A Loss of Twins and Missed Opportunities for Cerclage

Recently, a reader left the comment below. The value and use of cervical cerclage continues to come into question. There are major institutions in this country where it is not considered to be a useful procedure and have abandoned or severely limited its use to selective patients in deference to ‘conservative management’, often now involving the administration of progesterone during the pregnancy. I have addressed my feelings on cerclage in a series published on this site many months ago (between August 18 and September 26, 2008). My feelings have not changed. There is a big difference between getting a couple of extra weeks to an extremely early pregnancy, or holding off delivery long enough to ‘get steroids on board’ for fetal lung maturation, and delivering a baby beyond 30 weeks gestation when the risk of long-term complications of prematurity are greatly diminished. Barely a week goes by on our service when a patient would have lost a pregnancy in the manner detailed below except for the timely recognition of cervical insufficiency and the placement of a cerclage in later midtrimester…

On June 10 Anonymous wrote:
I have read one of your previous articles regarding cervical cerclage. I was diagnosed with endometriosis, treated with laproscopy and subsequently underwent many IUIs and one cycle of IVF without success. My second IVF cycle was successful, but due to preterm premature rupture of membranes (PPROM) at 21 weeks, lost healthy twins. No history of diabetes or hypertension. Doctors could not diagnose the reason for PPROM, may be due to cervical incompetence. I was on total bed rest, but had some vaginal bleeding at 11 weeks. I just wanted to know if cervical incompetence could have been diagnosed before and cervical cerclage would have been useful. What are my chances of undergoing normal conception?

To anonymous June 10:
Conception and successful carriage of a pregnancy are separate issues. It sounds like you had (and may still have) cervical insufficiency with the twin pregnancy. I firmly believe that all multiple gestations, particularly those resulting in infertility patients, should be carefully evaluated for premature cervical changes by transvaginal ultrasound beginning as early as 16 weeks. If cervical changes were picked up early enough, a cerclage may well have been successful in preventing your pregnancy loss.

Twenty-five years ago, detecting and treating cervical incompetence in a 'first pregnancy' was rarely successful. The diagnosis of cervical incompetence (insufficiency) was a diagnosis of exclusion, usually after one or more premature deliveries or midtrimester pregnancy losses. But because of the increased surveillance by ultrasound, it is almost a weekly event on our service.

With a subsequent pregnancy, I would recommend serial cervical evaluation by ultrasound even if you have a single baby. You might also be a candidate for an elective/prophylactic cerclage at 13-14 weeks if you have any other risk factors such as a congenital uterine abnormality or previous cervical surgery (e.g., LEEP or conization). In addition, even if you and your providers decide only upon serial ultrasound evaluation, you might consider weekly injections with 17-OH-progesterone caproate beginning at 16-18 weeks as well. I am sorry for your loss, but with careful follow-up and pregnancy management, you should be successful in the future.
Kind regards,
Dr T

Labels: 17-hydroxyprogesterone caproate, 17P, cerclage, cervical incompetence, cervical insufficiency; premature labor

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Low hCG and PAPP-A in a Patient with an Autoimmune Disorder

The following comment was left on an old post "Affect of Smoking on PAPP-A Levels in First Trimester." The case is interesting because it reminds us that abnormal maternal serum markers in first trimester might be the result of factors unrelated to fetal chromosomal abnormalities....

Hi! I just got my 1st trimester screening test results today, and would love you thoughts.

I will be 40 years old when I deliver. I was 12 weeks when I did the testing.

NT: 1.5
Nasal Bone present
Free b - HCG: 0.29 MoM
Papp A - 0.34 MoM

Down syndrome risk: 1:1600
Trisomy 18 & 13: 1:50 (normal for my age 1:150)

I have an autoimmune condition, polymyositis that is under control. I am taking 12.5mg prednisone 1xday. I am ANA and Jo-1 positive.

Can you please help me understand these results? I am trying to look at the fact that it is only a 2% chance of the trisomy abnormalities, but it is difficult.

Are there any other reasons that my blood levels would be so low for both? Is there anything I should be precautionary about through the rest of my pregnancy because of this?

