Alagille Syndrome

Other Names

• Arteriohepatic dysplasia

Airway

• Pointed mandible

Cardiovascular

• Pulmonary stenosis
• Peripheral pulmonary stenosis
• Occ. TOF, ASD, VSD, Coarctation

Gastrointestinal

• Intrahepatic biliary atresia, hypoplasia
• Cirrhosis
• Esophageal varices

Endocrine

• Hypercholesterolemia
• Hyperlipidemia

Neurological

• Intracranial hemorrhage
• Hepatic encephalopathy
• Carotid/intracranial aneurysms

Musculoskeletal

• Growth retardation

Considerations

• Cardiac, hepatic, renal status
• SBE prophylaxis
• Bleeding issues