Unilateral Lung Agenesis Research Articles

Unilateral lung agenesis – rare congenital malformation diagnosed in infants

Pulmonary agenesis is a congenital anomaly defined by the complete absence of lung parenchyma, as well as bronchial and vascular structures. Right lung agenesis is less frequent compared to left lung agenesis, and usually has a worse prognosis being associated with other congenital anomalies. The study focuses on the clinical case of a child with left lung agenesis.

Outcomes of slide tracheoplasty for congenital tracheal stenosis in 80 children: A 22-year single-center experience

PurposeThe application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes. MethodPatients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records. ResultsEighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival. ConclusionAlthough STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention.

Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

BackgroundTotal anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare.Case presentationWe present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction.ConclusionWe report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

PREGNANCY MANAGEMENT AND OUTCOME IN A WOMAN WITH CONGENITAL UNILATERAL LUNG AGENESIS: A CASE REPORT

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Congenital unilateral lung agenesis is a rare condition characterized by the complete absence of a lung and the bronchovascular tree. It is often associated with cardiac and bronchopulmonary malformations, resulting in significant morbidity and mortality. Pregnancy, due to the physiologic changes during gestation and childbirth, poses additional risks to these patients. We report a case of a woman with congenital right lung agenesis (CRLA) whose successful pregnancy could offer guidance to clinicians in the interdisciplinary management of a high-risk condition (1). CASE PRESENTATION: A 26-year-old G1P0 was referred to our Maternal Fetal Medicine (MFM) clinic for her CRLA with resultant dextrocardia and left lung hyperinflation. She was asymptomatic and was able to tolerate daily activities without concerns. Pre-conceptional imaging studies showed results consistent with her medical history.There were no obstetric concerns with normal prenatal labs and normal fetal anatomy ultrasound. She was recommended to undergo cardiopulmonary evaluation and follow up with the relevant specialists.The patient remained clinically stable throughout the gestation. Delivery and postpartum planning was done in the early third trimester at a multidisciplinary conference with the MFM, Anesthesia, Cardiology, Pulmonology, Obstetrics, and Critical Care teams.The patient was electively induced at 39 weeks resulting in an uncomplicated vaginal delivery. Careful monitoring of the patient's hemodynamic status was performed during the intra- and postpartum periods due to the concern of volume overload from intravenous fluid infusions during and autotransfusion following delivery. Her postpartum course was uncomplicated. DISCUSSION: Several cardiopulmonary physiological changes occur in pregnancy to accommodate the growing fetus. An increase in cardiac output with a decrease in pulmonary and systemic vascular resistance leads to an increase in pre-load and increased pulmonary capillary permeability. The risk of pulmonary edema is highest in the second stage of labor due to the autotransfusion into the systemic circulation and the relief of pressure on the inferior vena cava (2). During pregnancy, there is a 40% increased oxygen requirement by the growing fetus, placenta, and maternal organs with a decreased reserve secondary to the increased consumption and decreased functional residual capacity. These changes are often the source of significant morbidity and mortality in women with compromised systems (3). For these reasons, a multidisciplinary care approach was of utmost importance for this patient. CONCLUSIONS: We advocate for the utility of a multidisciplinary approach with frequent follow-up and testing with each specialty to ensure the best care for a successful pregnancy outcome in this patient with CRLA. REFERENCE #1: Wert S. E. (2016). Normal and Abnormal Structural Development of the Lung. In Polin R. A., Abman S. H., Rowitch D. H., Benitz W. E., & Fox W. W., Fetal and Neonatal Physiology (pp. 627-641). Elsevier. https://doi.org/10.1016/B978-0-323-35214-7.00061-5 REFERENCE #2: Silversides C. K. & Colman J. M. (2007). Physiological changes in pregnancy. Oakley, C. & Warnes C. A. (2007). Heart Disease in Pregnancy (pp. 6-16). Blackwell Publishing Limited. REFERENCE #3: Gabbe, S. G., Niebyl, J. R., Simpson, J. L., Landon, M. B., Galan, H. L., Jauniaux, E. R., ... & Grobman, W. A. (2016). Obstetrics: normal and problem pregnancies e-book. Elsevier Health Sciences. DISCLOSURES: No relevant relationships by Kajal Angras, source=Web Response No relevant relationships by Kelly Ca, source=Web Response No relevant relationships by Cherise Hatch, source=Web Response No relevant relationships by VANI MOVVA, source=Web Response

