CASE REPORT
Successful Pregnancy, Epidural Anaesthesia, Labour, and Delivery in a Woman With Sturge-Weber Syndrome and Previous Hemispherectomy Aly Shah Aziz, MD,1 Dini Hui, MD, FRCSC,2 Vinod Chinnappa, MBBS, MD, FCARCSI, FRCPC,3 Richard I. Aviv, MBChB, MRCP, FRCR, FRCPC,4 Richard H. Swartz, MD, PhD, FRCPC5 1
Department of Neurology, The Hospital for Sick Children, University of Toronto, Toronto ON
2
Department of Obstetrics and Maternal Fetal Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto ON
3
Department of Anaesthesia, Women’s College Hospital, University of Toronto, Toronto ON
4
Department of Medical Imaging, Sunnybrook Health Science Centre, University of Toronto, Toronto ON
5
Department of Medicine (Neurology), Sunnybrook Health Sciences Centre, University of Toronto, Toronto ON
Abstract
Résumé
Background: The outcomes of pregnancy and subsequent delivery of healthy neonates in women who have undergone previous near total hemispherectomy for Sturge-Weber syndrome (SWS) have rarely been reported.
Contexte : Les issues de grossesse et l’accouchement subséquent d’enfants en santé chez des femmes ayant au préalable subi une hémisphérectomie presque totale en raison du syndrome de Sturge-Weber (SSW) n’ont que rarement fait l’objet de signalements.
Case: A woman with SWS had two successful and uncomplicated pregnancies and deliveries under epidural anaesthesia after undergoing a near total hemispherectomy with subsequent unilateral cerebral palsy. Non-contrast MRI of the brain revealed post hemispherectomy changes with no residual meningeal angiomatosis. Conclusion: As more women who undergo surgical resection for underlying SWS grow into the child-bearing years, additional reports of pregnancy and delivery outcomes in this patient population can be expected and will be valuable.
Cas : Une femme présentant le SSW a connu deux grossesses et accouchements réussis et sans complications sous anesthésie péridurale, après avoir subi une hémisphérectomie presque totale ayant subséquemment donné lieu à une infirmité motrice cérébrale unilatérale. Une IRM du cerveau menée sans produits de contraste a révélé la présence de modifications posthémisphérectomie, sans angiomatose méningée résiduelle. Conclusion : Au fur et à mesure que nous verrons un nombre de plus en plus important de femmes, ayant subi une résection chirurgicale en raison d’un SSW sous-jacent, atteindre l’âge de procréation, nous pouvons nous attendre à d’autres signalements d’issues de grossesse et d’accouchement portant sur cette population de patientes; ces signalements prendront alors toute leur valeur.
Key Words: Hemispherectomy, Sturge-Weber syndrome, cerebral palsy, anaesthesia, epidural Competing Interests: None declared. Received on June 14, 2013 Accepted on July 4, 2013
J Obstet Gynaecol Can 2013;35(10):917–919
OCTOBER JOGC OCTOBRE 2013 l 917
Case Report
INTRODUCTION
S
turge-Weber syndrome (SWS) is a rare sporadic neurocutaneous syndrome characterized by facial angiomas, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angioma.1 Seizures are the primary neurological manifestation; medically refractory epilepsy is amenable to surgical removal of the leptomeningeal angiomatosis and underlying damaged cortex as a form of therapy.2 We report here the case of a woman with SWS diagnosed at birth who underwent near total left hemispherectomy for treatment of refractory seizures as an infant, with good results. She went on to have two successful pregnancies, with labour and vaginal deliveries under epidural anaesthesia. THE CASE
The patient, a 31-year-old woman, was born with a portwine stain on the left side of her face in the V1 distribution of the trigeminal nerve, and SWS was subsequently diagnosed. She had intractable generalized tonic-clonic seizures refractory to anti-epileptic medications, and at approximately one year of age underwent a near total leftsided hemispherectomy. Her seizures stopped but she had subsequent cerebral palsy on the right side, manifested as right-sided pyramidal weakness, right homonymous hemianopsia, and spastic gait. From a neurological perspective, the patient remained seizure-free until the age of 18, when she presented with generalized tonicclonic seizures after a mechanical fall. She was treated successfully with prophylactic phenytoin, and