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Primary Tracheomalacia T h o m a s H . Cogbill, M . D . , Frederick A . M o o r e , M . D . , Frank J . A c c u r s o , M . D . , a n d J o h n R. Lilly, M . D . ABSTRACT Tracheomalacia is a rare congenital malformation of the tracheobronchial cartilages in cheomalacia have been seen at our institution which the supporting cartilaginous rings permit ex- over the past four years. The clinical symptoms piratory collapse of the airway. The condition is usu- of the 4 patients were remarkably similar and ally mild and self-limited. There is a severe variant, highly distinctive. Moreover, therapeutic inter- however, that is life-threatening and warrants sepa- vention was eventually identical in each in- rate categorization. Four children with severe pri- stance. The purpose of this article is to review mary tracheomalacia were treated recently. The clin- briefly the clinical features in the 4 patients, to ical symptoms, diagnostic findings, and eventual describe the diagnostic process, and to discuss treatment of these patients were highly distinctive the therapeutic management of infants with se-and almost identical in all 4, permitting us to make the following observations: (1) primary severe tracheomalacia must be suspected in infants with unexplained respiratory distress manifested by Patient 1 stridor and cyanosis; (2) symptoms are not present at A 10-week-old female infant was hospitalized birth but appear insidiously after the first weeks of for progressive stridor and respiratory distress life, are markedly aggravated by respiratory tract in- over a 3-week period. On admission she had fections, and are made worse by agitation; (3) bron- expiratory stridor, wheezing, and intercostal re- choscopy is essential for definitive diagnosis and traction at rest. Symptoms became worse with should be employed early in the diagnostic process; agitation. Chest roentgenogram and barium (4) tracheostomy is probably essential in most in- swallow were normal. At bronchoscope exami- stances; and (5) resolution, although spontaneous, nation, expiratory collapse of the mainstem does not occur until after 2 years of age. bronchi and distal trachea was noted, as well as indistinct tracheal rings. A small, clinically un- Primary tracheomalacia is a rare disease of in- important subglottic mass was also visualized. fancy that is believed to be due to immaturity of Postoperatively, stridor gradually became more the tracheobronchial cartilage [1]. Tracheal nar- severe and was not responsive to racemic epi- rowing occurs with expiration and causes nephrine, isoetharine inhalations, and par-stridor, cyanosis, and respiratory distress. An- enteral prednisolone. At repeat bronchoscopic teroposterior tracheal collapse and indistinct examination five days later, the narrowing of tracheobronchial rings are visualized broncho- the distal trachea and mainstem bronchi had be- scopically. The disease is self-limited, generally come worse. There was now severe edema of resolving within the first 2 years of life [1]. the tracheobronchial mucosa and moderate There are different degrees of clinical severity, bronchial secretions, which grew parainfluenza however, and a high mortality has been associ- ated with the more severe variants [2, 3]. Despite the tracheostomy, the patient's respi- rator}' status deteriorated. Positive-pressure ventilation with pressures of 60 cm H 2 0 was necessary to provide an arterial oxygen tension From the Departments of Surgery and Pediatrics, University of 45 mm Hg on 100% forced inspiratory oxy- of Colorado Health Sciences Center, Denver, CO. gen. Cardiopulmonary arrest ensued, which was reversed only by emergency rigid bron- Address reprint requests to Dr. Cogbill, Department of Surgerv, Gundersen Clinic, 1836 South Ave, La Crosse, choscopy through the tracheostomy wound. The bronchoscope mechanically opened the al- 539 Cogbill et al: Primary Tracheomalacia most totally collapsed mainstem bronchi, thus bronchoscopic examination that showed the permitting aeration of the distal tracheobron­ tracheomalacia to be moderately improved. The Over the next 11 days, the patient's symp­ toms improved. Bedside bronchoscopy with Patient 3 tracheobronchial aspiration of thick sputum was A 21-month-old boy was hospitalized for recur­required on numerous occasions. Over the fol­ rent upper respiratory tract infections, cyanotic lowing weeks, the airway lumen gradually di­ episodes, apnea, and failure to thrive. At 6 lated because of resolution of mucosal edema weeks of age he was hospitalized for persistent and inflammation. The patient was removed cough, expiratory stridor, and cardiomegaly. A from positive-pressure ventilation and subse­ diagnosis of "laryngomalacia" was made. Sev­ quently discharged. Ten months later, bron- eral subsequent hospitalizations for respiratory choscopic examination demonstrated some distress and viral pneumonia occurred over the improvement of tracheomalacia and nearly next 18 months. complete resolution of the subglottic tumor. At On admission the patient exhibited moderate 2 years of age, the patient had a bronchoscopic respiratory distress with a respiratory rate of 40 examination, the findings of which were almost breaths per minute, and there was perioral normal. The tracheostomy tube was success­ cyanosis. Cardiomegaly and enlargement of the fully removed, and the patient has been well for pulmonary artery were noted on the chest the past 5 months. roentgenogram. Progressive carbon dioxide re­tention finally prompted nasotracheal intuba­ tion. At subsequent bronchoscopic examina­ A 3-month-old male infant was hospitalized for tion, severe tracheomalacia combined with failure to thrive and frequent cyanotic episodes. minimal subglottic stenosis was seen, and a tra­Symptoms began at 16 days of age with crying cheostomy was done. Postoperatively the pa­spells followed by choking and expiratory tient improved moderately, and congestive stridor. On examination the patient had expira­ heart failure was treated successfully by diuret­ tory stridor and grunting with severe chest re­ traction. The respiratory rate was 52 breaths per minute. Chest roentgenogram and barium swal­ months over the next 18 months. Tracheal mat­ low were normal. At bronchoscopic examina­ uration was first noted when the patient was 33 tion there was nearly total collapse of the distal months old. Subsequently the tracheostomy trachea, and the tracheal rings were indistinct. tube was removed successfully. At ЗУг years of A tracheostomy was done. Postoperatively the age, the patient has minimal symptoms. His patient improved moderately but continued to heart is entirely normal. have respiratory distress with wheezing and copious tracheal secretions that only gradually Patient 4 improved. A 36-week-old premature female infant was At 10 months of age, the patient had a bron­ seen with progressive cyanosis, apnea, and ex­ choscopic examination that showed almost piratory stridor. Nasotracheal intubation was identical findings. For reasons that are unclear, eventually necessary. At bronchoscopic exami­the tracheostomy tube was removed subse­ nation, findings were severe tracheomalacia quently at another institution. The patient did with marked tracheal collapse and mainstem well for 2 months and then had a sudden respi­ bronchial narrowing. The patient could not be ratory arrest. Emergency tracheostomy through removed from controlled ventilation and devel­ the old tracheostomy wound saved his life. oped congestive heart failure resistant to di- At 23 months of age, the patient developed a goxin and furosemide. A tracheostomy was viral pneumonia that required mechanical venti­ done, and there was gradual improvement. She lation, theophylline, isoetharine, and postural was discharged on mask oxygen. She devel­drainage. At 26 months of age, he had another oped a mild upper respiratory tract infection 4 Clinical Data on 4 Patients with Severe Primary Tracheomalacia3 months later and died at home of respiratory there is a definite worsening of symptoms with crying and agitation, an observation that has The courses of all 4 patients are summarized not been reported previously. Each of our pa- tients required sedation; in fact, Patient 1 re-quired temporary paralysis to control agitation As noted earlier, tracheomalacia is characterized Symptomatology is also markedly aggravated by abnormal tracheal collapse secondary to in- by respiratory tract infection. Three of our pa- adequate supporting cartilaginous and myo- tients had serious clinical deterioration during elastic elements [1]. The condition has been di- otherwise mild flu-like illnesses, and 1 died con- vided into primary (congenital) and secondary sequently. Presumably, the tracheobronchial lu- (acquired) subgroups. Primary tracheomalacia men, already narrowed by tracheomalacia, be-is probably due to immaturity of the tracheo- comes