[Pseudoexfoliation syndrome in Moroccan patients with cataract scheduled for sugery]. / Le syndrome pseudo-exfoliatif chez les patients marocains programmés pour chirurgie de la cataracte.

PURPOSE: To evaluate the prevalence of pseudoexfoliation syndrome (PES) in Moroccan patients with age-related cataract scheduled for surgery. PATIENTS AND METHODS: In a retrospective study, 837 consecutive eyes with age-related cataract scheduled for surgery were evaluated for the prevalence of PES, type of cataract, intraocular pressure (IOP) and operative complications. RESULTS: Exfoliation material was detected in 82 patients (9.8 %). The mean age was 69.3 years with no sex predilection. The condition was unilateral in 44 cases (53.7%) and bilateral in 38 cases (46.3%). The prevalence of PES increased with age (p=0.02). Patients with hypermature cataract had significantly more PES (p <0.001). Mean IOP was significantly higher in eyes with PES (17.7 +/- 6.4 mmHg) than those without PES (14.3 +/- 2.9 mmHg) (p<0.001). Operative complications occurred more frequently in patients with PES: 14 (17 %) than in those without PES: 58 (6.9 %). DISCUSSION: PES is a generalized degenerative fibrillopathy bound to age. The ethiopathogeny remains even unknown. The epidemiological data concerning the prevalence of the SPE in the African populations are scarce, and have not been published in Morocco, what motivated this study. Our study shows that this syndrome is common in Moroccan patients scheduled for cataract surgery with a prevalence of 9.8%. Its presence constitutes an important risk factor of primary open-angle glaucoma, and exposes more to peroperative complications at the time of surgery of cataract, independently of the technique used. CONCLUSION: There is great variation in the prevalence of PES among people of different regions and ethnic groups, although many factors interfere in the comparative analysis of results, hence the interest to achieve other complementary epidemiological studies.

Orbital osteoma: three case reports.

INTRODUCTION: Osteoma is the most frequent benign tumor of paranasal sinuses. It is generally asymptomatic and usually discovered by chance during radiological imaging. Three cases with orbital extension are reported and discussed. OBSERVATIONS: 1st observation: Mrs. M.N. is an 18 years-old caucasian female who presented a stony orbital deformity associated with chronical dacryocystitis. Tomography of the orbit revealed a probable ethmoidal osteoma compressing the lacrimal canals. The management consisted in a surgical excision of the whole tumor by external approach, associated with a dacryocystorhinostomy. The patient's follow-up for the last 12 months was normal. 2nd observation: Miss K.A. is a 16 years-old caucasian female who came to consultation for a swelling of the medial angle of the left eye. Computed tomography images showed a fronto-ethmoidal process displaying a bone density consistent with an osteoma. The tumor was removed through an external ethmoidectomy. The patient was free of symptoms at 6 months follow-up. 3rd observation: Mrs. F. Z. is a 45 years-old patient who presented a 5 years history of right painful exophthalmos. The computed tomography was consistent with a fronto-ethmoidal osteoma with intraorbital extension near the optic nerve. The surgical excision was limited to the intraorbital portion. Postoperative complications included ptosis and diplopia. DISCUSSION: Osteomas most commonly affect the fronto-ethmoid sinuses. They rarely show intraorbital extension or cause intracranial complications. They are generally asymptomatic. Symptoms are generally of late onset and are a consequence of tumoral growth and compression of neighbouring organs, as it is the case in our patients. Tumoral exophtalmos is the major ophthalmological sign. The computed tomography is of a great contribution, not only for diagnosis but also for the choice of the surgical approach. The classical surgical technique consists generally in a surgical excision of the osteoma. This surgery may induce ocular or neurochirurgical complications. CONCLUSION: Osteomas of the paranasal sinuses are usually asymptomatic. If they become voluminous, they may cause orbital manifestations and serious complications. The rarity of ethmoidal osteoma with orbital growth made our cases interesting to report.

[Tyrosinemia type II. Case report]. / La tyrosinemie type II. A propos d'un cas.

Tyrosinemia type II or Richner-Hanhart syndrome is a rare hereditary disease characterized by the association of pseudoherpetiform corneal ulcerations and palmoplantar hyperkeratosis. We report the case of a 12 year-old young man presenting a superficial punctate keratitis and a corneal dystrophy in both eyes, associated with a palmoplantar hyperkeratosis. The dosage of the serum level of tyrosine is meaningfully raised to 1236 micromol/l. A dietary treatment restraining tyrosine and phenylalanine is started with favorable results after an evolution of 6 months. Tyrosinemia type II is an autosomal recessive disease, due to an enzymatic deficit in tyrosine aminotransferase. The diagnosis is based on the clinic and high level of serum and urinary tyrosine as well as of its urinary metabolites. This disease must be suspected in all cases of dentritic keratitis not reacting on the antiviral treatment, and more especially if it is associated with cutaneous lesions such as palmo-plantar keratosis.

[Bourneville's tuberous sclerosis. A case report]. / La sclérose tubéreuse de Bourneville. A propos d'un cas.

INTRODUCTION: Bourneville's tuberous sclerosis (BTS) is an autosomal dominant phakomatosis characterized by the development of a benign hamartoma-like tumor, which is usually located in the skin, kidney, heart, brain, and eyes. We present here a case of a retinal BTS of late diagnosis. We also discuss the clinical course and progress of this condition. OBSERVATION: A 28-year-old man was a known BTS patient followed in the dermatology clinic. The ophthalmologic exam and the angiography with fluorescein revealed retinal astrocytic hamartomas bilaterally. Dermatologic examination also showed facial angiofibromas. The remainder of the physical examination was unremarkable. The chest X-ray, renal scan, heart scan and a CT scan of the brain failed to show any other localizations of the disease. The lesions described had remained unchanged over a period of 6 years. CONCLUSION: Retinal lesions during BTS are dominated by the presence of astrocytic hamartomas essentially around the papillae. They are often an incidental diagnosis and evolve slowly. Except in complicated cases, their prognosis is usually satisfactory.