Cervical agenesis: Sepsis and death following surgery

Abstract

<h3>Background</h3> Cervical agenesis is a rare variation of müllerian anomaly which may be associated with complete or partial vaginal agenesis. When the lower vagina is present, it is essential that the upper tract anatomy be defined prior to surgical intervention to correct the obstruction. We present a case of an obstructive müllerian anomaly diagnosed as a transverse vaginal septum, which was actually agenesis of the cervix and upper vagina. The surgical procedure resulted in sepsis and death from attempted connection of the vagina to a uterus with unrecognized cervical agenesis. Diagnosis and management of this rare müllerian anomaly is reviewed. <h3>Case</h3> An II year old female with a history of an imperforate anus and partial sacral agenesis presented with pelvic pain and by CT and MRI was found to have a hematometra and hematosalpinges felt to be secondary to a transverse vaginal septum or vaginal agenesis. The "septum" was excised and a drainage tube was placed from the endometrial cavity into the vagina. After 2 months of normal periods the tube occluded and was removed. Within 24 hours the patient presented with fever and tachycardia and was admitted to the Intensive Care Unit. She was in septic shock and underwent an ultrasound guided transvaginal drainage of an infected hematometra with placement of a closed transvaginal drainage tube. Blood and aspirated hematometra cultures grew E. coli and MorganelJa morgani. She developed Adult Respiratory Distress Syndrome, hemodynamic instability requiring pressors. Disseminated Intravascular Coagulopathy, acute renal failure, acrocyanosis, pancreatitis, and transaminitis. She underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy with pathology showing no identifiable cervix and necrotizing endomyometritis. Despite aggressive support she died on hospital day 31. <h3>Conclusions</h3> Cervical agenesis is a rare occurrence. If cervical agenesis is diagnosed, an option of suppression of menses and maintenance of the uterus in-situ, or a surgical procedure to create a patent epithelialized endocervical canal may preserve future reproductive function with the use of assisted reproductive technologies. Ultrasound and MRI should be employed to assist in the defmition of the upper tract anatomy prior to surgical correction of the obstruction. It may be difficult to defmitively identify the anatomy when a hematometra occurs. The gynecologic surgeon must be aware of the risks of creating a vaginal-uterine fistula in the absence of a cervix, as bacterial contamination of the upper tract may occur leading to sepsis and possible death.