Mesenteric and portal vein thrombosis, a rare complication in a patient with polycystic ovary syndrome: A case report and review of literature

Abstract

Introduction and importance

One of the rare complications of polycystic ovarian syndrome (PCOS) treated with combination contraceptives is venous thrombosis. However, there is currently no information on intestinal necrosis and portal venous thrombosis in polycystic ovary syndrome patients, and diagnosis is frequently delayed in these situations.

Clinical presentation

We report a case of a 30-year old female patient who experienced a sudden onset of rectal bleeding and severe abdominal pain. Superior mesenteric vein thrombosis was detected with Doppler ultrasonography. Right portal vein thrombosis was discovered on contrast-enhanced tomography of the abdomen; it was treated with enoxaparin sodium without improvement. A colonoscopy was then conducted; it revealed a distal descending colon with proximal sigmoid colon ischemia alterations. During a laparoscopic, ischemic portion were removed. On follow-up after two weeks, the patient was still on enoxaparin sodium (80 mg twice daily) in good general condition.

Clinical discussion

Portal vein thrombosis (PVT) and Superior mesenteric venous thrombosis (MVT) are rare forms of venous thrombosis and unusual conditions. Superior MVT related to hormonal contraception and PCOS is uncommon. To best of our knowledge, here we report the first case of PCOS presented with acute intestinal ischemia related to MVT.

Conclusion

Except for the correlation between PCOS and the use of combination contraceptives, no predisposing factor for portal vein thrombosis was found. Our case report indicates the need for clinicians to consider acute intestinal ischemia in patients with polycystic ovarian disease who have acute abdominal pain and atypical site of thrombosis.

Highlights

• •The risk of venous thrombosis increases by polycystic ovary syndrome and combined oral contraceptives.
• •Deep vein thrombosis and pulmonary embolism are the two common thromboses in polycystic ovary syndrome
• •Portal vein thrombosis and superior mesenteric vein thrombosis as a consequence of polycystic ovary syndrome is uncommon.

1. Introduction

Virchow's triad—blood flow stasis, endothelial damage, and hypercoagulability—leads to venous thrombosis. In some cases, particularly those with thrombophilia, a history of thrombosis, or those who have had cancer, venous thrombosis can also develop [1].

However, the risk of venous thrombosis and cardiovascular illness is also known to be increased by PCOS and combined oral contraceptives. Irrespective of the other risk factors, the prevalence of venous thrombosis was greater among women with PCOS than among women without PCOS [1]. According to one study, women with PCOS who used combined oral contraceptives had a 2-fold higher risk of venous thromboembolism than those who did not, having a 1.5-fold higher risk of venous thromboembolism [2].

Deep vein thrombosis of the lower limbs and pulmonary embolism are the two most common manifestations of PCOS-related venous thrombotic events. We report a patient with PCOS who received combined hormonal contraceptives as a first-line treatment and developed portal vein thrombosis and superior mesenteric vein thrombosis as a consequence. This triggered generalized ischemia of the intestine.

This case has been reported in line with SCARE criteria (see Methods section).

2. Case presentation

A 30-year-old female presented to the emergency department with a 5-day history of pain in her left lower abdomen. The pain was acute in onset, stabbing, not radiating, and increasing in severity for the previous three days, associated with multiple bilious vomiting throughout the day and rectal bleeding measuring about 250 ml. She also had an episode of black stools.

Doppler ultrasound was done, which showed superior mesenteric vein occlusion, so an abdominal and pelvic contrast Enhanced CT Scan (CECT) scan was done (Fig. 2), which revealed left-sided colitis and right intrahepatic posterior branch right portal vein thrombosis. She was given enoxaparin sodium (140 mg per day), a Proton pump inhibitor, anti-emetics drugs, and antibiotics. Moreover, the patient had a three days history of obstipation, and she had an episode of chills with a fever that was relieved with paracetamol. She also complained of unilateral left-sided headache, and she was given metamizole. After that, the patient was referred to our hospital and admitted into the surgical intensive care unit for further management.

Fig. 2.

Indicate the portal vein thrombosis in the CT scan.

Past medical history is significant for the polycystic ovarian syndrome, diagnosed in January 2022. The patient was on Metformin and combined oral contraceptive Pills for 25 days. She was on regular follow up by a Gynecologist. The patient had no known food or drug allergies. She had a positive past family history of premature coronary artery disease, in which her father suffered from myocardial infarction at the age of 39 years. There was no history of a similar previous attack, irritable bowel syndrome, abdominal pain, weight loss, past surgical history, or recent upper respiratory tract infection, particularly covid-19. And apart from the mentioned drugs, she doesn't take any other medication.

