N Scott Adzick1, Diva D De Leon2, Lisa J States3, Katherine Lord2, Tricia R Bhatti4, Susan A Becker2, Charles A Stanley2. 1. Department of Surgery and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. Electronic address: adzick@email.chop.edu. 2. Department of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. 3. Department of Radiology and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. 4. Department of Pathology and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Abstract
BACKGROUND: Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI. METHODS: From 1998 to 2018, 500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HI patients had pancreatectomies tailored to the PET scan and biopsy findings. RESULTS: The vast majority of pancreatectomies for focal HI were < 50%, and many were 2%-10%. 97% of focal HI patients are cured. For diffuse disease patients, 31% were euglycemic, 20% were hyperglycemic, and 49% required treatment for hypoglycemia; the incidence of diabetes increased with long-term follow-up. All 15 insulinoma patients were cured. CONCLUSIONS: Our approach to patients with focal HI can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and for some cases of atypical HI. LEVEL OF EVIDENCE: Level IV.
BACKGROUND:Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI. METHODS: From 1998 to 2018, 500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HIpatients had pancreatectomies tailored to the PET scan and biopsy findings. RESULTS: The vast majority of pancreatectomies for focal HI were < 50%, and many were 2%-10%. 97% of focal HIpatients are cured. For diffuse diseasepatients, 31% were euglycemic, 20% were hyperglycemic, and 49% required treatment for hypoglycemia; the incidence of diabetes increased with long-term follow-up. All 15 insulinomapatients were cured. CONCLUSIONS: Our approach to patients with focal HI can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and for some cases of atypical HI. LEVEL OF EVIDENCE: Level IV.
Authors: R J Ferry; A Kelly; A Grimberg; S Koo-McCoy; M J Shapiro; K E Fellows; B Glaser; L Aguilar-Bryan; D E Stafford; C A Stanley Journal: J Pediatr Date: 2000-08 Impact factor: 4.406
Authors: A Grimberg; R J Ferry; A Kelly; S Koo-McCoy; K Polonsky; B Glaser; M A Permutt; L Aguilar-Bryan; D Stafford; P S Thornton; L Baker; C A Stanley Journal: Diabetes Date: 2001-02 Impact factor: 9.461
Authors: H N Lovvorn; M L Nance; R J Ferry; L Stolte; L Baker; J A O'Neill; L Schnaufer; C A Stanley; N S Adzick Journal: J Pediatr Surg Date: 1999-05 Impact factor: 2.545
Authors: C Crétolle; C Nihoul Fékété; D Jan; M C Nassogne; J M Saudubray; F Brunelle; J Rahier Journal: J Pediatr Surg Date: 2002-02 Impact factor: 2.545
Authors: Mariko Suchi; Courtney M MacMullen; Paul S Thornton; N Scott Adzick; Arupa Ganguly; Eduardo D Ruchelli; Charles A Stanley Journal: Mod Pathol Date: 2006-01 Impact factor: 7.842
Authors: Timo Otonkoski; Kirsti Näntö-Salonen; Marko Seppänen; Riitta Veijola; Hanna Huopio; Khalid Hussain; Päivi Tapanainen; Olli Eskola; Riitta Parkkola; Klas Ekström; Yves Guiot; Jacques Rahier; Markku Laakso; Risto Rintala; Pirjo Nuutila; Heikki Minn Journal: Diabetes Date: 2006-01 Impact factor: 9.461
Authors: Mariko Suchi; Courtney MacMullen; Paul S Thornton; Arupa Ganguly; Charles A Stanley; Eduardo D Ruchelli Journal: Pediatr Dev Pathol Date: 2003 Jul-Aug
Authors: Kara E Boodhansingh; Zhongying Yang; Changhong Li; Pan Chen; Katherine Lord; Susan A Becker; Lisa J States; N Scott Adzick; Tricia Bhatti; Show-Ling Shyng; Arupa Ganguly; Charles A Stanley; Diva D De Leon Journal: Eur J Endocrinol Date: 2022-06-27 Impact factor: 6.558
Authors: Joanna Yuet-Ling Tung; Sophie Hon Yu Lai; Sandy Leung Kuen Au; Kit San Yeung; Anita Sik Yau Kan; Florence Loong; Diva D DeLeón; Jennifer M Kalish; Arupa Ganguly; Brian Hon Yin Chung; Kelvin Yuen Kwong Chan Journal: Int J Pediatr Endocrinol Date: 2020-07-10
Authors: Mary Ellen Vajravelu; Jung-Jin Lee; Lauren Mitteer; Babette S Zemel; Kyle Bittinger; Diva D De León Journal: Pancreas Date: 2021-01-01 Impact factor: 3.243
Authors: Maria Luisa Brandi; Sunita K Agarwal; Nancy D Perrier; Kate E Lines; Gerlof D Valk; Rajesh V Thakker Journal: Endocr Rev Date: 2021-03-15 Impact factor: 19.871
Authors: Jelena Sikimic; Theresa Hoffmeister; Anne Gresch; Julia Kaiser; Winfried Barthlen; Carmen Wolke; Ilse Wieland; Uwe Lendeckel; Peter Krippeit-Drews; Martina Düfer; Gisela Drews Journal: Front Endocrinol (Lausanne) Date: 2020-10-27 Impact factor: 5.555