Evaluation of Clinical Findings in Children with Chylothorax: A Descriptive Study
Halime Nayir Büyükşahin1, Nagehan Emiralioğlu1, H Nursun Özcan2
1Department of Pediatric Pulmonology, Hacettepe University, School of Medicine, Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
Insights
This study analyzed childhood chylothorax, finding varied causes like surgery and Gorham-Stout disease. Early diagnosis and a multidisciplinary approach are key for effective treatment and good prognoses, especially for Gorham-Stout disease.
Area of Science:
- Pediatric Medicine
- Thoracic Surgery
- Rare Diseases
Background:
- Chylothorax, the accumulation of chyle in the pleural space, presents diverse etiologies in children.
- Understanding age-specific causes and treatments is crucial for pediatric patients.
Purpose of the Study:
- To evaluate clinical manifestations, causes, and treatments of childhood chylothorax.
- To compare differences between two age groups (<24 months and >24 months).
- To assess clinical and radiologic features of Gorham-Stout disease presenting as chylothorax.
Main Methods:
- Retrospective review of 22 pediatric chylothorax cases over 31 years.
- Patients categorized into two age groups: under 24 months and over 24 months.
- Analysis of etiologies, treatments, and clinical/radiologic findings.
Main Results:
- Congenital heart surgery and Gorham-Stout disease were common etiologies.
- Younger children: congenital heart surgery, congenital chylothorax, Gorham-Stout disease.
- Older children: Gorham-Stout disease, congenital heart surgery, heart failure, and others.
- Primary treatments: medium-chain triglyceride diet (younger) and sirolimus (older).
Conclusions:
- Childhood chylothorax has varied causes, necessitating a multidisciplinary diagnostic approach.
- Postoperative causes and Gorham-Stout disease were frequent in this cohort.
- Gorham-Stout disease, identified via chylothorax and bone lesions, showed a favorable prognosis.
Objective:
Chylothorax refers to the presence of chyle in the pleural space. There are multiple etiologies of chylothorax. Our aim in this study was to evaluate the clinical manifestations, causes, and treatment of chylothorax in childhood and also to show the differences between the 2 age groups admitted to a tertiary care children's hospital. The second aim was to evaluate the clinical and radiologic features of patients diagnosed as having Gorham-Stout disease via chylothorax.
Materials And Methods:
The archives were reviewed for chylothorax documented in the last 31 years. Twenty-two patients (11 girls and 11 boys) were included. Patients were divided into 2 groups: the younger group aged under 24 months and the older group aged over 24 months.
Results:
A total of 22 patients had chylothorax, and 10 were aged younger than 24 months. In the younger group, etiologies were in order congenital heart surgery, congenital chylothorax, and Gorham-Stout disease. In the older group, etiologies were Gorham-Stout disease, congenital heart surgery, heart failure, heart transplantation, thrombus, intestinal lymphangiectasia, and idiopathic. The most common treatment in the younger group was the medium-chain triglyceride diet (70%), and in the older group, it was sirolimus (50%).
Conclusion:
There is a wide variety of underlying etiologies in childhood, so a multidisciplinary approach is important to identify the underlying diagnosis. The common etiologies were postoperative and Gorham-Stout disease in our study. All patients with Gorham-Stout disease had a good prognosis. Gorham-Stout disease should be considered in patients of any age with a diagnosis of chylothorax who have bone lesions.
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