[Uveitic glaucoma in Behçet's disease: When everything gets complicated]. / Glaucome uvéitique dans la maladie de Behçet : quand tout se complique .

INTRODUCTION: Behçet's disease is a systemic disease of unknown etiology. Its ocular manifestations are multiple and polymorphic, with uveitis at the top of the list. Classically, uveitis in Behçet's disease does not cause elevation of intraocular pressure. However, this may be found in 10 to 20% of cases, secondary to trabeculitis, trabecular obstruction by inflammatory cells, anterior or posterior synechiae, or induced by prolonged use of corticosteroids. Its occurrence is a major complication, the management of which is especially delicate when glaucoma occurs. The purpose of this study was to study the factors predictive of glaucoma in Behçet's disease. MATERIALS AND METHODS: This is a retrospective study of all the charts of patients consecutively hospitalized for Behçet's disease over a period of 8years in the ophthalmology A department of the specialty hospital of Rabat university medical center. RESULTS: Fifty patients were treated for Behçet's disease during the study period. There were 41 men (82%), and 9 women (18%). Glaucoma was diagnosed in 11 patients (22%). The mean age of this subgroup was 31years, with a gender ratio of 6 women/5 men. The glaucoma was associated with anterior synechiae in 4 cases, primary open-angle glaucoma in 5 cases, neovascular glaucoma in 1 case and seclusio pupillae in 1 case. Therapeutically, all patients were on glaucoma eye drops, and five underwent filtering surgery. Statistically, on univariate analysis, ocular hypertension was observed particularly in patients with low initial visual acuity (less than 1/10) (P=0.001), with severe posterior segment involvement, especially retinal vasculitis (29% vs. 6%, P=0.04), macular edema (40% vs. 4%, P=0.001), and optic disc edema (50% vs. 15%, P=0.02). A high number of recurrences was also associated with glaucoma (1.0 vs. 3.0, P<0.0001). On multivariate analysis, low baseline visual acuity, presence of retinal vasculitis, and high number of recurrences were independent risk factors for ocular hypertension in our patients followed for Behçet's disease. CONCLUSION: Glaucoma can complicate the management of Behçet's disease. This glaucoma will be more difficult to manage in this delicate pathological context. It requires special vigilance in patients with severe posterior segment inflammation, to institute effective management to avoid its potentially blinding complications.

[Prognostic factors of ocular involvement in Behçet's disease]. / Facteurs pronostiques de l'atteinte oculaire dans la maladie de Behçet.

INTRODUCTION: Behçet's disease is an idiopathic systemic inflammatory disease. Ocular involvement is part of the classification criteria for this disease. The uveitis is potentially severe. The goal of our study was to study the prognostic factors associated with low final visual acuity (VA) of less than 1/10 during the follow-up of patients with Behçet's disease. MATERIALS AND METHODS: This is a retrospective study including all consecutive patients hospitalized for Behçet's disease from January 2009 to December 2017 on a university hospital service. The age of onset, initial visual acuity, ophthalmologic manifestations, therapeutic management and progression were recorded. The prognostic factors associated with a final AV less than 1/10 were subjected to univariate analysis. Variables with p less than 0.05 were then included in a multivariate analysis. RESULTS: Fifty patients were included. The mean age was 30, with a male predominance of 82 %. The univariate analysis showed that there was a statistically significant difference between the group of patients with a final VA less than 1/10 (n=28) and those with a final VA greater than 1/10 (n=22) with regard to young age at onset of the disease (95 % CI 0.80-0.94, P=0.001), high number of ocular recurrences (95 % CI 1.67-10, 12, P=0.002), and the presence of occlusive vasculitis (62.9 % vs. 21.4 %, P=0.002). Multivariate analysis confirmed that young age of onset of the disease and the number of recurrences were independent prognostic factors of final visual acuity less than 1/10. CONCLUSION: Severe uveitis is a progressive step in Behçet's disease which may compromise the functional prognosis of these patients. Young age at onset of the disease as well as a high number of recurrences appear to be major prognostic factors in these patients.

