Congenital tracheo-esophageal fistula/esophageal atresia (TEF/EA) with concomitant pulmonary agenesis is exceedingly rare and has a high mortality rate. While there are several reported cases of successful repair, all but one patient had right-sided pulmonary agenesis. In the case of left-sided pulmonary agenesis, the patient had incomplete agenesis and underwent repair through a left thoracotomy. We present the first successful repair of TEF/EA with complete left-sided pulmonary agenesis. This patient also underwent elective pre-operative veno-venous extracorporeal membrane oxygenation (ECMO) and subsequent repair of the TEF/EA. We discuss the management, anesthesia risks, and role of periprocedural ECMO in pediatric patients who are high anesthetic risk.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1177/02676591231176241 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Unique imaging observations in an incidentally detected bilateral upper lobe agenesis.
BMJ Case Rep
November 2024
Radiology, Narayana Hrudayalaya Health City Bangalore, Bengaluru, Karnataka, India
Article Synopsis
- - Bilateral upper lobe pulmonary agenesis is a rare birth defect typically discovered incidentally in adults, with non-specific and subtle findings on traditional x-rays.
- - Diagnosis is often missed on regular radiography, but chest CT scans performed for other reasons can reveal this condition.
- - Contrast-enhanced CT is the best method for diagnosing and detailing the features of pulmonary agenesis, and understanding related pulmonary anomalies is crucial in treating patients with congenital heart issues.
Rare Presentation of Schizophrenia in a Patient with Congenital Right Lung Agenesis: A Case Report.
Indian J Psychol Med
May 2024
Dept. of Pediatrics, All India Institute of Medical Sciences, Bibinagar (Hyderabad Metropolitan Region), Bibinagar, Telangana, India.
Sex-specific differences in the severity of pulmonary hypoplasia in experimental congenital diaphragmatic hernia and implications for extracellular vesicle-based therapy.
Pediatr Surg Int
October 2024
Developmental and Stem Cell Biology Program, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, M5G 0A4, Canada.
Purpose: Amniotic fluid stem cell extracellular vesicles (AFSC-EVs) hold regenerative potential to treat hypoplastic lungs secondary to congenital diaphragmatic hernia (CDH). This study aims to investigate sex-specific differences in pulmonary hypoplasia severity and responses to AFSC-EV administration in an experimental CDH mouse model.
Methods: C57BL/6J dams were fed with nitrofen + bisdiamine (left-sided CDH) or olive oil only (control) at embryonic day (E) 8.
Prenatal Ultrasound Diagnosis of Unilateral Pulmonary Agenesis.
J Med Ultrasound
November 2023
Department of Obstetrics and Gynecology, University of Zulia, Maracaibo, Venezuela.
Pulmonary agenesis (PA) is a rare developmental malformation, with a frequency of approximately 1 in 10-15,000 pregnancies. Unilateral PA is often associated with other congenital anomalies, whereas bilateral PA is fatal. Prenatal diagnosis is rare and is diagnosed more frequently in the postnatal period than in the prenatal period.
View Article and Find Full Text PDFGiant omphalocele with right lung agenesia and bronchial tracheal hypoplasia: A case report.
Radiol Case Rep
November 2024
Radiology Unit, "Santobono-Pausilipon" Children's Hospital, Naples, Italy.
The omphalocele is an abdominal wall defect at the base of the umbilical cord, with the worldwide prevalence of 2.6 per 10,000 births. Omphalocele contains herniated abdominal organs and is classified in small and giant based on the size of the defect.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!