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Anaesthesia for caesarean section in a patient with systemic amyloidosis secondary to familial Mediterranean fever
International Journal of Obstetric Anesthesia (1998) I, 271-274 0 1998 Harcourl Brace & Co. Ltd
CASE REPORT
Anaesthesia for caesarean section in a patient with systemic amyloidosis secondary to familial Mediterranean fever F?S. Weir, C.C. McLaughlin Department of Anaesthetics, Belfast City Hospital, Belfast, UK SUMMARY. The anaesthetic management of a 33-year-old primigravid woman at 29 + 5 weeks’ gestation with familial Mediterranean fever (FMF), secondary amyloidosis, renal allograft with deteriorating renal function and cardiac impairment for emergency caesarean section is described. Pathophysiology and management options are discussed. Cautious induction of epidural anaesthesia together with continuous invasive monitoring produced a good outcome for mother and baby.
INTRODUCTION
pregnancy. Her blood pressure had since risen to 200/l 15 mmHg, her serum creatinine concentration had risen to 167 mmoYL (normal range 40-100) and urea to 10.0 mmoYL (normal range 3.3-8.8) and she had developed marked proteinuria and dependent oedema. In 1987 she developed intermittent supraventricular tachycardia. A myocardial biopsy showed this to be due to deposition of amyloid. She was prescribed propranolol which controlled her symptoms. In 1989 asthma was diagnosed and she was given terbutaline by inhaler. An echocardiogram carried out in 1993 showed a left ventricular ejection fraction of 60% with mild mitral regurgitation. A more recent echocardiogram had been carried out but the report was not available on the evening of surgery. A preoperative resting 1Zlead ECG showed left atria1 enlargement but was otherwise normal. There was some concern that her cardiac amyloid had worsened since the original echocardiogram, as on questioning, the patient complained of occasional palpitations and some chest tightness and shortness of breath on climbing stairs. In 1995 the patient was found to be hyperthyroid and was treated with propylthiouracil until January 1996. At the time of surgery, she remained clinically euthyroid. Her medication on admission consisted of prednisolone, azathioprine, propranolol, nifedipine, terbutaline and ranitidine. Laboratory investigations showed a haemoglobin concentration of 10.5 g/dL, a platelet count of 363 x 109/L and elevated serum creatinine and urea. Her prothrombin time, activated partial thromboplastin time, thrombin clotting time, fibrinogen and fibrin degradation products were all within normal limits.
Familial Mediterranean fever (FMF) is an inflammatory condition affecting serosal surfaces such as the peritoneum, pleura and joints. It is very rare in northern Europe, but when the condition does occur here, its well-established association with amyloidosis may have implications for the anaesthetist. We report the management of a patient with FMF, secondary systemic amyloidosis, renal allograft and asthma for emergency caesarean section. The patient presented with cardiac amyloid, hypertension and deteriorating renal function.
