Hafedh Jemel

Hemangiopericytoma of the Cerebellopontine Angle: A Wolf in Sheep's Clothing.

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.

Calcified Suprasellar Xanthogranuloma Presenting with Primary Amenorrhea in a 17-Year-Old Girl: Case Report and Literature Review

BACKGROUND Xanthogranuloma, also known as cholesterol granuloma, is an extremely rare intracranial neoplasm most commonly located in the middle ear, petrous apex, or choroid plexus. Exclusively suprasellar xanthogranulomas are exceptional and this report presents a very rare case in the pediatric population, particularly unique due to the presence of calcification. CASE DESCRIPTION A 17-year-old girl presented with primary amenorrhea with computed tomography and magnetic resonance imaging showing a large calcified enhancing suprasellar mass, which was presumptively diagnosed as a craniopharyngioma on the basis of its clinical and radiologic appearance. Gross total resection of a well-encapsulated, exclusively suprasellar tumor was achieved, without postoperative neurologic deficits. Histologic examination found fibrous tissue with abundant cholesterol clefts, multinucleated giant cells, and hemosiderin deposits but no epithelial cells. The final histologic diagnosis was a xanthogranuloma. CONCLUSIONS Xanthogranuloma, although extremely rare in the pediatric population, may present as a calcified suprasellar mass and manifest with primary amenorrhea. The prognosis after gross total resection is likely favorable; however, long-term follow-up is indicated for these rare neoplasms.

Cerebellar liponeurocytoma with extracranial extension: Case report

Cerebellar liponeurocytoma is a newly recognized, rare clinicopathological entity commonly described in the cerebellar hemispheres or the vermis. We present a rare case of cerebellar liponeurocytoma arising from the left cerebellar amygdala with extracranial extension. Such a condition has never been previously reported.

Spontaneous involution of a sinus pericranii

Because of the rarity of sinus pericranii, accepted guidelines or recommendations concerning the management, diagnosis, and treatment of are still lacking.Most patients are asymptomatic. However, it may cause fatal complications such as thrombosis, traumatic air embolism, or massive hemorrhage [1]. Authors recommend surgery usually with the aim of cosmetic improvement and for prophylactic purposes to prevent such complications [2, 3]. There have been no large observational studies that can help us to predict its natural history. Although the anomaly has a tendency to grow gradually, some authors have described spontaneous regression of sinus pericranii [4, 5]. We published a case of an 11-month-old boy, who was referred for a slowly growing mass on his scalp that the patient’s mother had noticed during his last 6 months of life. There was no history of trauma. Examination revealed a 1 × 2 cm frontal, superficial, nonpulsatile, painless soft tissue masswhich became larger in recumbent position or crying, and the skin over the lesion was normal as was neurologic examination. CT scan with three-dimensional CT (3D-CT) reconstruction (Fig. 1a–d) and MRI (Fig. 2e–g) were performed showing a vascular feature of the lesion with a focal enhancement of the portion of the sinus pericranii nearest the connectionwith the superior sagittal sinus. In our case, the radiographic studies supported a direct communication between the extracranial mass and intracranial venous systems and shown that no tumor or other significant abnormalities were present. Because of a limited esthetic prejudice and the absence of any functional disorder, our patient went on to be treated conservatively and will be seen for further follow-up [6]. Thus, we decided to observe the evolution with repeated imaging. At age 3 years, clinical examination revealed complete involution of the frontal mass. An angio-CT scan with three-dimensional CT reconstruction (Fig. 3a–d) was done 24 months later, which showed involution of the frontal lesion in size. The CT scan revealed the closure of the anterior fontanelle with complete disappearance of the communication between the extracranial mass and the superior sagittal sinus. However, in our case, because of the intramembranous ossification during anterior fontanelle closure, it may have gradually occluded the abnormal venous connection between the pericranii sinus and the superior sagittal sinus. * Sofiene Bouali sofienebouali@hotmail.fr

Temporal Dermoid Cyst with Unusual Imaging Appearance: Case Report.

