|Year : 2015 | Volume
| Issue : 4 | Page : 219-222
Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient
Ha Son Nguyen, Andrew Lozen, Ninh Doan, Michael Gelsomin, Saman Shabani, Dennis Maiman
Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI, USA
|Date of Web Publication||21-Oct-2015|
Dr. Ha Son Nguyen
9200 West Wisconsin Avenue, Milwaukee, WI 53226
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Dural ectasia is frequently associated with connective tissue disorders or inflammatory conditions. Presentation in a patient without known risk factors is rare. Moreover, the literature regarding the treatment options for symptomatic dural ectasia is controversial, variable, and limited. A 62-year-old female presents with intractable, postural headaches for years. A lumbar puncture revealed opening pressure 3 cm of water. A computed tomography myelogram of the spine demonstrated erosion of her sacrum due to a large lumbosacral dural ectasia. An initial surgery was attempted to reduce the size of the expansile dura, and reconstruct the dorsal sacrum with a titanium plate (Depuy Synthes, Westchester, PA, USA) to prevent recurrence of thecal sac dilatation. Her symptoms initially improved, but shortly thereafter recurred. A second surgery was then undertaken to obliterate the thecal sac distal to the S2 nerve roots. This could not be accomplished through simple ligation of the thecal sac circumferentially as the ventral dura was noted to be incompetent and attempts to develop an extradural tissue plane were unsuccessful. Consequently, an abundance of fibrin glue was injected into the thecal sac distal to S2, and the dural ectasia was marsupialized rostrally, effectively obliterating the distal thecal sac while further reducing the size of the expansile dura. This approach significantly improved her symptoms at 5 months follow-up. Treatment of dural ectasia is not well-defined and has been variable based on the underlying manifestations. We report a rare patient without risk factors who presented with significant lumbosacral dural ectasia. Moreover, we present a novel method to treat postural headaches secondary to dural ectasia, where the thecal sac is obliterated distal to the S2 nerve roots using an abundance of fibrin glue followed by marsupialization of the thecal sac rostally. This method may offer an effective therapy option as it serves to limit the expansile dura, reducing the cerebrospinal fluid sump and the potential for intracranial hypotension.
Keywords: Dural ectasia, intracranial hypotension, marsupialization
|How to cite this article:|
Nguyen HS, Lozen A, Doan N, Gelsomin M, Shabani S, Maiman D. Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient. J Craniovert Jun Spine 2015;6:219-22
|How to cite this URL:|
Nguyen HS, Lozen A, Doan N, Gelsomin M, Shabani S, Maiman D. Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient. J Craniovert Jun Spine [serial online] 2015 [cited 2020 Apr 4];6:219-22. Available from: http://www.jcvjs.com/text.asp?2015/6/4/219/167887
| Introduction|| |
Dural ectasia involves the enlargement of the spinal canal, concavity of the posterior vertebral body, reduction of cortical bone thickness of the pedicles and laminae, expansion of the neural foramina, formation of a meningocele, scoliosis, spondylolithesis, or vertebral fractures.  Lumbosacral dural ectasia is commonly associated with connective tissue disorders, such as Marfan syndrome, ,,,,,,, osteogenesis imperfecta, Loeys-Dietz syndrome, ,,,, ankylosing spondylitis (AS), , neurofibromatosis, , fibromuscular dysplasia,  and Larsen syndrome;  however, the pathology has also been linked to vertebral fracture, spine surgery,  trisomy 13,  scoliosis,  and trauma.
The pathology may be asymptomatic,  but can cause postural headaches due to a "cerebrospinal fluid sink" mechanism, as well as back pain, leg pain, or rectal pain. , If significant arachnoid cysts are present, the compression of lumbosacral nerve roots may lead to neurological deficits, including cauda equina syndrome (CES). , Moreover, dural ectasia may induce structural spinal instability. ,, Conservative management has been successful, including flat bed rest, hydration, and blood patch. ,, Surgery has been performed for persistent symptoms or CES. General methods have revolved around surgical bony decompression, followed by thecal sac reconstruction , with possible lumboperitoneal shunt placement. 