CASE HISTORY In April 1996 a 33-year-old primigravid patient at 29 + 5 weeks gestation was admitted to the labour ward at 19:00 for urgent caesarean section, the indications being uncontrolled hypertension and deteriorating renal function. She gave a complex past medical history. In 1983 she had been investigated for severe arthralgia and myalgia, and a diagnosis of FMF had been made. She subsequently developed rapidly progressive renal failure due to amyloid deposition and received a renal transplant in 1984. The renal allograft had functioned well until the second trimester of her FFARCSI, Queen’s University of Belfast, Department of Anaesthetics, Whitla Medical Building, 97 Lisburn Road, Belfast BT9 7BL, UK. Fax: 01232 329605; E-mail: [email protected]
Dr P. S. Weir
271
272
International Journal
qf Obstetric Anesthesia
With the limited amount of information and time available, it was decided that the safest way to proceed with this case was under epidural anaesthesia. Preoperatively, a 16 gauge intravenous cannula, a triple-lumen right internal jugular catheter and a 22 gauge radial arterial cannula were inserted. A urinary catheter was already in place. ECG, arterial blood pressure, central venous pressure and peripheral oxygen saturation were monitored throughout. Arterial pressure was initially 200/l 15 mmHg and her CVP on insertion of the central line was 19 cm H,O. Oxygen 6 L/min was given by face mask. A fluid pre-load of human plasma protein fraction (HPPF) 400 ml was given over 15 min. An 18 gauge epidural catheter was placed at the L,, interspace through a 16 gauge Tuohy needle. Over the next 20 min 32 ml of lignocaine 2% with adrenaline 1:200 000 and fentanyl 50 l..tgwere given epidurally. A sensory block to T, was obtained. A further 1000 ml of Hartmann’s solution was given during surgery. No vasopressors were required at any stage. Surgery was uneventful. Maternal blood pressure fell gradually to 132/87 mmHg at the time of skin incision, then rose slowly again to 165/95 mmHg at the end of surgery. The CVP fell to 8 cmH,O. The patient remained in sinus rhythm throughout. A baby girl weighing 1370 g was delivered, with Apgar scores of 4 at 1 min and 7 at 5 min. She was intubated and transferred to the special care baby unit. She subsequently progressed well and was discharged home with no apparent problems. At the end of surgery the mother was admitted to the adult intensive care unit. Postoperative analgesia was provided by a continuous epidural infusion of bupivacaine 0.1% with fentanyl 5 @ml, and dopamine was infused at a rate of 5 yg.kg-‘.min-’ overnight to improve renal function. Her blood pressure remained elevated, requiring a hydralazine infusion. Intravenous fluids were titrated according to the patient’s CVP, urinary output, serum urea and electrolyte concentrations. Over the next 3 days her blood pressure and serum creatinine gradually fell to prepregnancy levels and she was discharged to the postnatal ward. Her subsequent recovery was uneventful.
DISCUSSION
The most disturbing aspect of this case is the lack of communication between the health care professionals involved in the management of this woman’s condition. As soon as she presented to the antenatal clinic for booking, a multi-disciplinary meeting should have been arranged involving nephrologists, cardiologists, obstetricians, midwives and anaesthetists to plan
the optimum management of her pregnancy. Unfortunately, in our hospital, no formal mechanism exists for such an eventuality. Some larger obstetric units hold joint clinics involving specialists from all disciplines likely to have contact with high-risk pregnancies; we rely on the good sense of our colleagues to recognize and discuss potential problems with us. Unfortunately, some patients, such as this woman, slip through the net. This provides further evidence of the need to establish such case conferences to identify and formulate management plans for high-risk cases. FMF is a chronic illness characterized by periodic repeated bouts of fever associated with inflammation of serosal surfaces, causing peritonitis, pleuritis, arthritis and skin lesions.’ It affects almost exclusively people of Mediterranean and Middle Eastern origin and has been shown to be an autosomal recessive condition, although recent evidence suggests that there may be alternative modes of inheiritance.2 Although born in Ireland, our patient’s mother is of Mediterranean extraction. Anaesthesia for patients with uncomplicated FMF does not present any particular problems. However 25% of FMF patients go on to develop amyloidosis.3 Deposition of AA amyloid, a fibrous nonimmunoglobulin protein, usually begins in the first or second decade of life and advances at an individual, genetically determined rate.4 Deposits are found in the intima and media of arterioles, the sub-endothelial region of venules and in glomeruli.3 Nephrotic syndrome and renal failure are the commonest causes of death in FMF patients,5 and early transplantation is recommended.6 More rarely, cardiac deposition can occur affecting muscle fibres, valves and conducting tissue. Clinically, it tends to present late in the disease process, and may manifest as restrictive cardiomyopathy, arrhythmias or left ventricular failure. Neurologic involvement in amyloidosis secondary to FMF is very rare,7 although optic neuritis has been reported.8 Interestingly, it has been reported that FMF may protect against asthma.9 Primary infertility is more common in patients with FMF than in the general population. If pregnancy does occur, there is a high incidence of severe maternal and fetal complications, such as asymmetrical intra-uterine growth retardation, superimposed preeclampsia, thromboembolism and resistant anaemia.*O In addition, it has been reported that pregnancy has a deleterious effect on renal function in amyloid nephropathy.” Overall, fetal wastage of up to 30% has been reported. ‘* Colchicine therapy modifies the natural history of FMF by decreasing the attack frequency and preventing amyloid deposition.13 Despite being a potent inhibitor of mitotic activity, it does not appear to harm mother or fetusI Our patient, however, was not on colchicine treatment.
