Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 9  |  Issue : 1  |  Page : 142-145

Anesthetic management for elective lower segment cesarean section in a patient with systemic lupus erythematosus and thrombocytopenia


Department of Anaesthesiology, B.J. Medical College, Ahmedabad, Gujarat, India

Date of Submission06-Apr-2014
Date of Acceptance28-May-2014
Date of Web Publication17-Mar-2016

Correspondence Address:
B Patel Kiran
50, Raichandnagar Society, Near Visat Petrol Pump, Sabarmati, Ahmedabad 382424, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1687-7934.178896

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  Abstract 

Systemic lupus erythematosus is a multisystem, chronic inflammatory disease characterized by autoantibodies directed against nuclear antigens. The disease is most frequently found in women of child-bearing age and therefore may coexist with pregnancy. The clinical symptoms are variable and depend on the severity of damage to organ systems such as the musculoskeletal, renal, hematological, neurological, cardiac, and respiratory systems. The pregnant patient may experience exacerbations of disease, neonatal loss, and obstetric complications such as pre-eclampsia.The anesthetic management will depend on the patient's clinical status and the well-being of the fetus. The patient should be thoroughly examined for the extent of end-organ damage, current medications, and the health of the fetus. Laboratory investigations, such as coagulation profiles and testing for renal functions, should be performed and enough amount of blood substitutes should be kept ready before anesthetic intervention if time permits. A multidisciplinary approach by the obstetrician, the anesthesiologist and the hematologist for regular antenatal checkups, proper preoperative evaluation, and rational preparation of blood substitutes, with the availability of enough resources to manage complications during the entire course of the peripartum period, is essential to optimize the outcome for both the mother and the newborn.

Keywords: elective lower segment cesarean section, general anesthesia, steroids, systemic lupus erythematosus, thrombocytopenia


How to cite this article:
Kiran B P, Tapan S, Ananyaruchi S S. Anesthetic management for elective lower segment cesarean section in a patient with systemic lupus erythematosus and thrombocytopenia. Ain-Shams J Anaesthesiol 2016;9:142-5

How to cite this URL:
Kiran B P, Tapan S, Ananyaruchi S S. Anesthetic management for elective lower segment cesarean section in a patient with systemic lupus erythematosus and thrombocytopenia. Ain-Shams J Anaesthesiol [serial online] 2016 [cited 2019 Jul 21];9:142-5. Available from: http://www.asja.eg.net/text.asp?2016/9/1/142/178896


  Introduction Top


Systemic lupus erythematosus (SLE) is a chronic multisystem inflammatory autoimmune disorder characterized by autoantibody production, which involves multiple body systems and a wide range of manifestations. Antibodies are produced against host antigens and an inflammatory response is induced, resulting in tissue injury. It has a strong female preponderance (10 : 1) and occurs primarily in the reproductive years and may cause fetal wastage [1] . SLE was first documented in the middle ages when it was termed lupus ('wolf' in Latin) to describe the appearance of the classical facial (malar) rash. It was suggested that the rash resembled the fur on the forehead and muzzle of the wolf. Others have suggested that the disease may have been named after a veil (loup) used by women in France to cover facial blemishes. It was not until 1872 that MÓric Kaposi, a Hungarian dermatologist, began to recognize and describe the systemic manifestations of the disease [2] . The prevalence of SLE is described as having an ethnic component, with black women affected three times more than white women [3] .

In pregnancy, profound immune-endocrine changes occur. Hormonal modifications during pregnancy are regulated by the feto-placental unit and depend on interactions between the mother and the fetus [4] . The physiological increase of cortisol, progesterone, and estradiol during pregnancy leads to Th2 cytokine polarization both at the feto-maternal interface and at the systemic level [5] . Indeed, suppression of the immune response mediated by Th1 cytokines may be essential for fetal survival. Some, but not all, investigators believe that some autoimmune diseases, such as SLE, which are mainly mediated by Th2 cytokines, tend to occur or relapse during pregnancy, whereas the Th1-mediated disease, such as rheumatoid arthritis, tends to improve [5] .

Consequently, the obstetric anesthesiologist is likely to encounter pregnant lupus patients with increasing frequency. The purpose of this article is to provide a working knowledge of this disease and its implications for the practicing anesthesiologists.


  Case report Top


A 24-year-old second gravida parturient, who was a booked case with a confirmed diagnosis of SLE, presented to the hospital at 36 weeks of gestation with hematuria and bleeding per vagina since the morning of the day of presentation.

The patient had been suffering from enteric fever every year for 5 years and was hospitalized every time. She had also received multiple blood transfusions every time she was hospitalized. She was diagnosed with SLE in 2009 and had been taking 20 mg of prednisolone (omnacortil) once a day since then. The patient had a history of miscarriage at 6 weeks of gestation 1½ years previously when she had presented with bleeding per vagina and inevitable abortion.

