Key Points:
- Patients with hereditary haemorrhagic telangiectasia (HHT) often
experience intractable episodes of epistaxis due to intranasal
telangiectasia
- There is no existing literature on surgical treatment for
rhinosinusitis in patients with HHT
- Endoscopic balloon sinuplasty has been shown to be a safe, minimally
invasive surgical approach to sinusitis
- We show that endoscopic balloon sinuplasty can be safely performed in
a patient with HHT
- This technique can be combined with other endoscopic treatment for
epistaxis, such as laser or conventional diathermy coagulation
Introduction:
Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant
disorder of angiodysplasia characterized by mucocutaneous
telangiectasia, recurrent epistaxis and arteriovenous malformations. (1)
Most commonly patients present with epistaxis due to intranasal
telangiectasia; however telangiectasia can also occur in the skin,
gastrointestinal and oral mucosa and arteriovenous malformations (AVM)
can occur in the liver, lungs and brain. (1) Patients with HHT are at
increased risk of bleeding during endoscopic sinus surgery. (2) Balloon
sinuplasty is a less invasive technique compared to traditional
endoscopic sinus surgery. (3) We report the use of balloon sinuplasty to
treat chronic frontal sinusitis in a man with HHT and a previous frontal
lobe abscess.
Objectives:
To demonstrate the safety and efficacy of endoscopic balloon sinuplasty
in the treatment of frontal sinusitis in a patient with HHT.
Methods:
A retrospective case report was performed and all data was obtained from
the routine care of the patient.
Ethics:
Institutional or ethical board review was not sought for this case
report. Written informed consent was obtained from the participant of
the study. All patient data has been checked and de-identified prior to
submission.
Reporting guidelines:
This case report utilizes the CARE guidelines for case reports. (6)
Results:
A 41 year old man with a diagnosis of HHT was referred to a tertiary
referral rhinology clinic after an emergency admission with a right
frontal lobe abscess which had been drained via a right temperoparietal
craniotomy. A 10.7 x 10.6 x 3.1 cm left lingula pulmonary AVM (PAVM) was
diagnosed during that admission and successfully embolized with an
AMPLATZER Vascular Plug (St. Jude Medical, Plymouth, Minnesota, USA).
Isolated opacification of his right frontal sinus was also identified on
serial magnetic resonance imaging (MRI) scans but there was no bony
erosion of the posterior table (Fig 1). It was not possible to determine
whether the PAVM or frontal sinus disease was responsible for the
frontal lobe abscess.
The patient reported recurrent epistaxis and crusting since childhood
but had never sought treatment for this. He denied all other nasal
symptoms, including prior to/during his admission. Examination revealed
multiple intranasal telangiectasia, septal deviation to the left and a
bulky right middle turbinate. There was no evidence of acute or chronic
sinusitis with no pus, oedema or polyps seen. CT sinuses confirmed an
isolated right frontal sinus opacification with opacification of the
frontal recess and an opacified mucous filled right middle turbinate
concha bullosa (Fig 2).
Treatment options were discussed with the patient. Given his lack of
symptoms and the potential bleeding risk associated with any
intervention, conservative management was trialled initially. At one
year there was no change to the persistent right frontal sinus
opacification. At that review, he revealed his epistaxis was beginning
to affect his quality of life. Management options were again discussed
with the patient and he opted for endoscopic potassium titanyl phosphate
(KTP) laser ablation for his epistaxis. At the same time, he was offered
endoscopic balloon sinuplasty to treat his chronic frontal sinusitis, as
a potentially less traumatic option than traditional endoscopic sinus
surgery. As a precaution he was also consented for both endoscopic and
external approaches to the right frontal sinus.
The patient was anaesthetized using total intravenous anaesthesia to
optimize blood pressure control. He was intubated with an oral RAE tube
and positioned supine in a reverse Trendelenburg position. The nose was
prepared with a modified Moffett’s solution containing 100mg of cocaine,
1mg of adrenaline and 8mL of normal saline topically for
vasoconstriction. A zero-degree 2.7mm endoscope was used. The right
concha bullosa was reduced laterally for access to the frontal recess.
