Spontaneous spinal epidural hematoma in infants: A case report and review of the literature.

Background: Spontaneous spinal epidural hematomas (SSEHs) are exceedingly uncommon, especially in infants, with only two reported cases. Diagnosis can be delayed due to the nonspecificity of presenting symptoms. Case Report: We present a case of SSEH in a 10-month-old boy admitted to the pediatric emergency department with a 5-day history of progressive lower extremity motor weakness. There was no history of prior trauma. Magnetic resonance imaging of the spine revealed a posterior epidural hematoma extending from C7 to L4. After hematoma evacuation, the patient's neurological status gradually improved, and no sensorimotor deficit was present 3 weeks postoperatively. Conclusion: Our case suggests that surgical intervention can lead to an excellent prognosis for SSEH in infants, even if the diagnosis is delayed.

Spinal epidermoid cyst associated with limited dorsal myeloschisis.

Background: Epidermoid cysts (ECs) are rare benign tumors arising from epidermal cells, associated with congenital abnormalities or acquired through trauma, surgery, or lumbar punctures. They represent <1% of all intraspinal tumors and may be associated with limited dorsal myeloschisis (LDM). Case Description: A 7-year-old neurologically intact male had a dorsal skin mass since birth located posteriorly in the midline of the inferior thoracic spine. The mass was palpable, painless, mobile, vascularized, and could be transilluminated. Thoracic magnetic resonance imaging showed an extensive intradural extramedullary cystic lesion extending from D6 to D8 that did not enhance with contrast, accompanied by a subcutaneous fluid collection at D8-D9 communicating with the subarachnoid space. The patient underwent gross total resection of the lesion, pathologically confirmed as an EC. The postoperative course was uneventful, with no recurrence 1 year postoperatively. Conclusion: LDM may be associated with ECs. Early diagnosis and surgical resection of these lesions are essential for favorable outcomes.

Pediatric extra-axial glioblastoma with bone invasion leading to a subcutaneous mass: A case report.

Background: Pediatric glioblastoma multiforme (p-GBM) is an exceptionally rare and aggressive brain tumor, with even fewer reported cases with radiographic and intraoperative characteristics that mimic those of extra-axial lesions, often posing a diagnostic challenge. Despite advancements in imaging technologies, the diagnosis of GBM can still be intricate, relying primarily on histopathological confirmation. Case Description: We present a unique case of a 15-year-old female who presented to our hospital with a new-onset focal-to-bilateral tonic-clonic seizure described as clonic movements of her left hemicorps; on clinical examination, a subcutaneous mass was evident in the right parietal region. Magnetic resonance imaging of the brain revealed a sizable extra-axial enhancing mass measuring 9 cm, located in the right parieto-occipital region with notable bone invasion. Moreover, the intraoperative findings revealed an extra-axial mass attached to the dura. Total en bloc resection was achieved. The histopathological analysis confirmed the diagnosis of glioblastoma multiforme. Subsequently, the patient underwent adjuvant radiotherapy in conjunction with temozolomide chemotherapy. Postoperatively, she exhibited clinical improvement and remained stable throughout the 6-month follow-up period. Conclusion: We present the first case of extra-axial p-GBM in a young patient, which remarkably led to the destruction of the bone and finally resulted in a sizable parietal subcutaneous lesion in the absence of prior surgery or radiation.

Stab injury to the lumbar spine without neurological involvement in a child.

Background: Stab wound injuries are extraordinary in the child, the thoracic and lumbar spine are the most observed. Patient could be asymptomatic and it could lead to a neurological deficit. Case Description: We report a case of a 15-year-old boy victim of a stab wound injury with a knife. He was neurologically intact but the local examination showed blood and clear fluid. The patient underwent surgery and the knife was removed with the reparation of a dural tear. Conclusion: Stab wound injuries in child are very rare, the management is clear if there is compression, bleeding or cerebrospinal fluid leakage, and the prognosis depends on the symptoms.

Dermoid cyst of the posterior fossa.

