Abstract: The purely endoscopic eyebrow supraorbital approach is an alternative to both the endoscopic endonasal and eyebrow microsurgical routes to the anterior and middle cranial base. It combines an enhanced visualization provided by the endoscope and the absence of cerebrospinal fluid (CSF) leaks and nasal complications.
Abstract: Spinal subdural hematomas (SSDH) are rare lesions occurring in association with a wide variety of conditions, including anticoagulation, coagulation disorders, spinal anesthesia, lumbar puncture, spinal tumors and vascular malformations. SSDH resulting from trauma are the exception. We present a 62-year-old woman with a rare post-traumatic focal SSDH at C1 with bulbomedullary compression, treated successfully with surgery. A review of the literature revealed 26 patients with traumatic SSDH. The aim of this report is to describe the clinical presentation, imaging characteristics and management of traumatic SSDH. The controversial pathogenesis is also discussed.
Abstract: During the last two decades, endoscopic endonasal approach has completed the minimally invasive skull base surgery armamentarium. Endoscopic endonasal skull base surgery (EESBS) was initially developed in the field of pituitary adenomas, and gained an increasing place for the treatment of a wide variety of skull base pathologies, extending on the midline from crista galli process to the occipitocervical junction and laterally to the parasellar areas and petroclival apex. Until now, most studies are retrospective and lack sufficient methodological quality to confirm whether the endoscopic endonasal pituitary surgery has better results than the microsurgical trans-sphenoidal classical approach. The impressions of the expert teams show a trend toward better results for some pituitary adenomas with the endoscopic endonasal route, in terms of gross total resection rate and probably more comfortable postoperative course for the patient. Excepting intra- and suprasellar pituitary adenomas, EESBS seems useful for selected lesions extending onto the cavernous sinus and Meckel's cave but also for clival pathologies. Nevertheless, this infatuation toward endoscopic endonasal approaches has to be balanced with the critical issue of cerebrospinal fluid leaks, which constitutes actually the main limit of this approach. Through their experience and a review of the literature, the authors aim to present the state of the art of this approach as well as its limits.
Abstract: Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder, can lead to the development of hamartomas in various organs, including the heart, lungs, kidneys, skin and brain. The management of subependymal giant cell tumors (SGCTs) is still controversial, and peri- and/or intraventricular neoplasms may lead to life-threatening hydrocephalus. In the last years, many progresses have been made in research into the tumorigenesis and behaviors of SGCTs. This review aims to clarify the specific role of neurosurgeons in the multidisciplinary management of SGCTs in children with TSC.
Abstract: BACKGROUND AND PURPOSE: Intramedullary spinal cord tumors (IMSCT) are relatively rare neoplasms, accounting for less than 5% of all central nervous system tumors. The optimum management of these tumors still remains controversial. Many decades ago, partial surgical resection followed by radiotherapy was the conventional management for IMSCT. Nowadays, maximal surgical resection of IMSCT without adjuvant therapy is the rule. We discuss the management of our cohort of 45 patients and review retrospectively the surgical outcome and survival. METHODS AND RESULTS: We reviewed the charts of 45 patients who underwent surgery for IMSCT in our institution since 1990. The study included 23 female and 22 male with a mean age of 28.7 years (range: 18 months-64 years). In 40 patients, the final diagnosis was based on the results of MR imaging. The cervical location of the tumor was the most common (20 cases). Surgical procedures included a gross-total resection in 31 cases, subtotal resection in six cases, partial resection in five cases and a biopsy in three cases. The large majority of patients had histologically-proven low-grade tumors composed essentially of astrocytomas (44,4%) and ependymomas (28,8%). There was no mortality related to surgery. Concerning the functional outcome at six months, we noted that 22.2% of our patients deteriorated, 47.3% stayed the same and 30.5% improved. We found that patients with mild or no preoperative deficits were exceptionally damaged by the surgical procedure. CONCLUSIONS: The gold-standard treatment of IMSCT remains maximal microsurgical resection without adjuvant therapy. For malignant or rapidly recurrent IMSCT, the optimum management is still controversial. Determinant predictors for a good outcome after surgery of IMSCT are histological type of lesion, total removal of the tumor and a satisfactory neurological status before surgery.