This time, the patient presented with hematuria at 22 weeks of gestation and was admitted to the hospital where she received 7 U of packed red cells and 7 U of platelet-rich concentrates. She also received 20 doses of the gammaglobulin intravenously over 5 days. Bleeding was then controlled and she was advised to continue with the pregnancy. She was started on prednisolone at a 100 mg/day dosage, which was gradually tapered to 40 mg/day by 36 weeks of gestation. On admission, the patient had a hemoglobin of 9.03 g/dl with a platelet count of 10 000/cm 2 . Prothrombin time was 12 (control was 13.5) with international normalized ratio 0.88. The patient was antinuclear antibody positive, anti-double-stranded DNA borderline positive, anti-SSA positive, and antiphospholipid antibody negative. Urine R/M showed moderate albuminuria and plenty of red blood cells. Renal function tests and liver function tests were normal. Emergency medical assessment was carried out and she was prescribed 4 U of platelet-rich concentrates and a daily dose of 50 mg of prednisolone. The patient received 2 U of packed red cells and 4 U of platelet-rich concentrates on the morning of the day of her elective cesarean section. General anesthesia was planned with ECG, pulse oximeter, noninvasive blood pressure, end-tidal CO 2 , temperature, and urine output monitoring. Intravenous access was secured with an 18 G cannula. In the operating room her blood pressure was 142/82 mmHg, heart rate was 82/min and respiratory rate was 14/min. The risks and benefits of general anesthesia were discussed with the patient and her family and informed consent was obtained from them. Appropriate antibiotic prophylaxis, and steroids in the form of hydrocortisone (200 mg) and dexamethasone (8 mg) were administered intravenously. The patient was premedicated with glyco (0.2 mg), ondansetron (4.0 mg), and ranitidine (50 mg) intravenously. After 3 min of preoxygenation, induction was carried out with thiopentone sodium (400 mg) and succinylcholine (100 mg) and the patient was subsequently intubated with an endotracheal cuffed tube of size 7.0 mm ID. Anesthesia was maintained with sevoflurane, 50% nitrous oxide in oxygen, and mechanical ventilation was instituted after atracurium (25 mg) stat and then 5 mg as needed. After delivery, methylergometrine (1 ml) stat IV was administered and an oxytocin drip was started. Sevoflurane was then discontinued and fentanyl (70 mg) was administered intravenously. Hemodynamic stability was maintained with dextrose (5%), lactated Ringer's solution (750 ml), 4 U of platelet-rich concentrates, 3 U of fresh frozen plasma, and 1 U of packed red cell infusion during the intraoperative period. Frusemide (20 mg) was administered IV stat. Urine output was 400 ml and was red in color. The patient's recovery from general anesthesia was uneventful and she was shifted to the postoperative room under observation with stable vitals and in a state of consciousness. She was advised to stay in the head-up position and was administered oxygen with a venturi mask for 6 h. The patient then received methyl prednisolone (1 g) once a day and tranexamic acid (500 mg) three times a day for 5 days. One unit of whole blood was administered very slowly in the postoperative period. After 6 h, the patient was stable with normal vitals and voided clear urine. The mother and baby were followed up for 5 days postoperatively and there were no remarkable observations.


  Discussion Top


The cardinal feature of SLE is immune-mediated tissue injury, and major components of the immune system include cellular elements (lymphocytes, plasma cells, and macrophages), humoral products of cells (immunoglobulins), and complements. These three components interact to rid the body of foreign material and pathogenic organisms. However, detrimental effects can occur if they cause inflammation and tissue damage or if the immune response is directed against autoantigens [6] . Immune system injury can occur by the following four mechanisms: anaphylaxis (type I), cytotoxic (type II), circulating immune complex (type III), and delayed hypersensitivity (type IV). SLE represents a type III or immune complex disease [6] .

In our case, patient had very low platelet count and also had fetal distress, hence the added trial of labor was avoided and the patient was directly posted for elective cesarean section. A precious pregnancy with bad obstetric history and a well-managed mid-trimester complication was also an indication for lower segment cesarean section. The following conditions must be looked for during evaluation of the patient: previous bad obstetric history, renal or cardiac involvement, pulmonary hypertension and interstitial lung disease, evidence of active disease, high-dose steroids and immunosuppressive therapy, antiphospholipid antibody syndrome, and anti-Ro-La antibodies. Each of these conditions has a specific implication in the management of the patient. High titer of anti-double-stranded DNA may predict lupus flares, especially when coupled with decreasing complement levels [7],[8] , which was seen in our case. However, exacerbations of maternal disease are said to occur in 30-50% of pregnancies [9],[10],[11] . This frequency is two- to three-fold greater if lupus is active at the time of conception, with a high proportion of exacerbation during the second and third trimesters and the postpartum period [12] . Pregnancy does not alter the long-term course of disease and, consequently, the continuation of pregnancy with manageable complications is possible. It is accepted that active disease during pregnancy does not require abortion but should be treated with the necessary dosage of steroids required for disease control, which was also the procedure carried out in our case. Maternal deaths have only been reported in patients who were not treated with steroids and subsequently developed lupus crisis unresponsive to therapy [10] . Mintz and Rodriquez-Alvarez [9] have recommended a minimum dose of prednisone for all pregnant lupus patients.

