[Conjunctival melanoma and xeroderma pigmentosum, a rare association]. / Mélanome de la conjonctive et xeroderma pigmentosum, une rare association.

Ocular involvement in the xeroderma pigmentosum (XP) can be located on the eyelids, conjunctiva or cornea. Malignant melanoma of the conjunctiva is a rare tumor, and even more so in children. Only five cases associated with XP have been published since 1960. We report a sixth case occurring in a girl aged 14. Surgical treatment consisted of an orbital exenteration extended to the eyelids and the evolution is marked by a good healing of the orbital cavity without local recurrence after four months.

[Tolerance of porous polyethylene orbital implants in children]. / Tolérance des implants orbitaires en polyéthylène poreux chez l'enfant.

INTRODUCTION: The purpose of our study is to determine the incidence of orbital complications that occurred after insertion of orbital porous polyethylene implant in children under the age of 15 years. MATERIAL AND METHODS: We report a series of 21 eyes of 21 patients younger than 15 years and in which a porous polyethylene implant is used for reconstruction of the orbital cavity after enucleation between January 2003 and December 2008.All patients were operated on by the same surgeon using the same technique. RESULTS: These 11 boys and 10 girls, whose average age is 5.7 years. Histopathologic diagnoses after enucleation are dominated by the retinoblastoma (10 eyes) and phthisis bulbi (6 eyes). After a mean follow up of 23 months it was observed two cases of implant extrusion in children enucleated for retinoblastoma. No cases of orbital cellulitis or enucleated syndrome have been reported. DISCUSSION: The most common complication of porous polyethylene implants in children is exposure. Risk factors may be related to surgical technique, infection, the implant, use of wrapping material and the association with adjuvant chemotherapy. Using a porous polyethylene implant uncovered remains an appropriate technique in children under 15 years for the reconstruction of the anophthalmic cavity, provided a rigorous surgical technique.

Hyphema revealing retinoblastoma in childhood. A case report.

We present a case dealing with an uncommon presentation of retinoblastoma. An 4-year-old boy presented to the ophthalmic department for a red painful eye following trauma. The examination showed decreased visual acuity, total hyphema and ocular hypertony. Ocular ultrasonography revealed an intraocular process. CT-scan of the orbit was consistent with a retinoblastoma. Treatment consisted of an enucleation and chemotherapy. This paper stresses the fact that presentation of retinoblastoma is not stereotypic. Every effort should be made to exclude a tumoral intraocular process in all cases of hyphema, even in cases of well documented ocular trauma.

[Post electrocution cataract. Case report]. / La cataracte post-electrocution. A propos d'un cas.

INTRODUCTION: Cataract caused by high tension electric trauma remains a rare entity. It is often bilateral and late-onset especially if the electric current passes through the head. PATIENTS AND METHODS: We report a case of a bilateral cataract secondary to high tension electric trauma. RESULTS: A 13-year-old child presented with bilateral cataract secondary to high tension electric trauma (22 000sVolts) two years earlier accidentally on the railroad. The electric current has crossed the head and both upper limbs. This had resulted in immediate burns on the right scalp and hand. Ophthalmological examination found a total white cataract in the RE and anterior subcapsular lens opacities in the LE. CONCLUSION: Cataract is a rare but serious complication of severe electric trauma requiring regular and prolonged eye monitoring owing to its possible late occurrence.

[Treatment of choroidal noevascularization secondary to angioid streaks with bevacizumab and response: Experience of the ophthalmology department of Hassan II university hospital]. / Traitement par bévacizumab et évolution des néovaisseaux choroïdiens compliquant les stries angioïdes : expérience du service d'ophtalmologie du CHU Hassan II.

