Annual congress report
Published: 2023-04-21
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Silicone septal splint for recurrent epistaxis in HHT patients: experience of a national referral centre

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
https://orcid.org/0000-0001-7876-0489
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
https://orcid.org/0000-0001-7265-591X
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
University of Pavia, Pavia, Italy
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
https://orcid.org/0000-0003-1743-5863
Molecular Medicine Department, General Biology and Medical Genetics Unit, University of Pavia, Pavia, Italy
https://orcid.org/0000-0001-5812-3175
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
https://orcid.org/0000-0002-6857-1729
Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy
https://orcid.org/0000-0002-2244-373X
HHT epistaxis septal perforation nasal splint surgery FID

Abstract

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.

Introduction

Rendu-Osler-Weber disease or hereditary haemorrhagic telangiectasia (HHT, Online Mendelian Inheritance in Man (OMIM) #187300) is an autosomal-dominantly-inherited disorder of the fibrovascular tissue 1,2. The genetic anomalies behind the disease lead to inappropriate maturation of blood vessels in response to angiogenic stimuli. The mutations found in HHT disrupt transforming growth factor-beta-mediated pathways in vascular endothelial cells 3. The result is an aberrant blood vessels development leading to extreme fragility and arteriovenous malformations. The vascular malformations of HHT consist in mucocutaneous telangiectasia, mainly involving nasal and oral mucosa and the skin of the face and fingers, and arteriovenous malformations most notably localised in lungs, liver, gastrointestinal tract and in the central nervous system 1-4.

The main feature of this rare disease is epistaxis, whose severity often leads to anaemia in almost half of adult patients with HHT. In about 80% of cases nosebleeds are the first clinical symptom of the disease and appear before the age of 20 in more than 50% of patients 5,6. Epistaxis varies in intensity, frequency and duration but its severity is often moderate to serious and significantly affects patients’ quality of life 7-11.

Preventive measures (i.e. air humidification, ointment and lubricants, avoidance of triggers etc.) and avoidance of injury to nasal mucosa are generally recommended for management of chronic and recurrent nosebleeds 12,13. Various medical treatments have been reported to treat HHT-related bleedings: angiogenesis inhibitors, hormonal and antihormonal therapies, antifibrinolytics, with different results in terms of bleeding control 14-23.

Surgical management of HHT-related epistaxis strongly depends on bleeding severity. As first-line approach, several forms of mini-invasive surgical techniques including argon plasma coagulation (APC), lasers and coblation can be used, while more invasive procedures, such dermoplasty and modified Young’s procedure, might be evoked in patients with severe and refractory epistaxis 2,24-30.

However, epistaxis in HHT remains a clinically unsolved problem; current therapies do not lead to a definitive resolution but only to temporary control of the condition.

Moreover, patients with recurrent nosebleeds who have already undergone multiple surgical procedures and have developed complications of surgery, such as septal perforation, are known to be a much more difficult court to treat 7.

The use of septal splints to cover the septal mucosa after surgical treatment of epistaxis in HHT patients have been anecdotally reported and could represent an additional strategy in the management of recurrent nasal bleeding 13,31.

The aim of this study was to report our experience in the positioning of silicone septal splint after endoscopic surgical treatment of epistaxis in a cohort of adult HHT patients with severe and refractory nosebleeds, analysing the effectiveness and tolerability of this surgical strategy.

Materials and methods

The present descriptive, observational cohort study was conducted at the Otolaryngology Department of Fondazione IRCCS Policlinico San Matteo (Pavia, Italy), national Reference Centre for diagnosis and treatment of HHT.

Study population

We retrospectively reviewed data of the HHT patients who underwent the endoscopic treatment for recurrent epistaxis between January 2000 and April 2022 at our department. Inclusion criteria were as follows: clinical diagnosis of HHT with at least three out of four Curaçao criteria or positivity at genetic testing 1,33 and the placement of silicone septal nasal splints in occasion of the endoscopic surgery.

Measurements

Demographics and clinical features, such as the number of previous surgical treatments, the distribution of nasal telangiectasias and the presence of septal perforation were reviewed. The enrolled patients were interviewed to evaluate the subjective tolerability and the most common complaints and complications after the splint positioning.

Post-operative outcomes in terms of epistaxis severity, haemoglobin levels and need for blood transfusion were analysed.

The FID score, based on the average frequency, intensity, and duration of epistaxis in the last three months, was used to grade nosebleed severity 33,34. According to the FID score, epistaxis severity is calculated as the sum of the scores (1, 2 or 3) for each value, and is considered low if the sum is 3, mild if 4-6 and severe if 7-9 33,34.

A 10 - millimiter Visual Analogue Scale (VAS), anchored at each end with verbal descriptors (“no symptom-0” and “extreme symptom-10”), was administered to investigate the subjective tolerability of the septal nasal splints and the possible presence and severity of cacosmia (VAS-C), pain (VAS-P), obstruction (VAS-O) and crusting (VAS-Cr) with the splint in place.

