Right Lung Agenesis with Tracheal Stenosis due to Complete Tracheal Rings and Postpneumonectomy Like Syndrome Treated with Tissue Expander Placement

Yashwant Agrawal,Jagadeesh K Kalavakunta,Sandeep Patri

doi:10.1155/2016/4397641

Abstract

Congenital lung agenesis is an extremely rare condition with an estimated prevalence of 34 in 1,000,000 live births. It is often associated with other congenital malformations of the skeletal, cardiovascular, urogenital, and gastrointestinal systems. We discuss the case of a 5-month-old who presented with increasing stridor over 1 month. Imaging revealed right lung agenesis, complete dextromalposition of heart, and compression of distal trachea. An intrathoracic saline tissue expander was placed which marked improved distal tracheal stenosis. In patients who are symptomatic it becomes imperative to perform surgeries to increase survival as was the case in this patient.

Highlights

Congenital lung agenesis is a rare condition with a mortality rate as high as 50% by age of 5
There were two major causes for respiratory distress in this case, one being the mediastinal shift caused by the unilateral lung agenesis and other being the presence of tracheal rings causing tracheal stenosis
In a retrospective study by Backer et al 15% out of their 71 patients with tracheal stenosis had the combination of complete congenital tracheal rings and unilateral lung agenesis or severe hypoplasia [14]

Summary

Introduction

Congenital lung agenesis is a rare condition with a mortality rate as high as 50% by age of 5. Initial diagnosis may be delayed as the clinical picture and imaging may mimic other common diagnosis like pneumonia with parapneumonic effusion, and so forth. Almost 50% of these patients can have associated congenital malformations most notably of the skeletal, cardiovascular, urogenital, and gastrointestinal systems. These associated anomalies along with mechanical effects on the tracheobronchial tree and esophagus from mediastinal shift can result in major morbidity to this group of patients. Tracheal stenosis can be caused simultaneously by extrinsic compression from an overstretched aorta due to mediastinal displacement and intrinsic narrowing due to complete tracheal rings as seen in this patient.

Case Report

Findings

Discussion

Conclusion