Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome
Identifieur interne : 009683 ( Main/Exploration ); précédent : 009682; suivant : 009684Chiari-like tonsillar herniation associated with intracranial hypotension in Marfan syndrome
Auteurs : Stéphanie Puget [France] ; Charles Kondageski [France] ; Alison Wray [Australie] ; Nathalie Boddaert [France] ; Thomas Roujeau [France] ; Federico Di Rocco [France] ; Michel Zerah [France] ; Christian Sainte-Rose [France]Source :
- Journal of neurosurgery. Pediatrics
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
Abstract
√ The authors describe the case of a 12-year-old girl with Marfan syndrome, sacral dural ectasia, and tonsillar hemiation, who presented with headache Initially, it was hypothesized that the headaches were secondary to the tonsillar herniation, and the patient consequently underwent surgical decompression of the foramen magnum. Postoperatively, the patient's condition did not improve, and additional magnetic resonance (MR) imaging demonstrated evidence of a cerebrospinal fluid (CSF) leak at the level of the dural ectasia. It was surmised that the girl's symptoms were due to spontaneous intracranial hypotension (SIH) and that the tonsillar herniation was caused by the leakage. The patient responded well to application of a blood patch at the level of the demonstrated leak, and her headache resolved. This appears to be the first reported case of a patient with Marfan syndrome presenting with a symptomatic spontaneous CSF leak complicated by tonsillar herniation. In this rare association of SIH and connective tissue disorders, recognition of the clinical signs and typical MR imaging features of SIH may lead to more appropriate and less invasive treatment, potentially avoiding surgery.
Affiliations:
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first="Stéphanie" last="Puget">Stéphanie Puget</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Kondageski, Charles" sort="Kondageski, Charles" uniqKey="Kondageski C" first="Charles" last="Kondageski">Charles Kondageski</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Wray, Alison" sort="Wray, Alison" uniqKey="Wray A" first="Alison" last="Wray">Alison Wray</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Neurosurgery, Royal Children's Hospital</s1><s2>Melbourne</s2><s3>AUS</s3><sZ>3 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName></affiliation></author><author><name sortKey="Boddaert, Nathalie" sort="Boddaert, Nathalie" uniqKey="Boddaert N" first="Nathalie" last="Boddaert">Nathalie Boddaert</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Roujeau, Thomas" sort="Roujeau, Thomas" uniqKey="Roujeau T" first="Thomas" last="Roujeau">Thomas Roujeau</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Di Rocco, Federico" sort="Di Rocco, Federico" uniqKey="Di Rocco F" first="Federico" last="Di Rocco">Federico Di Rocco</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Zerah, Michel" sort="Zerah, Michel" uniqKey="Zerah M" first="Michel" last="Zerah">Michel Zerah</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Sainte Rose, Christian" sort="Sainte Rose, Christian" uniqKey="Sainte Rose C" first="Christian" last="Sainte-Rose">Christian Sainte-Rose</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades</s1><s2>Paris</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author></analytic><series><title level="j" type="main">Journal of neurosurgery. Pediatrics</title></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Journal of neurosurgery. Pediatrics</title></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Arnold Chiari malformation</term><term>Child</term><term>Intracranial hypotension</term><term>Marfan syndrome</term><term>Patch</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Hypotension intracrânienne</term><term>Marfan syndrome</term><term>Arnold Chiari syndrome</term><term>Patch</term><term>Enfant</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">√ The authors describe the case of a 12-year-old girl with Marfan syndrome, sacral dural ectasia, and tonsillar hemiation, who presented with headache Initially, it was hypothesized that the headaches were secondary to the tonsillar herniation, and the patient consequently underwent surgical decompression of the foramen magnum. Postoperatively, the patient's condition did not improve, and additional magnetic resonance (MR) imaging demonstrated evidence of a cerebrospinal fluid (CSF) leak at the level of the dural ectasia. It was surmised that the girl's symptoms were due to spontaneous intracranial hypotension (SIH) and that the tonsillar herniation was caused by the leakage. The patient responded well to application of a blood patch at the level of the demonstrated leak, and her headache resolved. This appears to be the first reported case of a patient with Marfan syndrome presenting with a symptomatic spontaneous CSF leak complicated by tonsillar herniation. In this rare association of SIH and connective tissue disorders, recognition of the clinical signs and typical MR imaging features of SIH may lead to more appropriate and less invasive treatment, potentially avoiding surgery.</div></front></TEI><affiliations><list><country><li>Australie</li><li>France</li></country><region><li>Victoria (État)</li><li>Île-de-France</li></region><settlement><li>Melbourne</li><li>Paris</li></settlement></list><tree><country name="France"><region name="Île-de-France"><name sortKey="Puget, Stephanie" sort="Puget, Stephanie" uniqKey="Puget S" first="Stéphanie" last="Puget">Stéphanie Puget</name></region><name sortKey="Boddaert, Nathalie" sort="Boddaert, Nathalie" uniqKey="Boddaert N" first="Nathalie" last="Boddaert">Nathalie Boddaert</name><name sortKey="Di Rocco, Federico" sort="Di Rocco, Federico" uniqKey="Di Rocco F" first="Federico" last="Di Rocco">Federico Di Rocco</name><name sortKey="Kondageski, Charles" sort="Kondageski, Charles" uniqKey="Kondageski C" first="Charles" last="Kondageski">Charles Kondageski</name><name sortKey="Roujeau, Thomas" sort="Roujeau, Thomas" uniqKey="Roujeau T" first="Thomas" last="Roujeau">Thomas Roujeau</name><name sortKey="Sainte Rose, Christian" sort="Sainte Rose, Christian" uniqKey="Sainte Rose C" first="Christian" last="Sainte-Rose">Christian Sainte-Rose</name><name sortKey="Zerah, Michel" sort="Zerah, Michel" uniqKey="Zerah M" first="Michel" last="Zerah">Michel Zerah</name></country><country name="Australie"><region name="Victoria (État)"><name sortKey="Wray, Alison" sort="Wray, Alison" uniqKey="Wray A" first="Alison" last="Wray">Alison Wray</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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