Acute dyspnea as a manifestation of Forestier's disease. A case report

(1)

1

DOI: 10.5604/01.3001.0014.4873 casestudy

POL OTORHINO REV 2021: 10 (1): 1-4

Acute dyspnea as a manifestation of Forestier’s

disease. A case report

Ostra duszność jako manifestacja choroby Forestiera.

Opis przypadku

Jagoda Kuryłowicz

^ABDEF

, Dominik Stodulski

^ABDE

, Ewa Garsta

^BD

, Bogusław Mikaszewski

^D

Chair and Clinic of Otolaryngology at the Medical University of Gdansk, Poland; Head: Bogusław Mikaszewski MD PhD

Article history: Received: 24.09.2020 Accepted: 29.10.2020 Published: 06.11.2020

ABSTRACT: Introduction: Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier’s disease, is a noninflammatory disease and is characterized by ossification of the anterolateral aspect of vertebral bodies, mostly in thoracic part of the spine.

Although, usually, DISH is asymptomatic, in rare cases osteophytes located in the cervical part of the spine can cause otolaryngological manifestations, such as dysphagia (most common), hoarseness and stridor

Case report: In differential diagnosis of upper respiratory tract symptoms, we should consider DISH. We present case of 82-years old male patient with acute dyspnea, who was diagnosed with osteophytes of C4–C7 vertebral bodies. In this case conservative therapy was not efficient, therefore successful surgical treatment was performed.

KEYWORDS: acute laryngeal dyspnea, diffuse idiopathic skeletal hyperostosis, dysphagia, Forestier’s disease, laryngeal oedema, otolaryngological symptoms, stridor, tracheotomy

STRESZCZENIE: Wprowadzenie: Rozsiana idiopatyczna hiperostoza szkieletu (ang. diffuse idiopathic skeletal hyperostosis; DISH), zwana również „chorobą Forestiera”, jest chorobą niezapalną, charakteryzującą się kostnieniem przedniobocznej powierzchni trzonów kręgów, najczęściej w piersiowym odcinku kręgosłupa. W większości przypadków nie jest ona asymptomatyczna, jednakże osteofity umiejscowione w szyjnym odcinku kręgosłupa mogą powodować objawy otolaryngologiczne, takie jak:

dysfagia, chrypka czy duszność.

Opis przypadku: Ze względu ma to, że u starszych chorych z objawami ze strony górnych dróg oddechowych w diagnostyce różnicowej należy brać pod uwagę również DISH, prezentujemy przypadek 82-letniego mężczyzny z nasiloną dusznością krtaniową, u którego zdiagnozowano obecność osteofitów trzonów kręgów C4–C7. Opisywany pacjent został poddany leczeniu zachowawczemu, a następnie, ze względu na brak zadowalającego efektu terapeutycznego, zabiegowi neurochirurgicznemu, po którym dolegliwości ustąpiły.

SŁOWA KLUCZOWE: choroba Forestiera, dysfagia, objawy otolaryngologiczne, obrzęk krtani, ostra duszność krtaniowa, rozsiana idiopatyczna hiperostoza szkieletu, stridor, tracheotomia

Authors’ Contribution:

A – Study Design B – Data Collection C – Statistical Analysis D – Manuscript Preparation E – Literature Search F – Funds Collection

ABBREVIATIONS

BMI – Body Mass Index CRP – C Reactive Protein CT – computed tomography

DISH – diffuse idiopathic skeletal hyperostosis GERD – gastro-esophageal reflux disease MRI – magnetic resonance imaging

NSAIDs – nonsteroidal anti-inflammatory drugs PPI – proton pump inhibitors

INTRODUCTION

Diffuse idiopathic skeletal hypMRerostosis (DISH), also known as Forestier’s disease, was first described under the name “senile ankylosing hyperostisis of the spine” by Jacques Forestier in 1950 [1]. It is a noninflammatory disease, characterized by ossification of the anterolateral aspect of vertebral bodies, mostly in thoracic part of the spine. It may also involve enthesopathy of the extre- mities. Forestier’s disease is a rather common condition, which affects approximately 40% of older (>65 years old) male patients.

