International Journal of Obstetric Anesthesia
References 1. Auerbach M. IV Iron in pregnancy: an unmet clinical need. Am J Hematol 2014;89:789. 2. Auerbach M, Ballard H, Glaspy J. Clinical update: intravenous iron for anaemia. Lancet 2007;369:1502–4. 3. Szebeni J. Complement activation-related pseudoallergy: a new class of drug-induced acute immune toxicity. Toxicology 2005;216:106–21. 4. Monofer: summary of product characteristics. http://www.monofer. com/media/55311/monofer_smpc_03-2014.pdf [accessed January 2015]. 5. European Medicines Agency. Assessment report for: Iron containing intravenous (IV) medicinal products; 2013 (EMA/549569/ 2013). http://www.ema.europa.eu/docs/en_GB/document_library/ Referrals_document/IV_iron_31/WC500150771.pdf [accessed January 2015]. 0959-289X/$ - see front matter
c 2015 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijoa.2014.12.008
Marfan syndrome presenting with postpartum aortic dissection following dural puncture headache and epidural blood patch We would like to highlight the unusual way in which a patient was diagnosed with Marfan syndrome during an apparently routine obstetric case. A 33-year-old nulliparous woman, who had received routine antenatal care and had been assessed as American Society of Anesthesiologists class 1, presented to obstetric triage at 41 + 3 weeks of gestation in spontaneous labour. Her cervix was fully dilated. She was hypertensive (145/98 mmHg) with proteinuria and elevated serum urate (0.47 mmol/L) and was treated with oral labetalol, nifedipine and intravenous fluid. Initial cardiotocography (CTG) was reassuring. An oxytocin infusion was started to aid uterine contraction. Deterioration of the CTG, meconium-stained liquor and an occipito-posterior presentation prompted a decision to attempt rotational forceps delivery. Spinal anaesthesia in the midline at the L4–5 interspace, using a 24-gauge Sprotte needle in the sitting position, was successful at the first attempt. Hyperbaric bupivacaine 13 mg and diamorphine 300 lg produced bilateral sensory block to cold up to T5 at 3 min with a bilateral motor block. Instrumental delivery was performed uneventfully. Two 5 U boluses of oxytocin followed by an infusion of 10 U/h and ergometrine 500 lg were used to treat uterine atony (600 mL blood loss). Postoperatively, a second oxytocin infusion and ergometrine 500 lg were given for a further 200 mL blood loss. Ten hours after spinal anaesthesia, the patient had symptoms suggestive of a post-dural puncture headache (PDPH). Conservative treatment was ineffective. An epidural blood patch (EBP) at L3–4, 38 h after the initial
197 spinal, using 20 mL autologous blood, produced resolution of the headache. Eleven hours after EBP, she developed acute thoracic back pain at T6–8 on movement. This was initially thought to be musculoskeletal but was not relieved by oral morphine 20 mg and diazepam 4 mg. Anaesthetic review, to exclude EBP-related complications, prompted consideration of a diagnosis of aortic dissection and underlying Marfan syndrome. A Stanford type B thoracic aortic dissection was subsequently confirmed on computerised tomography angiogram (Fig. 1). She was admitted to the intensive care unit for medical management of the dissection. Bedside echocardiography showed a tricuspid aortic valve with trivial regurgitation, mild mitral regurgitation and a 4.4 cm dilated aortic root at the sinus of Valsalva. Marfan syndrome was confirmed based on clinical features and positive genetic testing for FBN-1 mutation. Following stabilisation and reassuring repeated imaging, she was discharged home on nifedipine and metoprolol 11 days postpartum with arrangements for vascular surgical follow-up. Aortic dissection is an uncommon event, for which pregnancy and Marfan syndrome are predisposing factors.1 We wish to highlight a few issues related to this case. The patient had not been diagnosed previously with Marfan syndrome and had no family history. Notably, she had no arched palate, cardiac murmurs, arachnodactyly or other stigmata other than her height (1.92 m) and myopia. While most pregnant patients remain well, rare high-risk conditions may present in pregnancy with potentially fatal consequences. In this case, the obstetric team thought the thoracic pain was musculoskeletal and prescribed analgesics and sedatives. The anaesthetic team were consulted to exclude com-
Fig. 1 Computerised tomography angiogram showing Stanford type B aortic dissection extending from 2 cm distal to left subclavian artery down to both common femoral arteries. The aortic arch was dilated at 3.5 cm.
