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CASE REPORT
Year : 2020  |  Volume : 11  |  Issue : 2  |  Page : 96-98

Ovarian hyperstimulation syndrome following natural conception


Department of Obstetrics and Gynaecology, Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan, India

Date of Submission20-Jan-2020
Date of Acceptance19-Dec-2020
Date of Web Publication25-May-2021

Correspondence Address:
Dr. Swati Garg
Department of Obstetrics and Gynaecology, Mahatma Gandhi Medical College and Hospital, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjmsr.mjmsr_2_20

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  Abstract 


Ovarian hyperstimulation syndrome (OHSS) is a disorder that usually occurs with ovulation induction. It is rare in pregnant women and only a very few cases have been reported in spontaneous unstimulated conceptions. We report a case of spontaneous OHSS with primary hypothyroidism in a 19-year-old primigravida, where the imaging and laboratory findings confirmed the diagnosis of “Van Wyk–Grumbach syndrome” with pregnancy. This rare event in pregnancy needs attention, as early institution of thyroid hormone replacement therapy results in resolution of cysts and avoids unnecessary evaluation and surgical misadventures.

Keywords: Hypothyroidism, natural conception, ovarian hyperstimulation syndrome, Van Wyk–Grumbach syndrome


How to cite this article:
Garg S, Jain A, Sharma U, Thakral V. Ovarian hyperstimulation syndrome following natural conception. Muller J Med Sci Res 2020;11:96-8

How to cite this URL:
Garg S, Jain A, Sharma U, Thakral V. Ovarian hyperstimulation syndrome following natural conception. Muller J Med Sci Res [serial online] 2020 [cited 2021 Jun 21];11:96-8. Available from: https://www.mjmsr.net/text.asp?2020/11/2/96/316690




  Introduction Top


Ovarian hyperstimulation syndrome (OHSS) is a well-known potentially life-threatening complication in women undergoing ovulation induction for assisted reproductive techniques, with an incidence of 0.2%–1%.[1] OHSS in the absence of exogenous gonadotropins is very rare, and only a few cases following natural unstimulated conception have been reported.[2] De Leener has classified spontaneous OHSS syndrome into three types based on clinical presentation and follicle-stimulating hormone (FSH) receptor mutation. Type I causes recurrent spontaneous OHSS and is associated with the mutated FSH receptors. Type II occurs in hydatidiform mole and multiple gestation. It is secondary to excessive human chorionic gonadotropin (hCG) production and is the most common type. Type III is related to hypothyroidism, which is present in our case. It is a rare example of Van Wyk–Grumbach syndrome (VWG syndrome), which is a syndrome of prepubertal age characterized by longstanding hypothyroidism, precocious puberty, ovarian cysts, and delayed bone age. Only a few cases of Type III spontaneous OHSS during pregnancy have been reported. We report one such case of spontaneous OHSS and primary hypothyroidism which occurred in natural unstimulated conception.


  Case Report Top


A 19-year-old primigravida was referred to us from nearby village for early pregnancy and bilateral large ovarian cysts. Her history revealed that she conceived spontaneously, 7 months after her marriage, and went to doctor in her village for checkup. Ultrasonography (USG) has shown 12 weeks' intrauterine live gestation with huge bilateral ovarian masses, for which she was referred here. On detailed past history, her parents admitted that few years back, she was diagnosed with hypothyroidism, as she had irregular menstrual cycles and started with thyroxin, which she stopped taking around 1 year back before her marriage. On examination, she is hemodynamically stable, 143 cm in height, and is anemic. Abdominal examination revealed cystic masses of 26–28 weeks' uterus size, arising from pelvis; uterus was not felt separately. Investigations revealed a hypochromic, microcytic anemia with hemoglobin of 9.6 g%, hematocrit of 28.5%, and serum thyroid-stimulating hormone (TSH) of 614 mIU/ml. Viral markers, blood sugar, and urine examination were normal. She was Rh positive and hemoglobin electrophoresis was normal. Ovarian tumor markers for malignancy were sent; CA – 125 (Cancer antigen 125) was 61.9 U/ml, CEA (carcinoembryonic antigen) was 5.65 ng/ml, AFP (alpha-fetoprotein) was 8.58 ng/ml, and β–hCG (Beta human chorionic gonadotropin) was 150,000 mIU/ml. USG revealed an intrauterine single live fetus of 12+5 weeks, with adequate liquor and placenta fundal grade 0, with bilateral grossly enlarged ovaries with multiple cysts and thin septations (right – 140 mm × 91 mm; left – 126 mm × 108 mm).

