Ann Thorac Surg 1999;67:1171-1173
© 1999 The Society of Thoracic Surgeons
Case Reports
Ministernotomy for aortic valve replacement in a patient with osteogenesis imperfecta
Mohammad Bashar Izzat, FRCS(CTh)a,
Song Wan, MD, PhDa,b,
Innes Y.P. Wan, FRCSa,b,
Kim S. Khaw, MDa,b,
Anthony P.C. Yim, MDa,b
a Department of Surgery, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong
b Department of Anesthesia, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong
Accepted for publication October 8, 1998.
Address reprint requests to Dr Izzat, PO Box 33831, Rawda, Damascus, Syria
e-mail: izzat{at}cyberia.net.lb
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Abstract
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Open heart operations in patients with osteogenesis imperfecta are associated with increased morbidity and mortality resulting from tissue friability and bone brittleness. We used a ministernotomy approach for aortic valve replacement in a patient with osteogenesis imperfecta, with clear benefits and a satisfactory outcome.
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Introduction
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Osteogenesis imperfecta is a generalized disorder of the connective tissues, which is caused by a defect of collagen synthesis and characterized by bone brittleness and predisposition to fractures. Aortic valve insufficiency resulting from dilatation of the aortic root or myxomatous degeneration of valve leaflets is another recognized manifestation of osteogenesis imperfecta [1], for which surgical correction is associated with a high risk of morbidity and mortality, mainly from tissue friability and bleeding [2, 3]. We report a case of aortic valve replacement through a J-shaped ministernotomy in a patient with osteogenesis imperfecta.
A 43-year-old man with typical osteogenesis imperfecta (history of recurrent rib and lower limb fractures, short stature, pigeon chest, blue sclerae, and deafness) and long-term aortic valve insufficiency was referred for aortic valve replacement. Because of rib fragility and to minimize trauma to the chest wall, a minimal access approach was used.
An 8-cm skin incision was made, extending from two finger breadths below the sternal notch to the level of the fourth rib. An upper J-shaped ministernotomy was then done from the sternal notch extending caudally to the fourth intercostal space and then to that space on the right side. The patient was cannulated for cardiopulmonary bypass directly through the incision, and ventricular distension was prevented by inserting a pulmonary artery vent. The aortic cross-clamp was applied directly, and intermittent warm blood cardioplegia was introduced into the coronary ostia. An excellent view of the aortic root was obtained, and the aortic valve, which had a dilated annulus, was replaced with a 29-mm bioprosthesis (Biocor; St Jude, St. Paul, MN). Aortic valve repair was not considered because valve leaflets are commonly affected by the connective tissue abnormality [2, 3].
Weaning from cardiopulmonary bypass was done easily, a single pericardial drain was inserted through a stab incision in the epigastrium, and the sternum was closed using four interrupted sternal wires. The patient was extubated 9 hours postoperatively, and mediastinal drainage was minimal. Postoperative recovery was uncomplicated; no analgesia was required beyond postoperative day 3 (Fig 1). Preoperative and postoperative (day 8) radioisotope bone scans were done as part of a larger study, and they showed no evidence of recent rib trauma or fractures (Fig 2).
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Fig 2. Postoperative radioisotope bone scan. There is increased uptake in the upper part of the sternum, but no evidence of recent rib trauma or fractures.
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Comment
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Tissue friability is an important and clinically relevant aspect of osteogenesis imperfecta when cardiac operations are required. In 22 cardiac operations in patients with osteogenesis imperfecta reported in the English-language literature, there was a 30% mortality rate, mainly from difficult hemostasis and bleeding [2, 3]. Furthermore, the stress placed on the rib cage by median sternotomy can readily cause multiple rib fractures because of bone brittleness, causing considerable consequent pain and morbidity. Therefore, prevention of tissue and chest wall trauma in patients with osteogenesis imperfecta is essential.
With the ministernotomy approach, sternal split is limited and mediastinal dissection is avoided, hence postoperative blood loss is reduced compared with median sternotomy [4]. Furthermore, with the restricted spreading of the sternum, chest wall trauma is minimized, rib fractures are avoided, postoperative pain is reduced, and recovery is expeditious compared with conventional median sternotomy [4]. The present patient, in whom we used the ministernotomy approach, had minimal postoperative bleeding, no rib fractures, and a satisfactory outcome. Another advantage of the ministernotomy approach is that it can be easily converted into a full median sternotomy if more extensive procedures, such as aortic root replacement, are deemed necessary. We believe that the ministernotomy approach for aortic valve operations is advantageous in the unusual but high-risk group of patients with osteogenesis imperfecta.
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References
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Hortop J., Tsipouras P., Hanley J.A., Maron B.J., Shapiro J.R. Cardiovascular involvement in osteogenesis imperfecta. Circulation 1986;73:45-61.
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Almassi G.H., Hughes G.R., Bartlett J. Combined valve replacement and coronary bypass grafting in osteogenesis imperfecta. Ann Thorac Surg 1995;60:1395-1397.[Abstract/Free Full Text]
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Moriyama Y., Nishida T., Toyohira H., et al. Acute aortic dissection in a patient with osteogenesis imperfecta. Ann Thorac Surg 1995;60:1397-1399.[Abstract/Free Full Text]
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Izzat M.B., Yim A.P.C., El-Zufari M.H., Khow K.S. Upper "T" mini-sternotomy for aortic valve operations. Chest 1998;114:291-294.[Abstract/Free Full Text]
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Abstract
Introduction
