Correlation of severity of epistaxis with nasal telangiectasias

in hereditary hemorrhagic telangiectasia (HHT) patients

Fabio Pagella, M.D.,* Andrea Colombo, M.D.,* Elina Matti, M.D.,* Georgios Giourgos, M.D.,*
Carmine Tinelli, M.D.,# Carla Olivieri, Ph.D.,§ and Cesare Danesino, M.D.§

ABSTRACT
Background: Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is an autosomal dominant
disease that leads to multiregional angiodysplasia. The presence of telangiectasias in nasal mucosa leads to recurrent epistaxis that affects up
to 96% of patients but with unpredictable severity. Some authors have previously explained that endonasal morphology and distribution of
telangiectasias can be variable too. The purpose of this study was to evaluate any possible relationship between the severity of epistaxis and
the different morphology and distribution of nasal telangiectasias in HHT patients.
Methods: A review was performed of nasal endoscopy records of 76 consecutive HHT patients treated for epistaxis between 2003 and 2007
at our institution. An evaluation was performed of severity of epistaxis in the same patient group using a questionnaire and considering
frequency, intensity, duration of nosebleeds, and need for blood transfusions. Comparison of data collected on morphology and distribution
of nasal telangiectasias with data collected on severity of epistaxis was performed.
Results: Morphology and distribution of nasal telangiectasias showed a statistically significant correlation with frequency and intensity
of epistaxis. Presence of telangiectasias endoscopically appearing as large and prominent correlates with higher frequency of epistaxis. An
increase in number of nasal subsites involved correlates with higher intensity of nosebleeds.
Conclusion: Our data suggest that to reduce frequency and intensity of epistaxis in HHT patients, treatments should be directed also at
lesions located in the posterior part of nasal fossae and especially on telangiectasias endoscopically appearing as large and prominent.
(Am J Rhinol Allergy 23, 52–58, 2009; doi: 10.2500/ajra.2009.23.3263)

Key words: Correlation, distribution, epistaxis, hereditary hemorrhagic telangiectasia, HHT, morphology, nose, Rendu Osler
Weber syndrome, severity, telangiectasias

H ereditary hemorrhagic telangiectasia (HHT; OMIM

187300 and 600376), also known as Rendu-Osler-Weber
syndrome, is an autosomal dominant disease and its preva-
lence was recently estimated in different populations between
1:5000 and 1:8000.1 HHT has an almost complete age-related
penetrance and leads to multiregional angiodysplasia. Clini-
cally, the occurrence of mucocutaneous and gastrointestinal
telangiectasias and of systemic arteriovenous malformations
is commonly observed. The presence of telangiectasias in
nasal mucosa leads to recurrent epistaxis that affects up to
96% of patients, but with unpredictable severity and wide
variability as to age of onset and frequency.1 As previously
described by only a few articles in literature,2–4 endonasal
morphology and distribution of telangiectasias can be vari-
able too. In this study we evaluated a possible relationship
between the severity of epistaxis and the different morphol-
ogy and distribution of nasal telangiectasias observed in a
series of HHT patients.
MATERIALS AND METHODS
From January 1996 to February 2008, 214 HHT patients
were evaluated at our department and 152 of these patients
From the *Department of Otorhinolaryngology, University of Pavia IRCCS Policlinico
S. Matteo Foundation, Pavia, Italy, and #Clinical Epidemiology and Biometric Unit,
IRCCS Policlinico S. Matteo Foundation, Pavia, Italy, and §General Biology and
Medical Genetics, University of Pavia, Pavia, Italy
Address correspondence and reprint requests to Fabio Pagella, M.D., Clinica Otorino-
laringoiatrica, Fondazione IRCCS Policlinico San Matteo, Viale Golgi 19, 27100 Pavia,
Italy
E-mail address: hht@smatteo.pv.it
Copyright © 2009, OceanSide Publications, Inc., U.S.A.
were treated for epistaxis. We performed a retrospective re-
view of nasal endoscopy records of the HHT patients we
evaluated between 2003 and 2007. Only subjects surely af-
fected by HHT according to the Curaçao criteria5 were in-
cluded in the study. Patients who presented nasal septum
perforation were excluded from the study. Patients who pre-
viously underwent nasal septodermoplasty or embolization
and patients who were treated for epistaxis during the last 12
months were excluded to reduce influence of treatments on
nasal morphology and distribution of telangiectasias. More-
over, patients with alteration of coagulation parameters were
excluded too, to avoid influence of altered coagulation on the
severity of epistaxis. For patients who underwent multiple
treatments, only the records concerning the first treatment
were considered.
All patients treated underwent nasal endoscopy during
preparation of the nasal cavities for the surgical procedure by
argon plasma coagulation. The patients, in anti-Trendelen-
burg position, had a careful and meticulous preparation of the
nasal cavities: crusts and old blood clots were gently removed
under endoscopic control. Decongestion of nasal cavities was
obtained by positioning of oxymetazoline hydrochloride–
soaked cottonoid pledgets. After preparation, all nasal endos-
copies were performed bilaterally with a 0° endoscope follow-
ing a two-step standard technique. The endoscope was first
inserted parallel to the nasal floor through the nasal fossa
until reaching rhinopharynx; this movement of the endoscope
allowed visualization of the nasal valve, the inferior part of
nasal septum, the floor of nasal fossa, the inferior turbinate,
the inferior meatum, and the rhinopharynx. Once retracted
the endoscope to the anterior nasal valve, in the second step,

