Puzzler: A sneaky cause of hemorrhagic shock
Can you arrive at the correct diagnosis?
Dr Takeshige is an attending physician at the Department of Obstetrics and Gynecology at Lincoln Medical and Mental Health Center, Bronx, New York.
Dr Rezai is a resident physician in the Department of Obstetrics and Gynecology at Lincoln Medical and Mental Health Center, Bronx, New York.
Mr Giovane is a medical student in Grenada, West Indies.
Dr Henderson is an attending physician at the Department of Obstetrics and Gynecology at Lincoln Medical and Mental Health Center, Bronx, New York.
None of the authors has a conflict of interest to report with respect to the content of this article.
The authors wish to thank Ms Judith Wilkinson, Medical Librarian, Lincoln Medical and Mental Health Center Science Library, New York, for her assistance.
Presentation
A 35-year-old nulliparous Spanish woman was referred to our gynecology clinic for evaluation of worsening symptomatic fibroid uterus. The presenting complaints included increasing abdominal pressure, upper and lower quadrant pain, dyspepsia with intermittent vomiting, frequent urination, and nausea for several months. Gynecologic history was significant only for a fibroid uterus diagnosed several years before presentation for which the patient had received no treatment. She had no significant medical history and no prior surgery. Family history was significant for fibroid uterus; both her sister and mother had had hysterectomies for fibroids.
Clinical course
The patient’s vital signs were stable and physical examination was remarkable for an abdomen that was soft without tenderness; uterus was enlarged and extended to the level of xyphoid process. The work-up included a pelvic sonogram, which was limited due to a large mass in the pelvis extending to the abdomen measuring 13x10x11 cm most likely representing fibroids. Computed tomography reported a multinodular mass arising from the uterus measuring 25x16x17 cm, most compatible with myomas.
The patient was scheduled for abdominal myomectomies with possible hysterectomy due to her desire to remain fertile. Lupron was not administered preoperatively because of the large size of the uterus and the potential side effects of menopausal symptoms.
To preserve endometrial integrity, the endometrium was inflated with methylene blue via ZUMI intrauterine manipulator, allowing for identification of the endometrium during resection. An irregular uterus measuring 28x30 cm and enlarged ovaries bilaterally with prominent varicosity were noted intraoperatively. The uterus was exteriorized with difficulty due to its large size. Pitressin was administrated into the uterus to reduce bleeding. A total of 17 intramural myomas were removed, with variable sizes of 15x12 cm, 13x10 cm, 15x10 cm, 10x6 cm, 6x6 cm, and several 2x2 cm. Hemostasis of the uterus was achieved after closure of 3 layers and application of surgiflow (Ethicon); however, bleeding persisted from the abdominal cavity.
Upon exploration, an apparent 15x12-cm pedunculated subserosal myoma was noted detached from the uterus with profuse bleeding. This myoma had been inadvertently detached from the posterior uterine wall and was firmly attached to the transverse colon. Bleeding was unresponsive to sutures and coagulation of the proximal end of the leiomyoma until it was completely removed from the transverse colon following initiation of a massive transfusion. Bleeding was controlled after this myoma was removed from the transverse colon. The fibroid weighed 458 g. The specimen was sent for frozen section.
The patient was transfused intraoperatively with 10 units of packed red blood cells (PRBC) and 2 units of fresh frozen plasma (FFP) with postoperative hemoglobin of 4.6 g/dL and hematocrit of 13.0%. The patient’s vital signs included pulse of 128 bpm and blood pressure of 99/57 mmHg. The patient was transferred to the SICU immediately following completion of the procedure.
Differential diagnoses
Leiomyoma, leiomyosarcoma, angiolipoma, giant cell tumor, adenomyosis, uterine carcinosarcoma.
NEXT: Final diagnosis >>
Final diagnosis
Parasitic myoma
Subsequent clinical course
The patient received 18 units of PRBC, 6 units FFP, 2 units platelets, and 10L of crystalloid within 24 hours of surgery. Vital signs on postoperative day 1 were: pulse 126 bpm and blood pressure 111/69 mmHg. On physical exam, abdomen was soft, with moderate tenderness and incision intact. A repeat hemogram showed hemoglobin of 6.7 mg/dL and hematocrit of 19.0%. Despite massive blood transfusion and no further blood loss, hemoglobin levels did not improve and the patient remained tachycardic on postoperative day 1. Intra-abdominal bleeding was ruled out by bedside sonogram with no free fluid noted. Thus, impression was resolving hemorrhagic shock. The patient remained in SICU until postoperative day 4 with no further complications. Her hemoglobin and hematocrit levels remained stable. The patient was transferred to the gynecology floor and discharged on day 8.
Discussion
Myomas are the most common pelvic tumors in women and occur in approximately 25% of women of childbearing age.1-3 Although myomas are typically asymptomatic4, they can become enlarged and symptomatic. Cullen in 19075,6 described a case of a large parasitic myoma with marked development of omental vessels some reaching 1 cm in diameter.5 Approximately half of affected patients with parasitic myomas develop abnormal uterine bleeding, anemia, and pelvic pressure. Other symptoms include pain, increased urinary frequency, and increased risk for adverse reproductive outcomes (Tropeano et al. 2008).4,7 Parasitic fibroids are unique in that they can produce symptoms similar to any fibroid but also mass effects based on their location.8 Examples include urinary obstruction due to urethral, bladder neck, or ureter compression.9 If symptoms are debilitating, surgery should be considered.
The histopathology of myomas is the benign monoclonal proliferation of smooth muscle cells within the uterus.3,9,10 Parasitic myomas are unique in that they are defined as any myomas not occurring in the uterus.9
Several theories exist regarding the origins of parasitic myomas although the exact mechanism remains elusive. The most commonly accepted theories consider parasitic myomas to be a subset of pedunculated subserosal myomas that have undergone torsion and adhere to the surrounding tissue, with the most common location being the broad ligament.1,8,11 This allows the myoma to develop an extrauterine vascular supply via neovascularization.1,8 This event can be triggered by GnRH agonist therapy or uterine artery embolization, leading to a loss of uterine vascular supply thus encouraging the myoma to parasitize extrauterine vascular supply.1 Location of the parasitic fibroid on the sigmoid colon and the urethra are very rare.4 Seeding of parasitic myomas may occur after surgical morcellation of the myomas either at time of myomectomy or hysterectomy, leading to an iatrogenic parasitic myoma.1,3,12-14 An increase in the incidence of parasitic myomas has been noted in patients who have undergone laparoscopic surgery.16,17 Rare vascular spread of myomas can cause benign metastasizing leiomyoma, disseminated peritoneal leiomyomatosis, and intravenous leiomyomatosis.18
The diagnostic modalities for detecting extrauterine myomas are ultrasonography, computed tomography, and magnetic resonance imaging.9,11,19 Parasitic myomas usually demonstrate low signal intensity similar to that of smooth muscle on T2-weighted image on MRI, which makes it valuable for diagnosis.
Many treatment methods for symptomatic myomas exist. Options include hormonal therapies, uterine fibroid embolization, myomectomy, and hysterectomy.17,20
In the 12 cases discussed by Kho and Nezhat in 2009, the diagnosis of parasitic leiomyoma was almost always made during laparoscopy for treatment of uterine fibroids.1 In this case, the patient had no risk factors and was diagnosed intraoperatively.
References
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