Duplication cyst of the pancreatic duct presenting as pancreatitis

Duplication cyst of the pancreatic duct presenting as pancreatitis


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P E R C U T A N E O U S E N D O S C O P I C G A S T R O S T O M Y (PEG) PLACEMENT IN CHILDREN WITH V E N T R I C U L O P E R I T O N E A L SHUNTS (VPS). JH Berman TS Gunasekaran, R Montes, JR Kraut. Division of Pediatric Gastroenterology and Nutrition, Lutheran General Children's Hospital, Park Ridge, IL. and Rockford Clinic, Rockford, IL

SUCCESSFUL DIAGNOSIS OF BILIARY ATRESIA BY CLINICAL, RADIOLOGICAL, AND PERCUTANEOUS LIVER BIOPSY EVALUATIONS (ID D'AGATA, IL MARTINEZ, JJ DAVIS,Division of Pediatric GI/Nutrition and Liver diseases, Valley Children's Hospital, UCSF-Fresno, Fresno, Calif). Extrahepatic biliary atresla (EHBA) is the most common indication for orthotopic liver transplantation (OLT) in children. Prior to OLT, most infants with EHBA undergo a perteentemstomy (PE). However, suecesful biliary drainage is achieved in close to 90% of patients only if PE is performed prior to 2 months of age. Traditionally, diagnosis is arrived at by a combination of clinieal,radinlogical,and percutaneons liver biopsy (PLB) evaluations. Recently, endoscopic retrograde cholangiopancrnatography (ERCP) has been advocated as a means by which to expedite the diagnostic process. We reviewed the outcomes of infants admitted to Valley Children's between 1992-96 with direct hyperbilirubinemla to determine how the diagnosis of EHBA was made, and the time frame in which it was reached. Twenty-four infants (13M, 1IF) were initially evaluated by ultrasound, and four were found to have a choledoeal cyst. The other 20 patients underwent diisopropyliminodiacetic acid scan (DISIDA), and PLB. Nine were diagnosed with EHBA, 10 with neonatal hepatitis, and I with delayed maturation of glucuronyl transferase. Of the patients with EHBA (6M, 3F): mean age at presentation was 64 + 3 days (R: 33-97 days), diagnosis of EHBA was made in 3 + 2 days. Ultrasound showed a gall bladder in 3 patients. DISIDA showed excretion at 24 hours in 2 patients. Although available, ERCP was not used to make a diagnosis in any of these patients. Of the 2 patients who had excretion on the DISIDA Scan despite having EHBA, 1 was diagnosed at PLB. The other patient was erroneously diagnosed with neonatal hepatitis (gall bladder on US, PLB consistent with hepatitis). Jaundice worsened, and repeat DISIDA 1 month later showed no excretion. All patients went to PE (mean 6 d from diagnosis). The outcome of the patients who underwent surgery was: OLT 3 alive and well, 4 with Successful and 2 with unsuccessful PE, awaiting OLT. Successful PE was determined by lack of jaundice at one year post PE. Ultrasound, radiological, and PLB evaluation allowed soecessful identification of the cause of conjugated hyperbilirabinemia in 23/24 infants (95.8%). ERCP may have been of help in only one ease in this series of patients.

Introduction: Placement of P E G ' s has become more common in pediatric patients with neurological impairment. There is little information about the safety of PEG placement in patients with VPS. Shunt disruption, malfunction or infection are all potential concerns. Methods: Five patients with VPS were referred for PEG placement between July 1995 and November 1996. Mean age was 43.0 mos (1-82 mos). All VPS were placed on the right side. Shunt placement preceded PEG by a mean of 21.3 months (10 days-6.5 years). All patients were significantly, developmentally delayed. Underlying diagnoses included carbon monoxide poisoning with persistent vegetative state, brain malformations (2), congenital hydrocephalus and meningitis. Results: All five patients underwent successful placement of P E G ' s under general anesthesia. Location of PEG in each case was to the left of midline. A 15 Fr. Bard, Surgitek or Wilson-Cook "pull-type" PEG was used. All patients had 24 hours of perioperative antibiotic coverage. Two patients developed brief ( < 24 hrs) fevers of up to 39~ postoperatively with negative cultures. At a mean follow-up of 8.8 months (1-16 mos) there have been no episodes of shunt infection or malfunction. One patient died from causes unrelated to PEG placement. Conclusion: P E G ' s may be safely placed in patients with previously placed VPS. The importance of VPS location and use of routine antibiotic prophylaxis needs to be explored.



