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Laporan Kasus Woakes syndrome

Article · October 2014

DOI: 10.32637/orli.v44i1.86

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RLI Vol. 44 No.1. Tahun 2014 ORLI Vol. 44 No.1. Tahun 2014
ORLI Vol. 44 No.1. Tahun 2014 Woakes syndrome Woakes syndrome ORLI Vol.
Woakes 44 N
syndrom

Laporan Kasus

Woakes syndrome

Retno Sulistyo Wardani, Ika Dewi Mayangsari, Trimartani Koento, Ervin Amouzegar
Otorhinolaryngology Head and Neck Surgery Department
Faculty of Medicine University of Indonesia – Dr. CiptoMangunkusumo Hospital
Jakarta

ABSTRACT
Background: Extensive nasal polyp growth in the paranasal sinuses can lead to bone erosion of
the sinus walls and cause facial disfigurement due to continuous pressure or chronic inflammation. This
extremely rare phenomenon is called Woakes syndrome. This syndrome consist of several symptoms
include the destruction of ethmoid sinus that cause broadening of the bridge of the nose, frontal sinus
aplasia and bronchiectasis. Purpose: To give complete information about the diagnosis and management
of Woakes Syndrome. Case: A 16-year-old boy with deformity of the left nose, nasal obstruction and
frequent episodes of rhinorrhea since 4 months before admission. Nasoendoscopic evaluation showed
huge nasal polyps filling the left nasal cavity, pushing the septum and narrowing the right nasal cavity.
Histopathology result was edematous polyp with necrosis and massive bleeding without signs of
malignancy. Management: Patient was managed in two stages operations. First, nasal polyp removal
by FESS technique in general anesthesia, and the second stage four months later, was septorhinoplasty
for aesthetic bridge reconstruction. Conclusion: Nasal polyps could be related to Woakes syndrome,
characterized by broadening of nasal bridge which needs functional and aesthetic surgery.

Keyword: Woakes Syndrome, nasal polyps, Functional Endoscopic Sinus Surgery,Septorhinoplasty

ABSTRAK
Latar Belakang: Polip hidung besar yang meluas dalam sinus paranasal dapat menyebabkan
erosi dinding sinus dan menyebabkan cacat wajah akibat tekanan terus-menerus atau peradangan
kronis. Fenomena ini sangat langka dan disebut sebagai sindrom Woakes. Sindrom ini terdiri dari
beberapa gejala termasuk kerusakan dinding sinus etmoid yang menyebabkan hidung melebar, aplasia
sinus frontal dan bronkiektasis. Tujuan: Untuk memberikan informasi yang lengkap tentang diagnosis
dan penatalaksanaan Woakes Syndrome. Kasus: Seorang anak laki-laki 16 tahun dengan deformitas
hidung kiri, hidung tersumbat dan pilek berulang sejak 4 bulan. Evaluasi nasoendokopi menunjukkan
polip hidung masif mengisi rongga hidung kiri, mendorong septum dan menyempitkan rongga hidung
kanan. Pemeriksan histo-patologi memperlihatkan polip edematosa dengan nekrosis dan perdarahan
masif tanpa tanda-tanda keganasan. Penatalaksanaan: Pada pasien dilakukan dua tahap tindakan.
Pertama, dilakukan Bedah Sinus Endoskopik Fungsional (BSEF) dan polipektomi dalam anestesi umum,
dan empat bulan kemudian pasien menjalani septorinoplasti untuk rekonstruksi wajah. Kesimpulan:
Polip hidung pada kasus ini kemungkinan terkait dengan sindrom Woakes, ditandai dengan pelebaran
pyramid hidung yang membutuhkan tindakan operasi fungsional dan estetika.

Kata kunci: sindroma Woakes, polip hidung, Bedah Sinus Endoskopik Fungsional, Septorinoplasti.

