DISHphagia and DISHphonia DISHguised as a stroke

Abdulrahman Khalid Alfadhel; Abdullah K Almanea; Rajkumar Rajendram; Abdulaziz G Alsaad

doi:10.4103/JNSM.JNSM_47_19

CLINICOPATHOLOGICAL PEARLS

Year : 2020 | Volume : 3 | Issue : 2 | Page : 139-141

DISHphagia and DISHphonia DISHguised as a stroke

Abdulrahman Khalid Alfadhel, Abdullah K Almanea, Rajkumar Rajendram, Abdulaziz G Alsaad
Department of Internal Medicine, King Abdulaziz Medical City, National Guard Health Affairs, Riyadh, Saudi Arabia

Date of Submission	14-Oct-2019
Date of Decision	02-Dec-2019
Date of Acceptance	16-Dec-2019
Date of Web Publication	02-Apr-2020

Correspondence Address:
Abdulrahman Khalid Alfadhel
PO Box 1291, Ar Rawdhah, Khurias Road, Riyadh
Saudi Arabia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JNSM.JNSM_47_19

Abstract

Diffuse idiopathic skeletal hyperostosis (DISH) is a noninflammatory condition that usually affects the elderly patients with several metabolic derangements. It is characterized by symptoms that mimic more sinister conditions such as stroke or malignancy. Indeed, this diagnosis can easily be “DISHmissed” if not considered in the differential diagnosis. Dysphagia is the most common symptom reported by patients with DISH. Always due to cervical osteophytes, dysphagia due to DISH has been described as DISHphagia. Extrapolation of this terminology to DISHphonia, DISHpnea, DISHpepsia, and DISHesthesia may increase awareness of the plethora of symptoms that may be caused by DISHplacement of anatomy. This is clinically significant because the prevalence of DISH is likely to increase, and DISH can be managed with conservative, medical, and surgical therapies.

Keywords: Diffuse idiopathic skeletal hyperostosis, dysphagia, stroke, swallowing

How to cite this article:
Alfadhel AK, Almanea AK, Rajendram R, Alsaad AG. DISHphagia and DISHphonia DISHguised as a stroke. J Nat Sci Med 2020;3:139-41

How to cite this URL:
Alfadhel AK, Almanea AK, Rajendram R, Alsaad AG. DISHphagia and DISHphonia DISHguised as a stroke. J Nat Sci Med [serial online] 2020 [cited 2021 Oct 19];3:139-41. Available from: https://www.jnsmonline.org/text.asp?2020/3/2/139/275400

Introduction

Diffuse idiopathic skeletal hyperostosis (DISH), first described as senile ankylosing hyperostosis by Forestier and Rotes-Querol, causes noninflammatory ossification and calcification of spinal ligaments.^[1],[2] The abnormal ossification in Forestier disease disrupts the anatomy causing a plethora of symptoms including odynophagia, dysphagia, aspiration, dysphonia, and airway obstruction.^[3],[4],[5]

Recent studies report more peculiar presentations such as sore throat, hoarseness, and total vocal cord paralysis.^[6],[7] These presentations often divert clinicians to consider any of a vast array of alternative diagnoses. This exposes patients to a battery of unnecessary investigations, prolongs admission to hospital, and often results in diagnostic error. This is particularly concerning because DISH can be managed with conservative, medical, and surgical therapies. To highlight this, we, therefore, describe the presentation of a patient with cervical DISHphagia who was initially misdiagnosed with a stroke.

Case Report

An 87-year-old obese Saudi man with hypertension, dyslipidemia, peripheral vascular disease, and hyperuricemia was admitted to hospital with “sudden onset” dysphagia to solids and liquids, which began 15 days before this presentation. This was associated with hoarseness of voice. His symptoms had been ascribed to a stroke at another hospital, although computed tomography (CT) of the brain was normal. Physical examination also revealed two smooth firm 1 cm × 1 cm mobile nodules on the right lower leg, an 8 cm × 6 cm ulcer on the medial aspect of the right thigh, packed with herbal medication and right inguinal lymphadenopathy. Full neurological examination, including assessment of the cranial nerves, was normal except for the patient's hoarse voice and inability to chew because of pain. However, there was oropharyngeal dysphagia with an obvious impediment to the elevation of the larynx on swallowing. On bedside swallowing assessment, the patient tolerated liquids well but had mild dysphagia with puree and so refused to try solids.

