Samir S. Hussein

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.

SPLENIC FUNCTION IN OMANI CHILDREN WITH SICKLE CELL DISEASE: Correlation with Severity Index, Hemoglobin Phenotype, Iron Status, and α -Thalassemia Trait

The prevalence of functional asplenia in Omani children with sickle cell disease (SCD) has not been previously defined. In this study, the authors aim to compare the natural history of splenic dysfunction in their patients to other reports. The splenic function was studied in 72 Omani patients with sickle cell disease (50 homozygous for hemoglobin S (HbS-S), 11 double heterozygotes for HbS and g 0 -thalassemia (HbS- g 0 -thal), 5 HbS- g + -thal, 5 patients with hemoglobin S-D disease, and 1 child with hemoglobin S oman trait) aged 4.8-16 years, using 99m Tc-labeled tin colloid scintigraphy. The study revealed 4 groups according to their colloid uptake: group I included 20 patients (28%) with normal splenic function; group II, 6 patients (8%) with mild hyposplenism; group III, 20 (28%) with severe hyposplenism; and group IV, 26 (36%) patients with functional asplenia. Overall, more than 60% of them had preserved splenic function. Except for HbS- g + patients, the developmental pattern of hyposplenism was not different among the different Hb phenotypes. Factors associated with preservation of spleen function in these patients were larger splenic size ( p < .01), less clinical severity ( p < .05), lower MCH ( p < .01), higher HbF ( p < .001), and presence of f -thalassemia trait ( p < .05).

Catecholamine-secreting carotid body paraganglioma: successful preoperative control of hypertension and clinical symptoms using high-dose long-acting octreotide

Summary A 48-year-old hypertensive and diabetic patient presented with a 10-year history of progressive right facial pain, tinnitus, hearing loss, sweating, and palpitations. Investigations revealed a 5.6 cm vascular tumor at the carotid bifurcation. Her blood pressure (BP) was 170/110, on lisinopril 20 mg od and amlodipine 10 mg od and 100 U of insulin daily. A catecholamine-secreting carotid body paraganglioma (CSCBP) was suspected; the diagnosis was confirmed biochemically by determining plasma norepinephrine (NE) level, 89 000 pmol/l, and chromogranin A (CgA) level, 279 μg/l. Meta-iodobenzylguanidine and octreotide scanning confirmed a single tumor in the neck. A week after giving the patient a trial of octreotide 100 μg 8 h, the NE level dropped progressively from 50 000 to 25 000 pmol/l and CgA from 279 to 25 μg/l. Treatment was therefore continued with labetalol 200 mg twice daily (bid) and long-acting octreotide-LA initially using 40 mg/month and later increasing to 80 mg/month. On this dose and with a reduced labetalol intake of 100 mg bid, BP was maintained at 130/70 and her symptoms resolved completely. CgA levels returned to normal in the first week and these were maintained throughout the 3 month treatment period. During tumor resection, there were minimal BP fluctuations during the 10 h procedure. We conclude that short-term high-dose octreotide-LA might prove valuable in the preoperative management of catecholamine-secreting tumors. To the best of our knowledge, this is the first report on the successful use of octreotide in a CSCBP. Learning points The value of octreotide scanning in the localization of extra-adrenal pheochromocytoma. Control of catecholamine secretion using high-dose octreotide. This is a report of a rare cause of secondary diabetes and hypertension.

All-trans retinoic acid-induced myositis

An 18-year-old girl was diagnosed with acute promyelocytic leukaemia. She was started on standard therapy with all-trans retinoic acid (ATRA) and idarubicin. While on ATRA, she developed a painful swelling of the left thigh accompanied by high-grade fever and grossly elevated C-reactive protein. Blood cultures were negative. The fever did not settle with a combination of broad-spectrum antibiotics. Ultrasound Doppler examination was negative for venous thrombosis. A technetium-tagged leucoscan was performed to identify any focus of infection. It showed increased uptake in the left thigh muscle (white arrow, top left). This was followed by magnetic resonance imaging of the left thigh, which showed marked oedema of skin and subcutaneous tissue and the adductor muscles (arrows, top right). A gadolinium-enhanced T1-weighted transverse image of the left thigh showed marked muscle necrosis (arrow, bottom left). As ATRA is known to cause myositis, it was discontinued temporarily and dexamethasone was commenced. Within 24 h of initiation of dexamethasone, marked improvement in the thigh swelling was observed with normalisation of temperature. Dexamethasone was tapered after complete resolution of the thigh swelling and ATRA was recommenced without any further toxicity. A repeat nuclear imaging (bottom right) confirmed normal appearance with no increase in uptake. This case highlights a rare cytokine-mediated toxicity of ATRA involving the muscles, which is eminently treatable with dexamethasone, similar to ATRA-induced pulmonary toxicity.

Frontotemporal dementia in Oman: Cognitive behavioural profile and neuroimaging characteristics; A prospective hospital-based study

Frontotemporal dementia is increasingly recognised as an important cause of early-onset dementia and is considered to be the second commonest neurodegenerative dementia after Alzheimers disease. We describe the cognitive, behavioural profile and neuroimaging characteristics of 6 patients with frontal variant of Frontotemporal dementia that were evaluated at the cognitive behavioural clinic at this tertiary referral teaching hospital. All patients underwent clinical, neuropsychological, structural/functional neuroimaging, and laboratory evaluations. The male to female ratio was 1:1; mean age of onset was 54 years, and the mean duration of symptoms were 30 months. The mean scores for Addenbrookes cognitive examination, Frontal Assessment Battery, and Mini-Mental State Examination were 70.5, 6.33 and 23.6 respectively. The mean VLOM ratio was 2.04. MRI revealed significant asymmetrical regional frontal/temporal atrophy supplemented by the evidence of circumscribed hypoperfusion in SPECT imaging. We conclude that a combination of behavioural and cognitive assessment using short bedside tests, along with structural and functional neuroimaging does facilitate early identification, and increase the diagnostic specificity of Frontotemporal dementia.

Mucinous Breast Cancer with Solitary Metastasis to Humeral Head: A Case Report

Breast cancer is the most common cause of metastatic deposits in the skeleton, and bone is the most common site of recurrence of breast cancer. Breast cancer metastasis most commonly affects the spine, ribs, pelvis, and proximal long bones; however, only 3.5% of breast cancer patients develop long-bone metastases. The humerus is the most common upper-extremity site for bony metastasis, and pathologic fractures can result. The patient in the current study presented with breast cancer and discovered to have humeral head metastasis during initial workup. The dilemma was in investigation the modality to confirm humeral head metastasis as there are many differential diagnoses with similar findings. After staging workup, the patient was treated with neoadjuvant chemotherapy followed by modified radical mastectomy and radiotherapy of the chest wall and the shoulder. The lesion in humerus was well healed.