Mounir Lezrek

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.

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.

Le syndrome d’Usher: à propos d’une observation

Usher syndrome is a genetic disease resulting in double sensory deprivation (auditory and visual) called deafblindness. We report the case of a 50-year old patient, born to consanguineous parents, presenting with congenital deafness associated with normal vestibular function and pigmentary retinopathy responsible for decreased bilateral visual acuity occurred at the age of 16 years. This association composes Usher syndrome type 2, a rare autosomal recessive disorder. Cataract surgery allowed visual acuity improvement in this patient.

Acute traumatic maculopathy.

A 14-year old child presented to the emergency department (ED), complaining of blurry vision 1 hour after he was victim of a blunt trauma of his right eye. Initially, visual acuity was Counting Fingers at 2m and 20/20 in the left eye, Slit lamp examination was normal; Funduscopy of the right eye revealed gray-white discoloration of macula and lower retina in posterior pole (A); Optical coherence tomography showed an increase in reflectivity of the inner and outer segment junction (B); 1 week later, the visual acuity improved to 20/25 in the right eye without any treatment.