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Congenital stenosis of the trachea
Congenital
Stenosis
of the Trachea*
JAMES R. CANTRELL, M.D. AND HARRIET G. GUILD, M.D., Seattle,
From tbe Department of Surgery, University of Wasbingion School of Medicine. Seattle. Wasbirxton. and Deaartment of Pediatrics, tbe’Jobns Hopkins “Uni;ersity School of Medicine, Baltimore, Maryland.
Washington
various aIIergens. Desensitization was begun and she was discharged to the care of her private physician. The chiId did poorIy, experiencing recurrent episodes of puImonary infection and demonstrating persistent evidence of aIIergic bronchitis. She was readmitted to the hospita1 in June 1958 because of pneumonia which responded satisfactorily to treatment with antibiotics. Angiocardiography performed after recovery reveaIed a retro-esophagea1 subcIavian artery and absence of the right innominate artery. Pectus excavatum was aIs noted at that time. AIthough the chiId was stiI1 somewhat younger than was considered ideaI, it was beIieved, in view of the repeated upper respiratory tract infections and the persistent diffrcuIty in breathing, that the trachea1 stenosis shouId be immediateIy corrected. She was discharged for a brief interva1 and was readmitted for surgica1 correction of the trachea1 anomaIy. PhysicaI examination at the time of that admission reveaIed the patient to be a somewhat thin but we11 deveIoped seven year oId chiId who was in constant miId respiratory distress. MiId wheezing was noted at rest, and on exercise she developed a noticeable stridor which was both inspiratory and expiratory. A moderateIy severe pectus excavatum was present. Chest fiIm reveaIed the Iung fieIds to be essentiaIIy cIear. The heart was not enIarged. There were no other abnormaIities. Laboratory examination revealed a hemogIobin of I I .5 gm. per cent. The remaining Iaboratory studies were normaI. On July II, 1958, the patient’s right chest was expIored through a posteroIatera1 approach. A generaIized fibrous reaction was noted about the trachea. The right upper Iobe bronchus originated from the mid-portion of the thoracic trachea and appeared to be entirely norma as did the trachea above that point. The trachea beIow this bronchus was symmetricaIIy narrowed for a distance of 3.2 cm., measuring 6 mm. in externa1 diameter, approximately one-third the diameter of the nor-
HE OCCURRENCE Of COngenitaI StenOSiS Of the trachea has been documented in the Iiterature for aImost a century, yet genera1 recognition of this defect is so poor that the majority of anatomy, embryoIogy and surgica1 texts fai1 even to mention its existence. Despite the rarity of this maIformation, such Iack of recognition is regrettabIe since certain types of the anomaIy are amenabIe to compIete correction. This capabiIity assumes even greater significance when it is reaIized that this Iesion is aImost uniformly IethaI if untreated. The purpose of this report is to emphasize the diagnostic features of the anomaly, to propose a method for surgica1 correction, and to present an exampIe of a patient successfuIIy treated.
T
CASE REPORT A seven year oId white girI was admitted to the hospital in Jury, 1958 for correction of congenita1 stenosis of the thoracic trachea. There was no history of significant disease in the famiIy and there were no other instances of congenita1 anomalies. The past history was essentiaIIy negative except as related to the present iIIness. The patient had been observed to have stertorous, wheezing respiration since birth. She had been admitted to the hospita1 for the first time at one year of age because of frequent upper respiratory infections. Thorough study at that time, incIuding bronchoscopy and bronchography, reveaIed marked stenosis of the distal 3 cm. of the trachea and anomaIous origin of the right upper Iobe bronchus from the trachea just above the stenotic segment. (Fig. I.) The child was noted to have asthmatic bronchitis and study was undertaken to determine the sensitivity of the chiId to
* Presented at the annua1 meeting of the Pacific Coast SurgicaI Association, February g-12, 1964.
297
American
Journal
San Francisco, OJ Surgery,
Volume
CaIifornia, 108,
August
1964
CantreII
and GuiId
A
B
FIG. I. A, posteroanterior
view of bronchogram demonstrating the marked stenosis of the distal trachea just above the bifurcation and the tracheal origin of the right upper lobe bronchus. The upper trachea and bronchi are of normal caliber. B, oblique view of bronchogram demonstrating point of maximum narrowing at upper end of stenotic segment.
FIG. 2. Appearance of tracheobronchia1 about stenotic area obscures abnormalIy
tree at operation. Fibrous shaped, fused cartiIages.
reaction
stei notic recognizabIe cartilaginous rings. The [ding area was markedly adherent to the surl mediastina1 structures. The right lung it self and the right pulmonary artery and veins L‘PPCtared normaI. The norma and stenotic segments of the trac :hea,
ma1 trach lea. (Fig. 2.) The stenosis continued to the point of bifurcation of this structure into a Ieft main and right intermediate bronchus, each of Jwhich appeared to be of norma caliber and stru cture. The area of the stenosis was grossly orma in structure. There were no externalIy 298
Congenital
Stenosis
of Trachea
FIG. 3. Operative technic, demonstrating obliqur lines of excision at each end of stcnotic area, technic of ventilation and rrrethc,d of snastomosis utilizing interrupted sutures of braided stainless steel.
the Ieft main bronchus and the right stem bronchus were isoIated. Despite the fibrosis of the upper tracheal segment, some ffexibihty was gained by this mobiIization and it was beIieved that it would be possibIe to resect the stenotic area and carry out an anastomosis between the divided ends. Additiona mobility was gained by division of the inferior puImonary ligament and mobilization of the other hiIar structures. It appeared preoperativeIy that it wouId be technicaIIy advantageous to perform the anastomosis over an endotrachea1 tube. Since the Iumen of the stenotic area had measured onIy 4 mm. at bronchoscopy, it was cIear that a tube would not pass through the stenotic area. A Iong tube of small caliber was therefore passed into the upper trachea, the trachea sectioned obIiqueIy at the upper end of the stricture and an obIique incision made in the anterior portion of the trachea at the lower end of the stricture. It was immediateIy apparent that the endotracheal tube was too Iarge to enter the left main bronchus. A smaIIer tube was therefore pIaced in the left main bronchus through the wound and fixed by means of a gently tied umbiIica1 tape. This tube was connected to the anesthesia machine across the wound. This provided effective ventiIation but was not optimal from the point of view of technica ease in performing the anastomosis. The upper trachea1 segment was anastomosed to the bifurcation, using interrupted sutures
tension exerted at the base of the carina, the two tracheal segments were brought into apposition and the sutures were tied. The anterior sutures
FIG. 4. Postoperative roentgenogram of chest just prior to discharge from hospital. Note the abnormally high position of trachea1 bifurcation at lower border of the third thoracic vertebra. Preoperative films reveaIed bifurcation level to be at midpoint of the fifth thoracic vertebra.
of No. 4-o braided stainless steel wire. (Fig. 3.) AI1 posterior sutures were pIaced, and with down-
ward traction upon the upper segment and upward
299
CantreII and GuiId deveIoped transient atelectasis in the Ieft Iower Iobe which responded to steam, expectorants and vigorous coughing. The patient’s trachea was not suctioned directly. By the eighth postoperative day the chiId was afebriIe and breathing cIearIy and without difficulty. At the time of discharge the lungs were entireIy clear (Fig. 4) and respiration was compIeteIy free. Gross examination of the resected tissue reveaIed a stenotic segment of tracheobronchia1 tree, the Iumen of which measured 3 mm. at its narrowest point. The cartiIaginous rings were abnorma1 in shape and were fused. HistoIogic sections showed miId chronic inflammation in the submucosa and an increased amount of dense coIIagen in the waI1 of the trachea. (Fig. 5.) The chiId has been seen on severa occasions since discharge. Her Iast return visit was in March, twenty-one months after I 960, approximateIy operation. Bronchoscopy performed at that time reveaIed an uncompromised Iumen equa1 in size to the lumen of the trachea above. There was no stenosis at the suture line. She has been foIIowed since that time through her private physician. The most recent contact was in January, 1964. The child, now thirteen years of age, has deveIoped normaIIy, has no respiratory symptoms whatever, carries on unIimited physica activity, has had no recent puImonary infections and has no further asthmatic symptoms. Her onIy difficulty is the progressiveIy deepening pectus excavatum.
