Thyroid Storm-induced Multi-organ Failure in the Setting of Gestational Trophoblastic Disease

Background. Gestational trophoblastic disease (GTD) which includes hydatidiform mole, invasive mole, placental site trophoblastic tumor, and choriocarcinoma is a rare cause of hyperthyroidism due to excess production of placental human chorionic gonadotrophin hormone (hCG) by tumor cells. Molecular mimicry between hCG and thyroid stimulating hormone (TSH) leads to continuous stimulation of TSH receptor by extremely high levels of hCG seen in these tumors. Consequently, biochemical and clinical hyperthyroidism ensues and it is potentially complicated by thyrotoxic crisis which is fatal unless urgent therapeutic steps are undertaken. Case Description. We present a 49-year-old perimenopausal woman who presented with recurrent thyroid storm and high output cardiac failure. The initial workup revealed suppressed TSH, high-free thyroxine (FT4), and free triiodothyronine (FT3) levels with increased vascularity of the normal-sized thyroid on ultrasonography. She was managed with parenteral beta blockers, steroids, and high-dose carbimazole. Her lower abdominal tenderness led to further investigations which revealed tremendously elevated beta-hCG and a snow storm appearance on transabdominal ultrasound suggestive of GTD. She underwent curative surgery and was diagnosed with complete hydatidiform mole postoperatively by histology. Conclusion. Recurrent thyroid crisis in gestational trophoblastic disease is an exceedingly rare presentation and that is highly fatal. This case highlights the importance of early detection and treatment of the etiology of thyrotoxicosis to eliminate mortality.

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Gestational Trophoblastic Disease-induced Thyroid Storm

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2019.9.43656 ◽

2019 ◽

Vol 3 (4) ◽

pp. 409-412

Author(s):

Carly Blick ◽

Kraftin Schreyer

Keyword(s):

Vaginal Bleeding ◽

The United States ◽

Gestational Trophoblastic Disease ◽

Definitive Treatment ◽

Thyroid Storm ◽

Trophoblastic Disease ◽

Trophoblastic Diseases ◽

Gestational Trophoblastic Diseases ◽

Life Threatening ◽

Life Threatening Complication

In the United States, gestational trophoblastic diseases (GTD), including molar pregnancies, occur in 121 out of 100,000 pregnancies.1 Many patients with GTD may develop hyperthyroidism. GTD-induced thyroid storm is a rare but life-threatening complication of GTD.2 Once patients are hemodynamically stable, the mainstay of definitive treatment is evacuation of the mole.3 We present a case of molar pregnancy-induced thyroid storm presenting as vaginal bleeding, fever, and tachycardia.

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Hyperthyroidism in gestational trophoblastic disease – a literature review

Thyroid Research ◽

10.1186/s13044-021-00092-3 ◽

2021 ◽

Vol 14 (1) ◽

Author(s):

Jarett Vanz-Brian Pereira ◽

Taylor Lim

Keyword(s):

