PANCREATITIS 2003

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    Management of Childhood Pancreatic Disorders:

    A Multidisciplinary Approach

    *Surender K. Yachha, *Kamal Chetri, *Vivek A. Saraswat, Sanjay S. Baijal, Sadiq S. Sikora,

    Richa Lal, and *Anshu Srivastava

    Departments of *Gastroenterology, Radiology, and Surgical Gastroenterology, Sanjay Gandhi Postgraduate Institute of

    Medical Sciences, Lucknow, Uttar Pradesh, India

    ABSTRACTIntroduction: Data on therapeutic endoscopy and radiologicinterventions for the management of childhood pancreatic dis-orders are relatively limited. This study focuses on the mul-tidisciplinary approach to the management of pancreatitis inchildren.Patients and Methods: Children with pancreatic disorderswere studied from January 1992 to May 2001. Acute pancre-atitis (AP) was diagnosed by clinical evaluation, serum amylasemore than three times normal, and morphologic abnormalitiesof the pancreas on imaging. Children with recurrent abdominalpain, pancreatic calcification or ductal stones on imaging, andpancreatic ductal changes on endoscopic retrograde cholangio-pancreatography (ERCP) were diagnosed with chronic pancre-atitis (CP). Patients were treated by gastroenterologists, sur-geons, and interventional radiologists. Pancreatic exocrine in-sufficiency was diagnosed in appropriate settings.Results: Fifteen children6 with AP (posttrauma, 3; gallstonedisease, 1; and viral, 1), 7 with CP, and 2 with pancreaticexocrine insufficiencywere diagnosed. Local complications

    observed in children with AP included pseudocyst in three, andinfected acute fluid collection, right-sided pleural effusion, and

    ascites in one patient each. Complications of AP were managedwith percutaneous catheter drainage (n 3; pseudocyst, 2;infected fluid collection, 1), additional pancreatic duct stenting(n 2), surgical drainage (n 1), and octreotide for pleuraleffusion (n 1). Signs of CP included abdominal pain (n 7), obstructive jaundice resulting from lower common bile ductstricture (n 2), and bleeding from gastroduodenal arterypseudoaneurysm (n 1). Pancreatic duct stenting relievedpain in one patient, and steel coil embolization arrested bleed-ing from the pseudoaneurysm. Common bile duct strictureswere managed by surgical bypass (n 2), one of which re-quired preoperative endoscopic bile duct stenting for manage-ment of cholangitis. Two other patients with CP required nointervention.Conclusion: A multidisciplinary approach of radiologic andendoscopic interventions and surgery are complimentary toeach other in achieving successful outcomes of complicatedchildhood pancreatitis. JPGN 36:206212, 2003. Key Words:PancreatitisChildRadiologyInterventionalRetrogradecholangiopancreatographyEndoscopic. 2003 Lippincott

    Williams & Wilkins, Inc.

    Advances in endoscopic, radiologic, and surgical in-terventions have revolutionized the diagnosis and man-agement of pancreatic disorders in adults. Complicationsof acute and chronic pancreatitis (AP and CP), whichcontribute significantly to morbidity and mortality, havebeen successfully managed by a combined approach.There are relatively limited published data regarding theusefulness of therapeutic endoscopy and radiologic in-

    terventions for the management of AP and CP in children(112). Our study focuses on the presentation, diagnosis,and multidisciplinary approach to the management ofacute and chronic pancreatic disorders in children.

    PATIENTS AND METHODS

    This is a hospital-based study of patients with clinicalsigns of pancreatic disorders who were admitted fromJanuary 1992 to May 2001. Our hospital is a tertiary carereferral center in northern India. AP was diagnosed byclinical evaluation, elevated serum amylase more thanthree times normal (13), and morphologic abnormalityon imaging of pancreas (ultrasonography [US] and

    contrast-enhanced computed tomography [CECT] of ab-domen). CP was diagnosed by presence of recurrent ab-dominal pain, low fecal chymotrypsin level (

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    were jointly treated with the active involvement of gas-troenterologists, interventional radiologist, and gastroin-testinal surgeon. Treatment of patients was individual-ized, and interventions were performed using standardprotocol (1,3). Diagnosis of other pancreatic disorderswas based on the presence of typical abnormalities onpancreatic imaging (US and CECT) supplemented withhematologic and biochemical changes.

