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Joint Bone Spine 76 (2009) 409e411

Case report

Dysphagia and hypervitaminosis A: Cervical hyperostosis

Daniel Wendling a,*, Chafika Hafsaoui b, Jean-Marie Laurain c, Michel Runge d,Nadine Magy-Bertrand b, Clement Prati a

a Service de Rhumatologie, CHU Jean Minjoz, EA 3186, Universite de Franche-Comte, Boulevard Fleming, 25030 Besancon, Franceb Service de Medecine Interne, CHU Minjoz, 25030 Besancon, France

c Centre de Chirurgie du Rachis, Clinique St. Vincent, 25000 Besancon, Franced Service de Radiologie B, CHU Minjoz, 25030 Besancon, France

Accepted 12 November 2008

Available online 16 March 2009

Abstract

Vertebral hyperostosis typically predominates at the thoracic spine and causes only minor symptoms. Involvement of the cervical spine maycause dysphagia due to pressure on the esophagus. We report three cases of dysphagia revealing cervical hyperostosis.Case reports: The patients were 3 men aged 54e73 years. Dysphagia was moderate in 2 patients and severe in 1 patient who had lost 4 kg over 6months. Stiffness of the neck with mild pain was present. One patient reported a neck injury in childhood and another had a brother and fatherwith similar symptoms. Radiographs showed exuberant anterior cervical hyperostosis. Two patients also had hyperostotic changes at the thoracicspine and pelvis. The skin and neurological evaluation were normal. Findings were normal from standard blood tests (C-reactive protein,calcium, and vitamin D). Tests were negative for the HLA-B27 antigen in all 3 patients. Serum vitamin A levels were high, ranging from 894 to1123 mg/L (normal, 489e720). None of the patients reported taking retinoids or having unusual eating habits.Discussion: Dysphagia can result from anterior cervical hyperostosis. A role for hypervitaminosis A in the genesis of hyperostosis has long beensuspected. In our patients, the absence of vitamin A supplementation suggests an abnormality in vitamin A metabolism.� 2009 Societe Francaise de Rhumatologie. Published by Elsevier Masson SAS. All rights reserved.

Keywords: Diffuse; Idiopathic skeletal hyperostosis; Dysphagia; Retinol

Vertebral hyperostosis, or Forestier’s disease, is character-ized by flowing ossification of the anterior longitudinal liga-ment that extends along several contiguous vertebral bodiesbut spares the intervertebral disk spaces. Although the thoracicspine is predominantly affected, other spinal segments may beinvolved. There is a diffuse variant of the disease called diffuseidiopathic skeletal hyperostosis (DISH), in which extra-axialsites are also involved [1]. Motion restriction is the mainsymptom. Patients may report moderate back pain. Less often,ossifications of the cervical spine may impinge on neighboringstructures, such as the esophagus. We report 3 cases revealedby dysphagia due to esophageal compression. All 3 patientshad hypervitaminosis A.

* Corresponding author. Tel.: þ33 381 66 82 41; fax: þ33 381 66 86 86.

E-mail address: dwendling@chu-besancon.fr (D. Wendling).

1297-319X/$ - see front matter � 2009 Societe Francaise de Rhumatologie. Publis

doi:10.1016/j.jbspin.2008.11.004

1. Case reports

1.1. Case 1

A 73-year-old man whose medical history was remarkableonly for a smoking habit was admitted for evaluation of solid-food dysphagia of 6 months duration with a weight loss of4 kg. He had no skin lesions or neurological abnormalities.Findings were normal from an otorhinolaryngological evalu-ation, upper digestive tract endoscopy, and a pulmonaryevaluation. Motion of the neck was painful and restricted in alldirections. Radiographs disclosed marked hyperostosis at theanterior aspect of the cervical spine extending from C2 to C7and predominating at C3eC4 (Fig. 1 and Fig. S1; see thesupplementary material associated with this article online).Moderate hyperostosis was noted at the thoracic spine. Abarium study showed impingement of the ossifications on the

hed by Elsevier Masson SAS. All rights reserved.

Fig. 1. Patient #1. Standard lateral radiograph of the cervical spine: anterior

hyperostosis extending from C2 to C7 and predominating at C3eC4.

Fig. 2. Patient #2. Standard lateral radiograph of the cervical spine: anterior

hyperostosis extending from C2 to C7 and predominating at C3eC4.

410 D. Wendling et al. / Joint Bone Spine 76 (2009) 409e411

esophagus. Findings were normal from standard blood testsincluding C-reactive protein and serum calcium and vitamin Dlevels. There was no evidence of metabolic or hepatic disease.The test for HLA-B27 was negative. Plasma vitamin A(retinol) was elevated to 1113 mg/L (normal, 489e720). Thepatient denied taking drugs or supplements or having eatinghabits likely to cause vitamin A poisoning. A dysphagia dietwas effective in ensuring weight regain.

