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Combined Surgical and Endoscopic Approach for Ring-Sling Complex

Giulio Porcedda1, Alice Brambilla1, Silvia Favilli1

  • 1Department of Pediatric Cardiology, Anna Meyer Children Hospital, Florence, Italy.

The Thoracic and Cardiovascular Surgeon
|February 12, 2019
PubMed

Insights

Pulmonary artery sling (PAS) with tracheal stenosis (TS) in children is complex. Surgical repair shows an 18% late mortality but good LPA flow, emphasizing multidisciplinary care.

Area of Science:

  • Pediatric Surgery
  • Cardiovascular Surgery
  • Thoracic Surgery

Background:

  • Pulmonary artery sling (PAS) is a rare congenital anomaly often associated with long-segment tracheal stenosis (TS).
  • High mortality rates in operated children are linked to severe tracheal hypoplasia and congenital heart defects (CHDs).
  • This study focuses on the treatment and outcomes of pediatric patients with PAS and severe tracheobronchial involvement.

Purpose of the Study:

  • To report the treatment experience and follow-up of pediatric patients with PAS and severe tracheobronchial involvement.
  • To analyze the impact of associated CHDs and tracheal hypoplasia on patient outcomes.
  • To evaluate the role of respiratory endoscopy in managing these complex cases.

Main Methods:

  • Eleven children with PAS and congenital TS underwent surgical intervention between 2005 and 2017.
  • Diagnostic tools included echocardiography, computed tomography, and bronchoscopy.
  • Tracheal reconstruction involved slide tracheoplasty, with or without cartilage grafts, and LPA reimplantation.

Main Results:

  • A late mortality rate of 18% was observed over a mean follow-up of 30 months; no early deaths occurred.
  • All patients demonstrated good flow through the LPA post-surgery.
  • Ten children required multiple operative bronchoscopies for stent management, granuloma treatment, and dilatation.

Conclusions:

  • Severe tracheobronchial stenosis and CHDs significantly influenced hospitalization and the need for repeated endoscopic procedures.
  • Management of the PAS/TS complex necessitates specialized centers with multidisciplinary teams.
  • Respiratory endoscopy is crucial for both pre-operative assessment and post-operative care in these patients.
Abstract

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