Ann Thorac Surg 2009;88:1345-1348. doi:10.1016/j.athoracsur.2009.02.095
© 2009 The Society of Thoracic Surgeons
Case Reports
Aortopexy for Tracheomalacia With Dextrocardia, Pulmonary Artery Sling, and Congenital Tracheal Stenosis
Kwang-Jong Lee, MDa,c,*,
Shoichiro Kamagata, MDa,
Seiichi Hirobe, MDa,
Miki Toma, MDa,
Takuo Furukawa, MDb,
Naoki Fukushima, MDb,
Yukihiro Inomata, MDc
a Department of Surgery, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan
b Department of Pediatrics, Sapporo City General Hospital, Sapporo, Japan
c Department of Transplantation/Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
Accepted for publication February 27, 2009.
* Address correspondence to Dr Lee, 1-1-1, Honjo, Kumamoto City, Kumamoto, 860-8556, Japan (Email: kwangjong{at}xrh.biglobe.ne.jp).
 |
Abstract
|
|---|
We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this surgery was different from the usual aortopexy for tracheomalacia, which lifts the tracheal wall with the aorta. The three-dimensional evaluation considering the patient's associated malformations led to a successful result.
|
Introduction
|
|---|
Pulmonary artery (PA) sling is a rare congenital vascular anomaly in which the left PA arises from the right PA and encircles the distal trachea. It is often associated with congenital tracheal stenosis (CTS) [1, 2]. Many cases are detected by respiratory symptoms, such as wheeze and cyanosis in the neonatal period and infancy. In some cases a "dying spell" occurs, which has a serious effect on intellectual development and overall prognosis. Although early surgical treatment is necessary to avoid the hazard of choking, the treatment policy is controversial due because of poor therapeutic results. We experienced a case with coexisting CTS, PA sling, and dextrocardia caused by right lung hypoplasia. It was important to three-dimensionally establish the anatomical position of the surrounding large blood vessels prior to treatment.
An infant boy was born at gestational age 38 weeks (weight, 2,128 g). Transverse colostomy was performed on the first day because of imperforate anus (rectobulbar urethral fistula type). On day 47 of life, he had an episode of cyanosis while crying. On day 53 of life, his arterial oxygen saturation was detected at below 40% while he was crying, and he was intubated with a 3-mm diameter tracheal tube. After a chest computed tomographic scan showed PA sling and CTS, he was transferred to our hospital under intubation on day 81 of life. A chest roentgenogram showed that the right hemithorax was opaque, with a shift of the mediastinum and the heart to the right (Fig 1A). A chest computed tomographic scan revealed a 2.5 cm segmental tracheal stenosis (40% of tracheal length) with a diameter of 2 mm (Fig 1B). Echocardiography showed PA sling, double superior vena cava, and no intracardiac anomaly. Bronchoscopy showed tracheal stenosis with complete circular cartilaginous rings at the level of the tracheal bronchus that supplied the azygous lobe to the low tracheal bifurcation (pseudocarina). The bridging bronchus that arose from the left main bronchus supplied the right upper, middle, and lower lobes. Left PA reimplantation and slide tracheoplasty were concurrently performed under cardiopulmonary bypass on day 92 of life. Because effective ventilation was obtained with a 3.5-mm diameter tracheal tube, an extracorporeal membrane oxygenator was not required after surgery.
View larger version (74K):
[in this window]
[in a new window]
|
Fig 1. (A) A right hemithorax was shown by a chest roentgenogram at admission. (B) A computed tomographic chest scan revealed segmental tracheal stenosis, low tracheal bifurcation, and bridging bronchus. The azygous lobe was found on the inner field of the right upper lobe.
|
|
He was extubated on postoperative day 17. He suffered respiratory failure after crying for 4 hours after extubation. Reintubation was performed smoothly and easily. Echocardiography showed no evidence of anastomotic stricture of the left PA. However, it was possible to ventilate adequately under sedation, and he fainted when crying, even as he was intubated. Tracheomalacia was strongly suspected, because it was possible to ventilate immediately after the fainting spells. Tracheobronchoscopy revealed tracheobronchomalacia over both main bronchi. During examination, the tracheal bronchus was especially compressed and collapsed when breathing. After thorough review of the chest computed tomographic scan, we considered that the aortic arch that crosses orthogonally in front of the trachea due to dextrocardia was most strongly affected (Fig 2A). Three months after slide tracheoplasty, an aortopexy was performed. Three stitch mattress sutures of the ascending aorta were performed and fixed to the third right rib, while observing with tracheobronchoscopy that the anterior tracheal wall was lifted (Figs 3A and 3B). A postoperative computed tomographic chest scan confirmed that oblique displacement of the aortic arch was obtained (Fig 2B). He was extubated successfully 20 days after aortopexy and left the hospital on postoperative day 41.
