Laboratory Studies
In relapsing polychondritis and chondroradionecrosis, no specific laboratory tests confirm the presence of the disease. However, some common laboratory findings in relapsing polychondritis include normochromic normocytic anemia, mild leukocytosis, and raised erythrocyte sedimentation rate (ESR).
Imaging Studies
CT scanning allows visualization of cartilage and soft tissue components and can be used to monitor the response to medical management, such as the use of steroid therapy in relapsing polychondritis. However, because MRI has been proven to be more effective at distinguishing between fibrosis, inflammation, and edema than CT scanning, some believe that MRI is a useful adjunct.
In 1993, Briggs demonstrated that CT scanning and MRI frequently do not differentiate between radionecrosis and recurrent tumor. [15] He cites problems with patient movement artifact secondary to pain, airway compromise, and aspiration during scanning.
Positron emission tomography (PET) scanning, according to a 1995 study by McGuirt, has proved most reliable, with the ability to differentiate tumor from radiation effect in more than 80% of cases. [1] The resolution of PET scanning, however, is 6 mm. Therefore, microscopic deposits of cancer may not be observed, and larger deposits may be masked by pooled saliva.
A modified barium swallow may be useful in the evaluation of a patient with laryngeal chondronecrosis, allowing the clinician to evaluate not only the patient's ability to swallow but also the ability to protect the airway. The images below depict modified barium swallow studies of a patient with thin and thick contrast, respectively, who presented with chondroradionecrosis of the larynx. Significant aspiration can be observed on both studies.
The images below are a videostroboscopy and a nasopharyngoscopy of the same patient. The patient is noted to have decreased laryngeal elevation, paralysis of the right true vocal cord, and limited mobility of the left true vocal cord. Pooling of saliva is present in the pyriform sinus, and her false and true vocal cords are edematous with punctate hemorrhages.
Diagnostic Procedures
In postradiation therapy, patients with persistent edema may require multiple direct laryngoscopies with biopsies to differentiate between underlying necrosis and tumor recurrence. Because this may damage the mucosa or perichondrium and damage has even reportedly been significant enough to necessitate a laryngectomy, repeated biopsies may be performed only reluctantly. However, given the low risk of inciting a fulminant perichondritis in contrast to the high incidence of residual or recurrent carcinoma in postirradiated patients with persistent edema, biopsy probably is warranted. In a 1997 report, McGuirt referred to biopsy only patients with grade IV characteristics and patients with grade III illness and positive PET scan findings. [16]
Histologic Findings
The elastic fibers of the tunica media show degeneration, and hyalinization of smooth muscle fibers develops in postirradiated larynges. This is more marked in veins than in arteries. Damage to the endothelium of the capillaries and lymphatics results in obliteration, atrophy, and fibrosis. In the presence of concomitant infection, overlying mucosal ulceration and purulent exudate is noted.
The histologic findings in relapsing polychondritis are loss of basophilic staining of the cartilage matrix, perichondrial round cell formation, and destruction of cartilage with replacement by fibrous tissue.
Necrotizing sialometaplasia has also been identified in association with relapsing polychondritis, as well as squamous cell carcinoma.
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Chondronecrosis of the larynx. Contributing factors leading to chondronecrosis.
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Chondronecrosis of the larynx. Modified barium swallow using thin contrast; the study demonstrates significant aspiration.
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Chondronecrosis of the larynx. Modified barium swallow using thick contrast showing persistent aspiration.
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Chondronecrosis of the larynx. Videostroboscopy showing decreased vocal cord mobility on the left with paralysis on the right.
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Chondronecrosis of the larynx. Nasopharyngoscopy showing decreased vocal cord mobility on the left with paralysis on the right as the patient attempts to perform a high-pitched E.
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Chondronecrosis of the larynx.
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Chondronecrosis of the larynx.
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Chondronecrosis of the larynx.
Tables
Grade |
Symptoms |
Signs |
Treatment |
Grade 1 |
Slight hoarseness, slight mucosal dryness |
Slight edema, telangiectasia |
Symptomatic care: humidity, antireflux therapy, smoking cessation |
Grade 2 |
Moderate hoarseness, moderate mucosal dryness |
Slight impairment of vocal cord mobility, moderate cord edema and erythema |
… |
Grade 3 |
Severe hoarseness with dyspnea, moderate odynophagia and dysphagia |
Severe impairment of vocal cord mobility or fixation of 1 cord, marked edema, skin changes |
Same as Grade 1 and 2 plus steroids/antibiotics, PET scan Laryngectomy if + Bx |
Grade 4 |
Respiratory distress, severe pain, severe odynophagia, weight loss, dehydration |
Fistula, fetor oris, fixation of skin to larynx, laryngeal obstruction and edema of occluding airway, fever |
Functional larynx: PET scan, possible laryngectomy, steroids, abxs Dysfunctional larynx: Laryngectomy |
