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BACKGROUND - In our institutional experience treating pediatric choledochal cysts over the past 12 y, we noted that seven of 32 patients (21.9%) had comorbid congenital cardiac anomalies. This association has not been previously described other than in isolated case reports. We aimed to quantify this association on a national level.
MATERIALS AND METHODS - We queried the 2009 Healthcare Cost and Utilization Project Kids' Inpatient Database. We identified patients with a diagnosis of choledochal cyst (ICD-9-CM 75169, 75162, and 75160) or biliary atresia (75161). We defined cardiac anomalies using the Clinical Classification Software code (CCS 213). Comorbid choledochal cysts or biliary atresia and congenital cardiac anomalies were quantified in both infant (<12 mo) and child (1-18 y) subpopulations.
RESULTS - Of 1646 estimated discharges for patients with choledochal cysts, 506 (30.7%) were for patients who also had congenital cardiac anomalies, compared with 2.6% in the general hospitalized population (χ(2); P < 0.001). The frequency of congenital cardiac anomalies was lower in 1973 hospitalizations for biliary atresia (13.8%) than in those for patients with choledochal cysts (χ(2); P < 0.001). We detected cardiac anomalies in 44.9% of choledochal cyst hospitalizations for infants <12 mo (versus 3.44% general hospitalized population; χ(2); P < 0.001), but in 6.9% of children ages 1-18 y (versus 1.3% general hospitalized population; χ(2); P < 0.001).
CONCLUSIONS - We observed a strong association between pediatric choledochal cysts and congenital cardiac anomalies that commonly manifests in infancy. When choledochal cysts are diagnosed either prenatally or in infancy, we suggest echocardiographic screening for cardiac anomalies, which may affect the timing of surgery and anesthetic planning.
Copyright © 2012 Elsevier Inc. All rights reserved.
PURPOSE - Given evolving imaging technologies, we noted significant variation in the diagnostic evaluation of pediatric choledochal cysts (CDC). To streamline the diagnostic approach to CDC, and minimize associated expenses, we compared typing accuracy and costs of ultrasound (US), intraoperative cholangiography (IOC), and magnetic resonance cholangiopancreatography (MRCP).
METHODS - Records of 30 consecutive pediatric CDC patients were reviewed. Blinded to all clinical data, two pediatric radiologists reviewed all US, MRCPs, and IOCs to type CDCs according to the Todani classification. When compared with pathologic findings, the concordance between and accuracy of each diagnostic test were determined. Inflation-adjusted procedure charges and collections for imaging modalities were analyzed.
RESULTS - Mean typing accuracy overlapped for US, IOC, and MRCP. Inter-rater reliability was 87 % for US (κ = 0.77), 80 % for IOC (κ = 0.62), and 60 % for MRCP (κ = 0.37). MRCP procedure charges ($1204.69) and collections ($420.85) exceeded IOC and US combined ($264.80 charges, p = 0.0002; $93.40 collections, p = 0.0021).
CONCLUSION - Our data support the use of US alone in the diagnosis of pediatric CDC when no intrahepatic biliary ductal dilatation is visualized. However, when dilated intrahepatic ducts are encountered on US, MRCP should be utilized to distinguish a type I from a type IV CDC, which may alter the operative approach.
A 10-month-old, previously healthy boy presented with one week of mild jaundice, light-colored stools and irritability. Abdominal sonography showed a large type I choledochal cyst and a separate, distinct cystic mass at the head of the pancreas. Magnetic resonance cholangiopancreatography was performed to evaluate the relationship of the two lesions. A type I choledochal cyst was confirmed, and a coexisting type III choledochocele was identified as the second cystic structure in conjunction with pancreaticobiliary malunion. The infant had complete resection of the type I choledochal cyst with Roux-en-Y hepaticojejunostomy, and anterior duodenotomy with marsupialization of the choledochocele. After five years of follow-up, the child is thriving and has had no recurrence of his symptoms. An exhaustive review of the literature identifies only one previous case of synchronous types I and III choledochal cysts, and this association is not clearly defined among the traditional classifications of type IV multiple choledochal cysts. Because operative management of a type III cyst requires the addition of a transduodenal approach, we encourage accurate reporting of mixed type choledochal cysts for the benefit of surgical planning, epidemiologic tracking and outcomes.