SPINE Volume 37, Number 19, pp E1218–E1220 ©2012, Lippincott Williams & Wilkins
CASE REPORT
Paraplegia in Pregnancy A Case of Spinal Vascular Malformation With Klippel–Trenaunay Syndrome Caner Feyzi Demir, MD,* Mustafa Yildiz, MD,† Hasan Özdemir, MD,* Oktay Kapan, MD,* Zülkif Bozgeyik, MD,‡ Said Berilgen, MD,* Banu Aygün, MD,§ and Fatih Serhat Erol, MD¶
Study Design. A case report. Objective. To present improvement of paraplegia due to spinal vascular malformation after delivery with no intervention. Summary of Background Data. Pregnancy has been reported rarely in patients with Klippel–Trenaunay syndrome (KTS). A combined case of spinal arteriovenous malformations (AVMs) within KTS has not been reported before. Methods. A case report of KTS with paraplegia is presented and the pertinent literature is then reviewed. Results. A pregnant woman who was diagnosed with KTS in childhood presented with newly developed paraplegia due to spinal AVMs. Magnetic resonance image showed a vascular malformation at the T9–T12 levels without evidence of spinal cord hemorrhage. Diagnostic angiography that was performed after delivery revealed a high-flow arteriovenous fistula with AVM. Conclusion. Pregnancy complicated by paraplegia is thought to be secondary to venous engorgement and to the resulting spinal cord ischemia in this case. Her neurological symptoms and signs gradually improved over the few days after cesarean delivery. Key words: Klippel–Trenaunay syndrome, spinal vascular malformation, pregnancy. Spine 2012;37:E1218–E1220
T
he vascular malformations of the spine and the spinal cord are rare diseases accounting for only 2% to 4% of all spinal diseases. Damage to the spinal cord occurs by bleeding, space-occupying effects, and venous congestion and rarely by steal effects.1 Klippel–Trenaunay syndrome is a congenital vascular disorder characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy. From the Departments of *Neurology; †Emergency; ‡Radiology; §Obstetrics and Gynaecology; and ¶Neurosurgery, Firat (Euphrates) University, Elazig, Turkey. Acknowledgment date: January 12, 2012. First revision date: February 29, 2012. Second revision date: April 3, 2012. Acceptance date: April 20, 2012. The manuscript submitted does not contain information about medical device(s)/drug(s). No funds were received in support of this work. No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript. Address correspondence and reprint requests to Caner Feyzi Demir, MD, Department of Neurology, Firat (Euphrates) University Hospital, 23119 Elazig, Turkey; E-mail:
[email protected] DOI: 10.1097/BRS.0b013e31825c66ab
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Klippel-Trenaunay syndrome was first described in 1900 by 2 French physicians, Maurice Klippel and Paul Trenaunay. The etiology of the syndrome is unknown. Several theories have been proposed including abnormalities of the sympathetic nerve system resulting in dilatation of the arteriovenous anastomosis or obstruction of the deep veins and persistence of fetal microscopic small arteriovenous anastomosis.2,3 Pregnancy has been reported rarely in patients with Klippel–Trenaunay syndrome (KTS), although the incidence is unknown.4,5 We report a case of pregnancy complicated by paraplegia due to spinal arteriovenous malformation (AVM) within KTS. Literature search revealed no case reports of selfimprovement of paraplegia after delivery.
