Deviated By Christoph

Trachea in Hypoplasia and Aplasia of the Right Lung: Airway Obstruction and Its Release by Aortopexy Dijhlemann,

Karl Mantel,

Karl Schneider,

Monika

Giintner,

Eckart Kreuzer, and Waldemar

C. Hecker

Munich, West Germany l Three cases of male infants with hypoplasia and aplasia of the right lung and dextrocardia are reported. The infants developed increasing obstructive respiratory distress in the first 4 months of life. In all three cases, there was an opaque right hemithorax with overinflation of the left lung, a posterior deviation of the trachea with pulsatile stenosis, and a posteriorly located aortic arch. Hypoplasia (two cases) or aplasia (one case) of the right pulmonary artery and an absence or a remnant of the right main bronchus were also observed. Associated malformations were esophageal atresia with an abnormal high pouch of the proximal esophagus in case 1, and hypertelorismus syndrome in case 2. Because of severe respiratory distress in all three patients, an aortopexy was performed at the ages of 5 months (one infant) and 10 months (two infants). The obstructive respiratory signs disappeared postoperatively in the following weeks. It has been observed that tracheal stenosis in aplasia or hypoplasia of the right lung may be caused by the dislocated aortic arch. An aortopexy can release the airway compression in such cases. Because of associated malformations, a careful evaluation of the airway and vessels is recommended. 0 1990 by W.B. Saunders Company. INDEX WORDS: Hypoplasia. lung; aplasia, aortic arch; aortopexy; respiratory distress.

lung; deviated

P

APLASIA is not incompatible ULMONARY with life.’ However, a history of wheezing respiration and sometimes early death in patients with aplasia of the right lung is not uncommon.1-5 This suggests obstructive lung disease, which may be due to compression or deviation of the airway by an overexpansion of the left lung and by dislocation of the aorta and/or the pulmonary artery. Nevertheless, only a few cases (n = 6) have been reported demonstrating airway compression due to anomalous or deviated great vessels.6“’ To illustrate its occurence, three, cases of infants with aplasia or marked hypoplasia of the right lung

From ihe Departments of Pediatrics, Pediatric Radiology, and Pediatric Surgery, Dr v. Haunersches Kinderspital. and the Department of Cardiac Surgery, Kfinikum Gro+Thadern, University of Munich. Munich, FRG. Date accepted: January 9.1989. Address reprint requests to Christoph Diihlemann, MD, Univ. Kinderklinik. Abtlgf. Kardiologie, Lindwurmstr. 4.8000 Minchen 2. FRG. 6 1990 by W.B. Saunders Company. 0022-3468/90/2503-0002$03.00/0

290

and airway compression are reported. The heart and the left outflow tract were rotated and shifted to the right, posteriorly diverting the aortic arch, which can lead to tracheal compression. In all three cases, successful release from airway obstruction was achieved by suturing the aorta to the undersurface of the sternum. Only two cases have been previously reported in which this sort of procedure was attempted: one case successfully,” the other with a complicated postoperative course.’ Therefore, more cases should be reported to give us more information about the outcome of these severely ill infants. CASE

REPORTS

Case I A boy (weighing 2,250 g) was born at gestational age 37 weeks to a 34-year-old woman (gravida 3, para 3) in September 1981. An amniocentesis was performed in the 14th week of gestation because of a free trisomy 21 of her second child. The l-, 5, and lo-minute Apgar scores were 4.8, and 10. Because of hydramnion and an unsuccessful pass of nasogastric tube, the infant was examined by x-ray, which showed a blind ending of the upper esophagus that projected very high on vertebra C7, with air in the stomach on the left side. The right hemithorax was opaque, with a shift of the mediastinum and the heart to the right. Bronchoscopy showed a tracheoesophageal fistula in the distal esophageal segment, and no detectable origin of the right main bronchus. Gastrostomy was performed in the same session on the first day of life. On the second day, heart catheterization was done, showing dextrocardia with situs solitus, normally related great arteries, a narrow patent duct with minor left-to-right shunt, and pulmonary hypertension (pulmonary artery pressure [PAP], SO/20 mm Hg; aorta descendens, 55/35 mm Hg). In the angiocardiogram, a normal-sized left pulmonary artery (LPA) with normal venous return in the left atrium (LA), and a hypoplastic right pulmonary artery (RPA) with no detectable venous return was observed. The aortic arch was dislocated to the right, and posterior. Surgical repair of the esophageal atresia and tracheoesophageal fistula was attempted, but a primary anastomosis of the esophagus was thought not feasible because of the high pouch and the long atretic segment. Therefore, a cervical esophagostomy and the division of the tistula were performed on the third day of life. The postoperative course was uneventful, and the newborn was extubated four days later and discharged home at 4 weeks of age. In the following weeks, a persistent stridor reoccured, with episodes of apnea and cyanosis, some of which were severe, that prompted readmittance to our hospital at the age of 4 months. There was an increase of overexpansion of the left lung, herniating to the right. Contrast imaging through the cervical esophagostomy excluded a dilatation of the proximal esophagus. Bronchoscopy showed a distinct pulsatile stenosis of the distal trachea from its anterior wall, a remnant of a right main bronchus,

