Biomedical subjects

A Amraoui

Publications and source records attributed to A Amraoui.

At least 19 recordsLinked to original sources

PubMed2006

[Fraser syndrome. A case report].

Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.

Abnormalities, Multiple↗

PubMed2005

[Primary hepatic localization of the PPNET (primitive peripheral neuroectodermal tumors). Case report].

Our purpose is to study, through the case of a patient operated for right hepatic tumour, the clinical, radiological, anatomopathologic and therapeutic aspects of the peripheral primitive neuroectodermal tumours (PPNET). This tumour (PPNET) is a neoplasm belonging to the Ewing's family tumours, whose histology is similar. Its diagnosis requires the contribution of histopathology, immunohistochimy and cytogenetic studies. The primary hepatic localization of this rare tumour (our case), has never been reported. The treatment, in theory copied on that of the Ewing's sarcoma, is complex and not yet codified, which makes it another disappointing aspect of this disease whose prognosis remains dark.

Diagnosis, Differential↗

PubMed2005

[Avulsion of the optic nerve: two case studies].

Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic nerve avulsion. The first one concerns a 5-year-old boy who presented ocular trauma after falling on the handlebars of a bicycle. His visual acuity was light perception in the right eye, and his right pupil was unresponsive to light. The anterior segment was normal. The ophthalmoscopic examination showed a total separation of the optic nerve head from the sclera with peripapillary hemorrhage. The second case concerns a 30-year-old man who was hit in the right eye with a stick. On admission, he had no light perception in the right eye, a right afferent pupil defect, a small laceration of the right lower eye lid and no abnormalities on the anterior segment. The fundus examination showed a mild vitreous hemorrhage. Ocular ultrasonography showed vitreous hemorrhage coming directly from the optic nerve head in a mushroom pattern. A CT scan of the orbit revealed a thickened optic nerve. Color Doppler ultrasonography documented slowing of blood flow in the central retinal artery. The two patients received 1 mg/kg/day of prednisone for 2 weeks. No improvement was noted. Optic nerve avulsion is often caused by sudden and forceful rotation of the eye with tearing of the optic nerve as it exits the globe. The nerve can be partially or totally avulsed. The prognosis is usually poor.

Adult↗

PubMed2005

[Apert syndrome: a reported observation].

Apert syndrome is a type of acrocephalosyndactylia that belongs to the group of craniofacial synostoses. It is characterised by craniofacial dysmorphia and syndactyly of hands and feet. It is an uncommon affection that is often transmitted through an autosomal dominant mode, but sporadic cases are frequent. We report the case of a 2 months old baby brought by his parents to the paediatric emergencies for respiratory distress occurring within the framework of a polymalformative syndrome. The examination showed brachycephaly, bilateral exorbitism, syndactyly of the hands and feet and an anal fistula. Echocardiographic examination showed a cardiovascular malformation (interventricular communication), the whole suggestive of Apert syndrome. The child was admitted in intensive care during five days. He died following a respiratory infection. Through this observation the authors illustrate the clinical and evolutionary aspects as well as the therapeutic difficulties of this affection.

Acrocephalosyndactylia↗

PubMed2004

[Macular damage in Behçet's disease].

INTRODUCTION: The macular damage in Behçet's disease requires particular attention because of its poor prognosis. PURPOSE: The purpose of our study was to specify the frequency of macular alterations in Behçet's disease and discuss the clinical, physiopathological and therapeutic aspects. MATERIALS AND METHODS: Our report is based on 244 patients with Behçet's disease examined between January 1995 and December 1997. The authors studied macular alterations in detail. RESULTS: Sixty-nine patients had a maculopathy affecting 108 out of 432 eyes (24%). The mean progression of Behçet's disease in these cases was 4 years. Visual acuity was<or=2/10e in 48 eyes (44.4%). Maculopathy was bilateral in 39 cases. Macular edema was the most frequently noted form in 62% of the eyes, followed by cystoid macular edema (17%) and macular hole (3.4%). DISCUSSION: Macular edema is the most frequent sign and complication arising from the macular area during Behçet's disease, which can evolve into cystoid degenerescence and occasionally macular hole. Macular lesions are often irreversible, making prognosis uncertain, despite the various treatments available. CONCLUSION: Macular damage is the major cause of visual morbidity in patients with Behçet's disease and should be systematically sought.