HISTOLOGIC CHARACTERIZATION OF THE SQUAMOCOLUMNAR JUNCTION (SCJ) IN CHILDREN. J Bomstein "~ D Winesett*, E RuedaPedrszat, V Rhol] t, C Sullivan', P Rogers', V Piileiro-Carrem~, "Dept. of Pediatrics, q:)ept, of Pathology, WRAMC, Wash., DC; *Dept. of Pediatrics, NNMC, Bethesda, MD and ~ of Pediatrics, USUHS, Bethesda, MD Standard practice for pediatric esophagugastroduedenoscopy (EGD) has been to biopsy the esophagus at least 3 centimeters above the SCJ due to the concern that the histology of the distal 2-3cm of the esophagus may normally have characteristics of reflux. As part of a larger prospective study, standard pinch biopsies were obtained at the SCJ and 3cm proximally in the distal esophagus (DE) from all patients presenting for routine EGD. Results of the first 44 patients biopsied have been reviewed. Methods: A standardized questionnaire was used to obtain information regarding signs and symptoms of gastroesophageal reflux (GER) prior to EGD. Biopsies were reviewed by one of two pathologists in a blinded fashion to evaluate for histologic characteristics of GER esophagitis [presence of eosinophils (EOS), basal cell hyperplasia (BCH), elongation of papillae (EPAP)], and for intruepithelial lymphocytes. Results: Fourteen of 44 patients had EOS found in biopsies of the SCJ. Eight of these 14 had EOS found on biopsy of the DE. Only 2 patients had EOS found in the DE biopsy without EOS noted at the SCJ. EOS were statistically more likely to be found at either biopsy site when signs/symptoms of GER were the indication for EGD as compared to all other indications.

Dupllcatlon Cyst of the Pancreatic Duct Presenting A s Pnncreutitis. JF dr Rosario. WB Silverman, J Adkins, E Wiener. Depts. of Pediatric ,e, Adult Gastroenterology ,r Surgery, Children's Hospital of Pittsburgh,

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The only characteristic of esophagitis that was statistically different between the SCJ and the DE was the more frequent presence of EPAP at the SCJ (P--0.0034). Intraepithelial lymphocytes were noted in all biopsies. Conclusions: The presence of EOS in esophageal biopsies correlated highly with signs/symptoms of GER. The presence of EOS at the SCJ appears to be specific to patients with GER symptoms. Although not statistically significant in this preliminary report, the higher frequency of EOS at the SCJ versus the DE may represent a more sensitive measure of esophagitis in GER. More patients will be necessary to make any further conclusions.

VOLUME 45, NO. 4, 1997

Presbyterian Hospital, University of Pittsburgh Medical Center.

Background: Enteric duplication cysts involving the pancreas are exceedingly rare. They may easily be mistaken for an inflammatory pseudocyst or choledoehal cyst so careful interpretation of imaging studies and thorough evaluation, which may include an ERCP, are necessary. Clinical Vignette: We present an 1l-year old white male with chronic diffuse abdominal pain that did not radiate to the back or flanks, and no associated nausea, vomiting, diarrhea, or fever. He had no history of abdominal trauma or alcohol abuse, and bad not been on any medications. Past medical history was notable for a right thoracotomy during the first two weeks of life to remove a right posterior mediastinal cystic mass, not contiguous with the gastrointestinal tract, that appeared intraoperatively to be an esophageal duplication cyst. Pathology disclosed a mature teratoma. Physical examination revealed a well-appcaring male with a soft, nontender abdomen. He had no organomegaly, rebound tenderness or guarding. The rest of the examination was normal. Laboratory tests were normal except for elevated amylase (638 U/L) and lipase (515 U/L) levels. CT of the abdomen revealed a 4cm x 5era x 11cm cystic mass thought to be an inflammatory pseudocyst, which remained unchanged after two weeks of therapy for pancreatitis. A choledochal cyst was ruled out after a HIDA scan failed to show uptake of dye by the cyst. An ERCP disclosed a 2.5cm x 10cm fusiform cyst lateral to the pancreas emanating from the paocreatie duet, and compressing the biliary system. At surgery, an accesory pancreatic duct was identified. Biopsy of the cyst wall revealed mucus epithelium. The patient was diagnosed with benign congenital duplication cyst of the pancreas. Roux-Y-cystojejunostomywas performed to drain the cyst because the cyst was too adherent to the porta bepatis and head oftbe pancreas to allow for complete excision. He had an uneventful recovery with resolution of the cyst. Conclusion: Duplication cysts of the pancreas result from failure of an enteric diverticulum of the pancreatic duct to regress. Although they rarely give rise to symptoms, sufficiently large cysts may result in pancreatitis, as in our patient.