[size=20px]A five-year old boy has classic hemophilia A. The parents requests a circumcision.

 

Which of the following is true reagrding procedures in patients with hemophilia A?

 

A with preoperative correction of factor VIII levels, same day discharge is possible

 

B preoperative desmopressin will usually correct the coagulation profile

 

C preoperative factor VIII levels should be greater than 60%

 

D fibrin glue is thrombolytic in this scenario

 

E a factor VIII level of 50% on postoperative day five is acceptable[/size]

(Untitled)

 

d

think again my dear

(Untitled)

 

e

e

correct

 

Guidelines for the perioperative management of children with hemophilia are available but there is a paucity of data. The World Federation of Hemophilia (WFH) recommends maintaining a preoperative factor VIII level of 80 to 100% of normal. For minor procedures ’adequate’ postoperative levels are recommended for five to seven days or until wound healing occurs. For major surgery, 10 to 14 days of postoperative control are necessary. The WFH recommends maintaining a factor VIII level of 60 to 80% for postoperative days one through three; 40 to 60% for days four through six and 30 to 50% for days seven to 14.

 

One “rule of thumb” is that each IU (international unit) of factor VIII or IX per kg of body weight will raise the levels by two percent. With adequate control of factor VIII levels safe circumcision is possible in most cases.

 

Even for minor procedures such as circumcision, normalization of preoperative factor VIII levels and several days of postoperative hospitalization are usually necessary. One small study of circumcision in this patient population used fibrin glue with excellent results.

 

Desmopressin is used as an adjunct in patients with mild hemophilia in order to minimize exposure to factor VIII and the development of inhibitors.