she was gradually weaned off phenytoin with no further seizures. There was no seizure recurrence thereafter, and the patient lived independently, worked full-time, and did not use an aid for mobility. At the age of 27, the patient had a first trimester missed abortion requiring suction dilatation and curettage. At age 29, she delivered a healthy baby girl at term under epidural anaesthesia after a normal pregnancy. There were no complications during labour, and delivery was vacuum assisted because of patient fatigue. There were no postnatal complications. Although the patient had completed a pregnancy successfully with epidural anaesthesia, she was referred to our neurology service during the third trimester of her third pregnancy to assess the risk of vascular malformations in the brain or spine that might complicate epidural anaesthesia or lead to an increased risk of intracranial bleeding during and after labour. A secondary question of the safety of using epidural anaesthesia was also raised. 918 l OCTOBER JOGC OCTOBRE 2013
The patient reached 38 weeks’ gestation having had normal ultrasound assessments and stable blood pressure throughout her pregnancy. She had no complaints on review of systems. On examination, her vital signs were stable, with a blood pressure of 125/85 mmHg. She had a port-wine stain on the left side of the forehead extending over the entire distribution of the V1 segment of the trigeminal nerve. She had no dermatological lesions overlying the vertebrae. Screening mental status examination, including language, was normal. Cranial nerve testing revealed a right homonymous hemianopsia and mild weakness of the right sternocleidomastoid. Motor system examination showed decreased muscle bulk in her right upper extremity, with contractures of the right hand, wrist, and ankle. She had right-sided upper extremity spasticity, and hyperreflexia and an upgoing plantar response on the right side. Power examination showed near-normal strength in the right biceps and triceps, and right knee flexor and right knee extensor, but no strength in the right wrist or finger extensors. The patient had had surgery on the tendon of her right ankle for contractures. Sensory examination revealed hyperesthesia of the right upper extremity and mild impairment of proprioception. Gait was spastic, with circumduction of the right leg requiring assistance for heel-toe walking. Systems examination was otherwise normal. Non-contrast brain MRI showed evidence of an extensive left hemispherectomy with fluid within the residual cavity (Figure). No contrast was used at the request of the patient. Wallerian degeneration of the left cerebral peduncle and crossed cerebellar diaschesis was noted. There were no susceptibility foci to suggest residual meningeal angiomatosis. MRI of the spine showed a syrinx from T4 to T9. No abnormal vascular structures were seen. The patient had spontaneous onset of labour at 39 weeks’ gestation. Epidural anaesthesia was used for analgesia with no complications during insertion of the epidural catheter. Labour was uncomplicated, and the patient had a normal delivery of a healthy baby girl weighing 4.3 kg. There were no postnatal complications for mother or baby. DISCUSSION
In the English literature, reports of patients with SWS associated with pregnancy are scant. Zanconato et al. reported the case of a 42-year-old primigravid woman with SWS who had an uneventful pregnancy and delivery by Caesarean section.3 Dolkart and Bhat reported the case of a 24-year-old woman with SWS and underlying complex partial epilepsy who had seizure recurrence during pregnancy; three days after delivery, she had a small left lateral intraventricular hemorrhage.4
Successful Pregnancy in a Woman With Sturge-Weber Syndrome and Previous Hemispherectomy
