Feminizing Genitoplasty in a young girl with Glanzmann's thrombasthenia-management of haemostasis

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...

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Bibliographic Details
Published in:Journal of the Pakistan Medical Association 2020-06, Vol.70 (6), p.1084
Main Authors: Turk, Kashaf Khalid, Nazir, Zafar
Format: Article
Language:English
Subjects:
Care and treatment
Case studies
Child
Complications and side effects
Dosage and administration
Factor VII
Female
Female genital diseases
Gynecological surgery
Hemorrhage
Hemostasis
Humans
Pediatric research
Platelet Transfusion
Postoperative Hemorrhage
Prevention
Recombinant Proteins
Risk factors
Thrombasthenia
Thrombasthenia - complications
Thrombasthenia - therapy
Tranexamic acid
Urogenital abnormalities
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Summary: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.
ISSN:0030-9982
DOI:10.5455/JPMA.34911