CASE REPORT A 28-year-old, 30-week-pregnant woman presented with 8 days of backache and acute paraplegia. The patient was diagnosed with KTS since childhood. She had no family history of KTS or any other familial genetic diseases. On examination, she was found to have skin-colored nodules and varicosities on the right upper limb, extending onto the front of the chest, mostly confined to the right side. There was atrophy affecting the right upper limb. Her right leg was swollen and hypertrophic. There was a difference in diameter of 3 cm between both the legs; also, there was difference in diameter of 1 cm between both forearms. Neurological examination revealed paraplegia of lower extremities, with marked loss of pinprick, vibration, and proprioception. There was sensory loss below the T10 level. The patient was flaccid but sphincter control was preserved. There was no motor or sensory deficit in the upper extremities. The nerve-conduction study was normal. The results of the laboratory evaluation were within normal limits, including complete blood cell count, blood chemistry tests, sedimentation rate, antinuclear antibodies, anti-DNA, Lyme titers, Schilling test, serum protein electrophoresis, Venereal Disease Research Laboratory test, and human T-cell lymphoma virus–1 test. Spinal magnetic resonance image suggested a vascular malformation without evidence of spinal cord hemorrhage. T1-weighted (Figure 1A) and T2-weighted sagittal and axial images of the spinal cord (Figure 1B, C) show hypointense lesions accompanied by flow voids that are intradural dilated vascular channels. T2-weighted sagittal image also shows abnormal hyperintense signals, which keeping with congestive changes September 2012
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CASE REPORT
Paraplegia in Pregnancy • Demir et al
Figure 1. T1-weighted (A) and T2-weighted (B) sagittal thoracic magnetic resonance (MR) images show a hypointense lesion accompanied by flow voids which are intradural dilated vascular channels. T2-weighted sagittal image (B) also shows abnormal hyperintense signals, which keeping with congestive changes and edematous swelling extend from T10 to L2 level of conus medullaris. T2-weighted axial image (C) shows engorged medullary veins corresponding to the other images. T1- and T2-weighted MR images also show atrophy of the spinal cord at the affected levels.
and edematous swelling extend from T10 to L2 level of conus medullaris (Figure 1B). During pregnancy, low-molecular-weight heparin nadroparin was started for ischemic injury of the spinal cord and prophylaxis against deep vein thrombosis (enoxaparin twicedaily subcutaneous dose of 0.4 mL) (Clexane 4000 Anti-XA IU/0, 4 ml, Sanofi-Aventis, Turkey). Doppler examination demonstrated no thrombosis. During the following 24 hours, she developed urinary and bowel incontinence. She was referred for an obstetrical opinion and cesarean delivery. Her neurological symptoms
continued to worsen. A cesarean delivery was performed 2 days later. General anesthesia was preferred because of the risk of spinal hemorrhage. She delivered a healthy female neonate weighing 3400 g, with an Apgar score of 8. In the postpartum period, prophylactic anticoagulation was continued. During the following 7 days, she had mild recovery of some power in her legs and her left leg could effort against gravity. Diagnostic angiogram revealed high-flow arteriovenous fistula and AVM, fed mainly by branches of thoracic aorta. The fistula drained into the epidural and paravertebral venous plexus (Figure 2). Her symptoms improved and she was able to walk with assistance. Endovascular embolization was not planned because of her neurological symptoms that gradually improved delivery, and conservative observation was proposed. Magnetic resonance imaging 3 weeks after delivery did not show any changes in the spinal dimensions (Figure 3).
DISCUSSION
Figure 2. Digital subtraction angiogram: later phases of T-11 artery injection revealing large tortuous veins draining to venous plexuses. Spine
The clinical signs and symptoms of congestive myelopathy can mimic a peripheral nerve disorder initially.6 First, we thought that our case was Guillain–Barré syndrome, so nerve conduction study was performed immediately. It was negative for all 4 extremities. We detected vascular malformations on magnetic resonance images of the spine. Because of her pregnancy, angiography which is more sensitive for vascular malformations was not performed before delivery. During the next few days while being considered for embolization of spinal AVM, signs showed improvement. The circulation to the spinal cord has unique features related to the rich anastomotic anatomy of the cord that results in relative rarity of spinal cord infarction in comparison with cerebral infarction. Spinal cord vascular malformations (arterial and venous) represent a heterogenous group of blood vessel disorders that affect the spinal cord parenchyma either directly or indirectly. Spinal AVMs may lead to spinal cord dysfunction by various pathophysiological mechanisms, www.spinejournal.com
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CASE REPORT
Paraplegia in Pregnancy • Demir et al
syndrome, resulting in an increased risk of thromboembolism and hemorrhage. Neurological opinion is secondary to this venous engorgement and to the resulting spinal cord ischemia in this case. It is well known that the surgical treatment of spinal AVMs carries a risk of neurological deficit of about 20% and that endovascular intervention has a complication rate of about 14%.10 So, conservative observation may be considered as an alternative to endovascular or surgical intervention, as represented in this case report. A multidisciplinary approach with the gynecologist, anesthesiologist, and neurosurgeon or neuroradiologist forms the mainstay of the management of these patients.
