Author: Karen Jeoffroy, MD
Editors: Philippe Ayres, MD and Alec Feuerbach, MD
Case:
A 27-year-old G2P1001 female, six weeks pregnant by LMP, presented to the ED with two weeks of worsening nausea and vomiting, vaginal bleeding, and mild, intermittent abdominal cramping. She reported a positive home pregnancy test; however, she had not yet received prenatal care. Her vital signs were a heart rate of 132 beats/minute and a blood pressure of 154/87 mmHg. On examination, she was in acute distress, with dry mucous membranes and a diffusely tender, gravid abdomen. There was moderate blood in the vault with clots but no cervical or adnexal motion tenderness and no cervical dilatation. The beta-hCG was 100,000 mIU/ml (notably higher than expected for the estimated gestational age) and free T4 was high and TSH was low. Bedside ultrasonography revealed a heterogeneous uterus with multiple, small anechoic structures throughout (Figure 1). The patient was treated with IV fluids and antiemetics. Ob/Gyn was consulted and the patient was admitted for dilation and curettage.
Figure 1: Ultrasound findings scattered, small, anechoic structures within a heterogeneous uterus. This finding is commonly described as a “snowstorm” or “honeycomb” appearance and is consistent with molar pregnancy
While this is a fairly rare gynecologic emergency (present in 0.6 to 2 in 1000 pregnancies [1]), it is one that emergency physicians must recognize and act upon.
Molar Pregnancy
Definitions and Pathophysiology:
Molar pregnancies are part of the larger spectrum of gestational trophoblastic diseases that occur from abnormal fertilization. The scope of gestational trophoblastic diseases ranges from partial and complete hydatidiform moles to gestational trophoblastic neoplasms.[2] The abnormal fertilization of the maternal ovum which causes molar pregnancies leads to the overproliferation of trophoblastic tissue. This excess tissue creates hydropic chorionic villi that appear like clusters of grapes.
Table 1: Spectrum of gestational trophoblastic disease (from: OBGYN Key [3])
Presentation:
Molar pregnancy classically presents with first or early second-trimester vaginal bleeding and larger than normal uterine size for gestational age. Patients may also report vaginal discharge of grape-like vesicles. Hyperemesis is common and it is thought that the elevated level of beta-hCG produced by the excess trophoblastic tissue may be the etiology. Symptoms and signs of preeclampsia (headache, hypertension, proteinuria) in the first or early second trimester can also be indicative of a molar pregnancy. Lastly, because beta-hCG is structurally related to TSH, it can weakly bind to TSH receptors resulting in hyperthyroidism.
Evaluation:
It is important to assess volume status and identify electrolyte derangements in the setting of hyperemesis. These patients are also at risk for anemia due to heavy vaginal bleeding. As a result, lab testing should include complete blood count, chemistry, type and screen, hepatic function tests, quantitative beta-hCG, and thyroid funcion tests. In addition to pelvic ultrasonography, the evaluation should also include a chest x-ray or CT chest if there is a suspicion of trophoblastic emboli.[4] Patients with suspicion for trophoblastic emboli show symptoms and signs of respiratory distress such as dyspnea, tachypnea, and hypoxia, and radiographic findings of pulmonary infiltrates may be seen.[4]
Imaging:
Ultrasonography is the standard imaging modality in identifying a molar pregnancy. The well-known “snowstorm” or “honeycomb” appearance is a common finding which describes the intrauterine mass containing multiple anechoic cystic spaces.
Figure 2: Ultrasonographic images of molar pregnancies compared to images from pathology samples (from: NIH [6])
Management
Ob/gyn consultation will be needed for definitive treatment of the molar pregnancy which is dilation and curettage. In patients that do not desire future pregnancy, hysterectomy may reduce the risk of malignancy. In the ED, our role is to deliver symptomatic treatment and resuscitation as needed. This may include IV hydration, antiemetics, electrolyte repletion, and blood transfusion. Hypertensive emergencies, thyrotoxicosis, and, rarely, trophoblastic pulmonary embolism are complications of molar pregnancy that should be treated immediately. Nonetheless, these conditions often resolve with definitive management.
Once definitive treatment is complete, these patients must also undergo serial beta-hCG monitoring on an outpatient basis to detect the development of gestational trophoblastic neoplasia. Beta-hCG levels are taken weekly until levels are undetectable, and testing may continue for six months to a year.[5] Patients are advised to refrain from pregnancy during this monitoring period as increased beta-hCG levels related to pregnancy make it difficult to interpret beta-hCG results to identify gestational trophoblastic neoplasia.
Summary:
– Molar pregnancy is part of a spectrum of gestational trophoblastic disease.
– Classic findings include vaginal bleeding, grape-like vaginal discharge, hyperemesis, and signs of preeclampsia or thyrotoxicosis.
– A diagnosis of molar pregnancy can be made with a pelvic ultrasonography showing the classic “snowstorm” appearance of the uterus. Elevated beta-HCG levels are also common.
– Complications associated with molar pregnancy include anemia, dehydration, electrolyte derangements, hypertensive emergencies, thyrotoxicosis, and, rarely, trophoblastic PE.
– Definitive treatment includes dilatation and curettage or hysterectomy.
– Patients will require ongoing serial beta-hCG testing to detect gestational trophoblastic neoplasia.
References
1. Chawla T, Bouchard-Fortier G, Turashvili G, Osborne R, Hack K, Glanc P. Gestational trophoblastic disease: an update [published online ahead of print, 2023 Feb 10]. Abdom Radiol (NY). 2023;10.1007/s00261-023-03820-5. doi:10.1007/s00261-023-03820-5
2. Ngan S, Seckl MJ. Gestational trophoblastic neoplasia management: an update. Current Opinion in Oncology. 2007;19(5):486-491. doi:10.1097/cco.0b013e3282dc94e5
3. Gestational trophoblastic disease. OBGYN Key. June 4, 2016. Accessed February 15, 2023.Molar Pregnancy and Gestational Trophoblastic Neoplasms | Obgyn Key
4. Jorens PG, Van Marck E, Snoeckx A, Parizel PM. Nonthrombotic pulmonary embolism. Eur Respir J. 2009;34(2):452-474. doi:10.1183/09031936.00141708
5. Soper JT. Gestational Trophoblastic Disease: Current Evaluation and Management [published correction appears in Obstet Gynecol. 2022 Jan 1;139(1):149]. Obstet Gynecol. 2021;137(2):355-370. doi:10.1097/AOG.0000000000004240
6. Rao, Asha et al. Pregnancy outcome with coexisting mole after intracytoplasmic sperm injection: A case series. NIH National Library of Medicine, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4601179/, Published 2015, July.
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