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377: Anesthetic management of cesarean delivery in a patient with Ellis-van Creveld syndrome and severe left ventricular outflow obstruction
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Posters
•
Obstetrics
331. Combined spinal epidural for caesarean delivery in a patient with intracranial arteriovenous malformation— best of both worlds
377. Anesthetic management of cesarean delivery in a patient with Ellis-van Creveld syndrome and severe left ventricular outflow obstruction
F.J. Emerantia Jacintha, M. Nicholas Anaesthetics Department, Birmingham Women’s Hospital, Birmingham, UK
R.D. Toledano, P.J. Balestrieri, M. Tiouririne, M.J. Eisenmenger Department of Anesthesiology, University of Virginia Health System, Charlottesville, VA, USA
Introduction: Intracranial haemorrhage from an arteriovenous malformation (AVM) during pregnancy is rare but results in significant maternal and fetal morbidity and mortality. In the untreated patient the best mode of delivery remains debatable with most obstetricians preferring a caesarean section to avoid Valsalva manoeuvres associated with vaginal delivery. We describe the use and implications of combined spinal epidural (CSE) for a parturient undergoing Caesarean section. Case Report: A 24 year old woman in her second pregnancy was diagnosed with a left frontal AVM at 20 weeks when she developed neurological symptoms. CT scan and cerebral angiogram revealed intracerebral haemorrhage in the left frontal lobe caused by an AVM in the left posterior frontal cortex. Operative management was deferred till after delivery. Elective caesarean section was planned at 36 weeks under CSE. After initiating arterial monitoring, CSE was instituted at the L3/4 interspace using intrathecal bupivacaine 0.5% (heavy)10mgs and fentanyl 20 micrograms and epidural extension with incremental boluses of plain bupivacaine 0.5%. She had an uneventful operation, was monitored overnight on HDU and discharged on the 3rd day with no sequelae. Discussion: Intracranial haemorrhage due to ruptured arteriovenous malformation (AVM) during pregnancy is rare but warrants prompt recognition. Management is primarily based on neurosurgical rather than obstetric considerations. No guidelines exist regarding timing of obstetric and neurosurgical intervention. Previous case reports reveal successful outcomes with spinal, epidural and general anaesthesia. Optimal anaesthetic management of these women involves providing haemodynamic stability. We discuss the rationale for using CSE as our preferred technique in this patient
References 1. Hatsukari I; Nagasaka H; Tsuchiya M; Taguchi M. Anaesthetic management for elective or emergent cesarean section in patients with intracranial arteriovenous malformation. Masui. 2000 Jan;49(1):33-6.
Background: Ellis-van Creveld syndrome (EVC) is an autosomal recessive chondroectodermal dysplasia marked by dwarfism, cardiothoracic malformations, and polydactyly. Anesthetic considerations in parturients with EVC include difficult intubation in patients already at increased risk for aspiration, restrictive lung disease, anatomic challenges to neuraxial techniques, and adult congenital heart disease. Results: A 19 year old with EVC associated with a uni-atrial heart and cleft mitral valve, status post repair, presented at 10 weeks gestation with shortness of breath and fatigue. Work-up revealed subaortic stenosis with a fixed left ventricular outflow tract (LVOT) gradient of 90mmHg and severe mitral regurgitation. The patient elected to proceed with the pregnancy, despite the high risk to both her and the fetus. A multidisciplinary team planned Cesarean delivery under general anesthesia at 32 weeks gestation, barring deterioration in the patient’s status. The patient’s LVOT gradient increased to 130mmHg over the course of her pregnancy, without changes in her symptomatology, and we proceeded with Cesarean section as planned. After placement of an arterial catheter, we induced general anesthesia with etomidate 14 mg and succinylcholine 140 mg. An internal jugular venous catheter and a transesophageal echo probe were placed after the trachea was intubated, and anesthesia was maintained with 0.5% sevoflurane and a remifentanil drip. The cardiopulmonary bypass team remained on stand-by throughout the procedure. Conclusions: Our patient presented with a high LVOT gradient and severe mitral regurgitation, in addition to the stigmata associated with EVC. Her care required a multidisciplinary approach and careful consideration of seemingly limited anesthetic options. She was discharged on postoperative day 5 after an uneventful Cesarean delivery and post-partum recovery.
