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Presentation of congenital diaphragmatic hernia past the neonatal period
Insights
Congenital diaphragmatic hernias (CDH) can occur beyond infancy, presenting with respiratory or gastrointestinal issues. Early diagnosis via imaging is crucial for managing this rare condition and its potential complications.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Gastroenterology
Background:
- Congenital diaphragmatic hernias (CDHs) are typically diagnosed neonatally.
- Late-presenting CDHs are uncommon, posing diagnostic challenges.
- This study examines CDH cases identified after the neonatal period.
Purpose of the Study:
- To investigate the clinical presentation, diagnosis, and outcomes of congenital diaphragmatic hernias presenting beyond the neonatal period.
- To identify distinct clinical groups based on age and symptoms.
- To emphasize the importance of considering CDH in pediatric patients with persistent respiratory or gastrointestinal problems.
Main Methods:
- Retrospective review of 11 pediatric patients with CDH diagnosed after the neonatal period.
- Analysis of clinical symptoms, diagnostic imaging (chest roentgenograms, GI contrast studies), and surgical findings.
- Categorization of patients into younger (respiratory symptoms) and older (GI complaints) groups.
Main Results:
- 11 of 83 pediatric patients presented with CDH beyond the neonatal period (13%).
- Two groups emerged: younger children with respiratory symptoms and older children with GI complaints.
- Diagnosis was preoperatively confirmed in 10 of 11 patients; malrotation was noted in 6.
- No peritoneal sacs were observed, and all herniated contents were viable; no pulmonary hypoplasia was evident.
Conclusions:
- Congenital diaphragmatic hernias can manifest significantly beyond the neonatal age.
- Consider CDH in children with unexplained respiratory or GI issues and abnormal chest X-rays.
- While mortality is low, morbidity associated with late-presenting CDH can be substantial.
Abstract:
Congenital diaphragmatic hernias (CDHs) presenting beyond the neonatal period are a rare and unusual problem; they occurred in 11 of 83 children at our institution. Two discrete clinical groups were apparent: (1) younger children, with mainly respiratory symptoms; and (2) older children with gastrointestinal (GI) complaints. Chest roentgenograms suggested CDHs, but GI contrast studies were necessary for confirmation in eight patients. The diagnosis was made preoperatively in ten of 11 children. At operation, no peritoneal sacs were found, the hernial contents were viable in all patients, and malrotation was present in six of 11 patients. None had evidence of pulmonary hypoplasia. Congenital diaphragmatic hernias do present beyond the neonatal age group in a significant number of cases (13% in our series), and a diagnosis of CDH should be considered in any child with persistent GI or respiratory problems and abnormal chest x-ray film findings. The mortality rate in these patients is small, but morbidity may be significant.