To our knowledge, no report exists regarding dural ectasia in a patient without known risk factors. We report such a patient, and describe a novel technique to obliterate the dural ectasia through marsupialization of the thecal sac followed by Tisseel glue (Baxter Healthcare Corp., Deerfield, IL, USA) isolation of the estasia from the spinal canal. We also review the surgical techniques employed in the literature.
| Case Report|| |
The patient is a 62-year-old female who complains of headaches for her entire life but recently became progressively severe over the last couple of years. Headaches become progressively severe after 20-30 min of being upright. After lying down, the headaches slowly subside. Magnetic resonance imaging brain and magnetic resonance angiogram neck without significant findings. A lumbar puncture revealed opening pressure 3 cm of water, concerning for low spinal pressure. A computed tomography (CT) myelogram of the spine demonstrated the erosion of her sacrum due to a large dural ectasia [Figure 1], which was felt to be the source of her postural headaches.
|Figure 1: (a) Computed tomography myelogram (sagittal plane) demonstrates large lumbosacral dural ectasia. (b) Computed tomography myelogram (axial plane) demonstrates large lumbosacral dural ectasia|
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Surgery was recommended, entailing a sacral laminectomy and reduction of the dural ectasia.
An incision was made over the sacrum, followed by deep tissue dissection and subperiosteal dissection on the residual sacrum. The dura was immediately identified. Approximately 5 cm of bone circumferentially around the ectasia was removed with Kerrison rongeurs. Subsequently, the dura was incised, and large amounts of cerebrospinal fluid (CSF) drained. An ellipse within the dorsal dura was then excised, and over-sewed with 5-0 nylon. Tisseel and Duragen (Integra Life Sciences, Plaisnboro, NJ, USA) were applied. Valsalva maneuvers showed adequate closure. Subsequently, a titanium mesh fitted to the bony defect and fastened with screws. The wound was closed in layers. Evoked potentials, electromyography, and motor response did not change during the procedure.
Positional headaches initially improved but recurred at 1 month. A repeat CT myelogram of the L-spine demonstrated recurrence dural ectasia, with a size comparable to preoperative imaging. Repeat surgery was recommended to obliterate the thecal sac distal to the sacral nerve roots. Dissection occurred down the prior surgical cavity, as well as more distally into the sacrum. The metal mesh was removed. The dura was opened from L5 to S3. A dural stay stitch was placed. Meticulous inspection revealed no arachnoid cyst. The nerve roots were dissected free from the lateral walls of the dura and positioned anteriorly using cottonoids. Additional dural tissue was resected to narrow the size of the thecal sac. An attempt was made to dissect the ventral dura free in order to ligate the thecal sac at approximately S2, coming below the lowest nerve roots. However, this was unsuccessful as the tissue was notably tenuous with clearly defined erosions. Subsequently, the surgical cavity distal to the S2 roots were filled with Tisseel and Surgicel (Ethicon, Somerville, NJ, USA), forming a solid mass and filling the distal extasia in its entirety. The thecal sac was then marsupialized using 5-0 prolene, reducing the size of the thecal sac by approximately 30-35%. Tisseel and Surgicel were applied along the dural suture line. Valsalva maneuvers showed no evidence of CSF leak. The wound was then closed in layers. The patient woke up well. At 5-month follow-up, patient notes minimal headaches, which responds to caffeine.
| Discussion|| |
The pathogenesis leading to dural ectasia is unclear. Given its frequent association with connective tissue disorders or inflammatory conditions, the prevailing theory implicates CSF pulsations as the primary cause of progressive distention of a weakened dural sac. Associated features include the enlargement of the spinal canal, concavity of the posterior vertebral body, reduction of cortical bone thickness of the pedicles and laminae, expansion of the neural foramina, the formation of meningoceles, fracture of posterior elements, spondylolithesis, and scoliosis.  The most caudal levels of the spine tend to be most affected since CSF pressures are greatest at these levels based on Pascal's law. , On the other hand, dural ectasia has been reported after trauma and after spinal surgery , as well. In additional, the pathology can also occur at cervical  or thoracic levels  without lumbar involvement. Curiously, our patient had no history of connective tissue disorder, and no signs/symptoms to suggest an undiagnosed disorder. Moreover, she had no prior spine trauma or spinal surgery. The literature is scarce regarding dural ectasia in patients without such risk factors.
