IntroductionLes disproportions cranio-encéphaliques (craniosténose iatrogène) sont une des complications du traitement de l’hydrocéphalie avec valve de dérivation ventriculaire. Lorsque la modification de la pression d’ouverture des valves ne permet pas d’amélioration clinique, une chirurgie de correction de la déformation crânienne doit être envisagée. Nous avons révisé la prise en charge ainsi que les résultats à long terme des cas de craniosténose iatrogène dans notre service et l’ont comparé avec les cas publiés. La base de données du service de neurochirurgie de l’hôpital universitaire de Fattouma Bourguiba Monastir a été consultée pour identifier les cas de déformation crânienne induite par un shunt ventriculaire.Cas cliniques2 cas de craniosténose secondaire ont été identifiés. Ils ont bénéficié tous les 2 d’une dérivation ventriculo-péritonéale à moyenne pression à l’âge néonatale dans le cadre d’une prise en charge d’hydrocéphalie congénitale. Au cours de leur suivi en consultation externe une scaphocéphalie a été identifiée chez les 2 patients sur leur TDM cérébrales de contrôle (avec reconstruction tridimensionnelle). On a noté que les sutures n’étaient pas totalement fermées. L’attitude conservatrice avec surveillance rapprochée était choisie. Sur un recul de 2 ans, une nette amélioration de la forme du crane chez nos 2 patients était observée sans preuve radiologique d’une fusion progressive des sutures.DiscussionBien que rare, la craniosténose post-shunt, est la première cause de survenue secondaire craniosténose. Le remodelage de la voûte crânienne n’est pas toujours nécessaire, surtout lorsque les sutures ne sont pas totalement fusionnées.

Le syndrome d'hyper-IgG4 appelé aussi maladie systémique associée aux IgG4 (MSA IgG4) est une entité de description récente caractérisée par la présence d'une ou plusieurs atteintes fibro-inflammatoires d'organe, associées le plus souvent à une élévation des IgG4 sériques. Les lésions tissulaires sont caractérisées par une fibrose et un infiltrat inflammatoire lymphocytaire et plasmocytaires exprimant des IgG4. Les tableaux cliniques peuvent être polymorphes et atypiques, rendant le diagnostic difficile et parfois tardif. Nous rapportons ainsi un cas de syndrome d'hyper-IgG4 découvert à l'occasion d'un trismus. Il s'agissait d'un patient âgé de 37 ans, sans antécédents pathologiques notables, qui avait consulté les urgences pour un trismus droit associé à une fièvre et des céphalées en casque d'installation brutale. L'examen neurologique était normal en particulier pas de syndrome méningé et aucun foyer infectieux au niveau de la sphère otorhinolaryngologique n'était descellé. Le fond d'œil pratiqué en urgence avait montré un œdème papillaire unilatéral droit. La biologie avait objectivé un syndrome inflammatoire biologique, une lymphopénie et une thrombopénie. Le scanner cérébral avait mis en évidence : un processus tissulaire de la paroi parasellaire de la petite aile sphénoïdale droite mesurant 5 × 2,3 × 3,5 cm qui s'étend latéralement au lobe temporal, en bas aux muscles ptérygoïdiens et masticateurs, en médial le nerf optique qu'il refoule et les sinus caverneux avec présence des quelques adénopathies sous angulomandibulaires bilatérales. Ainsi, une biopsie chirurgicale était pratiquée objectivant une inflammation chronique fibrosante avec présence des multiples lymphocytes (n = 35) et plasmocytes IgG4 + . Le dosage sérique des IgG4 n'était pas fait. Le diagnostic de syndrome d'hyper-IgG4 était retenu. Un complément d'imagerie thoracoabdominopelvienne a révélé une splénomégalie sans autres signes de fibrose médiastinale ou rétro péritonéale. Une ponction sternale pratiquée avait confirmé le caractère périphérique de la thrombopénie. Le patient était traité par corticothérapie et des boli mensuels de cyclophosphamide. Une rééducation fonctionnelle était de même entamée avec une bonne évolution après 6 mois de recul. La MSA IgG4 est une pathologie rare, de diagnostic difficile qui est généralement corticosensible. Son pronostic est conditionné par les atteintes des organes nobles tel que le cœur, les poumons et les reins dont le traitement précoce permet de prévenir la fibrose définitive.

The coexistence of a pituitary adenoma and a meningioma is a rare event. In all previously reported cases, only 1 of these 2 tumors was symptomatic. We present the case of 61-year-old woman with no significant medical history who was treated for a simultaneously symptomatic pituitary nonfunctioning adenoma and foramen magnum meningioma. Such an association has not been reported previously.

