10.14639/0392-100X-suppl.1-43-2023-03
Objective. To report our experience in the use of silicone septal splint for recurrent severe epistaxis in hereditary haemorrhagic telangiectasia patients (HHT). Methods. This is a descriptive analysis carried out at the Otorhinolaryngology Department of Fondazione IRCCS Policlinico San Matteo in Pavia, a reference centre for the treatment and diagnosis of HHT. We retrospectively evaluated HHT patients who underwent silicone septal splint positioning after the endoscopic surgical treatment of epistaxis from 2000 to 2022. Results. Of the 506 patients surgically treated in the period of analysis, 74 patients underwent silicone septal splint positioning and 37 were post-operatively interviewed. With a mean of 2.4 previous surgical treatments and a mean epistaxis severity of 7.38, the majority of patients presented with septal perforation (71.6%). On average, patients maintained the splint in place for 54.5 months, with a good tolerability and a significant reduction in epistaxis severity, need for blood transfusion and improvement of haemoglobin levels. Conclusions. In HHT patients with recurrent severe epistaxis and with septal perforation, the placement of septal splints offers a useful additional strategy in the management of nosebleeds.
text/xml
1827-675X
2604
en
Fabio Pagella
http://creativecommons.org/licenses/by-nc-nd/4.0
ACTA Otorhinolaryngologica Italica
FID
Silicone septal splint for recurrent epistaxis in HHT patients: experience of a national referral centre
Text.Serial.Journal
https://www.actaitalica.it/
43
Annual congress report
2023-04-21
2023-04-19
2023-04-28
2023-04-28
S28-S33
1
Fabio Pagella
2023/04/21
10.14639/0392-100X-suppl.1-43-2023-03
S28
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
1827-675X
FID
S33
Silicone septal splint for recurrent epistaxis in HHT patients: experience of a national referral centre
https://www.actaitalica.it/article/download/2604/940
43
ACTA OTORHINOLARYNGOLOGICA ITALICA 2023;43(SUPPL.1):S28-S33; doi: 10.14639/0392-100X-suppl.1-43-2023-03




Rhinology section


Silicone septal splint for recurrent epistaxis in HHT
patients: experience of a national referral centre
Lamine settali in silicone nel trattamento dell’epistassi ricorrente nei pazienti con HHT:
l’esperienza di un centro di riferimento nazionale
Elina Matti1, Eugenia Maiorano1, Bogdan Nacu1,2, Andrea Luceri1,2, Fabio Sovardi1, Vera Siragusa1,2, Anna Ferrauto2,
Giuseppe Spinozzi1, Carla Olivieri3, Marco Benazzo1,2,, Fabio Pagella1,2
1
    Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; 2 University of Pavia, Pavia, Italy;
3
    Molecular Medicine Department, General Biology and Medical Genetics Unit, University of Pavia, Pavia, Italy