Adult↗

PubMed2004

[A case of a retinitis pigmentosa and asteroid hyalosis].

Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10e in the right eye and 4/10e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.

Aged↗

PubMed2003

[Metastasis of medullar thyroid carcinoma (case report)].

We report the case of a 38 year-old man treated for medullar thyroid carcinoma in 1990. Nine years later, this patient developed metastases in the parotid, cervical nodes and bones, with a choroidal metastasis in the left eye one year later. Polychemotherapy was performed without good outcome.

Adult↗

PubMed2003

[Involvement of the optic nerve in the course of Behcet's disease (presentation of 148 cases)].

Behçet's disease is a multisystemic vascularitis of still unknown etiopathogeny. Among 400 cases of Behçet disease, 148 cases presented an optic nerve involvement during a period of eight years (1992-1999). The goal of this work is to contribute to the study of optic nerve involvement in Behçet's disease. The involvement is higher in males (64%) with median age of 27 years. The involvement of the optic nerve is noticed in 37% of cases. It's isolated in 7% of cases and occurs on average after 5 years of evolution of the disease. The diagnosis is based on the clinical examination, visual field, visual evoked potentials, retinal angiography and neuro-imaging (TDM and/or MRI). It can be an acute anterior neuropathy, stasis papilledema complicating a benign intracranial hypertension, neuroretinitis or retrobulbar optic neuropathy. The extraocular systemic manifestations were dominated by oral aphthosis (94%), genital aphthosis (70%), joint manifestations (40%) and central nervous system involvement (32.4%). The prognosis is reserved, 44% of patients having vision lower than 1/10 in spite of treatment. The authors insist on the therapeutic emergency that this involvement represents and the interest to consider it in all patients having an unexplained visual loss.

Adolescent↗

PubMed2003

[Ocular and systemic complications of homocystinuria: a report of five cases].

Homocystinuria is an autosomal recessive disorder of methionine metabolism due to cystathionine B-synthetase deficiency. It is the second most common inborn error of amino acid metabolism after phenylketonuria. In addition to the eyes, the skeletal, central nervous and vascular systems are usually affected by homocystinuria. We report two family cases of two sisters and two brothers with homocystinuria revealed by lenticular dislocation into the anterior chamber, associated with mental and growth retardation, and an isolated case of homocystinuria revealed by poor vision with Marfan syndrome and mental retardation. They all underwent surgical lensectomy with anterior vitrectomy under general anesthesia with anaesthesic precautions to prevent vascular thrombosis. Ocular complications are common in patients with homocystinuria. Treatment must include dietary changes to reduce the incidence of ectopia lentis and mental retardation. Surgical treatment with modern microsurgical techniques should be considered in advanced ocular manifestations of homocystinuria.

Child↗

PubMed2002

A novel frameshift founder mutation in the cytochrome P450 1B1 (CYP1B1) gene is associated with primary congenital glaucoma in Morocco.

Primary congenital glaucoma (PCG) is a heterogeneous autosomal recessive disorder caused by unknown developmental defect(s) of the anterior chamber of the eye. A member of the cytochrome P450 gene family, CYP1B1, was found to be mutated in PCG patients in different populations, albeit to a variable extent. In this study, CYP1B1 mutations were searched for in 32 unrelated PCG patients from Morocco. Two mutations were detected in 11 (34%) patients. One, 4339delG, is novel and causes a frameshift at residue 179. The other, G61E, was previously found in patients from Turkey and Saudi Arabia. Seven patients were homozygous for 4339delG and two other patients for G61E, whereas the two remaining patients were compound heterozygotes. The close association of 4339delG with a rare allele of D2S177, a microsatellite marker located 270 kb upstream of CYP1B1, strongly suggested a founder effect for 4339delG. The occurrence of this mutation was tentatively dated at between 900 and 1700 years ago. Typing 4339delG and G61E mutations should help to prevent blindness resulting from a delayed diagnosis of PCG in Morocco.

Aryl Hydrocarbon Hydroxylases↗

PubMed2002

[Subperiosteal hematoma of the orbit associated with subfrontal extradural hematoma].

