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Biomedical subjects

A Berraho

Publications and source records attributed to A Berraho.

At least 19 recordsLinked to original sources

[Uveitis and chronic intestinal inflammatory diseases: three case studies].

INTRODUCTION: The most frequent inflammatory chronic bowel diseases are Crohn disease and ulcerative colitis, which present extra intestinal manifestations, particularly, arthritis and ocular inflammation. Anterior uveitis is the main ocular manifestation and remains exclusively an inflammatory process. MATERIAL AND METHODS: [corrected] We report three cases of bilateral uveitis, with ulcerative colitis in two cases and Crohn disease in the last case. In the two first cases, uveitis occurred after 8 years of bowel disease which was stabilized by sulfasalazopyridine and 6 months after bowel surgery. In the third case, the patient was followed for Crohn disease for 3 years with anterior uveitis relapse concomitant to bowel relapse. RESULTS: In the two first cases, there was no recurrence after corticosteroids topical therapy. In the third case, topical and systemic corticosteroids provided adequate control of ocular inflammation, but the follow-up was marked by pupillary and inflammatory glaucoma, which required gonio-surgery. DISCUSSION: Anterior uveitis is the most frequent ocular manifestation in chronic inflammatory bowel disease: approximately 1.9-4.9% in Crohn disease and 1.6% in ulcerative colitis. The uveitis course is synchronous with bowel relapse and its treatment requires anti-inflammatory agents. Nevertheless, in severe relapse, recovery is obtained after the resection of the entire involved bowel segment.

Adult↗

[Langerhans-cell histiocytosis of the orbit. A case study].

PURPOSE: Langerhans' cell histiocytosis is a rare disease representing less than 1% of orbital tumors. METHODS: We report a case of Langerhans cell histiocytosis with orbital involvement in a 9-year-old boy. He presented with an inflammatory swelling if the left lateral orbital wall. The computed tomographic scan revealed an orbital cellular mass with lytic bone lesion within the orbital roof and intracranial enlargement. RESULTS: The cytological study after a biopsy showed infiltrates of histiocytes derived from Langerhans cells. Diagnosis was confirmed by immunohistochemistry, which identified positive staining with anti-S100 and anti-CD1a antibodies. The rapidly expanding orbital tumor, posing a threat of ocular compression as well as intracranial spreading, was treated by chemotherapy (Vinblastine) combined with a steroid. CONCLUSION: A 2-year follow-up showed no evidence of recurrence or systemic involvement. According to this observation, the authors describe the clinicopathological and histological features of orbital involvement in Langerhans cell histiocytosis.

Child↗

[Macular serous detachment revealing acute lymphoblastic leukemia].

BACKGROUND: Leukemias are a group of malignant diseases caused by immature hematopoietic cells proliferating in the blood marrow. Some manifestations result from ocular-orbital involvement, which usually occurs through the central nervous system. Other manifestations stem from vasculopathy and/or hemorheologic disorders (anemia, thrombocytopenia, hyperviscosity). OBSERVATION: We report a case of a 42-year-old women presenting with loss of vision caused by serous macular detachment. The investigations showed the diagnosis of acute lymphoblastic leukemia. Steroids and chemotherapy led to complete remission with normal visual acuity during a follow-up of 29 months. DISCUSSION: Ocular involvement is seen in 28%-80% of leukemia cases. The most obvious findings are the presence of retinal hemorrhages, which are most commonly located in the posterior pole. The intraretinal hemorrhage may contain a white component that usually is a white dot in the center of the hemorrhage, but other clinical features are described. Serous detachment of the neuroepithelium is seldom reported, and can be the first symptom of the disease. Other ocular findings include infiltrative involvement of the chorioretina, anterior segment involvement, and orbital location. Ocular location is estimated to be an equivalent of central nervous system involvement, and subsequently requires adequate treatment (steroids, chemotherapy and radiotherapy of the central nervous system). CONCLUSION: Ocular manifestations of leukemia are frequent but rarely reveal the disease. Serous detachment is rarer. However, the diagnosis of leukemia should be considered in case of pigmentary epithelium involvement.

Adult↗

[Diagnostic difficulties in pigmented tumor of the optic nerve head. A case report].