SUMMARY
Objective. To report our experience in the use of silicone septal splint for recurrent severe
epistaxis in hereditary haemorrhagic telangiectasia patients (HHT).
Methods. This is a descriptive analysis carried out at the Otorhinolaryngology Department of
Fondazione IRCCS Policlinico San Matteo in Pavia, a reference centre for the treatment and
diagnosis of HHT. We retrospectively evaluated HHT patients who underwent silicone septal
splint positioning after the endoscopic surgical treatment of epistaxis from 2000 to 2022.
Results. Of the 506 patients surgically treated in the period of analysis, 74 patients under-
went silicone septal splint positioning and 37 were post-operatively interviewed. With a
mean of 2.4 previous surgical treatments and a mean epistaxis severity of 7.38, the major-
ity of patients presented with septal perforation (71.6%). On average, patients maintained
the splint in place for 54.5 months, with a good tolerability and a significant reduction in
epistaxis severity, need for blood transfusion and improvement of haemoglobin levels.
Conclusions. In HHT patients with recurrent severe epistaxis and with septal perforation, the
placement of septal splints offers a useful additional strategy in the management of nosebleeds.
KEY WORDS: HHT, epistaxis, septal perforation, nasal splint, surgery, FID
                                                                                                     Received: January 30, 2023
                                                                                                     Accepted: February 2, 2023
RIASSUNTO
Obiettivo. Riportare la nostra esperienza nell’utilizzo delle lamine settali in silicone nel trat-
                                                                                                     Correspondence
tamento dell’epistassi ricorrente nei pazienti con teleangectasia emorragica ereditaria (HHT).       Eugenia Maiorano
Metodi. Si tratta di uno studio retrospettivo svolto presso la SC di Otorinolaringoiatria            Department of Otorhinolaryngology, Fondazione
della Fondazione IRCCS Policlinico San Matteo, centro di riferimento per la diagnosi e la            IRCCS Policlinico San Matteo, piazzale Golgi 19,
cura dell’HHT. Sono stati valutati i pazienti con HHT sottoposti dal 2000 al 2022 al posi-           27100 Pavia, Italy
zionamento di lamine settali in silicone nella gestione delle epistassi ricorrenti.                  E-mail: eugenia_maiorano@libero.it
Risultati. Dei 506 pazienti trattati chirurgicamente, 74 sono stati sottoposti al posizionamen-
to delle lamine e 37 hanno aderito allo studio. Con una media di 2,4 precedenti trattamenti            How to cite this article: Matti E, Maiorano E,
chirurgici ed un valore medio di gravità delle epistassi di 7,38, il 71,6% dei pazienti presen-        Nacu B, et al. Silicone septal splint for recur-
tava una perforazione settale. Mediamente, i pazienti hanno mantenuto le lamine in sede per            rent epistaxis in HHT patients: experience of a
54 mesi, con una buona tollerabilità e una significativa riduzione della gravità delle epistassi,      national referral centre. Acta Otorhinolaryngol
del numero medio di trasfusioni e un miglioramento nei valori medi di emoglobina.                      Ital 2023;43(SUPPL.1):S28-S33. https://doi.
Conclusioni. Nei pazienti HHT con epistassi gravi ricorrenti e con perforazione settale,               org/10.14639/0392-100X-suppl.1-43-2023-03
il posizionamento di lamine di silicone a protezione della mucosa settale rappresenta una
valida strategia nel controllo delle epistassi.                                                      © Società Italiana di Otorinolaringoiatria
                                                                                                     e Chirurgia Cervico-Facciale
PAROLE CHIAVE: HHT, epistassi, perforazione settale, lamine, chirurgia, FID
                                                                                                                    OPEN ACCESS

                                                                                                     This is an open access article distributed in accordance with
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Introduction                                                                                         Commercial-NoDerivatives 4.0 International) license. The
                                                                                                     article can be used by giving appropriate credit and mentio-
                                                                                                     ning the license, but only for non-commercial purposes and
Rendu-Osler-Weber disease or hereditary haemorrhagic telangiectasia (HHT,                            only in the original version. For further information: https://
Online Mendelian Inheritance in Man (OMIM) #187300) is an autosomal-dom-                             creativecommons.org/licenses/by-nc-nd/4.0/deed.en




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                                                                               Silicone septal splint for recurrent epistaxis in HHT patients