[Os odontoideum: clinical and radiological aspects]. / Os odontoïdeum : aspects cliniques et radiologiques.

Few reports of os odontoideum have been made. We report two cases where this affection was revealed by cervical pain and hemiparesis in one case and acute tetraparesis in the other. Patients with os odontoideum usually present with neurological signs, but some have only cervical pain and some others remain asymptomatic. Radiological exams, including radiograms, cervical scanner and MRI lead to the diagnosis. Different surgical treatment can be proposed to symptomatic patients. Prophylactic surgical treatment is not indicated.

[Infected cervical epidural Ewing's sarcoma (case report)]. / Sarcome d'Ewing épidural cervical infecté (à propos d'un cas).

INTRODUCTION: Ewing's sarcoma is a relatively rare osseous malignant tumour. The age of onset is generally in the youth. Epidural localisation of the malignant osseous tumour Ewing's sarcoma is exceptional. Only 29 cases were reported in surgical literature including only one infected case. OBSERVATION: We report a case of a 24 years old patient who displayed a complete flaccid tétraplégia with T1 sensitive level. Spinal cord MRI documented a cervical posterior epidural process with peripheric gadolinium enhancement. Patient was operated in emergency. A C6 and C7 laminectomy allowed complete tumour resection with its purulent intratumoural collection revealed during the operation. A total tumour removal was performed. The immediate postsurgical evolution was remarkable, with a quasi complete recovery in the upper limbs. DISCUSSION: Firstly described by James Ewing in 1921, the spinal epidural locations of the Ewing's sarcoma are rare without specific clinical signs generally presenting as a radiculo-medullary compression. Our case is the second infected epidural Ewing sarcoma reported in the literature. However the cause remains unexplained. For this spinal epidural extraosseous tumour, X-ray radiography failed to show any osseous lesions. On MRI, the usually found aspect is a T1 and T2 isosignal with gadolinium enhancement. CONCLUSION: Through our observation, we underlined the scarcity of this pathology in the cervical epidural localization and the association with infection. We also emphasized on the complete removal, in order to give the best functional and vital outcome.

[Double localization of a cerebral germinoma. Case report]. / Double localisation d'un germinome cérébral. A propos d'un cas.

A germinoma located in both the pineal and suprasellar regions remains rare. The clinical expression can be polymorphic, although brain imaging reveals specific signs. We report the case of a 16-year-old boy who presented with diabetes insipidus of six months' standing, associated with visual loss and Parinaud's syndrome. Brain MRI showed a tumor in both the pineal and suprasellar regions. A stereotaxic biopsy targeting the infundibular growth confirmed the diagnosis of ectopic germinoma, which was successfully treated by radiotherapy and chemotherapy. The incidence of bifocal germinoma, its clinical and radiological characteristics as well as the therapeutic strategies to adopt are discussed.

[Uveitis during the course of sarcoidosis in a child: difficult diagnosis of a case]. / Uvéite au cours de la sarcoidose chez l'enfant: difficultés diagnostiques à propos d'un cas.

Sarcoidosis is a multisystemic granulomatous disorder affecting young adults. It is very unfrequent in children. The authors report the case of an 11-year-old boy with a history of a pulmonary affection who was referred from the department of paediatrics for red eyes. Ocular examination disclosed phlyctenular keratoconjunctivitis, ocular dryness, posterior uveitis and choroidal granulomas confirmed by fluoresceinic angiography. The diagnosis of pulmonary, ganglionnary and ocular sarcoidosis was made. The authors recalled the clinical particularities of sarcoidosis in childhood and emphasized the difficulties of the differential diagnosis between sarcoidosis and tuberculosis.

[Ocular abnormalieis associated with cutaneous hyperelasticity and joint hypermobility. A report of three observations]. / Les atteintes oculaires associées a l'hyperélasticité cutanée et l'hyperlaxité ligamentaire. A propos de trois observations.