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Figure 1. Punctate pattern (view of the left nasal fossa through a 0°
endoscope: presence of flat and punctate telangiectasias in corre-
spondence of the head of middle turbinate, anterior nasal septum,
and region of the agger nasi).
Figure 2. Large pattern (view of the right nasal fossa through a 0°
endoscope: presence of large and prominent telangiectasias in cor-
respondence of the anterior nasal septum, the head of the inferior
turbinate and the nasal valve).

the operator adjusted the instrument position to form an ⬃45°

angle above the horizontal plane; this movement of the endo-
scope allowed visualization of the agger nasi area, the supe-
rior part of nasal septum, and the head of middle turbinate;
then, readjusting progressively the instrument position and
advancing parallel to the nasal floor through the nasal fossa,
visualization of remaining middle turbinate and nasal sep-
tum, of middle meatum and of sphenoethmoidal recess was
reached. Images obtained during nasal endoscopy were re-
corded using a Tricam SL camera (Karl Storz GmbH & Co.,
Tuttlingen, Germany) and a Betacam UVW-1400 AP recorder
(Sony Corp., Tokyo, Japan). Two expert rhinologists reviewed
the records defining concurrently the presence or absence and
the morphology of telangiectasias. Presence or absence of
telangiectasias was assessed considering six subsites in nasal
fossae: nasal valve, floor of nasal fossae, anterior and posterior
nasal septum (considering as limit a coronal plane tangential
to the heads of middle turbinates), superior lateral wall (con-
sidering middle turbinate, region of the agger nasi, and mid-
dle meatum), and inferior lateral wall (considering inferior
turbinate and inferior meatum). Morphology of telangiecta-
sias was endoscopically classified as punctate and large: we
defined as “punctate lesions” telangiectasias that appeared as
punctate and flat lesions; the others, bigger and prominent, Figure 3. Mixed pattern (view of the anterior left nasal fossa
were defined as “large.” Patients were then divided into three through a 0° endoscope: presence of both flat and prominent
groups: punctate pattern, large pattern, and mixed pattern telangiectasias).
(Figs. 1–3). Patients who presented confluence of lesions were
included in the “punctate,” “large,” or “mixed” pattern con- naire (Table 1), including data about frequency, intensity, and
sidering the endoscopic aspect of single telangiectasias com- duration of nosebleeds and about previous need for blood
posing the lesions. For each pattern, as well as for the number transfusion. Except for duration of nosebleeds, parameters
of involved sites, we evaluated any correlation with the se- were chosen combining those used by Bergler et al.6 and
verity of epistaxis. Rebeiz et al.7 For each parameter three different levels of
To evaluate severity of epistaxis, all patients, at the time of severity were considered to obtain a score for the severity of
admission to our institution, were asked to fill out a question- epistaxis.

