Symptomatic Inferior Vena Cava Stenosis after Scoliosis Surgery in a Marfan’s Syndrome Patient with Pectus Excavatum

Objectives: Marfan’s syndrome (MFS) is associated with a variety of cardiovascular symptoms as well as deformities of the spine and the chest. A congenital stenosis of the inferior vena cava (IVC) in an asymptomatic patient with MFS has only been described once (Ghazal et al, 2015). We report a case of a 15-year-old boy with MFS, presenting with a symptomatic IVC compression after surgical scoliosis correction in the present of an asymmetric funnel chest (FC).

Methods and Results: A 15-year-old Caucasian MFS patient (aortic diameter Z = 4.54, systemic score = 9, and FBN1-positive) with a severe scoliosis and an asymmetric FC underwent scoliosis surgery in an outside hospital. During the procedure, the patient experienced severe hemodynamic instability so that he was emergently transferred to our pediatric intensive care unit. On arrival, he was found to have severe ascites and extended edema of the lower limbs, which were resistant to diuretics. Imaging findings on a postoperative CT scan suggested a subtotal compression of the IVC in proximity to the right ventricle, between the sternum and the spine. Echocardiography demonstrated focal narrowing of the IVC with turbulent flow and a reduced diastolic filling of the right ventricle caused by a severe IVC constriction. Being a well-known patient in our interdisciplinary Marfan center, it was common knowledge that a previous MRI had shown a constriction of the IVC on the height of the diaphragmatic passage, which had been clinically asymptomatic at all times—an information that was not available at the outside hospital. Conservative treatment failed to mitigate the disastrous hemodynamic status caused by the inferior inflow congestion. Thus, a surgical decompression of the IVC via correction of the FC with a Nuss procedure was performed, leading to a significant improvement of the clinical symptoms. The clinical course proceeded without complications, and the patient was discharged 3 weeks after surgical decompression.

Conclusion: To our knowledge, this is the first case of symptomatic IVC compression in a patient with MFS after surgical correction of scoliosis. In the case of simultaneously existing FC and scoliosis in MFS, the FC correction should be performed before the scoliosis correction to avoid a hemodynamic deterioration. The indication for surgical corrections in MFS patients should generally be discussed in an interdisciplinary Marfan center so that the full spectrum of the condition is taken into account.