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Successful treatment of large abdominal lymphatic malformations and chylous ascites with intra-abdominal lymphovenous anastomosis

https://doi.org/10.1016/j.jvsv.2020.05.017Get rights and content

Abstract

Large abdominal lymphatic malformations (LMs) are rare and may occasionally cause life-threatening illness, especially when they involve the central lymphatic system, lumbar trunks, cisterna chyli, thoracic duct, and their major tributaries, forming complex lymphatic anomalies. These LMs are often accompanied by chylous leak in various locations, and treatment remains challenging. We report a case of large abdominal LM with chylous ascites, protein-losing enteropathy, vaginal chylous leak, and lower limb lymphedema successfully treated with microsurgical intra-abdominal lymphovenous anastomosis and discuss the technical details of the procedure.

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Case report

A 10-year-old girl with a large abdominal LM associated with superficial chylous leak from the vagina and unilateral left lower limb lymphedema presented with progressive abdominal distention, exacerbation of vaginal leakage, and refractory lymphorrhea from trivial scratches on her leg.

One year earlier, we performed LVA on her left lower limb to reduce flow to the central lymphatic system, resulting in decreased lymphedema and vaginal chylous leakage. However, after menarche, she suddenly

Discussion

The observations and the biopsy result in our case suggested central conducting lymphatic anomaly (CCLA) or generalized lymphatic anomaly.4,11, 12, 13, 14, 15, 16, 17 At present, both diseases are characterized by a broad spectrum of anomalies involving the central lymphatic system, lumbar trunks, cisterna chyli, thoracic duct, and their major tributaries. Because of the rareness and similarity of these kinds of LMs, there is overlap in diagnostic criteria, making precise diagnosis difficult.

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Author conflict of interest: none.

Additional material for this article may be found online at www.jvsvenous.org.

The editors and reviewers of this article have no relevant financial relationships to disclose per the Journal policy that requires reviewers to decline review of any manuscript for which they may have a conflict of interest.

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