Guillen et al.

Clinical and Molecular Allergy 2013, 11:5

http://www.clinicalmolecularallergy.com/content/11/1/5

CMA

CASE REPORT

Open Access

The silent sinus syndrome: protean manifestations

of a rare upper respiratory disorder revisited
Denisse E Guillen1, Paulette M Pinargote1 and Juan C Guarderas2*

Abstract
Silent Sinus Syndrome (SSS) is known to be a rare clinical condition, characterized by spontaneous and progressive
enophthalmos and hypoglobus associated with atelectasis of the maxillary sinus and alteration of the orbital floor.
Most of the patients with this syndrome present with ophthalmological complaints without any nasal sinus
symptoms, and it typically has a painless course and slow development, ergo the term silent. Here we present a
case report of a patient with occasional coughing spells as the presenting symptom of Silent Sinus Syndrome,
which has not been previously described in the literature. The CT scan findings suggested chronic rhinosinusitis.
The radiological findings were suggestive of maxillary sinus hypoplasia, with evidence of maxillary sinus atelectasis.
Awareness of this syndrome is important for specialists who work with nasal sinus disease, since its management is
different than chronic rhinosinusits.

Background
Silent Sinus Syndrome (SSS) is known as a rare clinical
condition, characterized by spontaneous and progressive enophthalmos and hypoglobus [1] associated with
atelectasis of the maxillary sinus and alteration of the
orbital floor. This syndrome was first described in 1964
by Montgomery [2], but it was named Silent Sinus
Syndrome in 1994 by Soparkar et al. [3]. Most of the
patients with this syndrome present with ophthalmological complaints without any nasal sinus symptoms,
and it typically has a painless course [3] and slow development, ergo the term silent. We review the concepts
of SSS and review the two most likely mechanisms of
this condition.
Its very common that these patients first present to
ophthalmology [4] due to the syndromes typical constellation of progressive enophthalmos and hypoglobus.
SSS typically presents unilaterally [5], with a slight predominance for presenting on the right maxillary sinus
(57%) [6], and its development is gradual and progressive. The physical exam shows some degree of orbital
asymmetry, with deepening of the superior orbital
sulcus and the consequent hypoglobus. Some other
ophthalmological signs can be eyelid retraction, lid lag,

and lagophthalmos [7]. Occasionally, exophthalmos

of the contralateral uninvolved eye is reported [8]. Although the visual function is typically unaffected, a few
patients have reported alterations in ocular motility or
muscle imbalance producing diplopia [9].
Radiographic findings

The computed tomography (CT) scans of the nose and

paranasal sinuses typically show opacification of the
maxillary sinus and inferior bowing of the orbital floor
[10]. The sinus can be developed or hypoplastic but is
opacified, and the infundibulum is obstructed. This obstruction is usually caused by a lateral retraction of the
uncinate process with its apposition in the inferiomedial
part of the orbit [11]. Sanchez et al. described an image
of a pseudo-pneumo-orbit that can also be seen due to
air trapped under the upper eyelid [12]. Both CT and
magnetic resonance imaging (MRI) scans allow physicians to perform a SSS diagnosis, but CT scans are considered the gold standard diagnostic method because
they provide a better view of the anatomical changes of
SSS that are needed for its diagnosis and for differentiation from other conditions [13].
Management and treatment

* Correspondence: [email protected]
2
Department of Otorhinolaryngology, Mayo Clinic, 4500 San Pablo Road,
Jacksonville, FL 32224, USA
Full list of author information is available at the end of the article

Treatment should address the obstruction of the sinus

and the resultant ocular consequences. Treatment consists of reaeration of the atelectatic sinus by endoscopic

2013 Guillen et al.; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative
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Page 2 of 4

sinus surgery. All authors agree that sinus pathology

should be treated endoscopically as the first step of the
treatment [14].
Limited antrostomy typically results in a release of negative sinus pressure and re-expansion of the collapsed cavity
leading to reduction of enopthalmus [15]. A wide antrostomy prevents future reobstructions, and good reaeration of
the sinus helps to avoid recurrent enophthalmos [16].
Timing for management of the orbital floor is still under
debate. As suggested by some authors, orbital floor reconstruction must be performed simultaneously with sinus
treatment [17]. Other authors think that only drainage of
the sinus should be enough [18]. According to Cardesin
et al., the need for orbital floor repair depends on the
severity of the diplopia, the degree of the cosmetic alterations, and the postsurgical evaluation of the sinus [19].