FIG. 5.
Photomicrograph of junction of normaI trachea (right) and stenotic area (left) revealing abnormal chronically inflamed and thickened epicartilage, theIium and submucosa and increased colIagen (extreme Ieft) in the stenotic area; magnification X 35. pIaced about the endobronchia1 tube were then and al1 but three sutures tied. The tube was then removed, the remaining sutures tied and ventiIation re-established through the endotrachea1 tube. AI1 Iobes of the right Iung immediateIy ventilated freely. The anastomosis seemed to be of good size. There was no air Ieak from the anastomosis and the mediastina1 pleura was cIosed IooseIy. Chest drainage was instituted and the wound cIosed in Iayers with interrupted silk sutures. ProphyIactic tracheostomy was considered but it was beIieved that in these circumstances the hazards of the tracheostomy might be greater than its virtues. It was therefore eIected to defer this procedure unless it became cIearIy indicated. The patient received 250 cc. of whoIe bIood during the procedure. The child’s postoperative course was satisfactory. She had some diffIcuIty in coughing and
COMMENTS
CongenitaI stenosis of the trachea is an unIesion. We have been able to find onIy twenty-four acceptabIy documented cases common
FIG. 6$‘Diagram of the three morphologic variants of congenital stenosis of the trachea. Left, generaIized hypopIasia. Center, funneI-Iike stenosis. Right, segmenta stenosis. The occurrence of anomaIous bronchi, as in the case presented, is apparently most common in the segmenta form.
300
CongenitaI
Stenosis
of Trachea the anomaIies are uncIear, as is the mechanism of development. The defect must develop at a relatively early period in embryonic life. Although the available reports are inadequate to permit a definite concIusion, no evidence of a fan&a1 tendency has been noted. Associated Anomalies. AbnormaI origin of the major bronchi is occasionaIIy seen. In addition to the presentIy reported case, one example of trachea1 origin of the right upper Iobe bronchus has been observed [4]. In one of the cases reported by HoIIinger [12], the entire right bronchia tree arose from the Ieft main bronchus. Each of these abnormalities was noted in association with segmenta stenosis. AnomaIies of the Iung have been noted in three instances. In the case reported by Gruber [8], the right Iung was apIastic and the Ieft Iung was without fissures. Agenesis of the right Iung was noted in the case reported by Putney and BaItzeII [17], and abnormat IobuIation of the lungs was reported by Weber [21]. A number of co-existing anomaIies of other systems have been reported. These incIude cIubfoot [9], congenita1 absence of the thumbs [IO], maIformation of the brain [6], MeckeI’s diverticuIum [IO], intestina1 stenosis [14], imperforate anus [2], abnorma1 IobuIation of the Iiver [IO], ventricuIar septal defect [r5], persistent truncus arteriosus [12], abnormal position of the left puImonary artery [ 191 and atria1 septa1 defect [13]. Pectus excavatum, a retroesophagea1 subcIavian artery and absence of the innominate artery were observed in the present case. It is important to note that with two exceptions, none of these co-existing anomalies were incapabIe of correction or inconsistent with Iife. Clinical Features. The most significant cIinica1 symptom associated with trachea1 stenosis is stridor which is both inspiratory and expiratory and often becomes apparent shortly after birth. Wheezing is aIso commonIy described. Depending upon the degree of obstruction, inspiratory retraction of the sternum and empIoyment of secondary muscIes of respiration may be noted. Shortness of breath is routine but may be paroxysma in nature, occasionaIIy having been reported to be primariIy nocturna1. Cyanosis may be present and may occur at rest or onIy on exercise. Feeding diffrcuIty is common, often resuIting in faiIure to gain weight and deveIop normaIIy. Respira-
in the literature [r,2,4-221 aIthough a few additional cases may exist in the older foreign literature which has been unavailable to us. Three morphoIogic variants may be seen. (Fig. 6.) J. Generalized Hypoplasia. AIthough this malformation should perhaps be considered as a separate entity, it has been considered in this category since the functiona impairment is simiIar in every way to the other forms of stenosis. In this anomaIy, the entire trachea is narrow in caIiber, posterior fusion of the individual cartiIaginous rings usuaIIy being noted. The diameter of the Iumen in the reported cases has varied from I to 3 mm. in newborn infants. HypopIasia appears to be uniformIy Iimited to the trachea itself, the major bronchi and dista1 tracheobronchia1 tree being of normaI size. Seven examples of this anomaIy have been reported [1,4-6,14,17,183. 2. Funnel-like Stenosis. In this form of stenosis the diameter of the trachea immediateIy beIow the cricoid is of norma size but the trachea becomes progressiveIy narrower to the IeveI of the bifurcation. Again, the membranous trachea may be partiaIIy absent with fusion of the trachea1 cartiIages in a portion of the stenotic area. The point of maximum constriction is found just above the bifurcation and the Iumen at that point may be as narrow as I mm. in diameter. Four documented cases of this type of stenosis have been reported [8,x1,13,22]. 3. Segmental Stenosis. SegmentaI stenosis, in which a portion of the trachea is narrowed in hourgIass fashion, wouId appear to be the most common form of the anomaIy, more than 50 per cent of the reported cases being of this type [4,7,9,10,12,15,16,19,20]. A significant proportion of these stenoses are associated with a fibrotic reaction in the region of narrowing. The cartiIages are usuaIIy abnorma1 in shape and form compIete rings. The Iength of the stenotic area varies from I to 5 cm., the majority of the reported cases being approximateIy 2 cm. in Iength. The stenotic area may be found in any portion of the trachea. Of the eleven cases in which the point of narrowing was adequateIy described, four occurred in the proxima1 trachea just beIow the cricoid, 3 occurred in the centra1 portion of the trachea and four were noted just above the bifurcation. The etioIogic factors in the production of 301