Intracranial dermoid tumors represent a rare clinical entity accounting for 0.1-0.7% of all intracranial tumors. Their location in the posterior fossa is uncommon. We report a 16-year-old male patient who presented with clinical signs of increased intracranial pressure and cerebellar symptoms. The CT scan revealed a median cystic lesion of the fourth ventricle causing an active triventicular hydrocephalus. The MRI showed a median well shaped cystic lesion, of low signal intensity compared to the CSF, with capsular contrast enhancement. He underwent endoscopic third ventriculostomy before subtotal removal of the lesion. The postoperative course was uneventful, and the histological diagnosis was a dermoid cyst. Through this observation, we aim to discuss the clinical, and radiological aspects of the posterior fossa dermoid cyst, and to review the therapeutic strategies.

Atypical radiological findings in cerebral hydatid disease.

Cerebral hydatid disease is very rare, representing only 2% of all cerebral space occupying lesions. The diagnosis is usually based on a pathognomonic CT pattern. Exceptionally, the image is atypical raising suspicion of many differential diagnoses such as intracerebral infectious, vascular lesions, or tumors. We report 2 atypical cases of cerebral hydatid cysts diagnosed in a 21, and a 24-year-old woman. The CT scan results suggest oligodendroglioma in the first case and brain abscess in the second. An MRI was helpful in the diagnosis of the 2 cases. Both patients underwent successful surgery with a good outcome. The hydatid nature of the cyst was confirmed by histology in both cases.

Posterior epidural migration of a lumbar disc herniation.

BACKGROUND: Posterior epidural migration of a lumbar disc fragment (PEMLDF) refers to the dorsal migration of disc material around the thecal sac that can lead to radiculopathy and/or cause a cauda equina syndrome. It is rare and the diagnosis is often just established intraoperatively. CASE DESCRIPTION: A 50-year-old male with a chronic history of low back pain and psychosis presented with PEMLDF originating at the L4-L5 level. CONCLUSION: Lumbar disc herniations rarely present as PEMLDF resulting in symptoms varying from radiculopathy to cauda equina syndrome. These should be included among the differential diagnostic considerations for dorsolateral epidural lesions.

Subdural Empyema Complicating a Giant Fronto-Ethmoidal Osteoma.

Osteomas of the paranasal sinuses rarely lead to intracranial manifestations. We present an unusual case of a giant frontal sinus osteoma leading to subdural empyema formation. Determine the origin and the optimal surgical approach of these unusual lesions by analyzing giant osteomas of the frontal and ethmoidal sinuses in the literature. We report a rare case of giant frontoethmoidal osteoma with intracranial extension in a 34-year-old man, revealed by seizures. Neuroradiological studies revealed frontoparietal subdural empyema associated to a large osteoma in the right frontal sinus. The patient underwent surgical evacuation of the empyema and resection of the osteoma in one stage operation of decompressive craniotomy. The patient recovered very well after surgery and postoperative antibiotic therapy. This case represents in the literature only the third-reported case of subdural empyema complicating frontoethmoidal osteoma. The surgical treatment options, including open surgery techniques and endoscopic approaches, as well as pathogenesis are discussed according to the relevant literature.

ATRT of lateral ventricle in a child: A Rare Tumor at a Very Rare Location.

Atypical teratoid/rhabdoid tumors (AT/RTs) of infancy are highly malignant central nervous system neoplasms that are most commonly seen during the first 2 years of life with limited therapeutic options. To date, only two cases have been described in the lateral ventricle. A 4-year-old boy presented with a 4-month history of increased intracranial pressure. Cerebral magnetic resonance imaging (MRI) revealed a huge intraventricular tumor, occupying the entire temporal horn and the body of the left lateral ventricle. The boy was operated through a left temporal transventricular approach with gross total removal of the lesion. The histopathological diagnosis was an AT/RT. The infant underwent adjuvant chemotherapy and radiation therapy. The 1-year MRI of control showed a local recurrence of the tumor. Then after, Gamma Knife radiosurgery was performed because of the small volume and the deep location of the lesion. At the 3-month follow-up, the MRI showed a significant growth of the tumor volume, and the child was given additional adjuvant chemotherapy. Unfortunately, he died 9 months later. AT/RT of the lateral ventricle is a very rare tumor in children, associated with a poor prognosis in spite of multimodal treatment. Gamma knife surgery (GKS) was rarely reported as a treatment modality of AT/RT. The aim of this work is to discuss about the rarity of this tumor and the best treatment strategy to improve prognosis.