View larger version (84K):
[in this window]
[in a new window]
|
Fig 2. (A) The aortic arch was located orthogonally in front of the trachea, which compressed the trachea and tracheal tube. (B) The aortic arch was obliquely displaced, and the trachea was spread after aortopexy.
|
|
View larger version (72K):
[in this window]
[in a new window]
|
Fig 3. (A) The trachea with granulation was especially compressed at the level of the tracheal bronchus. (B) The anterior tracheal wall was lifted after aortopexy.
|
|
|
Comment
|
|---|
Pulmonary artery sling is a rare disease that is commonly associated with CTS. Treatment of either condition (sometimes both) is performed after evaluation, which is more life-threatening among PA sling and CTS [3]. Pulmonary artery sling associated with CTS is frequently associated with other multiple anomalies, including cardiac malformations; therefore, it is generally difficult to carry out a full preoperative assessment of serious cases. Since 1998, when slide tracheoplasty was introduced at our institution [4], we have concurrently performed left PA reimplantation and slide tracheoplasty in all 5 patients with PA sling among 13 cases of CTS. Four of the 5 patients survived, and 1 with associated congenital heart disease died from pulmonary hypertension after surgery. In the context of associated malformation, this patient did not have severe congenital anomalies apart from imperforate anus and double superior vena cava.
It is not unusual for CTS to be associated with tracheomalacia preoperatively or postoperatively [5]. The causes of tracheomalacia are generally divided into intrinsic abnormality and extrinsic compression. In the present case, full investigation was performed after failure of extubation, and revealed that tracheomalacia was mainly caused by vascular compression. The treatment for tracheomalacia caused by large blood vessels is generally selected aortopexy or an external stent. In the present case, aortopexy that aimed to lift the trachea together with the aorta was not expected to be effective, because there was no adhesion shortly after left PA reimplantation and slide tracheoplasty. On the other hand, external stenting was not selected in consideration that a stent material continuously contacts the large blood vessels. The unique feature of the present case was having right lung hypoplasia coexistent with CTS and PA sling. The frequency of association of right lung hypoplasia with PA sling is 8% to 14% [3, 6]. In the context of association with right lung hypoplasia and tracheomalacia, it has been reported that 3 patients with right lung hypoplasia and severe respiratory disorder who did not have coexistent PA sling or CTS were all extubated soon after aortopexy displaced the aortic arch that was orthogonally in front of the trachea [7]. Eventually aortopexy was chosen in our case; the purpose of this choice was displacement of the aortic arch, and not lifting the tracheal wall together with the aorta.
We performed a two-stage operation. It has been reported that aortopexy and left PA reimplantation were performed simultaneously in a case of PA sling with severe tracheomalacia [8]. Although it may be technically possible to perform a one-stage operation of left PA reimplantation, slide tracheoplasty, and aortopexy, the preoperative evaluation must be difficult.
|
References
|
|---|
- Valletta EA, Pregarz M, Bergamo-Andreis IA, Boner AL. Tracheoesophageal compression due to congenital vascular anomalies (vascular rings) Pediatr Pulmonol 1997;24:93-105.[Medline]
- Berdon WE, Baker DH, Wung JT, et al. Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex Radiology 1984;152:57-64.[Abstract/Free Full Text]
- Fiore AC, Brown JW, Weber TR, Turrentine MW. Surgical treatment of pulmonary artery sling and tracheal stenosis Ann Thorac Surg 2005;79:38-46.[Abstract/Free Full Text]
- Toma M, Kamagata S, Hirobe S, et al. Slide tracheoplasty for congenital tracheal stenosis-perioperative management and problems [Japanese] Nihon Shounigeka Gakkai Zasshi 2007;43:136-141.
- Wright CD, Graham BB, Grillo HC, Wain JC, Mathisen DJ. Pediatric tracheal surgery Ann Thorac Surg 2002;74:308-314.[Abstract/Free Full Text]
- Backer CL, Mavroudis C, Dunham ME, Holinger LD. Pulmonary artery sling: results with median sternotomy, cardiopulmonary bypass, and reimplantation Ann Thorac Surg 1999;67:1738-1745.[Abstract/Free Full Text]
- Döhlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: airway obstruction and its release by aortopexy J Pediatr Surg 1990;25:290-293.[Medline]
- Campbell DN, Lilly JR, Heiser JC, Clarke DR. The surgery of pulmonary artery "sling." J Pediatr Surg 1983;18:855-856.[Medline]
Top
Abstract
Introduction