Abstract: Langerhans cell histiocytosis is a systemic disease resulting from the oligoclonal proliferation of Langerhans cells, occurring most commonly in children and young adults. The focal form of the disease, also known as eosinophilic granuloma, most frequently involves the calvaria. We present two cases of calvarial eosinophilic granulomas that were surgically removed. These tumors are reputed to have an excellent prognosis, even if local recurrences and systemic dissemination can occur during the follow-up. The authors discuss the pathogenesis and the evolutive profile but also the therapeutic management of solitary eosinophilic granuloma of the calvaria.
Abstract: The trapped ventricular horn syndrome is characterized by the exclusion of a compartment of the ventricular tract from the rest of the CSF pathways. This syndrome has been reported in a variety of diseases including meningitis, intraventricular haemorrhages and tumours. Only two previous cases of neurosarcoidosis-related trapped temporal horn syndromes have been reported. The authors add a third case of a trapped temporal horn revealing neurosarcoidosis, presenting as a space-occupying process requiring urgent temporal tip lobectomy.
Abstract: Cerebrospinal fluid (CSF) leak is usually post-traumatic, but rarely it can be tumourous or spontaneous.
Complications of nasal polyposis are exceptional. The association between CSF leak and nasal polyposis has been rarely described.
Herein, we report the case of a woman with a history of recurrent meningitis and CSF leak, secondary to a huge benign nasal polyposis treated in a single neurosurgical procedure.
Abstract: Brainstem gliomas are a heterogeneous group of tumours commonly found in children, comprising about 10% of central nervous system tumours in paediatric patients, but less than 2% in adults. Pilocytic astrocytomas usually involve the midbrain and the medulla, and their surgical resection, when feasible, is generally curative. Thin calcifications can be normally found within low grade gliomas, but densely calcified pilocytic astrocytomas of the brainstem have been only rarely reported. We present the case of a young man presenting with a large brainstem calcification involving the medulla, which was subtotally resected using a posterior suboccipital approach. The definitive pathological diagnosis was calcified pilocytic astrocytoma.
Abstract: We report on a 22-day-old Tunisian boy born to consanguineous (first-cousin) parents (F = 1/16). The patient presents wide forehead with frontal encephalocele, wide anterior fontanel, marked hypertelorism, coloboma of the upper lids, proptosis, congenital glaucoma, broad nose, syndactyly between fingers 3 and 4, hypoplastic 3rd, 4th and 5th toes with nail dysplasia, hypospadias with cleft glans, bifid scrotum. Brain MRI showed right frontal encephalocele with anomalies of the cortical gyration without any corpus callosum abnormality. Normal chromosomes and parents' consanguinity are suggestive of autosomal recessive inheritance. Facial midline anomalies associated with limb and genitourinary anomalies is very uncommon. We present the third case reported in the literature.
Abstract: Ventriculoperitoneal (VP) shunt surgery is the most used technique for the treatment of hydrocephalus. This procedure is associated with a large amount of complications. Bowel perforation caused by a peritoneal shunt catheter is one of these complications, sometimes fatal, and is usually difficult to recognize, except when protrusion of the peritoneal catheter through a natural orifice occurs. This report presents the case of a 2-year-old boy who had undergone a VP shunt and later presented with protrusion of the peritoneal catheter through his mouth. The shunt device was removed and an external shunt procedure was achieved, using the original ventricular catheter kept in place. The diagnosis of bacterial meningitis was retained and an antibiotic therapy was started. The evolution was fatal in 15 days secondary to a bacterial ventriculitis. Through the reported cases of bowel perforation, many risk factors were individualized, such as age, congenital etiology of the hydrocephalus, silicon allergy or the length of the peritoneal catheter. Bowel perforation is a serious complication of VP shunt surgery, leading sometimes to a fatal outcome.