Primary spinal epidural hydatid cyst with intrathoracic extension.

Spinal epidural localization of hydatid cyst is quite rare. We report a case of a 33-year-old patient who experienced paraparesis over 2 years, with an umbilical sensitive level. A CT scan and MRI showed an intrathoracic multilobar lesion, probably of intra-spinal origin. An anterolateral transpleural surgical approach confirmed the hydatic character of the observed lesion and enabled total spinal cord decompression. No osseous involvement was noted. We report a case of spinal epidural hydatid cyst successfully managed by an anterior approach, and we discuss epidemiological, diagnosis, and therapeutical features of this rare localization of hydatid cyst.

Spinal Arteriovenous Fistulas of the Filum Terminale: Case Report and Literature Review.

Spiinal arteriovenous fistulae (AVF) are an uncommon cause of myelopathy that require a high degree of suspicion to diagnose. Treatment strategies have not yet been established. Only a few cases of AVFs of the filum terminale (FT) have been reported. In this review, we describe clinical presentation, imaging, and treatment options for this rare type of spinal AV shunt. A 43-year-old male patient presented with progressive low back pain and paraparesis with gradually worsening bilateral foot paresthesias and sphincter dysfunction. He underwent magnetic resonance imaging, which revealed a hypersignal in the thoracolumbar cord and angiography diagnosed a microfistula of the FT. Surgery was preferred over endovascular treatment and we realized an L5 laminectomy to open the dura mater and found a hypertrophic FT. After identifying the fistula which was closely related to cauda equina, and dissecting the root from the fistula, a permanent clip was placed on the proximal part of the arterialized vein. Surgery was uneventful, and 6 months postoperatively, the patient has fully recovered. FT AVFs although rare should be considered as a differential diagnosis of progressive paraparesis, and successful surgery through clipping relies on the angioarchitecture of the shunt and the clinical manifestations of the patient.

[Spinal schwannomas: case series]. / Shwannomes rachidiens: étude de séries.

Spinal schwannomas are benign tumors accounting for 30% of all spinal tumors. They originate from the shwann cells of the spinal roots. We report our experience in managing patients with spinal shwannomas, from diagnosis to treatment modalities, in the Department of Neurosurgery at the University Hospital Hassan II in Fez over a period of 13 years. The patients with spinal shwannomas accounted for 19.5% of those with spinal cord compression treated surgically over the same period. The average age of patients was 45 years, with a discreet female predominance. Spinal schwannomas had an insidious onset, then the median of consultation time was 18 months. The main symptoms were spinal and radicular pain. Half of our patients had neurological deficits. Medullary MRI was performed in all of our patients. Spinal schwannomas occurred predominantly in the chest (40%); 62% of shwannomas were intradural lesions, rarely extradural lesions (8%) and mixed lesions(4%). Complete Surgical resection was performed in 96% of cases with osteosynthesis in two cases and arthrodesis in a single case. Histological examination confirmed the diagnosis of benign neurinoma in 23 cases, malignant shwannoma in one case and neurofibroma in one case. Outcome was favorable in the majority of cases, two patients had complications, an infection of the wall and neurological worsening. The interest in the subject of our study is to highlight the features of these lesions and to compare the results of our case series with the data in the literature.

Variation of Ventricular Size after Surgical Treatment of Chronic Subdural Hematoma.

BACKGROUND: Surgical removal is the treatment of choice for chronic subdural hematoma (CSDH). Despite clinical improvement after surgery, computed tomography (CT) scan control often showed residual collection, which may discuss the possibility of failed surgery. The aim of this study is the assessment of ventricular size before and after surgery and to study its relation with residual hematoma. METHODS: In this prospective study (2013-2016), 63 patients who had burr-hole drainage of CSDH were sequentially allocated to either two groups; Group 1 with CT scan control under the 3rd day of surgery and Group 2 with delayed CT scan control (from 4th to 7th day). Linear measure of ventricular size was assessed by Evans' index. We reviewed and analyzed the data between both groups. RESULTS: There were 33 patients in Group 1 and 30 patients in Group 2. Preoperatively, the average thickness of hematoma was 20.5 mm in Group 1 versus 19.9 mm in Group 2 (P = 0.67); the mean midline shift was 8.5 mm in each group; Evans' index was 26.7% in Group 1 and 27% in Group 2 (P = 0.7). Postoperatively, the mean thickness of the residual hematoma was 7.7 mm in Group 1 and 8.4 mm in Group 2 (P = 0.57); the mean midline shift was 3.3 mm in Group 1 and 1.9 mm in Group 2 (P = 0.08); Evan's index was 28.5% in Group 1 and 32.1% in Group 2 (P = 0.002). CONCLUSION: The adoption of Evans' index, for assessing the variation of ventricular size after surgery, by neurosurgeons appears to be a good and simple method for evaluation and following the success of surgical removal of CSDH, despite the observation of some residual collection in early CT scan control.