Correspondence address: DR. Dr. Retno S. Wardani, SpTHT-KL(K), Rhinology Division ENT
Department Faculty of Medicine University of Indonesia, retno.wardani@ui.ac.id

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ORLI Vol. 44 No.1. Tahun 2014
INTRODUCTION
Woakes syndrome or ethmoiditis was first
described by Woakes in 1885. The clinical
hallmark of this entity with severe recurrent
growth of nasal polyposis is broadening of the
nose and hypertelorism. Polyps in Woakes’
syndrome do not differ histologically from
nasal polyps but are more often caused by a
non-eosinophilic dominated inflammation.1
Woakes syndrome is a very rare case. No
more than 10 cases had been reported in the
published journals. Abud-Neme et al in 1986
reported one case of 17-year-old girl with
Woakes syndrome who underwent resection
of polyps and fibrous tissue and reconstruction
of the nasal pyramid. Kellerhals et al in
1977 reported a 5-year-old-girl with Woakes
syndrome managed with polyp resection and
planned for reconstruction after puberty.2, 3
The occurrence of nasal polyps is a
multifactorial disease resulting from chronic
immune inflammatory processes within the
paranasal sinuses. The pathophysiological
processes of the underlying inflammation
causing chronic hyperplastic sinusitis and
nasal polyposis are still unclear, and the patho-
etiology of this syndrome is also still obscure.
Some authors have suggested genetic factors,
as the syndrome has occurred more frequently
among sisters, as mentioned by Groman et
al,1,4 and others implied infectious origin,
primarily syphilitic. External noxious agents
and allergies, could accelerates the growth of
polyps. However, in many cases, no agents or
allergy could be found. This indicates that the
syndrome with its deforming and recurrent
polyps is only a clinical entity. The extreme
broadening of the nose is explained as the
result of the chronic pressure of the polyps.
The chronic pressure also leads to a bolding
of the lamina papyracea with progressive
protrusiobulbi to the lateral side and to a
risk of slowly progressing blindness due to
compression of the optic nerve.1
Woakes syndrome Woakes syndrome Woakes syndrom
The etiology of nasal polyps is unknown.
Some theories consider polyps as consequence
of conditions which cause chronic inflammation
in the nose and nasal sinuses characterized by
stromal edema and variable cellular infiltrate.
While many aspects have been documented
to support this theory, the initiating cause
remains unknown and might be different in
many cases.1-4 Medical conditions commonly
associated with polyps include asthma,
bronchiectasis, and cystic fibrosis There is
a well recognized subgroup of patients with
Samnter’s Triad comprising polyposis, asthma,
and aspirin hypersensitivity which makes up
almost 10% of cases of nasal polyps.5-8
Extensive polyp growth in the paranasal
sinuses and facial disfigurement due to
continuous pressure or chronic inflammation
and ethmoiditis.The clinical hallmark of
this entity with severe recurrent growth of
nasal polyposis is broadening of the nose and
hypertelorism. The syndrome has also been
described with bronchiectasis in children.1
Caversaccio et al,1 reported following four
characteristics (1) bilateral nasal polyps in the
middle meatus beginning during childhood,
(2) ethmoiditis, (3) hypertrophic process with
nasal pyramid deformation and (4) therapeutic
failure with constant and rapid recurrences.
Patient with Woakes syndrome can
complaint about abundant nasal discharge and
the secretions being of a rubber-like viscosity.
Further examination revealed frontal sinus
aplasia, bronchiectasis with clubbed fingers,
deformation of the nasal bridge and no sign of
allergies.3,4
Laboratory findings showed no signs of
allergic disease. AlI blood examination seems
to be normal. CT scan showed a complete
obliteration of the paranasal sinuses on both
sides with a bulging of the ethmoid walls into
the orbits.1
Management of Woakes syndrome consists
of two stages. The first is by complete
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ORLI Vol. 44 No.1. Tahun 2014 Woakes syndrome Woakes syndrome ORLI Vol.
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resection of the nasal polyps, best executed by Physical examination revealed

