Ranjan William

Orbital involvement in sickle cell disease: A report of five cases and review literature

Purpose To present five cases of orbital infarction in sickle cell disease and review relevant literature.Method We reviewed the hospital records of 5 patients with sickle cell disease who developed a periorbital swelling during a vaso-occlusive crisis and were managed at our hospital between April 1992 and June 2000.Results The 5 patients (4 with homozygous sickle cell disease and 1 with sickle cell-β-thalassaemia disease) were aged 6-15 years with a history of multiple admissions for vaso-occlusive crises. The periorbital swelling spread to the orbit in 4 cases and resulted in proptosis (2 cases), restriction of ocular motility and visual impairment. In all 4 cases, computed tomography and/or magnetic resonance imaging of the orbits showed a mass adjacent to the orbital wall. In 2 cases the mass was identified as a haematoma. Orbital wall infarction was demonstrated in 3 cases by bone/bone marrow scintigraphy. Epidural haematomas were detected by computed tomography in one case. All patients received intravenous fluids, analgesics, broad spectrum antibiotics and steroids, as well as simple or exchange transfusion, and responded well to medical management.Conclusions Infarction of orbital bones during vaso-occlusive crises in sickle cell disease presents acutely with a rapidly progressive periorbital swelling. Haematomas frequently complicate the condition and, along with the inflammatory swelling, may lead to orbital compression syndrome. The condition is therefore sight-threatening, and necessitates prompt diagnosis and appropriate management for resolution without adverse sequelae. Imaging techniques are invaluable in the evaluation of patients. The majority of cases resolve with conservative treatment that includes steps to combat the vaso-occlusive crisis and use of systemic steroids under antibiotic cover.

Orbital Infarction in Sickle Cell Disease

PURPOSE To determine the role of hematological and genetic factors in the development of orbital infarction in sickle cell disease. DESIGN Retrospective, noncomparative case series. METHODS Fourteen sickle cell disease patients were diagnosed with orbital infarction during a vaso-occlusive crisis. Clinical and radiological findings were reviewed retrospectively. Sickle cell disease patients without orbital infarction were recruited as controls after matching for disease severity. Sickle haplotypes were determined for all patients. Differences between groups were evaluated statistically. RESULTS Patients with orbital infarction in sickle cell disease presented with acute periorbital pain and swelling with or without proptosis, ophthalmoplegia, and visual impairment during a vaso-occlusive crisis. Radiological findings included orbital soft tissue swelling (100%), hematoma (orbital, 36%; intracranial, 21%), and abnormal bone marrow intensities. Severity of orbital involvement was unrelated to that of the systemic disease (Pearson correlation coefficient, -0.1567). Affected patients predominantly had the Benin haplotype (P < .00782). CONCLUSIONS Orbital infarction is a potential threat to vision in sickle cell disease patients. Magnetic resonance imaging is more specific than computed tomography or nuclear scintigraphy in the evaluation of orbital changes. The degree of severity of the orbital manifestations appears unrelated to the severity of sickle cell disease. Patients with the Benin haplotype are more likely to develop orbital infarction during vaso-occlusive crises.

Thoracic Ectopic Kidney in an Adult

We are presenting an adult patient with a rare form of renal ectopy. Our patient had a thoracic kidney with complications. He presented with symptoms unrelated to the urinary system and his chest x-ray showed an intrathoracic mass presenting just above the diaphragm. An excretory urogram may be necessary for the diagnosis in such patients with similar chest x-ray findings.

Hydroxyurea or chronic exchange transfusions in patients with sickle cell disease: role of transcranial Doppler ultrasound in stroke prophylaxis.

Hydroxyurea is increasingly used in patients with sickle cell disease and acts by several underlying mechanisms. Its usage has been reported to eliminate transfusion needs in children with beta-thalassemia major. It has also been used in sickle cell disease patients on stroke prophylaxis with exchange transfusions who develop transfusional iron overload and is now being considered as a possible alternative to chronic transfusions for secondary stroke prophylaxis. Our case demonstrates a pitfall when using hydroxyurea without monitoring intracranial cerebral vessels for vasculopathic changes; presence of which can predict stroke risk and alert the need for chronic blood transfusions as primary stroke prophylaxis. Transcranial Doppler is a crucial investigation that can reveal elevated cerebral arterial flow velocities.

Transcranial Doppler Ultrasonography in Sickle Cell Disease: A Study in Omani Patients

Changes on Transcranial Doppler (TCD) ultrasonography have been proposed as significant predictors of cerebrovascular complications in sickle cell disease (SCD). However, consensus with regards to the TCD criteria to recognize abnormalities in cerebral vasculature is lacking. We studied the TCD characteristics of cerebral arteries among Omani patients with SCD and correlated them with cerebrovascular events. TCD was performed through the temporal and suboccipital windows using a 2 MHz probe (DWL). Thirty-three of 59 patients (56%) with SCD had neurological symptoms including stroke—12 (20%) and epilepsy—7. Fifteen patients (25%) had significant TCD abnormalities including: markedly increased velocities—11 (3 with stroke); turbulent flow—2; and reversal of flow—2. No patient had a time averaged maximum mean velocity of >200 cm/s in anterior circulation. On applying a modified definition of “abnormal TCD” to anterior and posterior circulation studies, increased TCD velocities in posterior circulation correlated with history of stroke (P<0.05). TCD velocities in the 18 adult patients ( older than 15 y) were significantly lower than in children. Logistic regression analysis revealed abnormal TCD in the left posterior cerebral artery to be an independent predictor of stroke in this cohort (P=0.035).

Lower Cranial Nerve Palsies

A 70 year old diabetic male presented with a three weeks history of left temporal pain radiating to the left eye and left side of the neck without any symptoms relating to the ear or nose. Two days later, the patient developed difficulty in swallowing and speaking with nasal regurgitation of fluids. Clinical examination revealed bilateral palatal weakness (more marked on the left side), bilateral weakness of sternocleidomastoids and bilateral weakness of tongue muscles (more marked on the right side). The gag reflex was diminished. There were no pyramidal or cerebellar signs and there was no neck stiffness. MRI of the brain showed bilateral otitis media with involvement of the petrous temporal bone. In addition, the patient had osteomyelitis of the clivus with increased uptake upon gallium scanning. He was treated with intravenous ceftriaxone 4 grams daily for three weeks, moxifloxacin 400 mgm daily and voriconazole 400 mgm daily for three months. The symptoms improved, but the patient was left with tongue weakness, more marked on the right side.