Problem: intravascular leiomyomatosis was discovered during laparoscopy

The patient presented with pelvic pressure and menorrhagia along with ultrasound findings confirming uterine fibroids. She desired definitive management and underwent a total laparoscopic hysterectomy and bilateral salpingo-oophorectomy (BSO).

Surgery revealed a 20-week sized myomatous uterus. Intravascular leiomyomatosis (IVL) within the infundibulopelvic ligament was suspected ( Figure 1 , Video Clip 1 ). As the extent of the disease was unknown, the initial procedure was limited to total laparoscopic hysterectomy and BSO. The patient then underwent postoperative computed tomography for further evaluation. Imaging demonstrated filling defects in the right and left ovarian veins. On the left side, this extended upward into the left renal vein and into the suprarenal inferior vena cava to the level of the adrenal gland.

Intravascular leiomyomatosis was suspected within right infundibulopelvic ligament.

Lakhi. Management of intravascular leiomyomatosis. Am J Obstet Gynecol 2013.

Our solution

Residual tumor within the patient’s inferior vena cava was removed during exploratory laparotomy. The liver was mobilized, and the entire inferior vena cava, from the diaphragm down to the bifurcation into the common iliac veins, was dissected ( Figure 2 , Video Clip 2 ). Vessel loops were used to gain circumferential control of vessels. Venotomy was performed to excise the leiomyomas. At the end of the procedure, all remaining tumor had been removed. Final pathology was consistent with benign IVL.

Dilated left renal vein, inferior vena cava (IVC), and left infundibulopelvic ligaments (IPL) are visible during exploratory laparotomy. Vessel loops were used to gain circumferential control of blood vessels.

Lakhi. Management of intravascular leiomyomatosis. Am J Obstet Gynecol 2013.

This case demonstrates the surgical management of IVL by both laparoscopic and open approaches. First described in 1896, IVL was said to be marked by “worm like” plexiform myomatous nodules growing into dilated channels of the uterus. These benign macroscopic smooth-muscle masses develop within the venules of the uterus and broad ligaments and can occasionally infiltrate the inferior vena cava and right atrium. Histologically, they resemble benign uterine leiomyomas; nuclear atypia and cellular pleomorphism are absent.

Patients normally present with symptoms of uterine fibroids, such as pelvic pain, menorrhagia, or abnormal uterine bleeding. Many cases can remain undiagnosed until the time of hysterectomy for presumed uterine leiomyomas. Right-sided congestive symptoms and ascites may manifest with intracardiac involvement. Tumors or thrombi in the upper segment of the inferior vena cava can lead to Budd-Chiari syndrome and lower-extremity edema.

The standard treatment for IVL is removal of the uterus along with excision of any extrauterine tumor when technically feasible. Since it was not possible to characterize the degree of our patient’s disease without adjuvant imaging, exploratory laparotomy was not attempted at the time of her primary procedure. Our goal was to first eliminate the pelvic IVL along with both ovaries. BSO is advocated as it reduces estrogen stimulation to intravascular leiomyomas. Many of these tumors are estrogen-receptor positive, and recurrences have been noted in patients with ovarian preservation. Removal of ovarian tissue can produce an antiestrogenic environment similar to the milieu created by gonadotropin-releasing hormone agonists.