Isolated amyloidosis of the gastro-intestinal tract.

Amyloidosis is a very rare condition, which, due to its rarity, is often missed or diagnosed in an advanced stage of the disease, causing significant morbidity and mortality. In this review we describe the existing types of amyloidosis focusing on the gastro-intestinal tract. Amyloidosis occurs when abnormal protein fibrils (amyloid) deposit in the muscularis mucosae. This can cause an array of symptoms ranging from (in order of occurrence): gastro-intestinal bleeding, heartburn, unintentional weight loss, early satiety, constipation, diarrhea, nausea, vomiting and fecal incontinence (1). Treatment is focused on the underlying condition (if any) causing the production and deposition of the abnormal fibrils, in combination of symptomatic treatment.

Thrombosis of an Arteria Lusoria with Secondary Subclavian Steal Syndrome and Swallowing Difficulties.

We report a case of an arteria lusoria causing swallowing difficulties known as dysphagia lusoria. Although the presence of an arteria lusoria is quite common, dysphagia lusoria is relatively rare. Interestingly, our patient also presented with a concurrent aneurysmal dilatation, known as a Kommerell's diverticulum, at the aortic origin. Complete thrombosis of the artery and flow reversal in the right cervical artery resulting in an asymptomatic subclavian steal syndrome was also seen. No underlying primary pro-thrombotic defects were identified but due to the presence of locally advanced prostate cancer, a paraneoplastic phenomenon was suspected.

Hemangiomatosis of the spleen in a patient with Klippel-trénaunay syndrome.

Klippel-Trenaunay syndrome is a rare disorder characterized by a triad of port-wine stains, varicose veins, and bony and soft tissue hypertrophy usually involving an extremity. Visceral involvement in Klippel-Trénaunay syndrome is rare, but has been described in the colon, small bowel, bladder, kidney, spleen, liver, mediastinum and brain. In this paper we present the case of a 45-year-old woman with Klippel-Trenaunay syndrome in whom routine physical examination unexpectedly revealed the presence of a left upper quadrant mass. Abdominal US, contrast enhanced CT and whole-body PET-CT demonstrated multiple large cystic lesions within a massively enlarged spleen. Based on the clinical history and imaging findings diffuse hemangiomatosis of the spleen was suspected. This diagnosis was confirmed by pathologic examination after splenectomy.