Insights into the pathophysiology and classification of type 4 choledochal malformation
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In brief
In brief
This study of 37 pediatric patients proposes subdividing type 4 choledochal malformations into cystic (4C) and fusiform (4F) subtypes based on distinct pathophysiological profiles. The cystic subtype presents with higher choledochal pressure and younger age, while the fusiform subtype shows elevated bile amylase and older presentation age.
Written by the GCMD Library team from the article.
Abstract
Introduction
Type 4 choledochal malformations (CMs) can be defined as extra- and intrahepatic biliary dilatation. They are of uncertain etiology but make up about 20% of most series. The aim of this study was to investigate the pathophysiology and their natural history following surgical intervention.
Methods
Ambispective review of a single-center series of type 4 CM between 1996 and 2017. Perioperative imaging, intraoperative pressure monitoring, bile amylase, and long-term follow-up data were analyzed based on extrahepatic morphology [(cystic (C), fusiform (F)]. Data were expressed as median (range). Statistical analysis was performed with nonparametric tests. P < 0.05 was considered significant.
Results
37 children had type 4 CM in the study period [age at surgery 4 (0.2–16) years] and could be further subdivided into cystic (4C) (n = 22) or fusiform (4F) (n = 15). There was significantly greater dilatation of the extrahepatic component in the cystic group [30 (11–94) versus 15 (8–90) mm; P = 0.0002] though there was no difference in left duct diameter [cystic 7.5 (0–17) mm versus fusiform 7.5 (3–16) mm; P = 0.86]. There was a trend to higher choledochal pressure in the cystic group [19 (4–40) versus 9 (6–25) mmHg; P = 0.09] and those in the fusiform group had higher bile amylase [8650 (3–890,000) versus 592 (1–123,000) IU/L; P = 0.01] and were older [4.1 (0.92–16.43) versus 2.4 (0.15–15.48) years; P = 0.03]. Children with type 4 CM were then separated simply on the basis of bile amylase into LOW (characterized by high pressure, cystic morphology and young age at surgery) and HIGH bile amylase (low pressure, fusiform morphology and older at time of surgery).
Conclusions
We propose further division of type 4 CM into 4C and 4F on clinical and pathophysiological grounds.
Level of evidence
II (prospective cohort).