Journal of Pediatric Surgery, Vol 25, No 3 (March). 1990: pp 290-293

DEVIATED TRACHEA IN HYPOPLASIA & APLASIA

and a fold of the closed tracheoesophageal fistula. The infant could not be extubated in the following 2 weeks. At age 4% months, a repeated heart catheterization showed no marked pulmonary hypertension (PAP, 35/20 mm Hg), and the ductus arteriosus was closed. Tracheography in combination with angiography demonstrated a posterior deviation of the trachea due to the abnormally right-sided and posterior-located aorta (Fig 1). Because of this finding, an aortopexy was undertaken at the age of 5 months. After anterolateral thoractomy in the second right intercostal

291

space, the operative findings showed no right-sided lung. The aorta was coming far from the right side, and its position was more posterior than normal. The ascending aorta and the proximal part of the truncus brachiocephalicus was fixed with seven prolene (4/O) sutures to the back of the sternum, so the vessels could be displaced anteriorly for 3 cm. No pulsatile stenosis was found during postoperative bronchoscopy. The patient was extubated 4 weeks later, and respiratory status improved during the following 3 months. At 2 years of age, a colon interposition of the long atretic segment

Fig 1. Angiotracheogrephy. Note pooterior devietion of the trachea. There is e hypoplestic RPA. (A) Fronts1 view, fB) lateral view. In (C,D) levogram the aortic erch can be identified, dialoceting the trachea dorsally. TV. tubus: LPA, left pulmonary artery; RPA. right pulmonary ertery: TR. trechaa: AO, aorta: LA, laft atrium: LV. laft ventricle; A0 DES, aorta descendens.

292

and a pyloroplasty were performed with an uncomphcated postoper-

Case 2 After the uncomplicated pregnancy of a 33-year-old woman (gravida 3, para 3). a full-term 2,800-g boy was delivered in May 1985, with 1-, 5, and lOminute Apgar scores of 6, 4;and 8, and respiratory distress. X-ray examination showed dextrocardia and aplasia or hypoplasia of the right lung. There were other dysplastic signs, suchas hemivertebrae of the thoracic column and scoliosis, a supernumerary digit at the base of the right toe I, a syndactyl toe I-IV on both sides, a marked hypertelorism and a broad nasal root (suggesting Greig’s syndrome of hypertelorism,‘3*‘4), moderately dilated cerebral lateral ventricles that were asymmetric, -and a partial agenesis of the corpus callosum (sonogaphy was performed at the age of 11 months)_Respiratory status improved, and the infant was diiharged home at 4 weeks of age. A short apnaic spelloccurred 3 weeks later, and the stridor became ‘more pronounced in the following weeks. At the time of transfer to our hospital at the age of 8 months, the boy was-intubated, with recurringstridorous breathing when extubation was attempted. Pluoroscopy showed the trachea to be posteriorly deviated without distinct stenosis in air contrast. Bronchoscopy showed a pulsatile anterior wall prolapseof the inferior trachea near length. A combined tracheography and angiography demonstrated the posteriorly deviated trachea and aorta. There was no RPA. The LPA and its venous connection to the left atrium were normal. No shunt was detected, and moderate pulmonary hypertension (PAP, 45/20 mm Hg; systemic pressure, 100/60 mm Hg) was measured. At the age of lo-months in March 1986, a right anterolateral thoracotomy was performed. The heart was shifted to the right and posteriorly; no right lung could be found. The left lung expanded to the right and was partly transferred back to the left by resecting some pleura1 tissue and fixing (Vicryl4/0) the remaining pleura at the left posterior part of the sternum. The ascending aorta, lying far posterior, was elevated by four Teflon adventitial sutures (4/O) to the right side of the retro sternum. Therefore, the dextrorotated apex of the heart was lifted, and its pericardium wes also fixed to the sternum. Interoperative bronchoscopy showed some lifting of the trachea and releasing of the tracheal stenosis. The infant was extubated 14 days later. Postoperative bronchoscopy showed approximately 30% stenosis of the left main bronchus, and no pelsatile stenosis of the trachea. The infant has done well since then, with no symptomes of respiratory obstruction.