Congenital glioblastomas are uncommon tumors of infancy with unique molecular features and usually better prognosis compared to their pediatric and adult counterparts. A 15-day old newborn harboring a congenital glioblastoma undiagnosed at pregnancy presented to our emergencies with rapid neurological deterioration. Radiological investigations revealed an 8 by 9 cm space occupying and enhancing lesion in the left temporoparietooccipital region with intratumoral hemorrhage. The patient was immediately transferred to the operative theater and was operated in a poor condition. Unfortunately he showed no improvement post-operatively and died within two days in the intensive care unit. As such presentation is unique; we present the case and review the relevant literature concerning the potential pitfalls in diagnosis, management strategies and improvements in outcome.

Background: Angiomatous meningiomas (AMs) represent a rare subtype of meningiomas in which the vascular component prevail. They represent less than 1% of all intracranial tumors and approximately 2.1% of all meningeal tumors (Hasselblatt et al., 2004).The purpose of this study was to determine the clinical characteristics, radiological features and prognosis of AMs based on a Tunisian multicenter experience in the management of 58 successive cases of intracranial AMs. To the best of our knowledge, this is the largest series reported to date.Methods: We retrospectively reviewed 58 patients of AM treated in the departments of Neurosurgery of The Tunisian National Institute of Neurology, Sahloul University Hospital and Fattouma Bourguiba University Hospital from January 2001 to December 2015. Clinical characteristics, radiographic features and treatment modality, in the form of radical surgery, were noted. Statistical analysis was done with regards to recurrence free survival (RFS) and overall survival (OS) using Kaplan-Meier survival analysis.Results: The median age of the patients on admission was 53.0 years (8-78). Twenty-two patients were males and thirty-six were females. The median duration of symptoms prior to presentation was six months. Signs of increased intracranial pressure were the most common presenting symptom followed by seizures, motor weakness and cranial nerves paresis. Cystic changes were observed in 50% of cases, moderate to severe peritumoral edema in 81% of cases and multiple signal voids suggestive of blood vessels in 86.2% of cases. Forty-two patients underwent Simpson I excision (72.4%) while fourteen had Simpson II excision (24.1%). A Simpson III resection was realized in two patients (3.5%). The surgical resection was hemorrhagic in 63.8% of cases and in 13.8% of cases, several blood transfusions were necessary to maintain hemodynamic stability. No adjunctive treatment was administered. Six out of the fifty-six patients of the Simpson I/II groups recurred while one of the two patients with Simpson III resection had tumor regrowth. Median duration of RFS was 103 months. Second surgery was realized in all cases with no subsequent recurrence. The extent of tumor resection and the location were found to correlate with the risk of developing recurrences (P=0.001).Conclusions: AMs represent a rare subtype of meningioma characterized by variable cystic components, large peritumoral edema and multiple areas of vascular signal voids.The mainstay of the treatment is gross total resection, ideally following a preoperative embolization.The fate of the tumor remnant after incomplete tumor resection still needs to be evaluated and we do not recommend the systematic use of post-operative adjuvant RT in all cases.As local recurrence can develop many years after initial treatment, Long-term follow-up is mandatory. (C) 2016 Elsevier B.V. All rights reserved.

BACKGROUND:Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy.CASE DESCRIPTION:We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up.CONCLUSIONS:Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Objective and importance: While bone invasion and hyperostosis are frequent phenomena in meningiomas, primary intraosseous meningiomas are rare and their occurrence in the skull base is an extraordinary exception. Moreover, radiation-induced meningiomas represent a unique clinical dilemma given the fact that patients with these tumors had often received a prior full course of radiotherapy.Clinical presentation: A 42-year-old man presented with a 3-month history of progressively worsening facial asymmetry. His medical history was consistent for a posterior cranial fossa irradiation at the age of 6 years for a non-confirmed brain stem tumor. On admission his Kamofsky performance status was graded as 50% and his neurological examination showed a complete right facial nerve paralysis and hearing impairment. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumor invading the whole right petrous bone without intracranial involvement.Intervention: As the tumor reached the external auditory canal, a tissue sample was obtained locally. Pathological examination of the lesion identified a grade II clear cell meningioma and the patient was consequently addressed for an intensity modulated radiation therapy. His condition remained unchanged till the most recent follow-up examination, 8 months later. Conclusions: To the best of our knowledge, a radiation induced osteolytic clear cell meningioma of the petrous bone has notbeen previously reported. As little literature exists regarding the use of adjuvant therapies for these tumors, intensity modulated radiation therapy remains an attractive treatment option in case of pervious irradiation and general status alteration. (C) 2016 Polish Neurological Society. Published by Elsevier Sp. z o.o. All rights reserved.

