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Dural sinus malformation with giant pouch (DSMGP): symptoms and treatment
Flavio Requejo1, Victoria Tcherbbis2, Maria Laura Gonzalez3
1Department of Interventional Radiology, Hospital Nacional de Pediatria JP Garrahan, Combate de los Pozos 1881, 1254, Buenos Aires, Argentina. frequejo@intramed.net.
Insights
Dural sinus malformation with giant pouch (DSMGP) is a rare condition in infants. Treatment varies from surgical excision to embolization, with some cases resolving spontaneously.
Area of Science:
- Vascular Malformations
- Pediatric Neurology
- Interventional Radiology
Background:
- Dural sinus malformation with giant pouch (DSMGP) is a rare vascular anomaly affecting infants.
- Characterized by a dilated, often thrombosed dural sinus with an arteriovenous fistula (AVF).
- Limited information exists regarding its clinical manifestations, optimal treatment, and prognosis.
Purpose of the Study:
- To describe the clinical features and treatment strategies for DSMGP.
- To analyze outcomes in pediatric patients managed at a tertiary care center.
Main Methods:
- Retrospective analysis of medical charts from January 2010 to January 2019.
- Inclusion of eight pediatric patients and one adult case.
- Review of clinical presentation, management, and outcomes.
Main Results:
- Commonly affects transverse and superior sagittal sinuses.
- Symptoms include mass effect, exophthalmos, congestive heart failure, epistaxis, and intracranial hemorrhage.
- Spontaneous resolution occurred in one pediatric case.
Conclusions:
- Management depends on whether the pouch is thrombosed or involves AVFs.
- Surgical excision is used for thrombosed pouches causing mass effect.
- Embolization is effective for AVFs, managing venous hypertension and cardiac overload.
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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