Intracranial dermoid cysts are benign, slow growing tumors derived from ectopic inclusions of epithelial cells during closure of neural tube. These lesions, accounting for less than 1% of intracranial tumors, have characteristic computed tomography (CT) and magnetic resonance imaging (MRI) appearances that generally permits preoperative diagnosis. However, the radiologic features are uncommon and the cyst can be easily misdiagnosed with other tumors in rare cases. Herein, we report a case of a left temporoparietal dermoid cyst in a 48-year-old woman that was peroperatively and histopathologically proven but not advocated on CT and MRI. Clinical, radiological and histopathological features of a dermoid cyst are reviewed.

A case of adult anaplastic cerebellar ganglioglioma

Background: Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity. Case Description: A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa. Conclusions: Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

Symptomatic pneumocephalus: A rare complication of discal herniation's surgery

Context: We report the case of a 40-year-old woman with no pathological history, operated from an L4-L5 disc herniation by a left unilateral approach. The dura mater enveloping the left L5 root was accidentally injured at its lateral face causing a breach with CSF leakage. This breach could not be sutured. A few hours after waking, the patient presented an agitation followed by three generalized tonico-clonic seizures. Cerebral imaging revealed pneumocephalus. The patient was hospitalized in an intensive care unit. The symptoms gradually faded and the patient was discharged 3 days after surgery. Findings: Pneumocephalus is defined by the presence of air inside the skull. The symptoms of pneumocephalus are generally non-specific and varied, and this complication should also be kept in mind to prevent potentially severe course. The prevention of postoperative pneumocephalus depends on a well-defined strategy in the case of iatrogenic dural tear. Conclusions: Symptomatic pneumocephalus is a very rare complication in the course of lumbar surgery. Conservative therapy may be appropriate even in severe symptomatic manifestations.

Hodgkin Lymphoma revealed by epidural spinal cord compression

Context: Hodgkin Lymphoma is rarely diagnosed as spinal cord compression syndrome. Caused by an epidural mass, this complication is often encountered in a late stage of the disease. We report the case of a 40-year-old man presenting with symptoms of low thoracic spinal cord compression due to an epidural tumor on the MRI. Findings: Emergent surgery was undertaken on this patient, consisting in laminectomy and tumor resection. After surgery, pain relief and mild neurological improvement were noticed. The histological study revealed a Hodgkin Lymphoma and the patient was referred to chemotherapy and radiotherapy. Conclusion: Though chemotherapy is the gold standard treatment for Hodgkin Lymphoma, surgical spinal decompression may be required in epidural involvement of the disease. Diagnosis may be suspected in the presence of lymphadenopathy and general health decay.

The role of endoscopy in the treatment of hydrocephalus associated with aneurysmal malformation of the vein of Galen

Dear Editor: Vein of Galen aneurysms may be defined as direct arteriovenous fistulas between choroidal and/or quadrigeminal arteries and an overlying single median venous sac [1]. It makes up approximately 1% of intracranial vascular malformation but 30% of all pediatric vascular malformation [1]. Malformations of the Galenic vein are believed to result from a dysembryogenic event involving the cerebral vasculature between 6 and 11 weeks of gestation [2]. The clinical manifestations of VGMs depend on age, with congestive heart failure commonly affecting newborns and hydrocephalus in older children/adults [2]. Both aquaductal obstruction and venous hypertension leading to an absorptive defect are plausible explanations as to the etiology of hydrocephalus [3]. However, while the management of vein of Galen aneurysms is firmly established with endovascular obliteration, the treatment of the concomitant hydrocephalus based on its pathophysiological implications remains the object of discussion in the neurosurgical literature. To date, ventriculoperitoneal shunting was the firstline treatment for all cases of hydrocephalus in children with vein of Galen malformations described in the English literature. It is fraught with shortand longterm dangers, including status epilepticus, intraventricular hemorrhage, subdural hematoma and hygroma, venous infarction, malignant dystrophic calcification, and worsening developmental delay [4]. The role of endoscopy in the treatment of hydrocephalus associated with vein of Galen malformations in childhood is not clear and little to no pediatric or neurosurgical literature has been published about this subject. Endoscopic third ventriculostomy has been described in two case reports for the treatment of hydrocephalus in adult patients with vein of Galen malformation with the resolution of symptomatic hydrocephalus [2, 3]: the first one described by Feletti et al. in a patient with a partially thrombosed vein of Galen malformation [2] and the second one has been described by Zeiler et al. in a man with triventricular hydrocephalus caused by an aneurysmal dilation of the great vein of Galen [3]. However, the efficacy of endoscopic third ventriculostomy in the infant patient population with non communicating hydrocephalus from vein of Galen aneurysms is not clear. Our patient was a 13-month-old girl. She was brought by his parents with complaints of increasing lethargy and vomiting for the last 5 days. There was no significant family history or history of any other sibling suffering from similar complaints. The child was born with full* Sofiene Bouali sofienebouali@hotmail.fr

