Background: Alagille´s Syndrome (AS) is a rare and inherited disorder. We report the case of a patient with AS and an acute upper gastrointestinal haemorrhage.
Case report: A 6 year-old 20 kg female with AS presented to the emergency service with one-day history of hematemesis. After evaluation was decided to make an exploration by the ORL team ( beacuse a recent history of Upper Arway infection) and later an upper esophagogastroduodenoscopy in an attempt to find the location of the bleeding. She was asymptomatic, alert and oriented. The physical examination revealed she was pale with normal arterial blood pressure and a taquicardia of 130 cpm. The cardiac, respiratory and central nervus system examination were unremarkable.The liver was enlarged. Mallampati test was I. Laboratory test revealed: anemia with Hb 6,2gr.dl, ,normal platelet count, liver function was normal as was seric Bilirubin.Creatinin 1.08 Urea 0.6. PT 13,8/ APTT 32,6
She underwent blanced general anaeshtesia.Was induced with propofol and fentanyl Iv and mantained with sevoflurane and remifentanil. An endotraqueal tube was inserted after Cistaracurium 0,15mg/Kg. No bleeding was found in the upper arway. The upper gastrointestinal endoscopy revealed bleeding with clots formation in the gástric chamber. Argon-plasma was applied in two angioectasias. The procedure was performed without complications and the patient was transferred to pediatric ICU.
Discussion: The main clinical features of AS are chronic cholestasis, congenital heart disease, butterfly vertebrae, characteristic fácies and posterior embriotoxon. The Anesthesia plan should be based on: The careful preoperative assesment of the airway and neck mobility. A ditailed physical examination.The choice of drugs should be careful and use those wich showed no changes in liver function.A muscular relaxante whose metabolism does not derectly depend on the kidney or liver is advisable in this situation.
References: Alagille syndrome and anesthesia management. Tulay S. Yildiz, Nurcan O. Yumuk, Duygu B, Mine S, Kamil T. Pediatric Anesthesia 2007 Vol: 17 (1) :91-92. 
Learning points: The anaesthetic teams faces the challenge to dealing with a not so common disese.With the need for an urgent intervention, the patient assesment was based on a detailed physical examination and laboratory test. There was a careful choosing of drugs and a concern for mantaining hemodynamic and hematological stability together with good control of body temperature.

A child with Alagille´s syndrome to undergo an urgent procedure: wich are the key points for a well suceeded anaesthetic management?