When having a baby can kill you

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Pregnancy is usually a happy time with the outcome being a healthy baby. However, some complications in pregnancy can be serious. Over the years I have cared for many couples with molar pregnancy (technically known as gestational trophoblast disease). This terrible complication of pregnancy not only results in grief from the “lost baby”, but can also have lasting physical, social and psychological consequences for both the mother and father (1,2,3,4). Untreated, molar pregnancy can cause death of the mother (1,2).

Of note, our research into molar pregnancy revealed that many fathers experienced lasting social and psychological symptoms following molar pregnancy (4). One reason for this is due to the origins of the disease.

Origins of molar pregnancy

There are two types of molar pregnancy, a complete mole and a partial mole. In both cases, the male sperm plays a key role.

In a complete molar pregnancy, sperm (one or two) fertilize an egg that has lost its female genetic material (DNA). Therefore all the genetic material in the fertilized egg arises from the male and none from the female.

In a partial molar pregnancy, a single egg is fertilized by two sperm causing an excess of male genetic material within the fertilised egg.

The incidence of molar pregnancy varies around the globe, from 1 in 200 to 1 in 2000 pregnancies (1,2).

Impact on pregnancy

Sadly, molar pregnancy never results in a normal baby except for the extraordinarily rare cases of twinning where one twin is a molar pregnancy and the other a normal pregnancy. Instead, the usual situation is that the uterus becomes full of abnormal placental tissue and no baby is present (complete mole) or some fetal development occurs, but the fetus  is  malformed and not viable (partial mole).

The abnormal placental tissue causes bleeding and can metastasis around to body to other organs such as the lungs, in the same way an untreated cancer may spread around the body.

Impact on women

Most molar pregnancies present with abnormal vaginal bleeding between 8 and 16 weeks of pregnancy. Initially most women are concerned about miscarriage.

The diagnosis may be strongly suspected following an ultrasound, where a characteristic pattern called a “snowstorm” may be seen within the uterus. However, the condition is not definitively diagnosed until a sample of the tissue within the uterus is sent for analysis (histopathology) and tissue that looks like a cluster of grapes (abnormal chorionic villi) are seen under the microscope.

Some women may present with signs of thyroid disease, as the abnormal placental tissue can produce thyroid-like hormones. Women may also present with excessive nausea and vomiting of pregnancy (hyperemesis) and rarely may present with abnormally high blood pressure readings under 20 weeks of pregnancy.

Management of molar pregnancy

Once a molar pregnancy is confirmed, management involves surgery, follow up surveillance and possibly chemotherapy.

The initial management is uterine suction curettage. This surgical procedure is necessary to confirm the diagnosis and exclude an even rarer form of gestational trophoblastic disease called choriocarcinoma. The surgery carries more risk than a usual suction curettage, as the abnormal placental tissue is very vascular, and therefore the risk of heavy bleeding is higher. This means that the attending gynaecologist will often cross match blood and organise an anaesthetic consultation to plan the safest time to perform surgery. Medication may be required following surgery to help contract the uterus and reduce post operative bleeding.

Following surgery, women receive a “risk rating” that is determined on a number of factors such as their levels of pregnancy hormone, blood group, the presence of metastatic disease and the histopathology of the molar pregnancy.

Based on the “risk rating” results, women enter a follow up surveillance program that involves monitoring with serial blood or urine pregnancy hormone levels.

If a women had a high initial risk score, or her pregnancy hormone levels rose or failed to fall during her surveillance period, then she will require chemotherapy. This is usually Methotrexate, but in some case will be combined chemotherapy.

The impact of molar pregnancy on women is often profound. This is particularly true as the risk of molar pregnancy increases as women become older. Some women may have been trying to conceive for many years and then discover their pregnancy is a molar pregnancy. Not only do they not have the baby they desire, they face surgery, prolonged surveillance during which pregnancy is contraindicated, and possible chemotherapy. They must defer trying to have a child until they have been cleared (3,4).

Impact on partners

Partners of affected women can also suffer due to delayed childbearing, prolonged stress and a feeling of guilt related to the male role in the origins of molar pregnancy (4,5,6).

In our research, we contacted 158 former patients in our service with molar pregnancy and through these women, interviewed  41 partners. We found many partners were as emotionally fragile as the woman. For full results click here.

In a thematic analysis we found several themes related to anxiety and fear, sadness and depression, and guilt.

Anxiety

Anxiety was the dominant theme, rather than depression. Anxiety arose in male partners from a sense of frustration consequent to experiencing loss of control over their fertility, particularly their anxiety that they, as a couple, may never have a child.

‘Wouldn’t have occurred to us before when we were just worried about possible health of a baby’

‘Words cannot describe how emotionally stressful it was… I witnessed my partner being torn apart emotionally.’

