The Laryngoscope
Lippincott Williams & Wilkins, Inc.
© 2005 The American Laryngological,
Rhinological and Otological Society, Inc.
Outcome of Septal Dermoplasty
in Patients With Hereditary
Hemorrhagic Telangiectasia
Maria Luisa Fiorella, MD; Douglas Ross, MD; Katharine J. Henderson, MS; Robert I. White, Jr., MD
Objectives/Hypothesis: Septal dermoplasty has
been recommended as the treatment of choice for life-
threatening epistaxis in patients with hereditary
hemorrhagic telangiectasia. The purpose of the study
was to evaluate the effectiveness and outcomes of
septal dermoplasty for management of transfusion-
dependent epistaxis. Study Design: Retrospective
study. Methods: Between 1994 and 2004, septal dermo-
plasty was performed on 67 consecutive patients with
severe epistaxis attributable to hereditary hemor-
rhagic telangiectasia. The numbers of units of blood
received 1 year before and 1 year after septal dermo-
plasty were obtained. A subjective appraisal of the
results of the surgery as well as second procedures
after septal dermoplasty was determined. Patients
were screened for pulmonary and cerebral arterio-
venous malformations, gastrointestinal tract bleed-
ing, and symptomatic liver disease. Results: Data
were obtained in 66 of 67 (98%) patients with a mean
age of 61.5 years (mean follow-up, 3.9 y). Accurate
transfusion requirements 1 year before and 1 year
after septal dermoplasty were available in 32 of 66
(48%) patients. In these 32 patients, the mean units of
blood received decreased from 21 units (range, 2–100
units) 1 year before septal dermoplasty to 1 unit
(range, 0 –10 units) in the year after septal dermo-
plasty (P < .001). Improved quality of life was claimed
in 57 patients. Second therapies, ranging from cau-
tery to repeat partial septal dermoplasty, were re-
quired in 15 patients during follow-up. Among the 67
patients, 31 (46%) had pulmonary arteriovenous mal-
formation, 14 (21%) had gastrointestinal tract bleeding,
From the Department of Otolaryngology (M.L.F.) Universita‘ degli
Studi di Bari, Bari, Italy; and the Sections of Otolaryngology (D.R.), De-
partment of Surgery, and Interventional Radiology (K.J.H., R.I.W.), Depart-
ment of Diagnostic Radiology, Yale University School of Medicine, New
Haven, Connecticut, U.S.A.
Supported in part by March of Dimes grant HHT-FY04 – 677 and
Josephine Lawrence Hopkins Foundation and General Clinical Research
Center NIH M01-RR-00125 grant (R.I.W. and K.J.H.).
Editor’s Note: This Manuscript was accepted for publication August
3, 2004.
Send Correspondence to Douglas Ross, MD, Section of Otolaryngol-
ogy, Department of Surgery, Yale University School of Medicine, Yale
Physician’s Building, 4th Floor, 800 Howard Avenue, New Haven, Con-
necticut 06519, U.S.A. E-mail: douglas.ross@yale.edu
DOI: 10.1097/01.mlg.0000154754.39797.43
Laryngoscope 115: February 2005
7 (10%) had symptomatic liver disease, and 5 (7%) had
cerebral arteriovenous malformation. During the
follow-up, 14 patients died of other complications of he-
reditary hemorrhagic telangiectasia (11 patients) and
unrelated causes (3 patients). Conclusion: Septal dermo-
plasty remains an effective way of reducing transfusion
requirements in patients with hereditary hemorrhagic
telangiectasia and subjectively improves their quality
of life. The otolaryngologist caring for patients with he-
reditary hemorrhagic telangiectasia should be familiar
with other organ involvement by hereditary hemor-
rhagic telangiectasia to prevent complications during
surgery. Key Words: Septal dermoplasty, epistaxis, he-
reditary hemorrhagic telangiectasia.
Laryngoscope, 115:301–305, 2005
INTRODUCTION
Hereditary hemorrhagic telangiectasia (HHT) is an
uncommon systemic autosomal dominant disorder of an-
giogenesis characterized by the presence of vascular tel-
angiectases in mucocutaneous tissues, visceral organs,
and the central nervous system.1 Epistaxis, caused by
spontaneous bleeding from telangiectases of the nasal mu-
cosa, is the most common manifestation of HHT. It may be
so severe as to require multiple transfusions and intrave-
nous iron supplementation. Bleeding becomes more severe
in later decades in approximately two-thirds of affected
persons.2 Although nasal manifestations of HHT are well
known to otolaryngologists, many of the other organ man-
ifestations of this disorder are less well known and may
pose issues for the physician treating the patient with
HHT.
