Unilateral Vocal Cord Paresis Caused by Diffuse Idiopathic Skeletal Hyperostosis: Case Report and Literature Review

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Background/Objectives: Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by calcification and ossification of ligaments and tendons, primarily affecting the spine. While often asymptomatic, DISH in the cervical spine can cause dysphagia and, more rarely, vocal cord paralysis due to compression of the recurrent laryngeal nerve at the cricothyroid joint. Here, we report cases of unilateral vocal fold paresis in two patients with DISH. Case Presentation: Our first case is an 80-year-old male presented with two months of dysphonia. Strobovideolaryngoscopy found left vocal fold paresis with glottic insufficiency. Computed Tomography (CT) imaging showed DISH with large anteriorly projecting osteophytes in the cervical spine causing rightward deviation of the laryngeal structures and compressing the cricothyroid joint. Second, a 30-year-old female with Turner Syndrome and subglottic stenosis who developed progressively worsening dysphonia over 6 months, characterized by diminished voice projection and clarity. Strobovideolaryngoscopy revealed a mild-to-moderate right vocal fold paresis. CT of the neck demonstrated multiple right-sided osteophytes projecting into the right tracheoesophageal groove, along the course of the right recurrent laryngeal nerve, in the absence of significant disc degeneration. Discussion and Conclusions: On our review of the literature, no other similar instances of unilateral vocal fold paresis were found. We present these cases to emphasize the need for early recognition and treatment to prevent symptom progression of DISH.

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  • 10.1542/neo.14-10-e521
Index of Suspicion in the Nursery
  • Oct 1, 2013
  • NeoReviews
  • Notie Erhahon + 8 more

A term male infant is transferred to the NICU for persistent hypoglycemia at age 3.5 hours. The infant had a blood sugar level of 21 mg/dL at age 3 hours and despite refeeding 20 mL of 20 calories per ounce of formula, his blood glucose level remained at 20 mg/dL at 3.5 hours. The infant was born via cesarean delivery for a nonreassuring heart rate tracing to a 19-year-old G1P 0 African-American mother. All prenatal laboratory results were negative, including an oral glucose tolerance test. Prenatal ultrasonography was performed during the second trimester and showed no gross fetal anomalies other than the presence of a two-vessel cord. The infant cried spontaneously at birth and had some transient respiratory distress. The infant weighs 2,960 g (50th percentile) with a length of 48 cm (50th percentile) and a head circumference of 32.5 cm (25th–50th percentile). Results of the physical examination are normal except for a small penis (stretched penile length of 1.5 cm, width of 0.6 cm) and an undescended left testicle. Blood glucose levels below 40 mg/dL persist despite two boluses of 10% dextrose and administration of continuous dextrose infusion with a glucose infusion rate of ∼5 mg/kg per minute. Glucose levels above 40 mg/dL are eventually achieved with a glucose infusion rate of 8 mg/kg per minute. The pediatric endocrinology service was consulted on the second day for persistent hypoglycemia. A 3,490-g term male infant is born to a 20-year-old primigravida mother. She received good prenatal care, and her pregnancy was uncomplicated. Antenatal screening for human immunodeficiency virus, hepatitis B, and syphilis were negative, and she had immunity against rubella. Results of the patient’s cervical culture were positive for group B streptococcus, but she received adequate intrapartum penicillin prophylaxis before delivery. There was no abnormality noted on the fetal cardiotocography. …

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  • Cite Count Icon 2
  • 10.3389/fsurg.2022.963399
Case Report: Diffuse idiopathic skeletal hyperostosis with ossification of the posterior longitudinal ligament in the cervical spine: A rare case with dysphagia and neurological deficit and literature review
  • Aug 9, 2022
  • Frontiers in Surgery
  • Chaoyuan Li + 4 more

Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by the calcification and ossification of ligaments and tendons. Progressive dysphagia caused by DISH-related anterior cervical osteophytes and deteriorating dysphagia caused by DISH combined with neurological dysfunction resulting from the posterior longitudinal ligament is rare. The initial diagnosis is misleading and patients often consult several specialists before spine surgeons. This study aims to provide a comprehensive review of the literature on this challenging pathological association. We also present a case illustration where a 53-year-old man presented with progressive dysphagia and foreign body sensation in the pharynx, accompanied by a neurological numbness defect in the right upper limb. Radiography and computed tomography confirmed the existence of osteophytes at the anterior edge of the C4–C7 pyramid and ossification of the posterior longitudinal ligament, in which the giant coracoid osteophyte could be seen at the anterior edge of the C4–C5 pyramid. The anterior cervical osteophyte was removed, and decompression and fusion were performed. The symptoms were relieved postoperatively. No recurrence of symptoms was found during the six-month follow-up. Spine surgeons should consider progressive dysphagia caused by DISH-related osteophytes at the anterior edge of the cervical spine as it is easily misdiagnosed and often missed on the first evaluation. When combined with ossification of the posterior longitudinal ligament, following cervical osteophyte resection it is necessary to consider stabilizing the corresponding segments via fusion.

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  • Cite Count Icon 6
  • 10.1097/corr.0000000000001719
An Unrecognized Ligament and its Ossification in the Craniocervical Junction: Prevalence, Patient Characteristics, and Anatomic Evidence.
  • Mar 15, 2021
  • Clinical Orthopaedics & Related Research
  • Bingxuan Wu + 9 more

Background In the craniocervical junction, the ligaments between the anterior foramen magnum and the anterior arch of the atlas are not well defined, and ossification of the ligaments in this region has rarely been reported. Characterizing the anatomy and ossification of these ligaments may help in the diagnosis and treatment of disorders in this region. Questions/purposes (1) What is the prevalence of an unrecognized ossification at the craniocervical junction in patients with cervical spine disorders, and what are the patient characteristics associated with this ossification? (2) Do patients with this ossification have a greater risk of ossification of other structures at the craniocervical junction or cervical spine? (3) Is there an unreported ligament at this ossified site? Methods We conducted a retrospective study of 578 hospitalized patients who underwent CT for cervical spine disorders between January 2016 and July 2020. Based on the inclusion criteria, 11% (66 of 578) were excluded because of a cervical or craniocervical tumor, deformity, infection, fracture or dislocation, or prior surgery, leaving 89% (512 of 578) for analysis. These 512 patients had diagnoses of cervical radiculopathy, cervical myelopathy, cervical spondylotic amyotrophy, cervical spinal cord injury without a radiographic abnormality, or axial neck pain. Their mean age was 57 years (range 22-90 years), and 60% of the patients were men. Patient characteristics including age, gender, and diagnosis were retrieved from a longitudinally maintained institutional database. CT images were used to assess the presence of a previously unrecognized ossification and ossification of other structures in the craniocervical junction and cervical spine, including the posterior longitudinal ligament, anterior longitudinal ligament, nuchal ligament, ligamentum flavum, transverse ligament, and apical ligament, as well as diffuse idiopathic skeletal hyperostosis (DISH). The association between these structures was also assessed. This unreported ossification was called the capped dens sign. It was defined and graded from 1 to 3. Grade 3 was defined as the typical capped dens sign. Cervical spine MRI was used to assess whether there was an unreported structure in the same region as where the capped dens sign was detected on CT images. In the database of a recent study, there were 33 patients younger than 41 years. Nine percent (three of 33) were excluded because they did not have cervical spine MRI. MRIs of the remaining 30 patients were assessed. Their mean age was 35 years (range 22-40 years), and 58% were men. All cervical spine CT images and MRIs were reviewed by one senior spine surgeon and one junior spine surgeon twice with a 2-week interval. Blinding was accomplished by removing identifying information from the radiographs and randomly assigning them to each examiner. Any discrepancy with respect to the grade of the capped dens sign was adjudicated by a third blinded senior spine surgeon. Intrarater and interrater reliabilities were assessed by calculating weighted kappa statistics. No ligament or membrane was reported at this site. MRI is not sensitive to identify thin tissue in this region, especially when severe degeneration has occurred. A cadaveric study was conducted to discover a potential ligament between the inferior margin of the foramen magnum and the anterior arch of the atlas, as prompted by the newly discovered ossification in the clinical analysis of this study. Six embalmed human cadaveric craniocervical regions (three male and three female cadavers; median age 56 years, range 45-78 years) were dissected by a senior anatomist and a senior anatomy technician. A mid-sagittal section of the craniocervical junction was created, allowing us to explore the interval between the anterior foramen magnum and anterior arch of the atlas. A histologic analysis was conducted in two of the six cadavers (a male cadaver, 45 years; and a female cadaver, 51 years). Slides were made with 4-µm sections and stained with hematoxylin and eosin. Results A novel capped dens sign was detected in 39% (198 of 512) of the patients and the most typical capped dens sign was detected in 19% (96 of 512) of patients. The prevalence of this sign was the highest in patients with cervical spondylotic amyotrophy (12 of 25 patients). The prevalence of ossification of the anterior longitudinal ligament, ligamentum nuchae, and apical ligament, as well as DISH, was higher in patients with a capped dens sign than in those without (p = 0.04, p < 0.001, p < 0.001, and p = 0.001, respectively). The capped dens sign was identified in 69% (18 of 26) of the patients with DISH. A thin and short band-like structure or osteophyte was detected on MRI in 87% (26 of 30), in the same region as the capped dens sign. In the cadaveric study, an unreported, distinct ligamentous structure was identified at this ossified site. It originated from the posterosuperior rim of the anterior arch of the atlas to the inferior margin of the foramen magnum, which we called the inter-atlanto-occipital ligament. It was found in all six dissected craniocervical junctions. The histologic analysis revealed dense connective tissue. Conclusion More than one-third of the patients in this series demonstrated CT evidence of a previously unrecognized ossification in the craniocervical junction, which we called the capped dens sign. Anatomic evidence of this sign, which was a previously unidentified ligament, was also newly discovered in this region. This study was conducted among Asian patients and specimens. Further studies among diverse ethnic groups may be needed to generalize the results. An additional well-designed prospective study will be needed to provide further evidence regarding the potential pathophysiology and clinical relevance of the capped dens sign. Furthermore, the cadaveric analysis in this study was only a preliminary report of the ligament; further biomechanical research is needed to investigate its function. Clinical Relevance Knowledge of this novel ligament may improve the diagnosis and treatment of craniocervical stability and dislocation. Ossification of this ligament is correlated with age, cervical spondylotic amyotrophy, and DISH. We wonder whether patients with cervical degenerative disorders who also have a capped dens sign may be at risk for the formation of osteophytes of an uncovertebral joint, which may result in palsy of the upper limb muscles. The capped dens sign may be the craniocervical manifestation of DISH. This possible association between the capped dens sign and DISH should be considered when performing surgery on patients with the capped dens sign.