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Table 1 Patient’s questionnaire
Epistaxis Blood Transfusion
Frequency Intensity Duration Number of Recent Need
Previous Blood for Blood
Transfusions Transfusions
Grade 1 Less than one Slight stains on the Less than 10 min No need No need
episode/wk handkerchief
Grade 2 At least one Soaked handkerchief From 10 to 30 min Less than 10 U From 1 to 5 U
episode/wk during the
last 3 mo
Grade 3 More than one Bowl or similar Over 30 min More than 10 U Over 5 U
episode/day utensil necessary during the
last 3 mo

Table 2 Answers of patients to the questionnaire

Grade
1 2 3
Epistaxis
Frequency score (%) 10 (14) 18 (24) 46 (62)
Intensity score (%) 7 (9) 14 (19) 53 (72)
Duration score (%) 23 (31) 23 (31) 28 (38)
Blood transfusions
Previous transfusions score (%) 54 (73) 12 (16) 8 (11)
Recent transfusions score (%) 62 (84) 8 (11) 4 (5)

To compare all of the categorical variables, we used the
␹2-test or the Fisher’s exact test, as appropriate. All of the
statistical tests were considered two tailed and were signifi-
cant when p ⬍ 0.05. The statistical analysis was conducted
using the STATA software (StataCorp., 2003, Stata Statistical
Software, release 8.0; StatCorp, College station, TX).
RESULTS
We performed a review of the records of 76 consecutive
patients satisfying the inclusion criteria; among them, two
records did not adequately describe patients’ nasal distribu-
tion of lesions and were excluded. Thus, the patients studied
included 46 men and 28 women, with a mean age of 50.9 years
(range, 15–79; median, 52 years). Answers of the patient to the
questionnaire are summarized in Table 2. Characteristics of
the patient group and differences between men and women
are summarized in Table 3. Distribution of telangiectasias
among nasal subsites are represented in Fig. 4.
No significant difference was observed between men and
women considering the severity of epistaxis (frequency score,
intensity score, and duration score) and the necessity for
blood transfusions (previous and recent); in addition, we did
not observe a significant correlation between the age of our
patients and the telangiectasias’ nasal pattern and distribu-
tion. A large pattern was the less represented in both sex
groups; however, we observed significant differences be-
tween sex groups considering the nasal pattern (p ⫽ 0.019)
with a relative predominance of the punctate pattern in the
female group. Fifty percent of patients with no sex prevalence
showed confluence of telangiectasias in at least one subsite of
the nasal fossae; no prevalence of large or punctate pattern
was observed in basic composition of confluent lesions. No
significant difference was observed between men and women,
considering number of sites involved.
We evaluated the differences in the single scores concerning
epistaxis (frequency, intensity, and duration scores) and need for
blood transfusions (previous and recent transfusion scores),
compared with nasal pattern. The large pattern presented a
significant correlation with the parameter “frequency of epi-
staxis” (p ⫽ 0.019): 100% of patients with large pattern declared
a frequency of nosebleeds of more than one per day while only
40% of patients with punctate pattern declared the same fre-
quency of epistaxis. The mixed group presented an intermediate
position with 66.67% of patients declaring the maximum fre-
quency of nosebleeds (Fig. 5). No other significant correlations
resulted between morphology and severity of epistaxis.
The same scores were evaluated also considering the num-
ber of involved sites. All patients presented with at least two
sites involved and these were the anterior nasal septum and
the inferior lateral wall. Furthermore, our data presented a
significant correlation between number of involved sites and
the intensity score of epistaxis (p ⫽ 0.011). Eighty percent of
patients with all six subsites involved declared the necessity
for bowl or something similar to collect nosebleeds, and only
50% of patients with two subsites involved declared the same
intensity of epistaxis. Moreover, increase in number of sub-

