Hyperreactio luteinalis in a spontaneously conceived pregnancy associated with polycystic ovarian syndrome and high levels of human chorionic gonadotropin
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Ioannis Kyvernitakis
,
Volker Ziller
Abstract
Aim: To report a case of hyperreactio luteinalis in a spontaneously conceived pregnancy.
Patient: A 29-year-old primigravida presenting with painful multicystic ovarian lesions.
Methods: Laparotomy and reconstruction of the ovaries.
Main outcome measures: Successful pregnancy and delivery of a 3650 g female infant at 39 weeks, without any abnormalities.
Results: Highly elevated human chorionic gonadotropin, estradiol, progesterone, testosterone, and free testosterone levels were observed. Ultrasound revealed bilaterally enlarged multicystic ovaries with several cysts of up to 9 cm in diameter.
Conclusions: In this case, laparotomy appeared to be an appropriate therapy to prevent further suffering in the mother without doing harm to the fetus.
Introduction
Ovarian hyperstimulation syndrome (OHSS) is a well-recognized and well-described condition occurring in assisted reproduction with follicle stimulating therapies. It is associated with polyfollicular growth leading to enlarged ovaries, elevated hormone levels, fluid shifts to the extravascular space, and consecutive hemoconcentration with elevated risk for thromboembolic events [1]. In severe cases, it leads to massive ascites, pleural effusion, and consecutive renal compression. Hyperreactio luteinalis (HL) is a rare condition characterized by multicystic ovarian enlargement mimicking OHSS in spontaneous pregnancies without ovarian stimulation or ovulation induction [5]. HL is considered to be a result of excessive ovarian luteinization due to hyperstimulation or hypersensitivity to human chorionic gonadotropin (hCG) [3].
Presentation of the case
We report on a 29-year-old woman gravida 1 para 0 who was referred to our obstetrics department at 13+3 weeks of gestation with multiple ovarian cystic lesions and abdominal pain and tightness for further evaluation and treatment.
The patient’s history revealed polycystic ovarian syndrome (PCOS) that was treated with oral contraceptives, which were interrupted about 2 years before the conception of a singleton pregnancy without ovulation stimulation. At 8 weeks, the patient had vaginal bleeding for 1 week but the pregnancy remained intact.
At admission, the ultrasound exam at our department revealed bilaterally enlarged multicystic ovaries with several cysts of up to 9 cm in diameter (Figure 1A). The cysts showed no criteria of malignancy. Color and pulsed Doppler studies disclosed normal uterine and ovarian vessels. Magnetic resonance imaging of the abdomen was performed, affirming the ultrasound findings and excluding further organ involvement (Figure 1B). Laboratory results showed normal values of hemoglobin, blood cell counts, hematocrit, creatinine, blood urea nitrogen, liver enzymes, and albumin concentration, but highly elevated hCG, estradiol, progestin, testosterone, and free testosterone levels (Table 1).
(A) Cystic lesions seen by color-coded transabdominal ultrasound showing a transversal plane of the enlarged ovarian masses. (B) Magnetic resonance image showing a transversal plane of the maternal abdomen. (C) At the start of abdominal laparotomy.
Laboratory results at admission.
| Laboratory parameters | Reference value | Unit | Result |
|---|---|---|---|
| hCG | –5 | U/L | 102,549 |
| FSH | 2.5–10.2 | U/L | 0.32 |
| LH | 1.9–12.5 | U/L | 1.6 |
| Estradiol | 21–114 | ng/L | 9905 |
| Progesterone | 0.2–0.8 | µg/L | 65 |
| Testosterone | –0.77 | µg/L | 2.9 |
| Free testosterone | –2.6 | ng/L | 3.1 |
| SHBG | 18–114 | nmol/L | 522 |
| TSH | 0.34–5.6 | mU/L | 1.9 |
The fetus was active without any sonographic signs of malformation or pathologic karyotype, e.g., values for nuchal translucency, nasal bone, fetal heart rate, Doppler of the ductus venosus, and tricuspid valve were within the reference range.
The patient was hospitalized, and as clinical symptoms aggravated a laparotomy was performed, whereby the cystic lesions were extirpated and the ovaries reconstructed (Figure 1C). The histopathological examination revealed HL of the ovaries without any signs of malignancy. The patient was discharged after a few days and the pregnancy continued without further complications. At 39 weeks, the patient spontaneously delivered a healthy girl of 3650 g. A sonogram post partum revealed normal uterine regression and no further cystic lesions. The mother was discharged the following day, breastfeeding the baby.
Retrospectively, we could conclude that HL is associated with PCOS and high levels of hCG and testosterone in a singleton pregnancy.
Discussion
The combination of polyfollicular ovaries and high hCG might be a possible explanation as PCOS is a well-established risk factor for OHSS in assisted reproduction, especially in combination with hCG application or elevated hCG levels as in twin pregnancies [2, 4]. In this case, no signs of fluid shifts or hemoconcentration were present and in combination with the histopathological diagnosis and the late onset in the early second trimester, the diagnosis was HL rather than spontaneous OHSS [3].
