Hepatocellular Cholestasis and Pruritus: Beyond Clinical Treatment

Introduction

Hepatocellular cholestasis in infants can be caused by hepatitis, alfa-1 antitrypsin deficiency, inborn errors of bile acid synthesis, drugs, or total parenteral nutrition. Occasionally, Alagille Syndrome and Progressive Familiar Intrahepatic Cholestasis (PFIC) may result in intrahepatic cholestasis, with an incidence of 1 in 90 000. The affected patients present in infancy or childhood with intermittent painless jaundice. The most apparent and debilitating symptom associated with this disorder is pruritus, which may be out of all proportion to the hyperbilirubinemia and often leads to self-mutilation from scratching. This results in a very poor quality of life, and treatment is very difficult because traditional drugs used to treat itching are typically ineffective [1] [2] [3].

Liver transplantation is an effective therapy for children with this condition, but due to the high comorbidities associated with this procedure, alternative techniques to treat the pruritus have been developed, consisting of diversion of the biliary flow, as this reduces the enterohepatic circulation and avoids liver damage from cholestasis, [1] [2] [3] [4]. Ileal exclusion (IE) seems to be a good choice to treat PFIC. It was primarily described by Hollands and consists of exclusion of the terminal ileum by means of an ileocolic diversion to interrupt the enterohepatic circulation.

This approach has the advantages of avoiding a stoma and the possibility to use it in a patient without a gallbladder, and additionally offers the possibility of reversing the intestinal transit if the procedure does not work or causes persistent diarrhea [2]. For these reasons we prefer this technique.

Due to the presentation of postoperative intussusception in one child with PFIC after ileal exclusion, we were impelled to make a modification to the original technique by adding a Noble procedure. The use of intestinal surgical plication to create controlled and predictable adhesions and treat and prevent adhesive intestinal obstruction was described in 1937 [5]. Based on that, we report 2 cases of PFIC with clinically intractable pruritus treated with IE and a Noble plication (Hollands modified technique).

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