Functional Endoscopic Sinus Surgery (FESS) polypoid mass filling the entire nasal cavity
followed by the second stage of reconstruction. with enlargement of the nose. Frontal view
Septorhinoplasty on Woakes syndrome has a appearance showed nose enlargement with no
goal in restoring nasal function by maximizing distinctive border of the left nose. From the basal
nasal airflow and improving cosmetic view appearance, the tip of the nose is deviated
appearance.9 to the right side. Sinus paranasal CT Scan taken
Six months earlier,
The aim ofpatient
publishingcould
this casenot
is to remind become severe.
on December 19thThere was no
, 2012 showed history
opacities on of
us about the nature of chronic polypoid the right and left nasal cavities, both choana
breathe through the nose.
inflammatory Thethatlast
disease mayfour
cause facial facial pain, allergy space,
and nasopharyngeal or trauma.
bilateral Patient
maxillarydid
disfigurement as distinctive as broadening of
months patient felt impaired sense of notsinuses,
complainbilateral ethmoid sinuses, left sphenoid
of nosebleeds, hearing loss,
nasal pyramid and its management. sinus and left frontal sinus. There were signs
smell and found that his left nose became double vision,
of erosion or septum,
of the nasal neck bilateral
lymph node
medial
maxillary sinus walls, left posterior maxillary
enlarged. HeCASE hadREPORT
moderate headache enlargement.
sinus wall, left orbital inferomedial wall, and
A 16 year-old-boy was referred from a
left inferior sphenoid sinus wall. There was
especially when the with
district hospital nasala diagnosis
blockage of sinonasal
tumor. Patient complained of recurrent common thickening of right sphenoid sinus mucosa.
Six months
colds withearlier, patient
nasal blockage since 3 could not
years before become severe. There was no history of
admission. Six months earlier, patient could not
breathebreathe
throughthroughthe nose.
the nose. TheThe last
last four four
months facial pain, allergy or trauma. Patient did
patient felt impaired sense of smell and found
monthsthatpatient felt impaired sense of
his left nose became enlarged. He had
not complain of nosebleeds, hearing loss,
moderate
smell and found headache especially
that his when the
left nose nasal
became double vision, or neck lymph node
blockage become severe. There was no history
enlarged. Hepain,had
of facial allergymoderate headache
or trauma. Patient did not enlargement.
complain of nosebleeds, hearing loss, double
especially when
vision, or thenodenasal
neck lymph blockage
enlargement.

Fig. 1 External nose enlargement Fig. 2 Anterior rhinoscopy

Physical examination revealed cavities, both choana and nasopharyngeal

polypoid mass filling the entire nasal
cavity with enlargement of the nose.
Frontal view appearance showed nose
enlargement with no distinctive border of
the left nose. From the basal view
Fig. 1 External nose enlargement
appearance, the tip of the nose is deviated
to the right side. Sinus paranasal
Physical CT Scanrevealed inferomedial
examination
78
th
taken polypoid
on December 19 , 2012theshowed
mass filling entire nasal sphenoidspace,
opacities on the right and left nasal
cavity with enlargement of the nose.
space, bilateral maxillary sinuses, bilateral
ethmoid sinuses, left sphenoid sinus and
left frontal sinus. There were signs of
erosion of the nasal septum, bilateral
medial maxillary sinus walls, left
Fig. 2 Anterior rhinoscopy
posterior maxillary sinus wall, left orbital
wall,
cavities, bothand leftandinferior
choana nasopharyngeal
sinus wall. There
bilateral was thickening
maxillary sinuses, bilateral
of right sphenoid sinus mucosa.
ethmoid sinuses, left sphenoid sinus and
RLI Vol. 44 No.1. Tahun 2014 ORLI Vol. 44 No.1. Tahun 2014
ORLI Vol. 44 No.1. Tahun 2014 Woakes syndrome Woakes syndrome Woakes syndrom

