Introduction Autoimmune liver disease (AILD) in children encompasses Autoimmune Hepatitis (AIH), Autoimmune sclerosing Cholangitis (ASC) or, where there are features of both, overlap syndrome (OS). Differentiating between these entities requires cholangiography. The gold standard for the diagnosis of ASC is Endoscopic Retrograde Cholangiopancreaticography which is invasive. Magnetic Resonance Cholangiopancreaticography can be used as an alternative but is not universally available and may require anaesthesia in young children. Anecdotally, we had found increased gall bladder (GB) length on ultrasound to be a useful marker of bile duct involvement in AILD.
Aim Our aim was to study the role of ultrasound measurement of Gall Bladder length as a screening tool for identifying ASC in children with AILD.
Method Children, under the age of 18 years at the time of presentation, diagnosed with AILD were identified from the departmental database. Cases for whom a fasting ultrasound at the time of presentation, was available on our radiology department's digital imaging system were included. Cases were categorised using established criterion into 2 groups; those with AIH and those with ASC with or without OS. A retrospective case notes review was performed on all eligible children, their ultrasound scans were reviewed by a single radiologist and GB length measured.
Results 50 cases were included. 32/50 (64%) had AIH type1 and 6/50 (12%) had AIH type2. 12/50 (24%) had ASC and 6/12 (50%) of these had OS. The average age at presentation was 10.18 years (range 1.3–16 years). The age at presentation was similar for both groups (AIH 10.1 yrs; ASC 10.2 yrs). Overall there were 22 males and 28 females but among the group with ASC there were 8 males and 4 females. The duration of symptoms before diagnosis was 5 months and was similar in both groups. The median (25th, 75th centile) GB length in children with ASC was 9.75 cm (7.2 cm, 10.6 cm) and in children with AIH was 6.8 cm (5.5 cm, 7.9 cm) [p value 0.003]. The normal GB length in children older than 1 year is up to 7 cm. GB length >7 cm was seen in 9/12 (75%) children with ASC compared to 15/38 (39.5%) of children with AIH (p=0.04). GB length >9 cm was seen in 7/12 (58.3%) children with ASC and 4/38 (10.5%) children with AIH (p=0.005).
|GB length||Sensitivity (%)||Specificity (%)||+ve predictive value (%)||−ve predictive value (%)|
Conclusion Summary: In AILD GB length is significantly increased in children with ASC. GB length >9 cm has 90% specificity for the diagnosis of ASC. GB length <7 cm is 88% exclusive of ASC.
Conclusion: GB length measurement is a useful screening test for ASC in children with AILD. We would recommend a further study with greater number of patients.
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