Management of pregnancy in case of multiple and giant uterine fibroids

Abstract

Uterine fibroids are common among women of reproductive age. During the pregnancy, the potential complications of fibroids, although rare, are of frequent clinical concern. Available studies describing management and obstetrical outcomes in pregnant women with giant fibroids are limited. We present the case of a 39-year-old pregnant woman with multiple and large uterine fibroids. During the pregnancy, there was adequate fetal development, without major maternal complications. Given the characteristics of the fibroids and breech position of the fetus, an elective caesarean section was decided, and postpartum hysterectomy planned. This challenging obstetrical case required a multidisciplinary approach.

We considered crucial discussing five main issues: preconceptional counselling, tailored pregnancy surveillance, decision of time and route of delivery, decision to perform a peripartum hysterectomy and management of decreasing blood loss perioperatively. Given the limitation of the published reports, we believe that sharing our experience, along with a literature review, is beneficial for other clinicians.

Background

Uterine fibroids, the most common benign gynaecological tumours, are found in 40%–60% of women of reproductive age.¹ The estimated prevalence in pregnancy is 10.7%, depending on ethnicity.² In older women undergoing medically assisted reproduction techniques, the incidence rises (25%).³

The potential implications of fibroids in pregnancy are a concern. Although most patients do not have fibroid-related complications, 10%–30% of cases are associated with significant adverse outcomes.⁴ In particular, multiple (more than three) and large (greater than 5 cm) fibroids increase the risk of spontaneous miscarriage, preterm labour, placental abruption, premature rupture of membranes, fetal malpresentation, labour dystocia, caesarean delivery and postpartum haemorrhage and hysterectomy.^{5 6} When larger than 10 cm, they may cause restriction of the uterine cavity, causing fetal deformities (limb reduction, caudal dysplasia, head deformation and congenital torticollis) from the long-term compressive force.⁷ The pregnant woman may complain of exaggerated abdominal distension or severe pain due to myoma degeneration or torsion. Morbidity in pregnancy appears to be related to fibroid number, size, location and relationship to placenta implantation.⁸

Since data reporting obstetrical outcomes in patients with giant fibroids are scarce, the optimal conduct is unknown. In these patients, some dilemmas might be present, such as clinical follow-up, route and time of delivery, and whether to preserve the uterus. We present a case report of a pregnancy in a woman with giant and multiple uterine fibroids.

Case presentation

A 37-year-old nulliparous African-American woman, with a personal history of uterine leiomyomas and abnormal uterine bleeding, refused a multiple myomectomy in October 2016 due to the substantial risk of hysterectomy. She had an enlarged uterus extending up to one finger above the umbilical scar, and an ultrasound revealed multiple transmural/subserosal fibroids (International Federation of Gynecology and Obstetrics (FIGO) 2–5), the largest with 10.7 cm of greater axis. Therapy with ulipristal acetate (UPA) 5 mg daily was proposed. After two cycles of UPA she was asymptomatic, and we reassessed by ultrasound but did not find significant change in the size of fibroids. Despite the risks of pregnancy, she refused to take contraception, and was lost to follow-up.

In January 2018, age 39, she sought our clinic because she had a positive pregnancy test.

From the beginning, the medical team was apprehensive about the pregnancy. In our initial ultrasound assessment (6th week), the uterus was deformed by four fibroids with subserous and intramural component (<50%), without involving the uterine cavity, the largest measuring 18.2 cm of greater axis. The concern was that the size of the myomas would prevent development of a healthy pregnancy, or that it would be hazardous to the woman because of the risk of adjacent structures compression and postpartum haemorrhage. Despite the risks being extensively explained, our patient decided to proceed with the pregnancy.

We performed pregnancy surveillance in our maternal–fetal medicine clinic, with monthly appointments until the third trimester and, from then onwards, biweekly consultations. In the first trimester (10th week), ultrasound revealed the pregnancy located in the right hypochondrium, with the whole abdominal cavity occupied by giant fibroids. In the 15th week, our patient underwent MRI, which showed four main fibroid nodules (21, 15, 12, and 8.5 cm of greater axis) (figure 1). The volume of the largest fibroid was 3062 cm³.

