Epistaxis in hereditary hemorrhagic telangiectasia

Case: 22 year old female with a past medical history of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is in ICU with urosepsis and vasodilatory shock. She developed epistaxis on the third day of the admission. Your next line of management? (select one)

A) Observe with nasal packing as it usually subsides spontaneously
B) Call ENT service as it may be a serious issue


 Answer: B

Epistaxis is the most common presenting symptom among younger patients with hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease. Bleeding in these patients can be hard to control. In the setting of high risks like sepsis and DIC, it would be prudent to get ENT service early in the course. The paradox of treatment in these patients may take bedside clinicians by surprise as the friable lesions may appear to bleed more with treatment than without! and expert opinion may be needed.

Epistaxis can be provoked by a variety of factors, besides acute sicknesses. It includes changes in external temperature, humidity, activity, change in diet, insertion of nasogastric tubes, and posture. Gushing bleed is usually a sign of life-threatening arterial bleed.

#ENT
#hematology


References:

1. Shah RK, Dhingra JK, Shapshay SM. Hereditary hemorrhagic telangiectasia: a review of 76 cases. Laryngoscope 2002; 112:767.

2. Fuchizaki U, Miyamori H, Kitagawa S, et al. Hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). Lancet 2003; 362:1490.

3. Hoag JB, Terry P, Mitchell S, et al. An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope 2010; 120:838.