Clinical Presentation
A chronic and slowly progressive airway compression is the usual
presentation of RG, and this can last many years in areas where iodine
deficiency is endemic23. A choking sensation
exacerbated by the supine position, globus, wheezing, and
exercise-induced dyspnea (both mistaken for asthma), or dysphagia are
the symptoms most commonly reported by patients, that on the other hand
are asymptomatic at presentation in over one-third of
cases5. Mechanisms of dyspnea are believed to be
direct extrinsic tracheal compression, dysfunction of the recurrent
laryngeal nerve, and lung atelectasis24. Instead, in
less than 5 % of cases the clinical presentation of RG may be acute:
spontaneous hemorrhagic enlargement of RG may occur during
pregnancy25,26 or for unclear
reasons27,28, acute asphyxia and cardiac arrest have
been described29,30, and some authors have
hypothesized a direct phrenic nerve compression by the RG as the main
cause of these dramatic scenarios31.
Rarer presentation may be constituted by thyrotoxicosis or symptoms of
superior vena cava syndrome (facial plethora and congestion, upper neck
cyanosis, or a positive Pemberton’s sign with facial flushing and
engorgement of superficial jugular veins upon raising the
arms)5. While it had been previously reported that
this syndrome was more often associated with malignancy, this remains
unproven21. Isolated dysphagia is also possible in
case of goiter growing only in the retro-esophageal
plane32,33, while the only presenting symptom may also
be represented by oropharyngeal bleeding because of pharyngolaryngeal
“downhill” (i.e ., without venous portal hypertension)
varices34. Furthermore lower and upper extremities
edema35, pericardial effusion, chylothorax, or
hemoptysis because of tracheal varices have been also
described21.