a 60-year - old asian woman , weighing 48 kg with a height of 147 cm , was scheduled for laparoscopic wedge resection of the stomach due to a gastrointestinal stromal tumor ( gist ) .
she had suffered from relapsing polychondritis for fifteen years and had been controlled with steroid therapy .
recently she presented aggravated dyspnea on exertion and had difficulty lying on her back because of orthopnea for a few of months . forced vital capacity ( fvc ) , forced expiratory volume in one second ( fev1 ) and fev1/fvc were 2.23 l ( 94% of predicted ) , 0.69 l ( 40% of predicted ) , and 31% , respectively .
a computed tomography ( ct ) scan of the neck also showed narrowing of the trachea at c5 - 6 level ( approximately 5.75 mm in maximal diameter ) ( fig .
an arterial blood gas test showed a ph 7.41 , paco2 37 mmhg , pao2 69 mmhg , sao2 94% , and hco3 24
owing to potential risks of airway compromise during intubation and/or extubation of an endotracheal tube under general anesthesia , the operation was converted from a laparoscopic surgery to an open laparotomy under epidural analgesia one day before operation . without premedication ,
the patient was reassured with sufficient explanation on the procedures and anesthetic technique . in the operating room , non - invasive blood pressure , electrocardiography , and pulse oxymetry
after placing the patient in the left lateral decubitus position , an 18 gauge tuohy epidural needle was inserted between the seventh and eighth thoracic vertebrae .
an epidural catheter was inserted in the epidural space through a needle and advanced 5 cm toward the cephalic direction . after a test dose injection of 3 ml of 2% lidocaine containing 1 : 200,000 epinephrine ,
after a catheter was fixed , 7 ml of 0.75% ropivacaine and 50 g of fentanyl was injected .
she was comfortable and did not complain of pain in the upper abdomen when the surgeon performed a midline skin incision above the umbilicus .
intraoperative vital signs were relatively stable with a blood pressure of 105 - 120/60 - 75 mmhg and a heart rate of 70 - 80 beats / min . during the operation ,
the stomach was pulled taut to resect the tumor because a mass was located at the posterior wall of the fundus .
being so , we infused remifentanil at 0.03 - 0.06 g / kg / min and spo2 slowly decreased to 95% from 98% . after applying a face mask at 5 l / min ,
after the operation , the patient was transferred to the post - anesthetic care unit and her vital signs including saturation were within acceptable ranges in room air .
the patient was discharged on the sixth day after the operation without any airway complications .
relapsing polychondritis is a disorder that has a variety of clinical manifestations and is considered an autoimmune disease resulting in cartilage breakdown . the disorder is usually presented with episodes of erythema , pain , and swelling of the ears , nose , and joints .
laryngotracheal symptoms are present in approximately 25% of the patients in the initial course of the disease , however , airway symptoms eventually occur in 50% of all patients with relapsing polychondritis .
the first is a stricture due to inflammatory swelling or scar formation in the glottic and subglottic areas .
accordingly , radiographic and bronchoscopic examinations may provide incorrect information for determining the degree of tracheal obstruction , as dynamic airway collapse occurs during respiration . however , inspiratory and expiratory flow - volume loops are useful in predicting the functional degree of intrathoracic and extrathoracic obstruction . in this case , the patient demonstrated severe airway obstruction on flow - volume loop that was well - correlated with her severe respiratory symptoms , including orthopnea .
the most common causes of death among patients with relapsing polychondritis are infection , airway compromise , and cardiac complications .
deaths from infection are frequently associated with laryngotracheal disease and pneumonia as well as corticosteroid therapy .
cardiovascular involvement , such as valvular disorders , aneurysmal dilatation of the great vessels and systemic vasculitis has been reported with relapsing polychondritis .
unfortunately , valve replacement surgery does not offer good prognosis because of the progression of underlying connective tissue degeneration .
