Infarction of the right hemisphere in a patient with antiphospholipid antibody syndrome

Summary Hemicraniectomy as a surgical treatment for intracranial pressure following large ischemic lesions is widely practiced in selected patients. The antiphospholipid antibody syndrome (APS), a disorder characterized by recurrent arterial and venous thrombosis, is a very rare cause of space occupying ischemic lesions. We present a case of a 35 year old female diagnosed with APS who initially presented with small ischemic lesions and within days developed a massive near-total infarction of the right hemisphere. Because of central nervous system, skin and systemic manifestations Sneddon’s syndrome and catastrophic antiphospholipid antibody syndrome (CAPS) remained a possible diagnoses. Sneddon’s syndrome is a non-inflammatory occlusive arteriopathy of small and medium size arteries predominantly of the skin and brain, whereas the catastrophic antiphospholipid antibody syndrome is characterized by acute multi-organ system thrombosis of small and large vessels. In addition to the diagnostic criteria for APS a heterozygous factor V Leiden mutation was found in this patient, which may be a contributing risk factor for cerebral ischemia. When considering invasive decompressive procedures the neurosurgeon has to be aware of the poor prognosis of some forms of APS with systemic manifestations.These authors contributed equally to this work.     

Does antiphospholipid antibody syndrome affect bioprosthetic heart valve?

Cardiovascular involvements in antiphospholipid antibody syndrome have been recognized as a major complication of this disease. Furthermore, some papers report bioprosthetic heart valve also seems to be affected. A 32-year-old female with aortic regurgitation presented to our hospital. Further examination revealed high titer of anticardiolipin beta 2 glycoprotein 1 antibody, and she was diagnosed as having primary antiphospholipid antibody syndrome since the patient failed to match the criteria of systemic lupus erythematosus. Cardiopulmonary bypass was uneventfully conducted under systemic heparinization of usual dosage. Administration of warfarin sodium was started on the third postoperative day, and international normalized ratio was controlled from 2.0 to 2.5. On echocardiographic examination at 1 month, mean systolic gradient was 17 mmHg. Although transesophageal echocardiography at 2 years after surgery revealed no sign of valvular destruction or sclerosis, transaortic gradient had increased to 26 mmHg. Bioprosthetic stenosis was suspected probably due to pannus formation and the patient may have to undergo another valve replacement in the near future.     

Does antiphospholipid antibody syndrome affect bioprosthetic heart valve?

Cardiovascular involvements in antiphospholipid antibody syndrome have been recognized as a major complication of this disease. Furthermore, some papers report bioprosthetic heart valve also seems to be affected. A 32-year-old female with aortic regurgitation presented to our hospital. Further examination revealed high titer of anticardiolipin beta 2 glycoprotein 1 antibody, and she was diagnosed as having primary antiphospholipid antibody syndrome since the patient failed to match the criteria of systemic lupus erythematosus. Cardiopulmonary bypass was uneventfully conducted under systemic heparinization of usual dosage. Administration of warfarin sodium was started on the third postoperative day, and international normalized ratio was controlled from 2.0 to 2.5. On echocardiographic examination at 1 month, mean systolic gradient was 17 mmHg. Although transesophageal echocardiography at 2 years after surgery revealed no sign of valvular destruction or sclerosis, transaortic gradient had increased to 26 mmHg. Bioprosthetic stenosis was suspected probably due to pannus formation and the patient may have to undergo another valve replacement in the near future.     

Catastrophic antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.     

Mitral stenosis and regurgitation with systemic lupus erythematosus and antiphospholipid antibody syndrome

Cases of valvular disease accompanied by systemic lupus erythematosus and antiphospholipid antibody syndrome are uncommon. Here, we present a surgical case of mitral stenosis and regurgitation with systemic lupus erythematosus and antiphospholipid antibody syndrome. Mitral valve replacement was performed, and the postoperative course was uneventful. Pathohistological findings of the mitral valve showed degenerative change due to chronic inflammation, proliferative fibrous change and calcification without thrombus formation.     

