Ann Thorac Surg 2007;83:1537-1538
© 2007 The Society of Thoracic Surgeons
Case Reports
One-Step Surgical Approach of a Thoracic Aortic Aneurysm in Wiskott-Aldrich Syndrome
Eduardo Bernabeu, MDa,
Miguel Josa, MDa,*,
Benet Nomdedeu, MD, PhDb,
José Ramírez, MD, PhDc,
Antonio García-Valentín, MDa,
Carlos A. Mestres, MD, PhDa,
Jaime Mulet, MD, PhDa
a Cardiovascular Surgery Service, Hospital Clinic, University of Barcelona, Barcelona, Spain
b Haematology Service, Hospital Clinic, University of Barcelona, Barcelona, Spain
c Department of Pathology, Hospital Clinic, University of Barcelona, Barcelona, Spain
Accepted for publication October 2, 2006.
* Address correspondence to Dr Josa, Servicio Cirugía Cardiovascular, Hospital Clinic Barcelona, C/Villarroel, 170, E-08036 Barcelona, España (Email: mjosa{at}clinic.ub.es).
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Abstract
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Wiskott-Aldrich syndrome is a primary immunodeficiency characterized by infections, thrombocytopenia, and eczema. We present a 33-year-old man with this syndrome who underwent a one-stage ascending aorta, aortic arch and descending aortic aneurysm repair under moderate hypothermia and continuous visceral and cerebral perfusion. Histologic examination showed the presence of an aortitis with granulomatous inflammatory response and multinucleated cells.
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Introduction
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Wiskott-Aldrich syndrome is a rare genetic disorder linked to the X-chromosome that includes eczema, thrombocytopenia, and immunodeficiency. Patients affected by this syndrome have been reported to experience aneurysm formation owing to vasculitis, although no association has yet been proven [1, 2]. Reports on aneurysm repair in these patients are scarce, and the operations are associated with technical difficulties and severe perioperative bleeding [3–5].
We report the case of a young patient in whom a complete repair of an aneurysm extending from the ascending aorta, aortic arch, and descending aorta was done in a single-stage procedure with continuous retrograde visceral and antegrade cerebral perfusion.
A 33-year-old man had been followed up in our institution since he was aged 15. Two younger brothers had died some years earlier owing to bleeding and infectious complications related to Wiskott-Aldrich syndrome. His personal medical history included an infectious acute nephritis by Klebsiella spp, a spontaneous hematoma of the left lower limb that needed drainage, and seizures during childhood.
On roentgenograms done a decade ago, no signs of thoracic abnormalities were detected (Fig 1A). He was found to have a mediastinal widening in a recent plain chest roentgenogram (Fig 1B). A thoracic aortic aneurysm was suspected and confirmed by a computed tomography (CT) scan. Measured maximal diameters were 48 mm in the ascending aorta, 40 mm in the aortic arch, and 53 mm in the descending thoracic aorta. At the level of the diaphragm, the morphology of the aorta was normal. Echocardiography showed a normal valvular and ventricular function.
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Fig 1. (A) Chest roentgenogram taken a decade earlier to surgery shows no dilatation of thoracic aorta. (B) Preoperative roentgenogram shows the dilated thoracic aorta.
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Elective surgical repair was indicated. A combined median sternotomy and left thoracotomy through the fourth intercostal space was used for optimal exposure. The left femoral artery and the right atrium were cannulated for institution of cardiopulmonary bypass (CPB). The left heart was vented through the right superior pulmonary vein. Cold blood cardioplegia was delivered into the coronary ostia every 20 minutes. Distal and proximal aortic anastomosis were performed on CPB, while antegrade cerebral perfusion through direct carotid cannulation was kept at 32°C. Continuous lower body perfusion was maintained through the femoral artery while the thoracic aorta was clamped proximal to the diaphragm. All supraaortic branches were anastomosed in a single patch of tissue, using a circulatory arrest time of 9 minutes. Total cross-clamping time was 122 minutes, and CPB time was 169 minutes.
The patient’s initial postoperative recovery was uneventful, and he was weaned from the ventilator within 6 hours after surgery. On postoperative day 2, an episode of tonic-clonic seizures occurred that led to a second brief period of ventilatory assistance. Medical therapy easily controlled seizures. Results of a head CT scan and electroencephalogram were normal.
