Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature

Bassani, Luigi; Graffeo, Christopher S.; Behrooz, Navid; Tyagi, V.V.; Wilson, Taylor; Penaranda, Saul; Zagzag, David; Rifkin, Daniel B.; Barcellos-Hoff, Mary Helen; Fatterpekar, Girish M.; Placantonakis, Dimitris G.

doi:10.4103/2152-7806.125629

Cited by 18 publications

(6 citation statements)

References 35 publications

(47 reference statements)

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“…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. [ 1 ] 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. [ 21 35 ] On the other hand, dural ectasia has been reported after trauma and after spinal surgery[ 21 36 ] as well.…”

Section: Discussionmentioning

confidence: 99%

“…[ 29 33 39 40 41 ] Where the pathology has been associated with postural headaches, conservative management has had some success, including flat bed rest, hydration, and blood patch. [ 1 31 32 ] 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. [ 24 31 42 ] However, if a site if discovered, percutaneous injection of fibrin glue and surgical repair of the leak have had success.…”

Section: Discussionmentioning

confidence: 99%

“…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. [ 1 ] Lumbosacral dural ectasia is commonly associated with connective tissue disorders, such as Marfan syndrome,[ 2 3 4 5 6 7 8 9 ] osteogenesis imperfecta, Loeys-Dietz syndrome,[ 10 11 12 13 14 ] ankylosing spondylitis (AS),[ 15 16 ] neurofibromatosis,[ 17 18 ] fibromuscular dysplasia,[ 19 ] and Larsen syndrome;[ 20 ] however, the pathology has also been linked to vertebral fracture, spine surgery,[ 21 ] trisomy 13,[ 22 ] scoliosis,[ 23 ] and trauma.…”

Section: Introductionmentioning

confidence: 99%

“…[ 28 29 30 ] Conservative management has been successful, including flat bed rest, hydration, and blood patch. [ 1 31 32 ] Surgery has been performed for persistent symptoms or CES. General methods have revolved around surgical bony decompression, followed by thecal sac reconstruction[ 33 34 ] with possible lumboperitoneal shunt placement.…”

Section: Introductionmentioning

confidence: 99%

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

Nguyen

Lozen

Doan

et al. 2015

J Craniovert Jun Spine

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Nguyen

Lozen

Doan

et al. 2015

J Craniovert Jun Spine

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“…Dural ectasia has been related to chronic lumbosacral pain syndrome, and it may give rise to complications in rare circumstances. For example, spontaneous cerebrospinal fluid leaks can result in intracranial hypotension presenting with postural headache; and giant anterior or lateral meningocele may cause constipation, urinary symptoms including retention or incontinence, or dysmenorrhea 94,95…”

Section: Team Membersmentioning

confidence: 99%

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Kodolitsch¹,

Rybczynski²,

Vogler³

et al. 2016

JMDH

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Marfan syndrome (MFS) is a rare, severe, chronic, life-threatening disease with multiorgan involvement that requires optimal multidisciplinary care to normalize both prognosis and quality of life. In this article, each key team member of all the medical disciplines of a multidisciplinary health care team at the Hamburg Marfan center gives a personal account of his or her contribution in the management of patients with MFS. The authors show how, with the support of health care managers, key team members organize themselves in an organizational structure to create a common meaning, to maximize therapeutic success for patients with MFS. First, we show how the initiative and collaboration of patient representatives, scientists, and physicians resulted in the foundation of Marfan centers, initially in the US and later in Germany, and how and why such centers evolved over time. Then, we elucidate the three main structural elements; a team of coordinators, core disciplines, and auxiliary disciplines of health care. Moreover, we explain how a multidisciplinary health care team integrates into many other health care structures of a university medical center, including external quality assurance; quality management system; clinical risk management; center for rare diseases; aorta center; health care teams for pregnancy, for neonates, and for rehabilitation; and in structures for patient centeredness. We provide accounts of medical goals and standards for each core discipline, including pediatricians, pediatric cardiologists, cardiologists, human geneticists, heart surgeons, vascular surgeons, vascular interventionists, orthopedic surgeons, ophthalmologists, and nurses; and of auxiliary disciplines including forensic pathologists, radiologists, rhythmologists, pulmonologists, sleep specialists, orthodontists, dentists, neurologists, obstetric surgeons, psychiatrist/psychologist, and rehabilitation specialists. We conclude that a multidisciplinary health care team is a means to maximize therapeutic success.

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Marfan syndrome: Evolving organ manifestations—A 10‐year follow‐up study

Vanem

Böker

Sandvik

et al. 2019

American J of Med Genetics Pt A

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The age‐dependent penetrance of organ manifestations in Marfan syndrome (MFS) is not known. The aims of this follow‐up study were to explore how clinical features change over a 10‐year period in the same Norwegian MFS cohort. In 2003–2004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology. Ten years later, we performed follow‐up investigations of the survivors (n = 48) who consented. Forty‐six fulfilled the revised Ghent criteria. Median age: females 51 years, range 32–80 years; males 45 years, range 30–67 years. New aortic root dilatation was detected in patients up to 70 years. Ascending aortic pathology was diagnosed in 93 versus 72% at baseline. Sixty‐five percent had undergone aortic surgery compared to 39% at baseline. Pulmonary trunk mean diameter had increased significantly compared to baseline. From inclusion to follow‐up, two patients (three eyes) developed ectopia lentis, four developed dural ectasia, four developed scoliosis, three developed incisional or recurrent herniae, and 14 developed hindfoot deformity. No changes were found regarding protrusio acetabuli, spontaneous pneumothorax, or striae atrophicae. The study confirms that knowledge of incidence and progression of organ manifestations throughout life is important for diagnosis, treatment, and follow‐up of patients with verified or suspected MFS.

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Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature

Cited by 18 publications

References 35 publications

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

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

The role of the multidisciplinary health care team in the management of patients with Marfan syndrome

Marfan syndrome: Evolving organ manifestations—A 10‐year follow‐up study

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