CantreII and GuiId tory infections are frequent and are usuaIIy the uItimate cause of death. Definitive Diagnostic Measures. The diagnosis of trachea1 stenosis may occasionaIIy be confirmed by examination of the IateraI roentgenogram of the chest in which the size and configuration of the infant trachea are usuaIIy we11 defined. Evans has pointed out, however, that diffIcuIty may be encountered in the interpretation of such fiIms unIess the examination is made during inspiration. Bronchoscopic examination or tracheograms wiI1 provide definitive confirmation but caution must be utiIized in advocating either of these procedures. The trauma associated with injudiciousIy performed bronchoscopy may precipitate acute fata trachea1 obstruction. If bronchography is performed, water-soIubIe media shouId be utiIized to avoid the mucosa1 reaction which occasionaIIy foIIows the instaIIation of oiIy contrast media. Because of the dista1 Iocation of the stricture, tracheostomy may be of no vaIue in reIieving airway obstruction which may foIIow these procedures. Diferential Diagnosis. Other Iesions which must be considered in the differentia1 diagnosis incIude a variety of causes of externa1 compression of the trachea (vascuIar rings, enIargement of the thymus and mediastina1 masses) as we11 as foreign body. Inspiratory stridor may aIso be seen in instances of tracheomaIacia. Prognosis and Therapy. If untreated, these anomalies are aImost invariabIy fata in earIy infancy or chirdhood. The eventua1 demise is usuaIIy the resuIt of repeated respiratory tract infections. In view of the high earIy mortaIity associated with this anomaIy, it may be concIuded that correction must usuaIIy be accompIished earIy in Iife. HoIIinger’s report [12] of apparentIy satisfactory resuIts foIIowing endoscopic dilatation in two instances is surprising in view of the abnorma1 structure of the stenosed segment which is routineIy observed. Furthermore, it wouId appear that diIatation may itseIf be extremeIy hazardous and that this approach shouId be recommended onIy with great caution. In the segmenta form of stenosis, optima1 results shouId be obtained by excision of the narrowed area with primary anastomosis of the divided ends of the trachea. The majority of the reported stenoses have been reIativeIy short and virtuaIly a11 shouId have been 302
amenabIe to this form of therapy. The feasibiIity and successfu1 resuIts of trachea1 anastomosis have been ampIy demonstrated. The experImenta evaIuation of this technic reported by CantreII and FoIse [3] wouId suggest that when properIy utiIized this method is remarkabIy safe and effective. The question of further growth of the anastomotic site cannot be answered at this time but it wouId appear that if a satisfactory anastomosis with primary heaIing is obtained, subsequent growth of the trachea at this point shouId not be hindered. In the funnel-type stenoses it wouId seem reasonabIe to resect the most narrowed dista1 portion of the trachea and to anastomose the proxima1 trachea to the bifurcation. Since a Iumen of no more than haIf of the norma diameter of the trachea is sufficient to provide adequate air flow, repair of this type shouId produce a good functiona resuIt. No attempts to carry out such a procedure have been reported in the Iiterature and we have had no occasion to dea1 with a chiId with this type of anomaIy. At the present time no satisfactory method of therapy appears avaiIabIe for the repair of the totaIIy hypopIastic trachea. SUMMARY I. Congenital stenosis of the trachea is a reIativeIy uncommon anomaIy yet one which shouId be recognized since in many instances the Iesion is susceptibIe to correction. 2. The presence of stridor, dyspnea and cyanosis in a newborn infant or smaI1 chiId shouId suggest the possibiIity of trachea1 stenosis. 3. Definitive diagnosis can be estabIished by IateraI roentgenograms of the chest, bronchoscopy and/or bronchography. 4. GmpIete correction of this anomaIy shouId be possibIe in many instances by excision of the stenotic area and reanastomosis of the trachea. 5. A case of segmenta stenosis of the trachea corrected in this manner is described. To our knowledge, this case is the first in which successfuI operative correction of such a defect has been accompIished. REFERENCES I. BEICKERT, P. Tracheal
Stenose durch KnorpelmissbiIdung. HNO. 7: 325, 1959. 2. BONNIER, M. Un cas de stknose cong&itaIe de la
CongenitaI
3.
4.
5.
6.
7.
8. 0. IO. I I.
Stenosis
trach& avec bronche du Iobe sup&ur droite atr(.sier et lobe supkrieur droite aplasique. Imperforation de I’anus. Ann. mtd.-cbir. Hbp. Sainte-Justine Mont&al, 5 : 13, 1947. CANTRELL, J. R. and FOLSE, J. R. The repair of circumferential defects of the trachea by direct anastomosis: experimenta evaIuation. J. Tboracic @ Cardiovas. Surg., 42: 589, 1961. EVARS, IV. A., JR. CongenitaI obstructions of the respiratory tract. I. Trachea1 malformations. Am. J. Roentgenol., 62: 167, rg4g. FINKELSTEIN, H. Lehrbuch der %ulingskrankheiten, 3rd ed., p. 621. Berlin, 1924. Julius Springer. GABRIEL, E. and FEYRTER, F. Angeborene tracheal Stenose bei einem 434 jahrigen Kinde. Jabrb. f. Kinderb., 121: zg, 1928. GREGOR, K. Ein FaII von angeborener Missbildung der Luftrijhre (circumscripte Derengerung mit FehIen der Pars membranacea). Jabrb. f. Kinderb., 49: 123, 1899. GRUBER, W. Oesterr. Ztscbr. f. prakt. Heilk., 16: 7, 1870. Cited by Haardt [zo]. GKJISEZ,J. Malformation congenitale de la trachbe. Bull. d’oto.-rhino.-laryngol.,~25: 289, 1927. HAARDT, W. Ueber angeborene Enge der Luftrchre. Wien. klin. Wcbnscbr.. $ I : 6~7. 10x8. HEIKEL, P. E. Congenital tracheal stenosis combined with truncus arteriosus cordis; a case diagnosed in vitam. Ann. paediat. Fenn., 3: 22,
1957. 12. HOLLINGER, P. H., JOHNSTON,K. C. and BASINGER, C. E. Benign stenosis of the trachea. Ann. Otol. Rbin. u Laryng., 59: 837, 1950. I 3. HOUSTON, I. B. and MACKIE, D. G. Congenital trachea1 stenosis. Thorax, 16: 94, 1961. 14. LERRO, E. Un case di stridore congenito da stenosi tracheale. Pediatria, 43: 440, 1935. 15. MONTANDON, A. Stbnose cong&nitaIe de Ia trachee et maIformations associbes. Pratt. oto-rbinolaryng., 6: 179, 1944. 16. Moussous, M. Sur un cas de &r&zissement cong&nitaIe de Ia trachde. J. mkd. Bordeaux, 37: 195, 1907. 17. PUTNEY, F. J. and BALTZELL, W. H. Agenesis of the Iung with trachea1 stenosis. Ann. Otol. Rbin. Laryng.; 61: 677, 1952. 18. SANKOTT, A. M. Ueber einen FaII mit angeborener Enge der Trachea und der Bronchien, FehIen der Paries membranaceus tracheae, DivertickeIbildung usw. Wien. klin. Wcbnscbr., 35: 391,1922. 19. SCHEID, P. Missbildung des TrachealskeIettes und der Iinken Arteria puImonaIis mit Erstickungstod bei 7 monate aItem Kind. Frankfurt. Ztscbr. Path;, 52: 114, 1938. 20. STEWART. S. and PINKERTON. H. H. UnusuaI stenos& of trachea in an aduIt. Brit. J. Anaestb.,
27: 492. ‘955.
21. WEBER, H. W. Ober kongenitaIe TracheaIstenosen.
Zentralbl. allg. Patb., 89: 148, 1952. stenosis of the trachea. Am. J.. Dis. Cbildy, 61: 1263, 1941.