‘Almost given up hope/plans of having a child at our age (maybe still some fear that another pregnancycould go awry).’

‘My world came crashing down.’

Guilt and blame

Partners felt guilty or blamed themselves for the occurrence of the molar pregnancy. Factors such as the male contribution in conception and individual genetic structures impacted on male participant’s view of cause and effect.

‘I somehow feel responsible in a way that it may have been my fault that it had something to do with my (works) my body that wasn’t right that caused this unusual pregnancy.’

Medical care

Themes relating to medical care centred around the actual treatment of molar pregnancy and the constant reminder of the diagnosis during the prolonged follow up perios that meant couples relived the experience. This delayed emotional recovery. The lack of clear information added to confusion and uncertainty.

‘…we are constantly reminded of our ‘failure’ through monthly urine samples, etc.’

‘I didn’t know as a individual at the time what was going on with my partner because we didn’t have enough information.’

Male partners’ displacement of feelings

Male partners felt a sense of indirect involvement in the management of the molar pregnancy. A new unfamiliar distancing occurred in a small percentage of couple relationships because of withdrawal from communication with partners during this time. This left partners feeling hopeless, unable to initiate appropriate actions to help their partner cope with trauma resulting from the diagnosis.

Partners felt they had to manage other financial and social matters additionally during this period of time. The male partner viewed himself largely as a supporter and made a distinction from being the patient.

‘I still cannot imagine what it would be like for my partner as she was the one carrying the pregnancy.’

‘It is hard for the husband to feel the same sense of loss as the wife because he has not had any physical contact with the “baby”.’

‘I felt very detached from it because it wasn’t my body going through the miscarry.’

Sexual function

Some men reported disparity in sexual functioning with their partner and described sexual tensions in their relationship.

‘My partner seems a little numb now, compared with before, and that makes it harder to feel good about sex and being close. I’m still keen but she seems less so…’

‘With all this happening inside her, she now seems less interested in sex, maybe that’s normal, but when I try she looks almost scared.’

‘…reduced desire by wife/apprehension re. sexual, even sensual contact…’

Positive role of children

The protective effect of children came through in our research. Subsequent delivery of a healthy child overcame the sense of loss.  This was reported both as an actual experience and as a hypothetically positive experience.

‘The scars only really started healing once we were given the gift of a beautiful baby boy nearly 2 years later.’

Summary

Most of the time pregnancy is a happy event, but occasionally things go wrong. It is important to remember that both mother and father may be deeply impacted and to provide support and follow up when things don’t go to plan.

Ultimately, providing support to ensure the couple are able to help each other through a sad and frightening experience is as important as getting the actual medicine right.

 References

  1. Berkowitz RS, Goldstein DP. Gestational trophoblastic diseases. In Principles and Practice of Gynecologic Oncology, Hoskins WJ, Perez CA, Young RC (eds.), Lippincott Williams & Wilkins: Philadelphia, PA, 2000; pp 1117–1137.
  2. Feltman CM, Growden WB, Wolfberg AJ et al. Clinical characteristics of persistent gestational trophoblastic disease after partial hydatidiform molar pregnancy. J Reprod Med 2006;51:902–906.
  3. Berkowitz RS, Marean AR, Hamilton N et al. Psychological and social impact of gestational trophoblastic neoplasia. J Reprod Med 1980;25:14–16.
  4. Quinlivan JA, Ung KA, Petersen RW. The impact of molar pregnancy on the male partner.Psychooncology. 2012 Sep;21(9):970-6. doi: 10.1002/pon.1992. Epub 2011 May 24.
  5. Wenzel L, Berkowitz RS, Robinson S, Bernstein M, Goldstein D. The psychological, social, and sexual consequences of gestational trophoblastic disease. Gynecol Oncol 1992;46:74–81.
  6. Wenzel L, Berkowitz RS, Robinson S, Goldstein DP, Bernstein MR. Psychological, social and sexual effects of gestational trophoblastic disease on patients and their partners. J Reprod Med 1994;39(3):163–167.

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Ovarian cysts in teenagers

ovcyst

Most ovarian cysts in teenagers do not require surgery. Yet, over the years as a clinician, I have seen many teenagers who have presented to emergency centres and been subjected to an unnecessary surgical procedure.

Ovarian cysts are common in teenagers

Ovarian cysts are common in teenagers. The vast majority are simple cysts that develop following ovulation of an egg from the ovary. These “functional” or “simple” cysts are usually smaller than 4cm in diameter.

The image above is a simple ovarian cyst from one of my patients (provided with consent). The finding of the simple ovarian cyst was incidental when she was having a laparoscopy for another indication. Needless to say, I left her ovary well alone and four months later an ultrasound reported the cyst had spontaneously resolved.

