Robot-assisted repair of a urogenital sinus with an anorectal malformation in a patient with McKusick-Kaufman syndrome

• Published in: Journal of pediatric urology 2019-10, Vol.15 (5), p.481-483
• Main Authors: Phillips, Michael R., Linden, Allison F., Vinocur, Charles D., Hagerty, Jennifer A.
• Format: Article
• Language: English
• ISSN: 1477-5131; 1873-4898
• DOI: 10.1016/j.jpurol.2019.08.001

A urogenital sinus (US) and an anorectal malformation (ARM) are a rare constellation of anomalies, and the optimal surgical approach is unclear. Open and laparoscopic approaches have been described for US and ARM, but no data exist to support robotic assistance in children. A 20-month-old Amish female presented to the study center with fever and abdominal pain. Abdominal ultrasound showed a large fluid-filled vagina, urinalysis was positive, and she was admitted for antibiotic therapy. Magnetic resonance imaging (MRI) confirmed hydrocolpos. An examination under anesthesia including cystoscopy demonstrated a short perineal body, an anteriorly displaced anus by muscle stimulation, and no vaginal opening. An ultrasound-guided, percutaneous vaginostomy tube was placed, and 650 cc of pus was drained. Vaginal and urine cultures grew similar strains of Escherichias coli. After a course of antibiotics, she underwent a robot-assisted mobilization of the intra-abdominal vagina and uterus, posterior sagittal anorectoplasty, vaginal pull-through, and a diverting colostomy. There were no intra-operative complications. Her Foley catheter was removed on post-operative day #3, and she voided spontaneously and was discharged in good condition. She remained in the hospital for ostomy teaching, but pain control and diet were not barriers to discharge after 12 h. Robotic mobilization of the intra-abdominal vagina in a pediatric patient with US and ARM is technically feasible and can be accomplished safely. Further comparative studies to other approaches are lacking. In this case, the robot allowed for good visualization, intra-operative collaboration between multiple specialties for complex patients with aberrant anatomy, and easy dissection in a narrow pre-pubertal pelvis and would be an approach that the study group uses in future cases.