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Pancreatic Disorders

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Pediatric Surgery

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Abstract

Pancreatic disorders can be congenital or acquired and can either affect the exocrine or the endocrine component of the gland. This chapter gives an overview of the main pancreatic conditions affecting children and adolescents that require surgical attention.

Structural pancreatic anomalies, such as annular pancreas, pancreas divisum, and pancreaticobiliary malunion, are rare but may cause severe symptoms and may require surgical correction.

Congenital hyperinsulinism (CHI) is a complex genetic disorder, characterized by excessive insulin secretion from pancreatic β-cells, which results in severe hypoglycemia. CHI can present as a focal, diffuse or atypical lesion. Near-total pancreatectomy is the procedure of choice for diffuse CHI, whereas a localized resection is curative for focal CHI.

Pancreatitis is defined as an inflammatory process secondary to the intraductal activation and secretion of pancreatic enzymes, which results in the digestion of the parenchyma. An increasing incidence of pancreatitis in children has recently been reported. In this chapter, we refer to the recent consensus statement of the INSPPIRE consortium for the diagnosis of childhood-onset acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis.

Pancreatic tumors are rare in the pediatric population, and their diagnosis is often challenging. Herein, we describe benign and malignant childhood tumors of the exocrine and endocrine pancreas according to the World Health Organization classification.

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References

  • Akbulut S, Yavuz R, Sogutcu N, Kaya B, Hatipoglu S, Senol A, Demircan F (2014) Hydatid cyst of the pancreas: report of an undiagnosed case of pancreatic hydatid cyst and brief literature review. World J Gastrointest Surg 6:190–200

    PubMed  PubMed Central  Google Scholar 

  • Awais M, Rehman A, Baloch NU (2019) Gastric-type enteric duplication cyst in the tail of a Bifid Pancreas in Association with complete intestinal malrotation. J Coll Physicians Surg Pak 29:489–490

    PubMed  Google Scholar 

  • Bohl CE, Federico SM, Robinson GW, Bahrami A, Shulkin BL (2018) FDG-PET CT in the evaluation of primary and secondary pancreatic malignancies. Pediatr Blood Cancer 65:e27115

    PubMed  Google Scholar 

  • Bülow R, Simon P, Thiel R, Thamm P, Messner P, Lerch MM, Mayerle J, Völzke H, Hosten N, Kühn JP (2014) Anatomic variants of the pancreatic duct and their clinical relevance: an MR-guided study in the general population. Eur Radiol 24:3142–3149

    PubMed  Google Scholar 

  • Cofer BR (2020). Pancreatic tumors. Pediatric surgery NaT. American Pediatric Surgical Association APSA Webapp. www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829508/all/Pancreatic_Tumors.)

  • Dabbas N, Davenport M (2009) Congenital choledochal malformation: not just a problem for children. Ann R Coll Surg Engl 91:100–105

    PubMed  PubMed Central  Google Scholar 

  • Dankovcik R, Jirasek JE, Kucera E, Feyereisl J, Radonak J, Dudas M (2008) Prenatal diagnosis of annular pancreas: reliability of the double bubble sign with periduodenal hyperechogenic band. Fetal Diagn Ther 24:483–490

    PubMed  PubMed Central  Google Scholar 

  • Diesen DL (2020a) Acute pancreatitis. Pediatric surgery NaT. American Pediatric Surgical Association. APSA Webapp. www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829507/all/Acute_Pancreatitis

  • Diesen DL (2020b) Chronic pancreatitis. Pediatric surgery NaT. American Pediatric Surgical Association. APSA Webapp. www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829601/all/Chronic_Pancreatitis

  • Dimitriou I, Katsourakis A, Nikolaidou E, Noussios G (2018) The main anatomical variations of the pancreatic duct system: review of the literature and its importance in surgical practice. J Clin Med Res 10:370–375

    PubMed  PubMed Central  Google Scholar 

  • Ellison EC, Johnson JA (2009) The Zollinger-Ellison syndrome: a comprehensive review of historical, scientific, and clinical considerations. Curr Probl Surg 46:13–106

    PubMed  Google Scholar 

  • Ferri V, Vicente E, Quijano Y, Ielpo B, Duran H, Diaz E, Fabra I, Caruso R (2019) Diagnosis and treatment of pancreas divisum: a literature review. Hepatobiliary Pancreat Dis Int 18:332–336

    PubMed  Google Scholar 

  • Flanagan SE, Kapoor RR, Hussain K (2011) Genetics of congenital hyperinsulinemic hypoglycemia. Semin Pediatr Surg 20:13–17

    PubMed  Google Scholar 

  • Hafezi M, Mayschak B, Probst P, Büchler MW, Hackert T, Mehrabi A (2017) A systematic review and quantitative analysis of different therapies for pancreas divisum. Am J Surg 214:525–537

