Managing incidentally diagnosed isolated factor VII deficiency perioperatively: a brief expert consensus report.

While isolated factor VII (FVII) deficiency is being more frequently diagnosed owing to improved preoperative screening procedures, there is no specific guideline for perioperative management of such patients. To complicate the issue, FVII activity levels seem to correlate less well with the risk of hemorrhage than the patient's past and family bleeding history do. We have devised expert consensus recommendations for managing such patients perioperatively, taking into consideration the personal and family bleeding history, the FVII activity level and the inherent bleeding risk of the procedure itself. We hope that clinicians will find this a useful tool in the decision-making process, thereby limiting the use of recombinant factor VIIa to those who need it most, and preventing possible thrombotic complications in those without a strong indication for its use.

Splenic rupture in a newborn with severe hemophilia--case report and review.

SUMMARY: Life-threatening splenic rupture is rare in neonates with severe hemophilia. There are only 3 cases of splenic rupture in neonates with hemophilia reported in the literature. We present the case of an infant, born to a hemophilia A carrier mother. The infant was asymptomatic until discharge at 48 hours of age, but presented on the third day of life with shock, abdominal distension, and severe anemia. Computed tomography of the abdomen confirmed the diagnosis of splenic rupture with hemoperitoneum. The infant recovered after extensive supportive care surgery and factor replacement.

Reversible posterior leukoencephalopathy syndrome in a child treated with bevacizumab.

Bevacizumab is a monoclonal antibody targeting vascular endothelial growth factor (VEGF). Hypertension is a well-recognized, common side effect of VEGF blocking agents. The reversible posterior leukoencephalopathy syndrome (RPLS) has been described as a rare but serious consequence of bevacizumab administration. We present a case of a 6-year-old child with refractory hepatoblastoma who developed hypertensive crisis, seizures and MRI changes consistent with RPLS while receiving bevacizumab with gemcitabine and oxaliplatin. Findings completely resolved without neurologic sequelae with stringent blood-pressure control. Better understanding of risk for RPLS, prompt recognition and aggressive management will be required as bevacizumab gains wider use in pediatrics.