Year : 2011 | Volume
: 1 | Issue : 1 | Page : 35--37
Anaesthetic implications of antiphospholipid antibody syndrome in pregnancy
Priyanka Garg, Prachi Gaba, Kirti N Saxena, Bharti Taneja
Department of Anaesthesiology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
KC-22B, Phase 1, Ashok Vihar, Delhi -110 052
Antiphospholipid antibody syndrome is an autoimmune disorder characterized by venous and/or arterial thromboses. When present in women of reproductive age, it is associated with recurrent loss of pregnancy. This case report summarizes the perioperative course and anaesthetic management in a patient with bad obstetrics history who had to undergo emergency caesarean section.
|How to cite this article:|
Garg P, Gaba P, Saxena KN, Taneja B. Anaesthetic implications of antiphospholipid antibody syndrome in pregnancy.J Obstet Anaesth Crit Care 2011;1:35-37
|How to cite this URL:|
Garg P, Gaba P, Saxena KN, Taneja B. Anaesthetic implications of antiphospholipid antibody syndrome in pregnancy. J Obstet Anaesth Crit Care [serial online] 2011 [cited 2020 Jun 2 ];1:35-37
Available from: http://www.joacc.com/text.asp?2011/1/1/35/84254
Antiphospholipid Antibody (APLA) Syndrome or Antiphospholipid Syndrome (APS) is an autoimmune disorder in which thrombotic events and recurrent fetal loss occurs in the presence of antibodies to negatively charged phospholipids.  It is probably the most common acquired hypercoagulable state.  There is limited literature on perioperative management of patients with antiphospholipid syndrome. We present the perioperative course and complications seen in a 31-year-old woman with primary anticardiolipin antiphospholipid antibody syndrome who underwent an emergency caesarean section.
A 30-year-old multigravida with 36 weeks amenorrhea, diagnosed with antiphospholipid antibody syndrome was scheduled for emergency caesarean section. Obstetric history revealed that she had only one live issue delivered by caesarean section 4 years back, following which she had three consecutive abortions. She was a known case of hypothyroidism on 50 μg of eltroxin orally for last 5 years. She was investigated in view of a bad obstetric history and was diagnosed with antiphospholipid antibody syndrome although there was no history suggestive of any thromboembolic phenomenon. Further workup ruled out any systemic involvement. To improve the fetal outcome she was receiving 40 mg of enoxaparin subcutaneously once a day and 70 mg of aspirin orally once a day. She was being monitored by serial bleeding time (BT), clotting time (CT) and activated partial thromboplastin time (APTT) measurements. Investigations revealed a Lupus Anticoagulant (LAC) value of 43.2 (normal- 36.8), IgM and IgG anticardiolipin antibody values were 8.3 μ/ml and 3.9 μ/ml, respectively.
In view of the bad obstetric history and precious pregnancy, an elective caesarean section was planned at term. However, she went into labor at 36 weeks of pregnancy and was to be taken up for emergency caesarean section. She received her usual dose of eltroxin and enoxaparin but aspirin was omitted. Preoperative investigations revealed a normal BT, CT and APTT but prothrombin time (PT) was 21 against a control value of 14. Liver function tests revealed mildly elevated liver enzymes. All other investigations were within normal limits. Adequate fresh frozen plasma was arranged and patient was shifted to the operation theatre (OT). In view of deranged PT, it was decided to administer general anaesthesia to the patient using rapid sequence induction. The patient was preoxygenated for 5 mins and anaesthesia was induced with thiopentone 250 mg intravenously followed by succinyl choline 75 mg to facilitate endotracheal intubation. Anaesthesia was maintained with 50% oxygen in nitrous oxide and vecuronium was given to maintain neuromuscular blockade. Analgesia was provided by fentanyl 100 μg administered after delivery of the baby. Measures were taken to prevent hypothermia by using warm intravenous fluids and a warming blanket. Surgery was uneventful with minimal blood loss. At the end of surgery, neuromuscular blockade was reversed using glycopyrrolate 0.5 mg and neostigmine 2.5 mg intravenously and trachea was extubated after return of adequate respiration and reflexes.