On arrival: Blood pressure was 123/76 mmHg, pulse was 94 beats/min, temperature was 37.5 °C, respiratory rate was 14 breaths/min, BMI was 25.8 kg/m2, and oxygen saturation was 96 %. On general examination, the patient looked well, conscious, and oriented. She was in pain but not in respiratory distress. The patient suffered from hirsutism, with no other skin, hair, or nail problems. On abdominal examination, her abdomen was distended with generalized tenderness more at left lower quadrant (LLQ), with no significance of palpable masses or organomegaly. There was no guarding or rigidity.

Routine investigations showed a white blood cell (WBC) count of 11,290/mm3 (N70), a hemoglobin (Hb) level of 15.2 mg/dl, platelet count was 343,000/mm3, and prothrombin time/international normalized ratio (PT/INR) was 14.7 s/1.41. Her liver function tests, renal function test, and amylase level results were normal. Lactate acid level of 31 mg/dl (ref, 5.7–22 mg/dl).CRP titer 141.1 mg/l and ESR 58 mm/h. Laboratory analysis for an inherited coagulopathy demonstrated negative findings for factor V Leiden, protein C, and protein S immunology and serology tests (Anti Cardiolipin IgM, Anti Cardiolipin IgG, B2-Glycoprotein IgG, B2-Glycoprotein IgM, and lupus AB) were normal. Tumor markers (CA 15-3, CA 19.9, and CEA), results were normal, except CA 125 which was elevated 171 u/ml, which is also expected in POCS patients [5]. Chest X-ray was within normal limits, and the ECG showed a regular sinus rhythm.

Furthermore, a colonoscopy was performed but stopped at the distal segment, showing a transitional area at the recto-sigmoid between the viable and gangrenous bowel within the sigmoid colon (Fig. 1). The procedure was well tolerated, and the patient was transferred into the recovery room.

Fig. 1.

Colonoscopy showing a transitional area at the recto-sigmoid between the viable and gangrenous bowel within the sigmoid colon.

An urgent laparoscopic colectomy was done, and it showed about 30–40 cm distal descending colon and proximal sigmoid colon ischemia with 200 ml bloody free fluids at the abdomen, small bowel, and omental adhesions were also seen at the ischemic segment with mass formation. The adhesions were released, and resection of the ischemic segments was done at the distal side with ligation by an Endovascular gastrointestinal anastomosis (Endo-GIA) stapler, with the creation of end colostomy. The resected colon segment “descending and sigmoid” was taken to the pathology department, where a manifestation showed that the serosa was of dark discoloration, the mucosa was congested, no mass or polyps were seen, the resection margins were viable, and no other remarkable pathology seen.

The patient's abdominal pain improved without recurrence of bloody diarrhea, and she was discharged from the hospital in stable condition. On follow-up after two weeks, the patient was still on enoxaparin sodium (80 mg twice daily) in good general condition.

Three months after the operation, the patient underwent second stage operation in which colo-rectal primary anastomosis was performed, with no significant Intra-abdominal pathology at exploration and healthy viable bowel.

3. Discussion

According to an epidemiological research, the prevalence of venous thromboembolism in reproductive-aged women who do not use contraception is approximately 3 per 10,000 women-years for women in their 30s and 5 per 10,000 for women in their 40s [3]. Among women with PCOS, the incidence of venous thromboembolism was 10.9/10,000 person-years. Women with PCOS who did not use oral contraceptives pills (OCP) had a 4.1/10,000 person-year incidence of venous thromboembolism [4].

Portal vein thrombosis (PVT) and Superior mesenteric venous thrombosis (MVT) are rare forms of venous thrombosis and unusual conditions. The most common risk factors for PVT and MVT are direct injury, local venous congestion, and hypercoagulability driven by thrombophilia or malignancy. However, Superior MVT related to hormonal contraception was first reported in 1963, three years after the first oral contraceptive pill was approved in the United States. As a result, hormonal medication may be the most common culprit triggering venous thrombosis in PCOS, particularly when it comes to the rarest position (porto-mesenteric venous system) [5,6].

A connection between PCOS and venous thrombosis may also exist. Three cases have been described: one involving cerebral sinus venous thrombosis and hereditary hypercoagulability; another involving thyroid vein thrombosis and inherited thrombophilic disorders; and a third involving an MTHFR gene mutation [7]. Moreover, a case of PCOS-related central retinal vein occlusion was described [8].