The VAS, as psychometric measurement tool, is widely used in the rhinology field to subjectively quantify patients’ symptoms severity and it has been proved highly correlated with more complex instruments assessing nasal specific variables, such as the Sino-Nasal Outcome Test (SNOT)-22 35.

Surgical procedure

The endonasal surgical procedure was performed in the operating theatre under local or general anaesthesia and under endoscopic endonasal control. Argon plasma, diode or thulium laser and quantum molecular resonance technology were used to selectively treat involved mucosa and telangiectasias along the nasal fossae as extensively described in a previous publication 24.

At the end of the procedure silicone splints (Silicon Splint, Medtronic Xomed, Inc. Jacksonville, FL 32216 USA) covering the maximum surface of the nasal septum were inserted and secured in place with a trans-septal non-absorbable stich.

Statistical analysis

Descriptive statistics were used for demographic characteristics. For quantitative variables the results were expressed as mean value and standard deviation (SD). Qualitative variables were summarised as counts and percentages. The comparisons were performed with chi-square test for categorical variables and Student’s t test (or Kruskall-Wallis test if data are skewed) for continuous variables. All tests were two-sided and p-values < 0.05 were considered statistically significant.

Results

Of the 506 patients surgically treated in the period of analysis, 74 patients met the inclusion criteria (23 females and 51 males). The mean age at splint positioning was 64.42 years (SD 10.0), ranging between 40 and 86 years. Before the splint positioning, each patient underwent an average of 2.42 (SD 2.37) surgical treatments to control epistaxis, ranging from 1 to 18 procedures. The mean FID score prior to surgery was 7.38 and the 71.62% of the patients presented with septal perforation. All the included patients had telangiectasias covering the nasal septum. The lateral nasal walls, the nasal valve and nasal floor were involved in 94.42%, 23.93% and 21.15% of cases respectively.

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 subjective tolerability of the nasal splint was 5 (SD 3.09).

On average, patients kept the splints in place for 54.48 months (range 5-180 months). During this time, 26 patients (70.27%) underwent splint repositioning in occasion of further endoscopic surgical treatments of lateral nasal wall or after a spontaneous extrusion of the nasal splints (45.94%, after a mean time of 23.35 months).

The 29.72% (11/37) of patients requested to remove permanently the splints, after a mean of 19.73 months (SD 17.84, range 5-63 months). In this subgroup of patients, there was little or no improvement in epistaxis severity (p = 0.38), Hb levels (p = 0.20) and the number of blood transfusion (p = 0.90) with the splint in place and the complications (VAS-C, VAS-P, VAS-O) were higher – even though not reaching statistical significance – compared to the others (Tab. II).

Discussion

Epistaxis is the most commonly reported symptom in HHT patients. The recurrence and severity of nosebleeds lead to repeated and frequent medical interventions and often translate into poor quality of life for patients with HHT 7-11.

Over the years, several Authors evaluated and applied different types of treatment for epistaxis in HHT patients. The intrinsic variability of the nosebleed, the scarcity of data in the literature regarding long-term follow-up, the small sample sizes, and the adoption of different parameters of evaluation, made nosebleeds in HHT still an unsolved issue.

The choice of therapy, therefore, must be guided by the overall consideration of various factors: the severity of epistaxis, the impairment of quality of life, the extent of endonasal manifestations of the disease, the patient’s general condition, the degree of invasiveness of the surgical treatment, the type of response obtained with any previous treatment 2,13,24,26-28.

Besides the preventive measures and local and/or systemic medical therapies, the literature shows that the first-line surgical approach of epistaxis in HHT patients is nowadays based on minimally invasive endonasal endoscopic procedures which guarantee a good control of nasal bleeding avoiding major complications and can be repeated over time, if necessary 13,24.

In more refractory and severe cases, the cost-benefit balance would lean towards more invasive and less tolerated procedures such as septodermoplasty, arterial embolisation and modified Young’s procedure 2,26,28,36-40.

At the moment, there are no intermediate surgical alternatives validated by the scientific community.

In patients poorly responding to first-line surgical treatments, with severe and recurrent epistaxis or who have developed complications of the surgery, such as septal perforation, the positioning of silicone splints covering the septal mucosa could represent a therapeutic option.

Several studies analysed the possible effects of septal perforation in the general population.

It is well known in literature that a perforation in the septal wall can cause a disturbed airflow in the area of perforation and behind 41,42.

In fact, according to Pousille’s law, septal perforations undermine the laminar flow across the anterior portion of the septum and increase turbulence, naturally occurring in this area, in particular along the posterior edge of the perforation .37,42.

The disturbed airflow and the ensuing vortex contribute to altering the normal physiological properties of the nasal mucosa, with crusting, ulceration and nosebleed due to the decreased temperature and humidity of inspired air and consequent damage and dehydration of the nasal mucosa 37,41,42.

As far as patients with HHT are concerned, the presence of septal perforation has been proven to be an important risk factor in worsening the severity of epistaxis 7.

Moreover, in HHT patients, the distribution of telangiectasias predominantly occurs in the anterior part of the septal mucosa where, as previously described, might be increased 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 of HHT patients poorly responsive to first-line surgical treatment of epistaxis 13,31.

