Clinical History
A 13-day-old female term neonate was admitted in the ER due to jaundice, “dark” urine, decreased food intake and lower vitality. In the observing room she weighed 3315 g (loss 11.6% of NP). The laboratory examinations revealed conjugated hyperbilirubinemia, elevated G-GT and lactate levels; urine protein and raised leukocytes.
Imaging Findings
She was admitted to the neonatology for additional studies, of conjugated hyperbilirubinaemia, with several diagnostic hypotheses, including late sepsis probably with a concurrent urinary infection (UI).
An abdominal ultrasound revealed a gallbladder with stratified thickened wall (4 mm) with high Doppler signal. It contained sludge and calculus; the main biliary duct was dilated (2 mm). The imaging findings were compatible with an acute cholecystitis.
The patient was transferred to the intensive care unit (ICU), some days later the ultrasound was repeated.
Ultrasound examination documented moderate hepatomegaly; distended gallbladder, with
stratified thickened wall (5.6 mm) and high Doppler signal together with hyperecogenic foci within the wall, associated with comet tail artefacts. Luminal contents were sludge and
calculus, and at the infundibulum there was a motionless calculus - obstructive? The main
biliary duct was dilated. These findings were suggestive of emphysematous cholecystitis.
The diagnosis of emphysematous cholecystitis was surgically confirmed.
Mid-term follow-up was favourable and she returned home some weeks later.
Discussion
Direct-reacting hyperbilirubinaemia (composed mostly of conjugated bilirubin) is defined as a direct bilirubin level> 2 mg/dL or 20% of the total bilirubin. This finding is always pathologic and should be assessed. Direct-reacting bilirubin is not neurotoxic for the infant but indicates a severe underling disorder associated with hepatocellular injury and/or cholestasis. More common aetiologies of conjugated hyperbilirubinemia in neonates are hyperalimentation cholestasis, CMV infection, other peri-natal congenital infections (TORCH- toxoplasmosis, other [syphilis, hepatitis, zoster], rubella, cytomegalovirus, and herpes simplex), inspissated bile from prolonged haemolysis, neonatal hepatitis and sepsis.
The evaluation includes the serum levels of liver enzymes, bacterial and viral cultures, metabolic screening tests, hepatic ultrasound, sweat chloride test, and occasionally liver biopsy [1, 2].
In neonates, inflammatory gallbladder disease is often related with concurrent systemic illness [1, 2].
Cholecystitis, inflammation of the gallbladder, may be acute or chronic and, although rare in the paediatric population, this entity does occur and must be recognized.
US has up to 100% sensitivity and specificity in diagnosing acute cholecystitis, showing
gallbladder wall thickening (> 3 mm), hydrops and Murphy vesicular sign [3, 4].
Intra- and extrahepatic biliary ducts should be carefully measured to exclude ductal dilatation.
The common bile duct should measure less than 1 mm in neonates, less than 2 mm in infants until 1 year old, less than 4 mm in older children, and less than 7 mm in teenagers and adults [2].
Small non-shadowing gallstones and biliary sludge may be difficult to distinguish.
Neonatal cholelithiasis may resolve without intervention [4].
Continuing right upper quadrant pain (> 6 h) with typical sonographic features suggests acute acalculous (15%) or calculous (85%) cholecystitis.
Acute cholecystitis-related complications include pericholecystic abscess, gangrene, emphysematous cholecystitis, empyema, impacted gallstone leading to cystic duct obstruction (Mirizzi syndrome), duodenal obstruction due to eroded gallstone (Bouveret syndrome) or gallstone ileus [4].
Emphysematous cholecystitis is surgically managed, and for patients too ill for surgery a
percutaneous cholecystostomy tube may be engaged.
Differential Diagnosis List
Emphysematous cholecystitis. Sepsis was assumed to be related with UI.
Acute cholecystitis
Emphysematous cholecystitis
Final Diagnosis
Emphysematous cholecystitis. Sepsis was assumed to be related with UI.