Thick retained mucous and polypoid tissue was debrided from the exposed
frontal recess in the middle meatus. A 6mm NuVent frontal sinus balloon
(Medtronic, Fridley, Minnesota, USA) was placed into the frontal recess
under direct vision and dilated for 3 seconds. Frank mucopus was
irrigated from the frontal sinus following removal of the balloon and
irrigation. Patency of the frontal recess was confirmed with 30-degree
and 70-degree endoscopes (Fig 3) (Supplemental annotated video).
Bilateral endoscopic KTP laser ablation of the nasal telangiectasia was
then performed using a standard technique (4). There was no significant
bleeding during the procedure. The patient was extubated in recovery and
discharged home the same day. He did not suffer any immediate or delayed
post-operative complications.
After one month he had not had any significant episodes of epistaxis.
Careful flexible endoscopy confirmed patency of the right frontal recess
and no mucopus was seen. Repeat MRI at six months revealed a clear right
frontal sinus with no residual opacification (Fig 4). He was reviewed in
clinic at one year post-operatively due to recurrence of his epistaxis,
albeit at a lesser frequency and severity. He was booked for another KTP
laser procedure.
Discussion:
Hereditary haemorrhagic telangiectasia (HHT) is not a risk factor for
acute or chronic rhinosinusitis, however it is a difficult problem to
treat in these patients. Routine endoscopic sinus surgery is complicated
by the presence of intranasal telangiectasia which, if traumatised, can
cause life threatening bleeding, impair visualization of the surgical
field and increase the risk of intra-operative complications. (5)
Balloon sinuplasty is a minimally invasive technique for the treatment
of chronic rhinosinusitis without polyposis which can even be used in
the outpatient setting. (3) With adequate topical decongestion and
vascoconstriction, the frontal ostium can be catheterized without the
need for endoscopic access or ablative surgery. At the time of the
procedure, any symptomatic telangiectasia can be treated with KTP laser
ablation or cautery. Here we demonstrate its efficacy in treating
isolated frontal sinus disease in a patient with a large intranasal
burden of telangiectasia. Careful endoscopic balloon sinuplasty allowed
atraumatic visualization and instrumentation of the frontal recess. The
ostium was widened to allow intraoperative irrigation of the mucopus
retained within the sinus and the frontal sinus remained patent six
months following surgery on a repeat MRI scan. This minimally invasive
technique also avoided the need for intranasal packing post-operatively
and reduced the risk of trauma to the anterior nasal mucosa. Whilst
traditional endoscopic surgical techniques were required to help deploy
the balloon, widening of the ostia and decompression of the sinus was
achieved with the balloon sinuplasty device.
Conclusion:
To our knowledge this is the first reported case of balloon sinuplasty
in a patient with HHT. Traditional sinus surgery is generally avoided in
this group if possible, given the potential risk of significant
intraoperative bleeding. In practice, this technique may be
transferrable to the treatment of maxillary sinus disease in patients
with HHT.
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sinus surgery for chronic rhinosinusitis: a 25-year experience.
Laryngoscope. 2011 Dec; 121(12):2684-701. doi: 10.1002/lary.21446.
Epub 2011 Nov 15.
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dilation of sinus ostia for chronic rhinosinusitis. Cochrane Database
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Figure 1 – Magnetic Resonance Imaging (MRI) findings of right frontal
sinus opacification (arrow head) pre-operatively and an opacified right
middle turbinate concha bullosa (arrow)
Figure 2 – Computed Tomography (CT) demonstrating right frontal sinus
and frontal recess (arrow head) opacification with obstruction of the
frontal recess by a concha bullosa of the middle turbinate (arrow)
without evidence of pansinusitis
Figure 3 – 70 degree endoscopic view intra-operatively confirming
patency of the right frontal ostium following balloon sinuplasty
Figure 4 – Post-operative MRI confirming a patent right frontal recess
(arrow head) six month following balloon sinuplasty