Fig. 3 Paranasal CT Scan of the patient with WoakesSyndrome

Fig. 3 Paranasal CT Scan of the patient with WoakesSyndrome

Patient underwent biopsy of the First post operative follow up was done
prepared for second stage surgery by Plastic
nasal mass in January 15th, 2013 with the on March 14th 2013 and nasoendoscopy
Patient underwent biopsy of the nasal Reconstruction ENT Division for rhinoplasty
resultin January
mass of chronic
15th, 2013rhinitis and of
with the result examination
correction. revealed wide left nasal
chronic rhinitis and
nasopharyngitis. nasopharyngitis.
FESS FESS
was performed in was cavity, there was clotting and no mass.
performed
March 8thin March Polypoid
, 2013. 8th, 2013. mass
Polypoidwasmass Right nasal cavity still narrow due to
was extracted with forceps. Inferior and medial
extracted with forceps. Inferior and septal deviation. Patient did not complain
turbinate was difficult to recognize due to the
medial turbinate
expansion of the was difficult
polypoid to recognize
mass. The bleeding of nasal blockage anymore and could
from
due the sphenopalatine
to the expansion artery
of thewas cauterized.
polypoid breathe through the nose. Four months
Uncinectomy
mass. The wasbleeding
performed fromwith backbiting
the after surgery, patient prepared for second
forceps, followed by middle meatal antrostomy
sphenopalatine artery was cauterized. stage surgery by Plastic Reconstruction
finding no polyp inside the maxillary antrum.
Uncinectomy
Histo-pathology was examination
performed with showed ENT Division for rhinoplasty correction.
edematous
backbiting polyps
forceps,with necrosis
followed by and massive
middle
bleeding and no signs of malignancy.
meatal antrostomy finding no polyp inside Fig.4 Polypoid tissue of surgical specimen.
Patient was discharged on the fifth day
the maxillary
after antrum. and was prescribed
hospitalization
Histo-pathology
coamoxiclav examination acid
3x625 mg, mefenamic Septorhinoplasty was performed on July 8th
3x500
showedmgedematous
and normosaline solution
polyps with for nasal
necrosis 2013. Graft augmentation was taken from left
rinsing. First post operative follow up was auricle cartilage. Transcollumelar incision was
and massive bleeding and no signs of
done on March 14th 2013 and nasoendoscopy carried out, followed by infracartilage incision.
malignancy. revealed
examination Patient was
widedischarged
left nasal oncavity, Septal cartilage was incised sparing superior
there was clotting and no mass. Right nasal
the fifth day after hospitalization and was and posterior part. Septal extension graft was
cavity still narrow
prescribed due to 3x625
coamoxiclav septal deviation.
mg, donePolypoid
Fig.4 with interdomal suture.
tissue of surgical Osteotomy was
specimen.
Patient did not complain of nasal blockage performed at right and left side. Nasal splint was
mefenamic acid 3x500 mg and
anymore and could breathe through the maintained for 2 weeks and removed on July
Septorhinoplasty was performed on
normosaline
nose. solution after
Four months for nasal rinsing.
surgery, patient 25th,th2013. Patient was satisfied with the result.
July 8 2013. Graft augmentation was

