Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.

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.

Lymphaticovenular anastomosis for lymph vessel injury in the pelvis and groin.

BACKGROUND: Lymphatic diseases due to lymph vessel injuries in the pelvis and groin require immediate clinical attention when conventional treatments fail. We aimed to clarify the effectiveness of and indications for lymphaticovenular anastomosis (LVA) to treat these lymphatic diseases. METHODS: We retrospectively evaluated six patients who underwent LVA for lymphatic diseases due to lymph vessel injuries in the pelvis and groin. Specific pathologies included groin lymphorrhea (N = 3), chylous ascites (N = 2), and retroperitoneal lymphocele (N = 1). The maximum lymphatic fluid leakage volume was 150-2600 mL daily. Conventional treatments (compression, drainage, fasting, somatostatin administration, negative pressure wound therapy, or lymph vessel ligation) had failed to control leakage in all cases. We performed lower extremity LVAs after confirming the site of lymph vessel injury using lymphoscintigraphy. We preferentially placed LVAs in thigh sites that showed a linear pattern by indocyanine green lymphography. Postoperative lymphatic fluid leakage volume reduction was evaluated, and leakage cessation was recorded when the drainage volume approached 0 mL. RESULTS: LVA was performed at an average of 4.3 sites (range, 3-6 sites) in the thigh and 2.7 sites (range, 0-6 sites) in the lower leg. Lymphatic fluid leakage ceased in all cases after a mean of 6 days (range, 1-11 days) postoperatively. No recurrence of symptoms was observed during an average follow-up of 2.9 (range, 0.5-5.5) years. CONCLUSIONS: LVA demonstrates excellent and rapid effects. We recommend lower extremity LVA for the treatment of lymphatic diseases due to lymph vessel injuries in the pelvis and groin.

Successful Management of a Severe Sacral Pressure Injury Penetrating to the Retroperitoneum.

INTRODUCTION: Reports of retroperitoneal infection related to a sacral pressure injury (PI) are rare, and none of the reports described the direct spread of infection through the sacrum to the retroperitoneum. The authors present, to their knowledge, the first report of a severely infected PI that showed full-thickness sacral destruction and direct retroperitoneal penetration. CASE REPORT: A 63-year-old female was referred for management of a stage 4 sacral PI complicated by a retroperitoneal abscess. The patient's comorbidities were diabetes mellitus and pemphigus foliaceus with steroid therapy-induced immunosuppression. Upon admission, the patient presented with a sacral PI producing copious purulent discharge that measured 5 cm × 3 cm. Magnetic resonance imaging revealed full-thickness sacral bone destruction and a massive retroperitoneal abscess, suggesting the sacral PI directly penetrated to the retroperitoneal space. Antibiotics were administered, and surgical debridement and sequestrectomy were performed. Negative pressure wound therapy (NPWT) with continuous saline irrigation was initiated. The patient's mesorectum was exposed within the retroperitoneal space. Therefore, a nonadhesive wound dressing was applied before placing the irrigation tube to avoid perforating the rectum. Because the patient had fragile skin secondary to long-standing pemphigus foliaceus and steroid treatment, a liquid skin protectant and hydrocolloid wound dressing were applied. The infection was successfully controlled with NPWT with saline irrigation. The patient experienced no rectal injury or skin rupture, and surgical closure was performed after 75 days. Although partial wound dehiscence occurred because of the poor condition of the skin, the resultant open wound was managed conservatively. The patient showed no retroperitoneal abscess recurrence 6 months later. CONCLUSIONS: A rare case of an intractable sacral PI complicated by retroperitoneal abscess was successfully managed in an immunocompromised patient. Notably, NPWT with saline irrigation was useful in controlling the patient's severe retroperitoneal infection.

Utility of negative-pressure wound therapy for orocutaneous and pharyngocutaneous fistula following head and neck surgery.

BACKGROUND: Because of the difficulty of airtight sealing and risk of salivary contamination, negative-pressure wound therapy (NPWT) has rarely been applied for postoperative fistula following head and neck surgery; thus, its utility remains unclear. METHODS: We applied NPWT in 34 patients who developed orocutaneous and pharyngocutaneous fistula after head and neck surgery. Here we retrospectively analyzed the utility of NPWT for managing those fistulas. RESULTS: Thirty-two patients (94.1%) underwent NPWT as scheduled without adverse events. In 28 patients (82.4%), fistula closure was completed only by NPWT, and the mean period to fistula closure was 30.4 days. The mean period to closure did not differ significantly between fistulas with (21.7 days) and without (39.1 days) previous irradiation. CONCLUSIONS: Airtight sealing can be maintained and postoperative fistula can be closed by NPWT with a high success rate, even after previous irradiation. NPWT is an effective and minimally invasive treatment for postoperative fistula.