Most “simple” ovarian cysts will resolve within six months and are asymptomatic.

Ovarian cysts can bleed or rupture

Occasionally “simple” ovarian cysts do cause problems.

This usually happens if they rupture, releasing fluid that irritates the lining of the abdomen to cause pain. Alternatively, there might  be bleeding from a small blood vessel lining the cyst wall. The blood causes the cyst to swell and become painful.

In both cases the pain is usually mild and resolves within few days.

However, occasionally pain may be more severe and result in hospital presentation.

This is where things can go astray. One typical scenario I have encountered after the event (that is after the teenager has had her ovary removed surgically!) involves the teenager being brought into the emergency department after hours by an anxious mother. The teenager reports she has developed pelvic pain. An ultrasound is performed and the “simple” ovarian cyst is misreported as being “complicated.” This is because blood inside the cyst causes ultrasound waves to be reflected and the cyst appears to have “internal echoes”.

A CT may be ordered to examine the cyst in more detail. Apart form exposing the teenager to a dose of radiation, this adds little to the diagnostic process. However, the intervention cascade that follows often ends up with emergency surgery, rather than the correct management of pain relief.

Even pain relief can go wrong. Keen young resident doctors frequently prescribe strong  codeine based analgesics. This can result in the patient re-presenting a few days later with a different cause of pain – constipation.  If a  CT was not ordered the first time around, it is certainly ordered on re-presentation. If the patient avoided surgery the first time, she may not be so lucky again facing an eager young registrar champing to operate.

The message is simple.

First, don’t operate on cysts in young women unless a consultant has reviewed the films and determined this is not a “simple” cyst or it is one of those very rare situations described below where emergency surgery is indicated.

Secondly, be careful with strong codeine based analgesia in young women. It leads to constipation and can confuse the presentation.

Ovarian torsion

Of course there are exceptions to every rule.

Ovarian torsion is the first exception.

Ovarian torsion occurs when the ovary twists around on its own blood supply. This stops blood flowing to the ovary and the lack of oxygen causes the ovary to become swollen and painful. It is a rare condition occurring in 4.9 per 100,000 women. It is more common in women over 20 years of age (1-3).

Ovarian torsion is a medical emergency as the ovary will die unless torsion is corrected.

Fortunately ovarian torsion can usually be distinguished by the clinical story (onset of pain is abrupt and there is frequently vomiting), the size of the cyst (larger cysts) and ultrasound features (abnormal blood flow seen on doppler of the ovarian vessels).

Ovarian tumours

These are rare in young women – exceedingly rare. Tumours usually look different on ultrasound. They may be solid, or have solid components with internal septations that delineate compartments within the cyst. Blood tests may demonstrate elevations in proteins called tumour markers, that rise in association with certain ovarian tumours.

Suspicious cysts in teenagers should always involve specialist management. It is usually wise to gather the correct information, and refer the teenager to a specialist hospital to be managed by gynaecology oncology staff, rather than rush and operate as an emergency. Even if specialist deem the cyst to be suspicious, and a decision is made to operate, it will still often be benign or harmless. In one series of 52 UK teenagers with suspicious ovarian cysts requiring surgery , only 3 tumours were eventually reported to be a malignant cancer (4).

Do unnecessary surgical procedures happen on simple ovarian cysts in teenagers?

The short answer is yes.

In one retrospective survey of children and teenagers presenting to the Royal Children’s Hospital in Melbourne with an ovarian cyst, 60% of the admitted patients had a “simple” cyst. despite this, many of the teenagers with “simple” cysts underwent a surgical procedure (5).

However, if the cyst is large, or the presentation is unusual, then consideration must be given to exclude a tumour.

So when in doubt, ask for a more senior opinion, and ask for your management options. Being admitted and observed is reasonable in some cases, rather than rushing to surgery.

References

  1. Hibbard LT. Adnexal torsion. Am J Obstet Gynecol. 1985;152:456–61.
  2. Dunnihoo DR, Wolff J. Bilateral torsion of the adnexa: a case report and review of the world literature. Obstet Gynecol. 1984;64:55S–59S.
  3. Kokoska ER, Keller MS, Weber TR. Acute ovarian torsion in children. Am J Surg. 2000;180:462–5.
  4. Tsikouras P1, Liberis V, Galazios G, et al. A 15-year report of pathological and benign ovarian tumors in teenagers. Eur J Gynaecol Oncol. 2008;29(6):602-7.
  5. de Silva KS, Kanumakala S, Grover SR, et al. Ovarian lesions in children and adolescents–an 11-year review. J Pediatr Endocrinol Metab. 2004 Jul;17(7):951-7.