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Postnatal management of prenatally diagnosed biliary cystic malformation
Hiromu Tanaka1, Hideyuki Sasaki1, Motoshi Wada1
1Department of Pediatric Surgery, Graduate School of Medicine, Tohoku University, Sendai, Japan.
Insights
Prenatal diagnosis of biliary atresia (BA) and choledochal cyst (CC) can be challenging. Ultrasonography can suggest BA if cyst size decreases and the gallbladder atrophies postnatally.
Area of Science:
- Pediatric Surgery
- Diagnostic Imaging
- Hepatobiliary Diseases
Background:
- Prenatal diagnosis of biliary atresia (BA) and choledochal cyst (CC) has improved with advanced ultrasonography.
- Distinguishing between BA and CC prenatally or neonatally remains difficult.
Purpose of the Study:
- Investigate diagnostic and therapeutic challenges in prenatal diagnosis of BA and CC.
- Evaluate ultrasonographic findings for differentiating BA and CC.
Main Methods:
- Retrospective study of 10 patients (4 BA, 6 CC) with hepatobiliary cysts.
- Sequential ultrasonographic assessment of cyst size and gallbladder morphology from diagnosis to surgery.
Main Results:
- Cyst size decreased between birth and surgery in BA, but increased in CC.
- Gallbladder atrophy was observed in BA cases.
- No significant differences noted in gestational age, prenatal cyst size, changes between birth and surgery, or liver fibrosis.
Conclusions:
- Decreasing cyst size and gallbladder atrophy postnatally suggest BA.
- Cholangiography is essential for definitive differentiation between BA and CC.
- Neonatal surgery is recommended for CC presenting with icterus and liver dysfunction.
Purpose:
Recent advances in ultrasonography have increased prenatal diagnosis of biliary atresia (BA) and choledochal cyst (CC). These conditions are not easy to distinguish before or just after birth. This study investigated diagnostic and therapeutic problems in prenatal diagnosis of BA and CC.
Methods:
We retrospectively studied clinical characteristics and progression of hepatobiliary cysts in 10 patients (4 cases of BA, 6 cases of CC) from the time of diagnosis. Chronological changes in cyst size and gallbladder morphology were assessed and measured sequentially by ultrasonography.
Results:
Three cases of BA were type I cyst and 1 case was type III-d. All cases of CC were type Ia. Cyst size decreased between birth and surgery in BA but increased in CC. The gallbladder appeared atrophic in BA. There was no significant difference in gestational age or cyst size at prenatal diagnosis, changes in cyst size between birth and surgery, and degree of liver fibrosis.
Conclusions:
BA should be suspected if cyst size decreases before and after birth and the gallbladder atrophies after birth. Cholangiography is the only reliable method to differentiate BA from CC. Neonatal surgery is indicated for CC with icterus and liver dysfunction.
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