Does a systematic algorithm matter for the management of ventriculoperitoneal shunt perforations of the gastrointestinal tract

Dear Editor: The gastrointestinal tract perforation is a rare complication of ventriculoperitoneal shunt, occurring in 0.1–0.7% of patients and can be fatal if unrecognized [1]. There have been less than 100 reports on migration of the distal catheter of the ventriculoperitoneal shunt to rectal orifice since this phenomenon was recognized 50 years ago, out of which 50% were children below 15 years of age [2, 3]. Colon is the most frequent site of perforation and clinical manifestations can be variable and nonspecific. Patients can be asymptomatic or may present with abdominal pain, rectal extrusion of the tubing, fever, diarrhea, shunt dysfunction, meningitis, or seizures [4]. The pathogenesis which was described as plausible explanations to the etiology of bowel perforation was related to adherence, and constant pressure of the catheter tip to the wall of viscera with local inflammatory reaction leads to erosion of the visceral wall and entrance of tip in the lumen [3]. Thin bowel wall of children, chronic irritation by the shunt, stiff shunt catheters, silicone allergy, and use of abdominal trocars had been described to increase the risk of bowel perforations [5]. Transanal catheter protrusion is a potential risk for peritonitis, ventriculitis, meningitis, or sepsis via retrograde migration of bacteria with a 15% risk of mortality [4, 6]. Regarding this rare complication, the treatment of ventriculoperitoneal shunt with bowel perforation based on its pathophysiological implications remains the object of discussion in the neurosurgical literature. Management must be individualized and depends upon multiple factors based on clinical presentation. Conservative management and surgery have been performed. In an asymptomatic case where patient’s exam remains benign, the shunt tubing should be immediately disconnected at the abdominal wall and the distal end should not be pulled back through the peritoneal cavity if at all possible, in order to prevent the retrograde spread of bacteria along the shunt system. The exteriorized end of a catheter can be attached to a longer filament suture and removed or through the orifice via colonoscopy [2–4]. Many authors demonstrated that the fistulous opening should close spontaneously after removal of the catheter [1, 4, 6]. Ventricular shunt externalization is necessary to maintain shunt patency. In this case, so that cerebrospinal fluid culture is sterile, there has been considerable discussion and controversy on the prescription of antibiotic therapy. Urgent surgery should be undertaken in patients with intraabdominal complications such peritonitis or abdominal abscess. Surgery must be performed when the fistulous tract does not close spontaneously [1]. Laparotomy, laparoscopy, and transanal repair of colonic perforation have been reported [2]. Some author recommend shunt replacement on opposite side, and others choose a different terminus outside the abdominal cavity such as the atrium or pleura, as there remains the concern that the factors leading to bowel perforation are still present, but there still no consensus about the timing. We report a case of a 6-month-old girl, who was referred to us with extrusion of a ventriculoperitoneal shunt catheter through the anus so as to add to the existing literature of this unusual complication. * Sofiene Bouali sofienebouali@hotmail.fr