22. WOLMAN, I. J. Congenital
DISCUSSION ROLAND D.
viewing
the
PINKHAM (SeattIe, Wash.): In cases at the Children’s HospitaI
rein
of Trachea SeattIe
I was able to find three cases of bronchial hypoplasia and three cases of bronchial stenosis due to segmental absence of the cartiIage with lobar emphysema. U’e have had no cases of congenital hypopiastic stenosis or narrowing of the trachea. The three cases of hypoplasia of the bronchi involved the entire right side in one case, and in the other two cases the hypoplasia involved the left upper and right upper Iobes, respectively. In these three cases onIy rudimentary lungs were found, but normaI segmental bronchia patterns were present though markedly hypoplastic. The first patient with hypoplasia of the entire right bronchus and lung was not operated upon for lack of symptoms and, unfortunately, she has been lost to foIIow-up. The other two cases are interesting since surgical removal of the involved Iobes was carried out in both instances and the anatomic and pathoIogic findings carefuIIy noted. In the case of hypoplasia of the Ieft upper lobe the indications for resection were recurrent infection. The findings at surgery were essentially a norma bronchial, arteria1 and venous pattern though miniature in size serving an air-containing but rudimentary Iung. The pathologic findings of the bronchia waI1 were practicaIIy identica1 with the findings just reported. There was extreme peribronchial fibrosis. Resection was performed two years ago, and it has been interesting to folIow this chiId since the Ieft lower Iobe was considerabIy smaIIer than norma but thought to be of adequate caliber. A recent bronchogram shows the Ieft lower Iobe bronchus and Iung have developed much more sIowIy than the right or norma side. The third case of bronchia hypoplasia involved the right upper lobe but was associated with the absence of the right cIavicIe and the anterior segments of the upper five ribs on the right. Because of the marked lung herniation through this defect, it was beIieved wise to repair the rib deformity and at the same time the rudimentary right upper Iobe and hypopIastic bronchus was removed. The lower and middIe Iobes appeared quite normal as did their bronchia segments, and to date they have deveIoped quite normaIIy. The segmenta bronchia pattern of the resected Iobe was anatomically normal though miniature in size. The histoIogic findings were again onIy bronchia fibrosis and deformed cartilaginous segments of the bronchia wall. In the other three cases, the disorder was simiIar showing segmenta absence or deformity of the bronchia cartiIage just at or distal to the main stem bronchus. In a11 three of these cases the reason for resection was marked Iobar emphysema due to entrapped air. In two of these cases the Ieft upper lobe was invoIved, and in the third, the
303
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right upper was stenosed. In two cases the lobectomies were performed as emergency life-saving procedures because of the overcrowding of the intrapuImonary space by the huge emphysematous Iobes. The balI vaIve-like action of the narrowed cartilaginous segment was 0nIy a few miIIimeters in length in each instance. The bronchia1, arterial and venous suppIy to the invoIved Iobes was otherwise normal. As the authors pointed out in their presentation, the complications of trachea1 or bronchia narrowing or stenosis are usuaIIy infection or air entrapment aIong with airway obstruction; these compIications demand surgica1 correction. Little is known of the fate of the hypopIastic bronchus and rudimentary lung in certain situations, and we are hopefuI in time to be abIe to answer some of these questions. Perhaps some of the bronchia or even trachea1 obstructions in ad&s that have been thought to be an acquired stenosis, in reaIity, stem from congenita1 origin, masked by the secondary infection. BENSON B. ROE (San Francisco, CaIif.): I wish to question Dr. CantreII as to the relationship of the disease he presents to so-caIIed tracheomaIacia to which he onIy brieffy aIIuded. We currentiy are folIowing two patients whose pIane bronchograms cIoseIy approximate those that Dr. CantreII demonstrated on his patient but whose cinebronchography through the transtrachea1 route shows a fIai1 segmenta area in the Iower trachea just above the bifurcation, and onIy during expiration does this narrowed appearance occur. One of these patients has now been foIIowed for nearIy eighteen months. We are trying to decide on the proper time to operate and, indeed, upon the proper operative procedure. The area of soft trachea is fairIy Iong, and in this patient there seems to be some evidence of progressive Ioss of elasticity and narrowing. I noticed that in the cross section of the photomicrograph Dr. CantreII showed, there was evidence of thickening and fibrosis in the membranous suggesting that the two tips of the portions, trachea1 cartiIages had been puIIed together; I wondered whether he has any evidence to suggest that perhaps these patients might have had the soft cartilage syndrome, foIIowed by narrowing, and that the stenosis was indeed not congenita1 but secondary. LYMANA. BREWER,III (Los AngeIes, CaIif.) : We have been interested in trachea1 probIems for a number of years, and I wouId Iike to make two points. The first concerns the management of hypoxia during trachea1 repIacement. With the resection of a portion of the trachea, there is proper positioning of an intratrachea1 tube in the distal segment or putting of a steriIe intratrachea1 tube in the distaI trachea through the open chest. With these maneuvers there is aIways a certain
304
amount of diffrcuIty in pIacing the tubes so that for varying periods of time the patient is inevitabIy hypoxic. For that reason we have operated upon these patients using Iight hypothermia, which greatly proIongs the period that the patient wiI1 toIerate hypoxia. A number of the members wiI1 remember that we presented some work on hypothermia some years ago at the Santa Barbara meeting. We continue to empIoy it for genera1 thoracic cases and find it very heIpfu1 for tracheobronchia1 reconstructions. The second point I wouId Iike to make is that I do not believe that patients with Iong stenoses of the trachea are inoperabIe. The criterion shouId be whether or not there is adequateIy functioning Iung dista1 to the stenosis. If a chiId has maIformed Iungs that are obviousIy not functiona1, for exampIe, there is nothing of course that shouId be done. However, if the dista1 puImonary tissue is functiona1, I think that even Iong stenoses of the trachea can be successfuIIy repaired with surgery. ProbabIy the idea1 trachea1 replacement has not yet been found. We have repIaced portions of the trachea in six patients. The Gebauer type of skin grafts reinforced with stainIess stee1 wire and Martex mesh prosthesis hlave not been as successfu1 in our experience as stainIess stee1 mesh covered with fascia Iata. JAMES R. CANTRELL (closing): I think an interesting point raised by both Drs. Pinkham and Roe concerns the degree of fibrosis in the area of these segmenta stenoses. I have no adequate answer to Dr. Roe’s question. I suspect that tracheomaIacia and these stenoses are different lesions. The reason is that stenotic Iesions have been found in newborn infants and even stiIIborn fetuses, and at that earIy date they have had the characteristics of the case which I described. TracheomaIacia is an interesting and, I think, different anomaIy, and some evidence in the Iiterature indicates that the ffaccidity of the trachea1 waI1 seen in such patients does tend to disappear with time. SeveraI exampIes of this have been reported by HoIIinger. Therefore, without attempting to sound very dogmatic in this respect, I think that these are different Iesions. The cause of fibrosis in these segmenta Iesions is diffrcuIt for me to understand. I think that it is an interesting point which must be recognized since it bears a direct reIationship to the technical features of repair. The abiIity to perform a primary anastomosis depends primariIy upon the amount of reIaxation which can be gained by mobihzation of the trachea and dista1 bronchi. In the very eIderIy person, for exampIe, virtuaIIy no trachea can be resected because the mobiIity of the trachea is so sIight that the degree of tension produced wiI1 resuIt in breakdown. This does impair to a
CongenitaI
Stenosis
certain extc%nt the ability to excise long segments, even in a Young chiId with congenital stenosis of the trachea. Dr. Brewer is really deserving of congratulations on the excellent result of virtually tota replaccment of th
of Trachea
case, he did leave a smalI posterior segment of membranous trachea. 1 think this is extremely important since it is necessary, as far as I can tell, for rc-epithelialization. Without epithelialization, I doubt whether these repairs will be successful. Also with respect to Dr. Brewer’s comments, I should like to say that the amount of fumbling that Ivent on when we found that our intratracheal tube was too large to go into the left main bronchus was quite significant. Only the necessity to contain our report within the allotted ten minutes permitted us to cxcIude this ulcerogenic aspect of the case.
