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).