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

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.