Subperosteal hematoma is rarely associated with extradural hematoma and orbital roof fracture. We report a case observed in a 16-year-old boy who developed exophthalmos, diplopia and visual loss after blunt head trauma. Computed tomography demonstrated the subperiosteal hematoma associated with a thin extradural hematoma and an orbital roof fracture. The hematomas were removed during the same procedure via fronto-orbito craniotomy. Surgery led to complete recovery without functional sequelae. We stress the importance of early diagnosis and treatment of post-traumatic exophthalmos.

Adolescent↗

PubMed2002

[Ocular impairment during type II membranoproliferative glomerulonephritis].

Membranoproliferative glomerulonephritis type II (MPGN) is characterized by dense deposits within glomerular basal membrane and Bruch's membrane which result in retinal lesions similar to drusens. We observed a 50-year-old patient with chronic renal deficiency who developed central bilateral serous retinopathy with diffuse punctiforme yellow subretinal lesions. Ophthalmoscopic and angiographic aspects led to an MPGN type II diagnosis. Specific posterior segment lesions are described during MPGN type II. Dense deposits concerned both lamina densa of glomerular basal membrane and Bruch's membrane with choriocapillaris. The main ocular complications were central serous chorioretinopathy and choroidal neovascularization. We review the clinical and evolutive aspects of this disease.

Bruch Membrane↗

PubMed2001

[Weill Marchesani syndrome. Report of a case].

Weill Marchesani syndrome is a congenital disease that combines microspherophakia and skeletal abnormalities. The authors report a 19-year-old male, born of a consanguineous marriage, with a progressive decrease in visual acuity. The general examination showed a squat look, dwarfism, muscle hypertrophy, short hands and feet, and joint stiffness. The ophthalmological examination showed that visual acuity was limited to hand motion in the right eye despite correction and no perception of light in the left eye. Intraocular pressure was 36 mmHg in the right eye and 40 mmHg in the left eye. The anterior chamber was irregular with iridophakodonesis and microspherophakia of both lenses. The zonula was partially ruptured in the right eye. The iridocorneal angle was narrow. Fundus eye examination showed a pale optic disc with an excavation of 9/10 on the right. In the left eye, the optic disc was totally excavated. Cardiovascular check-up revealed rheumatic aortic valvular cardiopathy. The therapy consisted of combined surgery: phakophagia with anterior vitrectomy plus trabeculectomy operated on the right eye. Weill Marchesani syndrome is a rare congenital affection with a recessive autosomal transmission. The visual prognosis is dominated by secondary glaucoma due to pupillary blockage by the mobile eye lens. This observation illustrates the seriousness of spontaneous progression in Weill Marchesani syndrome, justifying the necessity of lens extraction before the onset of complications.

Abnormalities, Multiple↗

PubMed2000

[Cockayne's syndrome with unusual retinal involvement (report of one family)].

Cockayne's syndrome is a very rare autosomal recessive affection. Ocular involvement is an essential element for positive diagnosis; the retina shows a typical salt and pepper retinitis with optical atrophy. We report a family with four brothers who had Cockayne's syndrome with unusual retinal involvement. The patients' parents were first cousins. Ophthalmologic examination of the mother showed unilateral left pigmentary retinopathy localized in the peripapillary region. The father's ophthalmological examination was normal. The four brothers presented disharmonious dwarfism, cutaneous hyperpigmentation of skin areas exposed to sun with old-appearance of the skin, sensorineural deafness, kyphoscoliosis, a cerebellar syndrome and mental retardation. The ophthalmological examination showed hypermetropia in all four brothers and bilateral maculopathy with no papillary or vascular abnormalities. The electroretinogram was in favor of cone dystrophy. Computed tomography showed one case of calcifications of the basal ganglia and cerebral atrophy. The karyotypes of the four brothers and the mother were normal. We discuss the ocular symptoms and the etiopathogenesis of this syndrome.

Adolescent↗

PubMed2000

[Oculomotor paralyses in the course of diabetes--concerning 12 cases].