Late presentation of unilateral lung agenesis in adulthood

BackgroundPulmonary agenesis is a rare congenital anomaly with a reported prevalence of about 1 in 100,000 births. It may be bilateral or unilateral. Among the unilateral form, left lung agenesis is more common (70%); however, it is the right lung agenesis which carries a dismal prognosis due to the frequent association with a gamut of other congenital anomalies and greater degree of mediastinal shift leading to tracheo-bronchial and vascular distortion. The patients of unilateral pulmonary agenesis usually present in infancy or early childhood. Presentation in late adulthood as seen in our patient is rare. We present a case of left pulmonary agenesis that was diagnosed in 4th decade of life.Case presentationA 36-year-old male presented with gradually progressive exertional dyspnea of 1 month duration. Clinical examination revealed tachycardia and tachypnea. Chest radiograph showed opaque left hemithorax with ipsilateral mediastinal shift. Computed tomography clinched the diagnosis by demonstrating absence of left main bronchus, lung and left pulmonary artery with shift of heart, and great mediastinal vessels into left hemithorax. The patient was managed conservatively and discharged with attachment to out-patient department for regular follow-up.ConclusionPresentation of unilateral lung agenesis in late adulthood, as seen in the present case is extremely rare. This case report highlights that, a rare condition like unilateral pulmonary agenesis, should be considered in the list of differentials in an adult presenting with opaque hemithorax with ipsilateral mediastinal shift on radiography.

Unilateral Lung Agenesis in an infant with prenatal diagnosis of Congenital Lobar Emphysema

Abstract Infants in respiratory distress presenting with congenital lobar emphysema (CLE) are surgical emergencies. However, several other congenital lung lesions (CLL) have similar prenatal imaging, highlighting the importance of early postnatal evaluation. We present a case of a newborn transferred for respiratory distress. Prenatal imaging suspected a CLE in the left upper lobe with concern for decompensation in the early postnatal period. Upon arrival, the patient demonstrated reasonable cardio-respiratory stability after initial apnea and cyanosis; due to prenatal concerns, we proceeded to CT imaging, which demonstrated congenital absence of the right pulmonary artery, agenesis of the right lung, and a larger left lung herniating into the right side. Surgical intervention was therefore not required. This case emphasizes the importance of postnatal imaging in the CLL workup. Providing “pathological” diagnoses to CLL can affect postnatal counseling and influence surgical decision-making. Here, early postnatal imaging clarified the diagnosis and prevented both unnecessary surgery and significant morbidity. The patient's clinical status should determine the urgency of CLL investigation. Stable patients may be followed as outpatients but should be assessed within 1–2 weeks after birth. Symptomatic patients should be transferred to tertiary care centers with pediatric surgical expertise for further investigation and possible intervention.

Unilateral lung agenesis: a case report and literature review

Pulmonary agenesis is an extremely rare malformation of lung with an estimated prevalence of only 34 per million live births. It is even more uncommon in females and on the right side. We report a case of this rare entity.

Complex surgical treatment of congenital tracheal stenosis with associated unilateral lung agenesis

Abstract Background The combination of congenital tracheal stenosis (CTS) and unilateral pulmonary agenesis is rare and fatal. Especially, right lung agenesis is associated with dextrocardia and often causes tracheal alignment disorder. The mortality is estimated with 12.5–65%. Slide Tracheoplasty (ST) has been improved the prognosis, however, the postoperative course of CTS with single lung remains unclear. Here, we describe the postoperative challenging situation with bronchial intervention and other devices. Case presentation A 9-month-old girl with CTS, right lung agenesis, dextrocardia, and bilateral superior vena cava underwent ST anterior to the aortic arch. On postoperative day 50, tracheostomy was performed for long-term airway pressure support. Six months after the ST, she showed severe bronchomalacia at the bifurcation of the lobar bronchi, which was sandwiched between the descending aorta and left pulmonary artery. Direct bronchopexy and mobilization of left pulmonary artery resolved the severe bronchomalacia. Additionally, the kinked trachea caused a tracheal ulcer and frequent accidental decannulations. The ulcer was made above the aortic arch by contact with the tracheal cannula. The customized Montgomery T-tube solved both problems and enabled the stable management for airway. Her condition eventually improved, and she was discharged home after 14 months of hospitalization. Conclusion This case presented a very rare and critical condition. This is the first report to address the severe condition after ST anterior to the aortic arch. Surgical interventions or other devices for treatment are thought to be modified according to each condition.

Congenital Lung Agenesis: Incidence and Outcome in the North of England.

Unilateral lung agenesis is an uncommon congenital abnormality, with a lack of reported accurate incidence estimates. Prognosis is also uncertain, with older literature reporting poor outcomes. The North of England register of congenital anomalies (Northern Congenital Abnormality Survey) records cases of congenital anomalies to mothers' resident in the region. We used the register to identify all patients with congenital lung agenesis born between 2004 and 2013 to calculate an accurate incidence estimate and report clinical outcomes with contemporary management. Four patients with congenital lung agenesis were born during the study period, giving an estimated incidence in the North of England of 1.22 per 100,000 live births (95% confidence interval, 0.33-3.11). Two patients had associated congenital heart disease requiring corrective surgery, and one had musculoskeletal anomalies. All four patients are alive and well without a regular oxygen requirement. Contrary to previous reports, the medium term outcomes in our patients have been good, even when lung agenesis is associated with other congenital anomalies. Long-term prognosis with modern management remains unknown, and the potential for the development of pulmonary hypertension remains a concern. Birth Defects Research 109:857-859, 2017. © 2017 Wiley Periodicals, Inc.