79
 was performed at right and left side. Nasal present clinically with complain
splint was maintained for 2 weeks and obstruction, congestion,
removed on July 25th, 2013. Patient was rhinorrhea, epistaxis, postna
RLI Vol. 44 No.1. Tahun 2014 ORLI Vol. 44 No.1. Tahun 2014
ORLI Vol. 44 No.1. Tahun 2014 Woakes syndrome Woakes syndrome ORLI Vol. 44 N
satisfied with the result. headaches, and snoring. Pat
Physical examination revealed clear
complained
polypoid mass filling the left nasalofcavity.
enlargement o
Nasal polyps typically present bilaterally
nose. Broadening
but can present unilaterally. Unilateral nasal of the no
masses may be benign unilateral nasal polyps
characteristic
in relation with chronic of Woakes syn
rhinosinusitis. Biopsy
from nasal and nasopharyngeal region
showed chronic described by Caversaccio et al.1
rhinitis and nasopharyngitis,
which conclude that the patient has unilateral
nasal polyp.7 Physical examination reve
The extreme broadening of the nose in
our patient is polypoid
explained by mass filling the left na
the ongoing
pressure of the polyps. This is in accordance
with hypertrophic Nasal
process polyps typically present
and nasal pyramid
deformation, one of the four characteristics
but 1 can present unilaterally.
of Woakes syndrome.
Paranasal sinus CT scan is needed to
prove the frontal nasal sinusmasses may be benign
aplasia. These
Fig.5 patient 16 years old boy with
Fig.5 patient 16 years old boy with Woakes Woakes findings added to the biopsy result suggest a
syndrome syndromeafter
after septorinoplasty
septorinoplasty Woakes Syndrome. nasalWoakes’polyps
syndromein was relation with
defined as necrotizing ethmoiditis and nasal
DISCUSSION
rhinosinusitis.
polyps with broadening of the nose. Biopsy
The from n
syndrome was defined by the following four
DISCUSSION6
According to Slavin et al as quoted
characteristics: nasopharyngeal
by Casale, nasal polyps are present in (1) bilateral nasal polyps region showed
approximately 1% to 4% of the population, in the middle meatus beginning during
According
more often in males to Slavin et alThis
than females. as isquoted rhinitis (3) and
childhood, (2) ethmoiditis, nasopharyngitis
hypertrophic
similar with the case reported in this paper. process with nasal pyramid deformation
6
by Casale, nasal
Case reports polypsjournals
in published are showed
present andin (4) therapeuticconclude thatconstant
failure with the patient has
age incidence of Woakes syndrome under 10 and rapid recurrences. In our case, frontal
1

approximately
years old.3 Our case1%was atomale teenager.
4% of the sinus aplasia was not found, but 7there was
nasal polyp.
Patient in this case presented with nasal full opacification of left frontal sinus without
population, more
blockage and frequent often
episodesinof rhinorrea
males than any polps inside it. The left frontal sinusitis
The extreme broadening o
since 3 years previously. He also complained is caused by obstruction of the frontal recess.
females.
loss This
of smell is and similar
moderatewith the caseWe extirpate intheour
headache. bulk of nasal mass by
patient is explained by th
According to Tritt et al,10 patients with nasoendoscopic procedure and sent it for
reported inpolyposis
nasal this paper. may present clinically with histo-pathological examination. Functional
pressure of the polyps. Th
complaints of nasal obstruction, congestion, Endoscopic Sinus Surgery (FESS) is
Case
hyposmia,reports
rhinorrhea, in epistaxis,
published journals
postnasal considered the gold standard for the surgical
accordance with hypertrophic pr
drip, headaches, and snoring. Patient also treatment of chronic rhinosinusitis with or
showedcomplained
age of enlargement
incidenceof hisof Woakes
left nose. without nasal polyps. It is performed with a
nasal pyramid deformation, on
Broadening of the nose is a characteristic rigid endoscope which allowed a complete
3 visualization of the nasal and paranasal
syndrome undersyndrome
of Woakes 10 years old.
as described Our
by case four characteristics of Woakes sy
Caversaccio et al.1 cavities. The aims of this surgical technique
is to restore normal sinus ventilation and
was a male teenager. Paranasal sinus CT scan