Caesarean section in a patient with systemic amyloidosis secondary to FMF Pregnancy in healthy women normally causes a sustained increase in glomerular filtration rate (GFR). Although transplanted allografts are ectopic, denervated and often derived from male donors, they too display a rise in GFR.15 Proteinuria and a reduction in GFR do occur in the third trimester but are usually transient and there is no evidence of pregnancy causing long-term deterioration in allograft function.16 However, preeclampsia occurs in 30% of pregnant women with renal allografts.is This condition, especially when superimposed on renal disease, is the disorder most often associated with serious maternal and fetal complications. In these cases, the allograft also behaves as a native kidney, with enlarged, swollen glomeruli at risk of ischaemia and a 25% reduction in GFR.” Proteinuria may be severe, and the patient may become profoundly hypo-proteinaemic, as well as having the contracted blood volume and increased peripheral vascular resistance associated with preeclampsia.‘* Thus, our priorities in this case were to provide adequate surgical anaesthesia without compromising a premature fetus, while controlling blood pressure, maintaining renal function and avoiding provocation of either arrhythmias or a reduction in cardiac output. Arterial and CVP monitoring were used throughout the peri-operative period. While the only echocardiogram available to us was 3-years-old and showed an ejection fraction of 60%, we were concerned that her cardiac performance may have deteriorated in the interim in view of her worsening symptoms. Although there can be discrepancies between CVP and pulmonary capillary wedge pressure in preeclampsia,18 we opted not to use a pulmonary artery catheter in this case and to insert one only if her cardiovascular status deteriorated. Although it is possible to obtund the hypertensive response to intubation in preeclamptic patientsi the effect of general anaesthesia on elevated levels of stress hormones, such as corticotrophin, P-endorphin and catecholamines is less clear. Regional anaesthesia, however, causes blunting of both haemodynamic and neuroendocrine responses,*Oand so it was decided in this case to perform a regional block to prevent rises in blood pressure and to avoid provoking supraventricular tachycardia. Although some authors have reported no difference in fetal outcome between epidural and spinal anaesthesia,*’ others have demonstrated greater neonatal acidaemia following spinal anaesthesia.** Although hypotension can occur in preeclamptic patients with either technique, the fall in blood pressure can be precipitous with rapid onset of sympathetic blockade. We, therefore, decided that gradual induction of epidural anaesthesia was the technique of choice in this case.
