Sofiene Bouali

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

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.

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

Embryonal tumor with multilayered rosettes: illustrative case and review of the literature

BackgroundEmbryonal tumor with multilayered rosettes (ETMR) is a very rare entity and has seldom been reported. It has been newly defined tumor entity included in the latest update (revised fourth edition) of WHO 2016 Classification of Tumors of the Central Nervous System which portends a uniform dismal prognosis and survival even with the best of multimodality approaches.Illustrative caseThis report documents the presentation of a 2-year-old girl with voluminous intracranial ETMR in the right parieto-occipital region. We describe clinical diagnosis, histological aspects, radiological features, and current management of this very aggressive tumor.ConclusionPediatric intracranial ETMR is a highly aggressive neoplasm, and it should be considered in the differential diagnosis of pediatric brain tumors.

Intradural Extramedullary Capillary Hemangioma in the Upper Cervical Spine: First Report

BACKGROUND The occurrence of intradural extramedullary capillary hemangiomas is exceedingly rare. To date, only 39 cases of intradural extramedullary capillary hemangiomas have been reported in the English literature, and all of these cases have been described at the lumbar and thoracic spinal levels. To our knowledge, this report is the first case of capillary hemangiomas of the cervical spine in the literature. In general, this entity is misdiagnosed preoperatively as a neoplasm. CASE DESCRIPTION A 29-year-old man presented with neck pain and progressive gait disturbance, and was diagnosed with an intradural extramedullary capillary hemangioma in the cervical region. CONCLUSIONS Although rare, our case demonstrates that capillary hemangioma should be considered in the differential diagnosis of intradural extramedullary tumor of the cervical spine.

Secondary Chondrosarcoma of the Upper Thoracic Costovertebral Junction with Neural Foraminal Extension and Compressing the Spinal Cord

BACKGROUND Chondrosarcoma is a rare malignant tumor of bone. This family of tumors can be primary malignant tumors or a secondary malignant transformation of an underlying benign cartilage tumor. Secondary chondrosarcoma arising from a benign solitary costal osteochondroma is extremely rare. Data show that the reported incidence of costal osteochondroma is very low and they are usually found in the anterior region at the costochondral junction. To our knowledge, however, there have been no previous reports, in English literature, describing osteochondroma malignant transformation located in the thoracic costovertebral junction. CASE DESCRIPTION We report the case of a man with chondrosarcoma arising from the malignant degeneration of an osteochondroma at the right first thoracic costovertebral junction with neural foraminal extension and compressing the spinal cord. CONCLUSIONS Although it is rare in solitary osteochondromas of rib, malignant transformation must always be considered.

Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study

BackgroundInvolvement of the orbit with Burkitt’s lymphoma is a very rare presentation of extra-nodal lymphoma.Illustrative caseWe report a case of a 2-year-old female presented an unusual location of sporadic Burkitt’s lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings.The mass was histologically confirmed as Burkitt’s lymphoma, and postoperative aggressive chemotherapy was initiated.We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor.ConclusionBecause of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.

Spinal epidural angiolipomas: Clinical characteristics, management and outcomes.

Purpose: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. Methods: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. Results: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. Conclusions: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.