Imaging of the neck CT [Figure 1] revealed that the posterior pharynx was indented by exuberant anterior osteophytosis of C2, C3, C4, and C5 vertebrae. Anterior spinal surgery was not appropriate. The risk of perioperative mortality and morbidity was high, independent of the coincident diagnosis of metastatic melanoma. Hence, the DISHphagia was managed conservatively. He was advised on head maneuvers to facilitate swallowing. Liquid and pureed diet was recommended, and the formulations of his medications were changed from tablets to syrups.

Figure 1: (a and b) Imaging of the neck computed tomography [Figure 1] revealed that the posterior pharynx was indented by exuberant anterior osteophytosis of C2, C3, C4, and C5 vertebrae

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Investigations also revealed anemia and iron and folate deficiency. A chest X-ray revealed a 1 cm round pulmonary nodule. Chest CT revealed bilateral nodules of different sizes. The presence of right inguinal lymphadenopathy was confirmed with CT, and biopsy subsequently demonstrated metastatic melanoma. The patient was, therefore, also referred to oncology for the consideration for chemotherapy.

What Is the Diagnosis?

Discussion

DISH is characterized by superfluous hyperostosis of the anterolateral spinal column that expedites ankylosis and ossification of spinal ligaments. Epidemiological data on DISH are scarce, which may reflect the lack of a clear definition of DISH. However, some data on the prevalence of DISH are presented in [Table 1].

Table 1: The prevalence of diffuse idiopathic skeletal hyperostosis

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Dysphagia is the most common symptom and is reported by 15%–25% of patients with DISH. While always due to cervical osteophytes,^[16],[17] there is no correlation between the size of the osteophytes and symptoms. However, age does correlate with symptom severity.^[8],[18]

Patients with DISHphagia will initially cope subconsciously by adjusting the position of their neck while swallowing. Observation of this maneuver narrows the differential diagnoses substantially. This may also explain reports of “sudden onset” DISHphagia when the patient eventually consciously realizes that they must change the consistency of the food they eat.

These conscious and subconscious strategies often delay presentation until the patient can no longer cope with severe and often atypical symptoms. For example, one case report described vocal cord paralysis as a late presentation of DISH.^[7] Such atypical presentations raise the suspicion of more sinister diagnosis such as stroke or malignancy. This often leads to a battery of unnecessary investigations and prolongs admissions. Misdiagnosis can be avoided by taking a careful history and performing a thorough physical examination to exclude more sinister diagnoses.

Risk factors for DISH include age, diabetes, cardiovascular diseases, hypertension, and obesity.^[19],[20] As the prevalence of these risk factors is increasing, the prevalence of DISH is also likely to increase. Hence, clinicians should have a high index of suspicion for DISH in patients presenting with symptoms that may be related to the displacement of normal anatomy.

Anatomical imaging (e.g., X-ray or CT of the spine) is required to confirm the diagnosis. Plain X-ray has a high sensitivity and specificity for the diagnosis of DISH. However, the sensitivity of CT is higher than that of X-ray alone.^[14] This anatomical assessment can be complemented such functional assessment as using barium swallow, videofluoroscopy, functional endoscopic evaluation of swallowing, nasopharyngoscopy, or laryngoscopy complements.^[18],[21]

In the vast majority of cases, the initial management should be conservative. Specific advice from speech therapists, swallowing therapists, and dietary modifications may be guided by the functional diagnostic assessment. While nonsteroidal anti-inflammatory drugs and steroids may be beneficial, osteophytectomy is the definitive treatment. The surgical option is reserved for severe cases due the possibility of severe complication including esophageal perforation and injury of the superior and recurrent laryngeal nerve.^[18],[21]

The term DISHphagia has been coined to describe dysphagia due to DISH.^[22] However, almost any symptom can be caused by DISH. We, therefore, advocate the extrapolation of this terminology to include DISHphonia, DISHpnea, DISHpepsia, and DISHesthesia. This would highlight the plethora of other symptoms that may be caused by DISH. The description of the pathophysiology as DISHplacement of anatomical structures will help both physicians and patients understand the cause of the symptomatology and the approaches to treatment.

Final diagnosis

DISH causing dysphagia.

Clinicopathological Pearls

DISH is a common condition characterized by superfluous hyperostosis of the anterolateral spinal column
Dysphagia is the most common symptom reported by patients with DISH
Many symptoms can occur as a result of the displacement of normal anatomy caused by DISH
The use of terminology such as DISHphonia, DISHpnea, DISHpepsia, and DISHesthesia would highlight the plethora of symptoms which may be caused by DISH
The description of the pathophysiology as DISHplacement of anatomical structures may facilitate understanding of the etiology of the symptomatology and the approach to treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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