Congenital
factor VII deficiency is an autosomal recessive serious disorder of blood coagulation with wide genotypic and phenotypic variations. The clinical presentation can vary from asymptomatic patients to patients with major bleedings in severe
deficiency (factor VII <1%). Investigations show prolonged PT and low
factor VII. Treatment modalities include FFP and repeated recombinant
factor VII infusions. We hereby report the first successful LRLT for
factor VII deficiency in an infant, the first-ever youngest baby reported worldwide. A six-month-old male child presented with easy bruisability, ecchymotic patches,
hematuria, and convulsions. CT of the head showed
subdural hemorrhage, which was treated conservatively. He had markedly increased PT (120 s) with normal platelets, and aPTT with
factor VII level <1%. Despite the treatment by
rFVIIa administration weekly, which was very expensive, he still had repeated life-threatening
bleeding episodes. LRLT was performed with mother as the donor, whose
factor VII level was 57%.
A factor VII infusion plan for pre-, intra- and postoperative periods was formulated and TEG followed. Postoperatively, his
factor VII started increasing from third day and was 38% on 24th day with PT <14 s. He had uneventful intraoperative and postoperative courses. LT is a safe and definite cure for
factor VII deficiency.