Thanks,
WJD

The combination of the low hCG and the low PAPP-A is typically a pattern seen in pregnancies complicated by trisomies 18 and 13 (in contrast to an elevated hCG coupled with a low PAPP-A in Down syndrome – trisomy 21). Both hCG and PAPP-A are produced by the trophoblasts of the placenta and low values could be the result of either a small placental mass or decreased production because of metabolic dysfunction. Both of these factors might be at work in trisomies 18 and 13.

However, in your case, there is also the possibility that your baby is chromosomally normal but has an abnormality of placentation that resulted from your autoimmune disorder. The immune system probably plays a very important role in normal placentation and the presence of certain autoantibodies (e.g., antiphospholipid antibodies, lupus anticoagulants, and anti-beta-2-glycoprotein-1) are thought to be associated with increased risk for abnormalities of placentation resulting from abnormal trophoblast migration and invasion of the maternal spiral arterioles. Indeed, if you have not been screened for these specific autoantibodies, I would recommend that you have that done. Such pregnancies are at increased risk for poor fetal growth (intrauterine growth restriction), fetal loss, pregnancy-induced hypertensive disorders, and early delivery. If you have any of these other autoantibodies, you might also be at increased risk for thromboembolic complications as well.

Whenever we find a pregnancy that has low hCG and PAPP-A levels and a chromosomally normal baby, it is recommended that fetal growth be followed at serial intervals, Doppler flow studies (e.g., umbilical, uterine , and fetal middle cerebral arteries) be done to evaluate impaired placental perfusion from either the fetal or maternal side and evidence of fetal blood flow redistribution (preservation of the brain at the expense of perfusion of ‘nonessential’ organs because of reduced placental transfer), and both mother and baby be monitored carefully for evidence of compromise. Your doctors can give you specific details of what should be done with regard to the latter.

Best of luck to you and please let us know how things turn out.
Dr T

Labels: first trimester screening, hCG, PAPP-A

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Return of Dr. T

It has been several months since I have written and I have missed all of you. I was a little burned out by trying to keep up with my interim Chair and clinical responsibilities as well as the blog, but the greatest reason was that ‘Blogger’ was misbehaving and had been for many months – you couldn’t leave your comments and I couldn’t respond. I felt like I was talking to the wall and it was the need for and the feedback I got from the interactions with all of you that had given me the energy and inspiration to keep going up to that point.

Well, we finally have a new Chair and last week, ‘Blogger’ must have gotten a fix because more than 900 comments showed up in my mailbox in two days! So, now I have another dilemma…there is NO WAY I can answer all of you. However, in one of the batches of mail was the note below from Danita. I want to share it with you because it reminded me what I envisioned and have attempted to provide as my role on this site and it has been a strong incentive to return to the keyboard today…..

Hello Dr. T--it's Danita.
I originally posted a comment on your blog on August 14, 2008. I just wanted to give you a quick update. My son who was born at 26 weeks 6 days, and weighed 2 lbs. 9 oz. is now a healthy 9 month old. I was told like many of the other women that my baby probably wouldn’t make it. I was admitted to the hospital at 23 weeks after my water broke, and I was immediately placed on bed rest. I continued to leak fluid, and eventually after a week I did not have any fluid surrounding my baby. I was nervous, scared, and honestly confused by all of the facts that were being presented to me. I just wanted to let you know that I truly appreciate the encouragement that you provided to me as well as the other women that have, or are experiencing these difficult times during what is supposed to be the happiest time of our lives. You were honest, and gave me a sense of hope. After spending 4 long months in the NICU we finally got to bring him home in December of 2008. My son left the hospital a healthy baby with no medication, no oxygen--nothing. This is the same baby that I was told probably would not make it…I just had to share my journey, and my experience. I would like to send my condolences to the women on this blog who have experienced a loss. I have experienced that pain, and I know it is a very emotional time. I want to tell those same women along with all the other women on the blog to keep their faith strong. I know it is hard--- I’ve been there…but I wanted to share my story with all of you. I am not here to give you false hope, but maybe I can give you hope period. Dr. T— keep doing your thing!
Thank you again,
Danita

So, I am going to renew my pledge to push on with Fruit of the Womb. Although I will not be able to respond to all of the comments that have been left, I will try to catch up with the more recent ones and will certainly go back through ALL of the others to find common themes and situations that might be of general interest or are uniquely intriguing and thought-provoking. Much has gone on in Maternal-Fetal Medicine since we have talked last and I will try to catch everyone up on some of the more exciting advances as well.

