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Does hepatic hilum morphology influence long-term prognosis in type I/I cyst biliary atresia?

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Abstract

Purpose

Some patients with cystic biliary atresia (BA) achieve exceptionally good postoperative courses. Early differentiation of pediatric patients with the favorable-type disease can lead to beneficial long-term postoperative management. We examined whether the hepatic hilum morphology affects long-term prognosis in type I/I cyst BA, atresia of the common bile duct with/without a cyst.

Methods

Of 253 BA patients identified since 1972, 40 were classified as having type I/I cysts and were divided into two subtypes according to hepatic duct size (subtype α: n = 18; duct diameter, ≥1 mm and subtype β: n = 22; duct diameter, <1 mm) to compare postoperative clinical courses.

Results

In subtypes α and β, jaundice disappeared in 16 (89 %) and 19 (86 %) patients, respectively (p = 0.81), and 13 (72 %) and 12 (55 %) survived with native livers (p = 0.18) at a mean age of 23.3 and 25.5 years, respectively (p = 0.42).

Conclusions

There were no significant differences in long-term outcomes between subtypes α and β, although approximately 40 % developed liver failure during the postoperative course. Thus, close long-term follow-up is essential in type I/I cysts, regardless of the hepatic hilum morphology.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryomachi, Aobaku, Sendai, 980-8574, Japan

    Masaki Nio, Motoshi Wada, Hideyuki Sasaki & Hiromu Tanaka

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  1. Masaki Nio
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  2. Motoshi Wada
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  3. Hideyuki Sasaki
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  4. Hiromu Tanaka
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Correspondence to Masaki Nio.

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Nio, M., Wada, M., Sasaki, H. et al. Does hepatic hilum morphology influence long-term prognosis in type I/I cyst biliary atresia?. Pediatr Surg Int 31, 931–936 (2015). https://doi.org/10.1007/s00383-015-3771-3

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  • DOI: https://doi.org/10.1007/s00383-015-3771-3

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