When the patient was being shifted to high dependency unit (HDU), the patient developed shivering. On examination, her hands showed severe peripheral cyanosis. She had spider like bluish discoloration bilaterally on her palms and forearm suggestive of livedo reticularis. There was no evidence of cyanosis anywhere else. Active rewarming of peripheries helped and the cyanosis faded away. The patient had an uneventful stay in the HDU. Enoxaparin was restarted in the postoperative period and continued for 6 weeks. Post-operative analgesia was achieved with patient-controlled intravenous morphine.
Antiphospholipid syndrome is a paradoxical disease state with in vitro prolongation of APTT and a strong predilection for in vivo thrombosis. , Primary APS presents with thrombotic phenomena in young patients in the absence of any other related disease, while the secondary APS is associated with systemic lupus erythematous or lupus-like disease.  It is a multisystem disorder characterized by recurrent systemic arterial and venous thrombosis, recurrent abortion, thrombocytopenia and neurological disorders.  Cardiac valves and skin may also be involved. More recently, a catastrophic antiphospholipid variant with a mortality rate of nearly 50% has been described.  This catastrophic variant poses a diagnostic and therapeutic dilemma, as it often leads to a rapidly progressive multi-organ failure. Antiphospholipid syndrome may be associated with other autoimmune disorders like hypothyroidism, as was seen in our patient.
Antiphospholipid syndrome is characterized by the presence of lupus anticoagulant. These antibodies adhere to phospholipids on platelet and endothelial membranes leading to thrombus formation. In the pregnant uterus the uterine vascular endothelium, the trophoblastic lining of intervillous space and fetoplacental endothelium are targeted, causing uteroplacental thrombosis, placental infarcts and fibrinoid necrosis of the vessel wall thus interfering with implantation of the embryo causing recurrent abortion, commonly at 6-12 weeks of gestation, as was seen in our patient. 
Skin involvement manifests as livedo reticularis which consists of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The condition may be normal or may be related to more severe underlying pathology like autoimmune diseases, heart diseases, cancers and endocrine disorders. It can also be associated with the presence of anticardiolipin antibodies (antiphospholipid syndrome). It may not only be aggravated by exposure to cold and occurs most often in the lower extremities but can also be seen on palms and forearm as was seen in our patient.
In women with a history of recurrent miscarriage and antiphospholipid antibodies, future live birth rate is significantly improved when a combination therapy of aspirin and heparin is prescribed. , Heparin, one of the treatment modality, potentiates the antithrombin effects of antithrombin III and other antithrombin factors, increases levels of factor Xa inhibitor, inhibits platelet aggregation and binds to the antibodies rendering them inactive, thus improving the pregnancy outcome. Aspirin is also beneficial in this regard due to its antiplatelet effect. ,, This therapy is withheld at the time of delivery to reduce blood loss and restarted after delivery and should be continued for as long as 6 weeks postpartum. ,
Perioperatively, APS leads to thrombosis which may occur spontaneously or may be precipitated (thrombotic storm) by infection, surgical intervention, any change in anticoagulation therapy or introduction of oral contraceptive pills. ,, High dose anticoagulation suitable for the long-term management of these patients presents a major problem in the perioperative period. Moreover, sub-optimal anticoagulation or withdrawal of anticoagulant leads to hypercoagulability and its attendant problems as well as the risk of catastrophic antiphospholipid syndrome. Treatment of this hypercoagulable state or thrombocytopenia,  a common feature of antiphospholipid syndrome may cause life-threatening hemorrhage. Intraoperatively, physical measures such as anti-embolic stockings, intermittent venous compression devices and adequate hydration are advised.  Adequate measures should be taken to keep the patient warm. Patients can develop recurrent thrombosis despite appropriate prophylaxis.
Antiphospholipid antibody (APLA) syndrome poses dilemma for an anaesthetist. Literature shows some case series where successful administration of neuraxial blockade to patients of APLA syndrome had been carried out.  Ringrose  in his case series concluded that "In the absence of coagulation defects secondary to clotting factor antibodies or platelet abnormalities, the in vitro phenomenon of prolonged APTT alone should not, in theory, predispose to hemorrhage or be a contraindication to epidural anaesthesia in patients with APLAS." However, the results of these studies do not prove that neuraxial techniques are safe in this setting as the study was done on a small number of patients.
