Dural Ectasia in Marfan Syndrome: A Case Control Study

Lundby, Rigmor; Rand‐Hendriksen, Svend; Hald, John; Lilleås, Finn; Pripp, Are Hugo; Skaar, S.; Paus, Benedicte; Geiran, Odd; Smith, Hans‐Jørgen

doi:10.3174/ajnr.a1620

Cited by 51 publications

(65 citation statements)

References 29 publications

(57 reference statements)

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“…All the patients were assessed for all subsets of the Ghent criteria (Ghent 1) 1 by the same group of physicians. [10][11][12][13][14][15][16] For assessing fulfilment of the major cardiovascular criterion in the Ghent nosology, information was collected about previous surgery related to dilatation or dissection of the ascending aorta, and electrocardiographic evidence of dilatation of the ascending aorta was recorded. 1,13 The assessment of fulfilment of the Ghent criteria included sequencing of the entire coding region of the gene FBN1 and searching for large deletions or duplications.…”

Section: Study Populationmentioning

confidence: 99%

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The pulmonary artery in patients with Marfan syndrome: a cross-sectional study

Lundby

Rand‐Hendriksen

Hald

et al. 2012

Genetics in Medicine

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Purpose:The objectives of this study were to establish the prevalence of pulmonary artery dilatation in Marfan syndrome using modern radiological methods and to correlate the diameter of the vessel with aortic disease. methods: Magnetic resonance or computed tomography imaging of the pulmonary artery and aorta was performed in 87 patients with proven Marfan syndrome. Diameters of the root and trunk of the pulmonary artery and of the aortic root were measured perpendicular to the long axes of the vessels. Pulmonary artery diameters were measured on axial images, and aortic diameters were assessed on oblique sagittal images. Results:As compared with normal values in the literature, 47 of the 87 patients (54%) had widening of the trunk of the pulmonary artery (≥30 mm). Of these 47, 15% had no sign of disease of the ascending aorta. The mean (SD) ratio between the diameters of the root and trunk of the pulmonary artery was 1.18 (0.155). Multivariate analysis showed that surgery of the ascending aorta and high body surface area were associated with dilatation of the trunk of the pulmonary artery.conclusions: Pulmonary artery dilatation is present in a high proportion of patients with Marfan syndrome as assessed using cutoff values based on measurements in the normal population. Severe disease of the ascending aorta correlates significantly with pulmonary artery trunk dilatation in patients with Marfan syndrome. 2012:14(11):922-927 Genet Med

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Section: Study Populationmentioning

confidence: 99%

“…8 This article presents data on pulmonary artery dimensions in the study population described in detail in previous studies. [10][11][12][13][14][15][16] All investigations in patients were carried out in the period [2003][2004]. Therefore all results reported in those studies are in accordance with the Ghent 1 criteria.…”

Section: Introductionmentioning

confidence: 99%

The pulmonary artery in patients with Marfan syndrome: a cross-sectional study

Lundby

Rand‐Hendriksen

Hald

et al. 2012

Genetics in Medicine

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“…A number of radiographic features identifying dural ectasia have been suggested including a dilatation of the dural sac with the diameter of the thecal sac at S1 level greater than the diameter at L4 level, 1,2 herniation of the dura along nerve root sleeves, and elevated dural sac ratios (the ratio between the dorsal sac diameter and vertebral body diameter at the same level). 1 Despite these various radiographic features, no gold standard for diagnosis of dural ectasia has emerged. Dural ectasia is as a key characteristic of Marfan syndrome but may also be associated with Ehlers-Danlos syndrome, neurofibromatosis, ankylosing spondylitis and osteogenesis imperfecta tarda.…”

Section: Discussionmentioning

confidence: 99%

“…3 Three large studies have observed dural ectasia in 63-92% of patients with Marfan syndrome. 1,4 The most common symptom is constipation, but other symptoms, including urinary retention or incontinence, dysmenorrhea, dyspareunia and pelvic or lower-back pain, can also occur from mass effects. 5,6 The differential diagnosis for an obstructing pelvic cyst includes dural ectasias extending into the pelvis as an anterior sacral meningocele, as well as other masses of genitourinary, gynecologic and gastrointestinal origin including ovarian cysts, bladder diverticuli and congenital arachnoid cysts.…”

Section: 2mentioning

confidence: 99%

Pelvic dural ectasia leading to bilateral ureteric obstruction in the pediatric patient