Risk Factors ◽

Early Detection ◽

Gestational Trophoblastic Disease ◽

Thyroid Stimulating Hormone ◽

Cochrane Library ◽

Thyroid Storm ◽

High Risk Women ◽

Trophoblastic Disease ◽

Full Text Review ◽

Thyrotropic Activity

Abstract Objective Gestational trophoblastic disease (GTD) is a group of pregnancy-related disorders that arise from abnormal proliferation of placental trophoblast. Some patients with GTD develop hyperthyroidism, a rare but potentially life-threatening complication requiring early detection and management. Existing literature on hyperthyroidism in GTD is scant. This review aims to analyse the epidemiology, pathophysiology and management of this phenomenon. Methods A comprehensive search of MEDLINE, EMBASE and Cochrane Library was performed to obtain articles that explored hyperthyroidism in GTD. A total of 405 articles were screened and 228 articles were considered for full-text review. We selected articles that explored epidemiology, pathophysiology and outcomes/management of hyperthyroidism in GTD. Results The pathophysiology of hyperthyroidism in GTD is well-investigated. Placental trophoblastic tissue secretes excessive hCG, which is structurally similar to thyroid stimulating hormone and also has enhanced thyrotropic activity compared to normal hCG. The incidence and prevalence of hyperthyroidism in GTD varies worldwide, with lower rates associated with high uptake of early antenatal screening and early GTD detection. No clear risk factors for hyperthyroidism in GTD were identified. While hyperthyroidism can be definitively managed with surgical evacuation of the uterus, severe complications associated with hyperthyroidism in GTD have been reported, including thyroid storm-induced multi-organ failure, ARDS, and pulmonary hypertension. Conclusion Early detection of GTD is critical to prevent development of hyperthyroidism and its associated complications. Hyperthyroidism should be recognised as an important perioperative consideration for women undergoing surgery for GTD, and requires appropriate management. Future studies should explore risk factors for hyperthyroidism in GTD, which may facilitate earlier identification of high-risk women.

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SUN-513 Severe Hyperthyroidism in a Complete Molar Pregnancy

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.996 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Nikoletta Proudan ◽

Kersthine Andre

Keyword(s):

Perioperative Complications ◽

Hydatidiform Mole ◽

Gestational Trophoblastic Disease ◽

Thyroid Storm ◽

Molar Pregnancy ◽

Free T4 ◽

Trophoblastic Disease ◽

Increased Risk ◽

History Of

Abstract Hydatidiform mole (HM), a type of gestational trophoblastic disease (GTD), is a rare cause of clinical hyperthyroidism. The development of hyperthyroidism requires an elevation of HCG >100,000 mlU/mL for several weeks. Complete mole has a marked HCG elevation compared to partial mole thus presents with a higher incidence of thyrotoxicosis. Surgical uterine evacuation is the treatment of choice for HM. However, untreated hyperthyroidism can pose a risk for the development of thyroid storm and high-output cardiac failure in the perioperative period. To our knowledge, there are no specific guidelines for management at this time. We present a case of hyperthyroidism secondary to complete molar pregnancy successfully treated with propylthiouracil (PTU), potassium iodide (SSKI), and atenolol in the preoperative period. A 42-year-old female with history of migraines presented to her gynecologist with a 3-week history of lower abdominal cramping, vomiting, loss of appetite, and abnormal vaginal bleeding. She also endorsed a 6-pound weight loss, intermittent tachycardia, exertional dyspnea, and increased anxiety. Pregnancy test was positive, and ultrasound was concerning for GTD. Laboratory work up was significant for HCG 797,747 mIU/mL (< 5mlU/mL), TSH <0.005 mIU/mL (0.4-4.0 mlU/mL), Free T4 3.09 ng/dL (0.9-1.9 ng/dL), and Free T3 11.48 pg/dL (1.76-3.78 pg/dL). The patient was admitted to the hospital and started on PTU 100 mg Q6H, SSKI 200 mg TID following the first dose of PTU, and atenolol 25 mg daily. She underwent an uncomplicated D & C the next day. On post-op day 1, HCG decreased to 195,338 mIU/mL and Free T4 to 2.39 ng/dL. The patient was discharged on the aforementioned doses of PTU and atenolol. One-week follow-up labs showed HCG 8,917 mIU/mL and Free T4 1.22 ng/dL. Surgical pathology confirmed a complete hydatidiform mole. PTU was decreased to 50 mg TID. On post-op day 14, HCG had risen to 15,395 mIU/mL with onset of nausea and vomiting. Repeat Free T4 remained within reference range. Patient was taken back to surgery for a laparoscopic total hysterectomy with bilateral salpingectomy. Pathology confirmed an invasive hydatidiform mole. Two-week follow-up lab work showed HCG 155 mIU/mL, TSH 1.5 mIU/mL, and Free T4 1.19 ng/dL. PTU and atenolol were then discontinued. The development of hyperthyroidism in molar pregnancy is largely influenced by the level of HCG and usually resolves with treatment of GTD (1). However, it’s crucial to control thyrotoxicosis to avoid perioperative complications. This case also highlights the importance of monitoring HCG levels following a complete molar pregnancy due to an increased risk for invasive neoplasm. 1. Walkington, L et al. “Hyperthyroidism and human chorionic gonadotrophin production in gestational trophoblastic disease.” British journal of cancer vol. 104,11 (2011): 1665-9. doi:10.1038/bjc.2011.139

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A Case of Thyroid Storm With Takotsubo Cardiomyopathy and Multi-Organ Failure: What Is the Primary Cause of Hemodynamic Instability and How Do We Treat it?