305
Stenosis
of the Trachea*
JAMES R. CANTRELL, M.D. AND HARRIET G. GUILD, M.D., Seattle,
From tbe Department of Surgery, University of Wasbingion School of Medicine. Seattle. Wasbirxton. and Deaartment of Pediatrics, tbe’Jobns Hopkins “Uni;ersity School of Medicine, Baltimore, Maryland.
Washington
various aIIergens. Desensitization was begun and she was discharged to the care of her private physician. The chiId did poorIy, experiencing recurrent episodes of puImonary infection and demonstrating persistent evidence of aIIergic bronchitis. She was readmitted to the hospita1 in June 1958 because of pneumonia which responded satisfactorily to treatment with antibiotics. Angiocardiography performed after recovery reveaIed a retro-esophagea1 subcIavian artery and absence of the right innominate artery. Pectus excavatum was aIs noted at that time. AIthough the chiId was stiI1 somewhat younger than was considered ideaI, it was beIieved, in view of the repeated upper respiratory tract infections and the persistent diffrcuIty in breathing, that the trachea1 stenosis shouId be immediateIy corrected. She was discharged for a brief interva1 and was readmitted for surgica1 correction of the trachea1 anomaIy. PhysicaI examination at the time of that admission reveaIed the patient to be a somewhat thin but we11 deveIoped seven year oId chiId who was in constant miId respiratory distress. MiId wheezing was noted at rest, and on exercise she developed a noticeable stridor which was both inspiratory and expiratory. A moderateIy severe pectus excavatum was present. Chest fiIm reveaIed the Iung fieIds to be essentiaIIy cIear. The heart was not enIarged. There were no other abnormaIities. Laboratory examination revealed a hemogIobin of I I .5 gm. per cent. The remaining Iaboratory studies were normaI. On July II, 1958, the patient’s right chest was expIored through a posteroIatera1 approach. A generaIized fibrous reaction was noted about the trachea. The right upper Iobe bronchus originated from the mid-portion of the thoracic trachea and appeared to be entirely norma as did the trachea above that point. The trachea beIow this bronchus was symmetricaIIy narrowed for a distance of 3.2 cm., measuring 6 mm. in externa1 diameter, approximately one-third the diameter of the nor-
HE OCCURRENCE Of COngenitaI StenOSiS Of the trachea has been documented in the Iiterature for aImost a century, yet genera1 recognition of this defect is so poor that the majority of anatomy, embryoIogy and surgica1 texts fai1 even to mention its existence. Despite the rarity of this maIformation, such Iack of recognition is regrettabIe since certain types of the anomaIy are amenabIe to compIete correction. This capabiIity assumes even greater significance when it is reaIized that this Iesion is aImost uniformly IethaI if untreated. The purpose of this report is to emphasize the diagnostic features of the anomaly, to propose a method for surgica1 correction, and to present an exampIe of a patient successfuIIy treated.
T
CASE REPORT A seven year oId white girI was admitted to the hospital in Jury, 1958 for correction of congenita1 stenosis of the thoracic trachea. There was no history of significant disease in the famiIy and there were no other instances of congenita1 anomalies. The past history was essentiaIIy negative except as related to the present iIIness. The patient had been observed to have stertorous, wheezing respiration since birth. She had been admitted to the hospita1 for the first time at one year of age because of frequent upper respiratory infections. Thorough study at that time, incIuding bronchoscopy and bronchography, reveaIed marked stenosis of the distal 3 cm. of the trachea and anomaIous origin of the right upper Iobe bronchus from the trachea just above the stenotic segment. (Fig. I.) The child was noted to have asthmatic bronchitis and study was undertaken to determine the sensitivity of the chiId to
* Presented at the annua1 meeting of the Pacific Coast SurgicaI Association, February g-12, 1964.
297
American
Journal
San Francisco, OJ Surgery,
Volume
CaIifornia, 108,
August
1964
CantreII
and GuiId
A
B
FIG. I. A, posteroanterior
view of bronchogram demonstrating the marked stenosis of the distal trachea just above the bifurcation and the tracheal origin of the right upper lobe bronchus. The upper trachea and bronchi are of normal caliber. B, oblique view of bronchogram demonstrating point of maximum narrowing at upper end of stenotic segment.
FIG. 2. Appearance of tracheobronchia1 about stenotic area obscures abnormalIy
tree at operation. Fibrous shaped, fused cartiIages.
reaction
stei notic recognizabIe cartilaginous rings. The [ding area was markedly adherent to the surl mediastina1 structures. The right lung it self and the right pulmonary artery and veins L‘PPCtared normaI. The norma and stenotic segments of the trac :hea,
ma1 trach lea. (Fig. 2.) The stenosis continued to the point of bifurcation of this structure into a Ieft main and right intermediate bronchus, each of Jwhich appeared to be of norma caliber and stru cture. The area of the stenosis was grossly orma in structure. There were no externalIy 298
Congenital
Stenosis
of Trachea
FIG. 3. Operative technic, demonstrating obliqur lines of excision at each end of stcnotic area, technic of ventilation and rrrethc,d of snastomosis utilizing interrupted sutures of braided stainless steel.
the Ieft main bronchus and the right stem bronchus were isoIated. Despite the fibrosis of the upper tracheal segment, some ffexibihty was gained by this mobiIization and it was beIieved that it would be possibIe to resect the stenotic area and carry out an anastomosis between the divided ends. Additiona mobility was gained by division of the inferior puImonary ligament and mobilization of the other hiIar structures. It appeared preoperativeIy that it wouId be technicaIIy advantageous to perform the anastomosis over an endotrachea1 tube. Since the Iumen of the stenotic area had measured onIy 4 mm. at bronchoscopy, it was cIear that a tube would not pass through the stenotic area. A Iong tube of small caliber was therefore passed into the upper trachea, the trachea sectioned obIiqueIy at the upper end of the stricture and an obIique incision made in the anterior portion of the trachea at the lower end of the stricture. It was immediateIy apparent that the endotracheal tube was too Iarge to enter the left main bronchus. A smaIIer tube was therefore pIaced in the left main bronchus through the wound and fixed by means of a gently tied umbiIica1 tape. This tube was connected to the anesthesia machine across the wound. This provided effective ventiIation but was not optimal from the point of view of technica ease in performing the anastomosis. The upper trachea1 segment was anastomosed to the bifurcation, using interrupted sutures
tension exerted at the base of the carina, the two tracheal segments were brought into apposition and the sutures were tied. The anterior sutures
FIG. 4. Postoperative roentgenogram of chest just prior to discharge from hospital. Note the abnormally high position of trachea1 bifurcation at lower border of the third thoracic vertebra. Preoperative films reveaIed bifurcation level to be at midpoint of the fifth thoracic vertebra.
of No. 4-o braided stainless steel wire. (Fig. 3.) AI1 posterior sutures were pIaced, and with down-
ward traction upon the upper segment and upward
299
CantreII and GuiId deveIoped transient atelectasis in the Ieft Iower Iobe which responded to steam, expectorants and vigorous coughing. The patient’s trachea was not suctioned directly. By the eighth postoperative day the chiId was afebriIe and breathing cIearIy and without difficulty. At the time of discharge the lungs were entireIy clear (Fig. 4) and respiration was compIeteIy free. Gross examination of the resected tissue reveaIed a stenotic segment of tracheobronchia1 tree, the Iumen of which measured 3 mm. at its narrowest point. The cartiIaginous rings were abnorma1 in shape and were fused. HistoIogic sections showed miId chronic inflammation in the submucosa and an increased amount of dense coIIagen in the waI1 of the trachea. (Fig. 5.) The chiId has been seen on severa occasions since discharge. Her Iast return visit was in March, twenty-one months after I 960, approximateIy operation. Bronchoscopy performed at that time reveaIed an uncompromised Iumen equa1 in size to the lumen of the trachea above. There was no stenosis at the suture line. She has been foIIowed since that time through her private physician. The most recent contact was in January, 1964. The child, now thirteen years of age, has deveIoped normaIIy, has no respiratory symptoms whatever, carries on unIimited physica activity, has had no recent puImonary infections and has no further asthmatic symptoms. Her onIy difficulty is the progressiveIy deepening pectus excavatum.
