Aagenaes syndrome, also called lymphoedema cholestasis syndrome 1 (LSC1), is 
characterized by neonatal intrahepatic cholestasis, often lessening and becoming 
intermittent with age and severe chronic lymphoedema, mainly affecting the lower 
extremities. The condition is autosomal recessively inherited, and the gene is 
located on chromosome 15q. The locus, LCS1, was mapped to a 6.6 cM region on 
chromosome 15. Angioid streaks are visible irregular crack-like dehiscences in 
bruch's membrane that are associated with atrophic degeneration of the overlying 
retinal pigment epithelium. Angioid streaks have been described to be associated 
with pseudoxanthoma elasticum, paget's disease, sickle-cell anaemia, acromegaly, 
Ehlers-Danlos syndrome, and diabetes mellitus, but also appear in patients 
without any systemic diseases. Patients with angioid streaks are generally 
asymptomatic, unless the lesions extend towards the foveola or develop 
complications such as traumatic bruch's membrane rupture or macular choroidal 
neovascularization.