[Birth-related retinal hemorrhages in the newborn: incidence and relationship with maternal, obstetric and neonatal factors. Prospective study of 2,031 cases]. / Hémorragies rétiniennes liées à l'accouchement chez le nouveau-né : fréquence et relation avec les facteurs maternels, néonataux et obstétricaux. Étude prospective de 2031 cas.

PURPOSE: To assess the prevalence, morphology and distribution of retinal hemorrhages in healthy newborns and their relationship to neonatal, maternal and obstetrical factors, and to determine their natural history. PATIENTS AND METHODS: The present study prospectively included 2,031 consecutive healthy newborns. Indirect ophthalmoscopy was performed within 24 hours after birth in all newborns. Infants with retinal hemorrhages were reexamined weekly until the hemorrhage resolved. Annual ophthalmologic follow-up was also scheduled in these children. Neonatal, maternal and obstetric parameters were analyzed in all newborns and compared between newborns with retinal hemorrhages and those without retinal hemorrhages. RESULTS: 31.8 % of newborns exhibited retinal hemorrhages. 72.6 % of hemorrhages were bilateral. They tended to be localized around the optic discs and in the posterior pole, but their distribution was variable. Retinal hemorrhages were of variable shapes. The prevalence of retinal hemorrhages was higher in newborns delivered with vacuum-assisted extraction (38 %, P<0.001), intermediate during normal vaginal delivery (32.6 %, P<0.001) and lower with cesarean section (20.8 %). Comparative analysis between elective cesarean section and emergency cesarean showed a higher incidence of retinal hemorrhages in the emergency cesarean group (P=0.006). On multivariate analysis, vacuum-assisted delivery was the only factor associated with a higher prevalence of retinal hemorrhages in newborns (P=0.045). Two thirds of hemorrhages had disappeared by one week after birth. Retinal hemorrhages had resolved in all newborns within four weeks. CONCLUSION: Birth-related retinal hemorrhages are common (1/3 of our newborns). Vacuum-assisted delivery is the main risk factor in this study. All hemorrhages resolved by one month of age. These findings may help in differential diagnosis with shaken baby syndrome.

[Acute toxic anisocoria]. / Une anisocorie aiguë d'origine toxique.

PURPOSE: To report clinical findings after accidental ocular instillation of Angel's trumpet (Datura suaveolens) sap. OBSERVATION: A 24-year-old man, with no particular past history, presented with sudden-onset of unilateral mydriasis. The clinical appearance, as well as the detailed history, made possible the etiologic diagnosis of ocular exposure to sap from Angel's trumpet, a plant containing natural alkaloids with parasympatholytic properties. DISCUSSION: A toxic etiology of sudden-onset anisocoria may be established by taking a detailed history and being familiar with possible causative agents. Accidental ocular instillation of sap from Angel's trumpet should be noted as a possible cause of sudden-onset mydriasis and/or systemic symptoms such as tachycardia in otherwise healthy patients.

[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.

[Langerhans cell histiocytosis located in the orbit]. / L'histiocytose Langerhansienne a localisation orbitaire.

Langerhan's cell Histiocytosis of the orbit. Langerhan's cell Histiocytosis is a rare condition that affects children and less frequently young adults. It is characterized by a proliferation of histiocytes derived from Langerhan's cells. Orbital involvement is described in 20% of cases where orbital eosinophilic granuloma located in the frontal bone is the most frequent. The malignant nature of this disease is not established. Its evolution is unpredictable and spontaneous regression after simple biopsy were described. The authors report four cases of Langerhan's cell Histiocytosis and will discuss on the clinical and radiological aspects as well as on the evolution of orbital histiocytoses X.

[Incontinentia pigmenti: a case study]. / Incontinentia pigmenti: à propos d'un cas.

Incontinentia pigmenti is a rare hereditary, dominant, X-linked disorder. It involves the skin, the teeth, the eyes and the central nervous system. The case we report is an infant girl aged 2 months. She had typical skin lesions associated with severe impairment of her left eye. We comment on the clinical, histological, genetic, and therapeutic characteristics of this rare disease. Ophthalmologic examination should be made early in order to diagnose ocular involvement at an early stage of the disease to provide for greater treatment possibilities.

[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.

[Bilateral posterior microphthalmos associated with papillomacular fold and severe hyperopia]. / Microphtalmie postérieure bilatérale associée à un pli papillo-maculaire et une forte hypermétropie.