FIG. 5.
Photomicrograph of junction of normaI trachea (right) and stenotic area (left) revealing abnormal chronically inflamed and thickened epicartilage, theIium and submucosa and increased colIagen (extreme Ieft) in the stenotic area; magnification X 35. pIaced about the endobronchia1 tube were then and al1 but three sutures tied. The tube was then removed, the remaining sutures tied and ventiIation re-established through the endotrachea1 tube. AI1 Iobes of the right Iung immediateIy ventilated freely. The anastomosis seemed to be of good size. There was no air Ieak from the anastomosis and the mediastina1 pleura was cIosed IooseIy. Chest drainage was instituted and the wound cIosed in Iayers with interrupted silk sutures. ProphyIactic tracheostomy was considered but it was beIieved that in these circumstances the hazards of the tracheostomy might be greater than its virtues. It was therefore eIected to defer this procedure unless it became cIearIy indicated. The patient received 250 cc. of whoIe bIood during the procedure. The child’s postoperative course was satisfactory. She had some diffIcuIty in coughing and
COMMENTS
CongenitaI stenosis of the trachea is an unIesion. We have been able to find onIy twenty-four acceptabIy documented cases common
FIG. 6$‘Diagram of the three morphologic variants of congenital stenosis of the trachea. Left, generaIized hypopIasia. Center, funneI-Iike stenosis. Right, segmenta stenosis. The occurrence of anomaIous bronchi, as in the case presented, is apparently most common in the segmenta form.
300
CongenitaI
Stenosis
of Trachea the anomaIies are uncIear, as is the mechanism of development. The defect must develop at a relatively early period in embryonic life. Although the available reports are inadequate to permit a definite concIusion, no evidence of a fan&a1 tendency has been noted. Associated Anomalies. AbnormaI origin of the major bronchi is occasionaIIy seen. In addition to the presentIy reported case, one example of trachea1 origin of the right upper Iobe bronchus has been observed [4]. In one of the cases reported by HoIIinger [12], the entire right bronchia tree arose from the Ieft main bronchus. Each of these abnormalities was noted in association with segmenta stenosis. AnomaIies of the Iung have been noted in three instances. In the case reported by Gruber [8], the right Iung was apIastic and the Ieft Iung was without fissures. Agenesis of the right Iung was noted in the case reported by Putney and BaItzeII [17], and abnormat IobuIation of the lungs was reported by Weber [21]. A number of co-existing anomaIies of other systems have been reported. These incIude cIubfoot [9], congenita1 absence of the thumbs [IO], maIformation of the brain [6], MeckeI’s diverticuIum [IO], intestina1 stenosis [14], imperforate anus [2], abnorma1 IobuIation of the Iiver [IO], ventricuIar septal defect [r5], persistent truncus arteriosus [12], abnormal position of the left puImonary artery [ 191 and atria1 septa1 defect [13]. Pectus excavatum, a retroesophagea1 subcIavian artery and absence of the innominate artery were observed in the present case. It is important to note that with two exceptions, none of these co-existing anomalies were incapabIe of correction or inconsistent with Iife. Clinical Features. The most significant cIinica1 symptom associated with trachea1 stenosis is stridor which is both inspiratory and expiratory and often becomes apparent shortly after birth. Wheezing is aIso commonIy described. Depending upon the degree of obstruction, inspiratory retraction of the sternum and empIoyment of secondary muscIes of respiration may be noted. Shortness of breath is routine but may be paroxysma in nature, occasionaIIy having been reported to be primariIy nocturna1. Cyanosis may be present and may occur at rest or onIy on exercise. Feeding diffrcuIty is common, often resuIting in faiIure to gain weight and deveIop normaIIy. Respira-
in the literature [r,2,4-221 aIthough a few additional cases may exist in the older foreign literature which has been unavailable to us. Three morphoIogic variants may be seen. (Fig. 6.) J. Generalized Hypoplasia. AIthough this malformation should perhaps be considered as a separate entity, it has been considered in this category since the functiona impairment is simiIar in every way to the other forms of stenosis. In this anomaIy, the entire trachea is narrow in caIiber, posterior fusion of the individual cartiIaginous rings usuaIIy being noted. The diameter of the Iumen in the reported cases has varied from I to 3 mm. in newborn infants. HypopIasia appears to be uniformIy Iimited to the trachea itself, the major bronchi and dista1 tracheobronchia1 tree being of normaI size. Seven examples of this anomaIy have been reported [1,4-6,14,17,183. 2. Funnel-like Stenosis. In this form of stenosis the diameter of the trachea immediateIy beIow the cricoid is of norma size but the trachea becomes progressiveIy narrower to the IeveI of the bifurcation. Again, the membranous trachea may be partiaIIy absent with fusion of the trachea1 cartiIages in a portion of the stenotic area. The point of maximum constriction is found just above the bifurcation and the Iumen at that point may be as narrow as I mm. in diameter. Four documented cases of this type of stenosis have been reported [8,x1,13,22]. 3. Segmental Stenosis. SegmentaI stenosis, in which a portion of the trachea is narrowed in hourgIass fashion, wouId appear to be the most common form of the anomaIy, more than 50 per cent of the reported cases being of this type [4,7,9,10,12,15,16,19,20]. A significant proportion of these stenoses are associated with a fibrotic reaction in the region of narrowing. The cartiIages are usuaIIy abnorma1 in shape and form compIete rings. The Iength of the stenotic area varies from I to 5 cm., the majority of the reported cases being approximateIy 2 cm. in Iength. The stenotic area may be found in any portion of the trachea. Of the eleven cases in which the point of narrowing was adequateIy described, four occurred in the proxima1 trachea just beIow the cricoid, 3 occurred in the centra1 portion of the trachea and four were noted just above the bifurcation. The etioIogic factors in the production of 301
