Rajae Daoudi

Persistance et hyperplasie du vitré primitif et syndrome d’Aicardi

Introduction Le syndrome d’Aicardi est caracterise par une triade symptomatique faite de spasmes en flexion, une agenesie du corps calleux et des lacunes chorioretiniennes. Cette maladie affecte les individus de sexe feminin avec une letalite precoce chez les individus de sexe masculin. Plusieurs anomalies generales et oculaires ont ete decrites en association avec cette affection. Ce tableau polymalformatif peut exceptionnellement comprendre un tableau de persistance et d’hyperplasie du vitre primitif. Observation Les auteurs rapportent l’observation d’une jeune femme, âgee de 30 ans, atteinte de syndrome d’Aicardi associe a un tableau de persistance et d’hyperplasie du vitre primitif. Discussion Le syndrome d’Aicardi est un tableau polymalformatif survenant a une periode precoce de l’embryogenese pouvant interesser les structures oculaires. Il est decrit essentiellement chez des enfants de sexe feminin qui atteignent rarement l’âge adulte. L’observation que nous decrivons se distingue par plusieurs elements dont l’âge de la patiente (30 ans) et l’association a un tableau de persistance et d’hyperplasie du vitre primitif qui est exceptionnellement decrit dans ce cadre. Conclusion A la lumiere des donnees de la litterature, les auteurs discutent les aspects cliniques, neuroradiologiques et pronostiques de ce syndrome polymalformatif, de meme que les differentes anomalies generales et oculaires associees en soulignant particulierement ceux de la persistance et d’hyperplasie du vitre primitif dans ce contexte.

Aniridie congénitale bilatérale familiale : à propos de 5 cas

Introduction L’aniridie congenitale est une affection rare se manifestant essentiellement par une hypoplasie de l’iris pouvant s’associer a d’autres desordres oculaires (glaucome, dystrophie de cornee, cataracte congenitale, ectopie du cristallin, hypoplasie maculaire et du nerf optique) et systemiques notamment urogenitales. Les auteurs rapportent cinq cas familiaux d’aniridie congenitale bilaterale. Observations Le pere, âge de 44 ans, presente de maniere bilaterale une dystrophie de cornee, une hypoplasie irienne et une cataracte totale. L’examen de sa fille, âgee de 12 ans, montre une acuite visuelle limitee a « voir bouger les doigts » en rapport avec une dystrophie de cornee importante et une cataracte congenitale bilaterale. L’examen de deux autres filles âgees de 18 et de 23 ans ainsi que de leur frere âge de 11 ans note une acuite visuelle basse et une ectopie du cristallin bilaterale. Le bilan general de cette famille ne montre pas d’anomalie extraoculaire. Trois patients sont operes de leur cataracte sans implantation intraoculaire, avec des suites favorables. Discussion Ces observations illustrent les differents aspects cliniques de l’atteinte oculaire au cours de l’aniridie congenitale de meme que les modalites therapeutiques, et notamment les difficultes de la chirurgie de la cataracte et de la correction de l’aphaquie au cours de cette affection. Conclusion L’aniridie est une maladie genetique qui s’accompagne d’autres atteintes oculaires et extra-oculaires compromettant le pronostic visuel voire vital, ce qui pose autant de problemes de prise en charge d’ou l’interet du conseil genetique.

Corps étranger intra-orbitaire végétal : à propos d’un cas

Un enfant de 12 ans fut victime d’un traumatisme orbitaire a gauche provoque par un morceau de bois. Il consulta 4 mois plus tard pour une cellulite orbitaire associee a une fistule cutanee. Le scanner montrait la presence d’un corps etranger orbitaire gauche etendu au sinus caverneux homolateral et en intracrânien. Une extraction du fragment de bois associee a un traitement antibiotique adapte a permis une amelioration clinique sans recuperation visuelle. La notion de traumatisme orbitaire et un syndrome inflammatoire orbitaire secondaire doivent faire suspecter la presence d’un corps etranger intraoculaire et motiver la realisation d’examens d’imagerie en urgence pour une prise en charge optimale.