Melanocytoma of the optic disc is a benign pigmented tumor located on the optic nerve head. Most cases are visually asymptomatic but in rare cases it can cause visual loss, which does not necessarily involve malignant transformation. We present a case of melanocytoma in a 49-year-old woman who presented with decreased vision in her left eye. The pigmented mass was in close continuity with the juxtapapillary adjacent choroid. Fluorescein angiography demonstrated hypofluorescence with adjacent disc edema. Ultrasonography disclosed a high internal reflectivity mass in the optic nerve head without retrobulbar extension. Magnetic resonance imaging (T1 weighted images) disclosed a hyperintense signal on the optic nerve head. The tumor was stationary for 20 months. This report emphasizes problems differentiating a melanocytoma from malignant melanoma of the optic nerve. Unusual features of melanocytoma (superior nasal location in the optic disc with an adjacent choroidal component, a decrease in visual acuity and disc edema surrounding the tumor) are discussed. Visual loss can be induced by optic neuropathy or retinal vascular obstruction. Melanocytomas grow very slowly over several years or remains stable, in contrast to malignant melanoma. In suspicious cases, close follow-up with serial fundus photographs is essential, although malignant transformation is exceptional.

Carcinoma, Papillary↗

[Isolated hypertrophy of the superior oblique muscle in Basedow disease].

INTRODUCTION: Proptosis is the most common feature of Graves' ophthalmopathy. We report an exceptional case of proptosis in relation with an isolated enlargement of the superior oblique muscle in Graves' ophthalmopathy. OBSERVATION: After ocular contusion a 37 years old man presented a progressive right exophthalmos. On examination there was an unilateral proptosis with dilated conjunctival vessels, without any clinical signs of inflammation and no decreased vision. Ocular movements were full. On general examination signs of hyperthyroidism were present. CT-scan showed a superior oblique muscle enlargement with enlarged superior ophthalmic vein. Cerebral angiography excluded the diagnosis of a carotid-cavernous fistula. Dosage of thyroid hormone revealed hyperthyroidism. DISCUSSION: This case has a double interest: --Diagnostic: a CT-scan picture mimicking a carotido-cavernous fistula in this particular clinical context (history of ocular trauma, enlarged conjunctival vessels). --Isolated enlargement of superior oblique muscle in Graves' disease, which is exceptional in the literature.

Adult↗

[Spontaneous orbital hematoma in an adult. A case report].

Spontaneous orbital hematoma is uncommon, especially in adults. It produces proptosis which progress rapidly and is often associated with ocular movement disturbance. The Authors report a case of spontaneous orbital hematoma in a 45-year-old woman, who had had no previous disease and had experienced sudden onset diplopia associated with moderate proptosis and pain in her right orbit. The diagnosis of orbital hematoma was made by computed tomographic scan. Further investigations revealed arterial hypertension. After a needle aspiration of this hematoma, proptosis resolved, ocular movement began to improve as well as visual acuity. Orbital hematoma revealing arterial hypertension is exceptional. It could be associated with ocular and orbital complications that lead the patient to consult an ophthalmologist. The diagnosis is based essentially on radiology. The other causes of orbital process, inflammation, tumor or vascular malformation, should be considered in the differential diagnosis. Therapeutic management could require surgical evacuation when visual function is compromised (compressive optic neuropathy).

Female↗

[Enucleations: epidemiologic investigation in Morocco. presentation of 183 cases].

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.

Adult↗

Gamma-D crystallin gene (CRYGD) mutation causes autosomal dominant congenital cerulean cataracts.

Congenital cataracts are a major cause of bilateral visual impairment in childhood. We mapped the gene responsible for autosomal congenital cerulean cataracts to chromosome 2q33-35 in a four generation family of Moroccan descent. The maximum lod score (7.19 at recombination fraction theta=0) was obtained for marker D2S2208 near the gamma-crystallin gene (CRYG) cluster. Sequencing of the coding regions of the CRYGA, B, C, and D genes showed the presence of a heterozygous C>A transversion in exon 2 of CRYGD that is associated with cataracts in this family. This mutation resulted in a proline to threonine substitution at amino acid 23 of the protein in the first of the four Greek key motifs that characterise this protein. We show that although the x ray crystallography modelling does not indicate any change of the backbone conformation, the mutation affects a region of the Greek key motif that is important for determining the topology of this protein fold. Our data suggest strongly that the proline to threonine substitution may alter the protein folding or decrease the thermodynamic stability or solubility of the protein. Furthermore, this is the first report of a mutation in this gene resulting in autosomal dominant congenital cerulean cataracts.

