The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–3, 2014Copyright � 2014 Elsevier Inc.

Printed in the USA. All rights reserved0736-4679/$ - see front matter

http://dx.doi.org/10.1016/j.jemermed.2014.01.025

RECEIVED: 18 JuACCEPTED: 30 Ja

ClinicalCommunications: Pediatrics

AN UNUSUAL CASE OF AN ICTERIC INFANT WITH ABDOMINAL DISTENTION

Gabriel Wardi, MD, MPH,* Paul Ishimine, MD,*† Daniel Lasoff, MD,* Chao Yuan, MD,* andColleen Campbell, MD, RDMS*

*Department of Emergency Medicine, UC San Diego Health System, San Diego, California and †Division of Pediatric Emergency Medicine,Rady’s Children Hospital, San Diego, California

Reprint Address: Gabriel Wardi, MD, MPH, Department of Emergency Medicine, UC San Diego Health System,200 West Arbor Drive, San Diego, CA 92103

, Abstract—Background: Jaundiced infants are uncom-mon in most emergency departments (EDs). Biliary ruptureremains one of the more rare and less described causes ofthis condition. Case Report: A 5-month-old male presentedto our ED with scleral icterus, increasing abdominal disten-tion, and increased irritability. A bedside ultrasound re-vealed a moderate amount of ascites and further imagingsuggested he had a rupture of his common bile duct. Surgicalexploration confirmed this and revealed the presence ofcholedocholithiasis, which was the likely cause of therupture. Why Should an Emergency Physician Be Awareof This?: Biliary rupture remains a rare but serious condi-tion in very young patients. Emergency physicians shouldconsider bedside ultrasound as an adjunct in undifferenti-ated abdominal distention or jaundice in this patient pop-ulation. � 2014 Elsevier Inc.

, Keywords—biliary rupture; choledocholithiasis; jaun-dice

INTRODUCTION

Jaundiced infants are uncommon in most emergency de-partments (EDs) and many of the underlying conditionsresult in significant morbidity if untreated or misdiag-nosed. In neonates, jaundice is usually physiologic andis frequently associated with hemolysis and breastfeed-ing; in older infants, it is more frequently pathologic.

ne 2013; FINAL SUBMISSION RECEIVED: 31 Octobnuary 2014

1

The majority of these cases are due to unconjugated hy-perbilirubinemia, and common causes include hemolysisfrom sickle cell disease and hereditary spherocytosis.Less encountered and more concerning is jaundicecaused by conjugated bilirubin, which is typically seenin cases of biliary obstruction and direct hepatocellularinjury. Surgical etiologies of jaundice are rare in infantsbut are often the most serious. The most common ofthese, biliary atresia, accounts for approximately 80%of such cases, but other conditions include choledochalcysts, inspissated bile syndrome, and biliary rupture (1).

CASE REPORT

A 5-month-old male presented to our ED with increasedabdominal distention and poor feeding during the previ-ous month. His mother reported that during the previousfew weeks, he had become less active and, in the past day,was unable to tolerate oral intake. She also noted his eyeshad a yellowish tint in the past few days. He had been seen2 weeks earlier for ‘‘swelling’’ in his testicles, but had anultrasound that showed ‘‘only fluid.’’ His mothermentioned a single bowel movement, possibly withsome blood earlier in the day, but his review of systemswas otherwise negative. His medical history was signifi-cant for a premature birth at 29 weeks, followed by amonth-long course in a neonatal intensive care unit, dur-ing which time he was given total parenteral nutrition

er 2013;

2 G. Wardi et al.

(TPN). His family history was remarkable for sickle celltrait.