    PubMed  Google Scholar 

  • Hameed S, Caro-Domínguez P, Daneman A, Zani-Ruttenstock E, Zani A, Navarro OM (2020) The role of sonography in differentiating congenital intrinsic duodenal anomalies from midgut malrotation: emphasizing the new signs of duodenal and gastric wall thickening and hyperechogenicity. Pediatr Radiol 50:673–683

    PubMed  Google Scholar 

  • Howard JM, Hess W (2002) The early surgeon-anatomists. In: Howard JM, Hess W (eds) History of the pancreas: mysteries of a hidden organ, 1st edn. Springer Science & Business Media, New York

    Google Scholar 

  • Hunter CJ, Connelly ME, Ghaffari N, Anselmo D, Gonzalez I, Shin C (2008) Enteric duplication cysts of the pancreas: a report of two cases and review of the literature. Pediatr Surg Int 24:227–233

    PubMed  Google Scholar 

  • Johnson PR, Spitz L (2000) Cysts and tumors of the pancreas. Semin Pediatr Surg 9:209–215

    CAS  PubMed  Google Scholar 

  • Karamanou M, Protogerou A, Tsoucalas G, Androutsos G, Poulakou-Rebelakou E (2016) Milestones in the history of diabetes mellitus: the main contributors. World J Diabetes 7:1–7

    PubMed  PubMed Central  Google Scholar 

  • Keim V, Witt H, Bauer N, Bodeker H, Rosendahl J, Teich N, Mossner J (2003) The course of genetically determined chronic pancreatitis. JOP 4:146–154

    PubMed  Google Scholar 

  • Kozu T, Suda K, Toki F (1995) Pancreatic development and anatomical variation. Gastrointest Endosc Clin N Am 5:1–30

    CAS  PubMed  Google Scholar 

  • Laje P, Scott Adzick N (2020) Congenital hyperinsulinism. Pediatric surgery NaT, American Pediatric Surgical Association. APSA Webapp. www.pedsurglibrary.com/apsa/view/Pediatric-SurgeryNaT/829506/all/Congenital_Hyperinsulinism

  • Laje P, Stanley CA, Palladino AA, Becker SA, Adzick NS (2012) Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr Surg 47:130–135

    PubMed  PubMed Central  Google Scholar 

  • Lecco TM (1910) Zur morphologie des pankreas annulare. Sitzungsb Akad Wissensch 119:391–406

    Google Scholar 

  • Lord K, Radcliffe J, Gallagher PR, Adzick NS, Stanley CA, De León DD (2015) High risk of diabetes and neurobehavioral deficits in individuals with surgically treated hyperinsulinism. J Clin Endocrinol Metab 100:4133–4139

    CAS  PubMed  PubMed Central  Google Scholar 

  • Lüttges J, Stigge C, Pacena M, Klöppel G (2004) Rare ductal adenocarcinoma of the pancreas in patients younger than age 40 years. Cancer 100:173–182

    PubMed  Google Scholar 

  • Márta K, Farkas N, Szabó I, Illés A, Vincze Á, Pár G, Sarlós P, Bajor J, Szűcs Á, Czimmer J, Mosztbacher D, Párniczky A, Szemes K, Pécsi D, Hegyi P (2016) Meta-analysis of early nutrition: the benefits of enteral feeding compared to a Nil Per Os diet not only in severe, but also in mild and moderate acute pancreatitis. Int J Mol Sci 17:1691

    PubMed  PubMed Central  Google Scholar 

  • Michailidis L, Aslam B, Grigorian A, Mardini H (2017) The efficacy of endoscopic therapy for pancreas divisum: a meta-analysis. Ann Gastroenterol 30:550–558

    PubMed  PubMed Central  Google Scholar 

  • Morikawa M, Nakazawa T, Kato S, Fujimoto D, Koneri K, Murakami M, Hirono Y, Maeda H, Goi T, Katayama K, Imamura Y, Tanizawa A, Yamaguchi A (2016) Acinar cell carcinoma of the pancreas in childhood. Int Cancer Conf J 5:140–145

    PubMed  PubMed Central  Google Scholar 

  • Morinville VD, Husain SZ, Bai H, Barth B, Alhosh R, Durie PR, Freedman SD, Himes R, Lowe ME, Pohl J, Werlin S, Wilschanski M, Uc A, INSPPIRE Group (2012) Definitions of pediatric pancreatitis and survey of present clinical practices. J Pediatr Gastroenterol Nutr 55:261–265

    PubMed  PubMed Central  Google Scholar 

  • Peranteau WH, Palladino AA, Bhatti TR, Becker SA, States LJ, Stanley CA, Adzick NS (2013) The surgical management of insulinomas in children. J Pediatr Surg 48(12):2517–2524

    PubMed  PubMed Central  Google Scholar 

  • Perez EA, Gutierrez JC, Koniaris LG, Neville HL, Thompson WR, Sola JE (2009) Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg 44:197–203

    PubMed  Google Scholar 

  • Pierro A, Nah SA (2011) Surgical management of congenital hyperinsulinism of infancy. Semin Pediatr Surg 20:50–53

