Original article
Pediatric cardiac
Tracheal Reconstruction in Children With Unilateral Lung Agenesis or Severe Hypoplasia

Presented at the Forty-fifth Annual Meeting of the Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.
https://doi.org/10.1016/j.athoracsur.2009.04.111Get rights and content

Background

Infants with congenital tracheal stenosis may also have unilateral lung agenesis or severe lung hypoplasia. The purpose of this review is to evaluate our results with these patients and compare their presentations and outcomes to those of tracheal stenosis patients with two lungs.

Methods

Our database was queried for patients undergoing tracheal stenosis repair since 1982. Patients were divided into two groups based on pulmonary anatomy of single lung (SL = unilateral lung agenesis or severe hypoplasia) or two lungs (BL = bilateral lungs) and analyzed to compare presentation and outcomes.

Results

From 1982 to 2008, 71 patients had tracheal stenosis repair. Bilateral lungs were present in 60 patients; 9 patients had an absent (4) or severely hypoplastic (5) right lung, and 2 patients had an absent left lung (SL = 11). Age at repair was similar between groups; median age 0.42 years in the SL group (mean 0.80 ± 1.0 years) versus 0.37 years in the BL group (mean 0.91 ± 2.1 years, p = not significant [ns]). In the SL group 8 of 11 (73%) were intubated preoperatively versus 15 of 60 (25%) in the BL group (p = 0.004). In the SL group 4 of 11 (36%) patients had pulmonary artery sling versus 20 of 60 (33%) of BL patients (p = ns). In the SL group 2 of 11 (18%) versus 14 of 60 (23%) in the BL group had intracardiac anomalies requiring simultaneous repair (p = ns). Procedures included pericardial tracheoplasty (2 vs 26), tracheal autograft (4 vs 16), slide tracheoplasty (3 vs 8), and tracheal resection (2 vs 10). Overall mortality (operative and late) was 2 of 11 (18%) SL versus 10 of 60 (17%) BL (p = ns). Median postoperative length of stay was 43 days SL (mean 48.6 ± 40) versus 30 days BL (mean 52.2 ± 65) (p = ns). The incidence of postoperative tracheostomy (SL group) was 0 of 3 for slide tracheoplasty and 5 of 8 for the other techniques (p = 0.12).

Conclusions

Despite the increased severity of pathology and increased critical presentation of tracheal stenosis patients with unilateral lung agenesis or severe hypoplasia, outcome measures of mortality and length of stay were similar to patients with two lungs. The incidence of associated pulmonary artery sling (1 of 3) and intracardiac anomalies (1 of 4) was similar. Unilateral lung agenesis or severe hypoplasia should not preclude operative repair of tracheal stenosis. Slide tracheoplasty is our current procedure of choice for these infants.

Section snippets

Material and Methods

This study was approved by the Institutional Review Board (IRB) of Children's Memorial Hospital as a retrospective chart analysis; requirement for consent was waived. Patients with tracheal stenosis were divided into two groups based on their pulmonary anatomy of one lung (SL = unilateral lung agenesis or severe hypoplasia) or two lungs (BL = bilateral lungs).

The two groups were then analyzed for differences in presentation and outcomes. Factors analyzed included preoperative intubation,

Results

Seventy-one patients underwent repair of tracheal stenosis secondary to complete congenital tracheal rings at Children's Memorial Hospital between 1982 and 2008. The SL group had 11 patients; 9 of these patients had absence of the right lung (4) or severe hypoplasia of the right lung (5) (Fig 1). The left lung was absent in 2 patients (Fig 2). The clinical characteristics of these patients are summarized in Table 1. Chest X-ray, axial computed tomographic (CT) image, and three-dimensional (3D)

Comment

In our overall series of 71 patients with tracheal stenosis secondary to complete tracheal rings, 15% had unilateral lung agenesis or severe lung hypoplasia. The purpose of this review was to compare the group of patients with tracheal stenosis and “one lung” with those who had tracheal stenosis and two lungs. We attempted to identify any trends with associated pathology and to evaluate and compare the outcomes of these two groups. One of the earliest reports of unilateral lung agenesis was

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    In this series, the nature of lung abnormality was predominantly hypoplasia (70%) if right-sided and agenesis (83%) if left-sided. Similar to previous studies, there was a high association of other congenital abnormalities (68.8%) in our study illustrating the complexity of the patients in the overall management [4,14]. In comparison to other studies, the requirement of preoperative ventilation (93.8%) was very high in our cohort (73% in the study published by Backer and colleagues in 2009 and 56% in the study published by DeMarcantonio and colleagues in 2016) [4,14] Although this may be due to a need for some of the patients to be transported safely over a long distance from other hospitals or due to an evolution of management over time, it is quite possible that it reflects the severity of airway obstruction.

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