The case we describe is the first documented case of a patient who underwent near total left hemispherectomy as an infant for refractory seizures because of underlying SWS and who subsequently had uncomplicated pregnancies. Leptomeningeal angioma is an intra-cranial vasculopathic feature in SWS. Given that affected vessels are thin walled and may be abnormally innervated, with impaired vascular responsiveness to hypertension, Batra et al. recommend a cautious approach in managing patients with SWS during anaesthetic procedures so as not to increase intracranial pressure, which may lead to intracranial hemorrhage.5 This is largely a theoretical argument as the incidence of intracranial hemorrhage due to hypertension in patients with SWS is not known. This does not exclude its possibility given the abnormal vasculature, and we therefore recommend vigilance in controlling hypertension in patients with SWS. In our patient, a definitive therapeutic procedure had been undertaken at one year of age, with clinical stability for more than 30 years subsequently. Her prior pregnancy had been uneventful, with an uncomplicated vaginal delivery under epidural anaesthesia. The MRI showed no evidence of residual angioma; however, the imaging was done without contrast at the patient’s request, limiting the study for sensitive detection of intracranial angioma.6 While gadolinium is not approved for use in pregnancy, some guidelines suggest that it is likely to be safe in the third trimester and that its use should be considered only after balancing risks against benefits.7 The use of epidural anaesthesia in patients with SWS is not well-documented in the English literature. Tadrous et al. described a 28-year-old primigravida with sudden onset of headache, acute right-sided weakness, aphasia, and blurred vision at 41 weeks’ gestation. 8 Neuro-imaging showed left-sided cerebral edema and a right parietooccipital lesion, most likely an angioma. Caesarean section was planned; general anaesthesia was avoided and a lumbar epidural block was administered but failed. A subarachnoid block was successfully performed and a healthy infant delivered.8 An MRI of the cervical, thoracic, and lumbar spines of our patient showed no vascular anomalies. To our knowledge, spinal leptomeningeal angiomas have not been reported in patients with SWS. The leptomeningeal abnormalities are classically localized to the trigeminal nerve territory, which includes the intracranial, but not spinal, leptomeningeal tissue. However, because spinal hemangiomas have been reported in overlap syndromes (for example, SWS associated with Klippel-Trenaunay syndrome9), some authors have recommended caution with epidural procedures in patients with SWS.8
Non-contrast axial T2 weighted brain MRI demonstrating fluid within the residual cavity (arrows).
This is the first documentation of a patient with SWS who has undergone a near total hemispherectomy with subsequent cerebral palsy going on to have two successful and uncomplicated pregnancies. It is likely that having excellent clinical stability and high functional status after definitive treatment for SWS as a child significantly lowered the pregnancy-associated risks for our patient. CONCLUSION
As more children treated with hemispherectomy grow into the child-bearing years, additional reports of pregnancy outcomes in women with SWS, both with and without childhood surgery, can be expected and will be valuable. ACKNOWLEDGEMENTS
The woman whose story is told in this case report has provided written consent for its publication. REFERENCES 1. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004;23(2):87–98. 2. Arzimanoglou A, Andermann F, Aicardi J, Sainte–Rose C, Beaulieu MA, Villemure JG, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology 2000;55(10):1472–9. 3. Zanconato G, Papadopoulos N, Lampugnani F, Caloi E, Franchi M. An uncomplicated pregnancy associated with Sturge-Weber angiomatosis. Eur J Obstet Gynecol Reprod Biol 2008;137(1):125–6. 4. Dolkart LA, Bhat M. Sturge-Weber syndrome in pregnancy. Am J Obstet Gynecol 1995;173(3 Pt 1):969–71. 5. Batra RK, Gulaya V, Madan R, Trikha A. Anaesthesia and the Sturge-Weber syndrome. Can J Anaesth 1994;41(2):133–6. 6. Nirmal N, Thaller SR. Sturge-Weber syndrome and associated congenital vascular disorder: review. J Craniofac Surg 2006;17(4):724–8. 7. Garcia-Bournissen F, Shrim A, Koren G. Safety of gadolinium during pregnancy. Can Fam Physician 2006;52:309–10. 8. Tadrous R, Ni Mhuirchteagh R, McCaul C. Anaesthesia for caesarean section in a patient with Sturge–Weber syndrome following acute neurological deterioration. Int J Obstet Anesth 2011;20(3):259–62. 9. de Leon-Casasola OA, Lema MJ. Anesthesia for patients with Sturge-Weber disease and Klippel-Trenaunay syndrome. J Clin Anesth 1991;3(5):409–13.
