Abstract
A patient with hereditary hemorrhagic telangiectasia is a rare presentation to the otolaryngologist in clinical practice. They almost present with epistaxis, which is recurrent, spontaneous and can be functionally and socially debilitating for the patient. Diode laser cauterization and Avastin intranasal injection showed significant improvement in epistaxis severity score. We report a case of hereditary hemorrhagic telangiectasia with chronic epistaxis (hemoglobin 3.4 mg/dl) managed with Diode laser and intranasal Avastin injection with great improvement in life quality and decrease in epistaxis attacks. Despite all treatment options absolute eradication of epistaxis attacks is difficult to obtain in these cases.
1
Introduction
Hereditary hemorrhagic telangiectasia or Osler–Weber–Rendu syndrome is a rare autosomal dominant genetic disease, which gives malformation of vascular tissues and makes the vessel walls more liable to spontaneous ruptures and injuries. It is a multiple systemic disease with recurrent epistaxis, cutaneous telangiectasia and visceral arteriovenous malformations . Epistaxis is the first clinical symptom of the disease in 80% of cases . Usually it is recurrent, spontaneous, or easily provoked and can be functionally and socially debilitating for the patient . There are many treatment modalities to treat chronic epistaxis. Recent studies showed that Diode laser and intranasal Avastin have significant improvement in epistaxis severity score with minimal side effects and can be repeated as the severity of the bleeding requires . We managed this case using diode laser and Avastin intranasal injection, which give great control of epistaxis attacks and significant improve in life quality in the long-term.
2
Case Presentation
A 73-year-old male with a known case of hereditary hemorrhagic telangiectasia presented to the emergency department with active epistaxis that started 4 hours prior to presentation.
Also associated with severe dizziness and shortness of breath increased with exertion, with history of recurrent spontaneous epistaxis on and off (3 to 4 times per day with moderate amount of blood) and chronic melena. There was no history of hematemesis or hemoptysis. Otherwise systemic review is unremarkable. Past medical history includes recurrent blood transfusion for chronic anemia.
On physical examination patient was conscious in distress with tachypnea, heart rate was 90 beats per min and oxygen saturation was 92%. General examination showed pale conjunctiva and multiple telangiectasia lesions. After applying intranasal decongestant bleeding stop, flexible laryngoscope showed multiple telangiectasia lesions in nasal cavity and supralaryngeal region with nasal septal perforation. Also, oral examination showed multiple telangiectasia lesions on the lips, hard palate, tongue and floor of the mouth ( Figs. 1, 2, 3 and 4 ).
Complete blood count showed hemoglobin 3.4 mg/dl with iron deficiency profile. Electrolyte, liver function test and creatinine were in the normal level. Electrocardiogram showed normal sinus rhythm. Urgent cross and match was done and 2 U of PRBC was transfused and the patient stabilized.
Diagnosis of chronic epistaxis had been made. After full pre-operation investigation and preparation, the patient had been taken to operation room for Diode laser (810 nm units) cauterization guided with rigid nasal endoscopy under general anesthesia and intranasal sub-mucosal Avastin (total 100 mg) injections to control bleeding sources. Post operation, the patient tolerated the procedure without any complication and hemoglobin reached 7.8 mg/dl. After two days patient was stable and ready for discharge with no attacks of epistaxis. So, patient was discharged from hospital with iron supplementation, nasal ointment, and educated about how to avoid epistaxis and follow up with ENT clinic. After 10 months patient hemoglobin reached 9.3 mg/dl with great improvement in patient quality of life and decrease in frequencies of epistaxis attacks.
2
Case Presentation
A 73-year-old male with a known case of hereditary hemorrhagic telangiectasia presented to the emergency department with active epistaxis that started 4 hours prior to presentation.
Also associated with severe dizziness and shortness of breath increased with exertion, with history of recurrent spontaneous epistaxis on and off (3 to 4 times per day with moderate amount of blood) and chronic melena. There was no history of hematemesis or hemoptysis. Otherwise systemic review is unremarkable. Past medical history includes recurrent blood transfusion for chronic anemia.
On physical examination patient was conscious in distress with tachypnea, heart rate was 90 beats per min and oxygen saturation was 92%. General examination showed pale conjunctiva and multiple telangiectasia lesions. After applying intranasal decongestant bleeding stop, flexible laryngoscope showed multiple telangiectasia lesions in nasal cavity and supralaryngeal region with nasal septal perforation. Also, oral examination showed multiple telangiectasia lesions on the lips, hard palate, tongue and floor of the mouth ( Figs. 1, 2, 3 and 4 ).