Mild and moderate epistaxis is treated by hormonal
therapy with estrogens,3 chemical cautery and electrocau-
tery, and laser coagulation.4 Life-threatening epistaxis,
which is associated with HHT, has been treated with
varying success using embolization,5 surgical ligation,6
Young’s surgical closure of the nostrils by a skin flap,7 and
microvascular free flaps.8
William Saunders pioneered a skin grafting tech-
nique for severe epistaxis in 1958; since then, this tech-
nique has undergone modification.9 The principle of this
procedure is to replace the fragile respiratory mucosa of
the nose with strong keratinized split-thickness skin
Fiorella et al.: Outcome of Septal Dermoplasty
301
grafts from the thigh that resist trauma and thereby pre-
vent bleeding.10
Since the early 1990s, the senior otolaryngologist
(D.R.) has used the Saunders method of septal dermoplasty
(SD), with his own modifications, to control transfusion-
dependent epistaxis.11 The purposes of the present study
were to describe indications and new variations of SD, to
characterize the phenotype of patients requiring this ther-
apy, and to report the long-term outcome of SD.

PATIENTS AND METHODS

The Yale Arteriovenous Malformation Center is an interdis-
ciplinary center of physicians caring for patients with HHT. From
a population of approximately 250 patients seen yearly, 67 con-
secutive patients with severe, debilitating epistaxis were referred
to the otolaryngologist and underwent SD. Human Investigations
Committee approval was obtained for a retrospective study that
included contacting all patients, as well as their primary care
physicians. Detailed information about total transfusions (units
of blood) 1 year before and 1 year after SD was requested. A
subjective appraisal of the results of the surgery was asked of the
patients and their families. Second therapies for control of nose-
bleeds after SD were also tabulated. Patients in the present study
were seen by the genetic counselor and senior physician in the
center and screened for pulmonary and cerebral arteriovenous
malformations12,13 and symptomatic liver disease.14

Surgical Technique
Surgical technique is shown in Figures 1 and 2. With the
patient under general endotracheal anesthesia, a 3 ⫻ 7-inch
split-thickness graft is taken from the lateral aspect of the right-
side thigh, using a Zimmer air dermatome on a setting of
141000of an inch. Xeroform and Telfa with Kerlex-wrapped
dressing are applied on completion of harvesting of the skin and
the graft, which is preserved in a saline-soaked sponge.
Bilateral inferior turbinectomies are performed in the fol-
lowing manner. Neo-Synephrine is placed into the nose for ade-
quate vasoconstriction; then the inferior turbinates are infil-
trated with 1% lidocaine and 1:100,000 epinephrine. The
turbinate is medialized and removed at its base using an anterior
straight scissor under endoscopic view, cutting anteroposteriorly,
as much as possible toward the lateral nasal wall. The free edge of
the remnant of the turbinate is then cut down using microderider. Fig. 1. (A and B) Sagittal views. (A) Nasal mucosa is removed with
a Faulkner curette (lateral wall). The nasal mucosa is resected, and
Meticulous hemostasis is achieved using Bovie electrocautery.
an inferior turbinectomy is performed. (B) The graft has been su-
The septal dermoplasty is performed. The nasal vestibule is tured anteriorly and applied to lateral wall.
injected with 1% lidocaine and 1:100,000 epinephrine. After 7
minutes a narrow circumferential strip of vestibular skin is ex-
cised from the septum and the floor and from the lateral wall of
both nostrils at the mucocutaneous junction. As much mucosa as 0.5 ⫻ 3-cm Neuropatties treated with mupirocin calcium oint-
possible is removed using a Faulkner curette, taking care to ment (Bactroban, Galaxo Smith Kline, Philadelphia, PA) are used
preserve the perichondrium of the septum that must nourish the for this task. The first pack is placed along the floor of the nose to
new grafts and the septal cartilage. anchor the graft inferiorly; then a second piece is placed superi-
Bleeding is controlled by the topical application of cotton orly, continuing in this alternate fashion until the nose is packed
soaked in epinephrine in one nostril and continuing the operation firmly. Packing is removed only after 5 days, when the grafts are
in the opposite side. Once the bleeding has slowed, the curetting well attached.
is continued until one is assured of adequate removal of mucosa. There is often “temporary” nasal obstruction after the packs
The skin graft is sutured in a U shape. A stitch is placed at are removed, because of excess skin at the posterior part of the
the inferior aspect of the rim incision with interrupted 3-0 chro- nose. This acts as an avascular small flap that eventually disin-
mic suture, and the skin graft is sutured circumferentially tegrates. When this occurs, the nasal airways improve.
around the rim incision of the nasal vestibule. The skin graft is
then inserted into the nose with a No. 2 bayonet forceps, and a Follow-Up Care
long nasal speculum is used to hold it in place for packing. Nothing more is performed for 10 to 14 days after surgery so
that the skin graft can become firmly attached to the nasal walls.