PURPOSE: To evaluate the efficacy of intravitreal bevacizumab in the treatment of choroidal neovascularization (CNV) secondary to angioid streaks. METHODS: We conducted a prospective study in the ophthalmology department of Hassan II university hospital in Fez, including 12 eyes of 7 patients with CNV associated with angioid streaks. We injected 8 eyes with bevacizumab 1.25mg; best corrected visual acuity (BCVA), fundus examination, fluorescein angiography and optical coherence tomography were performed and analyzed for all patients before and after treatment. The goal of the study was to determine the percentage of patients with stabilization or improvement in BCVA and decreased central macular thickness. RESULTS: Visual acuity on admission was <1/10 in 75% of patients. We injected 8 eyes with anti-VEGF, whereas abstention was appropriate for 4 eyes with disciform scarring. The average number of bevacizumab injections was 4.3±1.3 over a mean follow-up of 14.57 months±5.3. After treatment, BCVA was <1/10 in 12.5% of cases, between 1/10 and 5/10 in 50% of cases, and in 37.5% of patients, it was ≥5/10. Mean central macular thickness decreased from 424.25±137.03µm on admission to 255.75 microns±50.14 post-treatment (P=0.005). CONCLUSION: Intravitreal bevacizumab is a promising and effective treatment option for the management of CNV associated with angioid streaks, with the requirement however of early treatment and extended follow-up.

[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.

[Screening for diabetic retinopathy by non-mydriatic retinal camera in the region of Fez]. / Dépistage de la rétinopathie diabétique par un rétinographe non mydriatique dans la région de Fès.

PURPOSE: The goal of our study was to determine the benefits of screening by non-mydriatic retinal camera in the Fez region and to present the results of this screening. PATIENTS AND METHODS: We report a prospective study of 430 diabetic patients, or 860 eyes, compiled between December 2012 and June 2013 in the Dokkarat health center, "integrated referral center for management of diabetes and hypertension" in the region of Fez. All patients underwent an examination by non-mydriatic fundus camera, obtaining two digital images per eye. The photos are taken by a nurse and a general practitioner. Interpretation of the images was performed in the ophthalmology department of the Hassan II University Medical Center of Fez. RESULTS: A total of 430 patients were screened (78.4% female, 21.6% male). The mean age was 54.7 years (range, 10-80 years). The average duration of diabetes was 7.7 years (±5.9). Most patients had type 2 diabetes (94%). Insulin therapy was found in 35.3%. The mean HbA1c (obtained from 421 patients) was 8.21%. 38,8% of patients had never consulted an ophthalmologist before. The fundus photographs were not interpretable for at least one eye in 75 cases (17.4%). Among the interpretable images, the prevalence of diabetic retinopathy (DR) was 44.6%. Proliferative diabetic retinopathy was present in at least one eye in 2.5% and diabetic maculopathy in 32 cases (9%). After screening, 69% of patients with DR were seen by the ophthalmologist. CONCLUSION: These data confirm the benefits of screening by non-mydriatic retinal camera, because it helps reintegrate patients into the healthcare system and thus provides a real benefit in terms of public health and prevention of complications.

[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.

[Marcus-Gunn Syndrome. Based on a case report]. / Syndrome de Marcus-Gunn. A propos d'un cas.

The Marcus-Gunn syndrome associates an unilateral congenital blepharoptosis and "jaw-winking" synkinesia. We report a 12-year-old girl presenting an unilateral Marcus-Gunn syndrome and discuss the clinic, pathogenesis and treatment of this syndrome.

[Enucleations: epidemiologic investigation in Morocco. presentation of 183 cases]. / Enucléations: enquêté épidémiologique marocaine. A propos de 183 cas.

INTRODUCTION: The authors report the results of an epidemiological study concerning 183 enucleated eyeballs. MATERIAL AND METHODS: [corrected] The study is realized over a 12-year period (1988-2000) in the department of Ophthalmology "B" (University Hospital--Rabat) on 183 enucleated eyes, only 90% having an histological examination. RESULTS: The aetiologies are: trauma (40%), malignant tumours (30%), atrophies and glaucoma (17%), panophthalmitis (9%) and corneal lesions (4%). The authors compare their results with those of the literature. CONCLUSION: The causes of enucleation are the same throughout the world. The frequency has lately decreased due to the development of early diagnosis and to the use of more conservative treatments.

