Patients with symptoms lasting for more than 1 week, aneurysmal dilatation more than 2 cm in diameter, and SMA stenosis are suitable candidates for surgical management.

Neurological crises do not occur in patients with tyrosinaemia type I treated with NTBC. We report an 8 month-old boy with severe neurological crisis after interruption of NTBC treatment including progressive ascending polyneuropathy and diaphragmatic paralysis, arterial hypertension, respiratory distress requiring mechanical ventilation who later also developed impaired liver function and tubulopathy. After re-introduction of NTBC the patient slowly regained normal neurological functions and recovered completely.

The use of an inverted limb in the bifurcated component of modular endografts may allow endovascular treatment in scenarios where there is insufficient space to deploy a standard bifurcated component. This design modification allows an adequate sealing zone between the iliac extension limbs and the bifurcated component.

No abstract