Intestinal malrotation in children is a rare aberration, due to a halt in the rotation and attachment of the primitive gut, it can be asymptomatic if the rotation terminates at 90 degrees, which manifests itself in unusual forms of appendicitis as in our observation, or dangerous in cases of inadequate common mesentery and worsened by small intestine volvulus. This 12-year-old boy experienced abdominal discomfort in the hypogastrium and left iliac fossa 4 days before admission. The pain had been developing in a feverish setting, and the clinical examination had revealed abdominal sensitivity. A biological inflammatory syndrome was detected throughout the biological workup, the CT scan allowed the diagnosis of acute appendicitis on a complete common mesentery, and the patient underwent a laparotomy appendectomy. Even though children frequently experience acute appendicitis in its conventional form, it is nevertheless highly challenging to identify in its atypical forms when intestinal malrotation is involved. An abdominopelvic CT scan is used to make the diagnosis, and appendectomy, preferably with laparoscopy, is the recommended course of action.
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