Maria Arutyunyan, MD
Emergency Medicine Resident
Department of Emergency Medicine
Medical College of Georgia
Larry Mellick, MD, MS, FAAP, FACEP
Professor of Emergency Medicine and Pediatrics
Medical College of Georgia
Pearl: When a pregnant woman presents complaining or shortness of breath and chest pain, consider the possibility of ovarian hyperstimulation syndrome.
Presentation: A 29-year-old woman was sent to the emergency department from the reproductive endocrinology clinic for evaluation of severe substernal chest pain and shortness of breath. She had recently undergone ovulation induction and implantation as part of the management of her infertility. Other than infertility, the patient had no other relevant medical or surgical history. She took no medications and denied the use of tobacco, alcohol, or recreational drugs. Several days prior to her presentation, the patient had complained of abdominal discomfort, diarrhea, nausea, and vomiting. At that time a small-to-moderate amount of ascites fluid was noted and she was scheduled for paracentesis in the reproductive endocrinology clinic. However the onset of acute chest pain and shortness of breath at presentation caused her to be diverted to the emergency department. The referring physicians expressed concern that the new symptoms might be consistent with pulmonary embolism.
In the emergency department, the patient demonstrated marked tachycardia and dyspnea. She had decreased breath sounds on the right side of her chest and a chest radiograph showed a large pleural effusion. (See Figure 1.) Her electrocardiogram (ECG) demonstrated a sinus tachycardia, and her cardiac enzymes were negative. The patient subsequently underwent a chest computed tomography angiogram (CTA) to rule out pulmonary embolism. CTA was negative for emboli; however, it did confirm a right tension hydrothorax with shift of the mediastinum. (See Figure 2.) The patient was admitted to the internal medicine service for thoracentesis and further management of her condition. She subsequently required several thoracentesis procedures, and almost 10 liters of fluid were removed.
Diagnosis: Severe ovarian hyperstimulation syndrome with tension hydrothorax
Discussion Ovarian hyperstimulation syndrome (OHSS) generally is considered to be an iatrogenic complication of assisted reproduction technology (ART). However, more recently, cases of ovarian hyperstimulation have been reported in patients who did not undergo ovarian induction and became pregnant spontaneously.1,2,3 OHSS usually develops several days following gonadotropin therapy. This syndrome is characterized by ovarian enlargement due to multiple ovarian cysts and acute fluid shift into the extravascular space. The severity of the syndrome varies from mild with minimal gastrointestinal symptoms to a more severe presentation with presence of ascites, pleural and/or pericardial effusion, electrolyte imbalance, hypovolemia, and, in some instances, hemoconcentration with thromboembolic phenomena and disseminated intravascular coagulation.1,4 Black women are more likely to get OHSS than white women, and the condition is also more commonly associated with ovulation disorders, tubal factors, and other unexplained factors.5
Although pathophysiology remains unknown, the underlying mechanism responsible for clinical manifestations is thought to be due to increased capillary permeability and an acute fluid shift from the intravascular to the third space.6,7 Certain vasoactive substances such as vascular endothelial growth factor, cytokines IL-2, IL-6, and IL-8, and tumor necrosis factor-alpha are secreted during maturation and luteinization of multiple follicles under the effect of gonadotropin.1,6,8
The incidence of severe OHSS varies depending on the study, but it is estimated to be between 0.06% and 0.24%.1 Most cases of OHSS are relatively mild and limited to only mild gastrointestinal discomfort, which resolves spontaneously within a few days. The most serious complications include massive ascites and hypovolemia with thromboembolic event.1,9
While there are several risk factors that have been implemented, polycystic ovary syndrome (PCOS) seems to be one of the most common culprits. The polycystic ovary contains multiple partially stimulated antral follicles and increased local estrogen concentration, which increase the sensitivity of these follicles to exogenous gonadotropins.4,8,10 According to some studies, when patients are known to have polycystic ovary syndrome, metformin appears to reduce the risk of overstimulation by reducing the level of estradiol.10
Treatment of OHSS is primarily prevention and other means outside the scope of this discussion.11 Some studies have shown that aspiration of follicles 36 hours after administration of gonadotropin may reduce the risk of developing OHSS.8 If it does develop, however, management needs to be tailored to specific symptoms.8,12
In conclusion, patients who present with severe OHSS usually have components of symptoms that include gastrointestinal discomfort, abdominal bloating, and often are found to have ascites, hydrothorax, or more severe manifestations. There are a few case reports of patients who presented with unilateral pleural effusion as a sole presentation of OHSS.9,12,13 Interestingly, the pulmonary effusions occur more commonly in the right lung. This is thought to be the result of decreased lymphatic drainage as compared to the left lung.14 It is important to have a high index of suspicion since early recognition of this condition will ideally prompt further diagnostic evaluations and appropriate therapeutic management.9,14
References:
- Botros Rizk. Ovarian hyperstimulation syndrome. Epidemiology, pathophysiology, prevention and management. New York: Cambridge University Press; 2006. p. 1-10.
