Presentation
Persistent and chronic headaches with vomiting.
Patient Data
Extensive diffuse thickening of diploic space of the skull vault extending to the skull base bones with no obvious space occupying lesions. The paranasal sinuses and the mastoid air cells are clear. The ventricular systems, sulci/ gyri pattern, Sella and the pituitary are normal and there is no infarction or vascular thrombosis. Visualized cervical spine, cranio-cervical junctions are uneventful.
Grey scale and color Doppler mapping out of the abdominal viscera shows normal right and the left liver lobes, normal pancreas, and both kidneys, the major abdominal vasculature and the urinary bladder. However, the splenic organ is not visualized within the entire abdominal-pelvic spaces. Mild homogenous low level echo dense fluid is noted within the gall bladder neck suggesting non-obstructive and non-calculus sludge content.
Case Discussion
Elaborately thickened diploic space of the skull bone reminiscing hair-on- end appearance and extending to the skull bones with no obvious masses favoring extramedullary hematopoiesis skull, most likely attributable to the current underlying sickle cell disease hemoglobinopathy or any types of chronic hemolytic disorders asmanifestations of extra-medullary hematopoiesis. The sonographic absence of the splenic organ suggests autosplenectomy as no previous history of surgical intervention was noted.
Other plausible radiological mimickers of this illustration may include hyperostosis or thalassemia
NB: No hydrocephalus CT features.
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