Packing and Removal During follow-up, cautious suctioning of crusts is performed to
An important aspect is careful nose packing, to avoid the facilitate breathing and success of graft take. Skin is not meant to
displacement of the skin graft while healing takes place. Multiple be in the nose, and crusting from desquamation frequently devel-

Laryngoscope 115: February 2005 Fiorella et al.: Outcome of Septal Dermoplasty

302

Fig. 2. (A and B) Coronal views. In 2002, we began performing
routine turbinectomies followed by mucosal resection. (A) The ex-
tent of mucosal resection before (right side) and after (left side) the
introduction of turbinectomies is evident. (B) The extent of skin graft
and packing placement both before (right side) and after (left side)
the introduction of turbinectomies are evident.
ops, requiring that the patient provide vigorous nasal hygiene.
Crusting may lead to nasal obstruction and odor; therefore, the
nose must be cleaned once or twice daily. Cleaning is facilitated
using cotton-tipped applicators and moisturizing substances such
as mineral oil, Borofax ointment, half hydrogen peroxide and half
water, or soap and water. Saline irrigations can also be used. This
cleaning cannot injure the skin, and it cannot be scraped off.
RESULTS
Between 1994 and 2004, SD was performed in 67
patients (30 men and 37 women) (Table I). Data were
obtained in 66 of 67 patients (98%). The mean age at the
time of SD was 61.5 years (age range, 35– 80 y). Transfu-
sion requirements either before or after SD were available
in 50 of 67 patients (75%). Complete data before and after
SD were available in 32 of 66 patients (48%) of this group.
Table II In 66 of 67 patients (98%), the mean follow-up
period was 3.9 years (range, 2 mo–9.8 y). Quality-of-life
information was available for all living patients and from
their families for patients who had died.
TABLE I.
Clinical Phenotypes in 67 Patients Undergoing
Septal Dermoplasty.
Male n ⫽ 30
Female n ⫽ 37
Age (y)
Mean 61
Range 35–80
Other organ: n (%)
Pulmonary arteriovenous malformation 31 (46%)
Cerebral arteriovenous malformation 5 (7%)
Gastrointestinal tract bleeding 14 (21%)
Symptomatic liver disease 7 (10%)
Laryngoscope 115: February 2005
TABLE II.
Transfusion Requirements Before and After Septal Dermoplasty.
No. of patients requiring blood transfusion before SD 38
No. of patients requiring blood transfusion after SD 11
No. of units of blood received before SD*
Mean 21
Range 2–100
No. of units of blood received after SD*
Mean 1
Range 0–10
*Data from 32 patients with accurate determination of blood before and
after septal dermoplasty (SD).
Wilcoxon signed rank test, P ⬍ .001.
Transfusion Requirements and
Quality-of-Life Outcomes
Thirty-eight of the 50 patients required blood trans-
fusions before SD, and 11 required them after SD (Table
II). In 32 patients we were able to determine the exact
number of units of blood received before and after SD. In
these 32 patients, the mean units of blood received de-
creased from 21 units in the year before SD (range, 2–100
units) to 1 unit (range, 0 –10 units) during the year after
SD. The Wilcoxon signed rank test demonstrated a signif-
icant decrease in the number of transfusions in these
patients (P ⬍ .001).
We were able to question 57 patients or families of
patients about quality of life: 53 reported that their qual-
ity of life had improved after SD, and 4 reported no im-
provement. Twenty-four of the 57 patients (42%) reported
no epistaxis (mean follow-up, 2 y [range, 2 mo– 8 y]); 29
patients (51%) reported decreased frequency and duration
of epistaxis (mean follow-up, 2 y, 9 mo [range, 1 mo– 8 y]),
and 4 patients (7%) had not improved since surgery (Table
II).
Clinical Phenotype
Other organ involvement. Pulmonary arterio-
venous malformations were present in 31 of 66 patients
(47%), and cerebral arteriovenous malformations were
found in 5 of 66 patients (7%). Symptomatic liver disease
was present in 7 of 66 patients (10%) (Table I). In addition,
14 patients (21%) had gastrointestinal tract bleeding be-
fore or after SD. Two patients had a prosthetic heart valve
and tolerated coumadin well after SD.