    RESULTS

    Of the 15 children diagnosed with pancreatic disor-ders, 6 had AP (boys, 3; median age, 8.5 years [range,514]); 7 had CP (boys, 4; median age, 11 years [range1.513]); and 2 had pancreatic exocrine insufficiency.

    Acute Pancreatitis

    Three children had posttrauma AP. One of the post-trauma patients (boy aged 10 years) was examined for

    respiratory distress on day 4 of illness and was found tohave right-sided pleural effusion. Imaging (US andCECT of the abdomen) showed an enlarged pancreas andperipancreatic edema. His dyspnea improved, and thepleural effusion resolved after 7 days of treatment withoctreotide (50 g subcutaneously every 8 hours). Theother two patients with posttrauma AP were examinedfor persistent fever, abdominal pain, and abdominal masson days 40 and 49 of illness, respectively. In one patient(boy aged 10 years), CECT showed two pseudocysts(Fig. 1), one in the lesser sac (8 7 6 cm), and the otheranterior to the stomach (4 2 1 cm). He underwentpercutaneous catheter drainage (PCD), transgastric forthe lesser sac pseudocyst, and transperitoneal for the

    pseudocyst anterior to the stomach. The transperitonealcatheter was removed after 7 days because the drain

    output decreased. However, the transgastric catheter con-tinued to have high output (300 mL/day) with high amy-lase (4,700 IU/dL), suggesting pancreatic duct (PD)communication, which required ERCP and PD stentplacement on day 10. After 3 days, the catheter outputdecreased, and the external drainage was internalized toa percutaneously placed cystogastric double Mallecotstent. The PD stent was removed within 6 months, andthe cystogastric stent was removed endoscopically after1 year. In another posttrauma patient (boy aged 5 years),abdominal US showed two pseudocysts, one in the lessersac (14 12 6 cm), and the other near the tail of thepancreas (5 3 3 cm). PCD was done for the lesser sacpseudocyst with an extragastric approach. The drain out-put decreased to

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    to drain pseudocysts and acute fluid collections, and highdrain output in children with AP suggests PD communi-cation (Table 2).

    DISCUSSION

    Childhood pancreatic disorders are uncommon, andthe exact incidence is not known (14). At our hospital,pancreatic disorders constituted an average of 1.6 cases

    per year and 0.9% of all admissions to the pediatric gas-troenterology department. Our series has highlighted APand CP with varying presentations and differences inmanagement. AP in children is known to result fromtrauma (1520%), biliary tract disorders (15%), idio-pathic cause (25%), and from other causes related todrugs, infections, and multisystem disorders (8,15). Inthree of our patients, AP was the result of trauma. Onepatient had gallstone pancreatitis, which is uncommon in

    TABLE 1. Summary of clinical features, investigations, management, and follow-up of patients with chronic pancreatitis

    Caseno. Age (y)/sex

    Symptoms,duration

    Findings(diagnostic study) Management Follow-up

    1 11/M Recurrent pa in in abdomen,1 y

    Low fecal chymotrypsin, PDdilated with two strictures(ERCP)

    Endoscopic PD stenting;removed after 1 y

    2 y; pain free

    2 13/M Recurrent pa in in abdome n,2 y

    Low fecal chymotrypsin;pancreatic calcifications,dilated PD with intraductalcalculi (CECT)

    Longitudinal Roux-en-Ypancreaticojejunostomy

    3 mo; pain free

    3 1.5/M Jaundice and pain inabdomen, 2 mo

    Serum bilirubin, 12.6 mg/dL;serum alkaline phosphatase,3,812 IU/dL; low fecalchymotrypsin, dilatedintrahepatic biliary radicalsand CBD stricture of thelower end (ultrasonography)

