The purpose of this retrospective review of the charts of 6 children who underwent surgical treatment of chylous ascites refractory to conservative measures between 1993 and 2006 was to evaluate the efficiency of fibrin glue application for control of lymph leakage. Five children had postoperative chylous ascites (neuroblastoma, 4; cystic lymphangioma, 1) and 1 had a congenital malformation. Surgical exploration revealed large areas of diffuse lymphatic leakage in all of the patients. Lymphatic fistula was not identified intraoperatively in any patient. Ingestion of lipophilic dye in a concentrated fatty meal was not helpful in locating a lymph fistula. Absorbable mesh was used in association with glue application in the last 3 patients treated. Control of ascites was achieved immediately in 2 patients and within 3 weeks in 2 patients. Repeat surgery was required in the remaining 2 patients. The mean follow-up time was 4.3 years. One patient died of tumor recurrence 12 months after surgical treatment without relapse of the ascites. Two mild late recurrences were observed at 6 and 11 months after surgery and were managed conservatively. The findings of this study show that fibrin glue application on absorbable mesh after dissection of the leakage zones is easy, safe, and effective. We recommend that surgery with glue application be repeated until control of ascites is achieved. We suggest fibrin glue application as a preventive measure against postoperative chylous ascites.
Primary lymphedema, a rare disease, has a genetic cause in ~40% of patients. Recently, loss‐of‐function mutations in PIEZO1, which encodes the mechanotransducer protein PIEZO1, were described as causing primary lymphedema, when gain‐of‐function PIEZO1 mutations were attributed to dehydrated hereditary stomatocytosis type‐1 (DHS), a dominant red cell hemolytic disorder, with ~20% of patients having perinatal edema. Lymphedema was diagnosed in a 36‐year‐old man from a three‐generation DHS family, with a PIEZO1‐allele harboring 3 missense mutations in cis. Four affected family members had severe fetal and neonatal edema, most severe in the proband, whose generalized edema with prevailing ascites resolved after 8 months. Our patient's intermittent lower limb‐lymphedema episodes during hot periods appeared at puberty; they became persistent and bilateral at age 32. Clinical Stemmer's sign confirmed lymphedema. Lower leg lymphoscintigraphy showed substantial dermal backflow in both calves, predominantly on the right. Noncontrast magnetic resonance lymphography showed bilateral lower limb lymphedema, dilated dysplastic lymphatic iliac, and inguinal trunks. Exome‐sequencing analysis identified no additional pathogenic variation in primary lymphedema‐associated genes. This is the first description of well‐documented lymphedema in an adult with PIEZO1–DHS. The pathophysiology of PIEZO1‐associated primary lymphedema is poorly understood. Whether it infers overlapping phenotypes or different mechanisms of gain‐ and loss‐of‐function PIEZO1 mutations deserves further investigation.
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