INTRODUCTION: Tracheal stenosis from complete tracheal rings is a rare congenital anomaly. The C-shaped cartilages are fused posteriorly replacing the membranous portion of the trachea, thus narrowing the tracheal diameter. Tracheal stenosis from congenital complete tracheal rings involving the entire length of the trachea is extremely rare in adults with only few cases described in the literature1.
CASE PRESENTATION: A 36 year old woman was referred to our hospital for surgical evaluation of hypertrophic cardiomyopathy that was refractory to medical management. The patient was taken to the operating room for septal myomectomy. During intubation for administration of general anesthesia, a 7.5 mm endotracheal tube (ETT) could not be placed despite good visualization of the vocal cords. It was felt that the patient had subglottic stenosis. After several attempts and considerable difficulty, a 6.0 mm ETT was placed with the aid of fiberoptic bronchoscopy. Patient underwent septal myectomy, closure of the patent foramen ovale and was discharged six days after surgery. She presented to the emergency room two days later with severe dyspnea, wheezing and chest tightness. Pulmonary medicine was consulted, and a detailed history was elicited. The patient reported dyspnea and palpitations with exertion that dated back to childhood. She could not participate in vigorous play or sports activities. She was diagnosed with asthma for which she was placed on chronic steroids. Over the years, her symptoms progressively worsened and she was unable to climb two flights of stairs. The clinical course was punctuated with episodes of exacerbations when her ambulation was restricted to less than 20 feet. Examination demonstrated inspiratory and expiratory stridor. Lungs were clear to auscultation. CT of the neck and chest showed complete cartilaginous rings in the trachea causing diffuse narrowing of the trachea. Pulmonary function tests demonstrated decreased TLC (74%) and decreased FEV1/FVC (70%) ratio with responsiveness to bronchodilator (+ 21%), suggestive of a mixed pattern with restriction and obstruction. Bronchoscopy did not show any subglottic tracheal stenosis. The trachea below cricoid showed severe diffuse narrowing all the way to carina and into right and left mainstem bronchus due to complete tracheal rings with absence of posterior membrane. She was also noted to have a tracheal take-off from the right upper lobe bronchus. The patient was discharged home several days later and was reassured that she did not have asthma. Oral steroids were tapered over the next several weeks.
DISCUSSION: Patients with long segment tracheal stenosis from congenital complete tracheal rings present with life-threatening respiratory distress in their first year of life. The mortality is very high, as physiologically this condition is usually incompatible with life2. Surgical correction may not be possible in patients with involvement of the entire length of the trachea. Most patients also have congenital cardiac abnormalities including patent ductus arteriosus, ventricular septal defect, atrial septal defect and tetralogy of Fallot. Other associated tracheobronchial anomalies include anomalous right upper lobe take-off as seen in our patient. To the best of our knowledge, our patient is the oldest patient with long segment complete tracheal rings. Her respiratory difficulties were compounded by hypertrophic cardiomyopathy. The combination of hypertrophic cardiomyopathy with tracheal stenosis from complete tracheal rings has not been described so far.
CONCLUSIONS: Patients with complete tracheal rings may present with symptoms akin to asthma. This anomaly should be considered in the differential diagnosis in patients in whom there is difficulty with intubation.
Reference #1 Nagappan R, Parkin G, Wright CA, Walker CS, Vallance N, Buchanan D, Nazaretian S. Adult long-segment tracheal stenosis attributable to complete tracheal rings masquerading as asthma. Crit Care Med. 2002 Jan;30(1):238-40.
Reference #2 Faust RA, Stroh B, Rimell F. The near complete tracheal ring deformity. Int J Pediatr Otorhinolaryngol. 1998 Oct 2;45(2):171-6.
DISCLOSURE: The following authors have nothing to disclose: Satish Chandrashekaran, Seher Iqbal, Andrew Limper
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