Samter’s syndrome is a clinical designation of the triad of bronchial asthma, nasal polyposis and allergic intolerance to salicylate derivatives. Persistent inflammatory rhino-sinusitis is regular feature of this syndrome and leads to mucosal engorgement and nasal obstruction. Polyps are frequently restricted to the sinus spaces. The occurrence of large polyps leading to mechanical displacement of the orbit has not been reported previously.
A 51-year old male with a life long history of nasal polyps, asthma and salicylate intolerance presented to the emergency room with a several day history of diplopia, rhino-sinusitis and frontal headache. There was no fever, chills, or nocturnal diaphoresis. Until the current presentation, he denied any headaches, visual disturbance or neurological symptoms. Past medical history included hepatitis C infection and chronic allergic rhinitis and salicylate induced urticaria. There was no family history of asthma. He was taking albuterol for asthma on as required basis. Physical Examination revealed an adult male in moderate distress with increased nasal lacrimation and nasal phonation. Blood pressure was 134/75, pulse; 89/min, respiration; 21/minute. Room air saturation was normal. He was afebrile. Head and neck revealed no jugular venous distension, thyromegaly or adenopathy. The nose revealed generalized mucosal inflammation and hypertrophic turbinates and multiple polyps occluding the both nasal cavities. The left eye demonstrated proptosis and inflammation with increased lacrimation. Chest revealed bilateral polyphonic wheezes. Cardiac showed regular rate and rhythm, normal heart sounds no murmurs and no gallops. Extremities; no tremor, dry palms, no finger clubbing, cyanosis or edema. Cranial examination was remarkable for diplopia in all planes of vision. The rest of the neurological examination was unremarkable. A computer tomogram (CT) scan of sinuses performed in the emergency room showed bilateral nasal polyps with a large left sided maxillary polyp measuring 1cm by 1.5 cm in a cranial caudal dimension and encroaching the left orbit supero-laterally causing orbital and superior rectus muscle displacement. Laboratory data; showed WBC; 96000/μl, Hgb; 15.9g/dl, platelet; 198,000/μl. He was evaluated by otolaryngologist in the emergency room and given hydrocortisone 100 mg intravenously and initiated on prednisone 40 mg qd. He underwent nasal polypectomy and orbital decompression with a resultant restolation of normal vision.
Samter’s syndrome comprises of the triad of asthma, nasal polyps and salicylate intolerance. The pathogenesis of Samter’s syndrome is thought to be caused by abnormalities of the cyclo-oxygenase enzyme system. It is considered that genetically mediated perturbations in cyclo-oxygenase bioactivity channel the metabolites of eicosapentanoic acids into the leukotriene pathway. These metabolic derangements culminate in the amplification of the biological effects of leukotrienes. The pathological effects of excess leukotrienes include bronchospasm, inflammation and the development of polyps. A preponderance of cell adhesion molecules on polyp surfaces has also been noted and suggests that pro-inflammatory cytokines play a role in the pathogenesis of Samter’s triad. The management of giant polyps with symptomatic mechanical effects includes evaluation for sinus fungal infection and the use of high dose corticosteroids as a bridge to polypectomy and sinus debridement. In the long term, leukotriene modifiers should be included in the treatment regimen for Samter’s syndrome. Despite aggressive treatment,polyps have a recurrent course.
This case suggests that in patients with a known history of polyps who present with ocular complaints, diagnostic consideration should be given to the possibility of mechanical effects of large polyps. High dose corticosteroids should be used while awaiting the surgery which is the definitive treatment.
Daniel Dube, None.