OCTOBER JOGC OCTOBRE 2013 l 919
Successful Pregnancy, Epidural Anaesthesia, Labour, and Delivery in a Woman With Sturge-Weber Syndrome and Previous Hemispherectomy Aly Shah Aziz, MD,1 Dini Hui, MD, FRCSC,2 Vinod Chinnappa, MBBS, MD, FCARCSI, FRCPC,3 Richard I. Aviv, MBChB, MRCP, FRCR, FRCPC,4 Richard H. Swartz, MD, PhD, FRCPC5 1
Department of Neurology, The Hospital for Sick Children, University of Toronto, Toronto ON
2
Department of Obstetrics and Maternal Fetal Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto ON
3
Department of Anaesthesia, Women’s College Hospital, University of Toronto, Toronto ON
4
Department of Medical Imaging, Sunnybrook Health Science Centre, University of Toronto, Toronto ON
5
Department of Medicine (Neurology), Sunnybrook Health Sciences Centre, University of Toronto, Toronto ON
Abstract
Résumé
Background: The outcomes of pregnancy and subsequent delivery of healthy neonates in women who have undergone previous near total hemispherectomy for Sturge-Weber syndrome (SWS) have rarely been reported.
Contexte : Les issues de grossesse et l’accouchement subséquent d’enfants en santé chez des femmes ayant au préalable subi une hémisphérectomie presque totale en raison du syndrome de Sturge-Weber (SSW) n’ont que rarement fait l’objet de signalements.
Case: A woman with SWS had two successful and uncomplicated pregnancies and deliveries under epidural anaesthesia after undergoing a near total hemispherectomy with subsequent unilateral cerebral palsy. Non-contrast MRI of the brain revealed post hemispherectomy changes with no residual meningeal angiomatosis. Conclusion: As more women who undergo surgical resection for underlying SWS grow into the child-bearing years, additional reports of pregnancy and delivery outcomes in this patient population can be expected and will be valuable.
Cas : Une femme présentant le SSW a connu deux grossesses et accouchements réussis et sans complications sous anesthésie péridurale, après avoir subi une hémisphérectomie presque totale ayant subséquemment donné lieu à une infirmité motrice cérébrale unilatérale. Une IRM du cerveau menée sans produits de contraste a révélé la présence de modifications posthémisphérectomie, sans angiomatose méningée résiduelle. Conclusion : Au fur et à mesure que nous verrons un nombre de plus en plus important de femmes, ayant subi une résection chirurgicale en raison d’un SSW sous-jacent, atteindre l’âge de procréation, nous pouvons nous attendre à d’autres signalements d’issues de grossesse et d’accouchement portant sur cette population de patientes; ces signalements prendront alors toute leur valeur.
Key Words: Hemispherectomy, Sturge-Weber syndrome, cerebral palsy, anaesthesia, epidural Competing Interests: None declared. Received on June 14, 2013 Accepted on July 4, 2013
J Obstet Gynaecol Can 2013;35(10):917–919
OCTOBER JOGC OCTOBRE 2013 l 917
Case Report
INTRODUCTION
S
turge-Weber syndrome (SWS) is a rare sporadic neurocutaneous syndrome characterized by facial angiomas, ocular abnormalities (glaucoma and choroidal hemangioma), and leptomeningeal angioma.1 Seizures are the primary neurological manifestation; medically refractory epilepsy is amenable to surgical removal of the leptomeningeal angiomatosis and underlying damaged cortex as a form of therapy.2 We report here the case of a woman with SWS diagnosed at birth who underwent near total left hemispherectomy for treatment of refractory seizures as an infant, with good results. She went on to have two successful pregnancies, with labour and vaginal deliveries under epidural anaesthesia. THE CASE
The patient, a 31-year-old woman, was born with a portwine stain on the left side of her face in the V1 distribution of the trigeminal nerve, and SWS was subsequently diagnosed. She had intractable generalized tonic-clonic seizures refractory to anti-epileptic medications, and at approximately one year of age underwent a near total leftsided hemispherectomy. Her seizures stopped but she had subsequent cerebral palsy on the right side, manifested as right-sided pyramidal weakness, right homonymous hemianopsia, and spastic gait. From a neurological perspective, the patient remained seizure-free until the age of 18, when she presented with generalized tonicclonic seizures after a mechanical fall. She was treated successfully with prophylactic