Initial vital signs included a rectal temperature of38.2�C, a heart rate of 177 beats/min, a respiratory rateof 70 breaths/min, and an oxygen saturation of 97% onroom air. He weighed 4.9 kg, appeared mildly cachectic,was icteric, and had a noticeably distended abdomenwithout evidence of hepatosplenomegaly. His stool wasguaiac positive. The remainder of his examination wasunremarkable. A bedside ultrasound revealed a largeamount of ascites, as seen in Figure 1. At this point, thepatient was transferred to the regional children’s hospitalfor admission and further diagnosis. It was extremelydifficult to obtain intravenous access in this patient, anda subclavian central venous catheter was eventuallyplaced for both venous access and laboratory draws.

Initial laboratory results revealed an alkaline phospha-tase of 671 U/L, an aspartate aminotransferase of 27 U/L,an alanine aminotransferase of 32 U/L, and lipase of 20U/L, a total bilirubin of 2.8 mg/dL, a direct bilirubin <0.1 mg/dL, an indirect bilirubin of 0.5 mg/dL, a whiteblood cell count of 18.0/mm3, and a C-reactive proteinof 18.1 mg/dL. A paracentesis was performed in which320 mL of bilious ascites was removed with a bilirubinof 15.2 mg/dL. The next day, a magnetic resonance chol-angiopancreatography was performed which revealedcholelithiasis, choledocholithiasis, and was suggestiveof a 2- to 3-mm biliary leak from the distal commonbile duct (CBD). The surgical service was consultedand believed that the patient required surgical exploration

Figure 1. Bedside ultrasound of the patient’s right upperquadrant. The arrow indicates the location of ascites, withthe gallbladder present just below this.

for definitive diagnosis and management. An intraopera-tive cholangiogram confirmed the presence of a 2-mmhole in the medial aspect of the CBD and a large gallstonejust distal to this. A cholecystectomy tube and a T-tube inthe 2-mm defect of the CBD were placed to aid withfurther drainage. He remained in the hospital for 1 month,and was discharged in good condition after a repeat chol-angiogram that revealed complete closure of the defect inthe CBD.

DISCUSSION

We present the case of a 5-month-old male with a ruptureof his CBD likely secondary to choledocholithiasis.There have been > 150 published cases of pediatricbiliary rupture in the literature since its initial descriptionin 1932 (2). It represents the second most common surgi-cal etiology of the jaundiced infant, behind congenitalbiliary atresia (which has a prevalence of 1 in 8000 to10,000 live births and accounts for up to 80% of the sur-gical causes of infantile jaundice) (1). The exact etiologyof perforation is uncertain, and numerous mechanismshave been proposed. The most accepted theory suggeststhat a congenital weakness of the biliary wall leads torupture when intraluminal pressures exceed a certainthreshold due to a temporary obstruction (congenital ste-nosis, gallstone, inspissated bile), which is most commonat the junction of the cystic duct and the CBD (3). Otherspostulate that the posterolateral blood supply of the CBDis more susceptible to ischemia, leading to increasedrupture at this site if there is any compromise (4,5).Infants typically present in the 1st to the 4th month oflife, although reports have ranged from 25 weeks to7 years (6). Approximately 80% of patients appearwell, with progressive jaundice, abdominal distention,and normal or acholic stools. A minority of patientswill present in extremis, with fever, vomiting, and perito-nitis (2). Typical laboratory findings include relativelynormal liver enzymes and bilirubin, but an elevated alka-line phosphatase. A bilirubin level in ascitic fluid that isgreater than serum levels is pathognomonic for the condi-tion. This is also an important distinction between thiscondition and biliary atresia and infantile hepatitis, asthe latter two will have abnormal liver transaminases. Ul-trasoundmay reveal generalized ascites or a localized andloculated fluid collection (7,8). The biliary tree istypically not dilated, as there is no obstruction; someeven postulate that the rupture of the biliary tree leadsto the formation of stones due to stasis distal to therupture (6). Scintigraphy, an imaging modality rarelyused in the ED, will confirm the leakage of fluid intothe abdomen. Management requires surgical correctionof the biliary leak, although there is no consensus intechnique. Clinical deterioration occurs secondary to

Icteric Infant with Abdominal Distention 3

dehydration, electrolyte abnormalities, and abdominaldistention. Treatment in the ED is focused on resuscita-tion if needed and arranging for definitive care. The useof bedside ultrasound in this case led to recognition ofthe severity of the problem and expedited the discoveryof the eventual diagnosis.

