ABSTRACT
Vascular Ehlers-Danlos Syndrome (EDS) causes fragility of the blood vessels, skin, and hollow organs, and the most common clinical feature is vascular rupture. We report a surgical case of a spontaneous ruptured dissection of the left common iliac artery (CIA) in a patient with vascular EDS. A 27-year-old man with documented vascular EDS with a mutation of the collagen type III alpha-1 [COL3A1] gene, presented with acute left lower abdominal pain. The enhanced computed tomography (CT) scan revealed a ruptured dissection of the left CIA with extravasation, and left iliopsoas hematoma. The enhanced CT performed 1 month previously showed no arterial aneurysm. The left CIA was anastomosed end-to-end using a 7 mm heparin-bonded polytetrafluorethylene (PTFE) graft (Fusion, MAQUET Cardiovascular LLC, USA). A PTFE graft was anastmosed end-to-side to the left external iliac artery (EIA). The distal side of the CIA was closed. The postoperative enhanced CT showed aneurysmal formation in the right inferior gluteal artery. The follow-up CT showed enlargement of the left EIA (from 7.5 to 12 mm in a diameter), and aneurysm formation in the proper hepatic artery. Therefore, a close follow-up based on noninvasive imagings is mandatory to detect life-threatening vascular complecations in patients with vascular EDS.
ABSTRACT
A 21-year-old woman found to be hypertensive was referred for hypertension. On examination, blood pressure was 170/110mmHg in the right arm, 160/100mmHg in left arm,and 120/70mmHg in legs. A grade 2/6 systolic ejection murmur was present at the left upper sternal border, and a chest x-ray revealed a rib notching on the inferior margin of 4th rib. Two-dimensional echocardiogram showed the coarctation of aorta beyound the origin of the left subclavian artery. Biplane TEE demonstrated a discrete narrowing of the descending aorta at the site of coarctation. The blood pressure was 169/86mmHg in ascending aorta and 118/84mmHg in descending aorta. Aortogram showed a localized coarcted aortic segment of 7mm in diameter and 5mm long just distal to the left subclavian artery. Balloon coarctation angioplasty was performed with 7F 30x15mm pediatric balloon dilatation catheter. Balloon position was confirmed on fluoroscopy by the hourglass appearance of the balloon inflation and thereafter, the balloon was inflated until the waist of the balloon disappeared. After procedure, a pull back pressure tracing across the coarctation of aorta revealed no pressure gradient between ascending and descending aorta with 141/90mmHg. Aortogram showed an increase in diameter of the coarctation of aorta to 18mm with aneurysmal formation. 3 months later, follow up aortogram showed no significant change in diameter of coarctation of aorts or aneurysmal formation. Nonsurgical balloon coarctation angioplasty appears to be an alternative therapy for the coarctation of aorta in adults.