80
RLI Vol. 44 No.1. Tahun 2014 ORLI Vol. 44 No.1. Tahun 2014
ORLI Vol. 44 No.1. Tahun 2014
drainage removing polyps or other tissue
obstructing the osteomeatal complex. FESS
could minimized the recurrence rate of the
disease. The extent of surgery varies with
the extent of disease, the surgeon’s expertise,
the available equipments and technology, and
patient’s condition and expectation.11-13
Four months afterwards septorhinoplasty
was performed. The goal of septorhinoplasty
and rhinoplasty are to restore nasal function
by maximizing nasal airflow and to improve
cosmetic appearance. There are two completely
different methods of rhinoplasty for correcting
nasal pyramid deformations; the first is in
which the osteo-cartilaginous resection of the
nasal dorsum is carried out and the second is
based on preservation of the dorsum.9,14 The
two techniques were never combined because
osteo-cartilaginous resection of the nasal
hump prevents complete disarticulation of
the septal cartilage from its attachment on the
perpendicular plate of the ethmoid bone and the
vomer, an essential step in Cottle septoplasty.
Indeed, once the septal cartilage is completely
detached from its posterior and inferior edges, as
performed in Cottle septoplasty, it can no longer
be separated from the upper lateral cartilages as
performed in nasal hump resection. This would
involve total detachment with major risk of
post-operative saddling and, moreover, stenosis
of the internal nasal valve due to the collapse of
the upper lateral cartilages.2,14
In the literatures reviewed, there was only
one case of Woakes syndrome which underwent
septorhinoplasty.2
In this paper, a case of Woakes syndrome
was reported. The patient underwent two
stages surgery, the first was FESS to clear out
the polyp mass and the second surgery was
septorhinoplasty to restore physiologic function
and aesthetic appearance of the nose.  Med Surg 2008; 29:230-2.
Woakes syndrome Woakes syndrome Woakes syndrom
REFERENCE
1. Caversaccio M, Baumann A, Helbling A.
Woakes’ syndrome and albinism. Auris Nasus
Larynx 2006; 34:245-8.
2. Abbud-Neme F, Reynoso VM, Reiss ED.
Woake’s syndrome. A case report in a
teenager. Int J Pediatr Otorhinolaryngol
1987; 12:327-33.
3. Kellerhals B, Utheman Bd. Woakes’
syndrome: the problem of infantile nasal
polyps. Int J Pediatr Otorhinolaryngol
1979; 1:79-85.
4. Groman JD, Bolger W, Brass-Ernst L,
Macek M Jr, Zeitlin P, Cutting G. Recurrent
and destructive nasal polyposis in 2 siblings:
a possible case of Woakes’ syndrome.
Otolaryngol Head Neck Surg 2004;
131:1009-11.
5. Walsh WE, Kern RC. Head & neck surgery.
4 ed. Bailey BJ, Johnson JT, Newland SD,
editors: Lippincott Williams & Wilkins; 2006.
6. Casale M, Pappacena M, Potena M,
Vesperini E, Ciglia G, Mladina R, et al. Nasal
polyposis: from pathogenesis to treatment,
an update. Inflamm Allergy Drug Targets
2011; 10:158-63.
7. Akarcay M, Ekici N, Miman MC, Firat Y,
Bayindir T, Selimoglu E. Do comorbidities
influence objective and subjective recovery
rates of nasal polyposis? J Craniofac Surg
2010; 21:71-4.
8. Newton JR, Ah-See KW. A review of nasal
polyposis. Ther Clin Risk Manag 2008;
4(2):507-12.
9. Chisholm E, Jallali N. Rhinoplasty and
septorhinoplasty outcome evaluation. Ear
Nose Throat J 2012; 91:10-14.
10. Tritt S, McMains KC, Kountakis SE. Unilateral
nasal polyposis: clinical presentation and
pathology. Am J Otolaryngol Head and Neck
81
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11. Ajaml. Nasal polyp-etiology-clinical finding.

Available from: http://medilinks.blogspot.
com/2011/07/nasal-polyp-etiology-clinical-
findings.html%5D.
12. Bechara Y. Ghorayeb M. Available from:http://
www.ghorayeb.com/antrochoanalpolyp.
html%5D.
13. Fokkens WJ, Lund VJ, Mullol J, Bachert
C, Alobid I, Baroody F, et al. Rhinology
Official Journal of the European and
International Societues European Position
Paper on Rhinosinusitis and Nasal Polyps
2012; 50:1-329.
14. Boulanger N, Baumann C, Beurton R, Elueque
H, Gallet P, Grosjean R, et al. Septorhinoplasty
by disarticulation: early assessment of a new
technique for morphological correction of
crooked noses. Rhinology 2013; 51:77-87.

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