Hereditary Hemorrhagic Telangiectasia & Surgical Bleeding | 29.07.22
Research Inquiry
26 years old white female, history of Hereditary Hemorrhagic Telangiectasia (Osler -Weber -Randu), made by ears, nose and throat physician, but no genetic confirmation. History of multiple nose bleeds, but no Iron Deficiency Anemia (IDA), and normal coags. The patient had a traumatic ankle fracture, and she needs ankle surgery. The patient took NSAIDs in the past without increased bleed.
This report will answer the following inquiries:
- What are her chances for increased surgical bleed?
- What is the treatment if she has increased surgical bleed
- Can she take Aspirin prophylaxis post-op, to prevent DVT?
Research Findings
What are her chances for increased surgical bleed?
Our meta-search in multiple database platforms didn’t find enough data about the exact chances of general post-operative bleeding related to Hereditary Hemorrhagic Telangiectasia.
The limited findings are also reflected in the Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia, published in Annals of Internal Medicine in 2020 [1], and in The European Rare Disease Network for HHT Frameworks for management of hereditary haemorrhagic telangiectasia in general and speciality care, published in European Journal of Medical Genetics in 2022 [2] – both didn’t mention any special considerations regarding surgical risk.
Other risk management was almost identical to non-HHT patients, except for nasal manipulations like nasal intubation, which can trigger severe episodes of epistaxis linked to mucous telangiectases. If the patient’s clinical condition requires the insertion of a nasogastric tube, it should be a soft, small diameter (unless clinical circumstances demand a large bore tube), and put in place with extreme caution.
What is the treatment if she has increased surgical bleed?
There was insufficient data in the literature about the acute management of general post-operative bleeding related to Hereditary Hemorrhagic Telangiectasia, so we included data related to the management of HHT-related epistaxis, which is relevant to our patient clinical manifestation.
An article published in the Journal of Anesthesia in 2013 [3] reviewed the management of patients with hereditary hemorrhagic telangiectasia undergoing general anesthesia, using a cohort of 74 patients with hereditary hemorrhagic telangiectasia who underwent 163 surgeries, and most had high levels of disease burden. In addition, most surgeries in the cohort were directly related to treating conditions associated with HHT, with the majority being procedures to the nasal mucosa for recurrent epistaxis, and only 3 patients had Orthopedic surgery for septic arthritis. In the procedures for epistaxis, which is the main manifestation of our patient, 11 units of packed red cells were used, and some patients were also treated with topical phenylephrine.
An article, published in the Journal of Otolaryngology-Head & Neck Surgery in 2016[4], reviewed the evidence-based methods of surgical management of Hereditary Hemorrhagic Telangiectasia, using a case study of a 37-year-old male, presented with recurrent epistaxis and Iron Deficiency Anemia. For the emergency management of the patient, he was stabilized with blood transfusions, and later on, he was offered to undergo a Septodermoplasty procedure. Bevacizumab was also offered as a potential treatment.
The use of blood transfusions for stabilization of Hereditary Hemorrhagic Telangiectasia acute bleeding is also advised in an article [5] that was published in Blood journal in 2021, which reviewed the latest HHT systemic therapies, guidelines, and standard of care.
An article published in SAGE Otolaryngology-Head and Neck Surgery in 2020 [6], reviewed the Clinical Practice Guideline of Epistaxis. The main suggested management algorithm:
Can she take Aspirin prophylaxis post-operation, to prevent DVT?
The guidelines mentioned above [1,2] recommend that Hereditary Hemorrhagic Telangiectasia patients receive anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with consideration of their individualized bleeding risks. It also mentions that bleeding in HHT is not an absolute contraindication for these therapies.
An article published in the New England Journal of Medicine, published in 2013 [7], presented the results of an Antiplatelet and Anticoagulant Agents usage online survey that was sent to 973 confirmed Hereditary Hemorrhagic Telangiectasia patients. According to the results, 65% of the patients who were treated with 300mg of Aspirin had their nosebleeds worsen and 35% stayed in the same status. In addition, 94.9% had no other hemorrhages except for their nosebleed.
Conclusion
Although the literature and guidelines are suggesting that Hereditary Hemorrhagic Telangiectasia is not a contra-indication for antiplatelet usage and other required medical procedures, every patient must be evaluated closely with clinical and laboratory tools. This approach will be making sure that the patient is not suffering from severe HHT complications, as mentioned in an article that was published in Haematologica in 20188, like Anemia, Pulmonary Arteriovenous Malformations, Liver Arteriovenous Malformations, Gastrointestinal bleeding, and Central nervous system manifestations, that can elevate the bleeding risk of the patient. In addition, blood transfusions are advised if there is a major bleeding complication.
References
https://pubmed.ncbi.nlm.nih.gov
https://www.sciencedirect.com
https://www.ncbi.nlm.nih.gov
https://journalotohns.biomedcentral.com
https://ashpublications.org
https://journals.sagepub.com
https://www.nejm.org
https://www.ncbi.nlm.nih.gov
