Marsupialization and distal obliteration of a lumbosacral dural ectasia in a nonsyndromic, adult patient

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.

[1]  Jeremy G. Stone,et al.  Giant pseudomeningocele causing urinary obstruction in a patient with Marfan syndrome. , 2015, Journal of neurosurgery. Spine.

[2]  F. Calenbergh,et al.  Posterolateral approach in a neurofibromatosis type-I patient with severe dystrophic thoracic kyphoscoliosis: A case report, cadaver study, and literature review , 2015, Journal of neurosciences in rural practice.

[3]  S. Blankenberg,et al.  Dural ectasia in Loeys–Dietz syndrome: comprehensive study of 30 patients with a TGFBR1 or TGFBR2 mutation , 2014, Clinical genetics.

[4]  B. Son,et al.  Cauda Equina Syndrome Associated with Dural Ectasia in Chronic Anlylosing Spondylitis , 2014, Journal of Korean Neurosurgical Society.

[5]  D. Chun,et al.  Inadequate spinal anesthesia in a parturient with Marfan's syndrome due to dural ectasia , 2014, Korean journal of anesthesiology.

[6]  A. Crawford,et al.  Dural Ectasia in a Child With Larsen Syndrome , 2014, Journal of pediatric orthopedics.

[7]  M. Venturini,et al.  Further delineation of Loeys-Dietz syndrome type 4 in a family with mild vascular involvement and a TGFB2 splicing mutation , 2014, BMC Medical Genetics.

[8]  G. Babis,et al.  Uncommon complication after revision hip surgery. , 2014, Orthopedics.

[9]  L. Holly,et al.  Posterior fossa reconstruction using titanium plate for the treatment of cerebellar ptosis after decompression for Chiari malformation. , 2014, World neurosurgery.

[10]  D. Milewicz,et al.  Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF‐β expression and connective tissue features , 2014, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

[11]  G. Fatterpekar,et al.  Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature , 2014, Surgical neurology international.

[12]  Y. Mizuno,et al.  Inadequate spinal anesthesia in a patient with marfan syndrome and dural ectasia. , 2014, A & A case reports.

[13]  A. Kono,et al.  Prevalence of Dural Ectasia in Loeys-Dietz Syndrome: Comparison with Marfan Syndrome and Normal Controls , 2013, PloS one.

[14]  T. Derlin,et al.  Pediatric Patients with Marfan Syndrome: Frequency of Dural Ectasia and its Correlation with Common Cardiovascular Manifestations , 2013, Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren.

[15]  P. Arnold,et al.  Marfan syndrome and symptomatic sacral cyst: Report of two cases , 2013, The journal of spinal cord medicine.

[16]  W. Schievink,et al.  Spontaneous intracranial hypotension in childhood and adolescence. , 2013, The Journal of pediatrics.

[17]  R. Whitehouse,et al.  Spinal bone defects in neurofibromatosis type I with dural ectasia: stress fractures or dysplastic? A case series , 2013, European Radiology.

[18]  J. Pretell-Mazzini,et al.  High-grade spondyloretrolisthesis in a 12-year-old girl with neurofibromatosis type 1: a case report and literature review. , 2013, Journal of pediatric orthopedics. Part B.

[19]  J. Carrino,et al.  Ten-year clinical and imaging follow-up of dural ectasia in adults with Marfan syndrome. , 2013, The spine journal : official journal of the North American Spine Society.

[20]  I. Davagnanam,et al.  Teaching NeuroImages: Bilateral pedicular fractures in severe lumbar dural ectasia , 2012, Neurology.

[21]  Arun Gupta,et al.  Anterior dural ectasia mimicking a lytic lesion in the posterior vertebral body in ankylosing spondylitis. , 2011, Journal of neurosurgery. Spine.

[22]  Samuel K. Cho,et al.  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. , 2011, The Journal of bone and joint surgery. American volume.

[23]  O. Mäkitie,et al.  Hajdu–Cheney syndrome with severe dural ectasia , 2011, American journal of medical genetics. Part A.

[24]  Chung-Ping Lo,et al.  Cauda equina syndrome and dural ectasia : rare manifestations in chronic ankylosing spondylitis , 2011 .

[25]  H. Dietz,et al.  Musculoskeletal findings of Loeys-Dietz syndrome. , 2010, The Journal of bone and joint surgery. American volume.

[26]  K. Sairyo,et al.  Significance of surgical treatment for severe dystrophic changes in the cervical spine associated with neurofibromatosis type I: a case report. , 2010, Journal of pediatric orthopedics. Part B.

[27]  K. Abul-Kasim,et al.  Dural ectasia in adolescent idiopathic scoliosis: quantitative assessment on magnetic resonance imaging , 2010, European Spine Journal.

[28]  L. Mainardi,et al.  A method for morphological characterization of dural ectasia in marfan syndrome , 2009, 2009 Annual International Conference of the IEEE Engineering in Medicine and Biology Society.

[29]  J. Hald,et al.  Dural Ectasia in Marfan Syndrome: A Case Control Study , 2009, American Journal of Neuroradiology.

[30]  D. Yousem,et al.  Neuroradiologic Manifestations of Loeys-Dietz Syndrome Type 1 , 2009, American Journal of Neuroradiology.

[31]  J. H. Yang,et al.  Grade 4 spondylolisthesis of the L5 vertebra associated with dural ectasia in neurofibromatosis. , 2009, Singapore medical journal.

[32]  N. Voermans,et al.  Postural headache in marfan syndrome associated with spinal cysts and liquor hypotension. , 2009, Neuropediatrics.

[33]  L. Concepcion,et al.  Dural ectasia in Marfan syndrome , 2008, Neurology.

[34]  G. Lot,et al.  Lumboperitoneal shunt for treatment of dural ectasia in ankylosing spondylitis , 2008, Journal of Clinical Neuroscience.

[35]  C. Cancés,et al.  Intracranial hypotension in a girl with Marfan syndrome: case report and review of the literature , 2008, Child's Nervous System.

[36]  N. Boddaert,et al.  Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome. Case report. , 2007, Journal of neurosurgery.

[37]  R. Koenigsberg,et al.  Lumbar Dural Ectasia Secondary to Spinal Fusion: A Report of Two Cases , 2006, Journal of neuroimaging : official journal of the American Society of Neuroimaging.

[38]  G. Andersson,et al.  Cauda Equina Syndrome Resulting From Treatment of Dural Ectasia With Fibrin Glue Injection , 2006, Journal of spinal disorders & techniques.

[39]  I. Cohen Caudal block complication in a patient with trisomy 13 , 2006, Paediatric anaesthesia.

[40]  Y. Kato,et al.  High-grade L5 spondylolisthesis associated with dural ectasia in neurofibromatosis , 2005, Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association.

[41]  E. Onikul,et al.  Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent , 2005, Journal of paediatrics and child health.

[42]  D. Langeloo,et al.  Asymptomatic massive dural ectasia associated with neurofibromatosis type 1 threatening spinal column support: treatment by anterior vascularized fibula graft. , 2004, Journal of spinal disorders & techniques.

[43]  P. Griss,et al.  Intrathoracic dural ectasia mimicking neurofibroma and scoliosis , 1995, International Orthopaedics.

[44]  L. Nallamshetty,et al.  Cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome): meta-analysis of outcomes after medical and surgical treatments. , 2001, Journal of spinal disorders.

[45]  L. Nallamshetty,et al.  Dural Ectasia Is Associated With Back Pain in Marfan Syndrome , 2000, Spine.