Ehlers-Danlos Syndrome Presenting with Torsion of Stomach

Ehlers-Danlos Syndrome with Surgical Repair of Eventration of Diaphragm and Torsion of Stomach

“…A 63-year-old female, who presented in 1970 with vomiting, was found to have type I Ehlers-Danlos syndrome which is the classic severe type inherited as an autosomal dominant trait. When she was first reported to the Royal Society of Medicine in 1974 (Linnemann 1975), there was a history of previous poor wound healing and life-long kyphoscoliosis (Figure 1) with the development of premature osteoarthrosis. She had skin hyperextensibility, calcified subcutaneous spheroids, kyphoscoliosis (with later development of C4-5 subluxation), genu valgum, eventration of the left diaphragm (Figures 1 & 2) and organo-axial torsion of the stomach (Figure 3).…”