We believe that choledocholithiasis was the underly-ing cause of the biliary rupture. In addition to the stonein the CBD, therewas also evidence of cholelithiasis, sug-gesting that the gallstone in the CBD was likely presentbefore the rupture. Gallstones in the pediatric populationare uncommon, and even more so in infants. Data suggestthat 0.15% to 0.22% of children younger than 16 years ofage have gallstones, and a more recent study place prev-alence as high as 1.9% (9). Of these, approximately 2% to7% of these will have choledocholithiasis (10). It isthought that the increased prevalence of this disease inthe pediatric population is due to rising obesity in theUnited States and increased use of improved imagingtechniques. However, in the infant younger than 6months,gallstones are thought to be formed by TPN, prior abdom-inal surgery, sepsis, prematurity, bronchopulmonarydysplasia, hemolytic disease, malabsorptive disease pro-cesses, and certain medications (notably ceftriaxone andfurosemide) (11). The exact prevalence in this age rangeis uncertain, although it is exceedingly rare. In this partic-ular case, we believe that the patient’s prematurity andmonth of TPN likely contributed to the formation of thegallstones, which in turn led to the rupture of the CBD.

WHY SHOULDAN EMERGENCY PHYSICIAN BEAWARE OF THIS?

Jaundice in the infant is an uncommon presentation in theED. Although the exact etiology of the jaundicewill often

not be determined in the ED, providers should recognizethat bedside ultrasound could be used to expedite theworkup and begin appropriate management. This partic-ular case represents a rare cause of jaundice with < 200reports in the literature; definitive treatment and diag-nosis is often delayed due to unfamiliarity with thecondition.

REFERENCES

1. Davenport M, Betalli P, D’Antiga L, et al. The spectrum of surgicaljaundice in infancy. J Pediatr Surg 2003;38:1471–9.

2. Cotta PE, Virginia M, Yan J, et al. Conservative management ofspontaneous bile duct perforation in infancy: case report and litera-ture review. J Pediatr Surg 2012;47:1757–9.

3. Haller JO, Condon VR, Berdon WE, et al. Spontaneous perfora-tion of the common bile duct in children. Radiology 1989;172:621–4.

4. Evans K, Marsden N, Desai A. Spontaneous perforation of the bileduct in infancy and childhood: a systematic review. J Pediatr Gastro-enterol Nutr 2010;50:677.

5. Colver Hugh D. Perforation of the biliary tract due to gallstonesin infancy—an established clinical entity. Ann Surg 1964;160:226.

6. Banani SA, Bahador A, Nezakatgoo N. Idiopathic perforation of theextrahepatic bile duct in infancy: pathogenesis, diagnosis and man-agement. J Pediatr Surg 1993;28:950–2.

7. Bahia JO, Boal KB, Karl SR, et al. Ultrasonographic detection ofspontaneous perforation of the extrahepatic bile ducts in infancy.Pediatr Radiol 1986;16:157–9.

8. Siegel MJ. Jaundice in infants and children. Ultrasound Clin 2006;1:431–41.

9. Wesdorp I, Bosman D, Graaff A, et al. Clinical presentations andpredisposing factors of cholelithiasis and sludge in children. J Pe-diatr Gastroenterol Nutr 2000;31:411–7.

10. Poffenberger CM, Gausche-Hill M, Ngai S, et al. Cholelithiasis andits complications in children and adolescents: update and case dis-cussion. Pediatr Emerg Care 2012;28:68–76.

11. Klar A, Branski D, Akerman Y, et al. Sludge ball, pseudolithiasis,cholelithiasis and choledocholithiasis from intrauterine life to 2years: a 13-year follow-up. J Pediatr Gastroenterol Nutr 2005;40:477–80.