Case presentation
A 66-year-old gentleman, non-smoker, with no known
allergies or significant respiratory medical history,
presented to the Allergy Medicine service with a chief
complaint of cough. He describes that he suffered a respiratory infection approximately three months prior to
the visit. After it was treated, the majority of symptoms
resolved, but the cough and coughing spells persisted
with post-nasal drainage and clearing of the throat. The
CT scan revealed an asymmetrically smaller and completely opacified left maxillary sinus with left-sided
periosteal thickening as well as lateral bowing/bone remodeling of the uncinate process. The opacified left
maxillary sinus had hyperdensities which could represent chronic dense secretions (Figure 1). The left ostiomeatal complex was occluded (Figure 2). The initial
diagnosis was chronic sinusitis. He was prescribed antibiotics for 10 days, was advised to have a new sinus CT
scan, and was referred to otorhinolaryngology.
Two months later when the patient presented to
otorhinolaryngology for follow-up, there had been no
clinical changes. The physical exam showed some asymmetry of both eyes with mild hypoglobus of the left eye.
His nose had a moderate anterior septal deformity to the
left with mucoid drainage. He was unaware of vision
changes, but testing demonstrated double vision when
looking to the extreme right. The rest of the exam was
normal.
The new sinus CT scan showed no significant change.
The interpretation was a persistent opacified hypoplastic
left maxillary sinus with obstructed left ostiomeatal unit
with lateralization of the uncinate process. The pattern was
consistent with type 2 maxillary sinus hypoplasia. A sinonasal endoscopy confirmed the previous imaging findings.
The patient underwent a turbinoplasty and endoscopic
sinus surgery (antrostomy) to address the total opacification of his left maxillary sinus. The nasal sinus

Figure 1 Examination reveals an asymmetrically smaller and

completely opacified left maxillary sinus with left-sided periosteal thickening, as well as lateral bowing/bone remodeling of
the uncinate process.

symptoms and cough resolved after the procedure. Correction of the enopthalmos was not necessary.

Discussion
SSS has two main theorized mechanisms: maxillary sinus
atelectasis (MSA)which could be idiopathic, posttraumatic, or post-surgeryor maxillary sinus hypoplasia (MSH).
Chronic rhinosinusitis (CRS) has a prevalence of 13.4%
in adults older than 18 years of age, according to a national health survey conducted in 2008 [20]. The causes

Figure 2 The left maxillary sinus is smaller and opacified. The

middle meatus space is wide and the medial wall of the sinus is
lateralized with contact with the lateral wall of the maxillary sinus.

Guillen et al. Clinical and Molecular Allergy 2013, 11:5

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and classification of CRS have recently been reviewed by

Hamilos [21]. Causes of CRS are frequently anatomical
and include septal deformity, Hallers cells, paradoxical
middle turbinate, and agger nasi cell. A hypoplastic maxillary sinus, an atelectatic maxillary sinus, and silent sinus
syndrome are infrequently recognized causes of CRS. A
description of these entities is reviewed in this paper.
Maxillary sinus hypoplasia (MSH) is an infrequent
congenital anomaly that Bolger et al. [22] noted in 10.4%
of 202 consecutive CT scans reviewed. MSH has been
classified as type 1 when there is a normal uncinate
process and a defined infundibular passage. Type 2 has a
hypoplastic or absent uncinate process with an opacified
affected sinus, and type 3 has an absent uncinate process
and profound hypoplasia of the sinus. This classification
has been supported by Erden (Table 1) [23].
Chronic maxillary atelectasis (CMA) is a term that
describes a persistent decrease in the sinus volume from
inwardly bowing antral walls [24]. In a 1997 study that
spanned over ten years at the Massachusetts Eye and
Ear Infirmary, 22 individuals were diagnosed with CMA,
and their literature review found 25 additional individuals who met their criteria. Their criteria included: sinus
opacification on CT scans or X-rays lasting more than
3 months and/or tenacious mucus secretions filling the
antrum in addition to lateral displacement of the medial
infundibular wall (MIW).
CMA, in reference to the Massachusetts Eye and
Ear work, was differentiated in 3 stages based on the
anatomical changes: Stage 1 (membranous deformity)
where there is a lateralization of the maxillary fontanel, Stage 2 (bone deformity) where there is inward
bowing of one or more osseous walls of the maxillary antrum, and Stage 3 (clinical deformity) where
enophthalmus, hypoglobus, and/or midfacial deformity is noted (Table 2) [24].
In this series of 22 patients, 19 had some degree of
sinus symptoms and five had findings of hypoglobus.
Silent sinus syndrome is a very uncommon clinical
entity [25]. The pathophysiology of this syndrome remains unanswered in part because there is rarely a presymptomatic CT scan that can be used to review the
stages of the process. A hypothesis for the pathophysiology is that hypoventilation of the sinus due to
Table 1 Types of maxillary sinus hypoplasia [23]
Maxillary sinus
hypoplasia type