Figure 1.

MRI at 15th week of pregnancy showing giant and multiple nodes of myoma and the pregnancy located in the upper half of the abdomen. The biggest nodule has an anterior location and measures 20.4×13.9×10.8 cm.

Notwithstanding the exuberant uterine leiomyomas, there was adequate fetal development and growth, with an estimated fetal weight at the 30th percentile at 33 weeks. The growth of the fibroids stabilised in the second and third trimester. Also, the placenta was posterior, and there were no signs of placental insufficiency. Photographs documented the evolution of abdominal distension (figure 2). Remarkably, despite the progressive growth of fibroids, the patient had only mild pelvic pain, particularly in the first trimester, which was well managed with analgesia, and mild dyspnoea in the third trimester.

Figure 2.

Evolution of abdominal distension during pregnancy (A) 15 weeks; (B) 21 weeks; (C) 27 weeks; (D) 36 weeks.

Treatment

Our primary goal was to deliver at term (37 weeks of gestation). However, the onset of frequent painful contractility led to an anticipation of the decision to terminate the pregnancy. A multidisciplinary team comprised gynaecologist-obstetricians, anaesthesiologists, paediatricians and immunohaemotherapy specialists was involved and decided to perform fetal lung maturity induction with corticosteroids and an elective caesarean section at 36 weeks and 5 days.

The medical team discussed with the patient the decision to perform a peripartum hysterectomy if significant haemorrhage occurred, which was accepted. However, she did not accept possibility of intraoperative blood and blood derivates’ transfusion. She signed a written consent regarding both situations.

Conservative approach with myomectomy was not considered because we expected significant bleeding complications.

We decided to perform the surgery on a working day for availability of support from the general surgery team. Internal iliac artery balloon occlusion or other radiological procedures were not available at the time of the delivery. The immunohaemotherapy department had a central role in optimising the patient’s preoperative haemoglobin (Hb 105 g/L), with iron supplementation and erythropoietin.

The surgery was performed under general anaesthesia, through a supra and infraumbilical midline incision. The uterus occupied the entire abdominal cavity, deformed by multiple myomas, the largest isthmic anterior exceeding 20 cm. The surgeons identified the single fibroid-free section in the upper third of the uterus and performed a corporal hysterotomy by a vertical incision. It was demanding to enlarge the hysterotomy considering the adjacent fibroids. Extraction of the fetus in breech presentation was laborious and occurred 9 min after skin incision. The baby was born with an Apgar score of 5/9/10 and weighed 2675 g. Placenta removal was uneventful.

After fetal and placental extraction, the patient had significant bleeding caused by uterus atony and placental bed haemorrhage. The anaesthetists administered prophylactic intravenous oxytocin (10 units in bolus and 10 units in perfusion at 500 mL/hour). The uterus was sutured with a haemostatic single-layer suture, followed by a total hysterectomy and bilateral salpingectomy. The surgery was technically challenging due to the lack of visibility and narrowed space, conditioned by the bleeding and the uterus size. The total surgical time was 145 min, and the estimated blood loss was 3 L. Intraoperatively, we administered 1 g of tranexamic acid and 2 g of fibrinogen. In the immediate postoperative period, the patient stayed at the postanaesthesia unit care, and we decided to give ferric carboxymaltose (1000 mg) because she had a Hb of 80 g/L.

Outcome and follow-up

On the first postoperative day, the patient was transferred to the puerperal ward.

On the third day post partum, because of persistent symptomatic anaemia (Hb 51 g/L), we discussed with the patient available medical approaches. She finally gave consent for a red blood cell (RBC) transfusion. After two RBC units’ transfusion, the patient had significant clinical improvement. These was no significant pain or vaginal bleeding. On the sixth day of puerperium, the patient was discharged with her newborn, asymptomatic, with a Hb 82 g/L.

