Behçet's disease and cerebral sinus vein thrombosis in children: a case study and review of the literature.
OBJECTIVE
Central nervous system (CNS) involvement, one of the most severe manifestations of Behçet's disease (BD), is uncommon in children. Because it is rare, the clinical features of this disease in children are not well characterised. Here we describe a teenager with BD which was disclosed following an episode of cerebral sinus vein thrombosis (CSVT) and review the available literature on children with CSVT associated with BD.


METHODS
A 12-year-old boy who presented with CSVT is described and the relevant literature, based on a Medline search from 1966 to January 2015 is reviewed.


RESULTS
Twenty-three well-documented reports of children with CSVT and BD are described. This manifestation affected mainly males (61%) with a mean age of 12 years (range 4-18). BD was first diagnosed simultaneously or following CSVT in the majority of cases (75%). Multiple sinuses were involved in 30% of the cases. Thrombosis of additional large vessel was identified in 5 of the 23 children. The most common presenting symptom and signs were headache (91%), lasting more than 3 days in most cases (75%), followed by papilledema (43%), seizures (17%), and personality changes (9%). A mixed pattern of CNS involvement including both parenchymal involvement and CSVT, was demonstrated in only two patients (9%). Management of CSVT differed between reports.


CONCLUSIONS
CSVT in children is a rarely reported manifestation of BD and has a characteristic clinical picture of a teenage boy presenting with prolonged headache, with no previous diagnosis of BD. A therapeutic approach has not been established yet.
Department of Paediatrics, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel.
Amihai
A
Rottenstreich
Department of Paediatrics A, Edmond & Lily Safra Children's Hospital, Chaim Sheba Medical Center, Tel Hashomer, Israel.
Keren
K
Machol
Department of Paediatrics, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel.
Eli M
EM
Eisenstein
Department of Paediatrics A, Edmond & Lily Safra Children's Hospital, Chaim Sheba Medical Center, Tel Hashomer, Israel.
Shai
S
Padeh
Department of Paediatrics, Shaare Zedek Medical Centre, Jerusalem, Israel.
Aharon
A
Klar
Medicine F, Chaim Sheba Medical Center, Tel Hashomer, Israel and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Avi
A
Livneh
Department of Paediatrics, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel. berkun@hadassah.org.il.
Yackov
Y
Berkun
D000367:Age Factors; D000925:Anticoagulants / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D002648:Child; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011237:Predictive Value of Tests; D012307:Risk Factors; D012851:Sinus Thrombosis, Intracranial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D016896:Treatment Outcome
D000925:Anticoagulants; D007166:Immunosuppressive Agents