Connective tissues disorders are various and characterized by skin hyperelasticity, joint hypermobility and easy bruising. Ocular abnormalities predominate in type VI Ehlers-Danlos syndrome, Marfan disease, brittle cornea, Lobstein syndrome. We report three cases of patients with presumed Ehlers-Danlos syndrome. The diagnosis was clinical and could be confirmed by histological findings in two cases. We expose various ocular involvements in this syndrome and insist that biochemical tests are helpful to make the diagnosis.

[Multiple intracranial ganglioglioma. Case report]. / Gangliogliomes multiples intracrâniens. A propos d'un cas.

Multiple intracranial ganglioglioma (GG) is an extremely rare condition, generally associated with van Recklinghausen neurofibromatosis. We report the case of a 26-year-old woman who presented with progressive tetraparesis, raised intracranial pressure and visual loss. The neuroradiologic workup demonstrated a multiple lesion involving the temporal lobe, the diencephalomesencephalon and the optical pathways. Computed tomography-guided stereotactic biopsy of a right temporal lesion led to the diagnosis of GG. In light of this case and the literature review, we discuss morphologic aspects, differential diagnosis and therapeutic options of GG. In patients with multiple cerebral tumors, search for multiple ganglioglioma is particularly important because of the therapeutic implications and the good prognosis of this tumor.

[Wolfram syndrome: four case reports]. / Le syndrome de Wolfram: à propos de quatre cas.

Wolfram syndrome, also known as DIDMOAD syndrome, is a rare disease characterized by the association of diabetes mellitus, diabetes insipidus, optic atrophy and deafness. The ophthalmologic findings are largely dominated by optic atrophy. Through four cases and a literature review, the authors describe the ophthalmologic findings in this disease and its clinical and genetic aspects.

[Open globe injuries in children: retrospective study of 62 cases]. / Les plaies du globe oculaire de l'enfant: étude rétrospective de 62 cas.

The open globe injuries in children are frequent and serious accidents. The purpose of this study is to investigate the epidemiologic and clinical characteristics and the visual outcomes of this accident. A retrospective study was conducted on 62 cases in the Ophthalmology department between January 2001 and December 2005. The mean age of admission was 7.5 years. Fifty-one patients were males and twelve were females. Open globe injuries occurred most frequently in the street. The wounds involved the cornea in 66.1% of the cases. There was iris hernia in 30 cases, hyphema in 20 cases and traumatic cataract in 15 cases; in 8% of the cases a foreign body was detected. Final visual acuity was 0.5 or better in 28%. This finding emphasizes that educative measures should be taken into consideration in order to prevent this accident.

[Cranial vault chondroma: a case report and literature review]. / Chondrome de la convexité: revue de la littérature à propos d'un cas.

Intracranial chondromas are unusual tumors, which most commonly arise from cartilage rets in the synchondrosis at the base of the skull. They are most likely found in the sellar and parasellar regions, usually located extradurally. In rare instances, these tumors originate from the dura mater of the convexity. In these cases their neuroradiological features may mimic other intracranial tumors. We present the case of a 50-year-old male presenting a large parasagittal tumor originating in the frontal convexity. MRI-scans revealed a cavitated tumor strongly enhanced after gadolinium infusion mimicking a parasagittal meningioma. There was no obstruction of the superior sagittal sinus as shown by cerebral angiogram. Total excision was achieved and the postoperative course was uneventful. No complementary treatment was therefore considered. The histological examination diagnosis was chondroma. Radiological workup performed at four years follow-up did not reveal any recurrence of the lesion. Pathogenic, clinico-radiological and therapeutic issues are discussed and the literature reviewed.

[Endovascular treatment of idiopathic intracranial hypertension. Analysis of eight consecutive patients]. / Traitement endovasculaire de l'hypertension intracrânienne bénigne étiquetée "idiopathique". Analyse de huit cas consécutifs.

