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The Management of Craniopharyngioma in a Child with Severe Factor XI Deficiency

1Istanbul University, Istanbul School of Medicine, Division of Pediatric Hematology&Oncology, ISTANBUL, Istanbul, Turkey, 2Istanbul University, Istanbul School of Medicine, Division of Pediatric Hematology&Oncology, Istanbul, Istanbul, Turkey, 3Istanbul University, Istanbul School of Medicine, Department of Neurosurgery, Istanbul, Istanbul, Turkey, 4Istanbul University, Istanbul School of Medicine, Division of Intensive Care Unit, Istanbul, Istanbul, Turkey, 5Istanbul University, Istanbul School of Medicine, Division of Pediatric Intensive Care Unit, Istanbul, Istanbul, Turkey

Abstract Number: PB0718

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders

Background: The bleeding phenotype in patients with severe factor XI deficiency (SFXID) is not correlated with FXI levels.Therefore,there is no standard protocol for perioperative management.

Aims: We present the management of a child with SFXID and diabetes insipidus (DI) who underwent surgery for craniopharyngioma.To our best knowledge,this is the first report of neurosurgery of SFXID.

Methods: A 12-year-old boy presented with headaches.He had a history of surgery for craniopharyngioma with residual mass,4 years ago.We were unable to obtain the hospital records despite our best efforts,but the family reported uneventfully(inspite of 1-month stay in ICU).The patient had been diagnosed as SFXID during that admission.Radiotherapy had been given,also.

Results: The patient had headaches and recurrent vomiting, 4years later.The suprasellar mass was increased in size,surgery was planned.FXI inhibitor was negative,FXI level was 0.6%.He had no bleeding history.Tranexamic acid(TXA) was given orally,one day before surgery. FXI concentrate is not available in Turkey.Therefore,FFPs was started for prophylactic replacement the day before surgery.FFPs were administered with close follow-up due to DI,desmopressin doses were adjusted. One additional FFP was given immediately before surgery.The desired FXI level immediately before surgery was achieved.In addition,TXA was administered intravenously.Intraoperative bleeding was within normal range.However, one unit of FFP was administered at the surgeon’s recommendation.Moreover,rVIIa(1 mg) was administered at the end of surgery due to superficial mildly increased bleeding during suturing.FXI levels were maintained above 45% with FFPs for 10 days(&TXA).No bleeding or thrombosis was observed.Postoperative cranial MRI were performed twice,revealed a near-total excision of the tumor and no bleeding.

Conclusion(s): Perioperative management and effective hemostasis is very challenging in patients with severe FXI deficiency.If FXI concentrate is not available,effective hemostasis can be achieved by using FFP and TXA.If the patient has excessive bleeding in spite of these interventions,one dose of rFVIIa (with consent for off-label use) may help control bleeding, but this matter warrants further research.

To cite this abstract in AMA style:

UNUVAR A, KARAMAN S, KULELI O, SENCER A, DEMIRKOL D, TANYILDIZ G, BILICI M, GENCAY G, KARAKAS Z, TUGCU D. The Management of Craniopharyngioma in a Child with Severe Factor XI Deficiency [abstract]. https://abstracts.isth.org/abstract/the-management-of-craniopharyngioma-in-a-child-with-severe-factor-xi-deficiency/. Accessed September 22, 2023.

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