Fibres à myéline péripapillaires étendues, myopie forte ipsilatérale et amblyopie réfractaire

Introduction Les fibres a myeline peripapillaires etendues peuvent s’associer a une anisometropie myopique avec amblyopie. Malgre l’occlusion intensive du bon œil, les resultats visuels sont souvent decevants, probablement en raison de l’existence d’anomalies structurales au niveau de la macula qui limitent le potentiel visuel. Observation Nous rapportons le cas d’une fillette âgee de 5 ans presentant des fibres a myeline peripapillaires etendues associees a une anisometropie myopique, et dont l’amblyopie s’est revelee refractaire a la correction optique et a une occlusion continue de 9 semaines du bon œil. Discussion Certains auteurs font etat de l’obtention de bons resultats avec un traitement agressif de l’amblyopie associee aux fibres a myeline peripapillaires etendues et a l’anisometropie myopique. Cependant, la majorite des rares series publiees dans la litterature rapportent que l’amblyopie liee a cette entite pathologique est particulierement refractaire au traitement comme le confirme le cas clinique presente. Conclusion Malgre le rapport de certains cas a evolution favorable dans le cadre de l’association des fibres a myeline peripapillaires/myopie ipsilaterale, nous pensons que le pronostic visuel de cette entite est reserve et que les patients et/ou leur famille doivent beneficier d’un conseil approprie.

Anterior Dislocation of the Lens

A 30-year-old man presented with sudden, painful vision impairment in his right eye 1 hour after vigorous exercise (long jump). He reported no direct ocular trauma.

Atypical Presentation of Ectopia Lentis in Homocystinuria

A 6-year-old boy presented with a history of intermittent strabismus since the age of 2 years. On examination, there was a bilateral displacement (ectopia) of the lense to the nasal and superior quadrants with a marked bilateral phacodonesis (tremulousness of the lens during eye movement) due to rupture of the zonular fibers in the inferotemporal quadrants holding the crystalline lens in place (Figure). General examination revealed a marfanoid morphotype with developmental delay, which was suggestive of a homocystinuria. This was confirmed by plasma and urine screening tests for homocysteine. The patient underwent bilateral phacophagia (surgical extraction of the lenses) and anterior vitrectomy. Iris fixated intraocular lenses were inplanted to manage the residual aphakia (absence of the crystalline lenses). Pyridoxine (vitamin B6) supplementation was started, along with a low-methionine and high-cysteine diet. Homocystinuria is an inborn error of methionine metabolism related to a deficiency of the cystathionine-bsynthetase, which converts homocysteine to cystathionine. Clinical features of this condition are related to the accumulation of the homocysteine and deficiency of cystine. The ocular manifestations are dominated by myopia and ectopia lentis. The latter is present in 90% of patients; it is an acquired and progressive abnormality. The suspensory system of the lens (zonula) contains high levels of cystine; thus, a deficiency of this component leads to an abnormal development of the zonular fibers, which alters the stability of the lens. This may lead, long-term, to a complete dislocation of the lens either to the anterior chamber or the vitreous, requiring a surgical extraction.

Cilioretinal artery and central retinal vein occlusion after sildenafil use

Its reported that sildenafil may cause non-arteritic anterior ischaemic optic neuropathy and transient changes in perception of colour hue or brightness.1 We report a case of cilioretinal artery and central retinal vein occlusion after sildenafil use. A previously healthy 40-year-old man, presented with acute visual loss of the left eye. He took one tablet of sildenafil (100 mg) for two consecutive days before the onset of symptoms. At examination the visual acuity was …

