A 17-year-old man was admitted to the Accident and Emergency Department due to a six-week history of occipital headache associated with vomiting and poor balance. He also reported decreased appetite and weight loss of approximately 28 lbs over a two-month period. He described transient tender skin nodules, which were present on the anterior tibial area, eight weeks prior to presentation. On direct questioning, he did reveal that he had oral ulceration previously, but this resolved spontaneously. Background medical history was unremarkable, as was his family history.
On examination in the Emergency Department, he appeared unwell. However, all his vital signs were within normal limits. Positive findings on examination included bilateral papilloedema and a seventh lower motor neuron lesion. There was bilateral scarring anteriorly on his skins. There was no evidence of oral or genital ulceration and abdominal examination at this time was normal. Hematological investigations were essentially normal except for a raised C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) at 119 mg/L and 84 mm/h, respectively. Further investigations performed included a lumbar puncture, Ziehl-Neelsen stain, autoantibody and infectious mononucleosis screen, all of which were non-contributory. He proceeded to have a CT brain and magnetic resonance studies, which demonstrated evidence of cerebral venous thrombosis. The “Empty Delta” sign on the post contrast MRI image is a characteristic finding in cerebral venous thrombosis (Figure ).
MRI post contrast with “Empty Delta” sign indicating a cerebral venous thrombosis.
A working diagnosis of vasculitis was formulated at this stage and the patient was placed on anticoagulation and steroids with proton-pump inhibitor cover. His symptoms gradually improved, but on day eight, he developed acute abdominal pain. On examination, his abdomen was distended, diffusely tender with absent bowel sounds. An erect chest X-ray demonstrated air under the right hemidiaphragm (Figure ).
Erect chest X-ray demonstrating free air under the right hemidiaphragm.
Marked fecal peritonitis was observed at laparotomy. There were five separate colonic perforations in the cecum, ascending, transverse and sigmoid colon. Surrounding these perforations were multiple areas of gangrenous colon with punched-out ulcerations extending throughout the colon to the level of the proximal sigmoid colon (Figure ). A sub-total colectomy and terminal ileum resection with an end ileostomy were performed.
Punched-out ulcer at laparotomy.
The histology specimen demonstrated a normal terminal ileum, but extensive ulceration in the proximal colon. The colon had numerous punched-out areas of ulceration, many of which were deeply penetrating with perforation. The intervening mucosa showed areas of normal mucosa. The distal segment of bowel, descending and sigmoid colon showed less ulceration than the proximal segment. However, there were nine distinct areas of necrosis, the largest of which was 2 cm in diameter. There was active inflammation with vasculitis, consistent with changes described in BS (Figure ).
Microscopic specimen demonstrating loss of normal mucosa.
The patient made a good post-operative recovery, but developed a pelvic collection four weeks after the procedure. This was treated with a transrectal drain and intravenous antibiotic therapy. The patient was discharged home after a 51-d hospital stay on warfarin, azathioprine and a decreasing dose of corticosteroids. He remained well two months post discharge on immunosuppressant therapy.