Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female) and 2 (a 54-old-female) presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male) showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male) had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.
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A case of pulmonary arteriovenous fistula in a 8-year-old boy who presented with easy fatigability and cyanosis for 4 months, is described. Grossly, there was a large vascular anomaly measuring 2x2 cm in the center of the removed right lower lobe. Microscopically, the lesion consisted of dilated arterial and venous structures resembling a cavernous hemangioma. Subintimal fibrosis and attenuated vascular structure lacking elastic fiber and representing A-V shunt were also noted in the lesion.
Diffuse neonatal hemangiomatosis is an uncommon disease that is characteristed by a diffuse nature of the lesions, and distinguished form a single or few, or superficial or deep, capillary, cavernous, or mixed hemangiomas occuring in early or adult life. We report an autopsy case of multiple hemangiomatosis, which is associated with massive osteolysis of right humerus and arteriovenous fistulae in surrounding soft tissues. The patient is a 23 day old female infant and had a 2.0x1.5 cm sized cystic destructive bony lesion which was located in the proximal shaft of right humerus. Right arm was hypertrophied, compared to the normal looking left.
There were multiple hemangiomas in right humerus, lung, cutaneous skeletal muscles and nerves. This case shares clinical characteristics of Gorham's disease.