(2)

casestudy

WWW.OTORHINOLARYNGOLOGYPL.COM

2

Fig. 1. Flexible nasopharyngoscopy of the hypopharynx and larynx prior to surgery. Fig. 3. MRI prior to surgery.

Fig. 2. CT pior to surgery. Fig. 4. Intraoperative image of osteophytes of the cervical spine.

tory disorder was progressing for 2 months. Moreover, patient complained of dysphagia and hoarseness, which were present for a year. The patient was diagnosed with GERD and treatment was administrated. Medical history was significant of duodenal ulcer with perforation and prostate cancer treated with hormo- nal therapy for 13 months. Flexible nasopharyngoscopy revealed large mass protruding from the posterior wall of hypopharynx and oedema, which concealed interarytenoid notch and partly rima glottis (Fig. 1.). No other abnormalities in ORL examination were present. Increased CRP level and impaired fasting glucose were found in laboratory tests during hospitalization. CT (Fig. 2.) and MRI (Fig. 3.) revealed massive osteophytes on the anterior part of vertebral bodies C4–C7 without intervertebral disc space narrowing, thickened vestibular folds and peri-arytenoid region.

Prevalence of DISH increases with age (56% for age >80 years old), BMI and blood pressure [2] and is associated with diabetes mel- litus, elevated insulin-like growth factor and hyperuricemia [3].

Although usually DISH is asymptomatic, in rare cases osteophytes located in the cervical part of the spine can cause otolaryngological manifestations, such as dysphagia (most common), hoarseness and stridor [4–10].

CASE REPORT

An 82-years old man was admitted to the Department of Oto- laryngology due to acute dyspnea with stridor at rest. Respira-

(3)

3

POL OTORHINO REV 2021: 10 (1): 1-4

casestudy

Fig. 5. CT following surgery. Fig. 6. Flexible nasopharyngoscopy of the hypopharynx and larynx at 3 months post-op.

X-ray revealed no changes in sacro-iliac joints. Hypopharyngo- scopy and microlaryngoscopy were performed in due to rule out neoplastic changes. Antibiotis, steroids and high doses of PPI (proton pump inhibitors) were administered, resulting in oedema decrease and resolution of the symptoms. After 7 months patient was hospitalized again, due to acute dyspnea and stridor. Increased CRP level was found in laboratory tests. Despite conservative therapy (corticosteroids, antibiotics and PPI) no significant respiratory improvement was achieved, therefore deci- sion on surgical treatment was made. Patient was referred to the Department of Neurosurgery where, after elective tracheotomy, cervical osteophytes (C2–C5) were removed, using an anterolateral transcervical approach, without any complications (Fig. 4.).

Because of postoperative laryngeal and hypopharyngeal oedema, steroid therapy was maintained. Decannulation was performed on 7th postoperative day, although patient complained of more severe dysphagia with aspiration while swallowing. Nasogastric feeding tube was inserted for 3 weeks. At the 6-month follow up dyspnea and dysphagia are absent, patient successfully swallows solid food and liquids. Endoscopic examination revealed only small protrusion and minor oedema on posterior wall of hypopharynx on the level of arytenoids (Fig. 5., 6.).

DISCUSSION

Forestier’s disease otolaryngological manifestations are extre- mely rare. The most common symptom is dysphagia, aside from it aspiration [11], dyspnea (with or without stridor) and hoarseness [4].

Our patient’s main complaint and reason of hospitalization was dyspnea, while dysphagia was secondary problem. Foregoing symptoms can occur not only due to osteophytes compression on larynx and esophagus, but also because of chronic inflammation and chronic or recurrent edema caused by mechanic irritation.

Other symptoms, such as aspiration while swallowing, can be caused by impaired movability of epiglottis or vocal chords [8, 11].

In presented case symptoms (especially dyspnoe) were increasing during upper respiratory tract infections and due to exacerbation of GERD.

In literature authors described similar cases, where inflammation led to oedema, chronic or remitting during infection, which worsened patient’s condition [12, 13] and caused respiratory decom- pensation, necessitating even urgent tracheotomy [10].