CantreII and GuiId tory infections are frequent and are usuaIIy the uItimate cause of death. Definitive Diagnostic Measures. The diagnosis of trachea1 stenosis may occasionaIIy be confirmed by examination of the IateraI roentgenogram of the chest in which the size and configuration of the infant trachea are usuaIIy we11 defined. Evans has pointed out, however, that diffIcuIty may be encountered in the interpretation of such fiIms unIess the examination is made during inspiration. Bronchoscopic examination or tracheograms wiI1 provide definitive confirmation but caution must be utiIized in advocating either of these procedures. The trauma associated with injudiciousIy performed bronchoscopy may precipitate acute fata trachea1 obstruction. If bronchography is performed, water-soIubIe media shouId be utiIized to avoid the mucosa1 reaction which occasionaIIy foIIows the instaIIation of oiIy contrast media. Because of the dista1 Iocation of the stricture, tracheostomy may be of no vaIue in reIieving airway obstruction which may foIIow these procedures. Diferential Diagnosis. Other Iesions which must be considered in the differentia1 diagnosis incIude a variety of causes of externa1 compression of the trachea (vascuIar rings, enIargement of the thymus and mediastina1 masses) as we11 as foreign body. Inspiratory stridor may aIso be seen in instances of tracheomaIacia. Prognosis and Therapy. If untreated, these anomalies are aImost invariabIy fata in earIy infancy or chirdhood. The eventua1 demise is usuaIIy the resuIt of repeated respiratory tract infections. In view of the high earIy mortaIity associated with this anomaIy, it may be concIuded that correction must usuaIIy be accompIished earIy in Iife. HoIIinger’s report [12] of apparentIy satisfactory resuIts foIIowing endoscopic dilatation in two instances is surprising in view of the abnorma1 structure of the stenosed segment which is routineIy observed. Furthermore, it wouId appear that diIatation may itseIf be extremeIy hazardous and that this approach shouId be recommended onIy with great caution. In the segmenta form of stenosis, optima1 results shouId be obtained by excision of the narrowed area with primary anastomosis of the divided ends of the trachea. The majority of the reported stenoses have been reIativeIy short and virtuaIly a11 shouId have been 302
amenabIe to this form of therapy. The feasibiIity and successfu1 resuIts of trachea1 anastomosis have been ampIy demonstrated. The experImenta evaIuation of this technic reported by CantreII and FoIse [3] wouId suggest that when properIy utiIized this method is remarkabIy safe and effective. The question of further growth of the anastomotic site cannot be answered at this time but it wouId appear that if a satisfactory anastomosis with primary heaIing is obtained, subsequent growth of the trachea at this point shouId not be hindered. In the funnel-type stenoses it wouId seem reasonabIe to resect the most narrowed dista1 portion of the trachea and to anastomose the proxima1 trachea to the bifurcation. Since a Iumen of no more than haIf of the norma diameter of the trachea is sufficient to provide adequate air flow, repair of this type shouId produce a good functiona resuIt. No attempts to carry out such a procedure have been reported in the Iiterature and we have had no occasion to dea1 with a chiId with this type of anomaIy. At the present time no satisfactory method of therapy appears avaiIabIe for the repair of the totaIIy hypopIastic trachea. SUMMARY I. Congenital stenosis of the trachea is a reIativeIy uncommon anomaIy yet one which shouId be recognized since in many instances the Iesion is susceptibIe to correction. 2. The presence of stridor, dyspnea and cyanosis in a newborn infant or smaI1 chiId shouId suggest the possibiIity of trachea1 stenosis. 3. Definitive diagnosis can be estabIished by IateraI roentgenograms of the chest, bronchoscopy and/or bronchography. 4. GmpIete correction of this anomaIy shouId be possibIe in many instances by excision of the stenotic area and reanastomosis of the trachea. 5. A case of segmenta stenosis of the trachea corrected in this manner is described. To our knowledge, this case is the first in which successfuI operative correction of such a defect has been accompIished. REFERENCES I. BEICKERT, P. Tracheal
Stenose durch KnorpelmissbiIdung. HNO. 7: 325, 1959. 2. BONNIER, M. Un cas de stknose cong&itaIe de la
CongenitaI
3.
4.
5.
6.
7.
8. 0. IO. I I.
Stenosis
trach& avec bronche du Iobe sup&ur droite atr(.sier et lobe supkrieur droite aplasique. Imperforation de I’anus. Ann. mtd.-cbir. Hbp. Sainte-Justine Mont&al, 5 : 13, 1947. CANTRELL, J. R. and FOLSE, J. R. The repair of circumferential defects of the trachea by direct anastomosis: experimenta evaIuation. J. Tboracic @ Cardiovas. Surg., 42: 589, 1961. EVARS, IV. A., JR. CongenitaI obstructions of the respiratory tract. I. Trachea1 malformations. Am. J. Roentgenol., 62: 167, rg4g. FINKELSTEIN, H. Lehrbuch der %ulingskrankheiten, 3rd ed., p. 621. Berlin, 1924. Julius Springer. GABRIEL, E. and FEYRTER, F. Angeborene tracheal Stenose bei einem 434 jahrigen Kinde. Jabrb. f. Kinderb., 121: zg, 1928. GREGOR, K. Ein FaII von angeborener Missbildung der Luftrijhre (circumscripte Derengerung mit FehIen der Pars membranacea). Jabrb. f. Kinderb., 49: 123, 1899. GRUBER, W. Oesterr. Ztscbr. f. prakt. Heilk., 16: 7, 1870. Cited by Haardt [zo]. GKJISEZ,J. Malformation congenitale de la trachbe. Bull. d’oto.-rhino.-laryngol.,~25: 289, 1927. HAARDT, W. Ueber angeborene Enge der Luftrchre. Wien. klin. Wcbnscbr.. $ I : 6~7. 10x8. HEIKEL, P. E. Congenital tracheal stenosis combined with truncus arteriosus cordis; a case diagnosed in vitam. Ann. paediat. Fenn., 3: 22,
1957. 12. HOLLINGER, P. H., JOHNSTON,K. C. and BASINGER, C. E. Benign stenosis of the trachea. Ann. Otol. Rbin. u Laryng., 59: 837, 1950. I 3. HOUSTON, I. B. and MACKIE, D. G. Congenital trachea1 stenosis. Thorax, 16: 94, 1961. 14. LERRO, E. Un case di stridore congenito da stenosi tracheale. Pediatria, 43: 440, 1935. 15. MONTANDON, A. Stbnose cong&nitaIe de Ia trachee et maIformations associbes. Pratt. oto-rbinolaryng., 6: 179, 1944. 16. Moussous, M. Sur un cas de &r&zissement cong&nitaIe de Ia trachde. J. mkd. Bordeaux, 37: 195, 1907. 17. PUTNEY, F. J. and BALTZELL, W. H. Agenesis of the Iung with trachea1 stenosis. Ann. Otol. Rbin. Laryng.; 61: 677, 1952. 18. SANKOTT, A. M. Ueber einen FaII mit angeborener Enge der Trachea und der Bronchien, FehIen der Paries membranaceus tracheae, DivertickeIbildung usw. Wien. klin. Wcbnscbr., 35: 391,1922. 19. SCHEID, P. Missbildung des TrachealskeIettes und der Iinken Arteria puImonaIis mit Erstickungstod bei 7 monate aItem Kind. Frankfurt. Ztscbr. Path;, 52: 114, 1938. 20. STEWART. S. and PINKERTON. H. H. UnusuaI stenos& of trachea in an aduIt. Brit. J. Anaestb.,
27: 492. ‘955.
21. WEBER, H. W. Ober kongenitaIe TracheaIstenosen.
Zentralbl. allg. Patb., 89: 148, 1952. stenosis of the trachea. Am. J.. Dis. Cbildy, 61: 1263, 1941.
