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Published on: February 5, 2021
Congenital right hemidiaphragmatic agenesis
Bilal Mirza1, Zahid Bashir, Afzal Sheikh
1Department of Pediatric Surgery, The Children's Hospital and The Institute of Child Health, Lahore, Pakistan.
Insights
This case study details the successful surgical repair of congenital diaphragmatic agenesis in an infant. The treatment involved suturing the remaining diaphragm to chest muscles, avoiding prosthetic materials.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Medical Case Reports
Background:
- Congenital diaphragmatic hernia (CDH) is a birth defect where abdominal organs move into the chest.
- Severe cases can involve complete diaphragm agenesis, a rare malformation.
- Infants with CDH often present with respiratory distress.
Purpose of the Study:
- To report a rare case of right hemidiaphragmatic agenesis.
- To describe a successful surgical management technique for this condition.
- To highlight a prosthetic-free approach for diaphragmatic defect repair.
Main Methods:
- A 45-day-old male infant presented with respiratory symptoms.
- Diagnostic imaging revealed a right-sided congenital diaphragmatic hernia.
- Surgical exploration identified agenesis of the right hemidiaphragm.
Main Results:
- A large defect was found, with absence of the diaphragm anteriorly and laterally.
- The posterior rim of the diaphragm was successfully sutured to intercostal muscles and ribs.
- The infant's condition was managed without the need for prosthetic material.
Conclusions:
- Hemidiaphragmatic agenesis is a severe form of congenital diaphragmatic hernia.
- Surgical repair by suturing the diaphragm remnant is a viable treatment option.
- This case demonstrates successful management without prosthetic mesh.
Abstract:
Congenital diaphragmatic hernia is a congenital defect of the diaphragm through which intestine and other viscera herniate into the chest. In extreme form of diaphragmatic maldevelopment, there might be a complete agenesis of diaphragm. A 45-day old male infant was presented with fever, cough and respiratory distress for a week. Chest radiograph showed right-sided congenital diaphragmatic hernia. The patient underwent surgical exploration and found to have an unusual and large defect of right hemidiaphragm. The diaphragm was absent on anterior and lateral aspects of the chest wall and only a small rim of diaphragm was present on posterior aspect. The defect was identified as agenesis of right hemidiaphragm and successfully managed by suturing the posterior rim of diaphragm to the intercostal muscles and ribs. This report describes successful management of hemidiaphragmatic agenesis without incorporating a prosthetic material.
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