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Published in: Child's Nervous System 2/2020

01-02-2020 | Arteriovenous Fistula | Original Article

Dural sinus malformation with giant pouch (DSMGP): symptoms and treatment

Authors: Flavio Requejo, Victoria Tcherbbis, Maria Laura Gonzalez, Romina Argañaraz, Juan Manuel Marelli, Beatriz Mantese

Published in: Child's Nervous System | Issue 2/2020

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Abstract

Objective

The aim of this study is to describe the clinical manifestations and treatment options of patients having dural sinus malformation with giant pouch (DSMGP) in a tertiary pediatric center.

Introduction

Dural sinus malformation with giant pouch (DSMGP) is a rare vascular malformation affecting fetuses, newborns, and infants. It is characterized by a dilated dural sinus frequently thrombosed with arteriovenous fistula (AVF) in its wall. There is a few information about symptoms, best treatment, and prognosis of the disease.

Material and methods

Medical charts of cases of DSMGP were retrospectively analyzed from January 2010 to January 2019. Our hospital is a pediatric tertiary center. An adult patient managed by the authors in another institution was added to the series.

Results

Eight pediatric patients from 0 to 9 months were managed, four were males. The adult patient was a 40-year-old male. Symptoms were mass effect in 4 pediatric cases. Exophthalmos was present in the pediatric case and adult case. Both cases had venolymphatic malformation of the orbit. Congestive heart failure (CHF), epistaxis and facial vein engorgement, and intracranial hemorrhage (ICH) were the symptoms in other 3 cases. A child has spontaneous resolution of the disease.

Discussion

Transverse sinus and superior sagittal sinus are affected more commonly. Patients with totally thrombosed pouch had mass effect symptoms. These cases were managed by surgical excision. When AVFs are present, clinical manifestations were secondary to cerebral venous hypertension or cardiac overload. If cavernous sinus drained the shunt (capture), epistaxis and facial veins engorgement could be present. AVFs are amenable to embolization, achieving the control of venous hypertension in most cases. Cavernous malformation could be present and must be controlled because its enlargement could be a sign of uncontrolled venous hypertension. On the other hand, DSMGP can be accompanied by venolymphatic malformation conforming a cerebral venous metameric syndrome.
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Literature
1.
go back to reference Agid R, Terbrugge KG (2007) Cerebrofacial venous metameric syndrome 2 plus 3: facial and cerebral manifestations. Interv Neuroradiol 13:55–58CrossRef Agid R, Terbrugge KG (2007) Cerebrofacial venous metameric syndrome 2 plus 3: facial and cerebral manifestations. Interv Neuroradiol 13:55–58CrossRef
2.
go back to reference Barbosa M, Mahadevan J, Weon YC, Yoshida Y, Ozanne A, Rodesch G, Alvarez H, Lasjaunias P (2003) Dural sinus malformations (DSM) with giant lakes, in neonates and infants. Review of 30 consecutive cases. Interv Neuroradiol 9:407–424CrossRef Barbosa M, Mahadevan J, Weon YC, Yoshida Y, Ozanne A, Rodesch G, Alvarez H, Lasjaunias P (2003) Dural sinus malformations (DSM) with giant lakes, in neonates and infants. Review of 30 consecutive cases. Interv Neuroradiol 9:407–424CrossRef
4.
go back to reference Fluncker S (2005) Malformation des sinus duraux. Diagnostic anténatalet devenir. Les Malformations congénitales. Diagnostic anténatal et devenir. Tome 3. Sauramps Médical, Montpellier Fluncker S (2005) Malformation des sinus duraux. Diagnostic anténatalet devenir. Les Malformations congénitales. Diagnostic anténatal et devenir. Tome 3. Sauramps Médical, Montpellier
7.
go back to reference Komiyama M, Ishiguro T, Kitano S et al (2004) Serial antenatal sonographic observation of cerebral dural sinus malformation. Am J Neuroradiol 25:1446–1448PubMed Komiyama M, Ishiguro T, Kitano S et al (2004) Serial antenatal sonographic observation of cerebral dural sinus malformation. Am J Neuroradiol 25:1446–1448PubMed
8.
go back to reference Komiyama M, Matsusaka Y, Ishiguro T, Kitano S, Sakamoto H (2004) Endovascular treatment of dural sinus malformation with arteriovenous shunt in a low birth weight neonate. Neurol Med Chir (Tokyo) 44:655–659CrossRef Komiyama M, Matsusaka Y, Ishiguro T, Kitano S, Sakamoto H (2004) Endovascular treatment of dural sinus malformation with arteriovenous shunt in a low birth weight neonate. Neurol Med Chir (Tokyo) 44:655–659CrossRef
11.
go back to reference Lasjaunias P, Brugge K, Berenstein A (2006) Clinical and interventional aspects in children. In: Surgical Neuroangiography, vol 3, 2nd edn. Springer, Berlin, p 39, 41, 47, 396-434, 478–502CrossRef Lasjaunias P, Brugge K, Berenstein A (2006) Clinical and interventional aspects in children. In: Surgical Neuroangiography, vol 3, 2nd edn. Springer, Berlin, p 39, 41, 47, 396-434, 478–502CrossRef
14.
go back to reference Merzoug V, Flunker S, Drissi C, Eurin D, Grangé G, Garel C, Richter B, Geissler F, Couture A, Adamsbaum C (2008) Multicentric study of the GRRIF (Groupe de Recherche Radiopédiatrique en Imagerie Foetale), part of SFIPP (Société Francophone d'Imagerie Pédiatrique et Prénatale). Dural sinus malformation (DSM) in fetuses. Diagnostic value of prenatal MRI and follow-up. Eur Radiol 18(4):692–699. https://doi.org/10.1007/s00330-007-0783-y CrossRefPubMed Merzoug V, Flunker S, Drissi C, Eurin D, Grangé G, Garel C, Richter B, Geissler F, Couture A, Adamsbaum C (2008) Multicentric study of the GRRIF (Groupe de Recherche Radiopédiatrique en Imagerie Foetale), part of SFIPP (Société Francophone d'Imagerie Pédiatrique et Prénatale). Dural sinus malformation (DSM) in fetuses. Diagnostic value of prenatal MRI and follow-up. Eur Radiol 18(4):692–699. https://​doi.​org/​10.​1007/​s00330-007-0783-y CrossRefPubMed
17.
go back to reference Okudera T, Pen Huang Y et al (1996) Developmental radiology of the posterior fossa dural sinuses in the human fetus: with special references to physiological enlargement of transverse and occipital sinuses, formation of emissary veins and development of superior jugular bulb from jugular sinuses. In: Hakuba A (ed) Surgery of the intracranial venous system. Springer, Berlin, pp 192–203 Okudera T, Pen Huang Y et al (1996) Developmental radiology of the posterior fossa dural sinuses in the human fetus: with special references to physiological enlargement of transverse and occipital sinuses, formation of emissary veins and development of superior jugular bulb from jugular sinuses. In: Hakuba A (ed) Surgery of the intracranial venous system. Springer, Berlin, pp 192–203
Metadata
Title
Dural sinus malformation with giant pouch (DSMGP): symptoms and treatment
Authors
Flavio Requejo
Victoria Tcherbbis
Maria Laura Gonzalez
Romina Argañaraz
Juan Manuel Marelli
Beatriz Mantese
Publication date
01-02-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 2/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04338-2

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