Deaths during follow-up. Fourteen of 66 patients
(21%) died during the follow-up after SD. Five of these
patients had gastrointestinal tract bleeding but died of
heart disease.
In 3 of 14 patients, death was attributed to pulmo-
nary hypertension associated with HHT. Liver disease
and intractable congestive heart failure were the causes of
death in 2 of 14 patients, respectively, and bleeding from
esophageal varices (1 of 14 patients) accounted for the
additional death in this group. Three of 14 patients died of
colon cancer, pancreatitis and pneumonia, and lung can-
cer, respectively.
Fiorella et al.: Outcome of Septal Dermoplasty
303
Second therapies after septal dermoplasty.
Multiple additional therapies have been performed in
15 patients (laser therapy in 10 patients, a second SD in 6,
embolization in 2, and maxillary artery ligation in 1 pa-
tient). Many of these therapies were performed at other
institutions because some of the patients did not return to
our center for follow-up.
DISCUSSION
Technique History and Modifications
Since the early 1960s, surgical techniques for man-
aging debilitating nosebleeds in HHT patients have
evolved, but epistaxis of this magnitude is still problem-
atic. Many authors have proposed alternative procedures
including laser cauterization, embolization, maxillary ar-
tery ligation, Young’s procedure, and microvascular free
flaps. Although each method has advocates in these pa-
tients, no method has provided a complete cessation of
epistaxis, and reporting of long-term outcomes in these
patients has been limited.
Currently, the most credible technique is septal der-
moplasty, first described by Saunders9 in 1958. Replace-
ment of the fragile nasal mucosa with a skin-thickness
graft from the thigh renders the nose more resistant to
trauma without altering the nasal respiratory function.
However, good result of SD depends not only on quantity
of mucosa covered by the skin graft but also on the
follow-up care. Skin graft desquamation produces odor
and crusting; therefore, daily cleaning is necessary to
avoid nasal obstruction and nose bleedings.
Presence of telangiectases in nasal mucosa not cov-
ered by the graft, as well as graft contracture, can cause
postoperative epistaxis, which is treatable by laser tech-
nique. Septal dermoplasty may be repeated in patients
with recurrent severe epistaxis. Modification of the tech-
nique has been performed recently to facilitate the skin
graft positioning, cleaning, and maintenance of airway by
inferior bilateral turbinectomies.11 Septectomy has been
useful for one patient with profuse nosebleeds and septal
perforation.
Outcomes
Our study is a large retrospective review of patients
with severe epistaxis attributable to HHT that was
treated by SD. To date, the data on SD reported in the
literature have been based on a small number of patients
and the duration of follow-up has been short. Our out-
comes are longer than previously reported and are based
on transfusion requirements before and after SD, as well
as subjective questioning of the patient or family. Cessa-
tion or marked reduction of epistaxis occurs in 90% of
patients with HHT during the first 2 years after SD. Four
patients did not benefit from SD. Our clinical experience
has demonstrated that, beyond the obvious health dam-
age, epistaxis of this magnitude seriously affects the qual-
ity of life of the patient with HHT.
Limitations of our study include the retrospective
design and the recall bias associated with the quality-of-
life assessment. Because some of our patients developed
repeat bleeding attributable to new telangiectases or par-
Laryngoscope 115: February 2005
304
tial graft take and have received adjunctive therapy at 1
year, we currently recommend that all patients be seen 1
year after surgery. Management of recurrent epistaxis by
laser or second partial SD is able to extend the time of
improvement and emphasizes the importance of follow-up
by the otolaryngologist performing the SD.
Importance of Phenotype
Many patients in the present series have had other
serious organ involvement, which ultimately contributes
to their disability and mortality. Symptoms such as dys-
pnea, fatigue, and congestive heart failure can be mani-
festations of pulmonary arteriovenous malformations, he-
patic arteriovenous malformations, gastrointestinal tract
bleeding, and iron deficiency anemia or some combination
of all of theses.15 In addition, in patients with pulmonary
arteriovenous malformations, the lung no longer has a
capillary filter, and small blood clots, bacteria, and, occa-
sionally, air can pass directly through the pulmonary ar-
teriovenous malformation into the systemic circulation,
causing ischemic events in various organs.1 Intracerebral
hemorrhages may also occur as a result of cerebral or
spinal arteriovenous malformations.1 In our study, three
patients had primary pulmonary hypertension, which is a
syndrome newly recognized in HHT.16
Patients with a history of pulmonary arteriovenous
malformations are prone to air or clot emboli during in-
travenous infusions. Antibiotics should be given prophy-
lactically for procedures. In most instances, the otolaryn-
gologist is the first physician to see a patient with HHT
because epistaxis is the most common symptom.5 First-visit
patients should be screened for other organ involvement.