Amino Acid Sequence↗

[Melanoma of the iris and pregnancy].

Iris melanoma is a malignant melanocytic tumor, making up 1.2%-6.6% of uveal malignant melanomas. The growth of choroidal melanoma during pregnancy is described and a hormonal influence is suggested. We present a case of iris melanoma occurring during pregnancy. A 32-year-old woman, in the 28(th) week of her fifth pregnancy, was referred to the ophthalmologist with a 2-month history of blurred vision and pain in her left eye. The visual symptoms became progressively worse with decreasing vision. Her visual acuity was 20/70 in her left eye, with a temporal, pigmented, prominent iris mass extending into the trabecular meshwork and the corneal endothelium. Elsewhere other pigmented localizations were found in the iris and in the trabecular meshwork. There was secondary glaucoma with intraocular pressure of 36mmHg and C/D=0.9. The right eye was normal. A general physical examination found no pigmented lesions. Chest radiography and hepatic ultrasonography revealed no metastasis. The diagnosis of iris malignant melanoma was made from the diffuse involvement extending into the trabecular meshwork and the advanced secondary glaucoma; an enucleation was performed and histopathological examination confirmed the diagnosis of iris melanoma with involvement of the trabecular meshwork. The patient had no further problems (follow-up of 11 months). From this case report, we discuss the differential diagnosis of iris melanoma and the hormonal influence on its growth.

Adult↗

[Solitary retinal astrocytic hamartoma simulating retinoblastoma].

Retinal astrocytic hamartoma and retinoblastoma may be very similar clinically and their differentiation in atypical cases can be difficult, even with the use of ultrasonography and computed tomography. In such cases, a close follow-up is recommended before enucleation. This paper reviews the case of a 18 month old girl who presented with a solitary retinal astrocytoma of the right eye, without any other physical or ocular disorder. The initial presentation simulated a retinoblastoma; nevertheless atypical patterns as yellow calcifications and the lack of tortuous and dilated feeding blood vessels were present. Ophthalmoscopic and ultrasound regular evaluation did not reveal any change after one year follow-up. Additional investigations performed in order to exclude tuberous sclerosis (neurological and dermatological examination, CT-scans) showed no other organ involvement, which ruled out a phakomatosis. The clinical appearance and course of astrocytic hamartomas, its differential diagnosis from other retinal tumors, especially retinoblastoma, and its association with tuberous sclerosis are discussed.

Calcinosis↗

[Ophthalmic manifestations of acute leukemia].

Orbital and ocular lesions are the third most frequent extramedullar locations of acute leukemia after the meninges and testicles. These lesions are treated as a central nervous system lesion; therefore diagnosis is essential to therapeutic adjustment. We present a retrospective study on charts of children treated for acute leukemia between 1996 and 1998. Ophthalmic examination was carried out when there were ocular symptoms. One hundred ninety-six children were treated for acute leukemia. Twelve children (6.1%) had an ocular and orbital lesion: 7 boys and 5 girls, with an average age of 6 years. Six had acute lymphoblastic leukemia, 6 had acute myeloid leukemia. Visual acuity was 1/10 in 9 children. Four children had an initial orbital lesion with a rapidly progressing exophthalmos. The hemogram and myelogram showed a granulocytic sarcoma. Two children who had been previously treated for acute leukemia presented a bilateral anterior uveitis with hypopyon; anterior chamber paracentesis showed blast cells and confirmed the ocular relapse. A corneal lesion was found in 2 children and in 2 cases, the optic nerve had edema; a decrease in visual acuity was the sign of the optic nerve lesion. Two patients had retinal infiltration with hemorrhages in the posterior pole. Prophylaxis consists of intrathecal injection of methotrexate and reinforcement of chemotherapy. Central nervous system irradiation is seldom used in children. Orbital and ocular lesions carry a poor prognosis according to the majority of authors. Two years after diagnosis of the ocular lesions, only 1 of the children studied remains alive.