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Table 3 Characteristics of patients group (men vs women)
Men Women
No. of Patients 46 28
Mean age (yr [SD]) 51.1 关14.11兴 50.6 关11.94兴
Epistaxis
Frequency score (mean 关SD兴) 2.50 关0.72兴 2.46 关0.74兴
Intensity score (mean 关SD兴) 2.63 关0.64兴 2.60 关0.69兴
Duration score (mean 关SD兴) 2.06 关0.88兴 2.07 关0.77兴
Blood transfusions
Previous transfusions score (mean 关SD兴) 1.32 关0.60兴 1.46 关0.79兴
Recent transfusions score (mean 关SD兴) 1.17 关0.44兴 1.28 关0.66兴
Nasal pattern
Punctate pattern (%) 10 (21.74) 15 (53.57)
Mixed pattern (%) 29 (63.04) 10 (35.72)
Large pattern (%) 7 (15.22) 3 (10.71)
Number of sites involved (mean 关SD兴) 4.76 关1.08兴 4.11 关1.10兴

Figure 4. Distribution of telangiectasias among nasal subsites.

sites involved seemed to correlate with a greater intensity of
nosebleeds (Fig. 6).
DISCUSSION
The term “HHT” was first proposed by Hanes in 1909 to
define the hemorrhagic syndrome with hereditary pattern
first recognized and described by Rendu, Osler, and Weber
between 1896 and 1907. HHT has a wide geographic distri-
bution among many ethnic and racial groups but white pa-
tients seem to be primarily affected. No significant difference
concerning prevalence of the disease in nasal mucosa has been
recognized between men and women.1
HHT is an autosomal dominant disease with an almost
complete age-related penetrance. Mutations that cause HHT
have been identified in at least two different genes, defining
two forms of the disease, HHT1 and HHT2, clinically indis-
tinguishable. HHT1 patients have mutations in the ENG gene
(chromosome 9q34.1) coding for endoglin (CD105), a TGF-␤
type III receptor,8–10 whereas HHT2 patients have mutations
in ACVRL1 (chromosome 12q11–14), coding for the activin-
receptor II–like kinase 1.11,12 Mutations in both genes are
thought to be responsible of aberrant TGF-␤1 signaling inter-
fering at some stage during vascular development and hemo-
stasis. This interference is considered to be the cause of a
vascular structure abnormality leading to characteristic mu-
cocutaneous telangiectasias and systemic arteriovenous mal-
formations (AVM). Histologically, in venules excessive layers
of smooth muscle cells without elastic fibers or incomplete
layer of smooth muscle cells have been described.1 These
structural abnormalities, associated with additional defects in
endothelial junction, seem to be responsible for telangiectasia
appearance and development of AVMs.

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 Figure 5. Frequency score of epistaxis considering nasal pattern.

Figure 6. Intensity score of epistaxis considering number of subsites involved.

As established in 1999 by the Scientific Advisory Board of
the HHT Foundation International, Inc., the criteria for diag-
nosis of HHT are based on the four main clinical features of
the disease comprising spontaneous recurrent nosebleeds,
presence of mucocutaneous telangiectasias, visceral involve-
ment, and an affected first-degree relative.5 These criteria are
also known as the “Curaçao criteria.” A “definite HHT” is
defined where at least three of these parameters are present, a
“suspected HHT” in patients with two criteria verified, an
“unlikely HHT” where only one criterion is identifiable.
Spontaneous and recurrent bleeding from telangiectasias
localized in nasal mucosa is the most common clinical mani-
festation of HHT and occurs in ⬃96% of individuals affect-
ed.1,13 Furthermore, recurrent nosebleed is the first clinical
sign of this disease in ⬃80% of cases and is present in ⬎50%
of patients before the age of 20 years.2 This clinical manifes-
tation has a great impact on quality of life in HHT patients
and it is referred to as the most important impediment in
daily activities; duration of epistaxis, more than frequency,
seems to play a major role in the physical aspects of quality
of life.14 Despite the high prevalence of nosebleeds, fre-
quency and severity of epistaxis in these patients are very
variable and although some patients experience daily oc-
currence of epistaxis or more, others report only occasional
nosebleeds.
The severity of epistaxis is hardly measurable because of
the difficulties in accurate quantifying of intensity of bleeds.
Frequency of epistaxis, with its variability, is difficult to de-
fine too. During the past years, some authors6,7,15,16 have tried
to cross these limits, evaluating different parameters. For the
present work we decided to consider the main previous used
parameters, so as to evaluate the severity of epistaxis and its
possible relationship with nasal morphology and distribution
of telangiectasias.
Similar to the systemic manifestation of the disease, the
presence of endonasal telangiectasias can be very variable too.
Despite this consideration, relatively few works have tried to
define the variability in nasal distribution and morphology of