Intradural extramedullary ependymoma: is there constantly a hormonal relationship?

BACKGROUND: Ependymoma is a glial tumor that occurs in the central nervous system. The intradural extramedullary location of this neoplasm is very rare. The authors report a case of spinal intradural extramedullary ependymoma in a male and discuss its pathogenesis as well as its clinical, radiological, and therapeutical features. CASE DESCRIPTION: A 31-year-old man was admitted at the author's institution. The patient has had 1-year history of cervical pain, progressive quadriplegia, and bladder disturbances. Magnetic resonance imaging revealed an enhanced cervical intradural extramedullary tumor extending from the bulbomedullary junction to the C3 level, with severe spinal cord compression. Emergency surgical resection was performed, and a total removal of the lesion was accomplished. One year and half later, a local recurrence associated to a small cerebellar lesion was noticed justifying a second spinal intervention. Both surgical interventions demonstrated an intradural extramedullary ependymoma without attachment to the spinal cord or to the dura mater. Adjuvant craniospinal radiotherapy was recommended to the patient. CONCLUSION: The insufficiency of hormonal theory to explain solely the pathogenesis of this tumor might reveal other potential factors that have not been discussed in earlier literature.

Tuberculous osteitis of the skull in a child.

Tuberculosis is endemic in developing countries. However, skull tuberculosis is uncommon with few cases reported in the literature. We report a 10-year-old boy admitted for a left parietal painless swelling. A CT scan demonstrated a left parietal bony defect, destroying both inner and outer tables. This was associated with an enhanced epidural collection and scalp swelling. The patient was operated, and the microscopic examination revealed typical tuberculosis granuloma. The clinical presentation and management of this rare location of tuberculosis are discussed.

Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.

BACKGROUND: Ewing sarcoma (ES) is a malignant bone tumor that is most commonly observed in the long bones, the pelvis, and the chest. Primary intracranial localization is extremely rare. OBJECTIVE: In the aim of identifying the prognostic factors of this rare localization, we conducted a systematic review of the literature for patients with primary intracranial peripheral primitive neuroectodermal tumor/ES. METHODS: A complete MEDLINE search was undertaken for all articles reporting data for primary intracranial peripheral primitive neuroectodermal tumor/ES. We divided the patient population into 2 groups: group I, patients who were free of disease; and group II, patients who died or had uncontrolled disease at the last available follow-up. We conducted a comparative statistical analysis of data between the 2 groups. RESULTS: A total of 48 patients were included in the review. There were 32 patients in group I and 12 in group II. Fifty percent of tumors in group II and only 16% of tumors in group I were infratentotorial (P = 0.03). Signs of bone involvement were observed in 19% of patients in group I and 54% in group II (P = 0.03). Total removal was accomplished in 29% of patients in group I and in no patients in group II (P = 0.03). Radiotherapy was performed in 73% of patients in group II and 81% in group I (P = 0.43). Chemotherapy was administered in 36% of patients in group II and 74% in group I (P = 0.03). CONCLUSIONS: Infratentorial localization and the presence of bone involvement were associated with poor prognosis; Surgery seems to be a predictive factor of prognosis; radiotherapy and chemotherapy must be performed whenever the tumor is not totally removed.

Infected Intradural Dermoid Cyst with Complete Dermal Sinus of Posterior Fossa.