Primary repair of tetralogy of Fallot combined with unilateral left lung agenesis with crossed-ectopic right lower lobe

Unilateral lung agenesis is a rare congenital anomaly often combined with congenital malformations of different systems. A case of tetralogy of Fallot repair with unilateral left lung agenesis via median sternotomy is described for the first time. In this case, the choice of surgical approach to the heart could be affected by the fears of not getting optimal surgical exposition via median sternotomy due to severe displacement of the chest organs. The case demonstrates the median sternotomy as an optimal surgical approach even in high degrees of organ displacement caused by a combination of left pulmonary agenesis and cardiac lesions.

Persistent Cloaca associated with Unilateral Lung Agenesis- A Rare Presentation

We report a case of full term female child having persistent cloaca who was diagnosed to have right lung agenesis on investigations.

Unilateral Agenesis of Lung, Kidney with Cardiac and Vertebral Defects a Rare Association

Unilateral lung agenesis initially misdiagnosed as unilateral massive pleural effusion with collapse of lung, and after several investigations, including ultrasonography or CT scan of thorax, fibre-optic bronchoscopy or bronchography, a final diagnosis of unilateral absence of lung is made. An anomaly scan may also reveal associated renal, cardiac and vertebral defects. Here we report a 7 months old female who presented with respiratory distress since birth and after a thorough investigation, she was found to have this rare association of pulmonary-renal-cardiacvertebral defect. DOI: http://dx.doi.org/10.3126/jnps.v34i1.8898 J Nepal Paediatr Soc 2014;34(1):77-79

Prenatal diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition

BackgroundFetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening.MethodsA total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings.ResultsThe left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis.ConclusionsThe systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.

A New Morphologic Classification of Congenital Tracheobronchial Stenosis

Congenital tracheal stenoses are rare and life-threatening anomalies, associated with considerable variation in both morphology and prognosis. They have been classified previously according to the length of the stenosis or the severity of the symptoms, but not according to bronchial involvement. Data from patients who underwent slide tracheoplasty for long-segment (>50%) congenital tracheal stenosis were collected. We identified four different types of bronchial arborization (normal, n=52; tracheal right upper lobe bronchus, n=10; carina with "trifurcation," n=14; and unilateral bronchial and lung agenesis, n=8). Each type included congenital tracheal (above the carina) or tracheobronchial (extending below the carina) stenosis. Eighty-four children were enrolled in the study. Preoperative ventilation was necessary in 44 patients (52.4%; 75% in patients with a single lung), and preoperative extracorporeal membrane oxygenation was needed in 10 patients (11.9%). Preoperative tracheostomy was present in 3 patients initially treated elsewhere (3.5%), and a left pulmonary artery sling was performed in 44% (37 of 84). The overall mortality was 13% (11 of 84), 7.9% in patients with tracheal stenosis and 28.6% with tracheobronchial stenosis. No deaths occurred in patients with right upper lobe bronchus anatomy. Endoscopic procedures after slide tracheoplasty were required in 34 patients (40.4%). Stents were placed in 18 patients (21.4%), with a higher incidence in those with bronchial trifurcation (42.8%, 6 of 12). This classification appears useful for the morphologic characterization of congenital airway stenosis and could be the benchmark for future prospective studies on the outcome of these patients.

First description of unilateral lung agenesis and anomalous pulmonary venous connection in a newborn with Goldenhar syndrome

We present a unique case of unilateral lung agenesis and anomalous pulmonary venous return in a newborn with Goldenhar syndrome.

Balloon dilatation of tracheal restenosis in a patient with unilateral lung agenesis – an anesthetic challenge

Balloon dilatation of tracheal restenosis in a patient with unilateral lung agenesis – an anesthetic challenge

Aphallia, Lung Agenesis and Multiple Defects of Blastogenesis

We report the association of aphallia, with imperforate anus, bilateral renal dysplasia and complete right lung agenesis in a fetus born of non-consanguineous parents. No maternal diabetes was present. The multiple mesodermal anomalies present in this male fetus, with a severe form of Uro-Rectal-Septum Malformation Sequence associated with unilateral lung agenesis, and rib segmentation anomaly is consistent with an extended defect of blastogenesis, with apparent common timing of the malformations around the 5th to 7th weeks of gestation.

