ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
Genetic disorders affecting dermal collagen, elastin or...
LD28.01 — Marfan syndrome
ICD-11 LD28.01 — Marfan syndrome
Marfan syndrome is a systemic disease of connective tissue characterised by a variable combination of cardiovascular, musculo-skeletal, ophthalmic and pulmonary manifestations. Cardiovascular involvement is characterised by 1) progressive dilation of the aorta accompanied by an increased risk of aortic dissection, which affects prognosis and 2) mitral insufficiency. Skeletal involvement is often the first sign of the disease and can include dolichostenomelia, large size, arachnodactyly, joint hypermobility, scoliotic deformations, acetabulum protrusion, thoracic deformity, dolichocephaly of the anteroposterior axis, micrognathism or malar hypoplasia. Ophthalmic involvement results in axile myopia, which can lead to retinal detachment and lens displacement.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: