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2017 ; 17
(2
): 157-161
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Diagnosis and Treatment of Hereditary Hemorrhagic Telangiectasia
#MMPMID28638289
Grigg C
; Anderson D
; Earnshaw J
Ochsner J
2017[Sum]; 17
(2
): 157-161
PMID28638289
show ga
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic
disorder known for its debilitating symptoms. More than 90% of patients with HHT
experience epistaxis, and they average up to 18 bleeds per month. We review the
current literature on the pathophysiology, clinical presentation, and management
of HHT. METHODS: We searched MEDLINE, EMBASE, and PubMed and identified 19
articles published since 2000 with current information on HHT. RESULTS: HHT is a
disease more commonly associated with significant morbidity rather than
mortality. The morbidity of the disease and decreased quality of life are the
result of the recurrent and potentially severe epistaxis that the majority of
patients with HHT experience. During active epistaxis, the effective emergency
techniques of locally applied pressure, nasal packing anteriorly and/or
posteriorly, and cauterization are effective. Medical treatment with antiestrogen
therapy has shown promising results, but further research is needed to determine
the long-term side effects and the limitations of lifelong therapy. Research
directed toward bleeding reduction and prevention has yet to have a breakthrough.
Although initial reports suggest that intranasal bevacizumab is an effective
agent, further research is required. CONCLUSION: Interventional treatments in
life-threatening and/or severe circumstances will continue to be used because of
their effectiveness. Research into the pathophysiology of HHT has led to the
development of potential therapies that prevent and decrease the severity of
epistaxis, but the current evidence is insufficient to ascertain best practice.
At present, appropriate management of acute epistaxis coupled with early
diagnosis and referral to an ear, nose, and throat specialist should be the
mainstay of treatment.
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