose to the cochlea of less than 4 Gy (90.9%). CONCLUSIONThis study shows that the probability of preserving functional hearing in the long term after GKS for patients presenting with unilateral vestibular schwannomas is very high. The positive predictive factors appear to be young age, an initial symptom other than hearing decrease, and a low dose to the cochlea.

REPEAT GAMMA KNIFE SURGERY FOR REGROWTH OF VESTIBULAR SCHWANNOMAS

OBJECTIVEGamma knife surgery (GKS) has become established as a minimally invasive treatment modality for patients with vestibular schwannomas. Treatment failure and/or tumor regrowth, however, is occasionally encountered, and microsurgical resection is usually warranted in such cases. The role of repeat GKS in these situations is still unclear. The goal of this study was to investigate whether repeat GKS is an effective treatment for recurrent vestibular schwannomas and to assess the conservation of residual neurological function. METHODSBetween July 1992 and December 2007, 1951 patients harboring a unilateral vestibular schwannoma were treated with GKS. Of these, 48 patients (2.5%) had to undergo a subsequent intervention because of progression or regrowth of the tumor. Repeat GKS was performed in a total of 15 patients, 8 of whom had more than 2 years of follow-up and were eligible to be enrolled in the present study. The median follow-up period after repeat GKS was 64 months, and the median interval between these interventions was 46 months. The median tumor volume was 0.51 and 1.28 mL at the initial and second GKS treatments, respectively. Patients received a median prescription dose of 12.0 Gy at both interventions. RESULTSWe report no cases of failure. Six patients demonstrated a significant reduction in tumor volume. In 1 patient, the final tumor volume was less than the initial volume. The other 2 patients showed stabilization of tumor growth. Useful hearing ability was preserved in only 1 of the 3 patients who had serviceable hearing ability at the time of the second GKS. Neither aggravation of facial nerve dysfunction nor other neurological deficits secondary to GKS were observed. CONCLUSIONThis is the first report to address repeat GKS for vestibular schwannomas. After long-term follow-up, repeat GKS with a low marginal dose seems to be a safe and effective treatment in selected patients harboring regrowth of small vestibular schwannomas that have previously been treated with GKS.

Management of intracranial arachnoid cysts: institutional experience with initial 32 cases and review of the literature.

Surgical indications and modalities in treatment of intracranial arachnoid cysts still remain controversial owing to limited understanding of the pathophysiologic mechanisms and natural history of this pathology. Current literature favours endoscopic interventions for arachnoid cysts. We retrospectively reviewed 32 intracranial arachnoid cysts managed over 11-year period in our institution. Post-therapeutic results were clinically and radiologically assessed. Supratentorial location of cysts was noted in 75% cases (n=24) while 25% cysts were located infratentorially (n=8). The mean cyst size was 54mm in largest dimension (range 10-100mm; median 50mm). Hemiparesis was noted in 37% cases, raised intracranial pressure and seizures in 34% cases each, while cranial nerve dysfunction was noted in 16% cases. Seventy five percent cases were surgically managed: excision and marsupialization was done in 53% cases (n=17), stereotactic aspiration in 12.5% cases (n=4), endoscopic fenestration in 6.25% cases (n=2) and cystoperitoneal shunting initially in 1 case (3%) and after recurrence of primarily excised cysts in 2 cases. Conservative treatment with regular clinical and imaging control was done in 25% cases (n=8). The mean follow-up was 72 months (range: 12-108 months). Good outcome was noted in 72% cases, 16% cases remained unchanged while only one case with giant suprasellar cyst worsened. With excision and marsupialization, 65% of cysts reduced in size (n=11/17), 17% cysts resolved completely (n=3/17). The overall recurrence rate was 29%. Surgery excision and marsupialization of symptomatic cases provided good results.

Intracranial meningioma in children: Different from adult forms? A series of 21 cases

