6 cases of forestier syndrome

1. CASE REPORT
Six cases of Forestier syndrome, a rare cause of dysphagia*
ERDI˙NC AYDI˙N1
, VOLKAN AKDOGAN1
, BABU¨ R AKKUZU1
, ISMAI˙L KIRBAS¸2
&
O. NURI˙ OZGI˙RGI˙N1
1
Department of Otorhinolaryngology and 2
Department of Radiology, Baskent University Faculty of Medicine, Bahcelievler-
Ankara, Turkey
Abstract
Dysphagia is a common presenting complaint in otolaryngology practice, and there are many causes. Forestier syndrome is a
rare cause of dysphagia. It is also known as diffuse idiopathic skeletal hyperostosis (DISH) syndrome or vertebral ankylosing
hyperostosis. Forestier syndrome consists of anterolateral perivertebral ligament calcification. It was first described by
Forestier and Rotes-Querol in 1950; diagnosis is primarily radiological and the etiology is unknown. In addition to
dysphagia Forestier syndrome has been reported to cause laryngeal stridor, dyspnea, snoring and hoarseness. Other
important symptoms associated with Forestier syndrome are stiffness and pain in the back, pain related to tendinitis,
myelopathy related to core compression associated with the ossification of the posterior longitudinal ligament, and pain
related to vertebral complications such as fracture or subluxation. We report six cases of Forestier syndrome as an etiologic
factor in dysphagia and present clinical and radiological findings.
Keywords: Dysphagia, Forestier syndrome, osteophyte
Introduction
Zahn was the first to hypothesize that exostoses of
the cervical spine might cause dysphagia [1]. In
1950, Forestier and Rotes-Querol reported the cases
of nine patients who presented with spinal rigidity
[1Á3]. Radiological studies showed that all these
patients had abundant osteophytes of the cervical
spine that were associated with calcification of the
anterolateral aspects of the perivertebral ligaments.
They labeled this a syndrome of ‘ankylosing hyper-
ostosis’ of the spine. The name for this condition
was later changed to Forestier syndrome.
In 1975, Resnick et al. described diffuse idio-
pathic skeletal hyperostosis (DISH) and identified
Forestier syndrome as a more global ‘enthesopathy’
(an inflammatory disorder of muscular or tendi-
nous attachments to bone [4]) than the condition
initially reported by Forestier and Rotes-Querol
[2,3]. Since then the name Forestier syndrome has
also been used to refer to cervical manifestations of
DISH, and instead of DISH. Patients with this
condition may have lesions that affect tendon and
ligament insertion sites in the appendicular or axial
skeleton [1].
Research indicates that 2.4Á5.4% of individuals
older than 40 years of age have Forestier syndrome
[2,5]. This condition is often associated with type II
diabetes and obesity, and is also known to be linked
with gallstones, systemic hypertension, atheroma-
tous vascular disorders, and other diseases of lipid
and purine metabolism [6]. However, the etiology
and pathogenesis of this syndrome are unknown
[6,7].
Approximately 0.1Á6.0% of adults are expected
to develop dysphagia due to Forestier syndrome at
some time point in their lives [1]. In fact, dysphagia
is the most common symptom of Forestier syn-
drome [8]. It is important that otolaryngologists
are familiar with the diagnosis and treatment of the
cervical manifestations of this disorder. Here we
describe six cases of Forestier syndrome associated
with dysphagia.
Correspondence: E. Aydin, MD, Department of Otorhinolaryngology & Head and Neck Surgery, Baskent University Faculty of Medicine, 6. Cadde No:72/2,
06490 Bahcelievler-Ankara, Turkey. Tel: '/90 312 223 85 34. Fax: '/90 312 215 75 97. E-mail: erdinca@baskent-ank.edu.tr
*
Presented at the XVIII World Congress of International Federation of Oto-Rhino-Laryngological Societies, Rome, Italy, 25Á30 June 2005.
Acta Oto-Laryngologica, 2006; 126: 775Á778
(Received 25 October 2005; accepted 27 November 2005)
ISSN 0001-6489 print/ISSN 1651-2551 online # 2006 Taylor & Francis
DOI: 10.1080/00016480500504192

2. Case reports
Case 1
A 55-year-old man presented with a 4-year history of
dysphagia that was more pronounced with solid
foods. He also complained of foreign body sensation
in his throat, aspiration of foods, and difficulty
speaking. The patient reported no weight loss. He
was not obese, and did not suffer from hypertension,
type II diabetes mellitus, or any other metabolic
disease. Otorhinolaryngological examination re-
vealed a mass obstructing the laryngeal opening
(Figure 1). Axial computed tomography (CT)
images of the neck showed large bridging syndesmo-
phytes at C3 to C7 level. These lesions were
indenting the esophagus. Sagittal multiplanar recon-
struction (MPR) CT images also showed this
indentation (Figure 2). The patient was referred to
the neurosurgery clinic, but he refused surgical
treatment.
Case 2
An 82-year-old man presented with dysphagia of 1
month’s duration. He had undergone surgery for
nasal polyposis 6 years previously. His medical
history included hypertension, coronary artery dis-
ease, and hyperlipidemia. A videolaryngoscopic ex-
amination revealed a mass in the posterior wall of the
pharynx. CT scanning demonstrated osteophytes at
C3 to C7. Nonsteroidal anti-inflammatory drugs
(NSAIDs) and anti-reflux treatment were used to
control the symptoms.
Case 3
A 67-year-old man presented with dysphagia that
was aggravated by intake of solid foods. His medical
history included type II diabetes and hypertension.
Otorhinolaryngological examination revealed a mass
in the posterior wall of the pharynx, and CT showed
osteophytes at C3 to T1 level. Treatment with
NSAIDs resolved his complaints.
Case 4
A 63-year-old man presented with foreign body
sensation and difficulty swallowing. A radiograph
showed osteophytes on the anterior aspect of the
cervical spine (Figure 3). The patient was treated
with NSAIDS, and his symptoms completely re-
solved during follow-up.
Case 5
A 72-year-old man presented with the complaints of
coughing and difficulty swallowing. CT scan showed
osteophytes of the cervical spine. The patient refusedFigure 1. Endoscopic view of the posterior pharyngeal mass
posteriorly obliterating the laryngeal entrance.
Figure 2. (A) Axial computerized tomography (CT) image
showing large flowing syndesmophytes (black arrow) and indenta-
tion of the esophagus (arrowhead). (B) Sagittal multiplanar
reconstructed CT image showing anterior syndesmophytes (ar-
rowheads).
776 E. Aydin et al.

