Yutaka Kanamori1, Toshihiko Watanabe2, Tohru Yorifuji3, Michiya Masue4, Hideyuki Sasaki5, Masaki Nio5. 1. Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura Setagaya-ku, Tokyo, 157-8535, Japan. Kanamori-y@ncchd.go.jp. 2. Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, 2-10-1 Okura Setagaya-ku, Tokyo, 157-8535, Japan. 3. Division of Pediatric Endocrinology and Metabolism, Children's Medical Center, Osaka City General Hospital, Osaka, Japan. 4. Department of Pediatrics, Kizawa Memorial Hospital, Gifu, Japan. 5. Department of Pediatric Surgery, Tohoku University, Miyagi, Japan.
Abstract
PURPOSE: Congenital hyperinsulinism is a rare disease, and the newly developed 18 fluoro-L-dihydroxyphenylalanine-positron emission tomography (18F-DOPA PET) examination can detect hyperplastic lesions. Our purpose was to report the results of a nationwide survey on surgical treatment of congenital hyperinsulinism in Japan. METHODS: A questionnaire was sent to the 159 accredited and affiliated training institutes certified as pediatric surgical institutes by the Japanese Association of Pediatric Surgeons, asking if they had encountered patients who underwent surgical treatment for congenital hyperinsulinism after 18F-DOPA PET examination from 2000 to 2017. Six institutes answered that they had treated such cases, and the total number of cases was 14. RESULTS: 18F-DOPA PET examination detected the focal lesion in 12 of the 14 cases. 18F-DOPA PET examination could accurately determine the site of the hyperplastic lesion in the pancreas in 11 (91.7%) of the 12 cases. All cases underwent surgical resection of the hyperplastic lesion at under 2 years of age. CONCLUSION: Surgical resection of a focal hyperplastic lesion in the pancreas was a safe and effective treatment if the hyperplastic lesion was a focal lesion. However, it is necessary to check the exact distribution of the lesion by intraoperative pathologic examination of frozen sections.
PURPOSE:Congenital hyperinsulinism is a rare disease, and the newly developed 18 fluoro-L-dihydroxyphenylalanine-positron emission tomography (18F-DOPA PET) examination can detect hyperplastic lesions. Our purpose was to report the results of a nationwide survey on surgical treatment of congenital hyperinsulinism in Japan. METHODS: A questionnaire was sent to the 159 accredited and affiliated training institutes certified as pediatric surgical institutes by the Japanese Association of Pediatric Surgeons, asking if they had encountered patients who underwent surgical treatment for congenital hyperinsulinism after 18F-DOPA PET examination from 2000 to 2017. Six institutes answered that they had treated such cases, and the total number of cases was 14. RESULTS:18F-DOPA PET examination detected the focal lesion in 12 of the 14 cases. 18F-DOPA PET examination could accurately determine the site of the hyperplastic lesion in the pancreas in 11 (91.7%) of the 12 cases. All cases underwent surgical resection of the hyperplastic lesion at under 2 years of age. CONCLUSION: Surgical resection of a focal hyperplastic lesion in the pancreas was a safe and effective treatment if the hyperplastic lesion was a focal lesion. However, it is necessary to check the exact distribution of the lesion by intraoperative pathologic examination of frozen sections.
Authors: Olga T Hardy; Miguel Hernandez-Pampaloni; Janet R Saffer; Joshua S Scheuermann; Linda M Ernst; Richard Freifelder; Hongming Zhuang; Courtney MacMullen; Susan Becker; N Scott Adzick; Chaitanya Divgi; Abass Alavi; Charles A Stanley Journal: J Clin Endocrinol Metab Date: 2007-09-25 Impact factor: 5.958