OBJECTIVE Intracranial meningiomas are very rare in children, comprising only 0.4 to 4.1% of pediatric tumors and only 1.5 to 1.8% to all intracranial meningiomas. The goal of this study of pediatric meningiomas was to establish their epidemiological profile as well as their clinical and radiological features, to assess the long-term outcome, and compare this result with adult meningioma. PATIENT AND METHODS We conducted a retrospective study from June 1983 to June 2007; during this period 521 patients underwent surgery for primary meningioma at the Rabat Hospital, Department of Neurosurgery. Twenty-one patients were under 16 years of age (4%). The clinical charts and imaging data were reviewed. RESULTS The mean age was 10.3 years (range: 2 to 16 years), with 13 boys and eight girls. In one patient a neurofibromatosis was associated. The mean delay to diagnosis was 4.6 months (range: 1 to 12 months). The most common clinical sign was raised intracranial pressure (90%). Of the meningiomas diagnosed, 47% were convexity meningiomas while 24% were parasagittal and 19% were skull-base meningiomas; in two cases (9.5%) the location was intraventricular. The mean tumor diameter was 6.6 cm (range: 3 to 10 cm). A large cystic component was found in 24% of the cases. Surgery achieved a Simpson grade I resection in 47%; 62% of the tumors were grade I and 24% were grade II based on World Health Organization pathological classification. The mean follow-up period was 33 months (range: 6 to 120 months). The recurrence rate was 33%. CONCLUSION Pediatric meningiomas are larger than those found in the adult population; there is a male predominance with high incidence of a cystic component and high-grade meningiomas, thus explaining the increased recurrence rate despite the multimodal treatment.

Sphenoid sinus aspergillosis simulating pituitary tumor in immunocompetent patient

Aspergillosis of the sphenoid sinus is rare in immunocompetent patients. It may be mistaken for a sellar region tumor. A 65-year-old, human immunodeficiency virus-negative man presented with a 3-week history of cranial nerve III paresis and visual deterioration. The patient had a long-term history of tobacco snuff abuse. CT scans and MRI demonstrated a space-occupying lesion of the sellar and sphenoid sinus region. Presumptive diagnosis of pituitary macroadenoma was made and the patient was operated on via a transnasal-transsphenoidal approach. After the sphenoid sinus was opened, a yellow-brownish gluey material with crumbly debris extruded and was aspirated. The dura was intact. Histopathology revealed numerous Aspergillus hyphae without tissue invasion. Postoperatively, the cranial nerve III paresis resolved in a few days and visual acuity improved. Sphenoid sinus aspergillosis should be included in the differential diagnosis of sellar region processes, even in immunocompetent patients. Early diagnosis and transsphenoidal removal provides good results without the need for systemic antifungal therapy in non-invasive aspergillosis.

Orbital hydatid cyst: Review of 10 cases

BACKGROUND Echinococcosis represents one of the most common human parasitoses in some geographical areas. Orbital involvement is extremely rare. MATERIALS AND METHODS In the records of our Medical University Centre between 1984 and 2006, we found 10 cases of orbital hydatid cyst. RESULTS Among them, 6 males and 4 females; the ages ranged from 2 to 60 years and 7 cases (70%) were less than 12 years of age. The main symptoms of orbital hydatid cyst were slowly progressive unilateral proptosis (100%) with visual loss (90%). The presumptive diagnosis was made on the images obtained from computed tomography and/or magnetic resonance imaging. Surgical removal was the main treatment; although, it is frequently complicated with cyst rupture and spillage of the contents. For this reason, we adopted the technique of cyst puncture with irrigation. A postoperative antihelminthic treatment has always been used. CONCLUSION Hydatid cyst is an endemic disease in Morocco. Orbital involvement should be considered in the differential diagnosis of proptosis especially in children known to have been in affected geographical areas.

SPINAL INTRADURAL EXTRAMEDULLARY HYDATIDOSISREPORT OF THREE CASES

OBJECTIVESpinal hydatid cyst is a serious form of hydatid disease affecting fewer than 1% of all patients with hydatid disease. We report 3 healthy patients who presented with progressive paraparesis attributed to a histologically proven intradural hydatid cyst. METHODSThere were 2 children (1 boy, 1 girl) and 1 adult with a mean age of 12 years. The median follow-up duration was 16 months. Spinal magnetic resonance imaging was performed in the 3 patients, and an anatomic and topographical diagnosis of the intradural hydatid cyst was made. RESULTSMagnetic resonance imaging scans revealed cystic lesions with peripheral contrast enhancement. Surgery was performed through laminectomy, complete resection was achieved, and antihelminthic treatment with albendazole 10 mg/kg−1 per day for 6 months was included in the postoperative treatment. The patients improved after surgery with normal motor function. CONCLUSIONThis localization is rare and serious, but its prognosis is excellent if diagnosis is made early enough and surgery is performed in time to prevent cyst rupture.OBJECTIVE: Spinal hydatid cyst is a serious form of hydatid disease affecting fewer than 1% of all patients with hydatid disease. We report 3 healthy patients who presented with progressive paraparesis attributed to a histologically proven intradural hydatid cyst. METHODS: There were 2 children (1 boy, 1 girl) and 1 adult with a mean age of 12 years. The median follow-up duration was 16 months. Spinal magnetic resonance imaging was performed in the 3 patients, and an anatomic and topographical diagnosis of the intradural hydatid cyst was made. RESULTS: Magnetic resonance imaging scans revealed cystic lesions with peripheral contrast enhancement. Surgery was performed through laminectomy, complete resection was achieved, and antihelminthic treatment with albendazole 10 mg/kg-1 per day for 6 months was included in the postoperative treatment. The patients improved after surgery with normal motor function. CONCLUSION: This localization is rare and serious, but its prognosis is excellent if diagnosis is made early enough and surgery is performed in time to prevent cyst rupture.

