Background: Xeroderma pigmentosum (XP) is a usually autosomal recessive disorder linked to a deficiency of the enzyme systems of DNA repair. The pathological sensitivity to the sun exposes the patient to develop multiple cancers. Materials and Methods: We conducted a retrospective study over a period from January 2006 to December 2014. A total of twelve patients were enrolled. Results: These were nine male cases and three female, with a sex ratio of 3/1. The average age was 7.9 years, ranging from 1 to 40 years. Consanguinity between the parents was found in ten cases (83.3%). The first non-tumor cutaneous manifestations appeared in eight patients before the age of six months. During follow-up, seven patients, including three (43%) at the age of eight years, died from metastasis. Conclusion: XP is complicated in the development of cancers, even in children, and is linked to the intensity of solar radiation in Niger. Key words: xeroderma pigmentosum; skin cancers; tongue amputation; Niamey; Niger
Notre objectif est de rapporter le cas d’une loase dans une zone sahélienne habituellement non endémique dans sa manifestation ophtalmologique. Il s’agissait d’un homme de 25 ans admis en consultation ophtalmologique pour sensation des corps étranger dans l’œil droit. Après examen ophtalmologique un ver translucide tortueux et mobile d’environ 4cm sous la conjonctive bulbaire à l’œil droit est observé. Après une extraction non traumatique chirurgicale, l’examen parasitologique confirme la loase. Il s’agit d’une parasitose des régions forestières essentiellement africaines. Suites aux mouvements des populations, elle peut être présente partout dans le monde. Il faut savoir la reconnaitre lors de nos consultations.
Cerebral venous thrombosis (CVT) in sickle cell disease has been rarely described in the literature. Some authors consider sickle cell disease as a risk factor for CVT. We report the case of a 20-year-old boy, known as sickle cell, followed at the National Reference Center for Sickle Cell Disease in Niger. Admitted in consultation for a decrease of acuity with the right eye with perception of a black spot evolving since about three (3) days, ophthalmological examination revealed sickle cell retinopathy associated with cerebral venous thrombosis on MRI. The rest of the balance showed normochromic anemia, ionic disturbance and a D-dimer at 1500 μg/l. Rehydration, strict rest, antibiotic therapy and analgesics were started with an internal medicine opinion for further treatment. There was an improvement in visual acuity of 6/10 in 48 hours with disappearance of black spot perception, after three weeks, an ad integrum recovery of retinal lesions to control imaging. Despite the ocular complications of sickle cell disease involving visual prognosis, cerebral venous thrombosis is another complication to be investigated in any sickle cell retinopathy.
Many general conditions have an impact on the fundus. The purpose of our work was to study the epidemiological profile of patients referred to our service for a review of the fundus and analyze the results. It was a retrospective study description of the examinations of the fundus during the period from January 2016 to December 2017. In the ophthalmology department of the Lomé University Hospital Center, during the study period, 1019 consultations were recorded, of which 727 were for fundus 6.84%. There were 336 men and 391 women, a sex ratio of 0.85. The age group 40-69 years accounted for 61.9%. The frequency of the main reasons for the request was hypertension 54.2% (N = 394); hypertension complicated or associated with other conditions (CKI, CKD, pregnancy and diabetes) accounted for 21.6% (N = 157), headache associated with a decrease in visual acuity 8.25% (N = 60), diabetes 6.46% (N = 47) and sickle cell disease 0.69% (N = 5). Hospitalized patients 52.41% (N = 381). The fundus was abnormal in 546 patients or 75.11%. Hypertensive retinopathy was found 80.21% cases in hypertensive patients, diabetic retinopathy 43.42 in diabetic patients. Diabetics Fundus examination in hospital practice found a retinal lesion in three-quarters of cases. It so important to ask the examination of the fundus most often in the current pactise.
Purpose: The aim of this study was to describe the management of a case of malignant melanoma of the conjunctiva at the National Hospital of Zinder in Niger and review of the literature. Observation: This was a young adult, 46 years old, male, from Agadez, with an unfavorable socioeconomic status, with no particular history referred for an upper left eyelid tumour. On examination, his general condition was preserved. On inspection, a mass hanging from the free edge of the left upper eyelid was noted; resulting in total mechanical ptosis preventing examination of the condition was preserved. On inspection, a mass hanging from the free edge of the left upper eyelid was noted, resulting in total mechanical ptosis preventing examination of the anterior segment. Palpation of the orbital rim did not note anything in particular, the lymph node areas were free. On the ophthalmological examination, on the right the visual acuity from afar without correction was 10/10 with a normal biomicroscopic examination, on the left the visual acuity was counting the fingers at one meter and a biomicroscopy hampered by the ptosis The assessment laboratory was normal, the orbitocerebral computed tomography showed the conjunctival origin of the tumor without notion of extension in the vicinity. Macroscopically complete excision was performed. Histopathological examination of the approximately 5 cm surgical specimen noted a malignant melanoma of the conjunctiva with impregnation of the margins (R1 resection). Adjuvant treatment with mitomycin C was instituted. The postoperative course was simple. After 6 months of postoperative follow-up, the ophthalmological examination was normal and the search for metastases was negative. Conclusion: Malignant melanoma of the conjunctiva is a rare neoplasia that can mimic a palpebral tumor. Histopathological examination remains important for treatment and monitoring.
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