Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is autosomal recessiveand clinically characterized by soft extensible skin, laxity ofjoints and kyp...

The presence of supernumerary teeth, also known as hyperdontia, is the condition of having teeth in addition to the regular number of teeth. The occurrence of multiple supernumerary teeth is often found in association with syndromes such as Gardner syndrome, Anderson-Fabry disease, Ellis-van Creveld syndrome, Ehlers-Danlos syndrome, incontinentia pigmenti, and Tricho-rhino-phalangeal syndrome. ...

Surgical management of ectopia lentis is one of the major challenges faced by cataract surgeons today. Ectopia lentis signifies a displacement or malposition of the crystalline lens, irrespective of cause. It may occur congenitally or as part of developmental anomalies, as found in Marfan syndrome, homocystinuria, Ehlers-Danlos syndrome, hyperlysinemia, sulfite oxidase deficiency, simple primar...

Sir, There are several hereditary connective tissue disorders (CTDs), which occur due to mutation of specific genes. Common CTDs, which may present to a dermatologist, include Marfan syndrome (MFS) and Ehlers–Danlos syndrome (EDS); hence, we need to become acquainted with the diagnostic clinical features of these conditions. However, on rare occasions, patients may present with overlapping feat...

A 38-yr-old man with an unusual type of Ehlers-Danlos syndrome presented for elective abdominal aortic aneurysm repair. During surgery he developed acute myocardial ischaemia, resulting in abandonment of the procedure. He was shown subsequently to have severe triple vessel coronary artery disease. Silent ischaemia associated with severe coronary artery disease, although rare, may be associated ...

Q1: There was extensive cystic bullous involvement of lungs in both the cases, what could be the possible diagnosis? In the first case a diagnosis of cutis laxa—that is, Ehlers-Danlos syndrome—was made in view of elastic skin, past history of being operated for bilateral inguinal hernia, aphakic eyes, and presence of mitral valve prolapse. In the second case the diagnosis of Marfan’s syndrome w...

Ehlers-Danlos syndrome hypermobility type (EDS-HT) is an underdiagnosed genetic connective tissue disorder that causes joint hypermobility and widespread pain. We present a patient with the chief complaint of shoulder pain, a long history of widespread joint pain, and associated comorbidities. EDS-HT provided a unifying diagnosis and direction for management.

We present three cases of recurrent instability of the elbow in association with the Ehlers-Danlos syndrome. The pattern of instability has not previously been reported. We describe our procedure for achieving stability using a bone graft to the olecranon fossa which gave a functional range of movement.

Aortocaval fistula (ACF) is a rare complication of spontaneous abdominal aortic aneurysm (AAA) rupture, with an incidence of 2-4%. A unique case of ruptured AAA complicated by multiple aortovenous fistulas involving the inferior vena cava and left internal iliac vein is presented, and is the first published report of a patient with Ehlers-Danlos syndrome undergoing surgical treatment for an ACF.

Ehlers-Danlos syndrome-hypermobility type (EDS-HT) is a connective tissue disorder associated with chronic musculoskeletal pain. The diagnosis is based on simple clinical examination, although it is easily overlooked. Herein we present a case of EDS-HT associated with hemorrhoids and suggest that there may be an association between the two conditions.