273
Despite our patient’s elevated CVP and deteriorating renal function, we elected to give a fluid pre-load prior to establishing the epidural block in an attempt to avoid hypotension. Colloid solutions tend to remain in the vascular compartment longer than crystalloids, which have to be given in larger volumes to achieve the same degree of plasma volume expansion, and may contribute more to oedema formation.23 In view of this, and the hypoproteinaemia associated with preeclampsia, we chose to give HPPF. We chose to use lignocaine rather than bupivacaine to increase the speed of onset and also because of its lesser tendency to cardiotoxicity. It has been shown that adrenaline is absorbed rapidly from the epidural space and significantly elevates maternal circulating adrenaline concentrations,24 and occurrence of a hypertensive crisis following its use in a hypertensive patient has been reported. 25 However, the addition of adrenaline produces adequate surgical anaesthesia with smaller doses of lignocaine, with lower maternal and fetal plasma lignocaine concentrations and with no adverse effect on feto-placental circulation or on fetal outcome.26 In our patient, no tachycardia or acute rise in arterial pressure was observed due to the added adrenaline. No vasopressors were required before or during surgery to maintain arterial pressure. In normal circumstances if hypotension did occur, our drug of choice would be ephedrine. However, we would be concerned that its p-stimulant effects may have precipitated arrhythmias. It could be argued that in this case an a-agonist, such as methoxamine, would have been a better choice. Its effect on utero-placental circulation is not significantly different from that of ephedrine in healthy parturients, and the most important factor for fetal well-being is the avoidance of hypotension.27 In retrospect, it may have been safer to avoid the use of adrenaline in this patient given the risk of exacerbating her hypertension. Our decision, made purely on clinical grounds, not to use a pulmonary artery catheter from the outset was later supported by the discovery that her most recent echocardiogram did not show any further deterioration in cardiac function. If, however, her condition had deteriorated at any stage, we would not have hesitated to insert one. Combined spinalepidural anaesthesia is safe in preeclampsia.19 It has been suggested that with this technique it is possible to provide better analgesia with much lower doses of local anaesthetic, and with a lower incidence of hypotension in healthy parturients. 28However, we are not sure that the technique offers significant advantages over a carefully established epidural block in a case such as this. Despite these reservations about our management of the case, we are happy to report a good outcome for both mother and baby.
274
International
Journal of Obstetric Anesthesia
REFERENCES 1. Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean Fever. A survey of 470 casesand review of the literature. Am J Med 1967; 43: 227-253. 2. Yuval Y, Heino-Zisser M, Zemer D, Sohar E, Pras M. Dominant inheiritance in two families with Familial Mediterranean Fever. Am J Med Gen 1995; 57: 455457. 3. Wolff S M. Familial Mediterranean Fever. In: Wilson J D, Braunwald E, Isselbacher K J et al (eds): Harrison’s Principles of Internal Medicine, ed 12. New York: McGraw-Hill, 1991; 1469-1471. 4. Gafni J, Ravid M, Sohar E. The role of amyloidosis in Familial Mediterranean Fever. A population study. Isr J Med Sci 1968; 4: 995-999. 5. Cohen A S. Amyloidosis. In: Wilson J D, Braunwald E, Isselbacher K J, et al (eds): Harrison’s Principles of Internal Medicine, ed 12. New York: McGraw-Hill, 1991; 1417-1421. 6. Schmueli D, Lustig S, Nakache R et al. Renal transplantation in patients with amyloidosis due to Familial Mediterranean Fever. Transplant Proc 1992; 24: 1783-1784. I. Gedalia A, Zamir S. Neurologic manifestations in Familial Mediterranean Fever. Pediatr Neurol 1993; 9: 301-302. 8. Lossos A, Eliashiv S, Ben-Chetrit E, Reches A. Optic neuritis associated with Familial Mediterranean Fever. J Clin Neuroophthalmoll993; 13: 141-143. 9. Brenner-Ullman A, Melzer-Ofir H, Daniels M, Shohat M. Possible protection against asthma in heterozygotes for Familial Mediteranean Fever. Am J Med Gen 1994; 53: 172-175. 10. Mordel N, Birkenfeld A, Rubinger D, Schenker J G, Sadovsky E. Successful full-term pregnancy in familial Mediterranean fever complicated with amyloidosis: case report and review of the literature. Fetal Diagn Ther 1993; 8: 129-134. 11. Livneh A, Cabili S, Zemer D, Rabinovitch 0, Pras M. Effect of pregnancy on renal function in amyloidosis of familial Mediterranean fever. J Rheum 1993; 20: 1519-1523. 12. Schwartz J. Periodic peritonitis, onset simultaneously with menstruation. Ann Intern Med 1960; 153: 407. 13. Garcia-Gonzalez A, Weisman M H. The arthritis of familial Mediterranean fever. Arthritis Rheum 1992; 22: 139-150. 14. Rabinovitch 0, Zemer D, Kukia E, Sohar E, Mashiach S. Colchicine treatment in conception and pregnancy: 23 1 pregnancies in patients with familial Mediterranean fever. Am J Reprod Immun 1992; 28: 245-246.
