We report peri- and post-operative management of haemostasis in a 11-year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post-operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting.
JPMA. The Journal of the Pakistan Medical Association
Turk, K. K.,
(2020). Feminizing genitoplasty in a young girl with glanzmann's thrombasthenia-management of haemostasis. JPMA. The Journal of the Pakistan Medical Association, 70(6), 1084-1085.
Available at: https://ecommons.aku.edu/pakistan_fhs_mc_surg_paediatr/51