Wirsching and Kühnel in 2017 described their experience in diagnosis and therapy of 97 patients with HHT and reported the outcome of 16 patients receiving silicone septal splint after the endoscopic treatment of nasal telangiectasias with Nd:YAG laser. In their case series the septal splints remained intranasally as long as they were comfortable for the patients, reporting a strong decline in epistaxis severity, with a maximum follow-up of 24 months 13.

Recently, Brahmabhatt et al. conducted a prospective study recruiting 7 patients undergoing nasal septal splint placement after coblation treatment of nasal telangiectasias. In the 12 months follow-up period, Authors reported a significant improvement in epistaxis severity and Hb levels. The splints were well tolerated despite nasal obstruction and crusting 31.

The possible discomforts associated with nasal septal splint, such as pain, crusting and obstruction, have been already reported in literature 45-47.

However, these studies were published in the early 1990s, before development of the thinner, flexible and biocompatible splints nowadays in use. Jung et al. in a recent randomised controlled trial with septal splint left in place after septal surgery, showed that splints were well tolerated without significant nasal discomfort score 48.

However, it is important to remember that the presence of septal perforation and altered airflow patter itself might cause nasal obstruction and crusting, depending on the size and site of the perforation 40.

In our experience, the placement of nasal silicone septal splints for protection of the nasal septum represents a valuable option in HHT patients with recurrent severe epistaxis and with septal perforation who no longer respond to first-line surgical treatment. This surgical strategy aims to offer a more effective intervention compared to the endoscopic telangiectasias coagulation alone, trying to maintain low invasiveness and preserving the nasal patency. In patients with severe epistaxis and septal perforation who do not respond to first-line treatment, the modified Young’s procedure is generally the treatment of choice. Unfortunately, in our experience, this procedure is often not accepted by patients who prefer to procrastinate nasal closure as long as possible.

The use of silicone septal splint after endoscopic treatment of epistaxis proved to be safe, well tolerated, and effective, with significant reduction of epistaxis severity, reduction of the need for blood transfusion and improvement in hemoglobin levels, despite the possible nasal discomfort. Proofs of the usefulness and tolerability of the nasal splints are the months of retention of the splints and the high rate of repositioning in subsequent surgical procedures aimed at treating telangiectasias located on the lateral nasal wall and therefore not covered by the laminae.

Our study has some limitations, indeed. The study is retrospective and therefore it is prone to recall bias. Our aim is to overcome this limit designing a prospective, randomised clinical trial.

Conclusions

Despite these possible limitations, we reported the largest case series on the use of septal splints for control of HHT related nosebleeds, with a long follow-up period. The present study adds interesting new insights into the field of treatment of HHT patients and confirms the efficacy and tolerability of septal splints in the management of recurrent severe epistaxis in this population.

Conflict of interest statement

The authors declare no conflict of interest.

Funding

The research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author contributions

FP, EM: patient care, study design, revision of the manuscript; EM: data collection, literature revision, drafting of the manuscript; CO, GS: data collection, revision of the manuscript; BN: data analysis, literature revision; AL: data collection, literature revision; FS, VS: data collection; MB: revision of the manuscript.

Ethical consideration

This study was approved by the Institutional Ethics Committee (Comitato Etico di Pavia, protocol n. 1-29/1/14).

The research was conducted ethically, with all study procedures being performed in accordance with the requirements of the World Medical Association’s Declaration of Helsinki.

Written informed consent was obtained from each patient for study participation and data publication.

Figures and tables

Figure 1.Main parameters evaluated before and after the positioning of the nasal splints.

N (%) Mean VAS Median VAS SD
Cacosmia 31 (83.78) 5.7 7 3.19
Pain 13 (35.14) 1.4 0 2.07
Obstruction 28 (75.68) 3.9 4 2.90
Crusting 30 (80.08) 5 5 2.96
VAS: visual analogue scale; SD: standard deviation.
Table I.Complaints and complications after the splint positioning.
N (%) VAS-C P VAS-P P VAS-O P VAS-Cr P
Patients requiring permanent removal of the splints 11 (29.72) 6.36 P = 0.44 1.73 P = 0.51 4.36 P = 0.50 4.09 P = 0.25
Patients maintaining splints in place 26 (70.28) 5.46 1.23 3.65 5.30
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.
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.

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Affiliations

Elina Matti

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Eugenia Maiorano

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Bogdan Nacu

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy

Andrea Luceri

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy

Fabio Sovardi

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Vera Siragusa

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy

Anna Ferrauto

University of Pavia, Pavia, Italy

Giuseppe Spinozzi

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Carla Olivieri

Molecular Medicine Department, General Biology and Medical Genetics Unit, University of Pavia, Pavia, Italy

Marco Benazzo

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy

Fabio Pagella

Department of Otorhinolaryngology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy; University of Pavia, Pavia, Italy

Copyright

© Società Italiana di Otorinolaringoiatria e chirurgia cervico facciale , 2023

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