    PubMed  Google Scholar 

  • Poddar U, Yachha SK, Borkar V, Srivastava A, Kumar S (2017) A report of 320 cases of childhood pancreatitis: increasing incidence, etiologic categorization, dynamics, severity assessment, and outcome. Pancreas 46:110–115

    PubMed  Google Scholar 

  • Poropat G, Giljaca V, Hauser G, Štimac D (2015) Enteral nutrition formulations for acute pancreatitis. Cochrane Database Syst Rev 3:CD010605

    Google Scholar 

  • Quest L, Lombard M (2000) Pancreas divisum: opinion divisa. Gut 47:317–319

    CAS  PubMed  PubMed Central  Google Scholar 

  • Rebours V, Boutron-Ruault MC, Schnee M, Férec C, Le Maréchal C, Hentic O, Maire F, Hammel P, Ruszniewski P, Lévy P (2009) The natural history of hereditary pancreatitis: a national series. Gut 58:97–103

    CAS  PubMed  Google Scholar 

  • Restrepo R, Hagerott HE, Kulkarni S, Yasrebi M, Lee EY (2016) Acute pancreatitis in pediatric patients: demographics, etiology, and diagnostic imaging. AJR Am J Roentgenol 206:632–644

    PubMed  Google Scholar 

  • Rustagi T, Njei B (2014) Magnetic resonance cholangiopancreatography in the diagnosis of pancreas divisum: a systemic review and meta-analysis. Pancreas 43:823–828

    PubMed  Google Scholar 

  • Sacco Casamassima MG, Goldstein SD, Yang J, Gause CD, Abdullah F, Meoded A, Makary MA, Colombani PM (2017) The impact of surgical strategies on outcomes for pediatric chronic pancreatitis. Pediatr Surg Int 33:75–83

    PubMed  Google Scholar 

  • Sadler TW (2019) Langman’s medical embryology, 14th edn. Wolters Kluwer, Philadelphia

    Google Scholar 

  • Testoni PA (2014) Acute recurrent pancreatitis: etiopathogenesis, diagnosis and treatment. World J Gastroenterol 20:16891–16901

    PubMed  PubMed Central  Google Scholar 

  • Trout AT, Serai SD, Fei L, Sun Q, Abu-El-Haija M (2018) Prospective assessment of normal pancreatic secretory function measured by MRI in a cohort of healthy children. Am J Gastroenterol 113:1385

    PubMed  Google Scholar 

  • Villatoro E, Mulla M, Larvin M (2010) Antibiotic therapy for prophylaxis against infection of pancreatic necrosis in acute pancreatitis. Cochrane Database Syst Rev 2010:CD002941

    PubMed  PubMed Central  Google Scholar 

  • Zani A, Nah SA, Ron O, Totonelli G, Ismail D, Smith VV, Ashworth M, Blankenstein O, Mohnike W, De Coppi P, Eaton S, Hussain K, Pierro A (2011) The predictive value of preoperative fluorine-18-L-3,4-dihydroxyphenylalanine positron emission tomography-computed tomography scans in children with congenital hyperinsulinism of infancy. J Pediatr Surg 46:204–208

    PubMed  Google Scholar 

  • Zani A, Yeh JB, King SK, Chiu PP, Wales PW (2017) Duodeno-duodenostomy or duodeno-jejunostomy for duodenal atresia: is one repair better than the other? Pediatr Surg Int 33:245–248

    PubMed  Google Scholar 

  • Zani-Ruttenstock E, Davenport M (2016) Biliary atresia and choledocal malformations. In: Guandalini S, Dhawan A, Branski D (eds) Pediatric gastroenterology, hepatology and nutrition, 1st edn. Springer, London

    Google Scholar 

  • Zyromski NJ, Sandoval JA, Pitt HA, Ladd AP, Fogel EL, Mattar WE, Sandrasegaran K, Amrhein DW, Rescorla FJ, Howard TJ, Lillemoe KD, Grosfeld JL (2008) Annular pancreas: dramatic differences between children and adults. J Am Coll Surg 206:1019–1025

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Canada

    Elke Zani-Ruttenstock & Augusto Zani

  2. Department of Surgery, University of Toronto, Toronto, Canada

    Elke Zani-Ruttenstock & Augusto Zani

Authors
  1. Elke Zani-Ruttenstock
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  2. Augusto Zani
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Correspondence to Augusto Zani .

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Editors and Affiliations

  1. Beacon Hospital, University College Dublin, Dublin, Ireland

    Prem Puri

  2. University Clinic of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria

    Michael E. Höllwarth

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Zani-Ruttenstock, E., Zani, A. (2023). Pancreatic Disorders. In: Puri, P., Höllwarth, M.E. (eds) Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-030-81488-5_84

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  • DOI: https://doi.org/10.1007/978-3-030-81488-5_84

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-81487-8

  • Online ISBN: 978-3-030-81488-5

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