National survey of hepatobiliary and pancreatic surgery in hemophilia patients in Japan

Abstract

Background Approximately 8300 hemophiliacs are registered in Japan, but no comprehensive reports on hepatobiliary and pancreatic surgery (HBPS) have been conducted. This report investigates the current status of HPBS in hemophilia patients in Japan. Methods The subjects were hemophiliac patients seen between January 1 2007, and December 31 2017, at facilities participating in this study among the facilities for performing high‐difficulty cases nationwide designated by the Japanese Society for HBPS. A retrospective examination of short‐term outcomes in 49 cases was conducted to assess patient background, disease, surgical procedure, and complications. Results The types of hemophilia were A: 43 cases, B: four cases, and von Willebrand disease: two cases (hemophilia severity: mild 32, moderate seven, severe 10). The target malignant diseases for surgery were hepatocellular carcinoma (HCC) in 20 cases, intrahepatic cholangiocellular carcinoma (CCC) in four cases, combined HCC‐CCC in two cases, hilar CCC in two cases, and pancreatic cancer in four cases. As for the surgical procedure, limited resection (subsegmentectomy and partial hepatectomy) was performed in 16 cases of HCC even with normal liver function tests. Pancreaticoduodenectomy and distal pacreatectomy were performed for pancreatic cancers as in the standard procedure. Postoperative complications were postoperative bleeding in two cases after hepatectomy and one after pancreatectomy in one case. When compared with Japanese National Clinical Data base, the complication rates after hepatectomy and pancreatectomy were not conspicuous in hemophilic patients. Conclusions As long as they are performed in qualified centers, complication rate is not increased in hemophilic patients undergoing HBPS.

Laparoscopic liver resection for a patient of hepatocellular carcinoma with von Willebrand disease: a case report

“…VWD is a primary hemostasis deficiency presenting with mucosal bleeding, easy bruising, menorrhagia, and heavy bleeding during labor [ 8 ]. In the literature, several studies have reported about the safety and availability of conventional liver surgery in patients with ICD, including VWD [ 7 , 9 ]. Furthermore, Denzer et al reported that mini-laparoscopy-assisted liver biopsy was safe for patients with ICD [ 10 ].…”

“…Kobayashi et al reported no significant difference in operative time, estimated blood loss, red blood cell transfusion rates, Clavien–Dindo classification 3–4 complications, or mortality rates between patients with and without ICD who underwent hepatectomy [ 7 ]. Yoshimoto-Haramura et al reported that the postoperative complication rate did not increase in patients with ICD who underwent hepato-biliary-pancreatic surgery at qualified centers [ 9 ]. When limited to patients with VWD, several reports have described hepatectomies for patient safety [ 7 , 12 , 13 ].…”

Evaluation of Periodontal Status and Oral Health Habits with Continual Dental Support for Young Patients with Hemophilia

“…According to a 2018 survey, there are approximately 6200 patients with hemophilia in Japan (5100 with hemophilia A and 1100 with hemophilia B) [4]. Every summer since 2008, our hospital has held a comprehensive outpatient clinic for children with hemophilia, at which pediatricians evaluate coagulation factor replacement therapy, orthopedic surgeons assess hemophilic arthritis, and physical therapists provide rehabilitation.…”