Acute Disease↗

[Cutaneous leishmaniasis of the lid].

We report 3 cases of patients presenting with lid cutaneous leishmaniasis in which clinical presentation was a true diagnostic problem. The patients were 3 women aged 46, 36, and 60 years. The first patient presented with an ulceration of the superior eyelid that had been treated as chalazion. The second patient had a chronic lesion of the eyelid, present for 1 year, fitting the criteria for tuberculosis, syphilis, or sarcoidosis. The last patient had an erosive lesion of the internal canthus, which was suspicious of basal cell carcinoma. The presence of other cutaneous lesions and the chronic progression led to a biopsy for anatomopathology and parasitology analysis. These analyses confirmed the diagnosis of cutaneous leishmaniasis. The patients were then treated with antimony derivatives. Progression of the disease was marked by the appearance of a pigmented nonretractile scar. The different clinical characteristics, epidemiology, and treatment of this affection are reviewed.

Adult↗

[Ocular relapse of acute lymphoblastic leukemia].

A bilateral leukemic hypopyon can be inaugural in the child's leukemia or reveal a relapse. A five years old child with acute lymphoblastic leukemia presented after 30 months of treatment a bilateral hypopyon. Anterior chamber paracentesis with cytological survey revealed leukemic cells and confirmed the ocular relapse. The treatment included the association of topical corticosteroids, chemotherapy and radiotherapy. This child died unfortunately 16 months later following a medullar relapse. We remind the different clinical aspects of leukemic invasion of the anterior segment and the therapeutic methods for this relapse.

Administration, Topical↗

[Orbital lymphangioma. A case report].

The diffuse form of orbital lymphangioma is well known for its difficult surgical treatment. A diffuse orbital lymphangioma was diagnosed in a 6-year-old girl, revealed by unilateral recurrent proptosis. The imaging procedure discovered a mass presumed to be vascular in nature. The initial incompleted surgical removal was followed by 3 recurrences motivating 3 reoperations. Finally, the tumor was removed incompletely, with an acceptable reduction of the proptosis. The pathologic analyses indicated lymphangioma. Some vascular orbital tumors such as lymphangioma, may be very difficult to manage because of local spreading and frequent recurrence.

Adolescent↗

[Tuberculous uveitis].

Ocular tuberculosis is relatively rare; however, the most common ocular lesion during ocular tuberculosis is uveitis. The recrudescence of this disease is probably caused by human immunodeficiency virus or long-term corticotherapy. The authors report a of chronic unilateral tuberculosis-related uveitis complicated by panophthalmia with subconjunctival abcesess and scleral fistula. A histopathological study revealed a granulomatous inflammation with caseous material. An extensive work-up revealed no extraocular lesion. A systemic bactericidal treatement associated with topical steroids decreased the local inflammation with phthisis bulbi, and with no spread of the infection. The authors discuss the etiopathogenic, clinical, and therapeutic aspects of tuberculosis-related uveitis.

Adult↗

[Epithelial cyst in the superior internal angle: a case report].

Most orbital cysts in childhood are epidermoids or dermoids. We report one uncommon case of an 8-years old girl with a lacrimal orbital cyst revealed by discrete proptosis and pseudo-ptosis in which the cystic histopathological study revealed a lacrimal structure.

Child↗

[Medulloepithelioma of the ciliary body. A case report].

Medulloepithelioma of the ciliary body is an uncommon intraocular tumor occurring during the first year of life. Malignant degeneration may occur. We report the case of a 4-year-old child who presented medulloepithelioma of the left eye disclosed by oesotropia at 2 years of age. Clinically, there was oesotropia, positive light perception and cataract with vascular membrane spreading to the nasal side of the irido-corneal angle. CT scan and ultrasound B revealed a ciliary body tumor involving the sclera and orbital fat. After exenteration, the pathology study reported malignant medulloepithelioma of the ciliary body with scleral extension. No local recurrence or metastasis has been observed at 8 months follow-up. We discuss the clinical, radiological and therapeutic features of this uncommon tumor.

Child, Preschool↗