Multiple thromboemboli associated to two occult tumors: a case mimicking catastrophic antiphospholipid syndrome

Thromboembolic events tend to arise during the natural lifetime of tumors. However, multiple thromboemboli mimicking catastrophic antiphospholipid syndrome is quite rare as a first manifestation of a tumor. Herein we describe the case of a 51-year old woman that presented with multiple thromboemboli affecting her brain, lung and kidneys. Despite bolus administration of corticosteroids, anticoagulant therapy and immunoglobulin infusion treatment, the patient died. She had suffered from two occult tumors, which could not be identified premortem: a lung adenocarcinoma and an intrahepatic cholangiocarcinoma. This case underscores the importance of determining the underlying etiology behind multiple thromboemboli. The most important prognostic factor is rapid initiation of treatment of the multiple thromboemboli, emphasizing treatment of their etiology.     

Acute postoperative biventricular failure associated with antiphospholipid antibody syndrome

Antiphospholipid syndrome is probably the most common acquiredhypercoagulable state, but information on perioperative managementis sparse. Minor alterations in anticoagulant therapy, infection,or a surgical insult may trigger widespread thrombosis. Theperioperative course of a 31-yr-old woman with primary anticardiolipinantiphospholipid antibody syndrome requiring a mitral valvereplacement is described. Postoperatively, she developed acuteglobal biventricular failure requiring extracorporeal membraneoxygenation support and plasmapheresis. The management of anticoagulationand cardiac surgery in this condition is reviewed. Br J Anaesth 2004; 92: 748–54     

Prevalence and clinicopathologic findings of antiphospholipid syndrome nephropathy in Thai systemic lupus erythematosus patients who underwent renal biopsies

AIM: To determine the prevalence of antiphospholipid syndrome nephropathy (APSN) in Thai systemic lupus erythematosus (SLE) patients who underwent renal biopsy and to compare the relationship of renal histopathology and other significant clinical parameters between SLE patients with and without APSN. METHODS: A retrospective analysis was undertaken in systemic lupus erythematosus patients (n = 150, 44 <15 years old, 106 0e;15 years old) who underwent renal biopsy. The specimens were evaluated for histological features of APSN and other significant clinical parameters. The result of antiphospholipid antibodies, clinical course, and renal function from chart review were analysed. RESULTS: The prevalence of APSN in systemic lupus erythematosus patients who underwent renal biopsies was 34% (16% in <15-year-old group, 41.5% in > or =15-year-old group). APSN was associated with more severe hypertension (P = 0.002 for systolic and P = 0.004 for diastolic blood pressure), acute renal failure (P = 0.003), persistent heavy proteinuria (P < 0.001 for 4+ proteinuria), severe lupus nephritis (class III and IV, P = 0.014, high activity and chronicity indices, P < 0.001) and a tendency to progress to end-stage renal disease. CONCLUSION: Systemic lupus erythematosus patients who underwent renal biopsies in our institute showed a prevalence of APSN comparable to those in western countries. The presence of APSN was significantly higher in the adult than in the paediatric population. Its association with poor prognostic indicators suggests poor renal outcome. Clinicians should be aware of this condition in order to give proper care to systemic lupus erythematosus patients.     

Anesthetic implications of the catastrophic antiphospholipid syndrome

The antiphospholipid antibody syndrome (or the anticardiolipin antibody syndrome) is characterized by the presence of autoantibodies. Its major association is with systemic lupus erythematosus. 'Catastrophic' antiphospholipid syndrome (CAPS) is defined as an accelerated form of APS usually resulting in multiorgan failure and can be precipitated by surgery. We present the case of a 12-year-old male child who presented for enucleation of his left eye for fungal endopthalmitis. This patient had a history of CAPS 2 months before surgery with myocardial, gastrointestinal, renal and laryngeal involvement that improved on aggressive treatment and was subsequently managed on an outpatient basis for 2 months before presenting for enucleation. To the best of our knowledge, this is the first case of CAPS in a child reported in the anesthetic literature. Further aspects of this puzzling condition and its anesthesia implications are discussed.     

