Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome

Author:

Miyashita Kayo1,Kadota Hideki1,Hanada Masuo1,Inatomi Yusuke1,Oryoji Chikafumi1,Morishita Aki1,Yoshida Sei1,Oda Yoshinao2,Kinoshita Izumi2

Affiliation:

1. Department of Plastic and Reconstructive Surgery, Kyushu University Hospital

2. Department of Anatomic Pathology, Pathological Sciences, Kyushu University Hospital, Fukuoka, Japan

Abstract

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.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Surgery

Reference11 articles.

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