15. Davison J M. Dialysis, transplantation and pregnancy. Am J Kidney Dis 1991; 17: 127-132. 16 Sturgis S N, Davison J M. Effect of pregnancy on long-term function of renal allografts. Am J Kidney Dis 1992; 19: 1677172. 17 Cunningham F G, Lindheimer M D. Current concepts: hypertension in pregnancy. N Eng J Med 1992; 326: 927-932. 18 Ramanathan J. Pathophysiology and anesthetic implications in oreeclamosia. Clin Obstet Gvnecol 1992: 35: 414425. 19 Wallace D H, Leveno K J, Cunningham F G, Giesecke A H, Shearer V E, Sidawi J E. Randomized comparison of general and regional anesthesia for cesarean delivery in pregnancies complicated by severe preeclampsia. Obstet Gynecol 1995; 86: 193-199. 20. Ramanathan J, Coleman P, Sibai B M. Anesthetic modification of hemodynamic and neuroendocrine responses to cesarean delivery in women with severe preeclampsia. Anesth Analg 1991; 73: 172. 21. Karinen J, Rasanen J, Alahuta S, Joupilla R, Joupilla P. Maternal and uteroplacental haemodynamic state in preeclamptic patients during spinal anaesthesia for caesarean section. Br J Anaesth 1996; 76: 616-620. 22. Ratcliffe F M, Evans J M. Neonatal wellbeing after elective caesarean delivery with general, spinal and epidural anaesthesia. Eur J Anesth 1993; 10: 175-181. 23. Griffel M I, Kaufman B S. Pharmacology of colloids and crystalloids. Crit Care Clin 1992; 8: 235-253. 24. Ramanathan S, Desai N S, Zakowski M. Systemic vascular uptake of epinephrine from the lumbar epidural space in parturients. Ree Anesth 1995: 20: 199-205. 25. Hadzic A, Vloka J, Pate1 N, Bimbach D. Hypertensive crisis after a successful placement of an epidural anesthetic in a hypertensive parturient. Reg Anesth 1995; 20: 156158. 26. McLintic A J, Danskin F H, Reid J A, Thorburn J. Effect of adrenaline on extradural anaesthesia, plasma lignocaine concentrations and the feto-placental unit during elective caesarean section. Br J Anaesth 1991; 67: 683-689. 27. Wright P M, Iftikhar M, Fitzpatrick K T, Moore J, Thompson W. Vasopressor therapy for hypotension during epidural anesthesia for cesarean section: effects on maternal and fetal flow velocity ratios. Anesth Analg 1992; 75: 5663. 28. Rawal N, Schollin J, Wesstrom G. Epidural versus combined spinal epidural block for caesarean section. Acta Anaesthesiol Stand 1988; 32: 61-66.
CASE REPORT
Anaesthesia for caesarean section in a patient with systemic amyloidosis secondary to familial Mediterranean fever F?S. Weir, C.C. McLaughlin Department of Anaesthetics, Belfast City Hospital, Belfast, UK SUMMARY. The anaesthetic management of a 33-year-old primigravid woman at 29 + 5 weeks’ gestation with familial Mediterranean fever (FMF), secondary amyloidosis, renal allograft with deteriorating renal function and cardiac impairment for emergency caesarean section is described. Pathophysiology and management options are discussed. Cautious induction of epidural anaesthesia together with continuous invasive monitoring produced a good outcome for mother and baby.
