Late-presenting cholangitis and Roux loop obstruction after Kasai portoenterostomy for biliary atresia

Biliary atresia: A transplant perspective

“…In selected cases, revision of the HPE may be useful in relieving partial and/or complete obstruction that may predispose to the refractory disease 46, 47. Hepatobiliary scintigraphy may identify circumstances under which revision may be successful 48. LT may be indicated as a means of treating highly recurrent and/or refractory disease.…”

Single-balloon enteroscopy-assisted endoscopic retrograde cholangiopancreatography for treatment of cholangitis in a patient with a Kasai portoenterostomy

“…Indeed, successful dilation of strictures using this technique has been reported for patients with a hepaticojejunostomy [8]. Finally, for those cases caused by an obstruction of the afferent limb, single-balloon enteroscopy could potentially diagnose the obstruction and treat it with balloon dilation [12].…”

“…The mechanism for this complication is not entirely clear, but likely contributors include intestinal bacterial stasis and incomplete biliary drainage [11]. Afferent limb intestinal obstruction at the mesocolic window has also been reported to cause cholangitis [12]. In addition to its immediate morbidity, episodes of cholangitis have been associated with worse outcomes and progression of portal hypertension, although this association has not been consistently reported [10,13].…”

Medical management of the 'failing' Kasai portoenterostomy

“…In such children, ultrasonography plays a limited role and hepatobiliary scintigraphy is indicated to assess biliary excretion and possible mechanical problems with bile flow. These children could benefit exceptionally from surgical exploration and refashioning of the Roux-en-Y loop, 12 consequently avoiding early LT. More frequently, the late-onset jaundice represents a sign of incipient hepatic decompensation and heralds urgent consideration for LT.…”