3. surgical treatment, and medical therapy did not
alleviate his symptoms.
Case 6
A 61-year-old man presented with sore throat,
dysphagia and coughing. In his examination acute
rhinosinusitis was diagnosed. The laryngeal exam-
ination showed edema of laryngeal structures and
right vocal cord paralysis that was related to a stroke
which the patient had experienced 10 years earlier.
He was treated with antibiotics, decongestants, and
anti-reflux drugs, but he did not benefit from this
treatment. On repeated physical examination the
edema had regressed. CT scans of the neck showed
osteophytes at the hypopharynx level.
Discussion
DISH, or Forestier syndrome, is a degenerative
disorder of unknown cause. Other synonyms of
Forestier and Rotes-Querol disease are dysmetabolic
hyperostosing polyenthesopathy, senile ankylosing
vertebral hyperostosis, coating vertebral hyperosto-
sis, spondylitis ossificans ligamentosa and dysmeta-
bolic hyperostosing spondylopathy [2,7,9,10].
In DISH there is a tendency towards ossification
of ligamentous insertions and ossification and/or
calcification of tendons, ligaments, and fasciae in
both the axial and the appendicular skeleton [9].
The median age of affected individuals is 65Á70
years, with male predominance [1].
Methods of diagnosis may include X-ray, video-
fluoroscopy, CT, MRI (magnetic resonance ima-
ging), and endoscopy. Evaluation of swallowing with
videofluoroscopy allows a differential diagnosis of
dysphagia to be made. Flexible or rigid endoscopy
must be performed carefully because of the risk of
trauma due to the osteophytes. CT and MRI are
necessary for diagnosis and avoidance of complica-
tions such as injury to the cervical spine, the spinal
cord, the internal carotid artery or spinal cord
compressive myelomalacia associated with surgery
for Forestier syndrome [7,11].
Diagnostic criteria for spinal involvement of For-
estier syndrome are: calcification and ossification
within the anterior longitudinal ligaments of at least
four contiguous vertebral bodies; preservation of
disc space height; normal or only mildly sclerotic
apophyseal joints; sacroiliac joints that are normal or
demonstrate para-articular osteophytes only in the
upper third [1]. Radiological signs of DISH can be
found in the appendicular skeleton but diagnosis
depends on the findings in the spine [9].
The etiology and pathogenesis of the disease are
not clear.
There are multiple therapeutic options for the
treatment of Forestier syndrome. NSAIDs, steroids,
skeletal relaxants, anti-reflux drugs, and surgical
treatment are all therapeutic options. For most
patients conservative treatment is both advisable
and suitable. For surgical treatment an anterolateral
approach, a posterolateral approach and, for a C2Á
C4 level Forestier syndrome, a peroral transphar-
yngeal approach have been described. However,
dysphagia in Forestier syndrome is not only a cause
of mechanical compression but also a result of
inflammation and denervation, so a decision for
surgery should be evaluated after trials of medical
therapy.
Surgical complications include laryngeal paralysis,
stroke, Horner syndrome, and cervical instability
[1,7,12].
Clinical manifestations due to Forestier syndrome
include spinal rigidity and significant cervical loss of
motility, acute or chronic cervical pain, medullar
compression and cervical myelopathy, dysphagia,
foreign body sensation, odynophagia, otalgia, dys-
phonia, and airway obstruction [1,2]. The most
common site of presentation of Forestier syndrome
extends from the third to the seventh vertebra. In
this location osteophytes compress the relatively
immobile portion of esophagus [4,8]. The dysphagia
is more severe for solids than for liquids [1,8], and it
Figure 3. Radiograph of cervical spine (lateral view) showing
flowing anterior osteophytes indicative of Forestier syndrome
(arrowheads).
Forestier syndrome: a rare cause of dysphagia 777

4. can be very variable from very mild to severe
difficulty that allows only swallowing of liquids
with loss of weight mimicking an oesophageal tumor
[2]. There is a relationship between swallowing
pathology and osteophyte size: aspiration can be
seen more often in patients with osteophytes larger
than 10 mm [12].
Dysphagia due to osteophytes may be the result of
two mechanisms: obstruction of the pharynx or
esophagus by extrinsic compression or deviation, or
inflammation of soft tissues overlying the bony mass
due to repetitive motion of the esophagus or pharynx
across the protruding osteophyte [1].
Forestier syndrome, even though rare, should be
kept in mind in the differential diagnosis of patients
with a complaint of dysphagia.
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