INTRODUCTION: Optic nerve sheath fenestration or ventricular shunting are classically proposed after failure of medical treatment. Idiopathic intracranial hypertension is caused by venous sinus obstruction in an unknown percentage of cases. Recently, endoluminal venous sinus stenting was proposed as an alternative treatment. PATIENTS AND METHOD: Between September 2003 and December 2004, eight consecutive patients with a diagnosis of idiopathic intracranial hypertension underwent MRI venography and direct retrograde cerebral angiography. RESULTS: There were five females and three males aged 22 to 55 years. All patients had vision disorders. The cerebrospinal fluid pressure ranged from 27 to 45 mmHg with normal composition. All patients presented at least one sinus stenosis. Endovascular stenting of the stenotic venous sinus was performed under intravenous heparin administration. Anti-platelet therapy was administered for 3 months post treatment. Intra-sinus pressures were invariably reduced by stenting. Mean follow-up was 18 months. All patients improved clinically. The cerebrospinal fluid pressure had normalized at 3-month follow-up in all patients. In all patients, multidetector row CT-angiography or MRI venography was performed at 3-, 6- and 12-month follow-up and demonstrated the patency of the stent. DISCUSSION: The importance of venous sinus disease as a cause of idiopathic intracranial hypertension is probably underestimated. Patients with idiopathic intracranial hypertension should be evaluated with direct retrograde cerebral venography and manometry. In patients with venous sinus lesions, treatment by an endoluminal venous sinus stent is a safe and effective alternative for amenable lesions.

[Prevalence of exfoliation syndrome and cardiovascular diseases in patients scheduled for cataract surgery]. / Prévalence du syndrome exfoliatif et profil cardiovasculaire des patients programmés pour chirurgie de cataracte.

PURPOSE: To study the prevalence of exfoliation syndrome (XFS) and its association with cardiovascular disease in patients scheduled for cataract surgery. MATERIAL AND METHODS: This is a retrospective, single center, descriptive study of 260 patients admitted to a tertiary teaching hospital in Rabat, Morocco, between January 2015 and March 2016 and scheduled for cataract surgery. All patients had a preoperative cardiovascular exam, an electrocardiogram and, if needed, echocardiography. Statistical analysis was performed using SPSS software. Results with a P-value<0.05 were considered statistically significant. RESULTS: This study included 246 patients: 146 women (59.3 %) and 100 men (40.7 %). The mean age (years) was 67.6±10.1. The percentage of XFS increased with age (P=0.02). Sixty-two percent of patients with cardiovascular disease also had exfoliation syndrome (P<0.001). Patients with XFS had hypertension in 46.5 % of cases. 14 subjects (19.7 %) had ischemic heart disease and XFS. Using multivariate logistic regression and after adjusting for age, diabetes mellitus, hypertension, ischemic heart disease and arrhythmias, smoking was the only factor associated with exfoliation syndrome (P=0.01, odds ratio=5.2, confidence interval 95 %=1.35-20.15). CONCLUSION: Exfoliation syndrome is a common condition in the aging cataract population. Ischemic heart disease and hypertension are frequently associated with XFS. Smoking could be another factor associated with this syndrome.

[Prognostic factors in uveitis]. / Facteurs pronostiques des uvéites.

PURPOSE: To evaluate prognostic factors of vision loss among patients with uveitis. MATERIAL AND METHODS: This descriptive and retrospective study included all patients diagnosed with uveitis who were seen at the teaching hospital of Rabat, Morocco, over a 5-year period. Information regarding demographic data, uveitis type, bilaterality of the disease, etiology and complications were gathered from patients' records. Statistical analysis was performed using SPSS software. RESULTS: One hundred and thirty-nine eyes of 89 patients were included. There were 60.7 % men and 39.3 % women. The mean age (years) was 31.1±16.8. Median follow-up was 11 months. Median visual acuity (logMAR) on admission was 1.7 [0.7-2] and 1 [0.4-1.7] on the last visit (P<0.001). Fifty-nine percent of eyes had final visual acuity (VA) equal to or less than 1/10. Panuveitis (85.4 %) and posterior uveitis (72.7 %) were responsible for final VA equal to or less than 1/10 (P<0.001). Cataract, posterior synechiae, vitreous opacities, epimacular membrane (ERM) and cystoid macular edema (CME) were the most frequent complications. Using multivariate logistic regression, the prognostic factors associated with severe vision loss were the type of uveitis (posterior and panuveitis), CME, ERM, macular scarring and optic atrophy (P<0.05). CONCLUSION: Although this study included a limited number of subjects, the results showed that final VA was equal or less than 1/10 in 59 % of eyes diagnosed with uveitis and was associated with risk factors including posterior segment involvement (panuveitis and posterior uveitis), CME and ERM.