It’s gonna be GREAT to be back!
Dr T

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Quintuplet Story: A Physician's Perspective - 4

On April 6, 2007, at 27 6/7 weeks, Joy began having more “pelvic pressure” and the decision was made to give her corticosteroids to accelerate fetal lung maturation. Serial ultrasounds had shown that one of the babies was developing intrauterine growth restriction and was having more difficulty pushing blood through its placenta as well, so the general sense was that delivery would probably be necessary soon. On that evening she began having regular contractions.

Initially magnesium sulfate was given to try to break her labor and then indomethacin was added but she continued to contract. At that point, a second dose of corticosteroids was given and I received a call at home to end my vacation early. By the time I arrived, Joy was having considerably more discomfort with her contractions. The cervix was noted to be stretched around the cerclage and the presenting baby’s head was well-applied to the cervix. As promised, I looked her in the eye and said, “It is time” and she nodded knowingly and agreed.

A “CODE 5” was called and the five NICU teams arrived within a short period of time from all over the city. At about 4:00 AM (4:11-4:13 AM to be exact) on April 7, 2007, Abram, Adal, Ian, Noelani, and Nadia were delivered to Joy and Andres Gonzalez at 28 weeks gestation by cesarean section in Greenville, South Carolina. The response and the entire “operation” went as smoothly as was planned and could have been hoped for and, fortunately, Joy had a relatively uncomplicated post-operative course.

The babies could not have been born into a stronger or more nurturing family. As difficult and dangerous as the pregnancy was for Joy, the time since the delivery (now almost two years) has proven how important the power of faith, love, and friendship can be to overcoming all obstacles. The first-born, Abram, had problems following delivery that led to a series of medical complications and hospitalizations that have stressed the family's resources, financially, and I am sure, personally, and may well continue to do so, but there has not been one instance during that time when we were visited by Joy, Andres, and all the children that there were not smiles on their faces, optimism in their voices, and enough love in their hearts to brighten all of our spirits.

Into each of our lives come events leading to memories that we carry with us until we’re gone. That has been especially true for me in medicine. There are many patients who, unbeknownst to themselves, sometimes gave to me more than I gave to them, and I will never forget those folks. Their memories are as clear to me now as the day they happened. They are the foundation of my approach to the practice of medicine, guiding my deductive and intuitive senses, and my inspiration to awaken early every day to a new set of challenges. They have made me a better physician and a better person. Such is the case with the Gonzalez family, and I am so grateful that they have let me be a part of their lives.
Dr T

Labels: quintuplets; multiple gestations

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Quintuplet Story - A Physician's Perspective - 3

...The following day, I had a long discussion with Joy and Andres about what to expect over the next 3-4 weeks, the discomforts, the psychological drain, the ever-present concerns for outcomes and the risks. The uterus is now the size of a term one. She had gotten to the point where she developed a rapid heart rate (tachycardia) and low blood pressure (hypotension) in almost any position. I explained that the placentas are like having 5 large arteriovenous (A-V) malformations (direct connections between the arterial and venous sides of the circulatory system) and that this was stressing her already stressed heart.

We talked about my concerns of uterine rupture, discussed plans for the delivery itself, hopefully under very controlled circumstances rather than as an emergency, and discussed the immediate peripartum concerns related to rapid fluid shifts that might increase her risk for congestive heart failure and pulmonary edema at the time of delivery. We revisited the concern that a hysterectomy might be necessary at the time of delivery as the result of uterine rupture, placenta accreta, or uterine atony with hemorrhage. I distinctly recall that she was not at all enthralled with that possibility if there was anything that could be done to prevent it. I informed them that earlier in the day, planning with Dr. Ohning from the NICU (Neonatal Intensive Care Unit) and his staff had begun in earnest now.

