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Year : 2001  |  Volume : 47  |  Issue : 3  |  Page : 196-8

Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension.

Department of Anaesthesiology & Intensive Care, Nizam's Institute of Medical Sciences, Hyderabad, India. , India

Correspondence Address:
R R Sherke
Department of Anaesthesiology & Intensive Care, Nizam's Institute of Medical Sciences, Hyderabad, India.
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Source of Support: None, Conflict of Interest: None

PMID: 11832624

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 :: Abstract 

The management of idiopathic thrombocytopenic purpura (ITP) during pregnancy, especially with ongoing bleeding diathesis, has not been highlighted sufficiently in the literature. Aortocaval compression and reduction in uteroplacental circulation resulting in foetal hypoxia and acidosis, Mendelson's syndrome due to gravid uterus, trauma to airway with resultant haemorrhage and aspiration into lungs, compromised airway due to short neck, anasarca and heavy breast, limitation in using invasive monitoring and regional anaesthesia and uncontrolled bleeding leading to placental hypoperfusion and foetal hypoxia are some of the important risks. In the present case report, anaesthetic management for splenectomy during pregnancy complicated with pregnancy induced hypertension and bleeding diathesis secondary to ITP is described with reference to above risks.

Keywords: Adult, Anemia, Hemolytic, complications,therapy,Anesthesia, General, methods,Case Report, Female, Hemorrhage, etiology,Human, Pre-Eclampsia, etiology,Pregnancy, Pregnancy Complications, Hematologic, therapy,Purpura, Thrombocytopenic, Idiopathic, complications,therapy,Splenectomy, Syndrome, Treatment Outcome,

How to cite this article:
Sherke R R, Rao M S. Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension. J Postgrad Med 2001;47:196

How to cite this URL:
Sherke R R, Rao M S. Anaesthetic management of splenectomy in Evan's syndrome during pregnancy with pregnancy induced hypertension. J Postgrad Med [serial online] 2001 [cited 2023 Sep 22];47:196. Available from:

Idiopathic thrombocytopenic purpura (ITP) accounts for 3% of all causes of thrombocytopaenia during pregnancy and occurs with the frequency between 1:1000 and 1:5000 pregnancies.[1] Occasionally, it is associated with autoimmune haemolytic anaemic (AIHA) constituting Evanís syndrome. Platelet substitution, corticosteroids and immunoglobulin have been used widely to restore platelet count allowing pregnancy to progress to full-term. Splenectomy, during pregnancy or at caesarean section, is also undertaken to normalise platelet count.[2] In this report, we discuss anticipated anaesthetic risks and its impact on anaesthetic management in presence of severely depleted platelet count and haemorrhagic diathesis.

  ::   Case report Top

A 22-year-old female presented with haemorrhagic diathesis in 22nd week of third pregnancy. Previously, she developed haemorrhagic diathesis perioperatively during caesarean section for prolonged labour at first pregnancy. Multiple fresh blood & platelet transfusions were required over many days postoperatively for control of this catastrophe. Second pregnancy resulted in spontaneous abortion in second trimester. She developed epistaxis, bleeding gums and malena in twelfth week of third pregnancy. Investigations revealed haematocrit 24%, platelet count 44,000/cmm and bleeding time (BT) 20 minutes. Clotting, prothrombin (PT) and activated thromboplastin time (APTT) were normal. Serum fibrinogen was 280 mg/dl (normal 200 to 400 mg/dl). Peripheral blood smear and bone marrow examinations were suggestive of haemolytic anaemia and ITP. She received treatment with prednisolone (1 mg/kg/day) along with fresh blood and platelet transfusions. Platelet count remained below 50,000/cmm but overt bleeding tendency subsided within a week only to recur by 22nd week of gestation.

On hospitalisation, clinical examination revealed blood pressure of 160/90 mmHg, pedal oedema, petechiae and splenomegaly. Laboratory evaluation revealed platelet count 17,000/cmm, haematocrit 20.6% and reticulocyte count 3%. Peripheral blood smear revealed dual population of red blood cells, markedly diminished platelet count and frequent giant forms. Coagulation profile was normal and the level of plasma fibrinogen degradation products was not elevated. Liver function tests and serum uric acid were normal. Elevated serum lactate dehydrogenase (1370 mg/dl) and positive Coombís test indicated AIHA. Antinuclear, anticytoplasmic and anticardiolipin antibodies were negative. Ultrasonography did not show retroplacental bleeding or intracranial haemorrhage in the foetus. Thrombocytopaenia continued despite continuous corticosteroids therapy and platelet count could be elevated only transiently with platelet transfusion. Financial constraint precluded immunoglobulin therapy. She was scheduled for splenectomy.