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telangiectasias. The reason for this lack is probably because of
the objective difficulties in observing completely the walls of
the nasal fossae. The introduction of nasal endoscopy has
contributed to the development of a more complete approach
in evaluating the nasal manifestations of the disease. In 1996
Haitjema et al.2 examined 58 patients with definite HHT by
nasal endoscopy to determine the presence or absence of
telangiectasias. The authors considered as subsites in nasal
fossae the vestibulum nasi, the nasal septum, the middle
turbinates, the inferior turbinates, and the posterior nasal
mucosa. For each site, they defined three degrees for the
presence of telangiectasias: “few,” with ⬍5 lesions; “moder-
ate,” with 5–25 lesions; and “many,” with ⬎25 lesions. The
greater concentration of telangiectasias was found at nasal
septum and the inferior turbinates. Severity of epistaxis and
morphology of telangiectasias was not considered. In 2005
Folz et al.3 examined 21 patients with HHT for endonasal
telangiectasias by videoendoscopy with rigid endoscopes.
The authors described for the first time the presence of wide
variations in the morphology of telangiectasias. In addition,
nasal endoscopies at different time intervals showed that
telangiectasias increase in size and have the tendency to con-
fluence. Gender did not seem to influence the morphology of
nasal lesions. Distribution of the lesions was described as
predominant within the anterior nasal cavity and with overall
density higher in patients ⬎40 years of age. The authors also
emphasized the difficulty in delineating the exact number of
singular telangiectasias because of the confluent nature of the
lesions. As in Haitjema’s work,2 severity of epistaxis was not
considered. In 2006 Mahoney et al.4 proposed a classification
of nasal vasculature patterns. The authors reviewed the
records of 40 patients affected by HHT who underwent nasal
endoscopy and Nd:YAG laser photocoagulation. They de-
fined three different patterns: type I, in patients who pre-
sented isolated punctate telangiectasias or individual small
arteriovenous malformations; type II, in patients who pre-
sented diffuse interconnecting vasculature with “feeder” ves-
sels; type III, in patients with large solitary arteriovenous
malformations, which may be associated with scattered telan-
giectasias. The most common nasal vasculature patterns were
types I and II. No significant difference in mean age was
observed among the vascular pattern groupings. The authors
also tried to evaluate a possible correlation between severity
of epistaxis and nasal morphology. Severity of nosebleeds
was evaluated using Rebeiz classification considering mild,
moderate, or severe degrees of epistaxis. In type II patients,
the authors underlined a higher disease severity, associated
with extended transfusions need and reduced response to the
Nd:YAG laser treatment. However, no analysis on distribu-
tion of telangiectasias among the nasal fossae was performed.
Considering the experiences of the previous cited works,
our impression is that defining nasal manifestation of the
disease requires analysis of both morphology and distribution
of telangiectasias considered together. In fact, a wide variabil-
ity can be found not only in the nasal pattern of telangiectasias
but also in the number of nasal subsites involved, even re-
garding patients with the same pattern. Furthermore, in our
experience we observed that different subsites in nasal fossae,
and even the same subsite, can present similar or different
patterns. Concerning morphology of nasal telangiectasias, we
observed the presence of endoscopically flat punctate and
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prominent large lesions, configuring two distinct basic pat-
terns; these patterns can be partially considered similar, re-
spectively, to type I and type III following the classification
proposed by Mahoney and coworkers.4 We observed also that
every basic pattern can present itself as isolated lesions or can
show tendency to confluence, as observed by Folz et al.,3 but
maintaining the flat or prominent aspect of the single compo-
nents composing the “interconnected vasculature.” Mahoney
and coworkers defined as “type II” patients who presented
diffuse interconnecting vasculature; we think that this group
can be divided on the basis of our observations considering