The histology report described an enlarged uterus with 8840 g, with multiple leiomyomas with extensive hyaline degeneration.

We performed a follow-up appointment 6 weeks after delivery, and both mother and baby were well.

Discussion

There is much controversy regarding the growth of uterine myomas during pregnancy, but it is fair to say it cannot be predicted. Most authors agree that significant growth of uterine fibroids in the first trimester is expected.^{6 9 10} De Vivo et al⁹ found that 71.4% of uterine myomas increased >10% of volume between the first and second gestational periods. However, other authors, like Hammoud et al,⁵ stated that uterine myomas commonly decrease in volume throughout pregnancy. Neiger et al¹¹ also concluded with their findings that the enlargement of fibroids during pregnancy is rare. The opinions regarding the second and third trimesters are more consensual. Most authors reported a decrease in growth in the second trimester, up to stabilisation, and even a regression in the size of fibroids during the third trimester.¹ The hormonal and molecular mechanisms involved in fibroid modifications during pregnancy are unclear. There is an interesting theory that associates the increase of serum embryogenic human chorionic gonadotropin (hCG) in early pregnancy with Luteinizing Hormone (LH)-hCG myomal receptors hyperstimulation.¹²

One of the characteristics that contributed to the absence of fetal complications in our case is the fact that fibroids predominantly had a subserous and intramural component (<50%), without being in contact with the uterine cavity or placenta. Submucous fibroids have the strongest association with impaired implantation and placentation.³ Retroplacental myomas are associated with a higher incidence of miscarriage, intrauterine growth restriction, intrauterine fetal demise, preterm labour, placental abruption and postpartum haemorrhage.^{4 8} Also, our patient never had significant abdominal pain.

Surgical resection of myomas should be reserved for those pregnant women with torsion of a pedunculated myoma, leiomyomal cell necrosis and peritoneal reaction, where performing an urgent myomectomy may be the only therapeutic option.¹ Most authors report a preference for the laparoscopic approach for the treatment of fibroids in pregnancy.¹¹

In the absence of published recommendations, one of the most difficult decisions was the optimal time to plan delivery. We adapted our local guidelines to other haemorrhagic complications, such as vasa praevia or the placenta praevia, and decided pregnancy termination time at 36–37 weeks.

Due to fetal malpresentation (complete breech), we determined that the route of delivery should be a caesarean section. Even though a higher caesarean section rate is found among pregnant women with fibroids, with reported ranges between 34% and 73%, a vaginal delivery is preferable.¹³ Vergani et al¹⁴ reported that multiple fibroids, large fibroids, and fibroids in the lower uterine segment are predisposing factors for caesarean delivery. However, there are no definitive recommendations about the delivery route.

Since several studies suggest a 10-fold increased risk of postpartum bleeding, a multidisciplinary approach is crucial.^{15 16} Surgical planning may also help to maximise patient outcomes and reduce complications. In particular, a well-placed uterine incision, preferably a vertical incision, is essential to aid in fetal extraction, and to reduce bleeding and the risk of an intrapartum myomectomy/hysterectomy.

Some authors have studied the safety and feasibility of intracaesarean myomectomy and concluded that it can have a role in well-selected patients. The reported advantages are to avoid the need for interval myomectomy, decrease the risk of complications associated with fibroids in successive pregnancies, and increase the possibility of vaginal delivery in subsequent pregnancies.¹⁷ It can also be preferred in case of intramural myomas to decrease the odds of postpartum bleeding. The patients that should benefit the most of this approach are less than 40 years, have small to medium subserosal fibroids, and babies with birth weights <4000 g.¹⁸ This procedure should be avoided in cervical and intramural fibroids.¹⁹ Myomectomy at caesarean delivery can also be needed to facilitate the safe delivery of the fetus or closure of the hysterotomy.⁴

