1/15. Malignant haemangioendothelioma of bone in a HbSC disease patient--a case report.We report here a 35-year-old man with sickle cell disease (SCD), who presented in 1989 with pain in the (R) hip of 7 years duration and swelling of the (R) calf of 3 months duration. Clinical examination revealed a hard tender mass in the (R) calf. histology of the (R) calf mass revealed haemangioendothelioma (HE), similar to the histology of the (R) iliac bone mass obtained in another institution previously. He was treated with external radiotherapy with the 1.25 megavoltage beam to antero-posterior fields of the (R) hemipelvis and (R) calf, with good response. Chemotherapy was subsequently administered using 6 cycles of VAC regime. The patient remained in remission for 12 months. In 1991 he had lymphoedema of (R) lower limb and received further radiotherapy and chemotherapy after an isotope bone scan had revealed disease activity in the (R) hemipelvis, (R) femur and (L) upper tibia. He responded again with complete regression of the lymphoedema and remained well until April 1993 when the lymphoedema recurred. He died while being evaluated for further treatment. Although there is no evidence to suggest that SCD confers any protection from development of neoplasms, the co-existence of SCD with a neoplasm is not common. We consider the occurrence of HE of bone, a rare malignancy, in a HbSC patient worthy of reporting.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
2/15. Dark-without-pressure fundus lesions.Seven black patients had dark brown homogeneous geographical areas of the fundus. Six cases were associated with sickle cell haemoglobinopathies and one was associated with systemic hypertension. These flat lesions were uniform in colour and occurred in the posterior pole or in the midperiphery. They appeared to be transient and often disappeared leaving no residue. The cause is unknown. By analogy with white-without-pressure fundus lesions, we have called these areas dark-without-pressure.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
3/15. Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease.patients with severe acute chest syndrome (ACS) requiring endotracheal intubation and erythrocytopheresis are at increased risk for neurologic morbidity. This study examines patients with sickle cell disease who developed severe episodes of ACS, leading to endotracheal intubation, ventilatory support for respiratory failure, and erythrocytapheresis. magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) studies, a neurologic examination by a pediatric neurologist, and cognitive testing were done in all patients. Five consecutive patients, aged 3 to 9 years, were identified with severe ACS. All patients developed neurologic complications resulting from ACS episodes, including seizures (n = 2), silent cerebral infarcts (n = 3), cerebral hemorrhage (n = 2), and reversible posterior leukoencephalopathy syndrome (n = 3). Children with severe ACS should have a magnetic resonance image of the brain, neurologic examination by a neurologist, and cognitive testing to detect the presence of neurologic morbidity.- - - - - - - - - - ranking = 43650.127737852keywords = leukoencephalopathy syndrome, posterior leukoencephalopathy syndrome, posterior leukoencephalopathy, leukoencephalopathy, posterior (Clic here for more details about this article) |
4/15. Sickle cell disease and aneurysmal subarachnoid hemorrhage.While neurological complications are common in sickle cell disease, aneurysmal subarachnoid hemorrhage has been rarely reported. A case is presented of a young man with sickle cell disease and subarachnoid hemorrhage found to have two mid-basilar aneurysms, the largest of which had bled. Available literature suggests that this patient may be quite representative as nearly one half of documented cases have been noted to harbor multiple aneurysms, and posterior circulation involvement appears to be common. These anatomic features may relate to the pathophysiology of arterial destruction common in sickle cell disease.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
5/15. Penile prostheses in the management of impotence in sickle cell disease.priapism lasting more than 24 h in adults with homozygous sickle cell disease usually results in impotence. The dense fibrosis of the corpora cavernosa which destroys the normal vascular erectile system of the penis also complicates insertion of penile prostheses. Our experience in 5 patients is reviewed. Sharp dissection and dilatation were necessary to form a tunnel for the prosthesis, which was more easily excavated anteriorly and posteriorly through a dorsal incision than for the length of the corpora through a traditional perineal-scrotal incision. Damage to the tunica was common and subsequent migration of the prosthesis necessitated an additional 11 procedures under general anaesthesia. Despite these technical difficulties, the procedure allowed satisfactory intercourse. The average interval between onset of impotence and implantation was 4 years. Early implantation before the dense fibrosis develops might give more satisfactory results.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