INTRODUCTION
pregnancy. Her blood pressure had since risen to 200/l 15 mmHg, her serum creatinine concentration had risen to 167 mmoYL (normal range 40-100) and urea to 10.0 mmoYL (normal range 3.3-8.8) and she had developed marked proteinuria and dependent oedema. In 1987 she developed intermittent supraventricular tachycardia. A myocardial biopsy showed this to be due to deposition of amyloid. She was prescribed propranolol which controlled her symptoms. In 1989 asthma was diagnosed and she was given terbutaline by inhaler. An echocardiogram carried out in 1993 showed a left ventricular ejection fraction of 60% with mild mitral regurgitation. A more recent echocardiogram had been carried out but the report was not available on the evening of surgery. A preoperative resting 1Zlead ECG showed left atria1 enlargement but was otherwise normal. There was some concern that her cardiac amyloid had worsened since the original echocardiogram, as on questioning, the patient complained of occasional palpitations and some chest tightness and shortness of breath on climbing stairs. In 1995 the patient was found to be hyperthyroid and was treated with propylthiouracil until January 1996. At the time of surgery, she remained clinically euthyroid. Her medication on admission consisted of prednisolone, azathioprine, propranolol, nifedipine, terbutaline and ranitidine. Laboratory investigations showed a haemoglobin concentration of 10.5 g/dL, a platelet count of 363 x 109/L and elevated serum creatinine and urea. Her prothrombin time, activated partial thromboplastin time, thrombin clotting time, fibrinogen and fibrin degradation products were all within normal limits.
Familial Mediterranean fever (FMF) is an inflammatory condition affecting serosal surfaces such as the peritoneum, pleura and joints. It is very rare in northern Europe, but when the condition does occur here, its well-established association with amyloidosis may have implications for the anaesthetist. We report the management of a patient with FMF, secondary systemic amyloidosis, renal allograft and asthma for emergency caesarean section. The patient presented with cardiac amyloid, hypertension and deteriorating renal function.
CASE HISTORY In April 1996 a 33-year-old primigravid patient at 29 + 5 weeks gestation was admitted to the labour ward at 19:00 for urgent caesarean section, the indications being uncontrolled hypertension and deteriorating renal function. She gave a complex past medical history. In 1983 she had been investigated for severe arthralgia and myalgia, and a diagnosis of FMF had been made. She subsequently developed rapidly progressive renal failure due to amyloid deposition and received a renal transplant in 1984. The renal allograft had functioned well until the second trimester of her FFARCSI, Queen’s University of Belfast, Department of Anaesthetics, Whitla Medical Building, 97 Lisburn Road, Belfast BT9 7BL, UK. Fax: 01232 329605; E-mail: [email protected]
Dr P. S. Weir
271
272
International Journal
qf Obstetric Anesthesia
With the limited amount of information and time available, it was decided that the safest way to proceed with this case was under epidural anaesthesia. Preoperatively, a 16 gauge intravenous cannula, a triple-lumen right internal jugular catheter and a 22 gauge radial arterial cannula were inserted. A urinary catheter was already in place. ECG, arterial blood pressure, central venous pressure and peripheral oxygen saturation were monitored throughout. Arterial pressure was initially 200/l 15 mmHg and her CVP on insertion of the central line was 19 cm H,O. Oxygen 6 L/min was given by face mask. A fluid pre-load of human plasma protein fraction (HPPF) 400 ml was given over 15 min. An 18 gauge epidural catheter was placed at the L,, interspace through a 16 gauge Tuohy needle. Over the next 20 min 32 ml of lignocaine 2% with adrenaline 1:200 000 and fentanyl 50 l..tgwere given epidurally. A sensory block to T, was obtained. A further 1000 ml of Hartmann’s solution was given during surgery. No vasopressors were required at any stage. Surgery was uneventful. Maternal blood pressure fell gradually to 132/87 mmHg at the time of skin incision, then rose slowly again to 165/95 mmHg at the end of surgery. The CVP fell to 8 cmH,O. The patient remained in sinus rhythm throughout. A baby girl weighing 1370 g was delivered, with Apgar scores of 4 at 1 min and 7 at 5 min. She was intubated and transferred to the special care baby unit. She subsequently progressed well and was discharged home with no apparent problems. At the end of surgery the mother was admitted to the adult intensive care unit. Postoperative analgesia was provided by a continuous epidural infusion of bupivacaine 0.1% with fentanyl 5 @ml, and dopamine was infused at a rate of 5 yg.kg-‘.min-’ overnight to improve renal function. Her blood pressure remained elevated, requiring a hydralazine infusion. Intravenous fluids were titrated according to the patient’s CVP, urinary output, serum urea and electrolyte concentrations. Over the next 3 days her blood pressure and serum creatinine gradually fell to prepregnancy levels and she was discharged to the postnatal ward. Her subsequent recovery was uneventful.
