--- a +++ b/processing/MACCROBAT/26714786.txt @@ -0,0 +1,29 @@ +A 64 year old female known for HHT is referred to our clinic for recurrent epistaxis for nearly 50 years. +She has had recurrent symptoms since the age of 16 when her condition was diagnosed. +All three of her siblings also were diagnosed with HHT and her mother passed away from an intracranial hemorrhage. +Eight years prior to presentation she had undergone a left-sided septodermoplasty via a lateral rhinotomy approach. +This operation had significantly reduced the frequency of her symptoms and for several years her epistaxis was under control with the use of low dose thalidomide. +However, she was referred to our clinic due to increased epistaxis severity and frequency over the prior 12 months necessitating more frequent transfusions. +At the time of consultation the patient was concerned about daily severe left sided epistaxis despite several months use of topical bevacizumab and oral tranexamic acid. +She required intravenous iron and blood transfusions every two months. +Her baseline hemoglobin at the time of our consultation was 75 g/L (normal = 120 – 160 g/L). +Her HHT epistaxis severity score [17] was severe (normalized score 9.49). +On examination, she had multiple telangiectasia on her fingers, face, lips and palate. +Her endoscopic examination revealed bilateral telangiectasia along the nasal septum. +There was extensive crusting along the entire left nasal cavity with which any manipulation resulted in immediate profuse epistaxis. +Given the severity of the patient’s epistaxis despite medical therapy, she was offered endoscopic left-sided septodermoplasty. +The surgical goals were to improve her quality of life by reducing the number and severity of epistaxis episodes while diminishing the need for blood transfusions. +The patient was content with the treatment plan and agreed to undergo surgical intervention. +The endoscopic procedure was performed under general anesthesia with endotracheal intubation. +The nasal cavity was prepared by inserting pledgets soaked in topical adrenaline (1:1000) placed in both nostrils for decongestion. +Using a zero degree endoscope the residual STSG and mucosa of the left septum was dissected in a supraperichondrial plane that resulted in the expected significant diffuse hemorrhage. +Immediate hemostasis was attained using a topical gelatin-thrombin matrix, Floseal (FloSeal Hemostatic Matrix; Baxter Healthcare Corporation, Deerfield, IL, USA) (Fig.1). +The mucosal defect (Fig.2) measured approximately 3 cm in anterior-posterior dimension. +A 4 x 2 cm split thickness skin graft was harvested from the right thigh, pie-crusted with a 15 blade and then placed endoscopically along the length of the septal defect. +As seen in Fig.3, the graft was placed with an overlap of the mucosa of the nasal floor and the residual superior septal mucosa. +Finally, 2 mL of fibrin sealant (TISSEEL fibrin sealant, Baxter Healthcare Corporation, Deerfield, IL, USA) was then applied first to the edges then central portion of the STSG (Fig.4). +Packing was not used post-operatively and the patient was discharged home on the same day of surgery. +Clinical follow-up two weeks after surgery (Fig.5) showed that the entire graft had taken and the left-sided epistaxis had dramatically diminished. +The patient was very content with the results of the procedure. +At 6 months follow-up, her baseline hemoglobin had improved to 102 g/L and she was requiring transfusions every 4 months with her hematologist’s intent to stop the transfusions if her hemoglobin remained greater than 100 g/L. +Her epistaxis severity score at 6 month follow up was mild (normalized score 3.05).