Treatment of dural ectasia is not well-defined and has been variable based on the underlying manifestations. Where the pathology has been associated with spinal instability/deformity, surgery has been directed to stabilize the spine through reconstruction, instrumentation, and fusion. ,,,, Where the pathology has been associated with postural headaches, conservative management has had some success, including flat bed rest, hydration, and blood patch. ,, Treatment assumes that the postural headaches due to a "cerebrospinal fluid sink" mechanism, which causes traction on pain-sensitive structures such as cranial nerves, dura, and meninges; however, imaging to seek the site of a CSF leak associated with dural estasia has predominantly been negative. ,, However, if a site if discovered, percutaneous injection of fibrin glue and surgical repair of the leak have had success.  For patients who developed dural ectasia after posterior fossa decompression for Chiari malformation More Details, the use of a titanium plate to support dura over the cerebellar hemispheres has been effective.  Where the pathology has been associated with leg pain/rectal pain, procedures have been directed at the dural ectasia. Several investigators have felt that reduction of the expansile dura would decrease pressure and improve symptoms. Wera et al.  reported a patient received a percutaneous injection of fibrin glue for treatment of dural ectasia for back and leg pain; the goal was to use fibrin glue to decrease the expanded dural space and strengthening the attenuated dura; unfortunately, the patient suffered CES after the procedure.
If significant arachnoid cysts are present, the compression of lumbosacral nerve roots may lead to neurological deficits, including CES. , However, CES may also occur without arachnoid cysts; the pathophysiology of this presentation remains unclear. Dural ectasia and CES have predominantly been reported among AS patients. According to Ahn et al.,  conservative management have had limited success for patients with AS and CES Dinichert et al.  and Ahn et al. placed a lumboperitoneal shunt in seven patients with AS with CES; the groups believed that CES was caused by injury to the spinal cord and nerves secondary to an arterial pulse wave transmitted to the CSF, in a dural sac with reduced elasticity due to chronic inflammation and residual adhesions; 6 of 7 patients had symptoms that improved. Ahn et al.  reported decompressive laminectomy for 11 patients with AS with CES (3 improved, 6 stable, 2 worsened). Ha et al.  reported a limited laminotomy followed by filum detethering for a patient with AS-CES, where symptoms did not improve. Similarly, Liu et al.  performed lysis of adhesions along a tethered conus, without clinical improvement. For patients with concurrent sacral cyst along with dural ectasia, the cyst has been treated with marsupialization  or resection. 
Our patient had a prolonged history of postural headaches. Imaging suggested a gradual erosion of her sacrum due to the dural ectasia. An intact sacral wall limited the size of expansion of the cyst and the sump of the CSF. However, once the cyst eroded through the sacrum completely, leading to an enlargement of the potential line of the cyst, the spinal headaches worsened. The initial surgery attempted to reduce the size of the expansile dura while buttressing the reconstruction with a titanium plate to prevent recurrence of thecal sac dilatation. This logic mirrors the use of titanium plate for those that developed dural ectasia after posterior fossa decompression for Chiari malformation. Unfortunately, this approach did not prevent the recurrence of debilitating postural headaches. The second surgery was designed to obliterate the thecal sac distal to the S2 nerve roots. This could not be accomplished through ligation of the entire thecal sac circumferentially since the ventral dura of the thecal sac could not be elevated successfully. Consequently, an abundance of fibrin glue was injected into the thecal sac distal to S2, and the thecal sac was marsupialized rostrally, effectively obliterating the thecal sac while further reducing the size of the expansile dura. This approach significantly improved her symptoms. Percutaneous injection of fibrin glue was not considered since there was no obvious site of CSF leak on her imaging. Lumboperitoneal shunting or laminectomy alone would not be effective since that would increase the intracranial hypotension from the CSF sump.
| Conclusion|| |
Treatment of dural ectasia is not well-defined and has been variable based on the underlying manifestations. We report a rare patient without risk factors who presented with significant lumbosacral dural ectasia. This pathology should remain on the differential for persisting postural headaches, even for patients without risk factors. Moreover, we present a novel method to treatment of postural headaches secondary to dural ectasia, where the thecal sac is obliterated distal to the S2 nerve roots using an abundance of fibrin glue followed by marsupialization of the thecal sac rostally. This method can be effective since the expansile dura is significantly reduced, reducing the CSF sump and the potential for intracranial hypotension.
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| References|| |
Bassani L, Graffeo CS, Behrooz N, Tyagi V, Wilson T, Penaranda S, et al.
Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with Marfan syndrome: Case report and review of the literature. Surg Neurol Int 2014;5:8.
Heck A, Clayton R, Robinson C. Dural ectasia in Marfan syndrome. Int J Obstet Anesth 2014;23:287-8.
Iacono MI, Passera K, Magrassi L, Dore R, Lago P, Arbustini E, et al.
A method for morphological characterization of dural ectasia in Marfan syndrome. Conf Proc IEEE Eng Med Biol Soc 2009;2009:5764-7.
Lundby R, Rand-Hendriksen S, Hald JK, Lilleås FG, Pripp AH, Skaar S, et al.