The total chest drainage was 1500 mL, mainly consisting of nonhematic pleural and pericardial effusion. The patient needed four units of packed red blood cells and a unit of pooled platelets.
The patient was discharged on the postoperative day 13. Ten months after surgery he remains in good condition.
The aorta distal to the repaired segment was of normal diameter, but the macroscopic appearance was that of a chronic inflammatory process. Histologic examination of the aortic wall from this area showed a thickened aortic wall with granulomatous rheumatoid-like structures and a mixed lymphocytic inflammatory infiltrate process spreading through the full thickness of the aortic wall. These granulomatous structures included degenerative fibromuscular tissue surrounded by a histiocytic reaction with few giant multinucleated cells. The inner layer of the aorta also showed a paucicellular fibrous lobulated thickening. Immunohistochemistry confirmed the presence of a mixed reactive pattern of the inflammatory cells. A histologic diagnosis of granulomatous aortitis was determined (Fig 2).
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Fig 2. Histologic section of the aorta: granulomatous inflammatory response surrounding degenerative tissue (hematoxylin and eosin stain, original magnification x100).
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Comment
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Wiskott-Aldrich syndrome is a primary immunodeficiency disorder linked to the short arm of the X-chromosome. Several blood cell lines are affected; both lymphocytes B and T, and platelets malfunction in this genetic disorder. Its clinical picture includes bleeding complications owing to low platelet counts, recurrent bacterial, viral, and fungal infections, skin eczema, and autoimmune vasculitis [1].
In the past, the life span of these patients was quite short, with an expected mean survival of 6.5 years [3]. Since the introduction of splenectomy and bone marrow transplantation, mean survival has increased to 25 years [6, 7]. As a consequence of their longer survival, these patients are at higher risk for lymphoproliferative diseases and vasculitis. Vasculitis and aneurysm formation are rare and life-threatening complications in Wiskott-Aldrich syndrome [2]. Surgical management of aortic aneurysm in these patients is more complex than in the general population because of their higher propensity to infectious and bleeding complications.
Three patients have been previously reported in the English literature [3–5], and all of them presented with progressive aortic dilatation. They were managed by a two-staged approach, replacing first the aortic arch aneurysm with an elephant trunk technique and repairing the descending aorta aneurysm in a second operation. We performed a complete repair in a single operation using moderate hypothermic circulatory arrest. Antegrade cerebral and distal retrograde body perfusion under moderate hypothermia associated with controlled cardioplegic hypothermic heart arrest allowed time to perform a comfortable one-step procedure, thus decreasing the risks of bleeding and surgical exposure to infection.
Endovascular prostheses are emerging as a new approach to both Stanford type B aortic dissections and thoracic aortic aneurysms. This possibility was considered in this patient as a second-stage procedure. Vasculitis and aortic disease in Wiskott-Aldrich syndrome is multisegmentary, however, and anchoring of these devices in a diseased aortic wall is not advisable. This fact was confirmed in our patient, as the macroscopic and microscopic examination of the aortic wall distal to the repair was compatible with chronic aortitis.
The presence of aortitis in areas of normal size aorta makes follow up of these patients with imaging techniques mandatory.
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References
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- Johnston SL, Unsworth DJ, Dwight JF, Kennedy CT. Wiskott-Aldrich syndrome, vasculitis and critical aortic dilatation Acta Pediatr 2001;90:1346-1348.[Medline]
- van Son JAM, O’Marcaigh AS, Edwards WD, Julsrud PR, Danielson GK. Successful resection of thoracic aortic aneurysms in Wiskott-Aldrich syndrome Ann Thorac Surg 1995;60:685-687.[Abstract/Free Full Text]
- Narayan P, Alwair H, Bryan AJ. Surgical resection of sequential thoracic aortic aneurysms in Wiskott-Aldrich syndrome Interact Cardiovasc Thorac Surg 2004;3:346-348.[Abstract/Free Full Text]
- Parkman R, Rappeport J, Geha R. Complete correction of the Wiskott-Aldrich syndrome by allogeneic bone-marrow transplantation N Engl J Med 1978;298:921-927.[Abstract]
- 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;302:892-896.[Abstract]
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Abstract
Introduction
