Sphenopalatine Artery Ligation for Life-Threatening Epistaxis in a 4-Year-Old Child With Glanzmann Thrombasthenia

Glanzmann thrombasthenia (GT) is a rare autosomal recessive platelet disorder affecting chromosome 17. The underlying defect lies in the altered quality and quantity of the glycoprotein IIb-IIIa receptor complex (also called integrin IIbβ3), which leads to impaired platelet aggregation. ¹ Glanzmann thrombasthenia is characterized by mucocutaneous bleeding in children manifesting as purpura, gingival bleeding, and epistaxis commonly requiring intervention. ²

In children with a history suggestive of GT, the biochemical diagnosis is made by a combination of normal platelet count and prolonged platelet factor assay. Failure of platelets to aggregate with agonists during light transmission aggregometry is the gold standard diagnostic tool, though it is limited to experienced laboratories. ³

We describe a multidisciplinary approach to GT and the use of sphenopalatine artery (SPA) ligation to treat intractable epistaxis. A 4-year-old Filipino girl was diagnosed with GT at 1 year of age. There was no family history of bleeding disorders or blood dyscrasias. Over the course of 1 year, she had recurrent epistaxis necessitated 6 emergency department (ED) presentations and nasal packing. On 2 occasions, she was admitted to intensive care unit (ICU) for hemodynamic instability and required multidisciplinary management from Emergency, ICU, Paediatrics, Haematology and Ear, Nose and Throat Units at the Royal Darwin Hospital, Northern Territory, Australia. Her clinical course over the last few admissions was complicated by febrile reactions to platelet transfusions with associated wheezing. She was identified to have class I and II human leukocyte antigen (HLA) alloantibodies with a negative screen for human platelet antigen antibodies as assessed by platelet immunofluorescence test. Her hemostasis was managed on subsequent admissions with recombinant activated factor VII (VIIa, NovoSeven, Baulkham Hills, NSW, Australia) at a dose of 90 μg/kg repeated every 8 hours with concurrent tranexamic acid.

In October 2017, she presented to ED with right-sided epistaxis, hypovolemic shock. Intraoral examination showed a large clot in the oropharynx with surrounding bright red ooze. Full blood count revealed a hemoglobin of 61 g/L. She was packed with a MeroPack nasal packing presoaked in tranexamic acid.

Given the recurrent nature and severity of her latest presentation, multidisciplinary input was sought from hematology, ICU, pediatrics, general surgery, and ear, nose and throat (ENT) review.

Fluid resuscitation was commenced in parallel with administration of a tranexamic acid 250 mg intravenous (IV) infusion and Novoseven RT/recombinant factor VIIa 1 mg IV.

Despite medical management, the epistaxis did not abate. Following consideration of her complicated and recurrent epistaxis, we proceeded with a nasal endoscopic right SPA identification and subsequent ligation (Figures 1 and 2).

Figure 1.

Right sphenopalatine (yellow arrow) identified and clipped.

Figure 2.

Right sphenopalatine (yellow arrow) identification with a nasal endoscopic approach.

On advice of hematology, her perioperative state was optimized by a further NovoSeven 2 mg IV infusion (90 μg/kg), tranexamic acid 250 mg IV every 6 to 8 hours, and 2 units of packed red cells.

Regular 3 monthly follow-up including outpatient clinic appointments, hemoglobin and platelet levels, and nasal endoscopy has been done for 2 years, and no evidence of further hemorrhage has been found.

Epistaxis is one of the most common manifestations in patients with GT having a frequency as high as 73% as per previously published case series, ¹ and much of the available literature is centered on medical and interventional methods to control this problem, largely by nasal packing. ⁴ Surgical management, however, has not been reported due to the rarity of the condition and the risks involved. ⁵

Performing emergency surgery in an unstable patient with ongoing bleeding can be unpredictable. ⁶ Hence, the perioperative optimization is vital. In our case, we elected to use recombinant factor VIIa (NovoSeven). 7 In conjunction with tranexamic acid, an antifibrinolytic agent, and blood products, the patient’s circulatory state can be stabilized prior to surgery. ⁸ This is of particular importance in sinonasal surgery, as clear endoscopic visualization is partly achieved through hypotensive bradycardic anesthesia. ⁹

In conclusion, endoscopic SPA ligation in GT children with intractable epistaxis is a safe and effective procedure. Adequate perioperative optimization and multidisciplinary approach is essential.