PURPOSE: Our purpose is to study the clinical features of the oculomotor paralyses in the course of the diabetes, to assess the risk factors and to show the importance of the diagnosis and the cerebral imaging. MATERIAL AND METHODS: We report a series of 12 diabetic patients with OMP collected at the department of ophthalmology in UHC Ibn Rochd in Casablanca between 1995 and 1998. RESULT: Our study was about 9 men and 3 women, the mean age was 58 years 6 months. The diabetes was type I in 2 cases and type II in 10 cases. All the patients complained of diplopia with headache. The paralysis of VI has been noticed in 8 cases, it was bilateral in 5 cases. Unilateral Ill palsy occurred in 4 cases. Brain computed tomography was normal in 10 cases and pathologic in 2 cases. Treatment consisted in alternating occlusion in all patients. Concomitance was achieved, after 4 to 6 months of treatment. CONCLUSION: The metabolic abnormalities due to hyperglycemia and the ischemic phenomena due to the diabetic macroangiopathy explain these palsies.

Adult↗

PubMed2000

[Acquired oculomotor paralysis: a new therapeutic approach. Apropos of 10 cases].

PURPOSE: The aim of this work is to report our rehabilitation scheme and outcome in patients with acquired oculomotor palsy. PATIENTS AND METHODS: We cared for 10 patients with oculomotor palsy between January 1996 and March 1998 at the Casablanca University Hospital. Our orthoptic rehabilitation scheme was based on reinforcing the patient's sensorial potential using a prism dioper and motor capacities by soliciting vergency and version movements. RESULTS: The 10 patients (7 males, 3 females, mean age 39.5 years) had unilateral IV palsy (n =3), bilateral IV palsy (n= 1), unilateral VI palsy (n= 2), bilateral VI palsy (n= 1), partial unilateral III palsy (n= 1) and dissociated bilateral III palsy (n= 2). Mean delay to initiation of rehabilitation was 49 days and mean duration for treatment was 3 months. We achieved total recovery in 50% of the cases and partial recovery in 40%. Intermittent diplopia persisted in 10 per thousand of the cases. These results differ slightly from those in the literature where total regression is reported in about 50% of the patients after therapeutic abstention. CONCLUSION: This small series is insufficient to validate our method. The results obtained do however show that rehabilitation is safe and should be applied more widely in patients with oculomotor palsy.

Adolescent↗

PubMed2000

[Ocular damage during HIV infection at the University Hospital Center of Casablanca. (Apropos of 400 cases)].

The objective of this study was to report ocular manifestations in Moroccan patients infected with HIV/AIDS. 400 patients were surveyed retrospectively from 1993 to 1998 as part of a co-operative study undertaken by the departments of infectious diseases and ophtalmology of the Casablanca Teaching Hospital. Of the 400 patients, 127 had an infection of the posterior segment and 7 showed signs of a palpebral infection. We also found 44 cases of opportunist retinal infections, dominated by the Cytomegalovirus (CMV) (18 cases). Our sample study indicates an intermediary position between western countries where the widespread use of the tritherapy has increased the life expectancy of patients and sub-Saharan Africa where epidemiological data are still characterised by high mortality and increasing endemic disease.

AIDS-Related Opportunistic Infections↗

PubMed2000

[Vogt-Koyanagi-Harada's disease: 3 cases].

BACKGROUND: Vogt-Koyanagi-Harada's disease is a bilateral chronic panuveitis sometimes associated with signs of meningo-encephalic, auditory and skin and nail involvement. We report 3 cases. CASE REPORTS: The first case was a 30-year-old woman who consulted for a red eye, low visual acuity, poliosis, and diffuse alopecia which had developed over 9 months. The ophthalmology examination disclosed anterior uveitis with retinal detachment. The second patient was a 9-year-old child who developed poliosis, canities, and achromic lesions over a 2 month period. The ophthalmology examination disclosed low visual acuity, irido-corneal synechiae and pigmented deposits on the anterior lens. The third patient was a 20-year-old man who consulted for alopecia, diffuse canities, and white body hair. The ophthalmology examination disclosed low visual acuity, anterior uveitis, and a serous chorio-retinal detachment. All three patients were given general corticosteroid therapy (1 mg/kg/d). The clinical course was favorable in all cases with however one case of recurrent ocular involvement and one case of pigmentation disorders. DISCUSSION: The diagnosis of Vogt-Koyanagi-Harada's disease was established in these three cases on the basis of the ocular and skin and nail signs. This rare disease usually occurs in young, often female, patients. The pathogenesis remains unknown. Among the three signs observed, ocular involvement is the most serious. Skin and nail signs are seen in two-thirds of cases. For rapid diagnosis and early treatment, this disease requires a multidisiplinary management associating the dermatologist and the ophthalmologist.

Adult↗