Hypertension was controlled with nifedipine and prednisolone was continued. Three units of platelet concentrate were administered preoperatively. Investigations immediately prior to surgery were haemoglobin 7.5 gm%, platelet count 44,000/cmm, BT 4 minutes 10 seconds, PT 10/11 seconds and APPT 26/40 seconds. Premedication included midazolam (2 mg), ranitidine (150 mg)), atropine (0.5 mg) and metoclopramide (10 mg). She was transported to operative room with a wedge under the right hip. Following adequate oxygenation, induction of anaesthesia was achieved with fentanyl 50 micrograms and thiopentone 300 mg. Aided with Sellickís manoeuvre, tracheal intubation was done with portex cuffed endotracheal tube No. 6 after adequate muscle relaxation with suxamethonium 100 mg. Anaesthesia was maintained with low dose isoflurane (0.2-0.6%), 50% nitrous oxide in oxygen and incremental doses of fentanyl and atracurium. Intraoperative monitoring included electrocardiogram, pulse oximetry, end-tidal carbon-dioxide concentration, urine output and ultrasonic Doppler monitoring of foetal heart sound. During first 60-90 minutes of surgery, she received two units of blood transfusion. Surgery was complicated by estimated blood loss of 800-1000 ml during ligation of splenic pedicle requiring further transfusion of 4 units each of platelet concentrates and fresh blood. During surgery, mean arterial blood pressure (between 86 and 94 mmHg) and oxygen saturation (more than 92%) were stable. Ultrasonic Doppler monitoring of foetal heart sound revealed no evidence of foetal hypoxia. Total blood loss was estimated between 1100 and 1300 ml. Haemoglobin and platelet count were 7 gm% and 40,000/cmm respectively on completion of surgery.

One week after surgery, investigations revealed haemoglobin 10.2 gm%, haematocrit 32.5% and platelet count 120,000/cmm. Coagulation profile continued to remain within normal range and haemorrhagic diathesis subsided. Prednisolone was withdrawn gradually over 8 weeks. A male baby was delivered by caesarean section at 36 weeks of gestation. Platelet count and coagulation profile were within normal range at caesarean section. There was no evidence of neonatal thrombocytopaenia.

  ::   Discussion Top

Thrombocytopaenia during pregnancy is not an uncommon finding. ITP responds to corticosteroids and immunoglobulin therapy. Immunoglobulin therapy has prominent role in rapid restoration of the platelet count during bleeding diathesis, as splenectomy sparing measure and for preoperative preparation to caesarean section, splenectomy or other surgeries during pregnancy. In developing countries, use of immunoglobulin is influenced by financial affordability due to its high cost. Splenectomy is used either as definitive therapy or as last resort after other forms of therapy failed.[3] In the present case, financial constraints precluded adequate evaluation and management for two years and immunoglobulin therapy during third pregnancy and splenectomy was planned immediately due to corticosteroids resistance and bleeding diathesis consistent with the recommendations of the American Society of Hematology ITP Practice Guideline Panel.[3]

The frequency of non-obstetric surgeries during pregnancy is low, approximately 2 per 1000 cases.[4] Haemorrhagic diathesis due to ITP unresponsive to medical management presents considerable risk to mother as well as foetal well-being and requires immediate surgical intervention. Abdominal surgeries have been undertaken during pregnancy without much risk of premature labour, and foetal morbidity and mortality and decision to proceed with surgery during pregnancy should be guided by indications rather reluctance of anaesthetists and surgeons resulting from understandable concern.[5],[6] Splenectomy may increase the risk of spontaneous abortions during the first trimester and can be technically difficult to do in the third trimester, but no data are available on the magnitude of risk. Embryotoxicity of anaesthetic drugs during general anaesthesia has also been demonstrated in early pregnancy.[7] Second trimester is recommended to be suitable period for splenectomy.[3],[8]