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  • Cite Count Icon 1
  • 10.1016/j.oooo.2018.07.029
DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS: AN INCIDENTAL FINDING ON CBCT RADIOGRAPHIC ASSESSMENT
  • Dec 14, 2018
  • Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
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DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS: AN INCIDENTAL FINDING ON CBCT RADIOGRAPHIC ASSESSMENT

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  • 10.1080/02656736.2017.1328130
Vocal cord paresis following single-session high intensity focused ablation (HIFU) treatment of benign thyroid nodules: incidence and risk factors
  • Jun 6, 2017
  • International Journal of Hyperthermia
  • Brian H H Lang + 2 more

Background: Vocal cord paresis (VCP) may occur following high intensity focused ultrasound (HIFU) of thyroid nodules. We hypothesised its occurrence relates to the distance of the focus point (FP) of the HIFU beams from the recurrent laryngeal nerve (RLN) and the thermal power that this point received. Their relationships were examined.Methods: One hundred and three patients who underwent HIFU for symptomatic benign thyroid nodule from October 2015 to March 2017 were analysed. All treatment images were captured and were later watched by 2 reviewers to identify three FPs closest to the tracheoesophageal groove (TEG) on transverse sonographic view. TEG was taken as the RLN position. After identifying these FPs, their distance (mm) from the TEG, thermal power (W) used and depth from skin (mm) were recorded. These parameters were compared between those with and without VCP. VCP was defined as a cord with reduced or no movement.Results: Four (3.9%) patients suffered from a unilateral VCP afterwards but they all recovered fully within 6 weeks. There were no significant differences in baseline characteristics and treatment efficacy between the two groups. The distance from TEG (OR = 1.706, 95%CI = 1.001 to 2.915, p = 0.050) was the only significant factor for VCP. None of the other variables including thermal power were significant.Conclusions: The incidence of VCP was 3.9% (4/103) and they completely recovered within 6 weeks. The distance between the FP and the TEG was the only related factor for VCP. The safe distance between FP and TEG should be ≥1.1 cm.

