There is considerable variability in the phenotype of<unk> , from mild features to severe systemic abnormalities. The primary manifestations of<unk>  are arterial tortuosity (winding course of blood vessels), widely spaced eyes (hypertelorism), wide or split uvula, and aneurysms at the aortic root. Other features may include cleft palate and a blue/gray appearance of the white of the eyes. Cardiac defects and club foot may be noted at birth. There is overlap in the manifestations of<unk><unk>  and Marfan syndromes, including increased risk of ascending aortic aneurysm and aortic dissection, abnormally long limbs and fingers, and dural ectasia (a gradual stretching and weakening of the dura mater that can cause abdominal and leg pain). Findings of hypertelorism (widely spaced eyes), bifid or split uvula, and skin findings such as easy bruising or abnormal scars may distinguish<unk><unk>  from Marfan syndrome. Affected individuals often develop immune system related problems such as allergies to food, asthma, hay fever, and inflammatory disorders such as eczema or inflammatory bowel disease. Findings of<unk>  may include: * Skeletal/spinal malformations: craniosynostosis, Scoliosis, spinal instability and spondylolisthesis, Kyphosis * Sternal abnormalities: pectus excavatum, pectus carinatum * Contractures of fingers and toes (camptodactyly) * Long fingers and lax joints * Weakened or missing eye muscles (strabismus) * Club foot * Premature fusion of the skull bones (craniosynostosis) * Joint hypermobility * Congenital heart problems including patent ductus arteriosus (connection between the aorta and the lung circulation) and atrial septal defect (connection between heart chambers) * Translucency of the skin with velvety texture * Abnormal junction of the brain and medulla (Arnold–Chiari malformation) * Bicuspid aortic valves * Criss-crossed pulmonary arteries
Loeys–Dietz syndrome
Ehlers–Danlos syndromes
Tietz syndrome
Marfan syndrome
Frank–Ter Haar syndrome
Crigler–Najjar syndrome
Letterer–Siwe disease
Wilson–Mikity syndrome