The study was designed to evaluate the long term morbidity of Choledochal Malformation (CM) operated children, by associating clinical problems with liver histology, monitoring imaging, headache, and biochemical.

A one-center follow-up study with all of the patients treated with CMs was conducted. For statistical analysis, the Mann-Whitney U test and Spearman rank correlation were used. Long-term problems occurred in 21% of patients, including cholangitis in 9 individuals, anastomotic stricture in 2 referred patients, and adhesive volvulus or hepatocellular cancer in 1 patient each. Nonoperative management of anastomotic strictures and thermoablation of hepatocellular carcinoma were both successful.

The diameters of both main intrahepatic ducts had shrunk dramatically to 3.0mm in postoperative magnetic resonance cholangiography (MRCP) done 6.4 years after hepaticojejunostomy, although a distal cyst stump was still present in 30% of patients with a length of 6.0mm, which was linked with surgery age and fusiform CM type. Follow-up ultrasound revealed modest dilatation of intrahepatic bile ducts in 6.3%, mildly to moderately abnormal liver biochemistry in 23%, and liver stiffness in 22%. While cholangitis was the most prevalent postoperative concern, some patients had more serious problems, and one-quarter of patients had signs of underlying liver failure.

Reference: https://journals.lww.com/jpgn/Abstract/2021/06000/Long_term_Morbidity_of_Choledochal_Malformations.9.aspx

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