During diagnosis of DISH with otolaryngologic manifestations, mirror laryngoscopy and fiberoscopy should be performed. It can reveal fine, firm protrusion on the posterior wall of pharynx, accompanied by oedema and impaired movability of larynx [8].

In differential diagnosis of protrusion on the posterior wall of pharynx we should consider retropharyngeal pathologies, such as malignant tumors (including lymphoma), benign tumors, meta- stases, congenital defects (e.g. vascular malformations), lympha- denopathy, retropharyngeal abscess or massive oedema. Imaging (CT, MRI) can rule out these diseases [14].

Forestier’s disease should also be distinguished from other pathologies, which involve vertebral bodies, including ankylosing spondylitis, osteophytes in osteoarthritis or osteomas [3]. Diffe- rential diagnosis can be based on Resnick’s radiological classifi- cation criteria of DISH [15]: presence of flowing calcification and ossification along with the anterolateral aspects of at least four contiguous vertebral bodies, relative preservation of intervertebral disc height in the involved vertebral segments and absence of apophyseal joint bony ankylosis and sacro-iliac joint erosion, sclerosis or bony fusion.

In cases with dysphagia being the main patient’s complaint, eso- phagogram with barium swallowing should be performed [5, 9, 11].

(4)

casestudy

WWW.OTORHINOLARYNGOLOGYPL.COM

4

References

1. Forestier J., Rotes-Querol J.: Senile ankylosing hyperostosis of the spine. Ann Rheum Dis., 1950; 9(4): 321–330.

2. Holton K.F., Denard P.J., Yoo J.U., Kado D.M., Barrett-Connor E. et al.: Diffu- se Idiopathic Skeletal Hyperostosis and Its Relation to Back Pain Among Ol- der Men: The MrOS Study. Semin Arthritis Rheum., 2011; 41(2): 131–138.

3. Holgate R.L.V., Steyn M.: Diffuse idiopathic skeletal hyperostosis: Diagnostic, clinical, and paleopathological considerations. Clinical Anatomy. John Wiley and Sons Inc., 2016; 29: 870–877.

4. Pulcherio J.O.B., Velasco C.M.M.O., Machado R.S., de Souza W.N., de Mene- zes D.R.: Forestier’s disease and its implications in otolaryngology: Literature review Doença de forestier e suas implicações otorrinolaringológicas: Revisão de literatura. Braz J Otorhinolaryngol., 2014; 80(2): 161–166.

5. Nelson R.S., Urquhart A.C., Faciszewski T.: Diffuse Idiopathic Skeletal Hype- rostosis: A Rare Cause of Dysphagia, Airway Obstruction, and Dysphonia. J Am Coll Surg [Internet]., 2006 Jun [cited 2020 Mar 6]; 202(6): 938–942. Ava- ilable from: https://linkinghub.elsevier.com/retrieve/pii/S1072751506001918 6. Caminos C.B., Cenoz I.Z., Louis C.J., Otano T.B., Esáin B.F. et al.: Forestier disease: an unusual cause of upper airway obstruction. Am J Emerg Med [Inter- net]., 2008 Nov 1 [cited 2020 Mar 6]; 26(9): 1072.e1–1072.e3. Available from:

https://linkinghub.elsevier.com/retrieve/pii/S0735675708002829

7. Naik B., Lobato E.B., Sulek C.A.: Dysphagia, Obstructive Sleep Apnea, and Difficult Fiberoptic Intubation Secondary to Diffuse Idiopathic Skeletal Hy- perostosis. Anesthesiology., 2004; 100(5): 1311–1312.

8. Anand V., Vikram Vel V.R., Purushothaman P.K., Rajesh Kumar M.S.: Cri- co arytenoid joint fixation in diffuse idiopathic skeletal hyperostosis (DISH):

A case report. Indian J Otolaryngol Head Neck Surg., 2011; 63: S55–7.

9. Calisaneller T., Ozdemir O., Tosun E., Altinors N.: Dysphagia due to diffuse idiopathic skeletal hyperostosis. Acta Neurochir (Wien)., 2005; 147(11): 1203–1206.

