Ann Thorac Surg 1995;60:685-687
© 1995 The Society of Thoracic Surgeons
Case Report
Successful Resection of Thoracic Aortic Aneurysms in Wiskott-Aldrich Syndrome
Jacques A. M. van Son, MD, PhD,
Aengus S. O'Marcaigh, MD,
William D. Edwards, MD,
Paul R. Julsrud, MD,
Gordon K. Danielson, MD
Division of Thoracic and Cardiovascular Surgery, Section of Pediatric Hematology and Oncology, Division of Anatomic Pathology, and Department of Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota
Accepted for publication February 16, 1995.
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Abstract
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A 23-year-old man with Wiskott-Aldrich syndrome, chronic aortitis, and severe aneurysmal dilatation of the thoracic aorta successfully underwent two-stage graft replacement of the ascending and descending thoracic aorta. Nine years postoperatively, he is asymptomatic and employed full time.
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Introduction
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The Wiskott-Aldrich syndrome is a rare disorder characterized by eczema, thrombocytopenia, immunodeficiency, and early death. We report a patient who had Wiskott-Aldrich syndrome and associated aortitis with severe aneurysmal dilatation of the ascending and descending thoracic aorta who underwent successful two-stage graft replacement of the aneurysms.
A 23-year-old man with Wiskott-Aldrich syndrome was first seen at our institution in 1983. Two brothers also had the Wiskott-Aldrich syndrome without a diagnosis of thoracic aneurysm. The patient had a history of recurrent skin and respiratory infections, eczema, easy bruising, and prolonged bleeding after injury. His platelet count had been only 6,000/µL. Earlier in the same year, he had undergone a splenectomy elsewhere for thrombocytopenia, and the platelet count rose to 250,000/µL. His chest radiogram showed a tortuous, aneurysmally dilated, calcified ascending and descending thoracic aorta; calcium was also seen in the thoracoabdominal aorta (Fig 1A
). Comparison of computed tomograms from 1979 and 1983 showed an increase in the maximum diameter of the ascending and descending thoracic aorta from 4.5 to 6 cm (Fig 1B) and from 6 to 9 cm (Fig 1C), respectively. Angiographically, there was severe aortic valve insufficiency.
Operation was performed on January 6, 1984. The ascending aorta was approached through a median sternotomy. The aorta was elongated and irregularly aneurysmal with a maximum diameter of 6 cm. The ascending aorta and arch were extensively calcified. The right and left coronary ostia appeared normal except the right ostium was close to the commissure between the right and noncoronary cusps. The aortic annulus was dilated, but there was no dilatation of the sinuses. Cardiopulmonary bypass was instituted through a single right atrial cannula and the right femoral artery, which was unremarkable. The ascending aorta was cross-clamped just proximal to the innominate artery and the aneurysm was opened anteriorly. The right and left coronary ostia were selectively perfused with multidose cold blood cardioplegia. The aortic valve was excised and a composite Björk-Shiley valved conduit (29 mm) was inserted. Side-to-side anastomoses were made between the coronary ostia and appropriate openings made in the conduit. Calcification of the aortic wall made suturing difficult, even with cutting-point needles. The distal conduit was cut obliquely and sewn to the inside of the aortic wall just proximal to the clamp. There was considerable bleeding from all tissues and from the interstices of the graft. Fifteen units of platelets, 12 units of fresh frozen plasma, and 10 units of autologous blood were required before hemostasis was achieved. The redundant portion of the aneurysm was resected and the remainder was closed tightly around the conduit.
The postoperative course was uneventful, and he was discharged from the hospital on the seventh postoperative day. The patient was given polyvalent pneumococcal vaccine and was advised to take amoxicillin for respiratory infections.
On April 19, 1984, the descending aorta was approached through a left posterolateral thoracotomy. By moving the skin incision, we could approach the proximal and distal thoracic aorta through fourth and seventh intercostal space incisions, respectively. The aneurysm arose 1.5 cm distal to the left subclavian artery and extended to within 1 to 2 cm of the aortic hiatus in the diaphragm; its maximum diameter was 9 cm. The lung was adherent to the aneurysm and there were adhesions between the aneurysm and thoracic wall.
Cardiopulmonary bypass was instituted through the left femoral artery and vein using a pump oxygenator. The aneurysm was extensively calcified, and there was no backbleeding from any of the intercostal arteries, which were occluded by the pathologic process; one tiny bronchial artery on the posteromedial aspect of the aorta was patent. The proximal clamp was placed at the origin of the left subclavian artery and the distal clamp at the aortic hiatus. A 26-mm low-porosity Dacron graft was sewn into place, the proximal anastomosis being 1 cm distal to the left subclavian artery and the distal anastomosis 2 cm proximal to the aortic hiatus. Multiple additional pledgeted sutures were required at the anastomoses to control suture hole bleeding, which occurred through the extremely calcified and degenerated aortic tissue. Bypass was discontinued without difficulty. Eight units of platelets, 9 units of fresh frozen plasma, and 5 units of autologous blood were required to establish hemostasis. The aneurysm was partially resected and closed over the graft. The postoperative course was uneventful, and he was discharged from the hospital on the seventh postoperative day.