198 plications of EBP but suggested the diagnosis of Marfan syndrome. This highlights the importance of multidisciplinary care, consultation with different specialities and consideration of alternative diagnoses when the patient’s condition fails to improve. Marfan syndrome confers up to a fourfold increased risk of PDPH given accidental or intentional dural puncture.2 Dural ectasia can lead to unpredictable and inadequate intrathecal anaesthesia with normal doses of local anaesthetic,3 theoretically due to increased cerebrospinal fluid volume.4 In our case, a 13 mg dose of bupivacaine was used given her extreme height. The larger dose may have mitigated problems posed by dural ectasia (subsequently seen on magnetic resonance imaging) but it is unclear if the block would have been adequate for caesarean delivery. It is fortunate that she did not have an accidental dural puncture during insertion of her EBP. The patient developed uterine atony and received two 5 U doses of oxytocin followed by an infusion, which is standard unit practice. The hypertensive effects of ergometrine are well recognised. Its use may have contributed to the dissection, although symptoms started approximately 48 h after her last dose. Aortic dissection can occur in 1–4.5% of Marfan syndrome pregnancies, dependent on aortic root diameter; gestational hypertension and preeclampsia are additional risk factors.1,5
Acknowledgments We would like to thank Dr Hamish Ireland for supplying us with the radiological image.
International Journal of Obstetric Anesthesia V. Humphrey, D. Falzon, V. Clark Simpson’s Centre for Reproductive Health Royal Infirmary of Edinburgh, Edinburgh, UK E-mail address: [email protected]
References 1. Curry RA, Gelson E, Swan L, et al. Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG 2014;121:610–7. 2. Youngblood SC, Tolpin DA, Cooper JR, Coselli JS, LeMaire SA. Complications of cerebrospinal fluid drainage after thoracic aortic surgery: a review of 504 patients over 5 years. J Thorac Cardiovasc Surg 2013;146:166–71. 3. Lacassie HJ, Millar S, Leithe LG, Habib AS, Muir HA. Dural ectasia: a likely cause of inadequate spinal anaesthesia in two parturients with Marfan’s syndrome. Br J Anaesth 2005;94:500–4. 4. Carpenter RL, Hogan QH, Liu SS, et al. Lumbosacral cerebrospinal fluid volume is the primary determinant of sensory block extent and duration during spinal anaesthesia. Anesthesiology 1998;89:24–9. 5. Chow SL. Acute aortic dissection in a patient with Marfan’s syndrome complicated by gestational hypertension. Med J Aust 1993;159:760–2. 0959-289X/$ - see front matter
c 2015 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijoa.2015.01.009
References 1. Auerbach M. IV Iron in pregnancy: an unmet clinical need. Am J Hematol 2014;89:789. 2. Auerbach M, Ballard H, Glaspy J. Clinical update: intravenous iron for anaemia. Lancet 2007;369:1502–4. 3. Szebeni J. Complement activation-related pseudoallergy: a new class of drug-induced acute immune toxicity. Toxicology 2005;216:106–21. 4. Monofer: summary of product characteristics. http://www.monofer. com/media/55311/monofer_smpc_03-2014.pdf [accessed January 2015]. 5. European Medicines Agency. Assessment report for: Iron containing intravenous (IV) medicinal products; 2013 (EMA/549569/ 2013). http://www.ema.europa.eu/docs/en_GB/document_library/ Referrals_document/IV_iron_31/WC500150771.pdf [accessed January 2015]. 0959-289X/$ - see front matter
c 2015 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijoa.2014.12.008
Marfan syndrome presenting with postpartum aortic dissection following dural puncture headache and epidural blood patch We would like to highlight the unusual way in which a patient was diagnosed with Marfan syndrome during an apparently routine obstetric case. A 33-year-old nulliparous woman, who had received routine antenatal care and had been assessed as American Society of Anesthesiologists class 1, presented to obstetric triage at 41 + 3 weeks of gestation in spontaneous labour. Her cervix was fully dilated. She was hypertensive (145/98 mmHg) with proteinuria and elevated serum urate (0.47 mmol/L) and was treated with oral labetalol, nifedipine and intravenous fluid. Initial cardiotocography (CTG) was reassuring. An oxytocin infusion was started to aid uterine contraction. Deterioration of the CTG, meconium-stained liquor and an occipito-posterior presentation prompted a decision to attempt rotational forceps delivery. Spinal anaesthesia in the midline at the L4–5 interspace, using a 24-gauge Sprotte needle in the sitting position, was successful at the first attempt. Hyperbaric bupivacaine 13 mg and diamorphine 300 lg produced bilateral sensory block to cold up to T5 at 3 min with a bilateral motor block. Instrumental delivery was performed uneventfully. Two 5 U boluses of oxytocin followed by an infusion of 10 U/h and ergometrine 500 lg were used to treat uterine atony (600 mL blood loss). Postoperatively, a second oxytocin infusion and ergometrine 500 lg were given for a further 200 mL blood loss. Ten hours after spinal anaesthesia, the patient had symptoms suggestive of a post-dural puncture headache (PDPH). Conservative treatment was ineffective. An epidural blood patch (EBP) at L3–4, 38 h after the initial