Capillary hemangiomas are benign vascular tumors that are most often encountered superficially in the soft tissues of the head and neck region and frequently follow trauma; epidural location is exceptional. We report an uncommon case of epidural capillary hemangioma in the thoracic spine masquerading as an osteolytic lesion. In addition, we discuss the pathogenesis and management of this unusual condition.

BACKGROUND AND IMPORTANCE:Although glioblastoma is the most common primary brain tumor, primary intraventricular locations are extremely rare; only 21 cases have been reported to date.METHODS:A retrospectively acquired database of all intracranial glioblastomas treated in 2 different neurosurgical departments during the last 10 years was queried. Patients with histologically proven intraventricular glioblastomas were included in the study.RESULTS:Eight patients were identified as having a histologically confirmed intraventricular glioblastoma. Patient age at diagnosis ranged from 6 to 74 years (mean 29.6 years) and the male/female ratio was 5:3. Increased intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. The tumor was located within the lateral ventricle in 6 cases and the anterior third ventricle in 2 others. Gross total tumor excision was achieved in 3 patients, whereas the surgical resection was subtotal in 4 cases and a surgical biopsy was performed in 1 patient. Postoperative adjuvant therapies were administered in 5 patients. Median survival time was 32.1 months, and 3 patients were alive at the end of study. All of them had isocitrate dehydrogenase-mutated tumors.CONCLUSIONS:Intraventricular glioblastoma is extremely rare and can affect younger individuals including children. This malignant tumor should be included in the differential diagnosis of intraventricular lesions, especially in the lateral ventricles. Radical surgical resection can be associated with remarkable disease-free survival, especially in isocitrate dehydrogenase-mutated tumors. Because recurrence virtually is unavoidable, long-term follow-up is mandatory.

Background/Aims: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. Methods: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. Results: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. Conclusion: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.

Primary non-Hodgkin lymphoma of the spine is very rare and occurs mostly in adults with strong male predominance. Here, we present the case of a 24-year-old girl harboring a primary diffuse B-cell lymphoma of L2 vertebral body, who was admitted in an emergency with cauda equina syndrome and completely recovered after total spondylectomy and adjuvant chemotherapy. Such findings have never been previously reported.

Giant cell glioblastoma is not encountered in everyday practice and probably comes as an unexpected diagnosis most of the times. We present a rare case of giant cell glioblastoma mimicking a convexity meningioma in a 35-year-old man with 10 years of free clinical and radiologic follow-up.

Background. Cerebral hydatid cysts are rare and less than 5% of them show calcifications. Since symptoms are similar to those of brain tumor, and can mimic these lesions on radiologic investigations, intracranial calcified hydatid cysts can be misdiagnosed. Case Presentation. A 39-year-old man presented to us with seizures. On examination, he had right-sided homonymous hemianopia. Computed tomography (CT) brain showed calcified lesion in the occipital region, reported as calcified meningioma. Magnetic resonance imaging (MRI) showed a focal lesion in the left occipital region which was hypo intense on T1W and T2W images. A right parieto-occipital craniotomy was performed revealing a cystic mass which membrane was fibrous and the contents were semi solid. The cyst was excised completely with gentle dissection. There were no postoperative deficits. Histology revealed multiple calcific deposits, and remnants of hydatid cyst. Conclusion. Calcified intracranial hydatid cysts can mimic a primary brain tumor so they should be suspected if patients live in or come from areas with endemic hydatid cyst disease. The pathological examination confirms the diagnosis when it was not considered.

Sacrococcygeal yolk sac tumor (YST) is an extremely rare malignant extra-gonadal germ-cell tumor, which usually succeeds to the degeneration of more common benign teratoma.We describe here an unprecedented case of conus medullaris compression by a spinal metastasis from a pure sacrococcygeal YST in a 1½ years old girl, which was misdiagnosed initially as an anal fissure and stress the need of a meticulous clinical examination and further screening in young patients presenting with sphincter disturbances.

