Persistent Neonatal Cholestasis in a Preterm Infant
Stephen E. Messier, MD; Susan Duffek, MD and Gary Neidich, MD
Sanford Children’s Hospital, Sioux Falls, South Dakota
A 30-day old infant was transferred to a children’s hospital. He had been born at 32 weeks’ gestation and arrived having never been fed, on total parenteral nutrition (TPN) and intravenous lipid emulsion 20%, furosemide, acetazolamide, morphine sulfate, and ibuprofen.
On day 5 of hospitalization (day of life 35), laboratory test results demonstrated a total bilirubin level of 8.8 mg/dL and a conjugated bilirubin level of 0.8 mg/dL, which then progressed to 6.6 mg/dL and 2.8 mg/dL, respectively.
Liver ultrasonogram highlighting a suspected gallstone in biliary system (arrow).
Initial abdominal ultrasonography results on day of life 36 demonstrated a thickened gallbladder wall with sludge. The conjugated bilirubin level improved with initiation of feedings and the discontinuation of TPN. Weeks later, however, elevated transaminase and direct bilirubin levels prompted abdominal ultrasonography (A). The results suggested choledocholithiasis, which subsequently was confirmed with magnetic resonance cholangiopancreatography (MRCP) (B). Biliary distention resolved on ultrasonograms, implying passage of the stone.
Neonatal choledocholithiasis was first reported in very sick infants as early as the 1830s,1 but with advent of modern radiologic imaging, it is diagnosed more frequently. It is a rare cause of cholestatic disease in premature neonates. Risk factors include prolonged fasting, phototherapy, hemolytic disease, TPN, furosemide, and Escherichia coli sepsis.2
MRCP scan highlighting a gallstone in the common bile duct (arrow).
Ultrasonography can be technically challenging in neonates with dilated bowel loops. While MRCP is not commonly used in neonates, the results of a number of small studies have demonstrated its utility in assessing the common duct, gall bladder, and biliary tree.3,4
Philpott and colleagues reported on the largest cohort of pediatric patients to date in whom MRCP was used to evaluate cholestasis.5 Although only 5% of patients in this cohort were infants, the retrospective study highlighted the utility of MRCP in diagnosing the causes of cholestasis, finding that only 13% of MRCP scans were nondiagnostic, usually secondary to breathing artifact.
While symptomatic cases of choledocholithiasis commonly are treated surgically, a more conservative watch-and-wait strategy may be warranted for several days to see whether the stone passes on its own. In such cases, long-term follow-up to assess for recurrence is recommended.1
Our case highlights the usefulness of MRCP to arrive at the diagnosis of choledocholithiasis.
References
1. Hughes RG, Mayell MJ. Cholelithiasis in a neonate. Arch Dis Child. 1975;50(10):815-816.
2. Gertner M. Farmer DL. Laparoscopic cholecystectomy in a 16-day-old infant with chronic cholelithiasis. J Pediatr Surg. 2004;39(1):E17-E19.
3. Takaya J, Nakano S, Imai Y, Fujii Y, Kaneko K. Usefulness of magnetic resonance cholangiopancreatography in biliary structures in infants: a four-case report. Eur J Pediatr. 2007;166(3):211-214.
4. Peng SS, Li YW, Chang MH, Ni YH, Su CT. Magnetic resonance cholangiography for evaluation of cholestatic jaundice in neonates and infants. J Formos Med Assoc. 1998;97(10):698-703.
5. Philpott C, Rosenbaum J, Moon A, Bekhit E, Kumbla S. Paediatric MRCP: 10 year experience with 195 patients. Eur J Radiol. 2013;82(4):699-706.