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1859 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A910-A911

Author(s):

Tiffany Purewal ◽

Christopher Lesniak ◽

Andrew Ravin ◽

Khin Zin ◽

Soemiwati W Holland

Keyword(s):

Organ Failure ◽

Takotsubo Cardiomyopathy ◽

Treatment Plan ◽

Ventilatory Support ◽

Hemodynamic Instability ◽

Thyroid Storm ◽

Shortness Of Breath ◽

Free T4 ◽

Multi Organ Failure ◽

New Onset

Abstract Introduction: Thyroid storm is a rare but life-threatening emergency. Multi-organ failure has been recognized as the most common cause of death, but conventional therapies can be limited depending on the clinical presentation. We present a case of a patient in thyroid storm who rapidly developed multi-organ failure, preventing her from obtaining potentially life-saving treatment. Case Presentation: A 68-year-old female with a past medical history of hypertension, hyperlipidemia, and Grave’s disease, who was non-compliant with medications, presented to a facility for shortness of breath after the unexpected death of her husband. She was diagnosed with a non-ST elevation myocardial infarction and new onset heart failure. At that time, her TSH level was <0.010 uIU/mL and Free T4 was 1.80 ng/dL. Imaging revealed a significantly enlarged thyroid gland measuring 8cm by 6.6cm. She was started on methimazole and discharged home. A few days after discharge, she underwent a cardiac catheterization and was found to have Takotsubo cardiomyopathy. On presentation to our facility 2 weeks later, the patient was experiencing worsening shortness of breath and anxiety. She was found to have new-onset uncontrolled atrial fibrillation with rapid ventricular response and a blood pressure of 77/38 mmHg. The Burch-Wartofsky Point Scale was calculated to be 55 points, highly suggestive of thyroid storm. TSH was < 0.010 uIU/mL, total T4 was 16.63 ug/dL, and free T4 was 3.28 ng/dL. She was initiated on propylthiouracil, cholestyramine, hydrocortisone, and esmolol. Within 12 hours, she developed fulminant multi-organ failure requiring ventilatory support and vasopressors. She also developed ischemic hepatitis and propylthiouracil was discontinued. Urgent therapeutic plasma exchange (TPE) and continuous renal replacement therapy (CRRT) were later attempted but both therapies were not initiated due to severe hemodynamic instability. A bedside echocardiogram revealed an estimated ejection fraction of 20-25%. Due to worsening cardiogenic shock, she was evaluated for extracorporeal membrane oxygenation (ECMO) but was not a candidate. She instead underwent an emergent Impella device implantation. Despite this intervention, the patient’s clinical condition did not improve after multiple vasopressors, and the patient’s family opted for comfort-focused measures. The patient died after 1 day of hospitalization. Conclusion: A multimodality approach to treatment is recommended for patients with thyroid storm but underlying conditions such as Takotsubo cardiomyopathy and fulminant multi-organ failure may complicate the treatment plan. The complexity of this case highlights the need to understand relative contraindications to salvage therapies, such as TPE, and the role for other treatment options when patients present with co-existing multi-organ failure.