DISCUSSION
The most disturbing aspect of this case is the lack of communication between the health care professionals involved in the management of this woman’s condition. As soon as she presented to the antenatal clinic for booking, a multi-disciplinary meeting should have been arranged involving nephrologists, cardiologists, obstetricians, midwives and anaesthetists to plan
the optimum management of her pregnancy. Unfortunately, in our hospital, no formal mechanism exists for such an eventuality. Some larger obstetric units hold joint clinics involving specialists from all disciplines likely to have contact with high-risk pregnancies; we rely on the good sense of our colleagues to recognize and discuss potential problems with us. Unfortunately, some patients, such as this woman, slip through the net. This provides further evidence of the need to establish such case conferences to identify and formulate management plans for high-risk cases. FMF is a chronic illness characterized by periodic repeated bouts of fever associated with inflammation of serosal surfaces, causing peritonitis, pleuritis, arthritis and skin lesions.’ It affects almost exclusively people of Mediterranean and Middle Eastern origin and has been shown to be an autosomal recessive condition, although recent evidence suggests that there may be alternative modes of inheiritance.2 Although born in Ireland, our patient’s mother is of Mediterranean extraction. Anaesthesia for patients with uncomplicated FMF does not present any particular problems. However 25% of FMF patients go on to develop amyloidosis.3 Deposition of AA amyloid, a fibrous nonimmunoglobulin protein, usually begins in the first or second decade of life and advances at an individual, genetically determined rate.4 Deposits are found in the intima and media of arterioles, the sub-endothelial region of venules and in glomeruli.3 Nephrotic syndrome and renal failure are the commonest causes of death in FMF patients,5 and early transplantation is recommended.6 More rarely, cardiac deposition can occur affecting muscle fibres, valves and conducting tissue. Clinically, it tends to present late in the disease process, and may manifest as restrictive cardiomyopathy, arrhythmias or left ventricular failure. Neurologic involvement in amyloidosis secondary to FMF is very rare,7 although optic neuritis has been reported.8 Interestingly, it has been reported that FMF may protect against asthma.9 Primary infertility is more common in patients with FMF than in the general population. If pregnancy does occur, there is a high incidence of severe maternal and fetal complications, such as asymmetrical intra-uterine growth retardation, superimposed preeclampsia, thromboembolism and resistant anaemia.*O In addition, it has been reported that pregnancy has a deleterious effect on renal function in amyloid nephropathy.” Overall, fetal wastage of up to 30% has been reported. ‘* Colchicine therapy modifies the natural history of FMF by decreasing the attack frequency and preventing amyloid deposition.13 Despite being a potent inhibitor of mitotic activity, it does not appear to harm mother or fetusI Our patient, however, was not on colchicine treatment.