[Late cervicodorsal metastasis of thyroid adenocarcinoma treated by anterior cervicotomy]. / Métastase cervico-dorsale tardive d'un adénocarcinome thyroïdien opérée par cervicotomie antérieure.

Metastasis from a thyroid adenocarcinoma is a rare entity with high mortality. We describe an unusual case in a woman displaying tetraparesis due to a late cervico-dorsal metastasis operated on via a simple anterior cervicotomy. Outcome was good at eighteen months follow-up. The histological diagnosis was follicular adenocarcinoma. The development of a vertebral metastasis from a thyroid adenocarcinoma 11 years after the treatment of the primitive cancer is rare. The anterior cervico-dorsal location in this patient required decompression via an anterior approach. Surgical treatment of the tumor and spinal stabilization were possible via a simple anterior cervicotomy which allowed adequate access to the third thoracic vertebra, without sternotomy or thoracotomy.

[T-cell acute lymphoblastic leukemia /orbital lymphoblastic lymphoma in children]. / Leucémie aiguë lymphoblastique T/Lymphome lymphoblastique orbitaire chez l'enfant.

CASE: The authors report a case of an 6-year-old pediatric patient with a history of acute onset of proptosis of his right eye. He was admitted at hospital 6 months ago for proptosis concomitant with orbital trauma. Computed tomography scan demonstrated a mass involving the right orbit, right maxillary sinus, and zygoma with endocranial extension. RESULTS: Incisional biopsy of the mass revealed after of histopathologic and immuno-histochemical evaluation a T-cell lymphoblastic lymphoma. Systemic examination and bone marrow aspirate show a acute lymphoblastic leukemia. The patient was treated with LMT96 Protocol. A complete Remission was observed after 13 months of follow up. CONCLUSION: Primary T-cell lymphoblastic lymphoma of the orbit is a rare entity in any age group, but it is very rare in children. When tumors occurs in the orbit, it presents a challenging diagnosis problem, especially in pediatric patients.

[Bilateral uveitis in relapsing polychondritis. A case report]. / Uvéite bilatérale au cours d'une polychondrite atrophiante. A propos d'une observation.

We report a rare ocular manifestation of relapsing polychondritis. A 28-year-old woman presented with a 5-year history of relapsing polychondritis with chondritis of the nose, ears and tracheobronchial system. The ocular symptoms were bilateral uveitis with macular involvement and papillaedema. Ocular manifestations of relapsing polychondritis occur in 60% of patients. Uveitis is a rare symptom, sometimes severe, which can lead to blindness. This case report with a literature review sheds light on the features of this disease.

[Persistent hyperplastic primary vitreous and Aicardi syndrome]. / Persistance et hyperplasie du vitré primitif et syndrome d'Aicardi.

INTRODUCTION: Aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum and chorioretinal lacunae. This disorder affects mostly females, with early embryonic lethality in males. Numerous general and ocular disorders may be associated with this affection. We present here a case of persistent hyperplastic primary vitreous (PHPV) in association with Aicardi syndrome in a 30-year-old woman. CASE REPORT: The authors report a case of a 30-year-old woman with Aicardi syndrome associated with persistent hyperplastic primary vitreous. DISCUSSION: Aicardi syndrome is a polymalformative disease occurring at an early period of embryogenesis. It can affect many ocular structures. This syndrome is essentially described in female children, who rarely reach an adult age. The observation we report is particular because of the patient's age (30-years-old) and the association with a persistent hyperplastic primary vitreous, exceptional in this context. CONCLUSION: With a review of the literature, the Authors discuss the clinical neuroradiological and prognostic aspects of this polymalformative syndrome and different associated general and ocular abnormalities, emphasizing particularly those of persistent hyperplastic primary vitreous in this affection.