further compromised by inflammation. bronchial cartilage [1]. Secondary tracheomala- In addition, tracheobronchial secretions under cia is due to degeneration of previously normal these circumstances may be cleared less effec- tracheal cartilages resulting from inflammatory processes [4-7], extrinsic vascular compression Baxter and Dunbar [1] employed fluoroscopy [8, 9], bronchial neoplasms [5, 9], and tracheo- and lateral inspiratory and expiratory chest roentgenograms to establish the diagnosis of There appear to be two distinct types of pri- tracheomalacia. In our patients static inspiratory mary tracheomalacia: the common or mild vari- and expiratory chest roentgenograms were nor- ant and an unusual, severe form. In 1963 Baxter mal, and the critical clinical status of these in- and Dunbar [1] reported 21 patients with pri- fants precluded the use of fluoroscopy. We be- mary tracheomalacia who displayed both types lieve that bronchoscopy is absolutely necessary of clinical symptomatology. The mortality de- for definitive diagnosis. Also, because of the pended upon the severity of the disease; there safety of present-day equipment, this technique were no deaths in patients with the mild form. should be utilized early in the diagnostic pro- Mortality in severe tracheomalacia has been re- There is little information about the treatment Severe primary tracheomalacia has an insidi- of severe forms of tracheomalacia. Most of the ous onset in an otherwise healthy infant, with patients reported by Cox and Shaw [11], Levin the appearance of progressively noisy respira- and colleagues [12], and Baxter and Dunbar [1] tions, episodic cyanosis, increased respiratory required no formal therapy, since they had the rate, intercostal retractions, and, finally, obvi- mild form of the disease. In treatment of severe ous stridor. The stridor is most noticeable dur- forms, Picot and associates [3] recommended ing expiration when maximal tracheobronchial hospitalization, oxygen, and, occasionally, tra-collapse normally occurs. In our experience cheostomy. Treatment with oxygen, postural 541 Cogbill et al: Primary Tracheomalacia drainage, percussion, and frequent endotra- cheal suctioning was unsuccessful in our pa- 1. Baxter JD, Dunbar JS: Tracheomalacia. Ann Otol tients, and tracheostomy was ultimately neces- 2. Agosti E, DeFilippi G, Fior R, et al: Generalized familial bronchomalacia. Acta Paediatr Scand The efficacy of tracheostomy appears to be a consequence of the tube's stenting effect on the 3. Picot C, Monnet P, Bethenod M, et al: La semiflaccid trachea. More effective tracheo- tracheomalacie du nourrisson. Arch Fr Ped bronchial aspiration is also provided, but this is probably less important. That tracheostomy is 4. Feist JH, Johnson TH, Wilson RJ: Acquired not curative, or may not even prevent subse- tracheomalacia: etiology and differential diag- quent respiratory embarrassment in the face of 5. Johnson TH, Mikita JJ, Wilson RJ, et al: Acquired intercurrent respiratory illness, was well illus- trated in our small series. The probable mechan- 6. Jokinen K, Palva T, Sutinen S, et al: Acquired ism for exacerbation of symptoms in this situa- tracheobronchomalacia. Ann Clin Res 9:52, 1977 tion is inflammatory narrowing of the mainstem 7. Nuutinen J: Acquired tracheobronchomalacia. bronchi. The bronchi are involved to some de- 8. Koopot R, Nikaidoh H, Idriss FS: Surgical man- gree in all severe cases of tracheomalacia, and agement of anomalous left pulmonary artery can be supported only partially by the stenting causing tracheobronchial obstruction. J Thorac In mild forms of tracheomalacia, symptoms 9. Vasko JS, Ahn C: Surgical management of sec- ondary tracheomalacia. Ann Thorac Surg 6:269, disappear in most patients by the age of 18 to 24 months [1, 3]. Duration of symptoms in the se- 10 Benjamin B, Cohen D, Glasson M: Tracheo- vere variants has not been reported. Resolution malacia in association with congenital tracheo- occurred in 2 of our patients between 2 and 3 esophageal fistula. Surgery 79:504, 1976 years of age, after which time the tracheostomy 11 Cox WL, Shaw RR: Congenital chondromalacia of tube was successfully removed. Another pa- the trachea. J Thorac Cardiovasc Surg 49:1033, 1965 tient, although improved, still demonstrates 12. Levin SJ, Adler P, Scherer RA: Collapsible bronchoscopic and clinical findings of tra- trachea (tracheomalacia). Ann Allergv 22:20,

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