Although imperfect, the modified Saunders dermo-
plasty, coupled with follow-up care at 1 year and in sub-
sequent years, is the best therapy for this subset of pa-
tients. In the near future, many new pharmacological
agents, with anti-angiogenesis properties will be devel-
oped and, perhaps, may offer a possible nonsurgical solu-
tion; However, until that time, SD that is specific for HHT
should be the mainstay for transfusion-dependent epistaxis.
CONCLUSION
The modified SD for transfusion-dependent epistaxis
in patients with HHT provides excellent palliation and
improves quality of life. The need for transfusions was
significantly reduced in the subset of 32 patients with
accurate blood transfusion records 1 year before and 1
year after SD. All patients with HHT who are undergoing
SD should be seen 1 year after SD because in as many as
25% of patients, laser or other secondary procedures will
be necessary. The otolaryngologist treating patients with
HHT should be aware of other organ manifestations,
which may influence management of the patient during
and after SD.
Acknowledgment
The authors thank James Dziura for his assistance
with data analysis.
Fiorella et al.: Outcome of Septal Dermoplasty
BIBLIOGRAPHY
1. Guttmacher A, Marchuk D, White R. Hereditary hemorrhagic
telangiectasia. N Engl J Med 1995;333:918 –924.
2. Ross D, Jassin B. Current trends in the diagnosis and man-
agement of Osler-Weber-Rendu disease (hereditary hem-
orrhagic telangiectasia). Curr Opin Otolaryngol Head Neck
Surg 1997;5:191–196.
3. Sadick H, Ramin N, Oulmi J, Karl H, Bergler W. Plasma
surgery and topical estriol: effects on the nasal mucosa and
long-term results in patients with Osler’s disease. Otolar-
yngol Head Neck Surg 2003;129:233–238.
4. Shah RK, Dhingra JK, Shapshay SM. Hereditary hemor-
rhagic telangiectasia: a review of 76 cases. Laryngoscope
2002;112:767–773.
5. Elden L, Montanera W, terBrugge K, Willinsky RA, Lasjau-
nias P, Charles D. Angiographic embolization for the treat-
ment of epistaxis: a review of 108 cases. Otolaryngol Head
Neck Surg 1994;111:44 –50.
6. Golding-Wood PH. The role of arterial ligation in intractable
epistaxis. J Laryngol Otol Suppl 1983;8:120 –122.
7. Young A. Closure of the nostrils in atrophic rhinitis. J Lar-
yngol Otol 1967;81:515–524.
8. Bridger GP, Baldwin M. Microvascular free flap in hereditary
hemorrhagic telangiectasia. Arch Otolaryngol Head Neck
Surg 1990;116:85– 87.
9. Saunders WH. Septal dermoplasty for control of nosebleeds
Laryngoscope 115: February 2005
caused by hereditary hemorrhagic telangiectasia or septal
perforations. Trans Am Acad Ophthalmol Otolaryngol
1960;64:500 –506.
10. Saunders W. Hereditary hemorrhagic telangiectasia: effec-
tive treatment of epistaxis by septal dermoplasty. Acta
Otolaryngol 1964;58:497–502.
11. Ross DA, Nguyen DB. Inferior turbinectomy in conjunction
with septodermoplasty for patients with hereditary hem-
orrhagic telangiectasia. Laryngoscope 2004;114:779 –781.
12. Nanthakumar K, Graham AT, Robinson TI, et al. Contrast
echocardiography for detection of pulmonary arteriovenous
malformation. Am Heart J 2001;141:243–246.
13. Moussouttas M, Fayad P, Rosenblatt M, et al. Pulmonary
arteriovenous malformations: cerebral ischemia and neu-
rologic manifestations. Neurology 2000;55:959 –964.
14. Garcia-Tsao G, Korzenik JR, Young L, et al. Liver disease in
patients with hereditary hemorrhagic telangiectasia.
N Engl J Med 2000;343:931–936.
15. Longacre AV, Gross CP, Gallitelli M, Henderson K, White R,
Proctor D. Diagnosis and management of gastrointestinal
bleeding in patients with hereditary hemorrhagic telangi-
ectasia. Am J Gastroenterol 2003;98:59 – 65.
16. Trembath RC, Thomson JR, Machado RD, et al. Clinical and
molecular genetic features of pulmonary hypertension in
patients with hereditary hemorrhagic telangiectasia.
N Engl J Med 2001;345:325–334.
Fiorella et al.: Outcome of Septal Dermoplasty
305