Abstract: Intracranial teratomas are rare germ cell neoplasms occurring more often during childhood. We report the case of a huge mature teratoma of the pineal region in a 10-year-old patient that was not correctly diagnosed preoperatively by stereotactic biopsy. The tumor was revealed by intracranial hypertension and a Parinaud syndrome. The tumor markers were within normal levels in the serum. A left transcortical parietal approach was used to completely resect the tumor. No adjuvant treatment was given. A complete neurological recovery was observed after the surgical procedure. Follow-up at 2 years did not show any recurrence. Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor. The treatment is exclusively surgical.
Abstract: Growing skull fractures are rare complications of head injury, affecting almost exclusively young children. This disease is characterized by the progression of a skull fracture with an underlying dural tear, leading to large cranial vault defects and parenchymal porencephalic damages. This report on three cases with a radiological follow-up depicts the difficulty in the management of neglected cases. Three young patients (two months, two years and six years old) were managed for growing skull fracture in our department since 2001. Even if the three patients were diagnosed early, the therapeutic management was complex because of a long delay due to several reasons. While a cranioplasty and dural repair were performed in all patients, one required a shunt procedure for a large leptomeningeal cyst associated with a unilateral hydrocephalus. The clinical and radiological features are reviewed, as well as pathophysiological hypotheses and therapeutic principles.
Abstract: Thalassemia is a chronic hemolytic anemia, endemic around the Mediterranean basin. Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that can involve many organs or tissues, including the epidural space, leading to a spinal cord compression syndrome. In almost all cases, the clinical and MR patterns are those of a lower dorsal spinal cord progressive compression secondary to an epidural expanding process. The management still remains controversial, including mainly blood transfusions, decompressive surgery and radiotherapy. We present a case of EMH in a thalassemic adolescent boy presenting with a T12 level of compression, treated successfully with blood transfusion, surgical decompression and radiotherapy.
Abstract: A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling right hemisphere with marked temporal herniation. The baby underwent a decompressive right hemicraniectomy with temporal cortical biopsies. The post-operative course was favourable. The histological findings were unspecific, showing a gliotic spongiosis with disseminated granular cells. The post-operative MRI depicted a right hemisphere atrophy. To our knowledge, a space-occupying presentation of HHE syndrome requiring surgical decompression has never been described before while only a few reports dealt with the neuropathological aspects of this syndrome.
Abstract: BACKGROUND: Spinal epidermoid cysts are rare tumors and they are exceptionally intramedullary. AIM: Report of news cases CASE REPORT: The diagnosis is established by magnetic resonance imaging and the treatment is surgical. The prognosis is generally good, with possible recurrence after many years. The author report five new cases of intramedullary epidermoide cysts operated between 1996 and 2001.
Abstract: Primary central nervous system hemangiopericytoma is rare, accounting for less than 1% of primary central nervous system tumors. Diagnosis is histological. Treatment is surgical excision, followed by radiotherapy. Long-term follow-up is mandatory for these tumors with a high potential for recurrence and metastasis. The sellar location is very rare, and can be confused with pituitary adenoma. We report the case of a patient presenting a sellar hemangiopericytoma, who underwent surgery via a transsphenoidal approach, then right frontal craniotomy followed by radiotherapy. There was no recurrence at one year follow-up.
Abstract: Space-occupying middle cerebral artery infarction represents about 10 to 15% of supratentorial ischemic strokes. This syndrome carries a high rate of mortality and requires aggressive surgical decompression. The authors present 6 patients with signs of trans-tentorial herniation operated on between February 2001 and August 2003. Neurological preoperative status was evaluated with Glasgow coma scale score and postoperatively with Barthel index. Three patients had excellent recovery (Barthel Index up to 70), one remained dependant and two died. Younger patients had better prognosis. Decompressive surgery, when done early, should improve mortality rate and even functional outcome. Optimal selection of patients, with the help of Diffusion-Weighted imaging, could vouch good results.
