Severe Spontaneous Ovarian Hyperstimulation Syndrome With Cervical insufficiency: A Case Report

Abstract

Background: Severe form of spontaneous OHSS (sOHSS) is a rare entity in singleton pregnancy and may cause laparotomy because of misdiagnosis such as ovarian tumors or severe complications. We present a rare case of severe OHSS complicated with cervical insufficiency in singleton spontaneous pregnancy. Case: A 21-year-old primigravid, conceived spontaneously, presented to our clinic at 11 weeks of gestation with the complaints about severe abdominal pain, progressive abdominal swelling and dyspnea. Her medical or surgical disease and family history was unremarkable. She hadn't had any medication for ovulation induction. Her menstrual period was regular. Ultrasound revealed singleton, alive intrauterine an 11 week pregnancy with bilateral enlarged multicystic ovaries and a large amount of ascitic fluid in the pelvis and abdominal cavity (Figure 1) consistent with severe sOHSS. Right ovary was measured 17 cm in long axis diameter and left one 16 cm (Figure 2). Preliminary blood tests revealed a hematocrit of 36.2%, white blood cell count of 10,660/mm3, sodium of 128 mmol/L, potassium of 4.5 mmol/L, calcium of 8 mg/dL, serum hCG of 630,631 mU/mL, total testosterone of 258 ng/dL, with other blood results including TSH within normal limits. She was admitted to hospital and managed with intravenous fluid replacement, albumin infusion, hydroxyethyl starch (HES), low molecular weight heparin for thromboprophylaxis with carefully monitored over the course of her stay by her symptoms, body weight, abdominal circumference, ultrasonography and laboratory tests. We obtained molecular genetic analysis and demonstrated that she was homozygous for PAI-1 4G/5G gene. There was no available testing method for FSH receptor mutation in our hospital. There wasn't any pathological finding on pituitary MRI. After three days, peritoneal fluid was drained by abdominal catheter about 1000-2000 mL/day to reduce the patients' discomfort and performed meticulous fluid balance daily. There were no malignant cells on cytology. Cabergoline 0.5 mg/day treatment was started. After three weeks from catheterization, abdominal ascites were decreased and catheter removed. The first trimester combined test result was 1/276 risk and fbetahCG was 3.93 MoM. At 16 weeks, amniocentesis and chorionic villous sampling were performed due to placental appearance and possible terms of molar gestation or a chromosomal abnormality. Karyotype and placental histopathology were normal. At seven weeks after admission, her complaints were dissolved. Ultrasonography revealed that normal size of ovaries without ascites. She was discharged and followed up outpatient. At 22+3 week of pregnancy, she underwent cerclage due to cervical failure with Y formation. The pregnancy progressed to term and a healthy female baby of 3400 g was delivered at 40 weeks of gestation. Postoperative placental histopathology was normal. Four weeks after delivery, on sonographic examination, both ovaries were normal and betahCG was negative. She was managed expectantly with no complications. Conclusion(s): Our case, as well as literature data, indicates the importance of early diagnosis and successful management in pregnant women with sOHSS which may develop rapidly and may lead to significant morbidity and mortality if left untreated. (figure present).