Five additional cases of OCP-associated intestinal ischemia related to mesenteric vein thrombosis were noted in the review of more recent published data [[11], [12], [13], [14], [15]]. Two of these cases did respond well to conservative treatment with heparin [9,15], while four patients required laparotomy [[11], [12], [13], [14]], and three required segmental bowel resections [[11], [12], [13],15]. In Table 1, the majority of patients had similar presentations of abrupt onset abdominal pain, with two of them also presenting with bloody diarrhea, and were all diagnosed after 10 days and treated with antibiotics first. Nobody has a notably significant medical history. Only our patient was identified to have PCOS. After early use, OCP was this patient's major risk factor for MVT.

Table 1.

Summarizing clinical characteristics of the acute mesenteric ischemia patients who use OCP.

Reference	Age	Medical history	Presenting signs and symptoms	Timing of AMI diagnoses	Imaging findings (CT with contrast)	Other sites of thrombosis	Treatment	Risk factors	Outcome
Case 1 [11]	52	Metrorrhagia	Epigastric pain and bloody diarrhea	10 days	Distended loops of small intestine with hyperemic mucosa and SMV thrombosis	PVT	Surgical resection	Smoking OCP 1 year	Discharge
Case 2 [12]	31	Knee arthroplasty and benign breast tumor excision.	Left lower quadrant abdominal pain	One day	Dilation and thickening of the small intestine, small pelvic fluid collection and free air	No	Surgical resection	OCP 3 month	Discharge
Case 3 [13]	38	No past medical history	Diffuse abdominal pain vomiting Fever	14 days	Showed acute venous mesenteric thrombosis	NO	intravenous heparin, Surgical resection	Smoking Obese OCP 4 month	Discharge
Case 4 [14]	16	No past medical history	Lower abdominal pain and hematochezia	11 days	Computed tomogram showed a distal small bowel obstruction, thickening of the terminal ileum, and a small pelvic fluid collection	No	Laparotomy	OCP	Discharge
Case 5 [15]	24	No past medical history	Epigastric pain	NR	Results revealed a thrombosed superior mesenteric vein and peripheral branches with retroperitoneal fat stranding about the vessel, which was compatible with thrombophlebitis	No	Placed on a heparin drip	Family history of DVT OCP	Discharge
Case 6 (our case)	30	PCOS	Lower abdominal pain, hematochezia vomiting	3 days	Left sided colitis, right intrahepatic posterior branch right portal vein thrombosis	PVT	Low molecular weight heparin, surgical resection	PCOS OCP	Discharge

NR: not reported, CT: computed tomography, PVT: portal vein thrombosis, SMV: superior mesenteric vein, PCOS: polycystic ovary syndrome, OCP: oral contraceptives.

Contrast-enhanced abdominal computed tomography, regarded as the gold standard and aids with diagnosis, reveals a distal small intestinal obstruction in some cases and minor pelvic fluid collection and free air in others. A thrombosed superior mesenteric vein was also found in the majority of them, and one patient had both PVT and MVT.

With systemic anticoagulation, the treatment is primarily conservative. Only those patients having intestinal infarction signs are eligible for surgical exploration. It is necessary to cease taking OCP. There should be a thorough screening for hypercoagulability [13].

4. Conclusion

The assessment of our case in light of the literature showed that the risk of VTE is very rare in patients taking OCP for PCOS, with a higher incidence during the early period of OCP use. Clinicians should keep in mind intestinal ischemia in female patients on OCP developing acute onset of severe abdominal pain without any evidence of another cause.

Ethical approval

This study is exempt from ethical approval at our hospital.

Source of funding

The study did not receive any funding.

CRediT authorship contribution statement

Data collection: Aseel Abuhammad, Mohammad Emar, Fahmi Jobran

Writing the manuscript: Osama N. Dukmak, Aseel Abuhammad, Mohammad Maraka

Study concept or design: Osama N. Dukmak, Saif Sayes

Review & editing the manuscript: Osama N. Dukmak, Aseel Abuhammad, Mohammad Maraka, Fahmi Jobran Saif Sayes

Guarantor

Dr. Mohammad Maraka.

Registration of research studies

N/A.

Methods

This case has been reported in line with SCARE criteria [16].

Consent

Written informed consent was obtained from the patient for reporting this case. The consent is available for review on request.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Declaration of competing interest

There is no conflict of interest to declare.