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  • Cite Count Icon 44
  • 10.1097/bsd.0000000000000701
Prevalence and Distribution of Diffuse Idiopathic Skeletal Hyperostosis on Whole-spine Computed Tomography in Patients With Cervical Ossification of the Posterior Longitudinal Ligament
  • Nov 1, 2018
  • Clinical Spine Surgery: A Spine Publication
  • Soraya Nishimura + 36 more

This was a retrospective multicenter study. To clarify the progression of diffuse idiopathic skeletal hyperostosis (DISH) using whole-spine computed tomography in patients with cervical ossification of the posterior longitudinal ligament (OPLL). DISH and cervical OPLL frequently coexist, and can cause ankylosing spinal fractures due to biomechanical changes and fragility of the affected vertebrae. The epidemiology and pathophysiology of DISH occurring with cervical OPLL are unclear. We used whole-spine computed tomography to determine the prevalence of DISH in 234 patients with a diagnosis of cervical OPLL based on plain cervical radiographs. We established a novel system for grading the progression of DISH based on a cluster analysis of the DISH distribution along the spine. We calculated the correlation coefficient between this grading system and patient age. The prevalence of DISH in patients with cervical OPLL was 48.7%. Patients with DISH were significantly older than those who did not have DISH (67.3 vs. 63.4 y; P=0.005). Cluster analysis classified the DISH distribution into 6 regions, based on the levels affected: C2-C5, C3-T1, C6-T5, T3-10, T8-L2, and T12-S1. DISH was observed most frequently at T3-T10. We defined a system for grading DISH progression based on the number of regions involved, from grade 0 to 6. DISH was distributed at T3-T10 in >60% of the grade 1 patients, whereas most patients with DISH at the cervical or lumbar spine were grade 4 or 5. There was a weak but significant correlation between the DISH grade and patient age. DISH was present in nearly half of the patients with cervical OPLL. DISH was more common in older patients. DISH developed at the thoracic level and progressed into the cervical and/or lumbar spine with age. Level III.

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  • Cite Count Icon 27
  • 10.1007/bf03009319
Diffuse idiopathic skeletal hyperostosis: an unusual cause of difficult intubation.
  • Jan 1, 1993
  • Canadian Journal of Anaesthesia
  • Edward T Crosby + 1 more

A case is reported in which anterior osteophytes on the cervical vertebra, in combination with a subglottic stenosis, resulted in distortion of the airway and led to unexpected difficulties during intubation. The osteophytes, associated with the syndrome of diffuse idiopathic skeletal hyperostosis (DISH) were centred at the midcervical level and resulted in anterior displacement of the larynx with an acute angulation of the trachea just below the larynx. This acute angulation, immediately above an unrecognized subglottic stenosis, rendered it impossible to pass all but the smallest endotracheal tube. Diffuse idiopathic skeletal hyperostosis is an ossifying diathesis leading to bone formation in spinal and extraspinal sites, paravertebral osteophyte formation and ligamentous calcification and ossification. Ossification of the anterior longitudinal ligament is common, may be discontinuous, and is often more marked in the thoracolumbar spine than elsewhere. However, isolated and predominant cervical spinal involvement may occur. Diffuse idiopathic skeletal hyperostosis occurs primarily in the elderly population and is often associated with the syndromes of osteoarthritis and ossification of the posterior longitudinal ligament (OPLL). Difficult intubation resulting from anatomical abnormalities of the cervical spine is rare. Although radiological evaluation may be useful in assessing the airway in patients deemed to be at risk for difficult intubation, it cannot be recommended for screening patient populations on a routine basis because of the cost and anticipated extremely low yield. Careful clinical evaluation of the airway before operation and having an approach to the unexpected difficult intubation are emphasized.

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Exploring the association between insulin resistance and diffuse idiopathic skeletal hyperostosis: a cross-sectional observational study.
  • Dec 1, 2025
  • The spine journal : official journal of the North American Spine Society
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Exploring the association between insulin resistance and diffuse idiopathic skeletal hyperostosis: a cross-sectional observational study.