Uterine artery embolisation (UAE) and intrauterine balloon tamponade are two emergent options to manage persistent postpartum haemorrhage. The first has the advantage to also lead to myomal shrinkage, despite contradictory reports on its success rates, particularly regarding long-term outcomes.⁶ On the other hand, it has the disadvantages of being invasive, costly and associated with complications such as postembolisation syndrome, thromboembolic events and uterine necrosis.⁵ The second is an inexpensive and less invasive option, and although one-third of women who undergo intrauterine balloon tamponade will need an additional intervention, particularly if multiple fibroids, this technique has the potential to control a severe bleeding.⁶

Unfortunately, in our case, the anticipation of delivery due to the suspicion that the pregnant woman might go into spontaneous labour at a time when the surgical team was not available, was associated with the privation of our intervention radiology team. UAE or intrauterine balloon tamponade would have been an asset in this case. However, the decision of anticipation and its pros and cons were well considered by the multidisciplinary team.

Also, we considered that an intracaesarean myomectomy was not feasible. Deciding whether to preserve the uterus or to perform a peripartum hysterectomy is also controversial. Considering the high risk of uterine atony with massive postpartum bleeding, in a 39-year-old woman with no future fertility desire, we proposed a peripartum hysterectomy as the most reasonable decision in case of bleeding and the patient gave her consent. We believe that, in addition to the multidisciplinary team’s judgement, it is essential to involve the patient in the decision, always explaining the risks involved.

Another challenging point, which we would like to draw attention to, is correction of anaemia in a woman who refuses blood transfusions. In these cases, epoetin alfa (40 000 units subcutaneous once per week) and supplementation with intravenous iron preparations, folic acid and vitamin B₁₂ (cyanocobalamin) are crucial.²⁰ Volume therapy (crystalloids or colloids) and correcting potentially reversible coagulopathy (identified through coagulation studies) are of utmost importance.²¹

In conclusion, the presence of uterine fibroids during pregnancy is not uncommon and is likely to grow in the coming years with increasing maternal age. However, fibroids which cause a significant impact in pregnancy are rare. In uncommon clinical situations decisions are challenging, and a multidisciplinary approach is essential. During the pregnancy management, we tried to find literature to help support our clinical decisions, but we found the available data to be scarce, often underpowered and heterogeneous, mixing small and single with large and multiple fibroids. From our point of view, more case reports and series should be published to share experiences and support the best medical practice. Also, adequately powered prospective studies are mandatory to clarify the issue.

Patient’s perspective.

Pregnancy has always been my dream, and although I was medically advised against it, I decided to proceed. Throughout my pregnancy, I often felt the concern shown by obstetricians. Against all expectations I had no particular pain during my pregnancy. I would like to thank my obstetricians for the thoughtful manner in which they managed my case and to take the opportunity to ask the other international obstetricians to consider my example as a positive outcome, and thus give opportunity to other women like me to fulfil the desire of motherhood.

Learning points.

• Ideally, treatment of fibroids should occur preconceptionally. However, in the case of an unplanned pregnancy the risks must be explained clearly to the pregnant woman.

• It is essential to signal pregnant women at higher risk of complications, usually depending on the number, size or location of uterine fibroids.

• Pain is the most common complication of fibroids during the pregnancy. The symptoms are usually controlled by conservative treatment.

• An individualised approach coordinated by a multidisciplinary team is fundamental in the decision to terminate the pregnancy. The multidisciplinary team should include gynaecologist–obstetricians, anaesthesiologists, paediatricians, interventional radiologists and immunohaemotherapy specialists. A need for vascular or general surgeons should also be considered.

• There is no consensus regarding the route of delivery. Although the vaginal route is preferable because it is associated with a lower risk of bleeding, women with large (>5 cm), multiple or fibroids in the low segment of the uterus are at higher risk of needing a caesarean section. In the case of surgery, proper surgical planning is fundamental. A vertical midline incision is preferable.