➢ Key Points The vascular malformations of the spine and the spinal cord are rare diseases accounting for only 2% to 4% of all spinal diseases. KTS is a rare congenital vascular disorder characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy. Pregnancy has been reported rarely in patients with KTS, although the incidence is unknown. Pregnancy complicated by paraplegia due to spinal AVM within KTS is a very rare clinical condition. Figure 3. Postdelivery T2-weighted sagittal thoracic magnetic resonance image shows intradural dilated vascular channels similar to magnetic resonance imaging findings in pregnancy.
including venous hypertension with secondary congestive myelopathy, venous congestion, hemorrhage, mass effect, and vascular steal leading to ischemia. Spinal AVMs may present with fluctuating symptoms or progressive myelopathy, but it is unclear whether ischemia or venous hypertension is the pathophysiological mechanism.7,8 Another possible cause for ischemic injury is abnormal, enlarged veins which are associated with dural AV shunts, or fistulas may cause reflux of arterial blood into the venous drainage of the cord. This results in increased venous pressure and often causes ischemic cord injury as seen in Foix-Alajouanine syndrome. 6,9 Venous stasis, not thrombosis, may be the other cause of infarction in this case. The varicosities may increase in size during pregnancy. KTS in a pregnant women is extremely rare. Pregnancy is discouraged because this rare disease puts a pregnant woman in an increased obstetric risk because pregnancy can exacerbate complications.5 The normal physiologic changes of pregnancy, such as increased venous pressure, leg edema, venous stasis, and cardiac output, exacerbate the problems of this
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References
1. Strom RG, Derdeyn CP, Moran CJ, et al. Frequency of spinal arteriovenous malformations in patients with unexplained myelopathy. Neurology 2006;66:928–31. 2. Klippel M, Trenaunay P. Naevus variquenx osteohypertophique. Arch G´en´erales deM´edecine 1900;3:641–72. 3. Jacob AG, Driscoll DJ, Shaughnessy WJ, et al. Klippel-Trénaunay syndrome: spectrum and management. Mayo Clin Proc 1998;73:28–36. 4. Torres-Farías E, Torres-Gómez LG, Burciaga-Sepúlveda AS. Klippel-Trenaunay syndrome and pregnancy. Case report. Ginecol Obstet Mex 2010;78:287–90. 5. Güngor GT, Jacquemyn Y. Klippel-Trenaunay syndrome and pregnancy [published online ahead of print December 19, 2010]. Obstet Gynecol Int 2010;706850. 6. Jellema K, Tijssen CC, van Gijn J. Spinal dural arteriovenous fistulas: a congestive myelopathy that initially mimics a peripheral nerve disorder. Brain 2006;129:3150–64. 7. Aminoff MJ, Barnard RO, Logue V. The pathophysiology of spinal vascular malformations. J Neurol Sci 1974;23:255–63. 8. Brainin M, Samec P. Venous hemodynamics of arteriovenous meningeal fistulas in the posterior cranial fossa. Neuroradiology 1983;25:161–9. 9. Mishra R, Kaw R. Foix-Alajouanine syndrome: an uncommon cause of myelopathy from an anatomic variant circulation. South Med J 2005;98:567–9. 10. Corkill RA, Mitsos AP, Molyneux AJ. Embolization of spinal intramedullary arteriovenous malformations using the liquid embolic agent, Onyx: a single-center experience in a series of 17 patients. J Neurosurg Spine 2007;7:478–85.
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