    At surgery, focal pancreatitisseen in head of pancreas;cholecystectomy andcholedochoduodenostomy

    2 y; no recurrence

    4 11/F Recurrent pain in abdomen,2 y; jaundice, 15 d

    Serum bilirubin, 4 mg/dL;serum alkaline phospatase,844 IU/dL; stricture in thelower end of CBD withproximal dilatation anddilated PD with stricture inthe head of pancreas(ultrasonography, ERCP)

    ERCP and CBD stentingfollowed by lateralpancreaticojejunostomy andhepaticojejunostomy

    1 y; no recurrence

    5 6/F* Mild, recurrent pain inabdomen, 18 mo;recurrent uppergastrointestinal bleeding,5 mo

    No definite cause identified(UGIE); pseudoaneurysm ofgastroduodenal artery (DSA);pancreatic calcification(ultrasonography, CECT)

    Steel coil embolization of thepseudoaneurysm (DSA)

    1 y; pain free, nobleeding

    6 11/M Recurrent pa in in abdome n,5 y

    Low fecal chymotrypsin; dilatedPD (ultrasonography, CECT,and ERCP)

    Pancreatic enzymesupplementation

    6 mo; partial painrelief

    7 5/F Pain in abdomen, 2 mo Undetectable fecalchymotrypsin; dilated PD(ultrasonography and CECT);side branch changes (ERCP)

    Pancreatic enzymesupplementation

    1 y; pain free

    * Data from Agarwal et al. (40).CBD, common bile ducts; CECT, contrast-enhanced computed tomography; DSA, digital subtraction angiography; ERCP, endoscopic retrograde

    cholangiopancreatography; PD, pancreatic duct; UGIE, upper gastrointestinal endoscopy.

    FIG. 3. Abdominal contrast-enhanced computed tomographyscan (plain section) of a patient with chronic pancreatitis showingpancreatic ductal dilatation and calcification in the head and tailregions of the pancreas.

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    children (16). Viral infections can also cause AP (1720). One of our patients examined in 1993 for acutehepatitis (hepatitis B and C negative) had coexisting AP.Hepatitis A and E viral infections are common in devel-oping countries in this age group (21,22). The etiologicrole of hepatitis A and E cannot be ruled out in ourpatients because the viral markers for these infectionswere not done.

    Approximately 80% of AP episodes are mild and re-solve with supportive care (23). All our patients (n 6)had some complication (pseudocysts in 3; infected fluidcollection, pleural effusion, and ascites with high amy-lase in one patient each), which may be the result ofreferral bias. Ascites caused by PD disruption or inflam-matory exudation and pleural effusion caused by pancre-aticopleural fistula are known complications of AP (24).In children, blunt abdominal trauma is the most commoncause of PD disruption and development of various in-ternal fistulae. One of our posttrauma AP patients hadright-sided pleural effusion. Octreotide, a somatostatinanalog that reduces pancreatic secretions, has been usedto treat patients with PD disruption (25). Because ourpatient responded to a 7-day treatment with octreotide,ERCP was not done to look for pancreaticopleural fis-

    tula. Another child with AP thought to have acute hepa-titis had ascites with high amylase content and recoveredcompletely. Ascites in this case was attributed to inflam-matory exudation resulting from relatively low asciticfluid amylase (830 IU/dL) and quick recovery.

    Infected fluid collections and symptomatic pseudo-cysts after AP require drainage. Studies in adults show2% to 15% of AP patients develop pseudocysts, whichresolve spontaneously in 40% to 50% (2630). Pseudo-

    cysts >6 cm and remaining longer than 6 weeks rarelyresolve and are at high risk for development of furthercomplications (infection, hemorrhage, and rupture) and,therefore, require drainage (27,29,30). Pseudocysts canbe drained by surgery or by radiologic and endoscopicinterventions. Percutaneous drainage of pseudocysts inchildren with fluoroscopic guidance has shown good re-sults, but the experience is limited to small case series(3,4,31,32). Various approaches, like transperitoneal,transgastric, or retroperitoneal, have been used for per-cutaneous drainage. The transgastric approach has theadvantage of placing a cystogastric stent percutaneously(known as internalization) through the same access afterremoval of the external drainage catheter (3,33,34). Thismethod has been shown to reduce recurrence of pseudo-cysts (35).