Dural ectasia in Marfan syndrome: A case control study. AJNR Am J Neuroradiol 2009;30:1534-40.
Mesfin A, Ahn NU, Carrino JA, Sponseller PD. Ten-year clinical and imaging follow-up of dural ectasia in adults with Marfan syndrome. Spine J 2013;13:62-7.
Sakurai A, Miwa T, Miyamoto Y, Mizuno Y, Ka K. Inadequate spinal anesthesia in a patient with Marfan syndrome and dural ectasia. A A Case Rep 2014;2:17-9.
Stone JG, Bergmann LL, Takamori R, Donovan DJ. Giant pseudomeningocele causing urinary obstruction in a patient with Marfan syndrome. J Neurosurg Spine 2015;23:77-80.
Veldhoen S, Stark V, Mueller GC, Derlin T, Bley TA, Weil J, et al.
Pediatric patients with Marfan syndrome: Frequency of dural ectasia and its correlation with common cardiovascular manifestations. Rofo 2014;186:61-6.
Yang HJ, Baek IC, Park SM, Chun DH. Inadequate spinal anesthesia in a parturient with Marfan′s syndrome due to dural ectasia. Korean J Anesthesiol 2014;67 Suppl:S104-5.
Erkula G, Sponseller PD, Paulsen LC, Oswald GL, Loeys BL, Dietz HC. Musculoskeletal findings of Loeys-Dietz syndrome. J Bone Joint Surg Am 2010;92:1876-83
Kono AK, Higashi M, Morisaki H, Morisaki T, Naito H, Sugimura K. Prevalence of dural ectasia in Loeys-Dietz syndrome: Comparison with Marfan syndrome and normal controls. PLoS One 2013;8:e75264.
Ritelli M, Chiarelli N, Dordoni C, Quinzani S, Venturini M, Maroldi R, et al.
Further delineation of Loeys-Dietz syndrome type 4 in a family with mild vascular involvement and a TGFB2 splicing mutation. BMC Med Genet 2014;15:91.
Rodrigues VJ, Elsayed S, Loeys BL, Dietz HC, Yousem DM. Neuroradiologic manifestations of Loeys-Dietz syndrome type 1. AJNR Am J Neuroradiol 2009;30:1614-9.
Sheikhzadeh S, Brockstaedt L, Habermann CR, Sondermann C, Bannas P, Mir TS, et al.
Dural ectasia in Loeys-Dietz syndrome: Comprehensive study of 30 patients with a TGFBR1 or TGFBR2 mutation. Clin Genet 2014;86:545-51.
Bele K, Pendharkar HS, Venkat E, Gupta AK. Anterior dural ectasia mimicking a lytic lesion in the posterior vertebral body in ankylosing spondylitis. J Neurosurg Spine 2011;15:636-40.
Sakellariou VI, Papadopoulos EC, Babis GC. Uncommon complication after revision hip surgery. Orthopedics 2014;37:e608-12.
Bamps S, Calenbergh FV, Loon JV, Paesschen RV, Vanderschot P. Posterolateral approach in a neurofibromatosis type-I patient with severe dystrophic thoracic kyphoscoliosis: A case report, cadaver study, and literature review. J Neurosci Rural Pract 2015;6:84-6.
Khoo Bao JN, Ogunwale B, Huson SM, Ealing J, Whitehouse RW. Spinal bone defects in neurofibromatosis type I with dural ectasia: Stress fractures or dysplastic? A case series. Eur Radiol 2013;23:3418-21.
Ganesh SK, Morissette R, Xu Z, Schoenhoff F, Griswold BF, Yang J, et al.
Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features. FASEB J 2014;28:3313-24.
Jain VV, Anadio JM, Chan G, Sturm PF, Crawford AH. Dural ectasia in a child with Larsen syndrome. J Pediatr Orthop 2014;34:e44-9.
Hong TA, Koenigsberg RA, Brown F, Dastur CK, Kanoff R. Lumbar dural ectasia secondary to spinal fusion: A report of two cases. J Neuroimaging 2006;16:357-60.
Cohen IT. Caudal block complication in a patient with trisomy 13. Paediatr Anaesth 2006;16:213-5.
Abul-Kasim K, Overgaard A, Ohlin A. Dural ectasia in adolescent idiopathic scoliosis: Quantitative assessment on magnetic resonance imaging. Eur Spine J 2010;19:754-9.
Arnold PM, Teuber J. Marfan syndrome and symptomatic sacral cyst: Report of two cases. J Spinal Cord Med 2013;36:499-503.
