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case report

Indian Pediatr 2013;50: 507-508

Marfan Syndrome with Spontaneous Rupture of Aneurysm of Common Iliac Artery


Soumya Patra, Ajit Pal Singh and BC Srinivas

From Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India.

Correspondence to: Dr Soumya Patra, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bannerghatta Road, Bangalore, Karnataka 560 069, India.
Email: [email protected]

Received: November 2, 2012;
Initial review: November 26, 2012;
Accepted: December 06, 2012.


 


Marfan syndrome rarely presents with peripheral artery and pulmonary artery aneurysms. We are presenting a case of a fifteen-year-old boy who presented to us with progressive lower abdominal pulsatile swelling with bruit in the right inguinal region for one month. He had typical marfanoid habitus, though there was no history of similar illness in family. CT angiogram revealed the presence having giant right common iliac aneurysm along with interlobar pulmonary artery aneurysm. He had spontaneous rupture of aneurysm in right common iliac artery.

Key words: Aneurysm; Iliac artery; Marfan syndrome; Pulmonary artery.

Marfan syndrome is an autosomal dominant, multisystem disease characterized by skeletal deformity like long bone overgrowth and other structural abnormalities, such as dislocation of the ocular lens, pneumothorax, mitral valve prolapse, and aortic arch dilatation [1]. Isolated iliac artery aneurysms are rare in the general population (0.03%) and represent 2% of all abdominal aortic aneurysms [2]. This association with Marfan syndrome is even rare. Pulmonary artery dilation is a minor cardiovascular criterion in the diagnosis of Marfan syndrome and very rarely seen [1]. We are reporting this case who had aneurysm of both pulmonary (right inter lobar) and right common iliac artery; along with sudden and spontaneous rupture of right common iliac artery.

Case Report

A 15-years-old boy presented to us with progressive swelling of lower abdomen for last one month. There was no history of fever, dysurea, hematuria, and dyspnea or chest pain. Examination revealed marfanoid habitus with reduced upper and lower segment ratio or arm span to height ratio greater than 1.05, presence of wrist and thumb sign, arachnodactyly, clinodactyly, pectus carinatum, scoliosis and high arched palate. Ocular examination didn’t reveal presence of ectopia lentis or other ocular disorder. Cardiovascular, respiratory and central nervous system examination was within normal limit. Per abdominal examination revealed presence of pulsatile mass in the pelvic area along with bruit in the right inguinal region. Peripheral pulses were equally palpable in the both the limbs. Routine blood investigation and chest x-ray was within normal limit. Echocardiography was also found to be normal. CT angiogram revealed the presence of giant aneurysm of right common iliac artery (Fig. 1) and peripheral pulmonary artery aneurysm of right inter lobar artery. Though he was posted for endovascular repair with stenting but suddenly he developed spontaneous rupture of common iliac artery with disappearance of abdominal swelling and increasing pallor. Finally he succumbed to his illness.

Fig. 1 CT peripheral angiogram showed giant aneurysm of right common iliac artery.

Discussion

The most common cardiovascular abnormalities in Marfan syndrome include aortic and mitral valvular disease along with aneurysm and dissection of ascending aorta. Aneurysm in other location is uncommon in Marfan syndrome [4]. Though there are several case reports of patient of Marfan syndrome having common iliac artery aneurysm [4-6] in the literature but presence of pulmonary artery aneurysm is very rare with approximately eight cases per 100,000 autopsies [1]. However, there is no reported case so far which is showing presence of aneurysm in both peripheral and pulmonary artery. In our patient we found that he had aneurysm in both right common iliac artery along with right inter lober pulmonary artery and he was a previously undiagnosed sporadic case without any positive family history of Marfan syndrome. There is a reported case of spontaneous rupture of dissecting aneurysm of left common iliac artery during playing in a forty years old male patient [4]. But in our case it was sudden and spontaneous rupture had occurred without any physical exertion. While lying down, he developed acute onset pain followed by sudden collapse with severe pallor and disappearance of swelling in the abdomen. The occurrence of such an aneurysm suggests that the inherent mural weakness in Marfan syndrome is more widespread in the arterial tree than is generally appreciated [3]. It is secondary to cystic medial necrosis in the aorta and in multiple visceral arteries with extensive mucoid degeneration of the media of arterial wall [4]. So, patients with Marfan syndrome should be followed for signs of weakness of the peripheral arterial system and sometimes even with minor straining aneurysmal arterial wall can rupture. The peculiarity in our case was that even during rest the aneurysm got ruptured and he died almost immediately before resuscitation. When we diagnosed this case, we had planned early endovascular treatment with covered stenting of right common iliac artery as per recent advancement [7], but due to financial constraint it was delayed. Even in the acute condition, if we could have diagnosed rupture of the aneurysm earlier, he could have been saved by immediate closure of that artery by balloon dilatation just before the ruptured segment followed by immediate surgery with simple excision and end-to-end repair.

References

1. Ting P, Jugdutt BI, Tan JL. Large pulmonary artery aneurysm associated with Marfan syndrome. Int J Angiol. 2010;19:e48-50.

2. Hatrick AG, Malcolm PN, Burnand KG, Irvine AT. A superficial femoral artery aneurysm in a patient with Marfan’s syndrome. Eur J Vasc Endovasc Surg. 1998;15:459-60.

3. Flanagan PV, Geoghegan J, Egan TJ. Iliac artery aneurysm in Marfan’s syndrome. Eur J Vasc Surg. 1990;4:323-4.

4. Aschwanden M, Wegmann W. Dissecting aneurysm of the common iliac artery in Marfan syndrome: rupture following sports activities. Schweiz Med Wochenschr. 1990;120:836-9.

5. Savolainen H, Savola J, Savolainen A. Aneurysm of the iliac artery in Marfan’s syndrome. Ann Chir Gynaecol. 1993;82:203-5.

6. Safioleas MC, Kakisis D, Evangelidakis EL, Vericocos CM,Manti CP. Thromboendarterectomy of the right common iliac artery in a patient with Marfan’s syndrome and restoration with a new technique. Int Angiol. 2001; 20:241-3.

7. Yoshida Rde A, Yoshida WB, Kolvenbach R, Vieira PR. Modified "stent-graft sandwich" technique for treatment of isolated common iliac artery aneurysm in patient with Marfan syndrome. Ann Vasc Surg. 2012;26:419.e7-9.

8. Bozzani A, Arici V, Odero A. Modified "stent-graft sandwich" technique for treatment of isolated common iliac artery aneurysm in patient with marfan syndrome. Ann Vasc Surg. 2012;26:884-5

 

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