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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 27, No 1, January/February 2016

52

AFRICA

This fibrinolytic process in IMMD is distinct from DIC.

2

Patients with IMMD are found to have decreased levels of

platelets and fibrinogen, as well as factors V and VIII.

2

This leads

to accelerated fibrinolysis, often manifest as reduced euglobulin

lysis time.

2

In contrast to DIC, positive fibrin monomer and

increased D-dimer levels are not seen in IMMD.

2,6,8

The surgical

team needs to be aware of the bleeding tendency of patients

with IMMD.

6

Also, the arterial wall often appears friable with a

tendency to dissect easily during suture repair. Meticulous suture

technique is therefore essential.

6,8

The coagulation profile and

platelet function should be carefully monitored peri-operatively

and diligently corrected.

8

A single case report by Katz

et al

. describes endovascular

repair of a gluteal artery aneurysm secondary to IMMD.

2

However, the standard of care in the treatment of IMMD-

related aneurysms is open surgical repair.

6,8

At our institution,

we have treated a few patients with IMMD-related thoraco-

abdominal aneurysms with hybrid procedures, comprising

visceral debranching and revascularisation followed by extensive

thoraco-abdominal stent grafting.

The prognosis depends on the extent of disease and the time

of presentation.

8

Acute presentation with dissection has higher

morbidity and mortality rates.

8

Adverse outcomes are related

to major blood loss requiring massive blood transfusions and

the associated complications, as well as multi-organ failure

secondary to shock.

6

At the time of submission for publication, this case report

possibly represents the first described case of IMMD affecting

the renal artery.

Conclusion

Although IMMD is a rare vascular disorder, it forms part of

the differential diagnoses in aortic aneurysm, especially in the

younger population and in the absence of conventional risk

factors associated with non-specific degenerative aneurysms.

Clinicians managing patients with IMMD should be aware of

the bleeding diathesis associated with this condition.

The authors thanks Ms Monica van Schalkwyk for her help in obtaining the

articles reviewed in this case report.

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2.

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