In our patient, PT was deranged, hence regional anaesthesia was ruled out and general anaesthesia was administered to the patient. Postoperatively, optimal analgesia for early mobilization should be advocated to prevent the associated risk of thrombosis.  Patients require close monitoring for both bleeding and thromboembolic complications postoperatively.
To conclude, APS requires a multidisciplinary approach for its diagnosis and management. This is another addition to the various medical conditions manifesting during pregnancy where use of anticoagulants may lead to dilemma of their perioperative continuation. Discontinuation of anticoagulants is a double edged sword and requires careful deliberation on the part of anaesthetist to reduce the risk of perioperative bleeding.
|1||Dornan RI. Acute postoperative biventricular failure associated with antiphospholipid antibody syndrome. Br J Anaesth 2004;92:748-54.|
|2||Shapiro SS, Thiagarajan P. Lupus anticoagulants. Prog Hemost Thromb 1982;6:263-85.|
|3||Brooker G, Ralph C. Thromboprophylaxis for patients with antiphospholipid syndrome during their pregnancy. Int J Obstet Anesth 2004;13:129-30.|
|4||Merkel PA, Chang Y, Pierangeli SS, Convery K, Harris EN, Polisson RP. The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am J Med 1996;101:576-83.|
|5||Asherson RA. A primary antiphospholipid syndrome. J Rheumatol 1988;15:1742-6. |
|6||Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol 1992;19:508-12. |
|7||Rawat RS, Dehran M. Anaesthetic management of a pregnant patient with antiphospholipid antibody syndrome for emergency caesarean section. Int J Obstet Anesth 2003;12:311. |
|8||Empson M, Lassere M, Craig J, Scott J. Prevention of recurrent miscarriage for women with antiphospholipid antibody or lupus anticoagulant. Cochrane Database Syst Rev 2005;18:CD002859.|
|9||Rai R, Cohen H, Dave M, Regan L. Randomized controlled trial of aspirin and aspirin plus heparin in pregnant women with recurrent miscarriage associated with phospholipid antibodies (or antiphospholipid antibodies). BMJ 1997;314:253-7.|
|10||Davies SR. Systemic lupus erythematosus and the obstetrical patient-implications for the anesthetists. Can J Anaesth 1991;38:790-6.|
|11||Khamashta MA, Mackworth-Young C. Antiphospholipid (Hugues') syndrome. BMJ 1997;314:244.|
|12||Derksen RH, Khamashta MA, Branch DW. Management of the obstetric antiphospholipid syndrome. Arthritis Rheum 2004;50:1028-39.|
|13||Agaba AE, Charaklias N, Babu-Victor A, Agaba PO, Deepchand V, Dabasia HS, et al. Antiphospholipid syndrome: A series of surgical emergencies and the current evidence for its management. Ann R Coll Surg Engl 2006;88:370-4.|
|14||Asherson RA, Piette JC. The catastrophic antiphospholipid syndrome: Acute multiorgan failure associated with antiphospholipid antibodies: A review of 31 patients. Lupus 1996;5:414-7.|
|15||Rojas Rodriguez J, Garcia Carrasco M, Ramos Casals M, Enriquez-Coronel G, Colchero C, Cervera R, et al. Catastrophic antiphospholipid syndrome: Clinical description and triggering factors in 8 patients. J Rheumatol 2000;27:238-40.|
|16||Finazzi G, Brancaccio V, Moia M, Ciaverella N, Mazzucconi MG, Schinco PC, et al. Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: A four-year prospective study from Italian registry. Am J Med 1996;100:530-6.|
|17||Menon G, Allt Graham J. Anaesthetic implications of the anti-cardiolipin antibody syndrome. Br J Anaesth 1993;70:587-90.|
|18||Ralph CJ. Anaesthetic management of parturients with the antiphospholipid syndrome: A review of 27 cases. Int J Obstet Anesth 1999;8:249-52.|
|19||Ringrose DK. Anaesthesia and the antiphospholipidsyndrome: A review of 20 obstetric patients. Int J Obstet Anesth 1997;6:107-11.|
|20||Kone A. Antiphospholipid antibody and anaesthesia. Acta Anaesthesiol Belg 2003;54:169-71.|