Abstract
INTRODUCTION: Menstruation marks a significant developmental milestone, typically indicating healthy and normal pubertal changes in females. Primary amenorrhea can arise from various causes, including outflow tract abnormalities, resistant endometrium, primary ovarian insufficiency, and disorders of the hypothalamus, pituitary gland, or other endocrine organs. This report presents an exceptionally rare Müllerian malformation that does not conform perfectly to existing classification systems, necessitating an individualized management approach. CASE PRESENTATION: We present the case of a 12-year-old girl with a rare Müllerian malformation, who presented with cyclic lower abdominal pain persisting for over 9 months, with acute exacerbation in the preceding day. Comprehensive clinical evaluation and preoperative pelvic CT imaging revealed multiple anomalies: an unilateral rudimentary horn uterus with cavity and unilateral absence of the ovary and fallopian tube. Digital 3D CT reconstruction was utilized to confirm the diagnosis, revealing a left rudimentary horn uterus with cavity, hydrosalpinx of the left fallopian tube, and absence of the right ovary, fallopian tube and hemi-uterus. Surgical management included laparoscopic resection of the left rudimentary horn uterus and left fallopian tube. Postoperatively, the patient achieved complete resolution of cyclic lower abdominal pain. CONCLUSION: Digital 3D CT reconstruction technology is instrumental in diagnosing rare Müllerian malformations, providing crucial anatomical insights for surgical planning and patient counseling.