Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.

Autor:	Miyashita K; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Kadota H; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Hanada M; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Inatomi Y; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Oryoji C; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Morishita A; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Yoshida S; From the Department of Plastic and Reconstructive Surgery, Kyushu University Hospital., Oda Y; Department of Anatomic Pathology, Pathological Sciences, Kyushu University Hospital, Fukuoka, Japan., Kinoshita I; Department of Anatomic Pathology, Pathological Sciences, Kyushu University Hospital, Fukuoka, Japan.
Jazyk:	angličtina
Zdroj:	Annals of plastic surgery [Ann Plast Surg] 2024 Feb 01; Vol. 92 (2), pp. 208-211. Date of Electronic Publication: 2023 Dec 28.
DOI:	10.1097/SAP.0000000000003744
Abstrakt:	Abstract: Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs).A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS. At 60 years of age, she developed chyluria (urine albumin, 2224 μg/mL) and hematuria. Lymphoscintigraphy showed a lymphatic-ureteral fistula near the ureterovesical junction. Conservative treatment was ineffective. She also developed left lower extremity lymphedema, which gradually worsened. Leg cellulitis and purulent pericarditis developed because of hypoalbuminemia (minimum serum albumin level, 1.3 g/dL).We performed 14 LVAs in 2 surgeries to reduce lymphatic fluid flow through the lymphatic-ureteral fistula. The chyluria and hematuria resolved soon after the second operation, and the urine albumin level decreased (3 μg/mL). After 28 months, she had no chyluria or hematuria recurrence and her serum albumin level improved (3.9 g/dL). Multiple LVAs can definitively treat chyluria caused by a lymphatic-ureteral fistula in patients with KTS. Competing Interests: Conflicts of interest and sources of funding: The authors received no grants for the research, authorship, and/or publication of this article. (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)
Databáze:	MEDLINE
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