Chronic Visceral Artery Obstruction Associated with Hypoplastic Aorta in a Pair of Monozygotic Twins

Article Outline

• Abstract
• Introduction
• Report
• Discussion
• References
• Copyright

We report a case of chronic visceral artery obstruction associated with a hypoplastic aorta in a pair of monozygotic twins. They had suffered from abdominal angina and intermittent claudication. The younger twin underwent simultaneous visceral artery reconstruction and right common iliac-femoral artery bypass at age 30. The older twin underwent a visceral artery reconstruction at age 34, 4 years after bilateral common iliac-femoral artery bypass. We used a temporary shunt in order to ensure perfusion for the visceral organs, because there was a rich collateral circulation to visceral organs from the inferior mesenteric artery (IMA) and the internal iliac arteries.

Keywords: Arterial occlusive diseases, Mesenteric vascular occlusion, Monozygotic twins

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Introduction 

Hypoplastic aorto-iliac syndrome (HAIS) is not uncommon and comprises 5–9% of the patients with aortoiliac occlusive disease.¹ The etiology of HAIS, however, remains controversial. We report a pair of male monozygotic twins with hypoplastic aorta who underwent successful surgery for chronic visceral and aortoiliac occlusive disease.

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Report 

A pair of male monozygotic twins were diagnosed with chronic visceral artery obstruction associated with a hypoplastic aorta. They had suffered from intermittent claudication since childhood. Recently they began to experience abdominal angina during exercise and after meals. Computed tomography (CT) showed general hypoplastic aorta with premature severe calcification. Aortography demonstrated celiac artery (CA) occlusion, superior mesenteric artery (SMA) stenosis, and hypoplastic aorto-iliac system with occlusive lesion for the younger twin. CA stenosis, SMA occlusion and iliac artery lesion were confirmed for the elder twin (Fig. 1, A). Neither twin exhibited any congenital cardiac abnormality. They had a normal renal function and had no atherosclerotic risk factors such as hypertension, diabetes mellitus, hyperlipidemia and cigarette smoking. Their parents and the older brother did not have a history of cardiovascular disease. They were referred to our department for surgical correction.

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• Fig. 1 

  A. Preoperative aortography demonstrating hypoplastic aorta. Collateral circulation are noted due to severe stenosis of the celiac artery (arrow) and the SMA (arrow head). The origins of the celiac artery and the SMA cannot be identified. B. Postoperative CT showing bypassed celiac artery (arrow) and SMA (arrow head). Bilateral common iliac-femoral artery bypass are also observed (asterisk).

The younger twin underwent simultaneous visceral artery reconstruction and right common iliac-femoral artery bypass at age 30. Median laparotomy was followed by medial mobilization of left abdominal viscera behind the kidney for exposure of the aorta. The abdominal aorta was approximately 15mm in diameter throughout its length. The aorta was cross-clamped at both sides of the origin of the celiac trunk. A temporary shunt was established in order to ensure perfusion for the visceral organs. We used a 10F right-angle cannula placed in the aorta just proximal to the clamp forceps, and its other end inserted in a 6mm Dacron graft which was anastomosed to the right common iliac artery in an end-to-side fashion. The origin of the celiac trunk was removed and a 12-6mm bifurcation graft was attached to the aorta with end-to-side anastomosis. CA and SMA reconstruction was completed by graft bypass. The histologic examination of the celiac trunk revealed atherosclerotic changes with no inflammatory reaction.

The older twin went through visceral artery reconstruction at age 34, which was 4 years after bilateral common iliac-femoral artery bypass. We performed the same operation using the same temporary shunt except that the distal side of the shunt was placed at the aortic bifurcation in the second case (Fig. 2, A,B). Postoperative CT showed the patent bypass (Fig. 1, B). Both men are now free from abdominal angina.

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• Fig. 2 

  A. Arrow, celiac artery. arrow head, SMA. asterisk, left renal vein. B. Celiac artery (arrow) and SMA (arrow head) bypassed with 12-6mm bifurcation graft.

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Discussion 

To our knowledge we report the first case of surgical intervention for chronic visceral artery obstruction associated with hypoplastic aorta in a pair of monozygotic twins. The symptoms and progressive calcification of the aorta warranted an aggressive surgical approach. We paid close attention to visceral perfusion during reconstruction of the CA and the SMA. The temporary shunt was effective because there was a rich collateral circulation to visceral organs from the IMA and the internal iliac arteries.

Hypoplastic aorto-iliac syndrome (HAIS) is a well-defined disease characterized by a rapid tapering of the distal aorta and iliac arteries together with a high position of the aortic bifurcation and prominent bifurcating lumber arteries at the lowest level. These features describe the congenital origin in which aortic hypoplasia may result from embryonic overfusion of the dorsal aorta.¹, ² In contrast, there is a literature that supports an acquired process.³ They suggest that the term “hypoplastic” be replaced by “small” because of the preponderance of middle-aged women and late occurrence of the symptoms. In the literature 7 out of the 9 patients in hystologic study presented lymphoplasmacellular infiltration in the media and the adventitia, mainly around the vasa vasorum. This fact indicates a possible role of inflammatory diseases such as Takayasu's aortoarteritis. Whatever the cause, premature atherosclerosis is observed at the aortic bifurcation and the iliac arteries with relative absence of generalized atherosclerotic disease. This feature is attributed to the hemodynamic stress caused by the abnormal vessel dimension with a smaller ratio of the sum of the cross-sectional areas of the common iliac arteries to that of the aortic bifurcation.¹ In our cases the entire abdominal aorta was hypoplastic, not just the infrarenal portion and demonstrated atherosclerosis with calcification. Aside from the aortic bifurcation and iliac arteries, suprarenal segment of the aorta and visceral arteries were also complicated by atherosclerosis with severe calcification.

These features are in striking contrast to the previously reported cases.¹, ², ³, ⁴, ⁵ Our cases could be a variation of HAIS and if that is the case, they should contribute to the congenital etiological theory.

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References 

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