Caesarean section in a patient with systemic amyloidosis secondary to FMF Pregnancy in healthy women normally causes a sustained increase in glomerular filtration rate (GFR). Although transplanted allografts are ectopic, denervated and often derived from male donors, they too display a rise in GFR.15 Proteinuria and a reduction in GFR do occur in the third trimester but are usually transient and there is no evidence of pregnancy causing long-term deterioration in allograft function.16 However, preeclampsia occurs in 30% of pregnant women with renal allografts.is This condition, especially when superimposed on renal disease, is the disorder most often associated with serious maternal and fetal complications. In these cases, the allograft also behaves as a native kidney, with enlarged, swollen glomeruli at risk of ischaemia and a 25% reduction in GFR.” Proteinuria may be severe, and the patient may become profoundly hypo-proteinaemic, as well as having the contracted blood volume and increased peripheral vascular resistance associated with preeclampsia.‘* Thus, our priorities in this case were to provide adequate surgical anaesthesia without compromising a premature fetus, while controlling blood pressure, maintaining renal function and avoiding provocation of either arrhythmias or a reduction in cardiac output. Arterial and CVP monitoring were used throughout the peri-operative period. While the only echocardiogram available to us was 3-years-old and showed an ejection fraction of 60%, we were concerned that her cardiac performance may have deteriorated in the interim in view of her worsening symptoms. Although there can be discrepancies between CVP and pulmonary capillary wedge pressure in preeclampsia,18 we opted not to use a pulmonary artery catheter in this case and to insert one only if her cardiovascular status deteriorated. Although it is possible to obtund the hypertensive response to intubation in preeclamptic patientsi the effect of general anaesthesia on elevated levels of stress hormones, such as corticotrophin, P-endorphin and catecholamines is less clear. Regional anaesthesia, however, causes blunting of both haemodynamic and neuroendocrine responses,*Oand so it was decided in this case to perform a regional block to prevent rises in blood pressure and to avoid provoking supraventricular tachycardia. Although some authors have reported no difference in fetal outcome between epidural and spinal anaesthesia,*’ others have demonstrated greater neonatal acidaemia following spinal anaesthesia.** Although hypotension can occur in preeclamptic patients with either technique, the fall in blood pressure can be precipitous with rapid onset of sympathetic blockade. We, therefore, decided that gradual induction of epidural anaesthesia was the technique of choice in this case.
273
Despite our patient’s elevated CVP and deteriorating renal function, we elected to give a fluid pre-load prior to establishing the epidural block in an attempt to avoid hypotension. Colloid solutions tend to remain in the vascular compartment longer than crystalloids, which have to be given in larger volumes to achieve the same degree of plasma volume expansion, and may contribute more to oedema formation.23 In view of this, and the hypoproteinaemia associated with preeclampsia, we chose to give HPPF. We chose to use lignocaine rather than bupivacaine to increase the speed of onset and also because of its lesser tendency to cardiotoxicity. It has been shown that adrenaline is absorbed rapidly from the epidural space and significantly elevates maternal circulating adrenaline concentrations,24 and occurrence of a hypertensive crisis following its use in a hypertensive patient has been reported. 25 However, the addition of adrenaline produces adequate surgical anaesthesia with smaller doses of lignocaine, with lower maternal and fetal plasma lignocaine concentrations and with no adverse effect on feto-placental circulation or on fetal outcome.26 In our patient, no tachycardia or acute rise in arterial pressure was observed due to the added adrenaline. No vasopressors were required before or during surgery to maintain arterial pressure. In normal circumstances if hypotension did occur, our drug of choice would be ephedrine. However, we would be concerned that its p-stimulant effects may have precipitated arrhythmias. It could be argued that in this case an a-agonist, such as methoxamine, would have been a better choice. Its effect on utero-placental circulation is not significantly different from that of ephedrine in healthy parturients, and the most important factor for fetal well-being is the avoidance of hypotension.27 In retrospect, it may have been safer to avoid the use of adrenaline in this patient given the risk of exacerbating her hypertension. Our decision, made purely on clinical grounds, not to use a pulmonary artery catheter from the outset was later supported by the discovery that her most recent echocardiogram did not show any further deterioration in cardiac function. If, however, her condition had deteriorated at any stage, we would not have hesitated to insert one. Combined spinalepidural anaesthesia is safe in preeclampsia.19 It has been suggested that with this technique it is possible to provide better analgesia with much lower doses of local anaesthetic, and with a lower incidence of hypotension in healthy parturients. 28However, we are not sure that the technique offers significant advantages over a carefully established epidural block in a case such as this. Despite these reservations about our management of the case, we are happy to report a good outcome for both mother and baby.
274
International
Journal of Obstetric Anesthesia
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