Abstract
INTRODUCTION: Cloacal and bladder exstrophy are rare embryological defects that can cause developmental disruption of surrounding organ structures, the pelvis, spinal cord, and small intestines being the most commonly affected. Duplicated appendix is another rare embryological defect that has historically caused confusing clinical presentations. Our case highlights a rare instance of a patient with cloacal exstrophy who presented with a bowel obstruction and an associated inflamed duplicated appendix. CASE PRESENTATION: A newborn male is born with omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex. As primary surgical reconstruction was pursued, the patient was found to have a non-inflamed duplicated appendix, which was left unremoved. In the following months, the patient experienced episodes of small bowel obstruction, eventually requiring surgical intervention. During this operation, the duplicated appendix was noted to be inflamed, prompting removal of both appendices. DISCUSSION: This case highlights the increased prevalence of duplicated appendix in a patient with cloacal exstrophy, as well as the utility of prophylactic appendectomy for patients incidentally found to have a duplicated appendix intraoperatively. The duplicated appendix may lead to increased rates of complications and atypical presentation of appendicitis, supporting the practice of prophylactic appendectomy in patients with an incidentally found duplicated appendix. CONCLUSION: We suggest clinicians be aware of the association and potentially atypical presentation of appendicitis in patients with a duplicated appendix, particularly in the setting of cloacal exstrophy. The decision to prophylactically remove an incidentally found, non-inflamed duplicated appendix may be beneficial in preventing confusing clinical presentations and future complications.