Case study
Chest wall reconstruction in Marfan
syndrome following aortic root
replacement
Asian Cardiovascular & Thoracic Annals
2014, Vol. 22(7) 872–874
ß The Author(s) 2013
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DOI: 10.1177/0218492313496586
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Laurynas Bezuska, Shaffi Mussa and Nagarajan Muthialu
Abstract
Chest wall and spine deformities are common in Marfan syndrome, and often coexist with cardiac defects. Surgery is
often needed to both the aortic root and the anterior chest wall, and early spinal surgery is not uncommon. We describe
a case of severe thoracic cage deformity with aortic root dilatation, which was managed by a staged approach, with a very
good final result.
Keywords
Aorta, thoracic, Dilatation, pathologic, Funnel chest, Marfan syndrome
Introduction
Chest wall and spine deformities are common in
Marfan syndrome, and are accompanied by heart disease in many patients. Often, a variety of surgical
approaches are proposed, and it is difficult to stage
these operations, considering the fact that reoperation
for aortic root or valve problems is not uncommon in
these individuals. We describe a staged approach in a
boy with complex cardiac and chest wall defects, with
difficulty even in supine positioning, with a good surgical result.
Case report
A 15-year old boy was referred to us with a chest wall
deformity. On assessment, he was found to have progressive aortic root dilatation, moderate to severe
kyphoscoliosis involving the thoracolumbar spine,
and severe pectus carinatum. The aortic root dilatation
was significant enough to warrant initial surgery in the
form of valve-sparing aortic root replacement using a
Hemashield graft (Maquet, Rastatt, Germany).
Following this, he was kept on assessment and
follow-up for deformities of the front and back of his
chest wall. While on follow-up, as per our protocol,
spinal surgery was planned for his kyphoscoliosis.
After anesthetizing, he could not be positioned in a
prone position because he developed a severe low cardiac output state secondary to his sternal deformity,
and so the entire surgical plan was reorganized. The
changed plan involved correcting his anterior chest
wall deformity by sterno-costo-chondroplasty, followed
by spinal correction. His anterior chest wall defect was
severe pectus carinatum, with a 90 -angulation of the
sternum at the xiphoid process (Figure 1), and extensive
lengthening of the ribs. There was mild asymmetry, and
the whole defect extended from the 2nd rib onwards.
Preoperative magnetic resonance imaging to assess the
existing cardiac status also helped in delineating the
extent of the skeletal abnormalities (Figure 2). Under
conventional anesthesia, he was placed supine for his
anterior chest wall correction. Surgery involved a long
transverse curvilinear incision in the submammary
region, and elevating a flap below the level of the pectoral muscles. The chondral ends of the ribs were
detached from the sternum and the xiphoid process.
A transverse osteotomy was made just below the
Department of Cardiothoracic Surgery, Great Ormond Street Hospital
for Children, London, UK
Corresponding author:
Nagarajan Muthialu, DNB, FRCSEd, Department of Cardiothoracic
Surgery, Great Ormond Street Hospital NHS Trust, Great Ormond
Street, London WC1N 3JH, United Kingdom.
Email: [email protected]
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Bezuska et al.
873
Figure 1. External appearance of the pectus carinatum before
correction.
Figure 2. Magnetic resonance imaging showing the skeletal
component of the pectus feature anteriorly and also the spinal
deformity.
manubriosternal angle to correct the angulation defect
of the sternum, followed by excision of excessive cartilage lengths on either side. A release osteotomy was
made from the 2nd rib down to the 7th rib to correct
a similar angulation defect of the ribs. Once the length
and angles were corrected, the cartilages and xiphoid
process were refixed to the sternum using Vicryl sutures
(Polyglactin 910, Ethicon, Inc., USA). After hemostasis, 2 suction drains were left in the submuscular plane,
and the wound was closed in layers. The final result was
symmetrical and acceptable (Figure 3). On follow-up at
3 months, he was completely comfortable and remained
cardiovascularly stable. He is pain-free and awaiting
spinal surgery.
Discussion
Chest wall deformities are not uncommon in Marfan
syndrome, and they add to the complexity of chest
Figure 3. Intraoperative photograph at the end of sternocosto-chondroplasty. Note the fixation of the anterior ends of
ribs and cartilages to the sternum.
interventions needed in the course of adolescence and
early adult life. Both pectus excavatum and carinatum
can exist, although excavatum deformities predominate. The general incidence of pectus deformities is 1 in
400 to 1 in 1000, while almost two-thirds of Marfan or
related connective tissue disorder patients are affected
by some form of pectus deformity.1 Aortic root dilatation can be a common feature in this syndrome, and it
adds to both the morbidity and mortality. While it is
common to find these individuals under routine followup for indexed screening of their aortic root dimensions
by imaging, Seliem and colleagues2 did not find any
convincing difference in the relationship of aortic root
dilatation in Marfan syndrome with or without pectus
deformities. These associations do not form part of the
Ghent nosology for diagnostic criteria in this connective tissue disorder. Further association with spinal
deformities, although commonly seen, can add to the
complexity of the correction.
The surgical approach depends on the severity of the
individual lesion. There is more awareness in recent
times of intervention on both the cardiac component
and the chest wall in a single stage.3 Javangula and
colleagues4 reported simultaneous correction of aortic
root dilatation and pectus deformity in Marfan syndrome, using an open method of correction for pectus
along with Gore-Tex strip reinforcements. However,
the added complexity of spinal curvature was not
included in the management of this complex thoracic
cage abnormality. Many reports have described correction of pectus excavatum using either a pectus bar or a
Nuss bar after correction of intracardiac defects.
Fukunaga and colleagues5 reported one incidence of
reoperation for aortic root dilatation following previous bar insertion as a second stage. However, we were
faced with a carinatum deformity that needed an open
method of repair using sterno-costo-chondroplasty.
Conventional repair of this kind involves fixation of
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874
Asian Cardiovascular & Thoracic Annals 22(7)
the ribs using a Stratos titanium bar (Strasbourg
Thoracic
Osteosynthesis
System;
MedXpert,
Heitersheim, Germany). The technique involves a
triple osteotomy, as reported by Brichon and colleagues,6 and a correction osteotomy to the sternum
in a transverse line to achieve planar fixation. This is
followed by an initial suture fixation of the osteotomized site using Vicryl suture and then titanium bars.
While the end result is quite satisfactory and provides
further stability to the chest wall, it is not always necessary to use this bar for stability, as shown in our
case. Our fixation offered rigid stabilization of the
sterno-coso-chondral apparatus with a good cosmetic
result, and allows rapid access without the need to go
across a metal bar, in case of future cardiovascular
reoperations.
In our patient, the aortic root progression was fast
enough to warrant early repair in the form of a valvesparing aortic root replacement. Although considered
significant, both the spinal and pectus corrections
needed attention individually, but the spinal curvature
was so severe that it was staged soon after the cardiac
repair. The difficulty in positioning the patient and the
hemodynamic changes when placed prone made surgery to the spine impossible. Hence the staged procedure was reaffirmed to perform anterior chest wall
surgery first to achieve not only a cosmetic repair but
also a hemodynamically viable chest wall to help future
spinal surgery. This report reinforces the fact that a
proper multidisciplinary approach to a complex congenital defect achieves a better long-term result.
Funding
This research received no specific grant from any funding
agency in the public, commerical, or not-for-profit sectors.
Conflict of interest statement
None declared
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