2. Surgical Approach to Mullerian Anomaly with Coexisting Renal Agenesis and VATER Syndrome

• Published in: Journal of pediatric & adolescent gynecology Vol. 34; no. 2; p. 240
• Main Authors: Beausang, Jasjit, Spearman, Margaret, Mama, Saifuddin
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-04-2021
• ISSN: 1083-3188; 1873-4332
• DOI: 10.1016/j.jpag.2021.02.006

We present the case of a 23-year-old female with severe cyclical pelvic pain and amenorrhea with multiple emergency room visits and hospitalization. She already had diagnoses of VATER, right renal agenesis, neurogenic bladder, and scoliosis of thoracolumbar region. Imaging demonstrated right sided hematometra, right and possibly left uterine horn, right renal agenesis, and hematosalpinx. Because of outflow obstruction, and resultant retrograde menstrual flow, causing severe pain, surgical management was indicated. Robot-assisted laparoscopy demonstrated endometriosis, significant adhesive disease, bilateral enlarged fallopian tubes, ovaries bilaterally, two uterine horns without midline fusion and no identifiable cervical tissue. Some surgical findings were anticipated, such as absent right ureter, endometrial implants, adhesive disease, enlarged right uterine horn. Other findings were unanticipated such as left uterine horn adjacent to the left round ligament inguinal insertion, obscured and atypical right-sided pelvic sidewall vasculature, and the additional embryologic tissue of unclear origin discovered adjacent to the right uterine horn and vagina. No cervical tissue was identified in either uterine horn and pathology confirmed this. Pathologic diagnosis of the additional embryonic tissue separate from the right uterine horn indicated Mullerian origin. Surgery was without complications, patient was discharged home without incident.
We display the challenges inherent in surgical management of cyclic pelvic pain due to retrograde bleeding from obstruction in a patient with multiple congenital anomalies. Co-occurrence of the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association (VATER) and mullerian abnormalities is rare and not well-characterized. We describe and demonstrate surgical findings in a patient with the rarely associated VACTERL/VATER spectrum and Mullerian anomaly. Familiarity with the embryological origins of pelvic structures was crucial to decision-making and guided surgery. Robotic assistance facilitated visualization of planes and allowed precise dissection. Because each case of embryologic anomaly presents with unique pre-op and intra-op findings, formulating a sound thought process and surgical technique is crucial to prevent injury and achieve the goals of surgery.