A 53-year-old Caucasian female with Ehlers Danlos syndrome presented with abdominal pain and vomiting to the Emergency Medicine Department. The pain was described as aching in character, initially beginning in the left iliac fossa and radiating to the left upper quadrant. The patient was unable to keep food down and had several episodes of vomiting over the course of the previous 24 hours. She felt distended but the bowel motion was unchanged with normal stools on the day of admission and no rectal bleeding. The patient recounted feeling feverish prior to admission. She had a previous history of diverticular disease and had seen her general practitioner (GP) four days earlier with abdominal pain. Her GP had started her empirically on oral Cefalexin and Metronidazole. Her past medical history consisted of diverticulosis within both the small and large bowel diagnosed on contrast studies (Figure ). She also had oesophagitis. The patient had also had two previous caesarean section deliveries which were uncomplicated and a right-sided inguinal hernia repair. The patient was neither a smoker nor drinker. The only history relating to her Ehlers Danlos was of thin skin and an increased bruising tendency, with mild difficulty in using her hands since childhood with occasional finger locking and decreased hand strength. There was no significant family history and none of connective tissue disorders.
This is a gastrograffin meal highlighting diverticulae in the duodenum, performed six weeks prior to admission for further evaluation of oesophagitis.
On examination the abdomen was soft and minimally tender with no guarding, rigidity or rebound tenderness. Bowel sounds were present. Rectal examination revealed soft stool in the rectum. Chest radiograph was normal and abdominal films showed distended bowel loops. The patient's bloods on admission showed a normal white cell count of 6.9 and a raised C - reactive protein of 491. The other bloods were normal. The surgical team organised admission and converted her to intravenous antibiotics and fluids.
Over the next 48 hours, the abdominal pain continued to increase and was associated with intermittent vomiting with an inability to eat or drink. C - reactive protein remained elevated. The patient continued to feel distended yet bowels continued to open normally. A repeat abdominal radiograph revealed progressive bowel distension so a CT scan was requested. This was performed the same day and suggested malrotation of the large bowel with a dilated caecum lying under the diaphragm on the left hand side and the ascending colon lying to the left of the midline (Figure ). Multiple loops of dilated jejunum and ileum were present. Engorged vessels were present in the root of the mesentery and tortuosity of the mesenteric vessels was also noted. There was no ascites or free intraperitoneal air and no obvious ischaemia of the bowel.
This is a CT Scan showing malrotated large loopy colon and distended small bowel, taken one day before operation performed on the patient.
In view of the continuing deterioration in conjunction with the imaging, a laparotomy was performed. Malrotation of the bowel was found as per the CT findings and the caecum was fixed in the left hypochondrium (Figure ). Numerous diverticulae in the duodenum were also noted (Figure ). Multiple perforations sealed with omentum were seen within a six-inch segment of distal ileum without perforations of diverticulae as such. The perforations occurred within relatively normal bowel (Figure ). There was massive dilatation of the small intestine with huge multiple diverticulae especially in the jejunum and proximal ileum (Figure ). Adhesions were then divided with sharp dissection and the bowel wall was noted to be extremely fragile. Serosal tears were evident with minimal handling (Figure ).
This is an intra-operative image showing malrotation with the caecum fixed to the left hypochondrium.
This is an intra-operative image showing large, thin walled, diverticulae in the duodenum with bowel contents visible through the wall of the diverticlae.
This is an intra-operative image showing the perforations were in the segment of distal ileum with no diverticulae.
This is an intra-operative image showing proximal ileum and jejunum showing large, thin walled diverticulae, which were fluid filled.
The small intestine segment with the multiple perforations was resected. A hand sutured double-layer anastomosis was performed. The mesenteric defect was also closed. Haemostasis was obtained and an abdominal lavage performed with two litres of warmed normal saline. A corrugated drain was brought out through the right iliac fossa. Mass closure was performed with loop 0 Nylon. Wound lavage was performed and clips used to appose skin.
Post-operatively, intravenous antibiotics were prescribed, and the patient was admitted to the intensive care unit. Total parenteral nutrition was started and the patient kept nil by mouth. Her bloods showed a decreasing CRP from 175 to 32 and her WCC remained stable. The patient's haemoglobin dropped to 6.0 gm/dl post-operatively while her platelets climbed to 1532. The patient refused a blood transfusion. Also during the post-operative period there was a developing mild reduction in peripheral oxygenation noted incidentally with accompanying blue hands and lips.
The patient made a good post-operative recovery with discharge to the ward on day 4 with commencement of a normal diet by post-operative day 7. Resumption of normal bowel habit began and patient was discharged home. Follow-up over the next three months confirmed no other complications.
Histology revealed a segment of small intestine measuring 16 cm in length × 3 cm in diameter. The serosal surface was noted to be haemorrhagic, with several perforations, the largest measuring 1.0 × 0.5 cm with associated white exudate on the serosa. The mucosa shows a submucosal haemorrhage measuring 1.0 × 1.0 cm, which was 4 cm from its closest margin. The second specimen revealed an omental mass with an appearance of white tissue measuring 2.5 × 1.5 × 1.0 cm.
On microscopy, the sections of small bowel show submucosal oedema and vascular dilatation with focal haemorrhage and perforation and organising inflammation in the serosal surface. The mucosa was preserved and showed no significant abnormality. The resection margins showed viable small bowel. There was no dysplasia or malignancy and there was no thrombus in the sections of the mesenteric vessels.
Further sections of the omental mass show organising inflammatory debris and granulation tissue. No malignant cells were seen. Within the small bowel resection a submucosal haemorrhage and focal perforation was noted. On microscopy of the omental mass, inflammatory debris was seen.