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Primary lymphatic dysplasia in children: chylothorax, chylous ascites, and generalized lymphatic dysplasia
Insights
Primary lymphatic dysplasia, a congenital condition, can cause fluid buildup. Conservative treatments show promise for isolated cases, but generalized lymphatic dysplasia remains a severe, often fatal, condition.
Area of Science:
- Medicine
- Pediatrics
- Genetics
Background:
- Lymphatic dysplasia is a congenital maldevelopment affecting lymphatic system function.
- It can lead to chyle or lymph effusion into limbs, pleural, or peritoneal cavities.
Observation:
- A review of 38 Mayo Clinic patients from 1955-1982 identified chylous effusion or lymphatic dysplasia.
- Cases were categorized as primary or secondary, and further into chylothorax, chylous ascites, or generalized lymphatic dysplasia.
Findings:
- Conservative therapies, including dietary modifications and parenteral nutrition with fluid drainage, achieved a 75% resolution rate in children with isolated lymphatic abnormalities, with no associated deaths.
- In contrast, all five previously reported children with generalized lymphatic dysplasia died. Of the three cases reported here, one died, and two experienced progressive worsening.
Implications:
- Isolated lymphatic abnormalities are often responsive to conservative management in pediatric patients.
- Generalized lymphatic dysplasia represents a severe, high-mortality condition requiring further investigation and therapeutic development.
Abstract:
Primary lymphatic "dysplasia", a congenital maldevelopment, interferes with function of the lymphatic system and causes effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. Between 1955 and 1982, 38 Mayo Clinic patients were found to have a chylous effusion or dysplasia of the lymphatic system. In 22, the condition was secondary to surgery or other medical problems and in 16 it was primary. These cases were separated into three categories: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Conservative therapy, such as a restricted fat diet or total parenteral nutrition with repeated thoracentesis or paracentesis, was effective in the children with isolated abnormalities of the lymphatic system (75% resolution rate, no deaths). All five children with documented generalized dysplasia reported in the literature had died; of the three reported here, one has died and two have become progressively worse.