Role of gamma knife radiosurgery in the management of pituitary adenomas and craniopharyngiomas.

INTRODUCTION Radical microsurgical removal of pituitary adenomas (PAs) and craniopharyngiomas (CPHs) is often difficult. In such cases radiosurgery can be used as a second-line treatment option. MATERIALS AND METHODS Our series included 436 PAs and 164 CPHs. The majority of patients had large or giant tumors and were treated with microsurgery. Additionally, between June 2008 and August 2011, a total of 29 PAs and 10 CPHs underwent radiosurgery using Leksell Gamma Knife PerfeXion. At the time of treatment the volume of the PAs varied from 0.6 to 26.0 cm3 (mean 5.9 cm3) and that of the CPHs from 0.19 to 17.0 cm3 (mean 6.6 cm3). The marginal doses ranged from 12 to 15 Gy (mean 14.5 Gy) for nonsecreting PAs, from 22 to 25 Gy (mean 24 Gy) for hormone-secreting PAs, and from 8 to 14 Gy (mean 11 Gy) for CPHs. RESULTS The postoperative mortality rates after surgical removal of PAs via the transspenoidal approach and craniotomy were 2.4 % and 8.0 %, respectively, whereas after surgery for CPH it was 5.9 %. No major complications were noted in our limited number of patients after radiosurgical treatment. Taking into consideration only cases with radiological follow-up of at least 12 months, shrinkage of the tumor was demonstrated in 5 of 11 patients with a PA and in 4 out of 6 patients with a CPH. CONCLUSION Radiosurgery is safe and effective second-line management option in cases of recurrent or residual PA or CPH. Occasionally, it can be applied even as a primary treatment in selected patients.

L’exentération orbitaire

PURPOSE Orbital exenteration is a disfiguring surgery. The surgery is mostly performed for advanced neoplasms of the eyelid in an attempt to achieve cure with tumor free margins. Reconstruction is a real challenge, especially in elderly patients with significant comorbidities. PATIENTS AND METHODS We operated 15 patients presenting with palpebral and orbital tumors, between January 2000 and December 2007. We collected the clinical data concerning patients, tumor, treatment, and recurrences. RESULTS Ten male and five female patients with a mean age of 56 years at diagnosis presented with ulcerative palpebral malignant tumor, and impaired ocular motility. Basal cell carcinoma was the most common (80%). All patients underwent exenteration, (subtotal three, total eight, and extended four patients). The cavity was filled with a temporal muscle flap in ten cases, Mustardé flap in three cases, latissimus dorsi myocutaneous free flap in one case, and a jugal V-Y flap in one case. The mean follow-up was 23 months with good healing without radiotherapy tissue alteration. Four patients had a recurrence and one patient died from metastases. DISCUSSION The goals of reconstruction are functional and esthetic. Given the initial tumoral extension, we choose to use a regional or microsurgical flap for functional reconstruction. The flap provides a good cutaneous coverage, rapid healing, closure of orbital nasal and sinus communications, or of orbital and cranial communications. It is not damaged by radiotherapy.

Un cas de dysplasie fibreuse kystique craniofaciale

INTRODUCTION Fibrous dysplasia is a benign, idiopathic, fibro-osseous disease. CLINICAL CASE A 17-year-old girl had presented with left proptosis for the previous two years, associated to homolateral hemicranial pain. Clinical examination was normal except for non-reducible axile exophthalmia. Computed tomography and magnetic resonance imaging of the head revealed an extensive cystic tumor of the left fronto-ethmoido-sphenoidal region, with compression of the left frontal lobe and medial orbital wall. The radiological appearance suggested a mucocele but histological examination, after surgery, proved a fibrous dysplasia. DISCUSSION Fibrous dysplasia with fronto-sphenoido-ethmoidal localization may be misdiagnosed as mucocele. Histology proves the diagnosis.