6/15. Epiretinal membranes in sickle cell retinopathy.We examined the hospital records (including fundus photographs) of 769 patients (1486 eyes) with sickle cell disease from June 1978 to July 1985 for evidence of epiretinal membranes in the macula (51 eyes) or extramacular region (four eyes) of the posterior pole. The incidence of epiretinal membranes, seen in the eyes of patients with hemoglobin SC, SS, and SB thalassemia, was 3.7%. Most epiretinal membranes were mild, and visual acuity was 20/40 or better in 78.2% of these eyes. Severe macular pucker was occasionally noted following retinal detachment surgery. One patient with SC disease developed a macular epiretinal membrane that led to a macular hole and eventually to macular and extra-macular retinal detachment. Epiretinal membranes are a relatively common cause of maculopathy in patients with sickle cell disease.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
7/15. Choroidal occlusive disease in sickle cell hemoglobinopathies.Two distinct episodes of posterior ciliary artery occlusion were studied in a 32-year-old man with hemoglobin SS disease and multiple episodes of amaurosis fugax. Although posterior ciliary artery occlusions have been observed following photocoagulation of sickle cell retinopathy, their spontaneous evolution in patients with sickling hemoglobinopathies has received little attention. The manifestations of posterior ciliar artery occlusion seen in this case and in other clinical and experimental situations are reviewed. Histopathologic examination of three additional eyes of patients with sickle hemoglobinopathies revealed changes which may have been the result of previous small posterior ciliary artery occlusions or small vessel occlusive disease related to the sickling hemoglobinopathies; these cases are also reported.- - - - - - - - - - ranking = 4keywords = posterior (Clic here for more details about this article) |
8/15. Retinal damage from a Q-switched YAG laser.A 42-year-old woman with sickle cell anemia and proliferative retinopathy underwent neodymium-YAG laser therapy for a taut posterior hyaloid membrane causing peripapillary and peripheral traction detachment of the retina. vitrectomy was not done because the patient required anticoagulation. A Q-switched YAG laser was capable of cutting holes in the taut membrane, but treatment 2 to 3 mm from the retina resulted in microperforation of a retinal vein and focal areas of damage to the retinal pigment epithelium. The damage to the retinal pigment epithelium was not immediately apparent, and ophthalmoscopically visible lesions were seen only when the patient was reexamined 48 hours later.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
9/15. Intrathoracic extramedullary hematopoiesis: report of a case in a patient with sickle-cell disease-beta-thalassemia.A case of paravertebral extramedullary hematopoiesis in a patient with sickle-cell anemia-beta-thalassemia is reported. Interesting aspects in this case, including the high level of Hb F and large number of nucleated erythrocytes in the peripheral blood, are discussed. Fifty-five cases of intrathoracic extramedullary hematopoiesis have been reported. Most of these cases occurred in patients with thalassemia or hereditary spherocytosis. Extramedullary hematopoiesis should be considered in the differential diagnosis of a posterior mediastinal mass in a patient who has chronic anemia or other syndromes associated with extramedullary hematopoiesis.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
10/15. Reversal of retinal and optic disc ischemia in a patient with sickle cell trait and glaucoma secondary to traumatic hyphema.A 14-year-old black boy with sickle cell trait, who sustained a traumatic hyphema, developed moderately elevated intraocular pressure that failed to respond to carbonic anhydrase inhibitors and osmotic agents. On the tenth postinjury day, a sudden increased cupping of the optic disc and partial central retinal artery obstruction caused painless loss of vision. Reversal of the cupping, the retinal ischemia, and the intraocular pressure was documented following anterior chamber paracentesis, and visual acuity returned to 6/6. Pathophysiology of the posterior ischemia is discussed. This case documents the potentially debilitating course of traumatic hyphema in "benign" sickle cell trait and its avoidance with proper management. The authors endorse recent suggestions for careful observation of any sickle cell patient with traumatic hyphema, and recommend anterior chamber paracentesis, supplemental oxygen, and avoidance of osmotic agents, if secondary glaucoma develops following the initial trauma.- - - - - - - - - - ranking = 1keywords = posterior (Clic here for more details about this article) |
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