# Polysplenia syndrome complicated by multiple intrahepatic bile duct stones in an adult: a case report

**Authors:** Jiahao Zhang, Jiliang Lu, Hongzhong Liang, Pengyu Chen, Zheng Wang, Naiqing Liu, Jinghua Liu

PMC · DOI: 10.3389/fmed.2026.1704503 · Frontiers in Medicine · 2026-02-17

## TL;DR

This case report describes an adult with polysplenia syndrome and multiple bile duct stones, highlighting the importance of recognizing complex liver and spleen abnormalities.

## Contribution

The novelty lies in documenting a rare association between polysplenia syndrome and hepatobiliary complications in an adult.

## Key findings

- The patient had multiple congenital abnormalities including polysplenia and truncated pancreas.
- Surgical treatment included partial hepatectomy and bile duct exploration.
- Postoperative bacteremia was resolved with targeted antibiotics.

## Abstract

Polysplenia syndrome is a rare congenital disorder characterized by multiple spleens and complex visceral and vascular anomalies. We report an adult case admitted for multiple intrahepatic bile duct stones. Imaging revealed multiple congenital abnormalities, including polysplenia, a truncated pancreas, and absence of the superior inferior vena cava segment. Intraoperative findings confirmed extensive intrahepatic bile duct stones and biliary malformations. Surgical treatment included partial hepatectomy, bile duct exploration, and T-tube drainage. Postoperatively, the patient developed bacteremia, which resolved with targeted antibiotics. This case highlights the link between polysplenia syndrome and hepatobiliary anomalies, emphasizing the need for careful anatomical evaluation, early recognition of biliary complications, and close clinical monitoring in affected patients.

## Linked entities

- **Diseases:** bacteremia (MONDO:0005229)

## Full-text entities

- **Genes:** GUSB (glucuronidase beta) [NCBI Gene 2990] {aka BG, MPS7}
- **Diseases:** adhesions (MESH:D000267), PC (MESH:D015324), neonatal jaundice (MESH:D007567), choledocholithiasis (MESH:D042883), intrahepatic bile duct stones (MESH:D002780), pancreas (MESH:D010190), BA (MESH:D001656), biliary complications (MESH:D008107), inflammation (MESH:D007249), HL (MESH:C538324), inflamed (MESH:C531841), anomalies (MESH:D000013), cirrhosis (MESH:D005355), abdominal pain (MESH:D015746), PDPV (MESH:C563407), stone (MESH:D007669), hepatobiliary anomalies (MESH:D004066), atrophic (MESH:D020966), congenital heart disease (MESH:D006330), biliary maldevelopment (MESH:C538059), infection (MESH:D007239), calculi (MESH:D002137), splenic infarction (MESH:D013159), gallbladder and bile duct stones (MESH:D042882), chills (MESH:D023341), congenital (MESH:D008209), choledochal cysts (MESH:D015529), malignancy (MESH:D009369), biliary stasis (MESH:D002779), bacteremia (MESH:D016470), splenic developmental defects (MESH:D013158), jaundice (MESH:D007565), bile stasis (MESH:D014647), biliary malformations (MESH:C537726), cardiac involvement (MESH:D006331), cholangiocarcinoma (MESH:D018281), renal colic (MESH:D056844), nutcracker syndrome (MESH:D059228), biliary tract abnormalities (MESH:D001660), multiple spleens (MESH:D013160), obesity (MESH:D009765), intestinal malrotation (MESH:C562456), nausea (MESH:D009325), stone formation (MESH:D058426), congenital disorder (MESH:D009358), Polysplenia syndrome (MESH:D059446), cyst (MESH:D003560), vomiting (MESH:D014839), visceral and vascular abnormalities (MESH:D007418), bile (MESH:D001649), cholangitis (MESH:D002761), hemolysis (MESH:D006461), chronic (MESH:D002908), cardiac abnormalities (MESH:D018376), malformations (MESH:C564254), biliary strictures (MESH:D003251), cholelithiasis (MESH:D002769), vascular anomaly (MESH:D020785), fever (MESH:D005334)
- **Chemicals:** cholesterol (MESH:D002784)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC12953520/full.md

## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12953520/full.md

## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12953520/full.md

---
Source: https://tomesphere.com/paper/PMC12953520