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A Case of Atypical Thyroid Storm Presentation With Subsequent DIC, Hypoglycemia, Kidney Failure and Shock Liver

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1852 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A907-A907

Author(s):

Liang Xue ◽

Samar Singh

Keyword(s):

Organ Failure ◽

Kidney Failure ◽

Thyroid Storm ◽

Atypical Presentation ◽

Diagnostic Challenge ◽

Treatment Patient ◽

Shock Liver ◽

Multi Organ Failure ◽

Facial Swelling ◽

Life Threatening

Abstract Background: Thyroid storm is a rare and life-threatening emergency requiring prompt intervention that is diagnosed based on a combination of clinical, physical and biochemical findings. Presented here is a case of thyroid storm which proved a diagnostic challenge due to its atypical presentation and whose management was further complicated by uncommon sequelae including DIC, hypoglycemia, kidney failure and shock liver. Clinical Case: A 37-year-old female with Graves’ disease presented to the Emergency Department with a chief complaint of facial swelling with concern for recent food allergy. Initial labs revealed elevated fT4 of 5.17 ng/dL (n 0.6-1.12 ng/dL) and suppressed TSH <0.01 uIU/mL (n 0.45-4.12 uIU/mL), but otherwise normal range BMP and CBC. While awaiting admission in the ED, she had normal cognition, stable vitals and scored 25 per Burch-Wartofsky scale. Given significant thyrotoxicosis and history of non-compliance with Graves’ treatment, patient was nevertheless empirically started on treatment for suspected thyroid storm in addition to treatment for possible allergic reaction. In the ED patient suddenly went into cardiac arrest with ROSC achieved after 2 rounds of CPR. Following ROSC, labs showed BG of 24 mg/dL (n<115 mg/dL), WBC to 24 thousand/mcL (n 4.0-10.5 thousand/mcL), lactate >10 mmol/L (n< 2mmol/L), D-dimer >20,000 ng/mL (n<500 ng/mL), AST 1869 U/L (n<52 U/L). Patient underwent a prolonged hospital course requiring treatment for hypoglycemia, shock liver, acute kidney injury, heart failure, atrial fibrillation, DIC and embolic CVA. Her initial shock liver improved and transitioned into a cholestatic picture, prompting a change in her thionamides from PTU to Methimazole, then back to PTU later on. Her multi-organ failure improved gradually over 3 weeks with mechanical ventilation, CRRT, blood transfusion, stress dose steroids and comprehensive critical care treatment. Patient was eventually discharged with close endocrine, ENT, cardiology, and neurology follow up. Conclusion: In diagnosing and treating this rare but life-threatening endocrine emergency, a strong clinical suspicion should not be detracted by an atypical presentation and prompt action is needed. It is vital to remember that this is first and foremost a clinical diagnosis that can be further supported with laboratory and physical findings. Furthermore, this case is an example of the extent of multi-organ failure that can result from thyroid storm.

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SUN-009 Variable Presentation of Two Patients with Gestational Trophoblastic Disease and Hyperthyroidism

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1641 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Bettina Chale-Matsau ◽

Samantha Mokoena ◽

Tanja Kemp ◽

Tahir S Pillay

Keyword(s):