Central nervous system germ cell tumors (GCTs) account for less than 5% of primary brain tumors in children and adolescents but continue to attract much attention. To the best of our knowledge, a primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle has never been previously reported. A 15-year-old boy presented with signs of increased intracranial pressure for the past 2 weeks complicated by 2 episodes of generalized tonic-clonic seizures 1 day before admission. Neurological examination was normal, and funduscopic examination disclosed a grade II papilledema bilaterally. CT and MRI revealed a well-demarcated and enhancing mass within the anterior third ventricle associated with a left lateral ventricle hydrocephalus. There was no evidence of tumor within the pineal or suprasellar region, and systemic and cerebrospinal fluid evaluation demonstrated normal levels of α-fetoprotein and human chorionic gonadotropin. Radical surgery was advised, and total tumor resection was achieved via a transcallosal transforaminal approach. The postoperative course was uneventful, and the final histological diagnosis was a pure embryonal carcinoma. Further screening showed no other location, and adjunctive high-dose chemotherapy was administered. The patient has been symptom free with no clinical or radiological sign of progression at the most recent follow-up examination 2 years after surgery. Primary pure and nonsecreting embryonal carcinoma can develop within the anterior third ventricle and should be considered in the differential diagnosis of anterior third ventricular masses especially in young patients. Accurate identification, radical surgery and high-dose chemotherapy can result in better tumor control and improve the postoperative outcome.

La dysplasie fibreuse des os est une maladie osseuse bénigne, congénitale non transmissible touchant à égalité de fréquence les deux sexes avec une prévalence estimée à 1/2000. Sa localisation au niveau de la voûte est exceptionnelle et la littérature est réduite à des reports de cas ou de courtes séries. Nous présentons notre expérience en exposant les quatre cas pris en charge à notre service au cours des trente dernières années. Il s’agit de quatre sujets : deux de sexe masculin et deux de sexe féminin âgés de 12 à 24 ans (âge moyen de 18 ans) Tous se sont présentés pour une tuméfaction indolore de la voûte, d’aggravation progressive (deux fois en frontal et deux fois en pariétal). La radiographie standard faite dans tous les cas a montré systématiquement une lésion lytique de la voûte. Le scanner a confirmé l’atteinte osseuse et l’intégrité des structures encéphaliques. Tous les malades ont bénéficié d’une exérèse complète de leur lésion avec cranioplastie. L’évolution était favorable dans tous les cas avec absence de récidive après un suivi moyen de 13 ans. La dysplasie fibreuse de la voûte est une maladie rare qui touche électivement les sujets jeunes. Une tuméfaction unilatérale progressive et indolore est le maître symptôme. Le traitement associe chirurgie d’exérèse et cranioplastie et est associé à un bon résultat clinique dans la majorité des cas.

L’encéphalocèle intra-orbitaire se définit par l’issue de tissu cérébral souvent contus dans la cavité orbitaire à travers un fracas du toit de l’orbite. Il s’agit d’une complication exceptionnelle, résultat d’un traumatisme crânien violent à point d’impact supra-orbitaire, pouvant être à l’origine de complications visuelles graves. Le but de ce travail est de préciser les caractéristiques cliniques, radiologiques, évolutives ainsi que les modalités thérapeutiques à travers l’étude de trois observations colligées dans le service de neurochirurgie de Monastir de juillet 1992 à février2003, et de discuter les résultats obtenus en fonction des données de la littérature. Notre série se compose de trois patients s’ajoutant aux 23 cas déjà rapportés dans la littérature. L’âge des patients était respectivement de 3, 8 et 23 ans. L’accident était toujours très violent. Le délai d’apparition de cette complication était de 4 jours dans deux cas, Pour le troisième cas, le diagnostic a été fait au troisième jour post-traumatique suite à l’installation précoce d’une hypertension intracrânienne. Les signes cliniques se résument essentiellement à des signes ophtalmologiques tels qu’une exophtalmie ou des troubles de l’oculomotricité. L’examen tomodensitométrique a permis de poser le diagnostic dans tous les cas en montrant du tissu cérébral dans la cavité orbitaire. Le geste chirurgical consistait à aspirer le tissu cérébral contus et œdématié faisant hernie dans la cavité orbitaire puis à réaliser une plastie ostéo-durale du toit de l’orbite. La correction des troubles morphologiques était immédiate et une amélioration clinique était observée dans deux cas. Les traumatismes du toit de l’orbite constituent une entité anatomo-pathologique particulière par leur mécanisme de leur survenue ainsi que par les complications qu’ils peuvent engendrer. Ce travail nous a permis de revoir les tableaux cliniques, les critères radiologiques ainsi que les modes évolutifs des encéphalocèles intra-orbitaires. La chirurgie est la seule modalité thérapeutique. Cependant, malgré un traitement immédiat, les séquelles peuvent être non récupérables.