[The eye and hepatitis C]. / Oeil et hépatite C.

Ocular diseases such as Sjögren's syndrome, Mooren's ulcer and acute retinal pigment epitheliitis have been associated with hepatitis C. We report the case of a 35-year-old male nurse with hepatitis C who developed Sjögren's syndrome which was confirmed by labial biopsy. The role of hepatitis C infection in Sjögren's syndrome was recently established, whereas in the two other diseases, its role remains obscure. Although the presence of the hepatitis C virus in the cornea is still incertain, hepatitis C serology in donor's is required before cornea transplantation can be undertaken.

[Spinal aneurysmal cyst]. / Kyste anévrysmal rachidien.

The authors report one case of aneurysmal rachidian cyst who concern a young girl of eleven years old presenting lumbagos with pyramidal irritation on a L2 and L3 lesion. The radiological exploration (CT, MRI) has a double interest: to find again lytical pictures of the rear arch blowing the cortical, liquids levels, logets which remind the macroscopical appearance of the aneurysmal rachidian cyst; to specify the regional extension of preoperative interest. The histological confirmation of the aneurysmal rachidian cyst after a surgical treatment and the conformity of anatomo-radiological descriptions, confirm the predictive value of the semeiology described in case of aneurysmal rachidian cyst.

[Scleral buckling for inferior rhegmatogenous retinal detachments]. / Les décollements de rétine rhegmatogènes inférieurs traités par cryo-indentation.

PURPOSE: To investigate the epidemiological, clinical, therapeutic and prognostic factors in cases of inferior rhegmatogenous retinal detachments (RD) treated by scleral buckling surgery. PATIENTS AND METHODS: A retrospective chart review was performed on 45 patients (45 eyes) with inferior RD with only inferior tears (4:00-8:00), who had been treated by scleral buckling surgery over a 6-year period from 2006 to 2011. The parameters studied included patient demographics, refractive status, time until consultation, clinical exam data, treatment modalities and functional and anatomic results. RESULTS: Forty-five cases were included in this study (45 eyes), with an average patient age of 44.5 years (14 to 75 years) and a slight male predominance (56%). Myopia was observed in 60%. Mean time until consultation was 3.5 months. Visual acuity on admission was less than 1/10 in 53.33%. Macular detachment was found in 80%. Causative lesions were holes in 26 eyes. Proliferative vitreoretinopathy was essentially stage B in 48.9%. Scleral buckling surgery was performed in all patients, with drainage of subretinal fluid in 37.8%. Retinal reattachment was obtained in 36 eyes (80%) with a final visual acuity greater than or equal to 1/10 in 71.11%. The mean follow-up in our study was 6.62 months. CONCLUSION: Inferior retinal detachment has a predilection for young myopes. The time until consultation is often long, and extraocular surgery, although difficult, exhibits documented efficacy.

[Orbital location of multivisceral Langerhans-cell histiocytosis]. / Localisation orbitaire de l'histiocytose langerhansienne dans sa forme multiviscérale.

Langerhans-cell histiocytosis is a rare disease accounting for less than 1% of orbital tumours. We report the case of an 18-month-old infant presenting with exophthalmos of the left eye lasting for 3 months in a context of deterioration of the general condition, polydipsia, and polyuria. The computed tomographic scan revealed an orbital cellular mass with a lytic bone lesion within the orbital roof and infiltration of frontopalpebral soft tissue. In addition, other secondary lesions were found at the skull, ribs, skin, liver, and spleen. The cytological study after biopsy showed infiltrates of histiocytes derived from Langerhans cells. Because the disease was multivisceral, the child was treated with chemotherapy (vinblastine) combined with a steroid and progressed well. At 30 months of follow-up, there was no evidence of recurrence or systemic involvement.