- Oztekin O, Soylu F, Tatli O. Spontaneous ovarian hyperstimulation syndrome in a normal singleton pregnancy. Taiwan J Obstet Gynecol. 2006;45:272-5.
- Lussiana C, Guani B, Restagno G, et al. Ovarian hyper-stimulation syndrome after spontaneous conception. Gynecol Endocrinol . 2009;2:1-5. [Epub ahead of print]
- Delvigne A. Symposium: Update on prediction and management of OHSS epidemiology of OHSS.Reprod Biomed Online. 2009;19:8-13.
- Luke B, Brown MB, Morbeck DE, et al; a SART Writing Group. Factors associated with ovarian hyperstimulation syndrome (OHSS) and its effect on assisted reproductive technology (ART) treatment and outcome. Fertil Steril . 2009 Jul 8. [Epub ahead of print]
- Vloeberghs V, Peeraer K, Pexsters A, et al. Ovarian hyperstimulation syndrome and complications of ART. Best Pract Res Clin Obstet Gynaecol. 2009;23:691-709. Epub 2009 Jul 25.
- Fainaru O, Hornstein MD, Folkman J. Doxycycline inhibits vascular leakage and prevents ovarian hyperstimulation syndrome in a murine model. Fertil Steril. 2008 Oct 18. [Epub ahead of print]
- Oyawoye OA, Chander B, Hunter J, et al. Prevention of ovarian hyperstimulation syndrome by early aspiration of small follicles in hyper-responsive patients with polycystic ovaries during assisted reproductive treatment cycles. MedGenMed . 2005;7:60.
- Roden S, Juvin K, Homasson JP, et al. An uncommon etiology of isolated pleural effusion. The ovarian hyperstimulation syndrome. Chest. 2000;118:256-8.
- Tang T, Glanville J, Orsi N, et al. The use of metformin for women with PCOS undergoing IVF treatment. Hum Reprod. 2006;21:1416-25. Epub 2006 Feb 24.
- Gera PS, Tatpati LL, Allemand MC, et al. Ovarian hyperstimulation syndrome: Steps to maximize success and minimize effect for assisted reproductive outcome. Fertil Steril . 2009 Apr 6. [Epub ahead of print]
- Jakimiuk AJ, Fritz A, Grzybowski W, et al. Diagnosing and management of iatrogenic moderate and severe ovarian hyperstimulation syndrome (OHSS) in clinical material. Folia Histochem Cytobiol . 2007;45 Suppl 1:S105-8.
- Wood N, Edozien L, Lieberman B. Symptomatic unilateral pleural effusion as a presentation of ovarian hyperstimulation syndrome. Hum Reprod. 1999;14:272-3.
- Tang HH, Tsai YC, Kang CY, et al. Atypical ovarian hyperstimulation syndrome with isolated pleural effusion but without ascites or hemoconcentration. Taiwan J Obstet Gynecol. 2007;46:180-2.
Online Resources:
- http://emedicine.medscape.com/article/1343572-overview
- http://www.medscape.com/viewarticle/524218
|