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  • 10.3390/jcm10184137
Associations between Clinical Findings and Severity of Diffuse Idiopathic Skeletal Hyperostosis in Patients with Ossification of the Posterior Longitudinal Ligament
  • Sep 14, 2021
  • Journal of Clinical Medicine
  • Takashi Hirai + 37 more

Background: This study investigated how diffuse idiopathic skeletal hyperostosis (DISH) influences clinical characteristics in patients with cervical ossification of the posterior longitudinal ligament (OPLL). Although DISH is considered unlikely to promote neurologic dysfunction, this relationship remains unclear. Methods: Patient data were prospectively collected from 16 Japanese institutions. In total, 239 patients with cervical OPLL were enrolled who had whole-spine computed tomography images available. The primary outcomes were visual analog scale pain scores and the results of other self-reported clinical questionnaires. Correlations were sought between clinical symptoms and DISH using the following grading system: 1, DISH at T3-T10; 2, DISH at both T3–10 and C6–T2 and/or T11–L2; and 3, DISH beyond the C5 and/or L3 levels. Results: DISH was absent in 132 cases, grade 1 in 23, grade 2 in 65, and grade 3 in 19. There were no significant correlations between DISH grade and clinical scores. However, there was a significant difference in the prevalence of neck pain (but not in back pain or low back pain) among the three grades. Interestingly, DISH localized in the thoracic spine (grade 1) may create overload at the cervical spine and lead to neck pain in patients with cervical OPLL. Conclusion: This study is the first prospective multicenter cross-sectional comparison of subjective outcomes in patients with cervical OPLL according to the presence or absence of DISH. The severity of DISH was partially associated with the prevalence of neck pain.

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A Case Report of Early Surgical Intervention for Diffuse Idiopathic Skeletal Hyperostosis of the Cervical Spine: Challenges in Management
  • Aug 5, 2023
  • Iranian Journal of Neurosurgery
  • Misagh Shafizad + 4 more

Background and Importance: Diffuse idiopathic skeletal hyperostosis (DISH) can compress the trachea and esophagus when located in the cervical spine. In this report, we investigated whether it is preferable to perform the early surgical intervention in symptomatic patients or to wait and administer supportive care and perform late surgical intervention regardless of whether symptoms progress or not. Case Presentation: We present the case of a 70-year-old patient with diffuse idiopathic skeletal hyperostosis (DISH) causing significant dysphagia and unilateral vocal cord paresis, resulting in dyspnea and stridor. Imaging diagnostics revealed large osteophytes anterior to the cervical spine from C3 to C6 compressing the cervical spine. Significant clinical improvement was observed following the anterior resection of the patient's osteophytes. Conclusion: In order to achieve higher success and less recurrence, it is preferable to perform surgical intervention earlier in the disease's progression. However, more studies are necessary to confirm this because most of the present results are from case report articles and have less evidence.

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Within Normal Limits
  • Sep 1, 2016
  • Emergency Medicine News
  • Danielle Williams + 2 more