phenytoin, and she was gradually weaned off phenytoin with no further seizures. There was no seizure recurrence thereafter, and the patient lived independently, worked full-time, and did not use an aid for mobility. At the age of 27, the patient had a first trimester missed abortion requiring suction dilatation and curettage. At age 29, she delivered a healthy baby girl at term under epidural anaesthesia after a normal pregnancy. There were no complications during labour, and delivery was vacuum assisted because of patient fatigue. There were no postnatal complications. Although the patient had completed a pregnancy successfully with epidural anaesthesia, she was referred to our neurology service during the third trimester of her third pregnancy to assess the risk of vascular malformations in the brain or spine that might complicate epidural anaesthesia or lead to an increased risk of intracranial bleeding during and after labour. A secondary question of the safety of using epidural anaesthesia was also raised. 918 l OCTOBER JOGC OCTOBRE 2013
The patient reached 38 weeks’ gestation having had normal ultrasound assessments and stable blood pressure throughout her pregnancy. She had no complaints on review of systems. On examination, her vital signs were stable, with a blood pressure of 125/85 mmHg. She had a port-wine stain on the left side of the forehead extending over the entire distribution of the V1 segment of the trigeminal nerve. She had no dermatological lesions overlying the vertebrae. Screening mental status examination, including language, was normal. Cranial nerve testing revealed a right homonymous hemianopsia and mild weakness of the right sternocleidomastoid. Motor system examination showed decreased muscle bulk in her right upper extremity, with contractures of the right hand, wrist, and ankle. She had right-sided upper extremity spasticity, and hyperreflexia and an upgoing plantar response on the right side. Power examination showed near-normal strength in the right biceps and triceps, and right knee flexor and right knee extensor, but no strength in the right wrist or finger extensors. The patient had had surgery on the tendon of her right ankle for contractures. Sensory examination revealed hyperesthesia of the right upper extremity and mild impairment of proprioception. Gait was spastic, with circumduction of the right leg requiring assistance for heel-toe walking. Systems examination was otherwise normal. Non-contrast brain MRI showed evidence of an extensive left hemispherectomy with fluid within the residual cavity (Figure). No contrast was used at the request of the patient. Wallerian degeneration of the left cerebral peduncle and crossed cerebellar diaschesis was noted. There were no susceptibility foci to suggest residual meningeal angiomatosis. MRI of the spine showed a syrinx from T4 to T9. No abnormal vascular structures were seen. The patient had spontaneous onset of labour at 39 weeks’ gestation. Epidural anaesthesia was used for analgesia with no complications during insertion of the epidural catheter. Labour was uncomplicated, and the patient had a normal delivery of a healthy baby girl weighing 4.3 kg. There were no postnatal complications for mother or baby. DISCUSSION
In the English literature, reports of patients with SWS associated with pregnancy are scant. Zanconato et al. reported the case of a 42-year-old primigravid woman with SWS who had an uneventful pregnancy and delivery by Caesarean section.3 Dolkart and Bhat reported the case of a 24-year-old woman with SWS and underlying complex partial epilepsy who had seizure recurrence during pregnancy; three days after delivery, she had a small left lateral intraventricular hemorrhage.4
Successful Pregnancy in a Woman With Sturge-Weber Syndrome and Previous Hemispherectomy
