PMC4588676_01
(A) Oblique coronal US image of the right renal region showing a heterogenous mass with a large echogenic fatty component and a less echogenic soft-tissue component with prominent vessels within it (arrow). (B, C) Oblique coronal color Doppler US image in the right and left renal region respectively, showing highly vascular fatty masses. (D) Subcostal US scan of the liver showing presence of small echogenic lesions in both lobes consistent with small angiomyolipomas.
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Emergency ultrasound (US) of the abdomen revealed multiple heterogeneously hyperechoic masses occupying the whole abdomen, showing internal tortuous vessels with aneurysmal dilations at places which were seen on color Doppler scans (Figure 1A-1C). The mass on the right side showed a large non-fatty soft tissue component along its superior aspect (Figure 1A). The liver revealed a few small well-defined hyperechoic lesions (Figure 1D).
(A, B) Coronal and axial non-contrast-enhanced CT scans showing massive bilateral predominantly fatty renal masses consistent with angiomyolipomas. (C, D) Axial non-contrast-enhanced CT of the brain showing calcified subependymal nodules (arrows).
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Those lesions measured 29x27.5x15.5 cm on the right side and 30x19x13 cm on the left side (Figure 2A, 2B). The urinary bladder showed hyperdense fluid with attenuation of 30-40 Hounsfield units suggestive of hemorrhagic contents. Multiple calcified subependymal nodules were noted in the brain on CT (Figure 2C, 2D), and thorax CT revealed variable-sized air-filled cysts throughout the parenchyma in both lungs (Figure 3) consistent with lymphangioleiomyomatosis (LAM).
Axial (A, B), coronal (C) and sagittal (D) CT images of thorax in lung window showing presence of multiple variable sized air filled cysts consistent with LAM.
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Multiple calcified subependymal nodules were noted in the brain on CT (Figure 2C, 2D), and thorax CT revealed variable-sized air-filled cysts throughout the parenchyma in both lungs (Figure 3) consistent with lymphangioleiomyomatosis (LAM).
(A) Coronal CT image of the skull, (B, C) sagittal CT images of spine showing multiple patchy sclerotic lesions throughout all bones.
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Multiple small ill-defined sclerotic lesions were seen throughout scanned axial skeleton including the skull, vertebrae and ribs (Figure 4).
Clinical photographs of the patients showing. (A) adenoma sebaceum, (B) dental pitting (arrowhead) and gingival fibromas (arrows), (C, D) subungual fibromas (arrows).
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On examination typical facial angiofibromas (adenoma sebaceum) (Figure 5A) were present over the face along with a few hypopigmented macules and patches over the trunk. There was presence of dental pitting over a few teeth in the upper jaw (Figure 5B) along with multiple periungual and gingival fibromas (Figure 5B-5D).