Figure: A lateral radiograph of the thoracic spine, left, shows bulky bridging osteophytes (arrowheads) along the anterior thoracic spine involving greater than 4 contiguous vertebral bodies compatible with DISH. A fracture along the inferior endplate of the T8 vertebral body is barely perceptible (arrow). A sagittal CT scan, center, of the thoracic spine shows an acute, minimally displaced fracture of the middle to inferior half of the T8 vertebral body which extends into and widens the disc space (arrow). Note the extent of DISH at levels above and below the fracture. A sagittal CT scan of the thoracic spine, right, further shows the extent of the fracture and intact bulky osteophytes above and below the fracture site (arrow).FigureFigureFigureA 66-year-old man was brought to the emergency department after being in a motor vehicle crash in which he was rear-ended with very low impact. He complained of point tenderness in the lower thoracic spine, and a trauma workup was performed according to routine protocol. Thoracic radiographs showed evidence of bulky bridging osteophytes consistent with DISH (diffuse idiopathic skeletal hyperostosis) but no evidence of a fracture at the site of pain. Further evaluation with CT showed a displaced fracture of the T8 vertebral body, which extended through the disc space. The patient was placed in a brace and managed for non-life-threatening injuries, and was then discharged home. DISH most commonly occurs in the thoracic spine, typically involving T7-T11 levels. Most patients with DISH are discovered incidentally on imaging because they are often asymptomatic. Occasionally, patients can present with pain, postural instability, neurological symptoms, or dysphagia. A DISH diagnosis is typically made on radiography, followed by cross-sectional imaging, namely CT or MRI. It is characterized by the presence of ‘‘flowing’’ bulky ossifications along the anterolateral margin of at least four contiguous vertebral bodies, preservation of the intervertebral disk height without any degenerative-related findings and absence of apophyseal joint ankyloses, or fusion at the costovertebral or sacroiliac joints. Imaging features are characteristic, but the differential diagnosis for DISH include seronegative spondyloarthropathies such as ankylosing spondylitis, reactive arthritis and psoriatic arthritis. Unlike ankylosing spondylitis, fusion of the sacroiliac, facet, and uncovertebral joints do not occur in DISH. Similarly, reactive arthritis has asymmetric sacroiliac joint involvement and psoriatic arthritis has a predilection for the cervical and lower lumbar spine, while DISH occurs most commonly in the thoracic spine. The fused spine in DISH or any preexisting fusion-type condition is more prone to fracture than a normal spine, and these fractures can involve multiple vertebral columns and can occur after even minor trauma. Patients with moderate to severe disease are more prone to fractures. The thoracic and cervical spine are most commonly involved. Hyperextension is the most common mechanism of injury resulting in DISH-related spinal fractures. Acute spinal fractures are not common, but can lead to neurological injury, nonunion, deformity, or death. DISH produces broad bridging osteophytes that encompass the anterior longitudinal ligament, annulus fibrosis, and paraspinal connective tissues. The bridging osteophytes are most robust at the intervertebral disk space, extending to the adjacent vertebral body. The proximal and distal thirds of a vertebral body, therefore, are typically covered by ossifications, leaving the mid-vertebral body above and below the site of attachment of the ossifications most vulnerable to fractures. Alternatively, fractures may occur at the end of a fused segment, involving the vertebral body at the junction of fused and unfused spine. Radiographs of the spine may be initially obtained if fractures are suspected following minor trauma. Fractures may not be easily detected with radiographs in the patients with DISH or any other spinal fusion because of overlapping bone and soft tissues and the presence of productive bone formation. It is therefore reasonable to conclude from this case that a low threshold must be used to order a CT scan of the spine if patients with spinal fusion sustain minor trauma. This will not only assist in making an early and accurate diagnosis but ensure appropriate and timely treatment for patients. Share this article on Twitter and Facebook. Access the links in EMN by reading this on our website or in our free iPad app, both available at www.EM-News.com. Comments? Write to us at [email protected].

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  • Cite Count Icon 14
  • 10.1002/ajmg.a.61274
Molecular characterization of known and novel ACVR1 variants in phenotypes of aberrant ossification.
  • Jun 26, 2019
  • American Journal of Medical Genetics Part A
  • Aditi Gupta + 10 more

Diffuse idiopathic skeletal hyperostosis (DISH) is a disorder principally characterized by calcification and ossification of spinal ligaments and entheses. Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disabling disorder characterized by progressive ossification of skeletal muscle, fascia, tendons, and ligaments. These conditions manifest phenotypic overlap in the ossification of tendons and ligaments. We describe herein a patient with DISH, exhibiting heterotopic ossification of the posterior longitudinal ligament where clinical whole exome sequencing identified a variant within ACVR1, a gene implicated in FOP. This variant, p.K400E, is a novel variant, not identified previously, and occurs in a highly conserved region across orthologs. We used sequence-based predicative algorithms, molecular modeling, and molecular dynamics simulations, to test the potential for p.K400E to alter the structure and dynamics of ACVR1. We applied the same modeling and simulation methods to established FOP variants, to identify the detailed effects that they have on the ACVR1 protein, as well as to act as positive controls against which the effects of p.K400E could be evaluated. Our in silico molecular analyses support p.K400E as altering the behavior of ACVR1. In addition, functional testing to measure the effect of this variant on BMP-pSMAD 1/5/8 target genes was carried out which revealed this variant to cause increased ID1 and Msx2 expression compared with the wild-type receptor. This analysis supports the potential for the variant of uncertain significance to contribute to the patient's phenotype.

