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Eppikajutsuto (TJ-28) is an herbal medicine recently reported to be effective in treating lymphatic malformations (LMs). We report our experience concerning the clinical efficacy of TJ-28 for LMs. Medical records of 10 LM cases treated with TJ-28 between 2016 and 2018 were reviewed. TJ-28 was given at 0.3 g/kg/day and then increased to 0.5 if no improvement was noted after the first three months of treatment. Their clinical data were collected, and LM volume indices (depth × width × height) were measured with the first (LMVI-F) and latest (LMVI-L) imaging studies. The response rates were calculated as 1-LMVI-L / LMVI-F (%). The median age at the diagnosis and treatment period was 1.5 years and 17.5 months, respectively. LMs were located in the neck (six), mesenterium or retroperitoneum (three), and inguinal region (one). The median response rate was 83%, including 100% in three cases and the apparent improvement of obstructive airway symptoms in one case. One case underwent surgery for insufficient improvement, and another that showed no effect is being considered for surgery. Most of the satisfactory outcomes were demonstrated in the first six months of treatment. TJ-28 seems to be effective in treating LMs in children, especially early in treatment. IV [ABSTRACT FROM AUTHOR]

An eight-year-old boy presented with a fever, abdominal pain, and vomiting. Imaging studies revealed a 10-cm cystic lesion in his right retroperitoneal area with no blood flow and with a pedunculated nodule inside the cavity. He required surgery to remove the lesion, when severe adhesion of the lesion to duodenum and a small vessel-like connection between the lesion and inferior vena cava were dissected to remove the lesion. A histopathological examination revealed that the wall of the main lesion and the vessel-like connection were composed of smooth muscle, elastic and collagen fiber, and stained positive with CD31, which led to the diagnosis of venous malformation. The pedunculated nodule was a fibrin clot with calcification. His postoperative course was uneventful. Venous malformation is a common vascular malformation, but that in the retroperitoneal area has rarely been reported in adults and never in children. Imaging studies often hardly make the correct diagnosis, and then surgery and pathological examination of the resected specimen deem necessary. Careful surgical maneuvers are mandatory due to the possibility of dense adhesions with the surrounding vital organs and tissue. [ABSTRACT FROM AUTHOR]