Posters
•
Obstetrics
331. Combined spinal epidural for caesarean delivery in a patient with intracranial arteriovenous malformation— best of both worlds
377. Anesthetic management of cesarean delivery in a patient with Ellis-van Creveld syndrome and severe left ventricular outflow obstruction
F.J. Emerantia Jacintha, M. Nicholas Anaesthetics Department, Birmingham Women’s Hospital, Birmingham, UK
R.D. Toledano, P.J. Balestrieri, M. Tiouririne, M.J. Eisenmenger Department of Anesthesiology, University of Virginia Health System, Charlottesville, VA, USA
Introduction: Intracranial haemorrhage from an arteriovenous malformation (AVM) during pregnancy is rare but results in significant maternal and fetal morbidity and mortality. In the untreated patient the best mode of delivery remains debatable with most obstetricians preferring a caesarean section to avoid Valsalva manoeuvres associated with vaginal delivery. We describe the use and implications of combined spinal epidural (CSE) for a parturient undergoing Caesarean section. Case Report: A 24 year old woman in her second pregnancy was diagnosed with a left frontal AVM at 20 weeks when she developed neurological symptoms. CT scan and cerebral angiogram revealed intracerebral haemorrhage in the left frontal lobe caused by an AVM in the left posterior frontal cortex. Operative management was deferred till after delivery. Elective caesarean section was planned at 36 weeks under CSE. After initiating arterial monitoring, CSE was instituted at the L3/4 interspace using intrathecal bupivacaine 0.5% (heavy)10mgs and fentanyl 20 micrograms and epidural extension with incremental boluses of plain bupivacaine 0.5%. She had an uneventful operation, was monitored overnight on HDU and discharged on the 3rd day with no sequelae. Discussion: Intracranial haemorrhage due to ruptured arteriovenous malformation (AVM) during pregnancy is rare but warrants prompt recognition. Management is primarily based on neurosurgical rather than obstetric considerations. No guidelines exist regarding timing of obstetric and neurosurgical intervention. Previous case reports reveal successful outcomes with spinal, epidural and general anaesthesia. Optimal anaesthetic management of these women involves providing haemodynamic stability. We discuss the rationale for using CSE as our preferred technique in this patient
References 1. Hatsukari I; Nagasaka H; Tsuchiya M; Taguchi M. Anaesthetic management for elective or emergent cesarean section in patients with intracranial arteriovenous malformation. Masui. 2000 Jan;49(1):33-6.
Background: Ellis-van Creveld syndrome (EVC) is an autosomal recessive chondroectodermal dysplasia marked by dwarfism, cardiothoracic malformations, and polydactyly. Anesthetic considerations in parturients with EVC include difficult intubation in patients already at increased risk for aspiration, restrictive lung disease, anatomic challenges to neuraxial techniques, and adult congenital heart disease. Results: A 19 year old with EVC associated with a uni-atrial heart and cleft mitral valve, status post repair, presented at 10 weeks gestation with shortness of breath and fatigue. Work-up revealed subaortic stenosis with a fixed left ventricular outflow tract (LVOT) gradient of 90mmHg and severe mitral regurgitation. The patient elected to proceed with the pregnancy, despite the high risk to both her and the fetus. A multidisciplinary team planned Cesarean delivery under general anesthesia at 32 weeks gestation, barring deterioration in the patient’s status. The patient’s LVOT gradient increased to 130mmHg over the course of her pregnancy, without changes in her symptomatology, and we proceeded with Cesarean section as planned. After placement of an arterial catheter, we induced general anesthesia with etomidate 14 mg and succinylcholine 140 mg. An internal jugular venous catheter and a transesophageal echo probe were placed after the trachea was intubated, and anesthesia was maintained with 0.5% sevoflurane and a remifentanil drip. The cardiopulmonary bypass team remained on stand-by throughout the procedure. Conclusions: Our patient presented with a high LVOT gradient and severe mitral regurgitation, in addition to the stigmata associated with EVC. Her care required a multidisciplinary approach and careful consideration of seemingly limited anesthetic options. She was discharged on postoperative day 5 after an uneventful Cesarean delivery and post-partum recovery.