Bilateral dislocation of lens under complete voluntary control in Marfan's syndrome with cardiovascular anomaly.

THE syndrome originally described by Marfan (1896) under the name "dolichostenomalia" was subsequently called "Marfan's syndrome", but Achard (1902) suggested the term "arachnodactyly" because of the spiderlike appearance of the extended fingers. Since the orginal description of Marfan, well over 300 cases have been recorded in the literature. The condition is characterized by abnormal height, absence of subcutaneous fat, poorly developed musculature, long slender extremities-particularly the fingers and toes-abnormal extensibility of the joints with a tendency to contractures, pigeon or funnel-shaped chest, kyphosis, scoliosis, winging of scapulae, dolichocephalic skull with prominent supra-orbital ridges, high arched palate, malformation of the external ear, pes planus, elevation of the patella, and in some instances congenital abnormalities of the heart and lungs. Piper and Irvine-Jones (1926) first associated the syndrome with congenital heart disease. It is now generally accepted that the condition shows a strong hereditary tendency affecting both sexes and transmitted as a dominant characteristic. Over 60 per cent. of the cases of Marfan's syndrome reported in the literature presented ocular abnormalities, those most frequently found being bilateral subluxated lenses, iridodonesis, and a small pupil dilating poorly with mydriatics. Anomalies of the lids and lashes, nystagmus, and atrophy of the iris stroma were also reported. Spontaneous dislocation of the subluxated lens (Takei, 1912, Handmann, 1914; Wessely, 1919; and others) is probably due to faulty development of the zonule. The dislocations occur into either the anterior chamber or the vitreous cavity. Sometimes the fibres of the zonule remain attached to the lens capsule, in which case they may become much elongated. More usually, however, the fibres of the zonule became detached from the lens (Maggiore, 1924). Lenses dislocated into the vitreous cavity can occasionally be coaxed into the anterior chamber by assuming a certain posture and may be imprisoned there by contracting the pupil (" wandering " lens: Duke-Elder, 1940). A case of Marfan's syndrome is here presented in which cardiac disorder