Posterior microphthalmos with papillomacular fold is a rare disorder that involves both eyes without other ocular or systemic abnormalities. It is characterized by a short posterior segment with a normal anterior segment. We report the case of a 3.5-year-old boy with high hyperopia. The fundus examination showed bilateral papillomacular fold and ultrasonography confirmed the posterior microphthalmos. We comment on clinical characteristics, pathogenicity, and inheritance modes of this rare congenital disorder.

[Neuroretinitis, Parry-Romberg syndrome, and scleroderma]. / Neurorétinite, hémiatrophie faciale progressive de Parry-Romberg et sclérodermie localisée. A propos d'un cas et revue de la littérature.

INTRODUCTION: Progressive hemifacial atrophy (Parry-Romberg syndrome) is a rare entity characterized by severe hemifacial atrophy affecting subcutaneous tissue. Its clinical manifestations are mainly neurological and ocular. The most common ocular finding is enophthalmos with eyelid modifications (ptosis, retraction, atrophy). Neuroretinitis remains a rare symptom in this affection. We report here a new case. CASE REPORT: A 22-year-old woman with progressive hemifacial atrophy (Romberg syndrome) is reported. The atrophy had begun 10 years before. At time of presentation, she was suffering from blurred vision in her left eye. She presented with mild enophthalmos associated with eyelid atrophy and loss of cilia. Biomicroscopic examination showed bilateral vitreitis and a typical spectrum of neuroretinitis in her left eye. There was no heterochromia. General examination revealed atrophy of the right part of her body. Etiological investigations showed the presence of antinuclear antibodies and a positive Rose-Waaler latex test without an inflammatory syndrome. Corticotherapy was proposed (1 mg/kg/day) with good progression. DISCUSSION: The authors report a new case of progressive hemifacial atrophy (Parry-Romberg syndrome). They emphasize the rarity of this disease, its etiology, which remains controversial, the diversity of its ophthalmologic manifestations, and its relations with scleroderma and autonomic nervous system disorders are discussed. Acute neuroretinitis remains rare and its pathogeny is unknown.

[Eales disease and tuberculous allergy]. / Maladie de Eales et allergie tuberculinique.

The authors report the case of a 25-year-old woman who presented unilateral Eales disease associated with biologically confirmed pulmonary tuberculosis. The patient, from a family with a history of tuberculosis, showed a painless and abrupt decrease in visual acuity of the right eye with venous and arterial vasculitis. Fluorescein angiography confirmed the existence of peripheral ischemia and vascular abnormalities. The biological exam showed a positive PPD and the BK was isolated. Antibiotic and corticosteroid drugs were used in the treatment. The clinical, immunopathological and therapeutic aspects of this disease are discussed.

[Bilateral congenital aniridia: 5 case reports]. / Aniridie congénitale bilatérale familiale: à propos de 5 cas.

INTRODUCTION: Aniridia is a rare familial or sporadic disorder affecting not only the iris but also the cornea, angle structures, lens, optic nerve, and fovea. This disorder may be associated with many other systemic abnormalities such as urogenital malignancies. CASE REPORTS: A 44-Year-old man who complained of visual loss presented bilateral congenital aniridia with corneal dystrophy and dense cataract. His 12-Year-old daughter also presented visual loss with severe bilateral corneal dystrophy and total congenital cataract associated with aniridia. Two other daughters, 18 and 23 Years of age, and an 11-Year-old son had nystagmus with bilateral congenital cataract and ectopia lentis. Clinical and radiological investigations did not disclose evidence of extraocular abnormalities in this family. Three patients underwent cataract extraction without intraocular implantation and the aphakia was corrected with glasses. All three patients progressed well. DISCUSSION: This family provided a good illustration of the different clinical spectra of ocular involvement in congenital aniridia and its management difficulties, above all those of cataract surgery and correction of aphakia. CONCLUSION: Aniridia is a genetic disease with possible association with other ocular and systemic disorders, which may compromise visual and vital prognosis. Genetic advice is very important in such families.

[Hyphema with secondary hemorrhage: think about sickle cell disease]. / Hyphéma traumatique avec hémorragie secondaire: penser à la drépanocytose.

