A 47-year-old healthy woman presented with sudden-onset, severe neck pain and stiffness associated with odynophagia, dysphagia, and low-grade fever (37.8°C). She described it as severe neck pain extending into the deep neck and radiating upward to the occiput and vertex of the head. She also complained of difficulty in opening her jaw and swallowing due to throat pain. There was no preceding history of trauma, upper respiratory tract infection, or dental infection. The laboratory data showed slight leukocytosis of 12,350 cells/μL (8,500 neutrophils/µL), as well as an increased erythrocyte sedimentation rate of 55 mm/h and C-reactive protein of 43 mg/L. On physical examination, there was a severely limited range of all neck motion, with marked nuchal rigidity. Kernig and Brudzinski signs were not clearly positive. Mouth opening was difficult, but oropharyngeal examination was normal. The patient was admitted with the differential diagnosis of meningitis, retropharyngeal abscess, or infectious spondylitis.
Assessment
Cranial computed tomography (CT) scan was normal. A lumbar puncture was performed and demonstrated an opening pressure of 18 cm H2O, without alterations in the cerebrospinal fluid. A flexible nasopharyngolaryngoscopy showed posterior pharyngeal wall swelling overlying arytenoids. The findings in a cervical CT (Figure 1) and magnetic resonance imaging (MRI; Figure 2) demonstrated marked thickening and swelling of the prevertebral soft tissues from C1 to C3 and calcific deposits adjacent to the cortical margin of the anterior arch of C1.
Diagnosis
The patient was diagnosed with acute calcific tendinitis of the longus colli, and infrequent presentation of basic calcium phosphate (BCP) crystal deposition disease.1, 2, 3 The longus colli muscle is a weak flexor of the neck, composed of three portions with superior, central, and inferior fibers. It extends from the level of the anterior tubercle of the atlas into the superior mediastinum to the level of the T3 vertebral body. Calcification involves mainly the superior fibers, which attach the tubercle of the atlas to the transverse processes of the C3-C5 vertebrae. Histopathologically, biopsy demonstrates a foreign-body inflammatory response to amorphous hydroxyapatite deposition in the longus colli muscle.
This rare condition usually affects patients who are between 30 and 60 years old, without gender predilection. The associated symptoms are usually nonspecific and include acute severe neck pain and stiffness, limited cervical motion, dysphagia, odynophagia, and occipital headache. In some cases, there may be fever, with increased inflammatory markers.
It can be misdiagnosed as other life-threatening conditions, including retropharyngeal abscess, infectious spondylitis, or meningitis, resulting in unnecessary medical or surgical interventions.
The diagnosis is usually made by detecting amorphous calcification anterior to the C1-C2 level and prevertebral soft tissue swelling extending from C1 to C5-C6 on plain radiographs and CT, of which CT is more sensitive than radiography for the detection of calcification and prevertebral soft tissue swelling. It represents the method of choice, as it also allows bone destruction or fracture to be ruled out. Retropharyngeal soft tissue edema and muscle inflammation can be better visualized by MRI, which eliminates retropharyngeal abscess and cervical spondylitis.
Thus, a correct diagnosis can be made easily by combining CT and MRI. The importance of recognizing this pathology lies in preventing its misdiagnosis and mismanagement.
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-Javier Narváez, MD, PhD, Isabel Morales-Ivorra, MD, Sergio Martínez-Yelamos, MD, PhD, Jose Antonio Narváez, MD, PhD
This article originally appeared in the September issue of The American Journal of Medicine.
