Pancreatic Lesions Valerie Jefford Pediatric Surgery Rounds June 6, 2003
Embryology 4 th week 2 buds of endodermal origin from caudal foregut Dorsal and ventral bud Ventral migrates dorsally with CBD (below/behind dorsal pancreas) Parenchyma fuses, then ducts fuse (duct of Wirsung) Residual duct (duct of Santorini) 10%
Congenital Variances Ectopic Pancreatic Tissue Bleeding or inflammation Annular Pancreas Anomaly of rotation of gland (most c.) Tissue surrounds duodenum (40% stenosis or atresia) Antenatal: polyhydramnios, double bubble Bilious or nonbilious emesis Treat with intestinal bypass if symptomatic
Congenital Variances Pancreatic Divisum Ducts fail to fuse 20% children (25% symptomatic) Abdominal pain Sphincteroplasty of both ducts
Pancreatitis Rare in children Pathophysiology: insult to gland > activation enzymes > autodigestion Sx: abdo/back pain, N/V Increased amylase & lipase AXR: stone, sentinel loop CXR, U/S, CT, ERCP
Pancreatitis Etiology Trauma Anomalies Biliary disease Infection Meds Metabolic Familial idiopathic
Pancreatitis - Treatment Medical Stop causative factor Supportive care (NPO, IV, NG, TPN, pain control, urine output, B/W, + abx) Surgical Failure medical treatment (necrotic tissue) Complication (abscess, pseudocyst) Uncertain dx
Chronic Pancreatitis Causes: trauma, hereditary, malformations, metabolic, endocrine, IBD Dx: labs, stool tests, pancreatic stimulation Imaging: AXR, U/S, CT, ERCP Surgery (relieve pain, complications) Sphincteroplasty Pancreatic drainage pancreatectomy
Pancreatic Cysts Congenital & Developmental Cysts Uniloculated or multiloculated, singular Epithelial-lined, sterile fluid, no inflamm. Symptoms if large (N/V, jaundice, etc.) Excise (body/tail) or internal drainage (head) Retention Cysts 2 0 chronic obstruction Ductal epithelium, exocrine excretions
Pancreatic Cysts Enteric Duplication Cysts Near stomach, communication panc. Duct Ectopic pancreatic tissue Sx: pain, reports of ulcer/perforation Tx: extirpation, pancreaticoduodenectomy
Pancreatic Cysts Pseudocysts Trauma, inflamm. reactive tissue Sx: pain, N/V, jaundice, bleeding, sepsis CT, U/S, ERCP Treatment Conservative 6/52 Internal drainage (Cystgastrostomy, Roux-en-Y) External drainage Distal pancreatectomy
Hyperinsulinism 1921 Banting & Best insulin/pancreas role Hypoglycemia > neurologic impairment Sx: irritable, pallor, sweating, poor feeding, seizures, coma DDx hypoglycemia Hyperinsulinism (50%) Inborn errors of metabolism Endocrine deficiencies (GH, panhypopit., etc)
Hyperinsulinism Dx Inappropriate high insulin (low glucose) Glucose infusion required Low plasma FFA & ketones Glycemic response to glucagon Treatment IV glucose, frequent feeds Diazoxide with chlorothizide Somatostatin (short-term) Mesoxalyl urea (cytotoxic ß-cells)
Hyperinsulinism- Causes Beckwith-Wiedemann Syndrome Macroglossia, gigantism,exomphalos Resolves spontaneously with time Nesidioblastosis (Neonatal Hyperinsulinism) Dysregulation of insulin storage & release 95% pancreatectomy if pts unable to sustain fast Look for adenomas prior to resection Complications: persistent hypoglycemia, MR, DM
Hyperinsulinism - Causes Islet Cell Adenoma (Insulinoma) Usually >4 y.o., malignancy rare in kids Solitary, multiple, or +nesidioblastosis U/S, CT (small), arteriography (60%) Intra-op U/S Enucleate or resect part of gland 80-90% distal pancreatectomy if no lesion (with intra-op insulin levels)
Pancreatic Neoplasms Hormonally Active Tumors Insulinomas Gastrinomas Zollinger-Ellison Syndrome MENI (malignant, multicentric, mets) Calcium infusion/ Secretin stimulation tests CT, venous sampling, gastrin assay to localize Medical tx, resect, may need gastrectomy if residual tumor or mets VIPomas
Pancreatic Neoplasms Cystic Neoplasms Cystadenoma/Cystadenocarcinoma Serous Mucinous Papillary-Cystic Epithelial Neoplasms Teratomatous Cysts
Pancreatic Neoplasms Carcinoma Islet Cell Carcinoma Functioning Nonfunctioning Adenocarcinoma pancreatoblastoma