The Authors report a case of a 13-Year-old white child who presented a grade I hyphema, anterior chamber inflammation after trauma. He presented a secondary hemorrhage with increased intraocular pressure that was not controlled within 48 h, thus requiring surgical intervention. A hemoglobin electrophoresis and hemostasis test showed a sickle cell trait. After hemorrhage resorption, the ocular fundus showed substantial retinal hemorrhage. The final visual outcome was poor and attributed to optic atrophy. Sickle cell trait is a significant risk factor for secondary hemorrhage, increased intraocular pressure, and permanent visual impairement in children who have traumatic hyphemas following blunt trauma.

[Uveitis in children: about 20 cases]. / Les uvéites de l'enfant. A propos de 20 cas.

INTRODUCTION: Uveitis in children is rare: 3%-8% of all cases. It requires the same diagnostic and etiological processes as uveitis in adults, with additional difficulties at examination and a sometimes insidious progression. MATERIAL AND METHODS: Retrospective study of 20 cases of childhood uveitis from 1995 to 2000. All patients received an ophthalmologic examination and an etiological search, with specific and/or symptomatic treatment and follow-up lasting from 6 months to 5 years. RESULTS: The 20 children presenting uveitis were 4-16 years old, with etiologies as follows: 8 cases of Behçet's disease, 2 cases of Vogt-Koyanagi-Harada's disease, 1 case of sarcoidosis, 1 case of uveitis associated with coeliac disease, 1 case of toxoplasmosis, 1 case of sympathetic ophthalmia, 1 case of uveitis with streptococcal infection, 3 cases of ocular toxocarosis, and 2 cases with unknown etiology. Treatment based on the etiological findings was started in the cases of toxoplasmosis and uveitis from streptococcal infection. The others were treated with high- and then digressive-doses of corticosteroids. ANALYSIS: We have noted the high incidence of Behçet's disease in our series. Progression was marked by frequent recurrence for one case of Vogt-Koyanagi-Harada's syndrome and one case of toxoplasmosis. This study also revealed a few cases of complicated cataract. DISCUSSION: Clinical characteristics, diagnosis, and treatment of uveitis in children are discussed.

[Choroidal granuloma revealing tuberculosis. A case report]. / Granulome choroïdien pseudotumoral révélant une tuberculose. A propos d'une observation.

BACKGROUND: The incidence of tuberculosis is still high in developing countries and a steady increase in new cases has been observed in industrial countries within the past decade. Among other reasons, the growing number of immunodeficient patients and migration from developing to industrial countries are both contributing factors. OBSERVATION: A 70-year-old female patient presented with a marked decrease in visual acuity of the left eye. There was no history of tuberculosis. Funduscopy of the left eye revealed a choroidal tumor with multiple choroidal lesions, mostly located at the posterior pole, and adjacent serous retinal detachment. Ultrasonography showed a solid elevated mass; magnetic resonance imaging showed the tumor with posterior extension. Because of blindness in this eye and the presence of posterior extension, the eye was subsequently enucleated. Intraoperative observation found caseum necrosis through scleral breaking. The histological study confirmed choroidal tuberculoma. The patient developed pulmonary tuberculosis 15 days after surgery. The outcome was favorable after antibiotic treatment. DISCUSSION: Confirming the diagnosis of tuberculous uveitis is often difficult. The differential diagnosis includes other granulomatous ocular inflammations. The detection of Mycobacterium tuberculosis and the clinical course make this diagnosis the most likely one. CONCLUSION: Choroidal granuloma is a rare and atypical location of tuberculosis. This case showed the diagnostic difficulties when there is no history of tuberculosis.

[Vogt-Koyanagi-Harada syndrome in children]. / Le syndrome de Vogt-Koyanagi-Harada chez l'enfant.

INTRODUCTION: Uveitis in children accounts for 5% to 10% of all uveitis cases. Some causes such as Vogt-Koyanagi-Harada syndrome rarely affect young children. We report two cases. CASE REPORT: No.1: A 9-year-old girl was followed up for severe and chronic total uveitis. General fundus depigmentation with several white and yellowish rounded lesions in peripheral fundus suggested the diagnosis of Vogt-Koyanagi-Harada, which was confirmed by the development of areas of vitiligo in the lumbar region. The patient was treated with a bolus of corticosteroid therapy. No. 2: A 12-year-old girl had bilateral uveopapillitis and the clinical initial examination and laboratory evaluation failed to provide a diagnosis. The patient was also treated with a bolus of corticosteroid therapy and then high-dose oral prednisone. After several months, the patient's examination showed a sunset glow fundus with several white and yellowish rounded lesions in peripheral fundus. DISCUSSION: Vogt-Koyanagi-Harada syndrome rarely affects young children, so the diagnosis may be difficult if the extraocular manifestations are not present. The bolus of corticosteroid therapy and cytotoxic agents may be necessary and the prognosis may be poor in severe cases.