Case 3 A 3,100-g full-term boy was born in May 1984 to a 28-year-old woman who had asthma bronchiale and occasionally required corticosteroids. &soprenalin, and A&spin. Because of prolonged delivery, a vacuum extraction was performed_ The 1-, 5-, and lOminute Apgar scores were 8, 9, and 10. The infant developed episodes of tachypnea. thoracic retractions, and thereby reduced PO, in the tirst 2 months. Initial x-rays showed the right thorax homogenously opaque, and in lateral projection, a posterior curvature of tbe trachea. Doppler cchocardiography showed no duct flow in the PA, a normal LPA, and a very small RPA (ratio, 41). Cranial ultrasound showed a moderately asymmetrical dilatation of the lateral ventricles. At the age of 2% months the infant was discharged home, but retractions and wheezing increased and a chronical loud cough started. Therapy with corticosteroids, Euphyllin, and 6agonist was unsuccessful, and episodes of severe respiratory distress occurred. There was posterior curvature of the trachea (air contrast) and the esophagus (contrast swallow). Bronchoscopy demonstrated

pulsatile compression of the anterior wall of the inferior trachea, with the right main bronchus as a blind pouch of a few millimeters in length. The infant could not be extubated, thereby necessitating an extreme delicate position of the endotracheal tube to allow adequate ventilation. Subtraction angiography showed the dislocated right portion of the heart and the aortic arch. Because of movement artifacts, the trachea could not be evaluated. Therefore, a combined tracheoangiography was undertaken that showed a posteriorly deviated trachea due to the deviated aortic arch wrapping around the trachea. The RPA was hypoplastic. There was no pulmonary hypertension. At the age of 10% months, an anterolateral thoracotomy in the second intercostal space was performed. The left lung was extremely +nflated and extended to the right thorax overlying the right-sided posteriorly deviated heart. Some pleural tissue was resected to move the left lung to the left thorax. The remaining pleura was reclosed, with fixation sutures, to the left posterior part of the sternum. The posteriorly located aorta and the tnmcus brachiocephalicus were lifted by four prolene adventitia (4/O) and fixed to the midline of the sternum. Intraoperative bronchosccpy showed some lifting of the anterior tracheal wall. The patient was extubated 7 days later, and the postoperative course was uncomplicated. Signs of respiratory obstruction dip peared during the following 2 months. DISCUSSION

The angiotracheographic and tracheoscopic findings of these three cases demonstrate a markedly posterior curvature of the trachea, due to a posteriorly dislocated aortic arch, tightly crossing the trachea. There was no compression by the innominate artery as in other cases involving tracheomalacia at the site of crossing of this vessel.ts@ Dextrocardia, with situs solitus and normally related great arteries without intracardiac malformation, may be secondary to factors such as agenesis or hypoplasia of the right lung. In normal development the heart shifts to the left in the fourth week of fetal life.‘7~‘8At this&me, the trachea is shown as a ventral diverticulum rising from the foregut.” A disturbance at this time could involve both the shift of the heart and the appearance of the right main bronchus. On the other hand, a deviated aortic arch may disturb the development of the right bronchus. The investigator must consider other cardiac and noncardiac anomalies.20q2’The possible association of esophageal atresia22’23 is important, and it is emphasized that sometimes (three cases in the literature, and case 1 of this report) a high collar blind pouch of the esophagus can occur with a long atretic segment, which makes a primary esophagostomia impossible.‘0*24Y2’ Associat&vascular anomalies are: ductus arteriosus with early pulmonary hypertension, a ligamenturn arteriosum causing compression of the airway, and an anomalous pulmonary venous return. The LPA may cross behind the trachea, causing a pulmonary sling.’ In such cases, the barium esophagogram may pro-

DEVIATED TRACHEA IN HYPOPLASIA

293

& APLASIA

vide important diagnostic information and should be performed very early. To exclude tracheal malformation as ring cartilage, a tracheobronchoscopy is needed to show the exact location and length of the tracheal stenosis, with special attention to additional stenosis or malacia of the left main bronchus. Heart catheterization with angiography is recommended, to delineate the PAP, the course of PA and veins (scimitar syndrome?), and the course of the aorta with special regard to the trachea. Computed tomography and magnetic resonance imaging can provide important diagnostic information, but sometimes it is difficult to scan at the optimal level. However, angiography is still indicated and should be performed early in an infant with aplasia and hypoplasia of the right lung and respiratory distress. If the natural history does not show any improvement of respiratory distress, an aortopexy should be undertaken without delay in cases with increasing respiratory distress. In this procedure, the trachea is