  • Research Article
  • Cite Count Icon 1
  • 10.32412/pjohns.v29i2.429
Diffuse Idiopathic Skeletal Hyperostosis: A Rare Cause of Dysphagia
  • Nov 30, 2014
  • Philippine Journal of Otolaryngology-Head and Neck Surgery
  • Anuar Khairullah + 2 more

Diffuse idiopathic skeletal hyperostosis (DISH) is a disease characterized by massive, non-inflammatory ossification with intensive formation of osteophytes affecting ligaments, tendons, and fascia of the anterior part of the spinal column, mostly in the middle and lower thoracic regions. However, isolated and predominant cervical spinal involvement may occur. It has predilection for men (65%) over 50 years of age and a prevalence of approximately 15-20% in elderly patients.1 A CT scan is one of the diagnostic tools. The radiographic diagnostic criteria in the spine include: 1) osseous bridging along the anterolateral aspect of at least four vertebral bodies; 2) relative sparing of intervertebral disc heights, with minimal or absent disc degeneration; and 3) absence of apophyseal joint ankylosis and sacroiliac sclerosis.2 We present a rare case of dysphagia over 2 years duration due to DISH.
 Case Report
 A 55-year-old Malay man presented with intermittent dysphagia for 2 years duration. He denied foreign body ingestion, globus sensation or any laryngeal trauma, shortness of breath, hoarseness or any neurological deficits. A solitary smooth mass on the right posterolateral pharyngeal wall that was hard in consistency was appreciated on oropharyngeal examination. (Figure 1) There was no significant cervical lymphadenopathy and the neurological examination was unremarkable. Cervical Radiographs and CT scan showed marked ossification at the right anterolateral aspect of cervical vertebral bodies C2 to C7 most probably representing a Diffuse Idiopathic Skeletal Hyperostosis. (Figures 2, 3) He was treated conservatively with 6-monthly follow up.
 Discussion
 Diffuse Idiopathic Skeletal Hyperostosis (DISH) is an ossifying diasthesis characterized by the thickening and calcification of soft tissue (ligaments, tendons and joint capsule) resulting in secondary formation of osteophytes. Most commonly it affects the paraspinal ligaments, predominantly the anterior longitudinal ligament and occasionally the posterior longitudinal ligament.2 It was first described as senile ankylosing hyperostosis of the spine by Forestier and Rodes Querol in 1950.3 In 1970 Resnick et al. coined the term DISH for this systemic entity. Radiologically, they established 3-diagnostic criteria which include 1) Presence of flowing ossification of anterior longitudinal ligament of at least four contiguous vertebral bodies, 2) Preservation of intervertebral disc height, and 3) Absence of apophyseal joint ankylosis or sacroiliac joint erosion, sclerosis or fusion.4
 Cervical anterior osteophytes accompanying DISH are mostly asymptomatic. They may present with cervical pain and stiffness. Large osteophytes however do cause dysphagia and it is the most common presenting complaint, affecting 17 – 28% of patients.5 Many different mechanisms have been suggested as the cause of the dysphagia including mass effect on the esophagus by the osteophytes and neuropathy due to recurrent laryngeal nerve impingement.5,6 According to LIn et al., in addition to distortion of laryngoesophageal anatomy and functions, osteophytes of cervical vertebrae can alter the mechanics of pharyngeal swallowing leading to secondary inflammation and edema of mucosa and soft tissue.6 Although airway symptoms in patients with DISH appear to be rare, clinicians should be aware of this condition and its potential for acute respiratory complications.
 The etiology of DISH is still unclear, however according to Calisanellerr et al. it may be associated with excessive mechanical stress, hyperlipidaemia, increased levels of insulin with or without diabetes mellitus, increased levels of insulin-like growth factor-1 and hyperuricaemia.7 A positive HLA–B8 has also been reported, and hypervascularity may also play a role in the etiopathogenesis of DISH.7,8,9
 Differential diagnosis of DISH includes ankylosing spondylitis, spondylosis deformans, osteoarthritis and esophageal malignancies where it should be considered when the dysphagia cannot be explained by small anterior osteophytes.5
 Treatment can be divided into conservative treatment with dietary modification, swallowing therapy sessions and analgesia for early stages of mild dysphagia. Chiropractic treatment and acupuncture are popular alternatives among patients. The benefit of chiropractic therapy may lie in its role in increasing range of movement of the spine and providing pain relief.10 When conservative treatment fails, surgical interventions such as osteophytectomy, tracheotomy and feeding tube insertion are indicted. Surgical excision via perioral transpharyngeal route for C1 and C2 vertebrae or anterior cervical approach for C3 to C7 vertebrae is preferred.6,11 The aim of the surgery is to provide satisfactory decompression of the esophagus.6 Recent studies have shown that patients treated surgically with osteophytectomy had marked improvement, if not complete resolution, of their upper aerodigestive disturbances.11 It should be remembered that surgical interventions harbor the risk of recurrent laryngeal nerve injury, Horner’s syndrome, cervical instability, persistent symptoms, and recurrence.11
 Dysphagia caused by diffuse idiopathic skeletal hyperostosis is an uncommon entity. Radiological evaluation specifically CT scans are diagnostic and can rule out other possible causes of oropharygeal mass. Surgical decompression may relieve the dysphagia when conservative treatments fail.