    Endoscopic PD stenting has been reported in a fewchildren (5,6,10,36). In our patients, endoscopic PDstenting was a valuable intervention in two settings: 1) ithelped to direct pancreatic secretions through a naturalroute in children with communication between PD andfluid collection and pseudocyst; and 2) it successfullyrelieved pain symptoms in one CP patient (case 1; Table1). Pancreatic duct stenting relieves duct obstruction,which decreases parenchymal pressure and consequentlyresults in decrease of pancreatic pain in CP patients(5,37).

    Two of our CP patients (cases 3 and 4; Table 1) hadobstructive jaundice resulting from CBD strictures sec-ondary to CP. Barkin et al. (38) reported obstructive jaundice (resulting from CBD stricture) in 64% (18 of28) of patients with idiopathic fibrosing pancreatitis, andthe majority of these patients were treated surgically.Advances in therapeutic biliary endoscopy have facili-tated management of cholangitis for better results of de-finitive surgery at a later date (39). We adopted thisapproach of biliary stenting while managing cholangitisbefore surgery in one child (case 4, Table 1). Both ofthese patients (cases 3 and 4; Table 1) underwent biliarybypass surgery. Another CP patient (case 2; Table1) hadlarge PD calculi, pancreatic parenchymal calcification,and dilated PD. He underwent lateral Roux-en-Y pan-creaticojejunostomy and the PD calculi were removed.Radiologic steel coil embolization successfully blockedactive bleeding from the pancreatitis-induced pseudoan-eurysm (case 5, Table 1; earlier reported by us) (40).Two other CP patients did not require intervention andwere treated with pancreatic enzymes only (Table 1).

    Endoscopic retrograde cholangiopancreatography hasbeen used in adults on a large scale; however, this pro-cedure has only recently been accepted as a therapeuticmodality for children. At our center, there is a partner-ship between pediatric endoscopists and experiencedadult therapeutic endoscopists to perform pancreatico-biliary procedures in children. This kind of partnershiphas been very successful at our center and is in accor-dance with the recommendations of the subcommittee on

    FIG. 4. Endoscopic retrograde cholangiopancreatography of apatient with chronic pancreatitis showing dilated bile duct and

    intrahepatic biliary radicals caused by stricture in the lower part ofcommon bile duct.

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    endoscopy and procedures of the North American Soci-ety for Pediatric Gastroenterology and Nutrition (36).

    CONCLUSION

    Pancreatic disorders are rare in children. Managementof AP and CP involves a multidisciplinary approach.Radiologic and endoscopic interventions and surgery arecomplimentary in achieving successful management ofthe complications of childhood pancreatitis.

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    TABLE 2. Usefulness of therapeutic, endoscopic, and radiologic interventions in pancreatic disorders in children (n = 6)

    Serialno. Age (y)/sex Diagnosis

    Radiologicintervention

    Endoscopic findings;management

    1 10/M Posttrauma AP PCD followed by internalization Communicating pseudocyst; PD stenting2 5/M Posttrauma AP PCD followed by internalization Communicating pseudocyst; PD stenting3 14/F Gallstone AP PCD followed by internalization Communicating fluid collection in lesser sac;

    PD stenting4 11/M CP PD strictures and dilatation; PD stenting5 11/F CP and obstructive jaundice Lower CBD stricture; CBD stenting*6 6/F CP and recurrent UGI bleeding

    from pseudoaneurysmSteel coil embolization of

    pseudoaneurysmDiagnostic UGIE; no intervention

    * Followed by surgery.AP, acute pancreatitis; CBD, common bile duct; CP, chronic pancreatitis; PCD, percutaneous catheter drainage; PD, pancreatic duct; UGI, upper

    gastrointestinal; UGIE, upper gastrointestinal endoscopy.

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