scholarly journals A Delayed Anatomic Diagnosis and Management Challenge in an Initially Asymptomatic Infant With Type II Pulmonary Artery Sling: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Xiaoqing Shi ◽  
Chuan Wang ◽  
Yimin Hua ◽  
Xiaoliang Liu ◽  
Hongyu Duan
Keyword(s):  
Pulmonary Artery ◽  
Tracheal Stenosis ◽  
Clinical Symptoms ◽  
Left Pulmonary Artery ◽  
Bronchial Tree ◽  
Middle Lobe ◽  
Pulmonary Artery Sling ◽  
Lobe Bronchus ◽  
Recurrent Wheezing ◽  
Poor Outcomes

Pulmonary artery sling (PAS) is a rare but fatal malformation. Patients with PAS tend to develop obstructive symptoms in few weeks of life. Conversely, some patients may be otherwise mild or asymptomatic in their early life. Currently, no consensus on the intervention timing and treatment strategy for asymptomatic and mild cases has been reached. Moreover, the extent of tracheal stenosis is another determining factor for the choice of intervention timing since clinical symptoms might not correspond well with the degree of stenosis. Lack of comprehensive assessment of entire airways confer underestimation of disease severity and in turn improper choice of treatment regimens and poor outcomes. Herein, we described an infantile case of PAS, who was scheduled initially for periodic outpatient follow-up on account of the absence of symptoms and inadequate imaging assessment at diagnosis. The patient developed recurrent wheezing and progressive respiratory distress at 7 months of age. After left pulmonary artery (LPA) reimplantation without tracheal intervention, bronchoscopy was performed due to failure to wean from mechanical ventilation, which demonstrated complete tracheal cartilage rings, a long segment tracheal stenosis, a low tracheal bifurcation at T6, and the absence of a separate right middle lobe bronchus. The patient was finally diagnosed with type IIb PAS and extubated successfully following conservative treatment. Miserably, neurological sequelae were devastating, leading to poor outcomes. Comprehensive airway evaluation using bronchoscopy is substantial to early identification of all components responsible for airway compromise in PAS anatomic subtypes. Considering severe concomitant maldevelopment of the bronchial tree in children with type IIb PAS, early and complete correction by surgery might decrease perioperative morbidities and mortalities of these patients.

Tracheostenosis and Bronchial Abnormalities Associated with Pulmonary Artery Sling

1976 ◽  
Vol 85 (5) ◽  
pp. 582-590 ◽  
Author(s):  
Seymour R. Cohen ◽  
Benjamin H. Landing
Keyword(s):  
Pulmonary Artery ◽  
Tracheal Stenosis ◽  
Main Bronchus ◽  
Left Pulmonary Artery ◽  
Surgical Correction ◽  
Left Main ◽  
Pulmonary Artery Sling ◽  
Lobe Bronchus ◽  
Normal Number ◽  
The Right

Three patients with aberrant left pulmonary artery (sling artery) are reported to illustrate associated tracheobronchial abnormalities. The clinical picture was that of severe episodic or progressive respiratory distress without dysphagia in early infancy. Striking narrowing of the trachea by complete “ring cartilages,” unrelated to compression by the abnormal pulmonary artery, was present. Tracheotomy and intubation failed to relieve the obstruction. In one patient the bronchi and bronchial segmentation pattern were normal, but in the other two patients, bronchial abnormalities included wide irregular cartilages in the main bronchi, forming more complete rings than is normal. In both, the right main bronchus was relatively longer than normal, and the bronchus intermedius showed poor cartilage ring formation and was relatively short compared to the main bronchus. This discrepancy did not appear to be due to distal displacement of the right upper lobe bronchus. In these two patients the left main bronchus was relatively short and wide with reduced number of cartilage rings (five-six vs usual normal number of nine), so that the right and left main bronchi were almost of equal length. However, the branch patterns of the lobar bronchi were within normal range. Bronchoscopy seems essential to demonstrate such ring tracheal cartilages (absence of the pars membranacea of the trachea), which when found should alert the examiner to the possible presence of an abnormal left pulmonary artery. Since surgical correction of tracheal stenosis of this type is not possible at present, the ultimate prognosis of patients with sling artery may depend more on the severity of the tracheal anomaly rather than on success of surgical correction of the abnormal left pulmonary arterial course. Although aberrant (sling) left pulmonary artery can occur in patients without respiratory tract symptoms, tracheal stenosis due to ring tracheal cartilages occurs in a significant fraction of patients with this arterial anomaly. Bronchoscopic study of the trachea, and possibly air or contrast bronchography of right and left main bronchi, can be of aid in diagnosis of this complex.