P18.11: Unilateral pulmonary agenesis: prenatal imaging and outcome in three cases

Objectives: To review the imaging findings and outcome of unilateral pulmonary agenesis in the fetus. Methods: Retrospective review at a single academic center. Results: Case 1: Presented at 29 weeks with severe IUGR, occipital encephalocele, left renal agenesis, arthrogryposis, small stomach and left lung agenesis. The infant died at 30 hrs of age, no intervention occured. Case 2: Presented at 18 weeks with unilateral left lung agenesis, small right lung, right renal agenesis, interrupted aortic arch, facial dysmorphism. The infant lived about 30 mins after resuscitation attempts ceased. Case 3: Presented at 20 weeks with left pulmonary agenesis, right renal agenesis, club foot, caudal regression sequence and other vertebral segmentation anomalies as well as a single umbilical artery. The infant survived; she had emergency repair of aortic coarctation and is alive at age 3. All had normal karyotype. Conclusions: Pulmonary agenesis is described as an isolated finding in the pediatric literature however 50% of live born infants have features of the VACTERL association. Our series suggests that the prognosis is guarded when the diagnosis is made prenatally. All three had significant associated abnormalities, some of which were not appreciated on fetal US or MRI. Two infants died in the neonatal period and the third had a difficult time during repair of an unanticipated coarctation of the aorta.

Letters to the Editor

11 December 2008 Dear Editor, UNILATERAL LUNG AGENESIS: COINCIDENCE OR ASSOCIATION WITH MATERNAL VALPROATE THERAPY? Management of epilepsy in pregnant women is a complex issue as use of antiepileptic medications increase the risk of congenital malformation in the foetus.1 The antiepileptic drug, sodium valproate, is a known teratogen with a constellation of recognised facial features and congenital malformations described in foetal valproate syndrome.2 Infants of mothers receiving valproate more than 1000 mg are at increased risk of congenital malformation, particularly neural tube defects.1 Exposure of foetus to valproate uncommonly results in pulmonary anomalies. Bilateral pulmonary hypoplasia with immature histology was reported in a series of three cases as a possible teratogenic effect of valproate.3 Other reported but rare pulmonary malformations in foetal valproate syndrome include tracheomalacia, abnormal lobulation of right lung, severe laryngeal hypoplasia and oligaemic right lung.4 Association of unilateral lung hypoplasia with maternal exposure to a combination of phenytoin and carbamazepine has been reported.1 A male infant was born at 38 weeks gestation with a birth weight of 2915 grams. The mother was a 32-year-old multiparous lady. During pregnancy, she continued to take sodium valproate, 1000 mg/day, to control grand mal epilepsy. This pregnancy was complicated by the detection of a possible left congenital diaphragmatic hernia on antenatal ultrasound at 18 weeks gestation. An amniocentesis performed at 19 weeks gestation demonstrated a normal male karyotpye. A foetal magnetic resonance imaging scan performed at 21 weeks gestation suggested hypoplasia of the left lung with a normal diaphragm. The infant delivered normally with Apgar scores of 6 and 6 at 1 and 5 min, respectively. There were no dysmorphic features of foetal valproate syndrome. He was managed in a neonatal intensive care unit with continuous positive airway pressure (CPAP) and received a single dose of surfactant. He was treated with phenobarbitone to manage symptoms of valproate withdrawal at approximately 24 h of age. Chest X-rays demonstrated dense opacification with loss of lung volume in the left hemithorax. The computed tomography scan showed absence of lung, bronchial tree, pulmonary artery and veins on the left side (Fig. 1). Echocardiogram showed a structurally normal heart with absence of the left pulmonary artery and left pulmonary veins. He was treated with nasal mask CPAP support for 7 weeks and was weaned successfully to room air at the age of 8 weeks. A polysomnography study showed no oxygen desaturation or hypercapnia while ventilating in room air. He was discharged home with paediatric respiratory follow-up. Computed tomography scan of chest showing absence of carina and left lung. Pulmonary agenesis is complete absence of carina, bronchial tree and lung tissue, and the trachea continues as main bronchus. In pulmonary aplasia, carina and rudimentary main bronchus are present, but lung tissue is absent.5 Pulmonary agenesis is a rare malformation. The prevalence of agenesis of lung was reported to be 0.0034% among hospital admissions.6 The aetiology of lung agenesis is not certain, but possible genetic predisposition has been described.6 Our literature review did not find any other case of unilateral lung agenesis in association with maternal valproate intake. Given the rarity of pulmonary agenesis, it is possible that the foetal exposure to valproate in this report is a chance association. However, this observation will add to previously known pulmonary pathologies seen in association with maternal valproate use and could be relevant from a prenatal counselling point of view.

Tracheal Reconstruction in Children With Unilateral Lung Agenesis or Severe Hypoplasia

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. 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. 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). 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.