Acute fulminant endophthalmitis complicating traditional lens couching

A 76-year-old woman presented to the department of ophthalmology in February, 2014, with painful unilateral exophthalmos, 5 days after undergoing couching of the right cataractous lens by a traditional healer. The following day her eye had become red and painful with reduced vision. On examination visual acuity in the right eye was light perception, the intraocular pressure was high (45 mm Hg; normal 10–20 mm Hg), and there was proptosis and ophthalmoplegia. Biomicroscopic exam ination showed eyelid oedema, conjunctival injection, total chemosis, abundant purulent secretions, and a greenish hypopyon occupying the anterior chamber, typical of pseudomonas infection (fi gure). CT showed preseptal and retroseptal cellulitis, exophthalmos grade II, and a dislocated cataractous lens in the lower vitreous. We took vitreous and aqueous humour samples and gave one injection of intravitreal antibiotics (vancomycin 1 mg/0·1 mL and ceftazidime 2·25 mg/0·1 mL). We started systemic antibiotics (intravenous ceftriaxone 1 g twice daily and levofl oxacin 500 mg twice daily) and a systemic hypotonic (oral acetazolamide 250 mg twice daily). 48 h later we gave a second dose of intravitreal antibiotics and started methylprednisolone 40 mg twice daily for 5 days to reduce the infl ammation. Vitreous cultures grew Pseudomonas aeruginosa. By the seventh day the patient’s pain, eyelid oedema, and proptosis had resolved (fi gure), but she had a persistent vitreous opacity. We did pars plana vitrectomy and iris-claw intraocular lens on the posterior side of the iris to compensate for the refractive disorder caused by absence of the crystalline lens. At last follow-up in May, 2014, her best corrected visual acuity was 6/60 in the right eye and intraocular pressure had returned to normal (16 mm Hg). Lens couching is the oldest technique of cataract surgery. It was fi rst practised by the Assyrians of Hammurabi in the 17th century BC, and then by the Hindu surgeon Sushruta around 600 BC. It consists of traumatising the zonula through the sclera using a pen so the lens falls into the vitreous and releases the visual axis, allowing the patient to see the light. The visual outcome of this technique, unfortunately still prevalent in some parts of the world, is often poor. Its complications are retinal detachment, secondary glaucoma, hyphaema, uveitis, optic atrophy, and endophthalmitis.

Secondary hemorrhage in traumatic hyphema

A 40-year-old man presented with 3 h history of acute painful vision loss of his right eye. He reported a blunt trauma of the same eye 7 days prior to admission. At examination his visual acuity was reduced to light perception. Slit lamp examination revealed a multilayered hyphema (collection of blood in the anterior chamber). A layer of fresh blood (empty arrows) was noted over the darker clot (white arrows) in the anterior chamber (Figure 1). The intraocular pressure (IOP) was increased (40 mmgh).

Unilateral bullous exudative retinal detachment in Behcet's disease.

We report an unusual case of Behcet disease presenting with retinal vasculitis and unilateral exudative retinal detachment. A 24 years old man presented with 1 week history of acute vision loss of both eyes. He reported a 3 years history of recurrent oral and genital ulcerations, with arthralgia of wrists and ankles. The best correct visual acuity was 6/30 in the right eye and 6/24 in the left eye. Slit lamp examination showed bilateral mild inflammation in the anterior chamber and vitreous. Funduscopy revealed bilateral extensive perivascular sheathing with scattered yellowish retinal infiltrates and hemorrhages. In the right eye there was, in addition, a bullous exudative retinal detachment involving the superior and inferior quadrants of the temporal retina (a, white arrows). Fluoresce in angiography (b) showed late staining of the retinal vasculature with diffuse dye leakage in both eyes. In the right eye, late frames showed an evident fluorescein pooling in the subretinal space (black arrows). The patient received intravenous bolus of methylprednisolone (1 g daily / 3days) leading to prompt clinical improvement. Azathioprine was also started at a dose of 150 mg daily. Exudative retinal detachment is an uncommon finding in Behcets disease, and only few cases were reported in the medical literature. This entity could reflect the severity of the underlying disease. In addition the early set of the disease and male gender are usually associated with poor long term prognosis. This justifies our choice of giving azathioprine as an immunosuppressive therapy.