Over the next three weeks, she did very well. She flirted with preterm labor and continued to have the expected discomforts associated with hypotension, tachycardia, and general discomfort. At that point, I sent the following letter to my colleagues…

Joy Gonzalez is 27 weeks today. I have seen her just about every day she has been here, but I will be on vacation next week (but mostly in town) and wanted to apprise you all of where she stands.

Currently, she is on a running dose of indomethacin 25mg q6h. We are scanning the babies twice weekly to confirm well-being, assess amniotic fluid, and to do Doppler flow studies, including evaluation of patency of the ductuses when we can. At this point, unless the amniotic fluid begins to drop, I would stay the course with the indomethacin. That and the combination of vistaril and stadol in the early evening have helped control her uterine activity and anxiety. If the amniotic fluid drops, and everything else looks good, consider stopping the indomethcin for 48-72h. If the indomethcin has been the contributing factor to the decline in fluid, it will correct in that period of time. If it does not, or if there is differential correction, then we are probably dealing with placental insufficiency problems.

I took her off metronidazole earlier this week because of nausea and light-headedness and that seemed to help a lot. That can be restarted if her vaginal discharge returns and is bothersome to her. She was complaining of more “pressure” today (and over the past several days). The presenting baby is sitting right on the cervix but is still ballotable. The cervix remains long, but is extremely soft. There is no cervical dilation or tearing because of the cerclage at this point.

She continues to gain weight and her laboratory parameters have been stable. Albumin remains in the 2.3-2.4 range. She has made a major effort with regard to caloric intake. Her platelet count has drifted down, but was back in the 170,000 range yesterday. She has no abnormalities of her LFTs (liver function tests), LDH, etc. We have not done a formal glucola screen on her because all of her labs have always had glucose in the 70-80 range until yesterday’s random sugar (within an hour or so of breakfast) returned at 124. I still don’t know if a screen is worthwhile, but we might consider a fasting and 2hr pc evaluation to see if she has developed some carbohydrate intolerance along the way.

Her blood pressure is good, there is no edema, but her reflexes have become brisk within the past week. She has no specific complaints and is in the best spirits I have seen since she was admitted.

I have not yet given her steroids (to accelerate fetal lung maturity). My feeling there has been that if she gets to the point where we use magnesium to stop contractions or perhaps at 28 weeks, if not needed sooner, we could give them to her then. As the next step in tocolysis, if that becomes necessary, I would avoid nifedipine, consider a single dose or two of terbutaline (very carefully) to assess her response and tolerance to this, but probably go with the magnesium. If she needs magnesium, the plan is to transfer her to L&D. It is possible to get FHR(fetal heart rate) tracings on ALL of the babies and I suggest we start doing this every other day from this point forward.

We have worked closely with the nursery, L&D, and anesthesia to prepare for the delivery and everyone appears to have their roles well-defined. The plan will be to deliver her in O.R. C, nursery staff will take the first 3 babies to the ‘NICU annex’ we have set up in the recovery room directly handed off by us from the operating table, and the last 2 babies will be placed by us in the two warmers set up in O.R. C. The goal will be to make EVERY effort to deliver her on L&D. If she has complications and needs to be recovered in the SICU, that can be done afterwards. Anesthesia was pushing us to deliver her downstairs in the main O.R., but that creates almost an untenable situation for the nursery (and believe me, we have looked into that option). So unless we are in an extraordinary situation, please insist on delivering her upstairs.

Joy has a special request that we attempt to deliver the babies in the ‘order’ in which they have been evaluated antepartum. We have periodically drawn the orientation of the babies on her abdomen AND THEIR POSITIONS HAVE NOT CHANGED. This is not an unusual request under these circumstances and if it can be honored, that would be much appreciated. She would also like us to do “everything possible” to preserve her uterus.

The last is a special request on my part. I will tell her today that I may not be readily available over the next 11-12 days. After my vacation this week, I am then scheduled to go to that CDC meeting on the 10th and 11th. However, over the next week or so, I will try to get in to see her periodically. I will have my beeper and cell phone on and would truly appreciate a call if there is any significant change in her status. I would also like to be able to come in to assist with her delivery if at all possible. She is a very special patient to me and I know she would appreciate it too if I could be there to help out.

Thanks!

Labels: quintuplets; multiple gestations

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