Single Stage Repair of Tetralogy of Fallot Associated With Left Pulmonary Artery Sling and Tracheal Stenosis

2013 ◽  
Vol 28 (5) ◽  
pp. 595-598 ◽  
Author(s):  
Ajeya Joshi ◽  
Saket Agarwal ◽  
Satish Kumar Aggarwal ◽  
Vishnu Datt ◽  
G. R. Sethi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Tracheal Stenosis ◽  
Left Pulmonary Artery ◽  
Single Stage ◽  
Pulmonary Artery Sling ◽  
Left Pulmonary Artery Sling

Aortotracheal Fistula after Slide Tracheoplasty in a Patient with Dextrocardia, Left Pulmonary Artery Sling and Tracheal Stenosis: A Case Report

2021 ◽  
Author(s):  
Yu-san Chien ◽  
Yen-Chun Chao ◽  
Kuo-Sheng Lee ◽  
Kung-hong Hsu
Keyword(s):  
Pulmonary Artery ◽  
Tracheal Stenosis ◽  
Circulatory Arrest ◽  
Aortic Surgery ◽  
Left Pulmonary Artery ◽  
Laser Vaporization ◽  
Recurrent Bleeding ◽  
Pulmonary Artery Sling ◽  
Slide Tracheoplasty ◽  
Left Pulmonary Artery Sling

Abstract Background: Aortotracheal fistula (ATF) is an uncommon and fatal complication of tracheal or aortic surgery, especially among pediatric patients. Case presentation: We reported a case in a 1-year-old boy with dextrocardia, left pulmonary artery sling and long segment tracheal stenosis. He received slide tracheoplasty at 9 months of age and had post-operative refractory granulation at distal trachea status post repeated balloon dilatation and laser vaporization. Episodes of hemoptysis occurred on post-operative day 81. Bronchoscopy revealed a pulsating pseudoaneurysm at lower trachea which ruptured during the procedure. Urgent surgical repair under cardiopulmonary bypass with deep hypothermic circulatory arrest was done. No recurrent bleeding or significant neurologic deficits noticed at a 4-month follow-up. Conclusion: Congenital anomaly that changes the spatial relationship between trachea and aorta could have contributed to formation of ATF. This warrant future attention when managing tracheal granulation with this not uncommon anatomy.

A Case of Pulmonary Artery Sling Anomaly with Tracheal Stenosis and Management of Difficult Airway

2020 ◽  
Author(s):  
Emine G. Torun ◽  
Mutlu U. Yazıcı ◽  
Ebru Azapağası ◽  
Utku A. Örün ◽  
Hasibe G. Çınar ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Difficult Airway ◽  
Tracheal Stenosis ◽  
Pediatric Intensive Care ◽  
Male Infant ◽  
Left Pulmonary Artery ◽  
High Morbidity ◽  
Pulmonary Artery Sling ◽  
Vascular Abnormality ◽  
Pulmonary Sling

AbstractPulmonary artery sling is a rare congenital vascular abnormality, where the left pulmonary artery originates from the right pulmonary artery, passes between trachea, and esophagus and reaching the left hilum. Cough, wheezing, and difficulty in feeding are three major symptoms. Untreated pulmonary sling carries high morbidity and mortality, most of which is due to the airway and other associated anomalies. Herein, we report a 40-day-old male infant who admitted to the pediatric intensive care unit with progressive respiratory distress and diagnosed with left pulmonary sling with tracheal stenosis. We discuss the diagnosis and management of pulmonary artery sling and present the successful use of laryngeal mask in difficult airway management.

Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling

2020 ◽  
Vol 55 (3) ◽  
pp. 747-753
Author(s):  
Naoya Fukushima ◽  
Naoki Shimojima ◽  
Makoto Ishitate ◽  
Tomoo Miyakawa ◽  
Seiichi Hirobe ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tracheal Stenosis ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Sling ◽  
Left Pulmonary Artery Sling ◽  
Structural Aspects

Pulmonary Artery Sling with Tracheal Stenosis Combined with A Large Ventricular Septal Defect in A Two-Month-Old Boy: A Case Report

2021 ◽  
Vol 24 (6) ◽  
pp. E1043-E1045
Author(s):  
Yi Zhang ◽  
Yong jun Qian
Keyword(s):  
Pulmonary Artery ◽  
Tracheal Stenosis ◽  
Respiratory Symptoms ◽  
Rare Case ◽  
Septal Defect ◽  
Left Pulmonary Artery ◽  
Pulmonary Artery Sling ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Cardiovascular Anomalies

Pulmonary artery sling (PAS) is a rare congenital cardiovascular abnormality. In typical PAS patients, the left pulmonary artery (LPA) arises from the right pulmonary artery and passes between the trachea and esophagus, which possibly causes tracheal stenosis and some respiratory symptoms. PAS typically associates with other cardiovascular anomalies, which may cause difficulties to the treatment of patients. This report described a rare case of a two-month-old boy with PAS, VSD, and tracheal stenosis simultaneously and underwent procedures without tracheoplasty.

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