inantly-inherited disorder of the fibrovascular tissue 1,2. The   Materials and methods
genetic anomalies behind the disease lead to inappropriate
maturation of blood vessels in response to angiogenic stimu-      The present descriptive, observational cohort study was
li. The mutations found in HHT disrupt transforming growth        conducted at the Otolaryngology Department of Fondazi-
factor-beta-mediated pathways in vascular endothelial cells 3.    one IRCCS Policlinico San Matteo (Pavia, Italy), national
The result is an aberrant blood vessels development leading       Reference Centre for diagnosis and treatment of HHT.
to extreme fragility and arteriovenous malformations. The
vascular malformations of HHT consist in mucocutaneous            Study population
telangiectasia, mainly involving nasal and oral mucosa and        We retrospectively reviewed data of the HHT patients who
the skin of the face and fingers, and arteriovenous malforma-     underwent the endoscopic treatment for recurrent epistaxis
tions most notably localised in lungs, liver, gastrointestinal    between January 2000 and April 2022 at our department.
tract and in the central nervous system 1-4.                      Inclusion criteria were as follows: clinical diagnosis of
The main feature of this rare disease is epistaxis, whose         HHT with at least three out of four Curaçao criteria or pos-
severity often leads to anaemia in almost half of adult pa-       itivity at genetic testing 1,33 and the placement of silicone
tients with HHT. In about 80% of cases nosebleeds are the         septal nasal splints in occasion of the endoscopic surgery.
first clinical symptom of the disease and appear before the
age of 20 in more than 50% of patients 5,6. Epistaxis varies      Measurements
in intensity, frequency and duration but its severity is often    Demographics and clinical features, such as the number of
moderate to serious and significantly affects patients’ qual-     previous surgical treatments, the distribution of nasal tel-
ity of life 7-11.                                                 angiectasias and the presence of septal perforation were
Preventive measures (i.e. air humidification, ointment and        reviewed. The enrolled patients were interviewed to evalu-
lubricants, avoidance of triggers etc.) and avoidance of in-      ate the subjective tolerability and the most common com-
jury to nasal mucosa are generally recommended for man-           plaints and complications after the splint positioning.
agement of chronic and recurrent nosebleeds 12,13. Various        Post-operative outcomes in terms of epistaxis severity, haemo-
medical treatments have been reported to treat HHT-related        globin levels and need for blood transfusion were analysed.
bleedings: angiogenesis inhibitors, hormonal and antihor-         The FID score, based on the average frequency, intensity,
monal therapies, antifibrinolytics, with different results in     and duration of epistaxis in the last three months, was used
terms of bleeding control 14-23.                                  to grade nosebleed severity 33,34. According to the FID score,
Surgical management of HHT-related epistaxis strongly             epistaxis severity is calculated as the sum of the scores (1, 2
depends on bleeding severity. As first-line approach, sev-        or 3) for each value, and is considered low if the sum is 3,
eral forms of mini-invasive surgical techniques including         mild if 4-6 and severe if 7-9 33,34.
argon plasma coagulation (APC), lasers and coblation can          A 10 - millimiter Visual Analogue Scale (VAS), anchored
be used, while more invasive procedures, such dermoplasty         at each end with verbal descriptors (“no symptom-0” and
and modified Young’s procedure, might be evoked in pa-            “extreme symptom-10”), was administered to investigate
tients with severe and refractory epistaxis 2,24-30.              the subjective tolerability of the septal nasal splints and the
However, epistaxis in HHT remains a clinically unsolved           possible presence and severity of cacosmia (VAS-C), pain
problem; current therapies do not lead to a definitive reso-      (VAS-P), obstruction (VAS-O) and crusting (VAS-Cr) with
lution but only to temporary control of the condition.            the splint in place.
Moreover, patients with recurrent nosebleeds who have             The VAS, as psychometric measurement tool, is widely
already undergone multiple surgical procedures and have           used in the rhinology field to subjectively quantify pa-
developed complications of surgery, such as septal perfora-       tients’ symptoms severity and it has been proved highly
tion, are known to be a much more difficult court to treat 7.     correlated with more complex instruments assessing nasal
The use of septal splints to cover the septal mucosa after        specific variables, such as the Sino-Nasal Outcome Test
surgical treatment of epistaxis in HHT patients have been         (SNOT)-22 35.
anecdotally reported and could represent an additional
strategy in the management of recurrent nasal bleeding 13,31.     Surgical procedure
The aim of this study was to report our experience in the         The endonasal surgical procedure was performed in the op-
positioning of silicone septal splint after endoscopic surgi-     erating theatre under local or general anaesthesia and under
cal treatment of epistaxis in a cohort of adult HHT patients      endoscopic endonasal control. Argon plasma, diode or thu-
with severe and refractory nosebleeds, analysing the effec-       lium laser and quantum molecular resonance technology
tiveness and tolerability of this surgical strategy.              were used to selectively treat involved mucosa and telangi-



                                                                                                                                       S29
E. Matti et al.