Definite strategy was planned prior to surgery in the present case after identification of anticipated risks. Unmodified supine position hampers uteroplacental circulation due to aortocaval compression by gravid uterus, which can be prevented greatly by lateral position.[9] Lateral position was not practicable in the present case because supine position was needed for surgical access. Hence, a wedge was put under right hip causing uterine displacement to the left and thereby avoiding aortocaval compression. Aspiration of gastric contents (Mendelsonís syndrome) was also another known complication of surgeries during pregnancy and its occasional fatal nature is described since long ago.[10] Preoperative medication, Sellickís manoeuvre and rapid sequence induction were used to prevent Mendelsonís syndrome. Risk of haemorrhage in respiratory tract due to thrombocytopenia and compromised airway was avoided by pretreatment with platelet transfusion and smooth, rapid and atraumatic intubation with the help of smaller endotracheal tube, adequate expertise and better muscle relaxation with suxamethonium scoline. Surgery and anaesthesia during pregnancy entails the monitoring of two patients i.e. mother and foetus. During intraoperative period, maintenance of adequate arterial pressure is mandatory to avoid uterine hypoperfusion and hypoxia, and thus premature labour.[6] After 16th week of gestation, continuous monitoring of foetal heart rate may be useful for early detection of foetal hypoxia in the perioperative period and was adapted in the present case.[5],[11]

In summary, management of refractory ITP during pregnancy must be individualised. Pertinent to developing countries, high cost precludes the use of immunoglobulin. Splenectomy may help these patients immensely and can be safely undertaken during second trimester of pregnancy. However, as a part of pre-anaesthetic evaluation, strategic planning is essential for anticipating difficulties like haemorrhage in respiratory tract, aspiration of gastric contents and foetal hypoxia, and for controlling these problems promptly.

 :: References Top

1. Gill KK, Kelton JG. Management of autoimmune thrombocytopenic purpura in pregnancy. Semin Hematol 2000; 37:275-289.  Back to cited text no. 1    
2.Gottlieb P, Axelsson O, Bakos O, Rastad J. Splenectomy during pregnancy: an option in the treatment of autoimmune thrombocytopenic purpura. Br J Obstet Gynaecol 1999; 106:373-375.  Back to cited text no. 2    
3.Diagnosis and treatment of idiopathic thrombocytopenic purpura: Recommendations of the American Society of Hematology. The American Society of Hematology ITP Practice Guideline Panel. Ann Intern Med 1997; 126:319-326.  Back to cited text no. 3    
4.Kammerer WS. Nonobstetric surgery in pregnancy. Med Clin North Am 1987; 71:551-560.  Back to cited text no. 4    
5.Barron WM. The pregnant surgical patient: medical evaluation and management. Ann Intern Med 1984; 101:683-691.  Back to cited text no. 5    
6.Gianopoulos JG. Establishing the criteria for anesthesia and other precautions for surgery during pregnancy. Surg Clin North Am 1995; 75:33-45.  Back to cited text no. 6    
7.Strunin L, Knights K, Strunin JM, Ward ME. General anaesthesia during early pregnancy. Br J Surg 1975; 62:471-473.  Back to cited text no. 7    
8.McMillan R. Therapy for adults with refractory chronic thrombocytopenic purpura. Ann Intern Med 1997; 126:307-314.  Back to cited text no. 8    
9.Smith BE. Obstetrics. In: Martin JT, Warner MA, editors. Positioning in anaesthesia and surgery, 3rd edn. Philadelphia; WB Saunders Company: 1997. pp267-279.  Back to cited text no. 9    
10.Robinson JS, Thompson JM. Fatal aspiration (Mendelsonís) syndrome despite antacids and cricoid pressure. Lancet 1979; 2:228-230.  Back to cited text no. 10    
11.Liu PL, Warren TM, Ostheimer GW, Weiss JB, Liu LM. Foetal monitoring in parturients undergoing surgery unrelated to pregnancy. Can Anesth Soc J 1985; 32:525-532.   Back to cited text no. 11    

This article has been cited by
1 A case of Evans syndrome in pregnancy refractory to primary treatment options
Boren T, Reyes C, Montenegro R, et al.
2 Laparoscopic splenectomy in a pregnant patient with immune thrombocytopenic purpura
Felbinger TW, Posner M, Eltzschig HK, et al.
3 Evans syndrome and pregnancy: A case report
Phupong, V., Sareepapong, W., Witoonpanich, P.
BJOG: An International Journal of Obstetrics and Gynaecology. 2004; 111(3): 274-276
4 Idiopathic thrombocytopenic purpura in pregnancy: A single institutional experience with maternal and neonatal outcomes
Ali, R., Özkalemkaş, F., Özçelik, T., et al
Annals of Hematology. 2003; 82(6): 348-352


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© 2004 - Journal of Postgraduate Medicine
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