Ahn NU, Sponseller PD, Ahn UM, Nallamshetty L, Kuszyk BS, Zinreich SJ. Dural ectasia is associated with back pain in Marfan syndrome. Spine (Phila Pa 1976) 2000;25:1562-8.
Ahn NU, Ahn UM, Nallamshetty L, Springer BD, Buchowski JM, Funches L, et al.
Cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome): Meta-analysis of outcomes after medical and surgical treatments. J Spinal Disord 2001;14:427-33.
Liu CC, Lin YC, Lo CP, Chang TP. Cauda equina syndrome and dural ectasia: Rare manifestations in chronic ankylosing spondylitis. Br J Radiol 2011;84:e123-5.
Martín-Fuentes AM, Pretell-Mazzini J, Curto de la Mano A, Viña-Fernández R. High-grade spondyloretrolisthesis in a 12-year-old girl with neurofibromatosis type 1: A case report and literature review. J Pediatr Orthop B 2013;22:110-6.
Modi HN, Srinivasalu S, Suh SW, Yang JH. Grade 4 spondylolisthesis of the L5 vertebra associated with dural ectasia in neurofibromatosis. Singapore Med J 2009;50:e287-92.
Rosa S, Davagnanam I. Teaching neuroimages: Bilateral pedicular fractures in severe lumbar dural ectasia. Neurology 2012;78:e11.
Cheuret E, Edouard T, Mejdoubi M, Acar P, Pienkowski C, Cances C, et al.
Intracranial hypotension in a girl with Marfan syndrome: Case report and review of the literature. Childs Nerv Syst 2008;24:509-13.
Milledge JT, Ades LC, Cooper MG, Jaumees A, Onikul E. Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent. J Paediatr Child Health 2005;41:68-71.
Cho SK, Stoker GE, Bridwell KH. Spinal reconstruction with pedicle screw-based instrumentation and rhBMP-2 in patients with neurofibromatosis and severe dural ectasia and spinal deformity: Report of two cases and a review of the literature. J Bone Joint Surg Am 2011;93:e86.
Voermans NC, Dijk KG, Bos MM, Geus-Oei LF, Verrips A, Lindert EJ. Postural headache in Marfan syndrome associated with spinal cysts and liquor hypotension. Neuropediatrics 2009;40:201-4.
Wera GD, Dean CL, Nho S, Ahn UM, Cassinelli EH, Liu RW, et al.
Cauda equina syndrome resulting from treatment of dural ectasia with fibrin glue injection. J Spinal Disord Tech 2006;19:148-50.
Udani V, Holly LT, Chow D, Batzdorf U. Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation. World Neurosurg 2014;81:836-41.
Inoue M, Sairyo K, Sakai T, Yasui N. Significance of surgical treatment for severe dystrophic changes in the cervical spine associated with neurofibromatosis type I: A case report. J Pediatr Orthop B 2010;19:270-5.
Helfen M, Götzinger R, Lütke A, Likoyiannis A, Griss P. Intrathoracic dural ectasia mimicking neurofibroma and scoliosis. A case report. Int Orthop 1995;19:181-4.
Avela K, Valanne L, Helenius I, Mäkitie O. Hajdu-Cheney syndrome with severe dural ectasia. Am J Med Genet A 2011;155A:595-8.
de Kleuver M, van Jonbergen JP, Langeloo DD. Asymptomatic massive dural ectasia associated with neurofibromatosis type 1 threatening spinal column support: Treatment by anterior vascularized fibula graft. J Spinal Disord Tech 2004;17:539-42.
Toyoda K, Taguchi T, Kaneko K, Kato Y, Imajo Y, Imagama T. High-grade L5 spondylolisthesis associated with dural ectasia in neurofibromatosis. J Orthop Sci 2005;10:233-6.
Puget S, Kondageski C, Wray A, Boddaert N, Roujeau T, Di Rocco F, et al.
Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome. Case report. J Neurosurg 2007;106 1 Suppl:48-52.
Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L. Spontaneous intracranial hypotension in childhood and adolescence. J Pediatr 2013;163:504-10.
Dinichert A, Cornelius JF, Lot G. Lumboperitoneal shunt for treatment of dural ectasia in ankylosing spondylitis. J Clin Neurosci 2008;15:1179-82.
Ha SW, Son BC. Cauda equina syndrome associated with dural ectasia in chronic anlylosing spondylitis. J Korean Neurosurg Soc 2014;56:517-20.