Spontaneous abortion is twice as frequent as that in the general population and is not influenced by active or inactive maternal disease [9] , as seen in our case. Prematurity is a constant hazard occurring in 35-50% of pregnancies and is associated with intrauterine malnutrition and is related to active maternal disease [10] . An abnormal partial tromboplastin time (PTT) can be identified early in pregnancy and is frequently associated with thrombocytopenia. With high-dose steroid therapy, PTT frequently returns to normal, whereas low platelet counts may be reversed with acetyl salicylic acid (ASA) [11],[13] . A few patients may develop low platelets in the first trimester of pregnancy, particularly those with a history of thrombocytopenia or lupus antibody (LA), which is resistant to steroid therapy. It is associated with mid-trimester fetal death and spontaneously reverts to normal postpartum. The development of thrombocytopenia during the latter weeks of pregnancy indicates pregnancy-induced hypertension and should be managed as such [14] . In our case, aspirin was not administered as the patient had repeated attacks of enteric fever.

Perioperative anesthetic management must be tailored to the individual patient and includes all types of anesthesia but must take into account the multisystem nature of the disease, severity of organ involvement, and drugs used in treatment. Also, the usual anesthetic considerations for the pregnant patient should apply. A careful systemic approach to the patient will identify the organs damaged by the disease and allow one to plan the anesthetic technique appropriately. Similarly, preanesthetic investigation and intraoperative monitoring must be tailored according to the disease manifestations in the patient. Appropriate monitoring should include five-lead ECG because unexpected intraoperative myocardial ischemia in such patients has been reported [15] . In our patient, PTT was normal but there was severe thrombocytopenia, which was corrected partially by blood substitutes, and then the patient was prepared for general anesthesia, which results in potential drug interactions with immunosuppresants and was fortunately not a worry in our case as no immunosuppressant was continued in the patient. Usually azathioprine, an antimetabolite immunosuppressor, may interact with muscle relaxants, and dose increases of 37% with atracurium, 20% with vecuronium, and 45% with pancuronium were required in one study [16] . Cyclophosphamide acts as a pseudocholinesterase inhibitor and this may explain the risk of prolonged apnea after succinylcholine use [17] . Coadministration of NSAIDs with methotrexate has known deleterious effects, with several case reports describing acute renal failure and pancytopenia and in proximity to nitrous oxide exposure may precipitate bone marrow suppression [18] . However, the anesthesiologist must be warned about unexpected difficult airways with subglottic stenosis or laryngeal edema, unrecognized myocardial ischemia, and thrombotic risk during general anesthesia. The patient is prone to atelectasis (vanishing lung syndrome) and pneumonia (due to leukopenia). Strict asepsis should be maintained for invasive procedures such as central line cannulation and arterial line insertion. Maintenance of normothermia by the use of warm fluids and covering of the exposed body parts decreases the impact of Reynaud's phenomenon [19] .

The serum of patients with SLE contains an array of antibodies [20] and a number of these bind to diverse nucleic acid antigens such as DNA, RNA, various nucleoproteins and histones; of these, anti-RO/SSA is responsible for the syndrome of neonatal lupus. RO/SSA is an RNA protein complex whose function in the cell is presently unknown. Antibodies against it are IgGs and are therefore able to cross the placenta and cause disease in the fetus. It is a relatively infrequent clinical syndrome found exclusively in infants of mothers with anti-RO antibody [11],[12],[20] . Congenital heart block is the most common manifestation [1],[21] and usually presents as a stable bradycardia noted on routine obstetrical examination. Occasionally, pericarditis and other structural cardiac abnormalities such as extensive endocardial fibrosis have been reported [20],[22] . Fortunately, even though our case is anti-RO/SSA positive, the baby did not develop any transient or permanent cardiac clinical manifestations during the 1-week observation period. Tranexamic acid was started in the postoperative period and the mother as well as the baby fared well during the 1 week of observation.


  Conclusion Top


Close antenatal clinical and laboratory monitoring of mother and fetus with judicious use of steroids, aggressive management of active disease, and improved neonatal intensive care have decreased maternal and fetal morbidity and have increased the survival of newborns.


  Acknowledgements Top


Conflicts of interest

There are no conflicts of interest.

 
  References Top

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Ozaki M, Minami K, Shigematsu A. Myocardial ischemia during emergency anesthesia in patients with systemic lupus erythematosus resulting from undiagnosed antiphospholipid syndrome. Anesth Analg 2002; 95:255.  Back to cited text no. 15
    
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Chauhan G, Gupta K, Kashyap C, Nayar P. Anaesthetic management of patient with SLE and thrombocytopenia for vaginal hysterectomy. Anesth Essays Res 2013; 7:136-137.  Back to cited text no. 19
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