the single components (flat, large, or mixed). In the present
work confluence was observed in 37 patients (50%) with equal
distribution between sex and age groups; however, we ob-
served no significant correlation between the presence of con-
fluence and the severity of epistaxis or the need for blood
transfusions, at variance with Mahoney, who reported in-
creased transfusions request in their type II patients. To cor-
relate, in some way, the nasal pattern with its distribution in
the nasal cavities examining all of the subsites, we propose to
define three types of patients: “punctate pattern,” when all
subsites involved present only flat punctate telangiectasias;
“large pattern,” when all subsites involved present only
prominent large lesions; and the “mixed pattern,” when both
flat punctate and prominent large telangiectasias are present
in the nasal fossae.
In the present work we classified our patients following
this proposal and evaluated any possible correlation between
nasal pattern and severity of epistaxis and need for blood
transfusions. The “mixed pattern” result was the most repre-
sented (52.7% of patients), whereas “large pattern” was least
represented (13.5%), similar to Mahoney’s observations on
type III.4 In the female group there was a relative and signif-
icant (p ⫽ 0.019) predominance of the punctate pattern
(53.57% in women versus 21.74% in men). This difference was
not previously reported; the high mean age of our female
group (50.6 years) likely excludes a recent role of hormonal
gender differences; confirmation of this finding on a larger
sample and further investigations on its etiology are needed.
Concerning severity of epistaxes, we observed a signifi-
cantly higher frequency of nosebleeds in patients presenting
the “large pattern” (p ⫽ 0.019), which, in our opinion, can be
due to a higher intrinsic fragility of telangiectasias endoscop-
ically appearing as large and prominent. In fact, the interme-
diate position of patients with “mixed pattern” seems to con-
firm the presence of large prominent telangiectasias as a
worsening factor on the frequency of nosebleeds. All of our
patients, as other authors previously described, evidenced
telangiectasias at the anterior portion of nasal fossae (nasal
septum and inferior lateral wall) but an increase in number of
subsites involved resulted, in our patients, in worsening of the
intensity of epistaxis (p ⫽ 0.011). In fact, grade 3 in the “the
intensity score” was steadily more represented with increas-
ing number of subsites involved. This result and the absence
of correlation between nasal morphology of lesions and in-
tensity of bleeding are to be interpreted. It is author’s though
that increasing in the telangiectasias’ number and subsites’
involvement may lead to a higher risk of concurrent multiple
bleeding from different lesions by the same causative effect.
Another possible explanation may be researched in the addi-
tional involvement of the posterior part of the nasal cavities;
57
in this case, the impossibility to pad effectively the bleeding
point by an external digital compression, as for anterior epi-
staxis, may lead to a higher loss of blood. Surprisingly, we did
not find a significant correlation between duration of nose-
bleeds and need for blood transfusions with a nasal pattern of
HHT; this observation suggests the concurrence of other pos-
sible (genetic or environmental) factors to determine their
final severity.
CONCLUSIONS
Spontaneous and recurrent bleeding from telangiectasias
localized in the nasal mucosa is the most common clinical
manifestation of HHT and has a great impact on quality of life
in HHT patients. Frequency and severity of epistaxis in these
patients are very variable. Recently, some authors2–4 showed
that nasal manifestations of HHT can be variable too. In this
work we proposed a new endoscopic classification of nasal
telangiectasias establishing a significant correlation with the
severity of epistaxis. Our data suggest a role of morphology
and distribution of telangiectasias in determining frequency
and intensity of nosebleeds. Consequently, our impression is
that to reduce frequency and intensity of epistaxis in HHT
patients, treatments should be directed also on lesions located
in the posterior part of nasal fossae and especially on telan-
giectasias endoscopically appearing as large and prominent.
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