Presentation
Four days of worsening of long-standing intermittent abdominal distension and diffuse abdominal pain.
Patient Data
Grossly dilated large bowel loops. Dilated bowel with fecal material, predominantly on the right. A kidney/coffee bean sign with a thick inner wall of opposed bowel loops compared single layered outer walls is identified. Additionally, the Frimann-Dahl sign with three dense lines of sigmoid walls converging towards the right pelvis.
Bilateral psoas shadows are obscured. No pneumoperitoneum or air-fluid levels. No abnormal soft tissue densities or calcifications. The chest X-ray shows no sub-diaphragmatic free air and is generally unremarkable.
On single contrast enema, there is a bird beak-like projection on AP imaging, a bird beak sign, outlining the site of obstruction. Minimal contrast is observed traversing past the "beak" towards the right of the abdomen. No opacification of the remainder of the large bowel to cecum or reflux into the small bowel. Contrast flow abruptly cuts off within the sigmoid colon. No extravasation of contrast. Free flow of water-soluble (Gastrograffin) contrast through the rectum. Normal recto-sigmoid ratio.
The appearance of a bird beak-like projection on AP imaging on single contrast enema, outlining the site of obstruction "bird beak sign of the sigmoid colon".
Case Discussion
The bird beak sign of the sigmoid colon, kidney/coffee bean sign, and the Frimann-Dahl sign are all signs of sigmoid volvulus. The imaging features are in keeping with intestinal pseudo-obstruction (IP) and given the patient demographics (African) and age (ten years old), African degenerative leiomyopathy, a distinctive form of acquired degenerative visceral myopathy of uncertain etiology, is considered the most likely diagnosis.
Differential considerations would include missed Hirschsprung disease. Biopsy (for histology), in particular, a suction biopsy is recommended to distinguish the underlying entity.
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