22. WOLMAN, I. J. Congenital
DISCUSSION ROLAND D.
viewing
the
PINKHAM (SeattIe, Wash.): In cases at the Children’s HospitaI
rein
of Trachea SeattIe
I was able to find three cases of bronchial hypoplasia and three cases of bronchial stenosis due to segmental absence of the cartiIage with lobar emphysema. U’e have had no cases of congenital hypopiastic stenosis or narrowing of the trachea. The three cases of hypoplasia of the bronchi involved the entire right side in one case, and in the other two cases the hypoplasia involved the left upper and right upper Iobes, respectively. In these three cases onIy rudimentary lungs were found, but normaI segmental bronchia patterns were present though markedly hypoplastic. The first patient with hypoplasia of the entire right bronchus and lung was not operated upon for lack of symptoms and, unfortunately, she has been lost to foIIow-up. The other two cases are interesting since surgical removal of the involved Iobes was carried out in both instances and the anatomic and pathoIogic findings carefuIIy noted. In the case of hypoplasia of the Ieft upper lobe the indications for resection were recurrent infection. The findings at surgery were essentially a norma bronchial, arteria1 and venous pattern though miniature in size serving an air-containing but rudimentary Iung. The pathologic findings of the bronchia waI1 were practicaIIy identica1 with the findings just reported. There was extreme peribronchial fibrosis. Resection was performed two years ago, and it has been interesting to folIow this chiId since the Ieft lower Iobe was considerabIy smaIIer than norma but thought to be of adequate caliber. A recent bronchogram shows the Ieft lower Iobe bronchus and Iung have developed much more sIowIy than the right or norma side. The third case of bronchia hypoplasia involved the right upper lobe but was associated with the absence of the right cIavicIe and the anterior segments of the upper five ribs on the right. Because of the marked lung herniation through this defect, it was beIieved wise to repair the rib deformity and at the same time the rudimentary right upper Iobe and hypopIastic bronchus was removed. The lower and middIe Iobes appeared quite normal as did their bronchia segments, and to date they have deveIoped quite normaIIy. The segmenta bronchia pattern of the resected Iobe was anatomically normal though miniature in size. The histoIogic findings were again onIy bronchia fibrosis and deformed cartilaginous segments of the bronchia wall. In the other three cases, the disorder was simiIar showing segmenta absence or deformity of the bronchia cartiIage just at or distal to the main stem bronchus. In a11 three of these cases the reason for resection was marked Iobar emphysema due to entrapped air. In two of these cases the Ieft upper lobe was invoIved, and in the third, the
303
CantreII
and GuiId
right upper was stenosed. In two cases the lobectomies were performed as emergency life-saving procedures because of the overcrowding of the intrapuImonary space by the huge emphysematous Iobes. The balI vaIve-like action of the narrowed cartilaginous segment was 0nIy a few miIIimeters in length in each instance. The bronchia1, arterial and venous suppIy to the invoIved Iobes was otherwise normal. As the authors pointed out in their presentation, the complications of trachea1 or bronchia narrowing or stenosis are usuaIIy infection or air entrapment aIong with airway obstruction; these compIications demand surgica1 correction. Little is known of the fate of the hypopIastic bronchus and rudimentary lung in certain situations, and we are hopefuI in time to be abIe to answer some of these questions. Perhaps some of the bronchia or even trachea1 obstructions in ad&s that have been thought to be an acquired stenosis, in reaIity, stem from congenita1 origin, masked by the secondary infection. BENSON B. ROE (San Francisco, CaIif.): I wish to question Dr. CantreII as to the relationship of the disease he presents to so-caIIed tracheomaIacia to which he onIy brieffy aIIuded. We currentiy are folIowing two patients whose pIane bronchograms cIoseIy approximate those that Dr. CantreII demonstrated on his patient but whose cinebronchography through the transtrachea1 route shows a fIai1 segmenta area in the Iower trachea just above the bifurcation, and onIy during expiration does this narrowed appearance occur. One of these patients has now been foIIowed for nearIy eighteen months. We are trying to decide on the proper time to operate and, indeed, upon the proper operative procedure. The area of soft trachea is fairIy Iong, and in this patient there seems to be some evidence of progressive Ioss of elasticity and narrowing. I noticed that in the cross section of the photomicrograph Dr. CantreII showed, there was evidence of thickening and fibrosis in the membranous suggesting that the two tips of the portions, trachea1 cartiIages had been puIIed together; I wondered whether he has any evidence to suggest that perhaps these patients might have had the soft cartilage syndrome, foIIowed by narrowing, and that the stenosis was indeed not congenita1 but secondary. LYMANA. BREWER,III (Los AngeIes, CaIif.) : We have been interested in trachea1 probIems for a number of years, and I wouId Iike to make two points. The first concerns the management of hypoxia during trachea1 repIacement. With the resection of a portion of the trachea, there is proper positioning of an intratrachea1 tube in the distal segment or putting of a steriIe intratrachea1 tube in the distaI trachea through the open chest. With these maneuvers there is aIways a certain
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amount of diffrcuIty in pIacing the tubes so that for varying periods of time the patient is inevitabIy hypoxic. For that reason we have operated upon these patients using Iight hypothermia, which greatly proIongs the period that the patient wiI1 toIerate hypoxia. A number of the members wiI1 remember that we presented some work on hypothermia some years ago at the Santa Barbara meeting. We continue to empIoy it for genera1 thoracic cases and find it very heIpfu1 for tracheobronchia1 reconstructions. The second point I wouId Iike to make is that I do not believe that patients with Iong stenoses of the trachea are inoperabIe. The criterion shouId be whether or not there is adequateIy functioning Iung dista1 to the stenosis. If a chiId has maIformed Iungs that are obviousIy not functiona1, for exampIe, there is nothing of course that shouId be done. However, if the dista1 puImonary tissue is functiona1, I think that even Iong stenoses of the trachea can be successfuIIy repaired with surgery. ProbabIy the idea1 trachea1 replacement has not yet been found. We have repIaced portions of the trachea in six patients. The Gebauer type of skin grafts reinforced with stainIess stee1 wire and Martex mesh prosthesis hlave not been as successfu1 in our experience as stainIess stee1 mesh covered with fascia Iata. JAMES R. CANTRELL (closing): I think an interesting point raised by both Drs. Pinkham and Roe concerns the degree of fibrosis in the area of these segmenta stenoses. I have no adequate answer to Dr. Roe’s question. I suspect that tracheomaIacia and these stenoses are different lesions. The reason is that stenotic Iesions have been found in newborn infants and even stiIIborn fetuses, and at that earIy date they have had the characteristics of the case which I described. TracheomaIacia is an interesting and, I think, different anomaIy, and some evidence in the Iiterature indicates that the ffaccidity of the trachea1 waI1 seen in such patients does tend to disappear with time. SeveraI exampIes of this have been reported by HoIIinger. Therefore, without attempting to sound very dogmatic in this respect, I think that these are different Iesions. The cause of fibrosis in these segmenta Iesions is diffrcuIt for me to understand. I think that it is an interesting point which must be recognized since it bears a direct reIationship to the technical features of repair. The abiIity to perform a primary anastomosis depends primariIy upon the amount of reIaxation which can be gained by mobihzation of the trachea and dista1 bronchi. In the very eIderIy person, for exampIe, virtuaIIy no trachea can be resected because the mobiIity of the trachea is so sIight that the degree of tension produced wiI1 resuIt in breakdown. This does impair to a
CongenitaI
Stenosis
certain extc%nt the ability to excise long segments, even in a Young chiId with congenital stenosis of the trachea. Dr. Brewer is really deserving of congratulations on the excellent result of virtually tota replaccment of th
of Trachea
case, he did leave a smalI posterior segment of membranous trachea. 1 think this is extremely important since it is necessary, as far as I can tell, for rc-epithelialization. Without epithelialization, I doubt whether these repairs will be successful. Also with respect to Dr. Brewer’s comments, I should like to say that the amount of fumbling that Ivent on when we found that our intratracheal tube was too large to go into the left main bronchus was quite significant. Only the necessity to contain our report within the allotted ten minutes permitted us to cxcIude this ulcerogenic aspect of the case.
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