Tran

Kavanagh

Steinbok

et al. 2013

CUAJ

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Large pelvic cysts and moderate to severe hydroureteronephrosis were found after investigating hypertension in a 16-year-old child with Marfan's and known pelvic dural ectasia. Follow-up magnetic resonance imaging demonstrated extensive ectasia of the dural sac at the sacral level with displacement of the bowel, bladder and bilateral ureteric obstruction with accompanying hydroureteronephrosis that was advanced compared to prior imaging. Postural headaches secondary to cerebrospinal fluid leak and progressive hydroureteronephrosis prompted a combined neurosurgical and urologic resection of the pelvic masses. In this report, we discuss the preoperative evaluation and management of this rare form of bilateral ureteric obstruction. Case reportFollowing aortic valve replacement and mitral valve repair, a 16-year-old female with Marfan's syndrome had a hypertensive crisis while under monitoring in the intensive care unit. Cardiac catheterization and aortograms were done which did not show any issues with the repair. A chest x-ray was performed the next day. An incidental finding of dilated and opacified renal collecting system was noted. This prompted an abdominal ultrasound revealing bilateral hydroureteronephrosis and a large cystic mass in the pelvis. Urology consult was requested for further management of the bilateral ureteric obstruction.The patient remained entirely asymptomatic with normal bowel and bladder function. Her genitourinary exam was completely normal, except for mild urethral prolapse. Neurologic examination revealed no abnormality. The renal function remained normal (creatinine 57 mmol/L). Magnetic resonance imaging (MRI) of her abdomen and thorax 1 year earlier for evaluation of her cardiac pathology revealed extensive ectasia of the dural sac at the sacral level (Fig. 1). This was determined to be an out-pouching through the S2 foramina on both sides into the presacral region as a large cystic area. No concominant hydroureteronephrosis was seen. A repeat MRI of the abdomen was performed showing enlargement of the pelvic cysts with bilateral hydroureteronephrosis (Fig. 2). Based on the progression of hydroureteronephrosis over the 1-year interval between scans and the development of hypertension, bilateral ureteric stents were inserted. This procedure was performed in an uncomplicated fashion and the patient was discharged from hospital.Two months later, she returned to the hospital with complaints of progressive severe postural headaches. Neurosurgical evaluation prompted a meningomyelogram which revealed clear communication of the pelvic dural ectasia with the spinal subarachnoid space. There was also evidence of recent blood in the cyst. Her headaches were considered to be low pressure headaches, caused by cerebrospinal fluid leak into a partially ruptured pelvic meningocele. Bleeding into the cysts may have been facilitated by warfarin, an anticoagulant which she was taking because of her artificial heart valve. She consented to have her pelvic cysts resected and drained in a combined...

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“…5 The prevalence of DE in patients with Marfan syndrome is estimated to be from 63-92%. 6 Although DE can occur at different levels of the lumbar spine, it is most common at the L5-S1 level. 7 The anesthetic management of these patients can be challenging, and the literature regarding the management of these cases is scarce.…”

Section: Résumémentioning

confidence: 99%

Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases

Baghirzada

Krings

Carvalho

2012

Can J Anesth/J Can Anesth

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Background The anesthetic management of women with Marfan syndrome and dural ectasia undergoing Cesarean delivery remains controversial. We present two cases of patients with Marfan syndrome and dural ectasia where neuraxial anesthesia was used successfully. Case 1 A 31-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 35 3/7 weeks' gestation. The magnetic resonance imaging of her spine revealed significant dural ectasia in the lumbosacral area with a mean lumbar dural sac area of 4.71 cm 2 . Intrathecal administration of 0.75% hyperbaric bupivacaine 9 mg produced only limited perineal analgesia. The epidural catheter was titrated, and 0.5% bupivacaine 150 mg were required to achieve a T4 sensory level. Case 2 A 34-yr-old G1P0 female with Marfan syndrome presented for elective Cesarean delivery at 37 weeks' gestation. The intrathecal administration of 0.75% hyperbaric bupivacaine 13.5 mg produced a T5 sensory level. Magnetic resonance imaging of her spine prior to discharge confirmed the diagnosis of moderate dural ectasia with a mean lumbar dural sac area of 3.61 cm 2 . Discussion The two patients described in this report responded differently to spinal anesthesia, most likely based on the severity of their dural ectasia. Although preoperative magnetic resonance imaging may help to identify patients at risk for a failed spinal, we suggest considering a combined spinal-epidural technique in cases of dural ectasia. RésuméContexte La prise en charge anesthe´sique de femmes ayant un syndrome de Marfan et une ectasie de la dure-me`re au cours d'un accouchement par ce´sarienne reste un sujet controverse´. Nous pre´sentons le cas de deux patientes ayant un syndrome de Marfan et une ectasie de la dure-me`re chez qui une anesthe´sie neuraxiale a e´te´re´alise´e avec succe`s. Cas n8 1 Une femme de 31 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`35 semaines et 3 jours de grossesse. L'imagerie par re´sonance magne´tique de la colonne verte´brale a re´ve´le´une ectasie significative de la dure-me`re dans la re´gion lombosacre´e avec une surface moyenne du sac dural lombaire de 4,71 cm 2 . L'administration intrathe´cale de 9 mg de bupivacaı¨ne hyperbare a`0,75 % n'a procure´qu'une analge´sie pe´rine´ale limite´e. La dose du cathe´ter pe´ridural a e´te´titre´e et 150 mg de bupivacaı¨ne 0,5 % ont e´te´ne´cessaires pour atteindre le niveau sensitif D4. Cas n8 2 Une femme de 34 ans, G1P0, ayant un syndrome de Marfan a e´te´admise pour accouchement par ce´sarienne programme´a`37 semaines de grossesse. L'administration intrathe´cale de 13,5 mg de bupivacaı¨ne Author contributions Leyla Baghirzada and Jose C.A. Carvalho cared for patients, reviewed the literature, and wrote the manuscript. Jose C.A. Carvalho and Timo Krings revised the manuscript, and Leyla Baghirzada submitted the final version of the manuscript. Timo Krings interpreted the magnetic resonance imagings for both patients.

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Dural Ectasia in Marfan Syndrome: A Case Control Study

Cited by 51 publications

References 29 publications

The pulmonary artery in patients with Marfan syndrome: a cross-sectional study

The pulmonary artery in patients with Marfan syndrome: a cross-sectional study

Pelvic dural ectasia leading to bilateral ureteric obstruction in the pediatric patient

Regional anesthesia in Marfan syndrome, not all dural ectasias are the same: a report of two cases

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