  • Research Article
  • Cite Count Icon 38
  • 10.1097/01.brs.0000214949.75361.f4
Diffuse Idiopathic Skeletal Hyperostosis Associated With Risk Factors for Stroke
  • Apr 1, 2006
  • Spine
  • Nobuhiko Miyazawa + 1 more

The incidence of risk factors for cerebrovascular diseases was investigated in patients with diffuse idiopathic skeletal hyperostosis (DISH) and patients with cervical spondylosis. To investigate the association between DISH and cerebrovascular disease. DISH is a common skeletal disease mainly affecting the anterior and lateral spinal longitudinal ligaments. The principal clinical features are nonradicular pain, stiffness, dysphagia (cervical portion), and associated ossification of the posterior longitudinal ligament. Age- and sex-matched patients were divided into three groups: 45 patients with DISH, 45 patients with cervical spondylosis Grade I and II, and 45 patients with cervical spondylosis Grade III and IV. Anthropometric, laboratory, and magnetic resonance (MR) imaging findings were analyzed. The values of uric acid (P = 1.60 x 10) and alkaline phosphatase (P = 2.00 x 10) were significantly greater in patients with DISH than in the other groups. Patients with DISH had a significantly higher incidence of ossification of the posterior longitudinal ligament (P = 5.21 x 10). Stiffness was significantly more common in patients with DISH and patients with spondylosis Grade III and IV than in patients with spondylosis Grade I and II (P = 0.000232). The incidence of infarction on MR imaging was significantly higher in patients with DISH than in the other groups (P = 0.0120). The incidence of stenosis or occlusion of a major cerebral artery on MR angiography was significantly higher in patients with DISH than in the other groups (P = 0.00264). DISH is associated with increased incidences of risk factors for stroke and cerebrovascular disease.

  • Research Article
  • 10.4081/reumatismo.2024.1692
Diffuse idiopathic skeletal hyperostosis is associated with greater complexity of coronary artery disease burden on coronary angiography.
  • Oct 1, 2024
  • Reumatismo
  • G Adami + 9 more

Diffuse idiopathic skeletal hyperostosis (DISH) is a common disorder characterized by ossification of tendons and ligaments. DISH has been largely associated with an increased risk of metabolic syndrome and type 2 diabetes. The objective of the present study is to investigate the role of DISH in the risk of coronary artery disease (CAD). We conducted an observational cross-sectional study of patients without a history of rheumatic musculoskeletal diseases who underwent coronary angiography between March 2016 and April 2021. The SYNergy between percutaneous coronary intervention with TAXus and cardiac surgery (SYNTAX) score was calculated based on coronary angiography images. DISH diagnosis was based on standard X-ray images and computed tomography scans (Resnick criteria). Demographic and clinical characteristics were retrieved from electronic medical records. Multinomial and binary logistic regression models were employed to determine the association between SYNTAX score (dependent variable) and DISH (independent variable). The study included 187 patients, 82.9% of whom were men, with valid radiological imaging. 83 (44.4%) patients had a confirmed radiological diagnosis of DISH. Diagnosis of DISH was associated with a higher SYNTAX score [adjusted odds ratio (aOR) 34.1, 95% confidence interval (CI) 1.41-79.2 p=0.049], independently from traditional cardiovascular risk factors. In patients aged <70 years, DISH was associated with a 7-fold higher risk of belonging to the highest category of SYNTAX (≥34), compared to non-DISH (aOR 7.23, 95% CI 1.08-48.4; p=0.041). The extension of vertebral calcification was significantly associated with SYNTAX score (r2 0.378, p<0.0001). DISH diagnosis is common in patients at high risk of cardiovascular disease or with definitive CAD. DISH was independently associated with higher CAD complexity.

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