Hereditary telangiectasia with massive nasal bleeding

The patient, a 50-year-old man, a native of India, entered the emergency room with severe nosebleeds.In the history and history of the disease, repeated nasal bleeding with a characteristic family history of the disease and telangiectasia on the nasal mucosa and paranasal cavities were found. Earlier he was diagnosed with hereditary hemorrhagic telangiectasia in connection with the presence of vascular malformations in the nose and sinuses found during endoscopy. After admission, his blood pressure was 90/60 mm Hg. the pulse 124 beats per minute, the arterial blood saturation of 96% oxygen. Laboratory examinations: hemoglobin 6.5 g / dl, hematocrit 16 g / dL. Auscultation of the chest revealed vesicular breathing from both sides, without crepitus. Start therapy: 500 ml of hydroxyethyl starch, followed by 3 units of erythrocyte mass and 4 units of fresh frozen plasma. The patient received a slow intravenous infusion of tranexamic acid to control bleeding, glycopyrrolate to reduce glandular secretion, pantoprazole as a prophylaxis of acute gastric ulcers and ondansetron to reduce reflux.

In parallel with the resuscitation, a surgical examination with bleeding control under general anesthesia was planned.Since the presence of blood in the stomach was possible, and when the patient ate it was unknown, an initial anesthesia was carried out with thiopental 5 mg / kg and succinylcholine 2 mg / kg. Intubation of the trachea was carried out, a urinary catheter was inserted, as well as an aspirate with an orogastric probe. Anesthesia was maintained by sevoflurane, nitric oxide and oxygen, as well as atracurium as a muscle relaxant.

Several telangiectasies were found in the anterior part of the nasal septum, which were completely burned by diathermocoagulation under the control of endoscopy. Next, a nasal septum dermatoplasty was performed to stop bleeding. Intraoperatively was applied tranexamic acid (1 g) in parallel with the local use of lignocaine and adrenaline. During the operation, controlled hypotension was provided with sevoflurane and propofol in bolus injections to maintain a systolic pressure of 90-100 mm Hg. Art. The postoperative period passed without any peculiarities.

Conclusions: Patients with Rundu-Weber-Osler disease or hereditary hemorrhagic telangiectasia can manifest withuncontrolled bleeding. Resuscitative measures along with stopping bleeding are the basis of treatment. Treatment includes blood transfusion, the use of antifibrinolytic drugs and surgical hemostasis.