ectasias along the nasal fossae as extensively described in a    (70.27%) underwent splint repositioning in occasion of fur-
previous publication 24.                                         ther endoscopic surgical treatments of lateral nasal wall or
At the end of the procedure silicone splints (Silicon Splint,    after a spontaneous extrusion of the nasal splints (45.94%,
Medtronic Xomed, Inc. Jacksonville, FL 32216 USA)                after a mean time of 23.35 months).
covering the maximum surface of the nasal septum were            The 29.72% (11/37) of patients requested to remove perma-
inserted and secured in place with a trans-septal non-ab-        nently the splints, after a mean of 19.73 months (SD 17.84,
sorbable stich.                                                  range 5-63 months). In this subgroup of patients, there was lit-
                                                                 tle or no improvement in epistaxis severity (p = 0.38), Hb lev-
Statistical analysis                                             els (p = 0.20) and the number of blood transfusion (p = 0.90)
Descriptive statistics were used for demographic character-      with the splint in place and the complications (VAS-C, VAS-
istics. For quantitative variables the results were expressed    P, VAS-O) were higher – even though not reaching statistical
as mean value and standard deviation (SD). Qualitative           significance – compared to the others (Tab. II).
variables were summarised as counts and percentages. The
comparisons were performed with chi-square test for cat-         Discussion
egorical variables and Student’s t test (or Kruskall-Wallis
test if data are skewed) for continuous variables. All tests     Epistaxis is the most commonly reported symptom in HHT
                                                                 patients. The recurrence and severity of nosebleeds lead
were two-sided and p-values < 0.05 were considered statis-
                                                                 to repeated and frequent medical interventions and often
tically significant.
                                                                 translate into poor quality of life for patients with HHT 7-11.
                                                                 Over the years, several Authors evaluated and applied differ-
Results                                                          ent types of treatment for epistaxis in HHT patients. The in-
Of the 506 patients surgically treated in the period of analy-   trinsic variability of the nosebleed, the scarcity of data in the
sis, 74 patients met the inclusion criteria (23 females and      literature regarding long-term follow-up, the small sample
51 males). The mean age at splint positioning was 64.42          sizes, and the adoption of different parameters of evaluation,
years (SD 10.0), ranging between 40 and 86 years. Before         made nosebleeds in HHT still an unsolved issue.
the splint positioning, each patient underwent an average        The choice of therapy, therefore, must be guided by the
of 2.42 (SD 2.37) surgical treatments to control epistax-        overall consideration of various factors: the severity of
is, ranging from 1 to 18 procedures. The mean FID score          epistaxis, the impairment of quality of life, the extent of
prior to surgery was 7.38 and the 71.62% of the patients         endonasal manifestations of the disease, the patient’s gen-
presented with septal perforation. All the included patients     eral condition, the degree of invasiveness of the surgical
had telangiectasias covering the nasal septum. The lateral       treatment, the type of response obtained with any previous
nasal walls, the nasal valve and nasal floor were involved in    treatment 2,13,24,26-28.
94.42%, 23.93% and 21.15% of cases respectively.                 Besides the preventive measures and local and/or systemic
                                                                 medical therapies, the literature shows that the first-line
Of the 74 patients who received surgical treatment with
splint positioning during the period of analysis, 19 (25.68%)
died at the time of writing and 37 (50%) were interviewed.
The remaining patients did not reply to our survey.
Analysing the data of the 37 patients interviewed, after the
surgical treatment with nasal splint positioning, the mean
FID score decreased from 7.38 (SD 1.55) to 5.11 (SD 2.04)
(p < 0.001), the mean haemoglobin (Hb) levels raised from
7.75 g/dl (SD 1.30) to 8.75 g/dl (SD 2.58) (p < 0.001) and the
mean number of blood transfusion in three months switched
from 3.13 (SD 4.73) to 2.04 (SD 4.89) (p < 0.001) (Fig. 1).
Regarding the complaints and complications after the splint
positioning, the mean VAS-C was 5.72 (SD 3.19), the mean
VAS-P was 1.38 (SD 2.07), the mean VAS-O was 3.86 (SD
2.89) and the mean VAS-Cr was 4.95 (SD 2.96) (Tab. I). The               FID: frequency, intensity, duration score; Hb: plasmatic haemoglobin value.
subjective tolerability of the nasal splint was 5 (SD 3.09).
On average, patients kept the splints in place for 54.48         Figure 1. Main parameters evaluated before and after the positioning of the
months (range 5-180 months). During this time, 26 patients       nasal splints.



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                                                                                                               Silicone septal splint for recurrent epistaxis in HHT patients