Ct Scan ◽

Metastatic Disease ◽

Pulse Rate ◽

Medical Oncology ◽

Gestational Trophoblastic Disease ◽

Thyroid Storm ◽

Molar Pregnancy ◽

Free T4 ◽

Trophoblastic Disease ◽

Β Hcg

Abstract Background: Gestational trophoblastic disease (GTD) represents a group of tumours caused by abnormal proliferation of trophoblastic cells, including molar pregnancy. Elevated β-hCG levels are an established marker for the presence of the disease and useful for monitoring. Due to the shared structural homology of β-hCG and TSH, hyperthyroidism can occur. Clinical Cases: We present two patients with GTD associated with hyperthyroidisim. Case 1, a 20 year old female (G1P0) presented to the emergency department complaining of vaginal bleeding associated with abdominal pain. She was estimated to be 13 weeks. Laboratory evaluation were β-hCG 648 324 IU/L, TSH 0.06 (0.35 - 4.94 mIU/L, free T4 23.2 (9.0 - 19.0 pmol/L, Hb 8.0 (11.6 - 16.4 g/dL). Ultrasound revealed molar pregnancy. She underwent uterine evacuation, thereafter complicated with thyroid storm (Burch Wartofsky score = 45). Post- operative vitals were BP 192/112, pulse rate 120 bpm and temperature 360C. She was managed in high care on labetolol, carbimazole, lugol’s iodine and hydrocortisone. She was subsequently referred to Medical Oncology for further management. Histology sample obtained in theatre confirmed complete molar pregnancy. Her staging CT scan indicated the presence of small lung nodules, suggesting metastatic disease. The patient’s FIGO/WHO score was III: 2. At the time of preparing this study, she had already received 7 weeks of methotrexate intramuscularly and still had detectable β-hCG levels. Case 2 was a 31 year old female presented similarly. This was her second pregnancy (G2P1), 12 weeks by dates. Her vitals were BP 141/74, pulse rate 110 bpm and temperature 36oC. The Ultrasound revealed larger for gestational age uterus with cystic structures in utero. Her quantitative β-hCG was significantly elevated (> 1 500 000 IU/L) she was thyrotoxic [TSH (<0.1 (0.34 - 4.94 mIU/L) free T4 (47.2 (9.0 - 19.0 pmol/L)], however did not develop thyroid storm (Burch Wartofsky score = 20). This patient also underwent uterine evacuation and did well post operatively. She was treated for her thyrotoxicosis with carbimazole, propranolol and thiamine. Further management was by Medical Oncology. Histological examination was in keeping with a partial mole. Her staging CT scan showed no metastasis, and had a FIGO/WHO score of 1: 4 due to her pre-treatment hCG of >1.5 million IU/L. She received 7 cycles of intramuscular methotrexate from which she achieved and maintained suppressed β-hCG levels (<1 IU/L). Conclusions: This study has demonstrated that the β-hCG levels may not always correlate with disease severity and prognosis. When comparing the two patients Case 1 had lower levels of β-hCG and of free T4 than Case 2, however was clinically more unwell, developed thyroid storm and had metastatic disease. Case 2 had hCG levels almost double those of Case 1, wsa stable and her levels decreased much quicker reaching undetectable levels

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Plasma exchange and early thyroidectomy in thyroid storm requiring extracorporeal membrane oxygenation

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-19-0051 ◽

2019 ◽

Vol 2019 ◽

Cited By ~ 1

Author(s):

Huilin Koh ◽

Manish Kaushik ◽

Julian Kenrick Loh ◽

Chiaw Ling Chng

Keyword(s):

Extracorporeal Membrane Oxygenation ◽

Organ Failure ◽

Plasma Exchange ◽

Beta Blockers ◽

Thyroid Storm ◽

Organ Support ◽

List Type ◽

Cardiovascular Collapse ◽

Membrane Oxygenation ◽

Multi Organ Failure

Summary Thyroid storm with multi-organ failure limits the use of conventional treatment. A 44-year-old male presented with thyroid storm and experienced cardiovascular collapse after beta-blocker administration, with resultant fulminant multi-organ failure requiring inotropic support, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy. Hepatic and renal failure precluded the use of conventional thyroid storm treatment and early plasma exchange was instituted. The patient underwent emergency thyroidectomy after four effective exchanges, with subsequent rapid reversal of multi-organ failure. The challenges of institution of plasma exchanges with ongoing ECMO support, dialysis and timing of thyroidectomy are discussed. This case highlights the important role of early therapeutic plasma exchange (TPE) as an effective salvage therapy for lowering circulating hormones and stabilization of patients in preparation for emergency thyroidectomy in patients with thyroid storm and fulminant multi-organ failure. Learning points: Administration of beta-blockers in thyroid storm presenting with congestive cardiac failure may precipitate cardiovascular collapse due to inhibition of thyroid-induced hyperadrenergic compensation which maintains cardiac output. TPE can be an effective bridging therapy to emergency total thyroidectomy when conventional thyroid storm treatment is contraindicated. End-organ support using ECMO and CRRT can be combined with TPE effectively in the management of critically ill cases of thyroid storm. The effectiveness of plasma exchange in lowering thyroid hormones appears to wane after 44–48 h of therapy in this case, highlighting the importance early thyroidectomy.

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