The pathology report on the heavily calcified tissue removed from the ascending and descending aorta (maximal wall thickness, 1.2 cm) described an idiopathic aortitis manifested by adventitial fibrosis and proliferative granulation tissue, adventitial and medial lymphocyte infiltrates with focal lymphoid follicle formation, adventitial and medial microabscesses with tissue destruction and extensive neutrophilic infiltrate, patchy medial destruction and fibrosis, and secondary intimal fibrosis (Fig 2). Special stains for bacteria, fungi, acid-fast bacilli, and spirochetes were all negative.
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Fig 2. . Photomicrograph of ascending aorta showing aortitis with fibrotic aneurysmal wall (Hematoxylin and eosin; x40 before 43% reduction.)
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At 9-year follow-up, the patient is in New York Heart Association functional class I and is working full time as a recreational therapist. However, recent computed tomograms showed a 5.8 cm aneurysmal dilatation of the thoracoabdominal aorta from the previous graft down to the renal arteries. Resection of the thoracoabdominal aneurysm was recommended; a 5% to 10% risk of mortality and a 10% risk of paraplegia were quoted.
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Comment
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The Wiskott-Aldrich syndrome is a rare X-linked recessive disorder that usually becomes manifest in early life as eczema, thrombocytopenia with platelets of reduced size and abnormal function, and an immunodeficiency characterized by a specific inability to make antibodies to carbohydrate antigens [1]. The syndrome is a disorder of T-cell function in which a deficiency of the surface glycoprotein CD43 has been described in lymphocytes and platelets of affected patients [2]. Most patients die in late childhood of infectious or bleeding complications. Splenectomy results in an improvement in both platelet number and size [3], and immunoglobulin replacement therapy, prophylactic antibiotics, and immunization can decrease the incidence of infections with encapsulated bacteria. The only specific therapy for Wiskott-Aldrich syndrome is bone marrow transplantation [4].
Systemic arteritis has rarely been described in other patients with Wiskott-Aldrich syndrome, nearly all of whom died in early life [5, 6]. One patient with aortitis and hypertension secondary to an abdominal aortic aneurysm with bilateral stenoses of the renal arteries underwent autotransplantation of the kidneys to the iliac arteries, but he died in the immediate postoperative period [6]. Our patient had successful treatment of thoracic aortic aneurysms associated with Wiskott-Aldrich syndrome. He has been asymptomatic for 9 years but now has aneurysmal dilatation of the thoracoabdominal aorta, for which operation has been recommended.
In general, in the absence of significant other pathology, asymptomatic patients with aneurysmal dilatation of the ascending aorta, aortic arch, or descending thoracic aorta measuring 6 cm in diameter or greater are advised to have operation, especially when a recent increase in size has been documented, because current hospital and late mortality is less than in the natural history of the disease [7, 8]. However, surgical treatment of patients with combined aortitis and Wiskott-Aldrich syndrome, such as the present patient, represents a significant challenge because the entire aorta including the aortic valve may require replacement, the tissues are extensively diseased and densely calcified, and there are increased risks for infection and bleeding. Our patient now faces a difficult decision regarding his thoracoabdominal aortic aneurysm, and he is currently considering his options.
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Footnotes
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Address reprint requests to Dr Danielson, Division of Thoracic and Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905.
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References
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- Cooper MD, Chase HP, Lowman JT, Krivit W, Good RA. Wiskott-Aldrich syndrome. An immunologic deficiency disease involving the afferent limb of immunity. Am J Med 1968;44:499–513.[Medline]
- Parkman R, Remold-O'Donnell E, Kenney DM, Perrine S, Rosen FS. Surface protein abnormalities in lymphocytes and platelets from patients with Wiskott-Aldrich syndrome. Lancet 1981;2:1387–9.[Medline]
- Lum LG, Tubergen DC, Corash L, Blaese RM. Splenectomy in the management of the thrombocytopenia of the Wiskott-Aldrich syndrome. N Engl J Med 1980;302:892–6.[Abstract]
- Parkman R, Rappeport J, Geha R, et al. Complete correction of the Wiskott-Aldrich syndrome by allogeneic bone-marrow transplantation. N Engl J Med 1978;298:921–7.[Abstract]
- Filipovich AH, Krivit W, Kersey JH, Burke BA. Fatal arteritis as a complication of Wiskott-Aldrich syndrome. J Pediatr 1979;95:742–4.[Medline]
- Lau YL, Wong SN, Lawton WM. Takayasu's arteritis associated with Wiskott-Aldrich syndrome. J Paediatr Child Health 1992;28:407–9.[Medline]
- Crawford ES, Svensson LG, Coselli JS, Safi HJ, Hess KR. Surgical treatment of aneurysm and/or dissection of the ascending aorta, transverse aortic arch, and ascending aorta and transverse aortic arch. Factors influencing survival in 717 patients. J Thorac Cardiovasc Surg 1989;98:659–74.[Abstract]
- Crawford ES, Hess KR, Cohen ES, Coselli JS, Safi HJ. Ruptured aneurysm of the descending thoracic and thoracoabdominal aorta. Analysis according to size and treatment. Ann Surg 1991;213:17–26.
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Abstract
Introduction