drawn anteriorly to release the compression and to shorten the upper airway to the left lung. In two of the three cases, the distended left lung was partly referred back to the left side, allowing some removal of the heart. This might be useful, but there is no proof that a combined relocation of heart and lung is superior to an aortopexy alone. An intraoperative bronchoscopy is difficult to perform because of the lateral position of the patient during the operation, but some releasing of stenosis was observed. Release of airway compression was evident in the postoperative evaluation. Prophylactic aortopexy is not recommended because some cases do not develop respiratory symptoms.’ It is concluded that hypoplasia of the right lung involving a deviated heart and aorta may lead to posterior dislocation of the trachea, when crossed by the aortic arch. This causes prolongation and obstruction of the airway, and can be released and shortened by aortopexy.

REFERENCES 1. Maltz DL, Nadas AS: Agenesis of the lung. Presentation of eight new cases and review of the literature. Pediatrics 42174-188, 1968 2. Valle AR: Agenesis of the lung. Am J Surg 89:90-100, 1955 3. Wexels P: Agenesis of the lung. Thorax 6:171-192,195l 4. Yaghmai J: Agenesis of the lung. AJR 108:564-568, 1970 5. Schaffer AJ, Rider RV: A note on the prognosis of pulmonary agenesis and hypoplasia according to the side affected. J Thorax Surg 33:379-382, 1957 6. Harrison MR. Hendren HW: Agenesis of the lung complicated by vascular compression and bronchomalacia. J Pediatr Surg l&813817.1975 7. Henchman DC, Macarthur EB: Agenesis of the right lung. Report of a case. Med J Aust 2:675-678,1964 8. Maier HC, Gould WJ: Agenesis of the lung with vascular compression of the tracheobronchial tree. J Pediatr 43:38-42,1953 9. Wheeler PC, Wolff LJ, Stevens EM: Pseudovascular ring resulting from right lung agenesis, normal aortic arch, and patent ductus arteriosus. AJR 98:365-368, 1966 10. McCormick TL, Kuhns LR: Tracheal compression by a normal aorta associated with right lung atresia. Pediatr Radio1 130:659-660, 1979 Il. Van Praagh R, van Praagh S, Vlad P, et al: Diagnosis of the anatomic types of congenital dextrocardia. Am J Cardiol 15:234247,1965 12. Kluth D: Atlas of oesophageal atresia. J Pediatr Surg 11:901919,1976 13. Kunze J, Kaufmann HJ: Greig cephalopolysyndactyly syndrome. Helv Paediatr Acta 40:489-495, 1945 14. Greig DM: Hypertelorism: A hithero undifferentiated congenital crania-facial deformity. Edinburgh Med J 31:560-593,1924

15. Berdon WE, Baker DH, Bordiuk J, et al: Innominate artery compression of the trachea in infants with strldor and apnoe. Radiology 92:272-278, 1969 16. Dohlemann C, Mantel K, Dusch T, et al: Trachealkompression durch den Truncus brachiozephalicus. Angiografische Beurteilung. Herz 3:127, 1978 17. Streeter GL: Developmental horizons in human embryos. Description of age groups XV, XVI, XVII and XVIII. Washington, DC, Carnegie Institute, Pub1 575, Contributions Embryo]. 32:133, 1948 18. Van Praagh R, van Praagh St, Vled P, et al: Anatomic types of congenital dextrocardia. Diagnostic and embryologic implications. Am J Cardiol 13:510-531, 1964 19. Reid L: The lung: Its growth and remolding in health and disease. AJR 128:777-788, 1977 20. Benson JE, Olsen MM, Fletcher BD: A spectrum of bronchopulmonary anomalies associated with tracheooesophageal malformation. Pediatr Radio1 15:377-380, 1985 21. Booth JB, Barry CL: Unilateral pulmonary agenesis. Arch Dis Child 42:361, 1967 22. Brereton RJ, Rickwood AMK: Esophageal atresia with pulmonary agenesis. J Pediatr Surg 18:618-620, 1983 23. Warner FS, McGraw TC, Peterson HG, et al: Lung ectopia and agenesis with heart dextrorotation. Am J Dis Child 101:514518,196l 24. Golladay ES, Seibert J: Agenesis of the right lung with tracheooesophageal fistula and tracheal agenesis. Z Kinderchir 30:3 15-320, 1980 25. Toyama WM: Esophageal atresia and tracheoesophageal fistula in association with bronchial and pulmonary anomalies. J Pediatr Surg 7:302-307,1972