In view of serum TSH of 614 mIU/ml, endocrinologist's reference was done. Incidentally, at the same time, the endocrinologist was reviewing magnetic resonance imaging of one prepubertal girl with juvenile hypothyroidism, precocious puberty, and ovarian masses. A diagnosis of VWG syndrome was made in both the cases. Our patient was started with thyroxin 200 μg for 5 days, followed by 100 μg daily. She was on continues follow-up, she remained clinically stable, and recovery was seen in serum examination and sonographic parameters. The TSH levels were slowly declining, the size of ovary gradually reduced, and the fetus was growing normally. Anomaly scan at 18 weeks was normal, and she was at low risk for chromosomal aneuploidies.

She delivered at term female child, weighing 2.8 kg at 39 +4 weeks of gestation. Postpartum TSH was 5.2 mIU/ml, and her ultrasound revealed regular and uniform uterus and normal ovaries.

To rule out mutation of FSH receptor gene, the patient was advised to have genetic sequencing, as this mechanism has implications related to the recurrence of the syndrome in future pregnancies, though at present severe hypothyroidism appears to be the cause in this pregnancy.


  Discussion Top


OHSS is an iatrogenic complication of ovulation induction and is associated with high morbidity. Less frequently, it is associated with spontaneous ovulatory cycles and is associated with molar and multiple pregnancies and hypothyroidism. Van Wyk and Grambach in 1966 first described the association of longstanding hypothyroidism, isosexual precocious puberty, and polycystic ovaries.[3]

The cause of spontaneous OHSS is proposed to be an increase affinity of ovarian receptor for hCG and/or TSH. hCG belongs to a family of glycoproteins that include FSH, luteinizing hormone (LH), and TSH. The receptors for these glycoprotein hormones have a similar structure. Under normal circumstances, hCG and LH bind to the LH receptor, while FSH and TSH bind to separate FSH and TSH receptors, respectively. Once the hormone binds to the receptor, the downstream signaling events are activated. High levels of hCG in molar pregnancies or multiple birth result in hyperactivation of FSH receptors in granulosa cells of the ovary by hCG, resulting in ovarian hyperstimulation. The same occurs with elevated TSH where hyperactivation of FSH occurs from the TSH receptors.

Hyperstimulated ovaries release vasoactive mediators and several proinflammatory cytokines under the influence of hCG which causes increased capillary permeating, intravascular fluid depletion and accumulation of fluid in the third space.[4] In spontaneous OHSS, unlike in iatrogenic, extravascular fluid, reference does not occur and there is hemodilution rather than hemoconcentration; therefore, patients are usually anemic, as was there in this patient.

It is important that all clinicians should consider hypothyroidism and other endocrine disorders in the differential diagnosis of adult females presenting with multicystic ovarian tumors to avoid unnecessary and catastrophic ovarian resection. Cases have been reported where the clinical presentation of spontaneous OHSS ascites, ovarian enlargement, and pleural effusion was misdiagnosed as advanced ovarian malignancy and may end up in exploratory laparotomy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ilanchezhian S, Mohan SV, Babu SR. Spontaneous ovarian hyperstimulation syndrome with primary hypothyroidism: Imaging a rare entity. Radiol Case Rep 2015;10:1050.  Back to cited text no. 1
    
2.
Sridev S, Barathan S. Case report on spontaneous ovarian hyperstimulation syndrome following natural conception associated with primary hypothyroidism. J Hum Reprod Sci 2013;6:158-61.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Kasum M, Oresković S, Jezek D. Spontaneous ovarian hyperstimulation syndrome. Coll Antropol 2013;37:653-6.  Back to cited text no. 3
    
4.
Orvieto R, Dratviman-Storobinsky O, Lantsberg D, Haas J, Mashiach R, Cohen Y. Interleukin-2 and SOCS-1 proteins involvement in the pathophysiology of severe ovarian hyperstimulation syndrome--A preliminary proof of concept. J Ovarian Res 2014;7:106.  Back to cited text no. 4
    




 

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