[Childhood Behçet's disease: clinical and evolutive aspects. About 13 cases]. / La maladie de Behçet chez l'enfant, aspects cliniques et évolutifs Agrave; propos de 13 cas.

INTRODUCTION: Behçet's disease is a systemic vasculitis which rarely occurs in childhood. The aim of this study was to evaluate clinical characteristics and outcome of Behçet's disease in Moroccan children. MATERIAL AND METHODS: A retrospective study of 13 cases of children, 10 males and 3 females with Behçet's disease followed up between 1990 and 1998. The diagnosis of Behçet's disease was based on the criteria of the international study group for Behçet's disease. All patients were studied by a complete clinical, ophthalmological and laboratory staging and treated with appropriate therapy. RESULTS: The mean age at diagnosis of Behçet's disease was 13.9 years. Familial forms were found in 30.7% of cases. Oral aphtae were noted in all cases while genital ulcers were present in 76% of cases. Cutaneous lesions were found in only 1 case and 53.8% of children had a pathergy test. Articular involvement was found in 30.7%, neurological features in 46% and vascular manifestations in 38.4%. Only one case of intestinal involvement was noted. Ocular features (76%) were bilateral in all cases and were dominated by panuveitis complicated by macular edema ant retinal vasculitis. CONCLUSION: Behçet's disease seems to have particular characteristics in childhood. Familial forms, articular and digestive manifestations appear to be more frequent in early stages of Behçet's disease in children. Neurological and vascular involvement with panuveitis seems more frequent in the older children.

[Therapeutic and prognostic problems of traumatic cataracts. Apropos of 45 cases]. / Problèmes thérapeutiques et pronostiques des cataractes traumatiques. A propos de 45 cas.

PURPOSE: The purpose of this study was to investigate clinical profile, prognosis and therapeutics problems of traumatic cataract. MATERIAL AND METHODS: A retrospective study was conducted in 45 cases of traumatic cataracts (1993-1996). Mean age was 19 years, principally male. We chose the extracapsular extraction with implantation in 28 cases. RESULTS: Overall results were satisfactory (62% with VA > 2/10); they are better in patients with implantation and poorer in infants. Postoperatively, most complications were major inflammatory and secondary capsular opacification essentially children. The importance of systemic corticotherapy and prevention of amblyopia in children is emphasized.

[Vogt-Koyanagi-Harada syndrome. Epidemiological, clinical and disease progression aspects. Twenty cases]. / Le syndrome de Vogt-Koyanagi-Harada. Aspects épidémiologiques, cliniques et évolutifs. A propos de 20 cas.

PURPOSE: We retrospectively compared outcome in 20 patients with Vogt-Koyanagi-harada syndrome with results reported in the literature. PATIENTS AND METHODS: The charts of 20 patients attending the ophthalmology A unit of a specialized hospital in Rabat over a 7-year period between 1990 and 1997 were retrospectively reviewed. RESULTS: There were 15 women (75%). Mean age was 30 years (18-40 years). The prodromal stage was characterized by headache (100%), meningism (25%) and psychiatric changes (10%). In 100% of cases, both eyes were involved with visual acuity less than 1/10 in 65% and serious retinal detachment in 95%. Integumentary signs occurred in 9 patients (45%), with alopecia (40%), poliosis (40%) and vitiligo (20%). Lumbar puncture showed cerebrospinal fluid pleocytosis in 16 patients (80%) and audiometry revealed sensory neural hearing loss in 11 cases (55%). HLA typing, performed in only 5 patients, showed HLA DR4 in 100%. All of our patients were treated with systemic steroids and two of them had received immunosuppressive therapy. Long-term complications were cataract (25%) and intra-ocular pressure elevation (30%). After a mean follow-up of 6 years, 87.5% of our patients have visual acuity of 5/10 or better. CONCLUSION: Vogt-Koyanagi-Harada syndrome is common in Morocco, and visual prognosis is fair in most of the cases. It appears that cases in Morocco are more similar to those in the Asiatic population than in the American population.