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Ann Thorac Surg 1995;60:1397-1399
© 1995 The Society of Thoracic Surgeons

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Case Reports

Acute Aortic Dissection in a Patient With Osteogenesis Imperfecta

Second Department of Surgery and First Department of Pathology, Faculty of Medicine, Kagoshima University, Kagoshima, Japan

Accepted for publication May 11, 1995.

	Abstract

Top Footnotes Abstract Introduction Comment References

 
A case of osteogenesis imperfecta complicated with acute type A aortic dissection is presented. Emergency graft replacement of the ascending aorta was performed successfully despite the anticipated difficulties with tissue friability. Therefore, such an operation is suggested to be worthy of consideration and feasible in patients with osteogenesis imperfecta.

	Introduction

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Osteogenesis imperfecta (OI) is a heterogenous group of disorders of collagen biosynthesis characterized by osseous fragility and a familial incidence [1, 2]. Although the primary clinical manifestations of OI include skeletal, ocular, cutaneous, otologic, and dental abnormalities, serious defects of the cardiovascular system have also been recognized occasionally [1–4]. We present here a patient with OI who had Stanford type A aortic dissection requiring an emergency graft replacement of the ascending aorta.

See also 1395 and 1439.

A 32-year-old man with known type I OI and a history of recurrent fractures was referred to our hospital for the evaluation of sudden onset of severe anterior chest pain. He had neither history of hypertension nor familial incidence of bone fragility. The blood pressure was 90/60 mm Hg, and the pulse was 110 beats/min and regular. The patient had multiple long bone deformities, kyphoscoliosis, a triangular head, and blue sclerae. Hearing and dentition were normal. There was no cardiac murmur or neurologic deficit. An electrocardiogram showed nondiagnostic ST-T changes in anterolateral leads with no features characteristic of left ventricular hypertrophy. A chest roentgenogram showed mild cardiomegaly with a cardiothoratic ratio of 0.52. A transthoracic echocardiogram revealed a slightly dilated ascending aorta (40 mm), a tricuspid competent aortic valve, and a massive pericardial effusion. Computed tomography with intravenous contrast medium confirmed the intimal flap in the ascending aorta with pericardial effusion.

At operation the ascending aorta was replaced by a 5.0-cm-long, collagen-impregnated, woven Dacron graft through a median sternotomy. The sternum was thin and friable. Intraoperative inspection of the ascending aorta confirmed the extensive subadventitial hematoma with a massive sanguineous pericardial effusion. The intima appeared almost normal, and a discrete 1.0 x 2.0-cm defect was identified about 2.0 cm beyond the ostia of both coronary arteries. The separated layers of aorta at the suture lines were approximated between inner and outer strips of Teflon felt. Microscopic examination of the aortic wall disclosed a focally slight reduction of the elastic fibers with mild deposition of mucopolysaccharides in the aortic media. The patient's postoperative course was complicated by prolonged respiratory failure due to bilateral phrenic nerve paralysis and sternal disruption. The phrenic nerve paralysis may have developed due to cold injury, despite application of an insulating pad; it resolved within 2 months. The sternal disruption did not result in mediastinitis. He finally recovered and was discharged from the hospital in good condition. Follow-up at 6 months showed good recovery. Periodic follow-up with computed tomographic surveillance in the future is planned.

	Comment

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Cardiovascular involvement is well known in some connective tissue disorders such as Marfan's syndrome. Although uncommon, cardiovascular abnormalities have also been described in OI. The structures involved are the aortic root [1, 4] and aortic and mitral valve leaflets [5–8]. The principal hemodynamic consequences are aortic or mitral insufficiency. According to our survey of the English-language literature [2–8], so far 20 cases of cardiovascular operation have been reported as being done under cardiopulmonary bypass, including the present case (Table 1). There were 18 male and 2 female patients, ranging in age from 19 to 63 years with a mean of 39 years. Thirteen of them (65%) were younger than 50 years old when they presented with cardiovascular abnormalities. The incidence of aortic disease in OI appears to be rare in comparison with that of valvular heart disease.

Cardiovascular operation in patients with OI poses certain special problems relevant to tissue friability and wound healing. In fact, serious problems with hemostasis after operation were reported in 6 of the 20 surgical cases (see Table 1). The nature of this disorder is still ill-defined and variable in individuals. Increased capillary fragility, decreased platelet function, and clotting factor deficiency have been suggested to be the prevalent abnormalities in these patients [5, 6]. Fortunately we have not had any coagulopathy and hemorrhagic complications in the present case, excluding the phrenic nerve paralysis and sternal disruption, which were probably related to tissue friability to some extent. Maximal support should be ensured at suture lines with the use of Teflon felts, biological glues, and so on. Our results suggest that such an operation is worthy of consideration and feasible even in patients with this connective tissue disorder.

	Footnotes

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Address reprint requests to Dr Moriyama, Second Department of Surgery, Faculty of Medicine, Kagoshima University, Sakuragaoka 8-35-1, Kagoshima 890, Japan.

	References

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1. Hortop J, Tsipouras P, Hanley JA, Maron BJ, Shapiro JR. Cardiovascular involvement in osteogenesis imperfecta. Circulation 1986;73:54–61.[Abstract/Free Full Text]
2. Koentges D, Van de Werf F, Stalpaert J, Goddeeris P, De Geest H. Aortic and mitral valve replacement in osteogenesis imperfecta: report of a case. Acta Cardiol 1986;41:147–53.[Medline]
3. Passmore JM, Walker WE, Fuentes F. Successful aortocoronary bypass in osteogenesis imperfecta. J Am Coll Cardiol 1987;9:960–3.[Abstract]
4. Ohteki H, Ohtsubo S, Sakurai J, Koga N, Kohchi K, Itoh T. Aortic regurgitation and aneurysm of sinus of Valsalva associated with osteogenesis imperfecta. Thorac Cardiovasc Surg 1991;39:294–5.[Medline]
5. Wood SJ, Thomas J, Braimbridge MV. Mitral valve disease and open heart surgery in osteogenesis imperfecta tarda. Br Heart J 1973;35:103–6.[Free Full Text]
6. Gerlach PA, Rosensweig J, Ramanathan KB. Successful aortic valve replacement in osteogenesis imperfecta: with special emphasis on peri-operative management. Can J Cardiol 1987;3:132–5.[Medline]
7. Jeyamalar R, Hashim R, Kannan P. Aortic valve replacement in osteogenesis imperfecta tarda: a case report. Sing Med J 1989;30:316–7.
8. Thibault GE. Clinical problem-solving. The heart of the matter. N Engl J Med 1993;329:1406–10.[Free Full Text]

This article has been cited by other articles:

				M. B. Izzat, S. Wan, I. Y.P. Wan, K. S. Khaw, and A. P.C. Yim Ministernotomy for aortic valve replacement in a patient with osteogenesis imperfecta Ann. Thorac. Surg., April 1, 1999; 67(4): 1171 - 1173. [Abstract] [Full Text] [PDF]

				R. J. Cusimano Repeat Cardiac Operation in a Patient With Osteogenesis Imperfecta Ann. Thorac. Surg., April 1, 1996; 61(4): 1294 - 1294. [Full Text]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Yukinori Moriyama Hitoshi Toyohira Akira Taira Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Moriyama, Y. Articles by Kuriwaki, K. Search for Related Content PubMed PubMed Citation Articles by Moriyama, Y. Articles by Kuriwaki, K. Related Collections Related Articles