10. Dagher W.I., Nasr V.G., Patel A.K., Flis D.W., Wein R.O.: An unusual and rare cause of acute airway obstruction in the elderly: Forestier’s disease. J Emerg Med [Internet]., 2014 May 1 [cited 2020 Mar 9]; 46(5): 617–619. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24548469

11. Seidler T.O., Pèrez Àlvarez J.C., Wonneberger K., Hacki T.: Dysphagia caused by ventral osteophytes of the cervical spine: Clinical and radiographic findings.

Eur Arch Oto-Rhino-Laryngology., 2009; 266(2): 285–291.

12. Marks B., Schober E., Swoboda H.: Diffuse idiopathic skeletal hyperostosis causing obstructing laryngeal edema. Eur Arch Oto-Rhino-Laryngology., 1998; 255(5): 256–258.

13. Kim Y.S., Lee J.J., Chung Y.H., Kim E.S., Chung I.S.: Postoperative obstructing laryngeal edema in patients with diffuse idiopathic skeletal hyperostosis of cervical spine-A report of two cases. Korean J Anesthesiol., 2011; 60(5): 377–380.

14. Hoang J.K., Eastwood J.D., Branstetter B.F., Raduazo P., Glastonbury C.M.:

Masses in the Retropharyngeal Space: Key Concepts on Multiplanar CT and MR Imaging. Neurographics., 2011; 1(1): 49–55.

15. Resnick D., Niwayama G.: Radiographic and pathologic features of spinal in- volvement in diffuse idiopathic skeletal hyperostosis (DISH). Radiology., 1976;

119(3): 559–568.

16. Lecerf P., Malard O.: How to diagnose and treat the symptomatic anterior cervical osteophytes? Ann Fr d’Oto-Rhino-Laryngologie Pathol Cervico-Facia- le., 2010; 127(3): 137–142.

Word count: 1119 Tables: – Figures: 6 References: 16 Access the article online: DOI: 10.5604/01.3001.0014.4873 Table of content: https://otorhinolaryngologypl.com/issue/13697

Some right reserved: Polish Society of Otorhinolaryngologists Head and Neck Surgeons. Published by Index Copernicus Sp. z o.o.

Competing interests: The authors declare that they have no competing interests.

The content of the journal „Polish Society of Otorhinolaryngologists Head and Neck Surgeons” is circulated on the basis of the Open Access which means free and limitless access to scientific data.

This material is available under the Creative Commons – Attribution-NonCommercial 4.0 International (CC BY-NC 4.0). The full terms of this license are available on: https://creativecommons.org/licenses/by-nc/4.0/legalcode

Corresponding author: Jagoda Kuryłowicz MD; Chair and Clinic of Otolaryngology at the Medical University of Gdansk; Smoluchowkiego street 17, 80-214 Gdansk, Poland; Phone.: +48 58 349 31 10;

E-mail: jagoda.kurylowicz@gmail.com

Cite this article as: Kurylowicz J., Stodulski D., Garsta E., Mikaszewski B.: Acute dyspnea as a manifestation of Forestier’s disease. A case report; Pol Otorhino Rev 2021; 10 (1): 1-4

Elective panendoscopy with biopsy should always be considered to rule out neoplasm [5, 16].

If DISH is diagnosed accidently in imaging, we can assume an expectant attitude. Conservative treatment with NSAIDs, steroids and dietary measures can be introduced in cases without weight loss and with minor respiratory symptoms. Gastroeso- phageal reflux can be symptom-worsening factor, therefore PPI treatment should be considered. If conservative treatment is not effective, osteophytes’ surgical removal is recommended, prefe- rably using anterolateral approach [16]. In our case, conservative therapy was not sufficient. Massive hypopharyngeal oedema and

stridor were present in spite of high doses of corticosteroids. Due to the pre-operative oedema we decided to perform an elective tracheotomy before neurosurgical intervention, to avoid possible complications [13].

It is remarkable, that after surgery dyspnea resolved after few days, whilst dysphagia and aspiration worsened in post-operative time and nasogastric tube insertion was necessary.

Authors present this case in aim to draw attention to Forestie- r’s disease as possible reason for respiratory and gastrointestinal symptoms among elderly people.