physiotherapy has a favorable effect on mucociliary clearance and may be useful for conditioning patients before elective surgical interventions . a tracheostomy or surgical interventions , such as airway stenting ,
airway stenting leads to considerable improvement in airflow , particularly during expiration if the collapsing segment can be broadened by a stent .
the anesthetic management of patients with relapsing polychondritis is challenging due to airway compromise , which is also one of the most causes of death in this disease . during induction of anesthesia ,
furthermore , after extubation , the potential for laryngeal obstruction may increase due to swelling , edema , and tissue injury of tracheal cartilage .
tanaka et al .   reported a case of relapsing polychondritis that underwent laparoscopic cholecystectomy under general anesthesia .
after extubation , the patient showed no respiratory complications and was discharged a few days later .
however , there was a distinct difference in the severity of disease between this case and tanaka 's case , as the patient in tanaka 's case had only mild dyspnea on exertion and no tracheal stenosis on the ct scan .
the patient in this report , in contrast , suffered from orthopnea for a few months and a ct scan revealed narrowing of the subglottic area of which the diameter was approximately 5.75 mm .
, we had to consider the possibility of failure of intubation and/or extubation of the endotracheal tube due to airway compromise . even if intubation was able to be done , the risks of respiratory failure and reintubation after extubation were also high . due to the risk of glottal or subglottal mucosal swelling induced by endotracheal intubation , local and regional anesthesia may be considered safer for patients with relapsing polychondritis than general anesthesia .
therefore , the possible side effects of endotracheal intubation should be weighed against its advantages and the expected procedure . in this case , the operation was converted from a laparoscopic surgery to an open laparotomy and was performed under epidural anesthesia after discussion with surgeon .
these have included a reduction in neurohormonal response to surgical stimuli , preservation of spontaneous breathing and airway reflexes , decreased blood loss and postoperative pain , a reduced risk of thromboembolism , and a more rapid recovery of gastrointestinal motility .
many studies have evaluated the usefulness of continuous epidural analgesia as the predominant anesthetic technique in abdominal surgery [ 10 - 12 ] .
other studies demonstrated that the postoperative deterioration of respiratory function and pulmonary complications decreased with thoracic epidural analgesia compared to general anaesthesia in abdominal or breast surgery . in this case , thoracic epidural blockade provided appropriate analgesia .
the patient felt sensations of nausea , discomfort or pain only when surgeons pulled on the mesentery or palpated the diaphragmatic surfaces of intraabdominal organs .
instead , we infused remifentanil at a low rate instead of a sedative bolus in order to avoid respiratory depression and this was effective in reducing discomfort and pain .
awareness of relapsing polychondritis , as a rare disorder , is significantly critical to conventional general anesthesia because dynamic airway collapse can occur .
we were able to put a patient with severe relapsing polychondritis for wedge resection of stomach under thoracic epidural analgesia and found no specific complications as a result thereof .
thus , we present that thoracic epidural analgesia can be safely utilized as an anesthetic technique for patients undergoing upper abdominal surgery with airway difficulties , reduced pulmonary function , or weak respiratory muscles due to neuromuscular disease .
 relapsing polychondritis is a rare disease characterized by progressive inflammation and destruction of cartilaginous structures such as ears , nose , and tracheolaryngeal structures . as a result , 
 tracheolaryngeal involvement makes anesthetic management a challenge . 
 anesthetic management of a patient with relapsing polychondritis may encounter airway problems caused by severe tracheal stenosis . 
 we present the case of a 60-year - old woman with relapsing polychondritis who underwent wedge resection of the stomach under epidural analgesia . 
 thoracic epidural blockade of the t4 - 10 dermatome was achieved by epidural injection of 7 ml of 0.75% ropivacaine and 50 g of fentanyl . 
 the patient was tolerable during the operation . 
 we suggest that epidural analgesia may be an alternative to general anesthesia for patients with relapsing polychondritis undergoing upper abdominal surgery . 