Central retinal vein occlusion in a patient on hemodialysis secondary to antiphospholipid syndrome: Case report

Central retinal vein occlusion (CRVO) is one of the most common retinal vascular disease. Macular edema associated is responsible of the major decrease in visual acuity. The main causes often implicated are high blood pressure and diabetes. Other etiologies should be sought including CRVO secondary to antiphospholipid syndrome (APS). This rare etiology is associated with a poor prognosis when late diagnosed. Owing to the high associated mortality, early diagnosis and prompt treatment are necessary. We describe a case of APS complicated by a catastrophic antiphospholipid syndrome in a patient who presented a decrease visual acuity.     

Asynchronous (heterochronic) bilateral renal infarction associated with primary antiphospholipid syndrome

We report the rare case of a 51-year-old man with asynchronous (heterochronic) bilateral renal infarction associated with primary antiphospholipid syndrome. He was treated for right renal infarction, but 2 months later, while under anticoagulant treatment, he had a recurrence on the other side of the renal infarction. The laboratory work-up confirmed antiphospholipid syndrome. Six months later the patient has not experienced any new thrombotic episodes and is receiving oral anticoagulants and antiplatelet therapy.     

Transient antiphospholipid syndrome in an infant with segmental small bowel infarction

The clinical picture of venous or arterial thrombosis in the presence of circulating antiphospholipid antibodies is referred to as the antiphospholipid syndrome. A 5-month-old baby girl who was quite healthy so far was referred to our clinic with irritability, vomiting, and abdominal distension for 30 hours. Surgical exploration exposed a gangrenous ileal segment about 15 cm long. The postoperative period was unremarkable. Investigation to identify the risk factors for mesenteric thrombosis found anticardiolipin antibodies (isotype Ig G) and decreased protein C level. Protein S and antithrombin III were within normal levels. Hb electrophoresis results showed no HbS, and neither Factor V Leiden nor prothrombin 20210 mutations were detected. Eight months postoperatively, anticardiolipin antibodies were found within normal levels. Lupus anticoagulant, ds DNA, and ss DNA were negative. Direct coombs test and protein C, C3, and C4 were also within normal levels. She had no thrombotic episode in the 24 months postoperatively, although no anticoagulant medication was administered. To the authors’ knowledge this case is the first report of segmental intestinal infarction in transient antiphospholipid syndrome in the pediatric population.     

Aortic valve replacement for aortic regurgitation in a patient with antiphospholipid antibody syndrome

Cardiac manifestations of antiphospholipid antibody syndrome (APLS) comprise a major complication. Herein we report our surgical treatment of aortic regurgitation in a patient with APLS. A 61-year-old woman was referred to our hospital with symptoms of congestive heart failure. Systemic lupus erythematosus had been diagnosed at the age of 36, and immunosuppressive therapy has been continuously performed. APLS was also diagnosed at the age of 55, after which cardiomegaly was noted on chest radiographs and aortic regurgitation was evident on echocardiography. Although immunosuppressive therapy had been continued, cardiac symptoms began to develop. With a presumed diagnosis of valvular disease associated with autoimmune disease, the aortic valve was replaced with a bioprosthesis. Noninfective endocarditis was confirmed in the excised specimen and was likely involved in APLS. The patient was discharged on postoperative day 26 without complications.     

Secondary mitral valve replacement in antiphospholipid syndrome and chronic renal failure

A 48-year-old woman admitted with progressive dyspnea had previously been diagnosed with systemic lupus erythematosus, antiphospholipid syndrome, and chronic renal failure, and had undergone mitral valve replacement with a Carpentier-Edwards pericardial bioprosthesis for mitral insufficiency 9 years before. She suffered a cerebral infarction 5 years earlier, despite appropriate anticoagulant therapy. On admission, echocardiography showed severe bioprosthetic stenosis. Repeat mitral valve replacement was conducted using a Mosaic bioprosthesis. On postoperative day 2, when heparinization was commenced, she suddenly had an epileptic fit. She also developed ischemic necrosis of the fingers and toes, considered secondary to microthrombosis. Aspirin was administered and heparin replaced by warfarin sodium. Necrosis gradually disappeared, and she was discharged 3 months after surgery. The original bioprosthesis showed degenerative changes with significant thrombus formation on cusps, thought to be mainly due to her hypercoagulable state. Considering the thrombophilic tendency in patients with antiphospholipid syndrome, strict management of anticoagulant therapy is required.