The case we describe is the first documented case of a patient who underwent near total left hemispherectomy as an infant for refractory seizures because of underlying SWS and who subsequently had uncomplicated pregnancies. Leptomeningeal angioma is an intra-cranial vasculopathic feature in SWS. Given that affected vessels are thin walled and may be abnormally innervated, with impaired vascular responsiveness to hypertension, Batra et al. recommend a cautious approach in managing patients with SWS during anaesthetic procedures so as not to increase intracranial pressure, which may lead to intracranial hemorrhage.5 This is largely a theoretical argument as the incidence of intracranial hemorrhage due to hypertension in patients with SWS is not known. This does not exclude its possibility given the abnormal vasculature, and we therefore recommend vigilance in controlling hypertension in patients with SWS. In our patient, a definitive therapeutic procedure had been undertaken at one year of age, with clinical stability for more than 30 years subsequently. Her prior pregnancy had been uneventful, with an uncomplicated vaginal delivery under epidural anaesthesia. The MRI showed no evidence of residual angioma; however, the imaging was done without contrast at the patient’s request, limiting the study for sensitive detection of intracranial angioma.6 While gadolinium is not approved for use in pregnancy, some guidelines suggest that it is likely to be safe in the third trimester and that its use should be considered only after balancing risks against benefits.7 The use of epidural anaesthesia in patients with SWS is not well-documented in the English literature. Tadrous et al. described a 28-year-old primigravida with sudden onset of headache, acute right-sided weakness, aphasia, and blurred vision at 41 weeks’ gestation. 8 Neuro-imaging showed left-sided cerebral edema and a right parietooccipital lesion, most likely an angioma. Caesarean section was planned; general anaesthesia was avoided and a lumbar epidural block was administered but failed. A subarachnoid block was successfully performed and a healthy infant delivered.8 An MRI of the cervical, thoracic, and lumbar spines of our patient showed no vascular anomalies. To our knowledge, spinal leptomeningeal angiomas have not been reported in patients with SWS. The leptomeningeal abnormalities are classically localized to the trigeminal nerve territory, which includes the intracranial, but not spinal, leptomeningeal tissue. However, because spinal hemangiomas have been reported in overlap syndromes (for example, SWS associated with Klippel-Trenaunay syndrome9), some authors have recommended caution with epidural procedures in patients with SWS.8
Non-contrast axial T2 weighted brain MRI demonstrating fluid within the residual cavity (arrows).
This is the first documentation of a patient with SWS who has undergone a near total hemispherectomy with subsequent cerebral palsy going on to have two successful and uncomplicated pregnancies. It is likely that having excellent clinical stability and high functional status after definitive treatment for SWS as a child significantly lowered the pregnancy-associated risks for our patient. CONCLUSION
As more children treated with hemispherectomy grow into the child-bearing years, additional reports of pregnancy outcomes in women with SWS, both with and without childhood surgery, can be expected and will be valuable. ACKNOWLEDGEMENTS
The woman whose story is told in this case report has provided written consent for its publication. REFERENCES 1. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004;23(2):87–98. 2. Arzimanoglou A, Andermann F, Aicardi J, Sainte–Rose C, Beaulieu MA, Villemure JG, et al. Sturge-Weber syndrome: indications and results of surgery in 20 patients. Neurology 2000;55(10):1472–9. 3. Zanconato G, Papadopoulos N, Lampugnani F, Caloi E, Franchi M. An uncomplicated pregnancy associated with Sturge-Weber angiomatosis. Eur J Obstet Gynecol Reprod Biol 2008;137(1):125–6. 4. Dolkart LA, Bhat M. Sturge-Weber syndrome in pregnancy. Am J Obstet Gynecol 1995;173(3 Pt 1):969–71. 5. Batra RK, Gulaya V, Madan R, Trikha A. Anaesthesia and the Sturge-Weber syndrome. Can J Anaesth 1994;41(2):133–6. 6. Nirmal N, Thaller SR. Sturge-Weber syndrome and associated congenital vascular disorder: review. J Craniofac Surg 2006;17(4):724–8. 7. Garcia-Bournissen F, Shrim A, Koren G. Safety of gadolinium during pregnancy. Can Fam Physician 2006;52:309–10. 8. Tadrous R, Ni Mhuirchteagh R, McCaul C. Anaesthesia for caesarean section in a patient with Sturge–Weber syndrome following acute neurological deterioration. Int J Obstet Anesth 2011;20(3):259–62. 9. de Leon-Casasola OA, Lema MJ. Anesthesia for patients with Sturge-Weber disease and Klippel-Trenaunay syndrome. J Clin Anesth 1991;3(5):409–13.
OCTOBER JOGC OCTOBRE 2013 l 919
