Vertebral Scalloping in Neurofibromatosis

Casselman, E S; Mandell, Gerald A.

doi:10.1148/131.1.89

Cited by 64 publications

(24 citation statements)

References 20 publications

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“…The association between vertebral anomalies and dural ectasias seem to be in accord with the hypothesis that both neuroectodermal and mesodermal dys- plasia may be operative in causing the disease [1,2,5]. The medulla appeared slightly compressed from the front and displaced to the left, agreeing with the direction of the scoliosis.…”

Section: Discussionsupporting

confidence: 80%

A case of a thoracic vertebral body dislocation without neurological signs in a child with neurofibromatosis

Colajacomo

Gugliantini

et al. 1986

Pediatr Radiol

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Section: Discussionsupporting

confidence: 80%

A case of a thoracic vertebral body dislocation without neurological signs in a child with neurofibromatosis

Colajacomo

Gugliantini

et al. 1986

Pediatr Radiol

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“…It is not known if this is a primary malformation or secondary to the vertebral abnormalities. Dural ectasia may be a primary mesodermal dysplasia of the meninges [Casselman and Mandell, 1979] skeletal abnormality, in which vertebral bodies may be so severely deformed as to resemble malformations. Weakening of spinal stabilizers (i.e., facets, pedicles, and ligaments), perhaps by dural ectasia with meningocele formation, may lead to kyphosis.…”

Section: Vertebral Defectsmentioning

confidence: 99%

Skeletal abnormalities in neurofibromatosis type 1: Approaches to therapeutic options

Elefteriou

Kolanczyk

Schindeler

et al. 2009

American J of Med Genetics Pt A

136

114

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The skeleton is frequently affected in individuals with neurofibromatosis type 1, and some of these bone manifestations can result in significant morbidity. The natural history and pathogenesis of the skeletal abnormalities of this disorder are poorly understood and consequently therapeutic options for these manifestations are currently limited. The Children's Tumor Foundation convened an International Neurofibromatosis Type 1 Bone Abnormalities Consortium to address future directions for clinical trials in skeletal abnormalities associated with this disorder. This report reviews the clinical skeletal manifestations and available preclinical mouse models and summarizes key issues that present barriers to optimal clinical management of skeletal abnormalities in neurofibromatosis type 1. These concepts should help advance optimal clinical management of the skeletal abnormalities in this disease and address major difficulties encountered for the design of clinical trials. © 2009 Wiley‐Liss, Inc.

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“…7 Dural ectasia has also been reported in patients with neurofibromatosis type 1, Ehler-Danlos syndrome, ankylosing spondylitis, trauma, tumours, and scoliosis. [12][13][14] The severity of DE can be evaluated by computed tomography or MRI using either quantitative (actual dural sac measurements) or qualitative (evaluating the prevalence of different features of DE, i.e., scalloping) criteria. There are no universally accepted quantitative criteria for diagnosing DE, and various methods have been used, including evaluation of dural sac ratio, 15 assessment of dural sac diameter at S1 and L4 levels, nerve root sleeve diameter, 16 and lumbar pedicle width.…”

Section: Discussionmentioning

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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Vertebral Scalloping in Neurofibromatosis

Cited by 64 publications

References 20 publications

A case of a thoracic vertebral body dislocation without neurological signs in a child with neurofibromatosis

A case of a thoracic vertebral body dislocation without neurological signs in a child with neurofibromatosis

Skeletal abnormalities in neurofibromatosis type 1: Approaches to therapeutic options

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

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