BACKGROUND: Intracranial dermoid cysts are benign embryologic tumors. They occur most often in children and young adults. Infected intradural dermoid cyst with complete dermal sinus of posterior fossa is rare. The main feature of this dermal sinus is the continuity from the intradural cyst through the occipital bone to the skin. METHODS: We report 2 cases of infected intradural dermoid cyst with complete dermal sinus of posterior fossa in children, with special attention to the clinical and anatomical features of this rare combination of anomalies. RESULTS: A 5-year-old boy presented with a midline cystic lesion responsible for triventricular hydrocephalus revealed by a cerebellar syndrome and a dermal sinus. The second case was a 36-month-old girl who had an occipital cystic lesion that was limited by a pseudo-wall with diffusion restriction revealed by a double dermal sinus. We inserted an external shunt to relieve the increased intracranial pressure then performed a suboccipital approach for excision followed by systemic antibiotic therapy. Both cases had satisfactory outcomes. CONCLUSIONS: Infected intradural dermoid cyst with complete dermal sinus of posterior fossa is rare, and a double dermal sinus has not yet published, to our knowledge.

[A rare cause of exophthalmia: intraorbital cavernous hemangioma (about a case)]. / Une cause rare d'exophtalmie: l'hémangiome caverneux intraorbitaire (à propos d'un cas).

Cavernous hemangioma is the most frequent primary benign vascular tumor of the orbit in the adult; the median age of diagnosis is 42 years with a female predominance. This tumor develops slowly and has no tendency for spontaneous regression; it is electively located at the level of the retro-ocular muscular cone but it can develop in the extraconic space. Clinically it appears as progressive irreducible, non-pulsating, painless (unless there's an unexpected complication) exophthalmia, associated with decrease in visual acuity in 2/3 of cases. The diagnosis is easily confirmed by imaging, which allows to precisely locate the tumor in relation to the optic nerve and the oculomotor muscles and to indicate the type of surgical approach. Surgical resection should be complete; it is usually simple because the tumor is limited and perfectly cleavable. Surgical approach is established on the basis of tumor volume and above all of the seat of the lesion. Functional prognosis is good and recurrences are rare. We here report the case of a 44-year old patient with orbital cavernous hemangioma revealed by exophthalmia.

[A rare cause of spinal cord compression: spinal epidural arachnoid cyst (about 3 cases)]. / Une cause rare de compression médullaire: kyste arachnoïdien épidural rachidien (à propos de 03 cas).

Spinal epidural arachnoid cyst (SEAC) is a benign condition whose pathophysiology is still uncertain. It is most commonly asymptomatic but it can cause severe neurological sequelae especially when treatment is not received in time. We conducted a retrospective study of three patients treated for SEAC conducted in the Neurosurgery Department, Hassan II University Hospital, Fez. We report the case of two male patients and a woman, with an average age of 35 years (range: 16-56 years), admitted with slow progressive spinal cord compression. All patients underwent spinal cord MRI showing epidural fluid collection, having the same signal as that of cerebrospinal fluid, compressing the opposite marrow. The collection was located in the chest in all cases. All patients underwent surgery via posterior approach for cyst resection and cyst neck ligation in two cases and dural plasty in a single case. Anatomo-pathologic examination showed arachnoid cysts. Postoperative outcome was simple in all cases. This study aims to update the current understanding of this pathology while insisting on the need for early management given its tendency toward gradual worsening in the absence of adapt therapy. It also aims to review the clinical, paraclinical and therapeutic features of this condition.

[Rare medico-surgical emergency: spinal epidural abscess (about 3 cases)]. / Une urgence médico-chirurgicale rare: l'abcès épidural rachidien (à propos de 03 cas).

The awareness about infections in the epidural space is increasing thanks to the development of neurosurgery, including MRI. Spinal epidural abscess is a rare pathology but extremely serious from a functional point of view and potentially life threatening. We report three cases of male patients (the first one aged 52 years, the second 57 years and the third 63 years) with diagnosed spinal epidural abscess. Two patients were admitted to the Neurosurgical Emergencies with slow progressive spinal cord compression evolving in the context of infection. The last patient complained of S1 sciatica pain in his right leg resistant to treatment associated with urinary incontinence. Entrance door of the infection wasn't identified during the initial assessment. All patients underwent spinal cord/radicular decompression surgery and evacuation of the epidural abscess via posterior approach. Bacteriological examination showed pyogenic germ justifying adequate prescription of antibiotic therapy in the three cases. The evolution was favorable in two cases. However one patient died three days after surgery due to severe sepsis.