Characteristics

Type 1

Normal Uncinate. Defined

infundibular passage

Type 2

Hypoplastic or absent uncinate.

Opacified sinus

Type 3

Absent Uncinate. Profound

hypoplasia of the sinus

Page 3 of 4

Table 2 Stages of chronic maxillary atectasis [24]

Characteristics
Stage 1

Membranous deformity where there is

lateralization of the maxillary fontanel

Stage 2

Bone deformity where there is inward

bowing of one or more osseous walls
of the maxillary antrum

Stage 3

Clinical deformity with enophthalmus,

hypoglobus, and/or midfacial deformity
is noted.

obstruction of the osteomeatal unit [26] creates a negative pressure [27] that leads to atelectasis [28] of the
sinus with a downward displacement of the orbital
floor [29]. There is disagreement over whether the obstruction of the osteomeatal unit (OMU) is caused by
hypoplasia and/or if there are any cases where a normally developed sinus due to trauma, surgery, or other
cause can be obstructed and consequently develop
atelectasis and SSS.

Conclusion
In this paper, we have reviewed the clinical and radiological presentation of SSS. Patients with SSS most often
present to ophthalmology practices due to complaints of
facial or ocular asymmetry such as hypoglobus or enophthalmos with little or no nasal sinus symptoms [30].
However, these patients may occasionally also present to
otorhinolaryngology or allergy medicine, with nasal sinus
symptoms suggestive of sinusitis. The mechanism of the
development of SSS has been thought to be atelectasis of
the maxillary sinus with or without the presence of maxillary sinus hypoplasia, especially in type 2.
Our patient presented to otolaryngology and allergy
medicine for evaluation of a chief complaint of chronic
cough with occasional coughing spells which have not
been described in the literature as a form of presentation
of Silent Sinus Syndrome. The CT scan findings suggested CRS. The radiological findings were suggestive of
MSH, and there was evidence of MSA.
The differentiation of SSS from CRS is important since
sinus surgery is the procedure of choice and clear knowledge of this anatomy is very important for the surgeon
to avoid entering into the orbit and since medical management alone is unlikely to produce a positive result. In
surgery, the use of an image-guidance system can also
help to avoid complications.
Consent
Written informed consent was obtained from the patient
for publication of this Case Report and any accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.

Guillen et al. Clinical and Molecular Allergy 2013, 11:5

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Abbreviations
CMA: Chronic maxillary atelectasis; CRS: Chronic rhinosinusitis; CT: Computed
tomography; MIW: Medial infundibular wall; MRI: Magnetic resonance
imaging; MSA: Maxillary sinus atelectasis; MSH: Maxillary sinus hypoplasia;
OMU: Ostiomeatal unit; SSS: Silent sinus syndrome.
Competing interests
JG serves on the editorial board for Clinical and Molecular Allergy. The authors
declare no other conflicts of interest.
Authors contributions
DG: conception and design; acquisition of data; analysis and interpretation of
data; drafting the manuscript. PP: acquisition of data; drafting the
manuscript. JG: analysis of data; revising the manuscript. All authors read and
approved the final manuscript.
Acknowledgments
The authors thank Victoria L. Jackson, MLIS (Academic and Research Support,
Mayo Clinic, Jacksonville, FL) for her assistance in the editing and preparation
of this manuscript.
Author details
1
Department of Otolaryngology, Mayo Clinic, 4500 San Pablo Road,
Jacksonville, FL 32224, USA. 2Department of Otorhinolaryngology, Mayo
Clinic, 4500 San Pablo Road, Jacksonville, FL 32224, USA.
Received: 12 July 2013 Accepted: 23 November 2013
Published: 9 December 2013
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doi:10.1186/1476-7961-11-5
Cite this article as: Guillen et al.: The silent sinus syndrome: protean
manifestations of a rare upper respiratory disorder revisited. Clinical and
Molecular Allergy 2013 11:5.

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