Unknown case: Part 2

摘要

The investigations confirmed superior mesenteric artery (SMA) syndrome, a rare but well-described complication of scoliosis. In Figure 2, note how there is a clear cutoff inflow of contrast along the third part of the duodenum; and in Figure 3, note the serpentine route of the duodenum under the SMA and over the abdominal aorta. First described by Carl Freiherr von Rokitansky1 in the 1800s, SMA syndrome is a rare and life-threatening disorder characterized by compression of the third part of the duodenum between the aorta and the overlying SMA. The pathophysiology of this condition is subject to debate, but general consensus is that retroperitoneal fat usually acts as a "cushion" between the SMA and duodenum and loss of this fat can lead to a reduction in the angle between the SMA and the abdominal aorta, resulting in compression of the duodenum. This can lead to a variety of upper gastrointestinal symptoms including, abdominal cramping, nausea, bilious vomiting, and early satiety. In severe cases, weight loss and severe malnutrition can result. This condition is associated with a high rate of morbidity and mortality with an estimated death rate of 1 in 3 patients.