197 spinal, using 20 mL autologous blood, produced resolution of the headache. Eleven hours after EBP, she developed acute thoracic back pain at T6–8 on movement. This was initially thought to be musculoskeletal but was not relieved by oral morphine 20 mg and diazepam 4 mg. Anaesthetic review, to exclude EBP-related complications, prompted consideration of a diagnosis of aortic dissection and underlying Marfan syndrome. A Stanford type B thoracic aortic dissection was subsequently confirmed on computerised tomography angiogram (Fig. 1). She was admitted to the intensive care unit for medical management of the dissection. Bedside echocardiography showed a tricuspid aortic valve with trivial regurgitation, mild mitral regurgitation and a 4.4 cm dilated aortic root at the sinus of Valsalva. Marfan syndrome was confirmed based on clinical features and positive genetic testing for FBN-1 mutation. Following stabilisation and reassuring repeated imaging, she was discharged home on nifedipine and metoprolol 11 days postpartum with arrangements for vascular surgical follow-up. Aortic dissection is an uncommon event, for which pregnancy and Marfan syndrome are predisposing factors.1 We wish to highlight a few issues related to this case. The patient had not been diagnosed previously with Marfan syndrome and had no family history. Notably, she had no arched palate, cardiac murmurs, arachnodactyly or other stigmata other than her height (1.92 m) and myopia. While most pregnant patients remain well, rare high-risk conditions may present in pregnancy with potentially fatal consequences. In this case, the obstetric team thought the thoracic pain was musculoskeletal and prescribed analgesics and sedatives. The anaesthetic team were consulted to exclude com-
Fig. 1 Computerised tomography angiogram showing Stanford type B aortic dissection extending from 2 cm distal to left subclavian artery down to both common femoral arteries. The aortic arch was dilated at 3.5 cm.
198 plications of EBP but suggested the diagnosis of Marfan syndrome. This highlights the importance of multidisciplinary care, consultation with different specialities and consideration of alternative diagnoses when the patient’s condition fails to improve. Marfan syndrome confers up to a fourfold increased risk of PDPH given accidental or intentional dural puncture.2 Dural ectasia can lead to unpredictable and inadequate intrathecal anaesthesia with normal doses of local anaesthetic,3 theoretically due to increased cerebrospinal fluid volume.4 In our case, a 13 mg dose of bupivacaine was used given her extreme height. The larger dose may have mitigated problems posed by dural ectasia (subsequently seen on magnetic resonance imaging) but it is unclear if the block would have been adequate for caesarean delivery. It is fortunate that she did not have an accidental dural puncture during insertion of her EBP. The patient developed uterine atony and received two 5 U doses of oxytocin followed by an infusion, which is standard unit practice. The hypertensive effects of ergometrine are well recognised. Its use may have contributed to the dissection, although symptoms started approximately 48 h after her last dose. Aortic dissection can occur in 1–4.5% of Marfan syndrome pregnancies, dependent on aortic root diameter; gestational hypertension and preeclampsia are additional risk factors.1,5
Acknowledgments We would like to thank Dr Hamish Ireland for supplying us with the radiological image.
International Journal of Obstetric Anesthesia V. Humphrey, D. Falzon, V. Clark Simpson’s Centre for Reproductive Health Royal Infirmary of Edinburgh, Edinburgh, UK E-mail address: [email protected]
References 1. Curry RA, Gelson E, Swan L, et al. Marfan syndrome and pregnancy: maternal and neonatal outcomes. BJOG 2014;121:610–7. 2. Youngblood SC, Tolpin DA, Cooper JR, Coselli JS, LeMaire SA. Complications of cerebrospinal fluid drainage after thoracic aortic surgery: a review of 504 patients over 5 years. J Thorac Cardiovasc Surg 2013;146:166–71. 3. Lacassie HJ, Millar S, Leithe LG, Habib AS, Muir HA. Dural ectasia: a likely cause of inadequate spinal anaesthesia in two parturients with Marfan’s syndrome. Br J Anaesth 2005;94:500–4. 4. Carpenter RL, Hogan QH, Liu SS, et al. Lumbosacral cerebrospinal fluid volume is the primary determinant of sensory block extent and duration during spinal anaesthesia. Anesthesiology 1998;89:24–9. 5. Chow SL. Acute aortic dissection in a patient with Marfan’s syndrome complicated by gestational hypertension. Med J Aust 1993;159:760–2. 0959-289X/$ - see front matter
c 2015 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijoa.2015.01.009