1.2. Case 2

A 54-year-old man presented with stiffness of the neck andpositional dysphagia of several months duration. He reporteda neck injury in childhood and a 20-year history of mechanicalneck pain. The physical examination showed motion restric-tion of the cervical spine. No cutaneous or neurologicalabnormalities were found. Radiographs showed marked ante-rior hyperostosis of the cervical spine (Fig. 2, Fig. S2) withevidence of progression over the last 20 years. The ossificationwas uniform and extended from C2 to C7. No hyperostoticlesions were found at other sites. Findings were normal fromblood tests including C-reactive protein, calcium, phosphate,vitamin D, and parathyroid hormone. The test for HLA-B27was negative. No signs of metabolic or hepatic disease werefound. Plasma retinol was elevated to 894 mg/L. He denied

taking drugs, supplements, or foods known to cause hypervi-taminosis A.

1.3. Case 3

This 65-year-old man with an unremarkable medicalhistory presented with stiffness of the neck and back, upperthoracic kyphosis, and moderate intermittent dysphagia.Motion restriction of the spine and hips was the only abnormalphysical finding. Radiographs showed anterior hyperostosisextending from C2 to C7 and predominating at C3eC4(Fig. 3). Additional hyperostotic lesions were found at thethoracic and lumbar spine and at the hips.

Findings were normal from laboratory tests includingC-reactive protein, calcium, phosphate, vitamin D, parathyroidhormone, and tests for metabolic and hepatic disease. TheHLA phenotype was B8, B44. Plasma retinol was 1123 mg/L.He denied taking medications.

2. Discussion

In our 3 patients, dysphagia and neck stiffness were thepresenting symptoms of cervical vertebral hyperostosis withradiological features meeting criteria for DISH [1,2]. Theossifications were diffused in 2 patients and confined to thecervical spine in 1 patient. DISH predominantly affects males.Prevalences range from 5% to 15% among individuals olderthan 65 years [3]. The thoracic spine is the main site of

Fig. 3. Patient #3. Standard lateral radiograph of the cervical spine: anterior

hyperostosis extending from C2 to C7 and predominating at C3eC4.

411D. Wendling et al. / Joint Bone Spine 76 (2009) 409e411

involvement, although the cervical spine is affected in 75% ofpatients with diffuse ossifications [4]. Dysphagia has beenreported in 17% of patients with cervical ossifications [1,4],and several case-series studies of DISH-related dysphagiahave been published. In a study of 10 patients, the symptomsdepended on the size of the anterior ossifications and on theage of the patients [3]. Dysphonia and dyspnea have beenreported also [3,5,7].

The mechanisms that may contribute to dysphagia inpatients with anterior cervical hyperostosis include impinge-ment, fibrotic adhesions, and impaired epiglottic mobility[3,5]. Dysphagia may occur in patients with osteophytes due todegenerative disease of the cervical spine [5] or trauma [10],in the absence of hyperostosis. Severe symptoms may requiresurgery [2,5e9], which relieves the dysphagia and ensuresa favorable outcome [5].

Apart from hypervitaminosis A, our patients had none of theother risk factors for DISH such as hyperlipidemia, diabetesmellitus [2,11], hyperuricemia, or obesity [1,3,12]. Mechanicalstress may be involved [1], and the neck injury sustained by ourpatient #2 during childhood may have contributed to the devel-opment of exuberant ossifications confined to the cervical spine.Studies done in France two decades ago showed high serumvitamin A levels in patients with hyperostosis, compared tocontrols [13,14]. Vitamin A stimulates osteoblast formation, andretinoid therapy can induce hyperostosis [15]. Furthermore,

a case of DISH was reported in a patient who had a familialabnormality in the retinol binding protein [16]. The absence ofvitamin A supplementation and retinoid therapy in our patients isconsistent with an underlying abnormality in vitamin A metab-olism. This metabolic abnormality may explain the exuberantnature of the ossifications and, therefore, the occurrence ofdysphagia. A lateral radiograph of the cervical spine should beobtained in patients who present with dysphagia. Features thatsuggest hyperostosis are lessening of the dysphagia upon forwardflexion of the neck and motion restriction of the cervical spine.

Appendix A. Supplementary material

Supplementary material (Figs. S1 and S2) associated withthis article can be found at http://www.sciencedirect.com, atdoi: 10.1016/j.jbspin.2008.11.004

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