title: Occult small bowel perforation in a patient with Ehlers Danlos syndrome: a case report and review of the literature.
abstract: Patients who present with a co-existing connective tissue disorder add a degree of complexity to operative intervention. We present an unusual case of a 53-year-old Caucasian female patient with Ehlers Danlos syndrome who presented with an occult perforation of the distal ileum. The patient had known small bowel diverticulae yet the perforation occurred within the normal bowel wall. The pre-operative CT only showed malrotation of the large bowel and did not correlate with the intra-operative findings. Our case has highlighted that although small bowel perforation is a rare occurrence, it may be more common in Ehlers Danlos and may present with atypical features. Perforation may also occur alongside normal bowel as well as diverticulae within the bowel. Where diverticulae exists within a patient with Ehlers Danlos syndrome and there is some diagnostic uncertainty, there should be a lower threshold for operative intervention. We present in the discussion a number of salient features and learning points.
authors.affiliation: Department of General and Colorectal Surgery, Princess Royal University Hospital, Farnborough Common, Orpington, Greater London, Kent, BR6 8ND, UK. tasneemtarun@hotmail.com.
authors.forename: Tessa Frances
authors.initials: TF
authors.lastname: Leake
authors.forename: Tarun
authors.initials: T
authors.lastname: Singhal
authors.forename: Aninda
authors.initials: A
authors.lastname: Chandra
authors.forename: Alexandra
authors.initials: A
authors.lastname: Ashcroft
authors.forename: Sudeendra
authors.initials: S
authors.lastname: Doddi
authors.forename: Abdulzahra
authors.initials: A
authors.lastname: Hussain
authors.forename: Frank
authors.initials: F
authors.lastname: Smedley