The management of this patient was based on the clinical deterioration rather than the sonographic or radiographic findings, namely a fast progression of abdominal pain and tightness. Laparotomy to restore the ovaries after extirpating the cysts appeared to be a successful therapy to prevent further suffering in the mother without doing harm to the fetus.
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Conflict of interest statement
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Authors’ conflict of interest disclosure: The authors have no potential conflicts of interest.
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Confirmation: We confirm to have written consent by the patient for publication.
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Design: Case report for the Journal of Perinatal Medicine.
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Setting: University hospital.
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The authors stated that there are no conflicts of interest regarding the publication of this article.
References
[1] American Society for Reproductive Medicine. Ovarian hyperstimulation syndrome. Fertil Steril. 2008;90(5 Suppl): S188–93.10.1016/j.fertnstert.2008.08.034Search in Google Scholar PubMed
[2] Check JH, Choe JK, Nazari A. Hyperreactio luteinalis despite the absence of a corpus luteum and suppressed serum follicle stimulating concentrations in a triplet pregnancy. Hum Reprod. 2000;15:1043–5.10.1093/humrep/15.5.1043Search in Google Scholar PubMed
[3] Haimov-Kochman R, Yanai N, Yagel S, Amsalem H, Lavy Y, Hurwitz A. Spontaneous ovarian hyperstimulation syndrome and hyperreactio luteinalis are entities in continuum. Ultrasound Obstet Gynecol. 2004;24:675–8.10.1002/uog.1759Search in Google Scholar PubMed
[4] Humaidan P, Quartarolo J, Papanikolaou EG. Preventing ovarian hyperstimulation syndrome: guidance for the clinician. Fertil Steril. 2010;94:389–400.10.1016/j.fertnstert.2010.03.028Search in Google Scholar PubMed
[5] Van Holsbeke C, Amant F, Veldman J, De BA, Moerman P, Timmerman D. Hyperreactio luteinalis in a spontaneously conceived singleton pregnancy. Ultrasound Obstet Gynecol. 2009;33:371–3.10.1002/uog.6325Search in Google Scholar PubMed
©2012 by Walter de Gruyter Berlin Boston
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Articles in the same Issue
- Masthead
- Masthead
- Editorial
- Editorial
- Case reports – Obstetrics
- Sonographic presentations of uterine rupture following vaginal birth after cesarean – report of two cases 12 h apart
- Prenatal diagnosis of thrombocytopenia-absent radius syndrome
- Cervico-isthmic pregnancy with cervical placenta accreta
- Prelabor uterine rupture and extrusion of fetus with intact amniotic membranes: a case report
- Hyperreactio luteinalis in a spontaneously conceived pregnancy associated with polycystic ovarian syndrome and high levels of human chorionic gonadotropin
- Should clinicians advise terminating a pregnancy following the diagnosis of a serious fetal cardiac abnormality?
- Absence of hemolytic disease of fetus and newborn (HDFN) in a pregnancy with anti-Yka (York) red cell antibody
- Congenital midgut malrotation causing intestinal obstruction in midpregnancy managed by prolonged total parenteral nutrition: case report and review of the literature
- Skin popping scars – a telltale sign of past and present subcutaneous drug abuse
- Botulinum toxin for the treatment of achalasia in pregnancy
- Thrombotic stroke in association with ovarian hyperstimulation and early pregnancy rescued by thrombectomy
- Normal pregnancy outcome in a woman with chronic myeloid leukemia and epilepsy: a case report and review of the literature
- Three-dimensional power Doppler assessment of pelvic structures after unilateral uterine artery embolization for postpartum hemorrhage
- Deep congenital hemangioma: prenatal diagnosis and follow-up
- Case reports – Fetus
- Diagnosis of cleft lip-palate during nuchal translucency screening – case report and review of the literature
- Vein of Galen aneurysm that was diagnosed prenatally and supracardiac obstructed total anomalous pulmonary venous return with pulmonary hypertension: case report
- A fetus with 19q13.11 microdeletion presenting with intrauterine growth restriction and multiple cystic kidneya
- Prenatal detection of periventricular pseudocysts by ultrasound: diagnosis and outcome
- Twin-to-twin transfusion syndrome and limb ischemia: a case report
- Prenatal surgery in a triplet pregnancy complicated by a double twin reversed arterial perfusion (TRAP) sequence
- A case of a four-vessel umbilical cord: don’t stop counting at three!
- Case reports – Newborn
- Supratentorial hemorrhage suggested on susceptibility-weighted magnetic resonance imaging in an infant with hydranencephaly
- Differential diagnosis of pseudotrisomy 13 syndrome
- Carey-Fineman-Ziter syndrome: a spectrum of presentations