Table I. Complaints and complications after the splint positioning.                          As far as patients with HHT are concerned, the presence of
                     N (%)        Mean VAS Median VAS               SD                       septal perforation has been proven to be an important risk
 Cacosmia              31 (83.78)            5.7               7              3.19           factor in worsening the severity of epistaxis 7.
 Pain                  13 (35.14)            1.4               0              2.07           Moreover, in HHT patients, the distribution of telangiecta-
 Obstruction           28 (75.68)            3.9               4              2.90           sias predominantly occurs in the anterior part of the septal
 Crusting              30 (80.08)             5                5              2.96           mucosa where, as previously described, might be increased
VAS: visual analogue scale; SD: standard deviation.                                          turbulence of airflow 31,43,44.
                                                                                             To date there are only two reports in literature taking into
                                                                                             account the placement of septal splints in the management
surgical approach of epistaxis in HHT patients is nowa-                                      of HHT patients poorly responsive to first-line surgical
days based on minimally invasive endonasal endoscopic                                        treatment of epistaxis 13,31.
procedures which guarantee a good control of nasal bleed-                                    Wirsching and Kühnel in 2017 described their experience
ing avoiding major complications and can be repeated over                                    in diagnosis and therapy of 97 patients with HHT and re-
time, if necessary 13,24.                                                                    ported the outcome of 16 patients receiving silicone sep-
In more refractory and severe cases, the cost-benefit bal-                                   tal splint after the endoscopic treatment of nasal telangi-
ance would lean towards more invasive and less tolerated                                     ectasias with Nd:YAG laser. In their case series the septal
procedures such as septodermoplasty, arterial embolisation                                   splints remained intranasally as long as they were comfort-
and modified Young’s procedure 2,26,28,36-40.                                                able for the patients, reporting a strong decline in epistaxis
At the moment, there are no intermediate surgical alterna-                                   severity, with a maximum follow-up of 24 months 13.
tives validated by the scientific community.                                                 Recently, Brahmabhatt et al. conducted a prospective study
In patients poorly responding to first-line surgical treat-                                  recruiting 7 patients undergoing nasal septal splint place-
ments, with severe and recurrent epistaxis or who have de-                                   ment after coblation treatment of nasal telangiectasias. In the
veloped complications of the surgery, such as septal perfo-                                  12 months follow-up period, Authors reported a significant
ration, the positioning of silicone splints covering the septal                              improvement in epistaxis severity and Hb levels. The splints
mucosa could represent a therapeutic option.                                                 were well tolerated despite nasal obstruction and crusting 31.
Several studies analysed the possible effects of septal per-                                 The possible discomforts associated with nasal septal
foration in the general population.                                                          splint, such as pain, crusting and obstruction, have been al-
It is well known in literature that a perforation in the septal                              ready reported in literature 45-47.
wall can cause a disturbed airflow in the area of perforation                                However, these studies were published in the early 1990s,
and behind 41,42.                                                                            before development of the thinner, flexible and biocompat-
In fact, according to Pousille’s law, septal perforations under-                             ible splints nowadays in use. Jung et al. in a recent ran-
mine the laminar flow across the anterior portion of the sep-                                domised controlled trial with septal splint left in place af-
tum and increase turbulence, naturally occurring in this area,                               ter septal surgery, showed that splints were well tolerated
in particular along the posterior edge of the perforation .37,42.                            without significant nasal discomfort score 48.
The disturbed airflow and the ensuing vortex contribute                                      However, it is important to remember that the presence of
to altering the normal physiological properties of the na-                                   septal perforation and altered airflow patter itself might
sal mucosa, with crusting, ulceration and nosebleed due                                      cause nasal obstruction and crusting, depending on the size
to the decreased temperature and humidity of inspired air                                    and site of the perforation 40.
and consequent damage and dehydration of the nasal mu-                                       In our experience, the placement of nasal silicone septal
cosa 37,41,42.                                                                               splints for protection of the nasal septum represents a valu-


Table II. Comparison of complaints and complications in HHT patients who asked to permanently remove the septal nasal splints vs those who maintained the
splint in place.
                                    N (%)             VAS-C           P              VAS-P            P             VAS-O              P             VAS-Cr               P
 Patients requiring
 permanent removal of             11 (29.72)          6.36                           1.73                            4.36                              4.09
 the splints                                                       P = 0.44                       P = 0.51                         P = 0.50                           P = 0.25
 Patients maintaining
                                  26 (70.28)          5.46                           1.23                            3.65                              5.30
 splints in place
HHT: Hereditary haemorrhagic telangiectasia; VAS-C: visual analogue scale for cacosmia; VAS-P: visual analogue scale for pain; VAS-O: visual analogue scale for obstruction; VAS-Cr:
visual analogue scale for crusting.




                                                                                                                                                                              S31
E. Matti et al.




able option in HHT patients with recurrent severe epistaxis      Ethical consideration
and with septal perforation who no longer respond to first-      This study was approved by the Institutional Ethics Com-
line surgical treatment. This surgical strategy aims to offer    mittee (Comitato Etico di Pavia, protocol n. 1-29/1/14).
a more effective intervention compared to the endoscopic         The research was conducted ethically, with all study pro-
telangiectasias coagulation alone, trying to maintain low in-    cedures being performed in accordance with the require-
vasiveness and preserving the nasal patency. In patients with    ments of the World Medical Association’s Declaration of
severe epistaxis and septal perforation who do not respond to    Helsinki.
first-line treatment, the modified Young’s procedure is gen-     Written informed consent was obtained from each patient
erally the treatment of choice. Unfortunately, in our experi-    for study participation and data publication.
ence, this procedure is often not accepted by patients who
prefer to procrastinate nasal closure as long as possible.       References
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The authors declare no conflict of interest.
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