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Published on: March 5, 2016
Successful management of lymphangiomatosis and chylothorax in a 7-month-old infant
Scott Burgess1, Margaret Harris, Carolyn Dakin
1Department of Respiratory and Sleep Medicine, Mater Children's Hospital, South Brisbane, Australia. sburgess@mater.org.au
Insights
Splenic lymphangiomatosis in an infant caused chylous effusion. Surgical spleen resection resolved the condition, demonstrating its efficacy for this rare disease.
Area of Science:
- Pediatric Surgery
- Medical Imaging
- Vascular Anomalies
Background:
- Lymphangiomatosis is a rare congenital anomaly characterized by lymphatic vessel proliferation.
- Infantile splenic lymphangiomatosis can lead to significant complications, including chylous effusion and respiratory compromise.
Observation:
- A 7-month-old infant presented with splenic lesions and chylous effusion.
- Magnetic resonance imaging (MRI) confirmed splenic lymphangiomatosis with numerous enhancing lesions.
- Conservative management with dietary changes and octreotide failed to control chyle production.
Findings:
- Surgical intervention, specifically splenectomy and partial pleurectomy, resulted in a dramatic clinical improvement.
- The infant showed no recurrence of lymphangiomas or chylothoraces at 27 months of age.
Implications:
- Splenectomy is a viable and effective treatment for symptomatic splenic lymphangiomatosis in infants.
- Early surgical intervention may prevent severe complications associated with untreated lymphangiomatosis.
- This case highlights the importance of considering surgical options when conservative measures fail.
Abstract:
A 7-month old infant presented with an effusion and multiple lesions in his spleen. A diagnosis of lymphangiomatosis was made based on chylous effusion and an MRI demonstrating numerous enhancing lesions within the spleen on T2-weighed images. Conservative measures, including the withdrawal of feeds and octreotide, did not significantly reduce the rate of chyle production and increasing requirement for respiratory support. Resection of the patient's spleen and partial pleurectomy were associated with a dramatic improvement in his condition. At 27 months the patient was well with no evidence of further lymphangiomas or a recurrence of his chylothoraces.
