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scientific articles

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

key information

source: Journal of the Royal Society of Medicine

year: 1979

authors: Phadke JG, Johnson VW, Young HB

summary/abstract:

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 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 and organo-axial torsion of the stomach.

Despite radiologically demonstrable persistence of the gastric torsion, she recovered from a total of nine episodes of vomiting treated conservatively over a seven-year period, but in 1977, when aged 71 years, operative intervention became imperative. In view of the tissue fragility it was decided to use a combination of Tanner’s methods of ‘keeling’ of the diaphragm followed by colonic displacement into the subphrenic space and gastropexy (Tanner 1968). The procedure was restricted to the transabdominal route because of her poor respiratory reserve and the operative risks.

organization: New Cross Hospital Wolverhampton

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