Indications

Recurrent epistaxis due to telangiectasia

Hereditary hemorrhagic telangiectasia (Osler Weber Rendu)

• Prevalence of 1/10,000 in Caucasians
• Recurrent epistaxis affecting 50-90% of patients
  • VEGF has been found to be elevated in HHT patients
  • Recurrent and severe epistaxis is the most common presentation of HHT, frequently leading to severe anemia requiring intravenous iron and blood transfusions

Pre-op evaluation

HHT is diagnosed by the presence of epistaxis; telangiectasias on mucosal surfaces: lips, oral cavity, nose; visceral lesions such as gastrointestinal telangiectasia; pulmonary, hepatic or cerebral AVMs; and family history of HHT in first-degree relatives and genetic testing.

The presence of any three of these findings is required to fulfill the Curacao criteria.

• Medical history
  • Anemia, blood transfusions
  • Internal organ bleeding
• Family history
• Physical exam - nasal exam
• Genetic testing = HHT is an autosomal dominant gene
• Radiographic imaging to document telangiectasias and bleeding sites throughout the body - screening the lungs and brain
• Labs: Hgb/Hct

Consent: Endoscopic laser control of epistaxis (with Avastin)

• Intranasal injection of submucosal bevacizumab is an off-label therapy in conjunction with KTP laser

Equipment

• KTP laser (potassium titanyl phosphate)
  • Uses a 1064-nm Nd:YAG source passed through a KTP crystal to emit light that is frequency-doubled with a wavelength of 532 nm. This quasi-CW laser system uses nanosecond pulses to destroy vascular targets. The KTP laser's 532-nm wavelength corresponds with the 542-nm absorption peak of oxyhemoglobin, which makes it relatively specific for cutaneous blood vessels.
• Protective eyeware
• Nasal tray
• Bipolar cautery (Silverglide) and monopolar cautery available
• Nasal packing available
• Avastin (bevacizumab) injection (antiVEGF)
  • Bevacizumab is a recombinant, humanized, monoclonal antibody that binds to and inhibits the activity of VEGF, which then prevents angiogenesis
  • Simonds et al. reported that patients receiving submucosal injections of bevacizumab at the time of laser treatment had less bleeding, required fewer blood transfusions, and had a greater improvement in their social life than those patients that underwent treatment with laser alone.

Anesthesia

• General anesthesia
• Can perform awake, but must be able to protect the airway and control hemorrhage

Disposition

• Outpatient procedure
• Lasering controls epistaxis, but does not cure it. 
• The laser treatment is normally repeated depending on the patient
  • Retreat for significant frequent recurrent epistaxis

Procedure

Case prep: Strip end of KTP wire  to expose laser fiber.

Patient brought back to OR by anesthesia. General endotracheal intubation introduced. Afrin (oxymetazoline) sprayed into bilateral nostrils. Nasal speculum used to visualize nasal passage on the right and left. Use saline irrigation to clear away crusting and improve visualization, if necessary. Do not place pledgets as this may cause trauma and bleeding, interfering with laser effect (bleeding absorbs KTP wavelength). KTP laser is set to 8 watts, 20 msecs, 2 pulse per sec. Telangiectasias seen on the septum and on the lateral nasal walls as well as floor are spot lasered with the KTP laser carefully used directly over the mucosa surrounding telangectasia in a centripetal spiral pattern circling inward toward telangiectasia. Do not laser on same spot of both sides of the septum to avoid septal perforation.

Bipolar cautery used as needed for deeper more prominent vessels that cannot be reached with the KTP laser. The nose is irrigated intermittently as needed to remove excess bleeding and is suctioned out. At the conclusion of the case Nasopore can be placed appropriately in the nasal cavity. Patient is awakened in the operating room and taken to post op recovery in stable condition.

Video of KTP laser:

Injection of Avastin:

One hundred mg of bevacizumab is diluted to a total volume of 10 mL in normal saline. The bevacizumab solution is injected submucosally throughout the nasal cavity using a 25-gauge spinal needle. The 100mgs of Avastin is typically delivered 50 mgs on each side into the lateral wall in several spots, along with the posterior septum and inferior and middle turbinate as well as nasal floor. Do not inject on over cartilaginous septum for fear of septal perforation. Do not perform other procedures in or around the nose or other areas of the body for fear that the VEGF inhibitor will interfere with wound healing.

Per UIHC OR pharmacy as of July 15, 2011, the cost of Avastin to the patient or insurer is $1412 for 100mg dose. 

References

http://drdavidson.ucsd.edu/Portals/0/Epistaxis%20Treatment%20for%20HHT(2).pdf

Davidson TM, Olitsky SE, Wei JL.  Hereditary hemorrhagic telangiectasia/avastin. Laryngoscope. 2010 Feb;120(2):432-5.

Simonds J, Miller F, Mandel J, Davidson TM.  The effect of bevacizumab (Avastin) treatment on epistaxis in hereditary hemorrhagic telangiectasia. Laryngoscope. 2009 May;119(5):988-92.

Al-Samkari H1, Kritharis A2, Rodriguez-Lopez JM3, Kuter DJ1.Systemic bevacizumab for the treatment of chronic bleeding in hereditary haemorrhagic telangiectasia.J Intern Med. 2019 Feb;285(2):223-231. doi: 10.1111/joim.12832. Epub 2018 Oct 9.