Midtrimester hemoperitoneum caused by placenta percreta in association with elevated maternal serum (X-fetoprotein level

Robert S. McDuffie, Jr., MD, Laurel Harkness, MD, Randi M. McVay, MD, and Albert D. Haverkamp, MD

Denver, Colorado

We report a case of hemoperitoneum in the second trimester due to placenta percreta which was associated with an elevated maternal serum a-fetoprotein. A 29-year-old woman, gravida 4, para 1-0-2-1, was seen at 17 weeks' gestation with an acute abdomen. Maternal serum a-fetoprotein in a sample drawn 1 week previously revealed a value of 5.0 multiples of the median. At laparotomy, placenta percreta was discovered. This case of placenta percreta diagnosed In the second trimester was associated with an elevated maternal serum a-fetoprotein level. PhYSicians counseling patients with unexplained elevated maternal serum a-fetoprotein levels should include placenta accreta or percreta in the differential diagnosis and should maintain an awareness of its existence in patients with acute abdomen in pregnancy. (AM J OBSTET GVNECOL 1994; 171 :565-6.)

Key words: Placenta previa, placenta accreta, maternal serum a-fetoprotein, hemoperitoneum

Unexplained elevated maternal serum a-fetoprotein (AFP) has been associated with a variety of adverse pregnancy outcomes. I Recently, Zelop et at." reported a series of cases of placenta accreta, increta, and percreta associated with elevated maternal serum AFP levels. We report a case of hemoperitoneum occurring in the second trimester as a result of placenta percreta in association with an elevated maternal serum AFP value.

Case report A 29-year-old woman, gravida 4, para 1-0-2-1, was

seen at 17 weeks' gestation with sudden onset of lower abdominal pain. The patient reported nausea, vomit­ing, and anorexia on the day of admission. On physical examination the abdomen was distended and tender, particularly in the right lower quadrant. Bowel sounds were decreased. There was tenderness over the uterus, but no rebound abdominal tenderness. Stool was nega­tive for occult blood. On pelvic examination the cervix was long and closed with cervical motion tenderness. Complete blood count showed a white blood cell count of 20,500/mm" and a hematocrit value of 27.8%. Ultra­sonography showed a viable intrauterine fetus at 17 weeks 5 days' gestation. The anterior fundal placenta was unremarkable. No fetal anomalies were seen. As­cites and dilated loops of small bowel were seen in the maternal abdomen.

From the Department of Obstetno and Gynecology, Kaiser Penna­nente/Saint joseph HOlpltal. ReCflved for publicatwn December 15, 1993; reVised February 17, 1994; acU'pted Februal)' 28, 1993. Reprmts not avazlahle from the authors. Copyrzght © 1994 by Mosby-Year Book, Inc. 0002-9378/94 $3.00 + 0 6/1/55638

The history included two elective abortions and a low-transverse cesarean section because of dystocia. The patient also reported a similar episode of pain 1 month before admission. A sample for maternal serum AFP measurement was drawn 1 week previously, and results were unavailable to the clinicians at the time of presentation. A value of 5.0 multiples of the median was reported subsequently.

Exploratory laparotomy was performed. At laparot­omy a hemoperitoneum was found and 2500 ml of blood was evacuated from the abdominal cavitv. The omentum was adherent to the uterus, and the right fundal region of the uterus was bleeding from "excres­cences." On frozen section chorionic villi were identi­fied and were consistent with placenta percreta. The lower uterine segment (site of previous cesarean sec­tion) was not involved. The abdominal organs and bowel were normal. A bilateral hypogastric artery liga­tion was performed initially, followed by hysterectomy. The ligation was performed because of extensive blood loss as a temporizing measure before hysterectomy. Estimated blood loss for the procedure was :3500 ml; the patient received 2 units of packed red blood cells and 1 unit of whole blood. Pathologic examination revealed a normal fetus and placenta percreta.

Postoperatively the patient received intravenous an­tibiotics for presumptive cuff cellulitis. She was dis­charged on postoperative day 7.

Comment

This case furthers the association between placenta accreta and percreta and unexplained elevation of the maternal serum AFP level. In contrast to previous cases reported at term, this case occurred in the midtrimeter

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Rizk and Johansen

as an acute abdomen with hemoperitoneum. In retro­spect, the elevated maternal serum AFP level was un­explained on the basis of a normal result of obstetric ultrasonographic examination. Patients with unex­plained elevations of maternal serum AFP demonstrate a high rate of adverse pregnancy outcomes, including pre term delivery, intrauterine growth retardation, abruptio placentae, preeclampsia, and placenta accreta, increta, or percreta. I. 2

We recommend that clinicians who are evaluating patients with unexplained elevations of maternal serum AFP counsel their patients that placenta accreta, in­creta, or percreta can occur, although the frequency is extremely low. They should maintain an increased

August 1994 Am J Obstet Gynecol

awareness of placenta percreta when patients have symptoms of acute abdominal pain at any time in pregnancy. Finally, in the particular setting of a prior cesarean section, anterior placenta previa, and an un­explained elevated maternal serum AFP level, the pos­sibility of placenta accreta, increta, or percreta may become even more likely.

REFERENCES 1. Katz VL, Chescheir NC, Cefalo RC. Unexplained elevations

of matneral serum alpha-fetoprotein. Obstet Gynecol Surv 1990;45:719-26.

2. Zelop C, Nadel A, Frigoletto FD, Paulker S, MacMillan M. Benacerraf BR. Placenta accreta-percreta/increta: a cause of elevated maternal serum alpha-fetoprotein. Obstet Gynecol 1992;80:693-4.

Maternal steroid sulfatase deficiency - Cause of high-risk pregnancy?

Diaa E.E. Rizk, MSc, and Karl A. Johansen, MD

Cardiff, United Kingdom

A pregnancy is described in a woman with X-linked steroid sulfatase deficiency and an unaffected but growth-retarded male fetus. Her previous affected pregnancy that resulted in intrauterine fetal death was reported. Urinary estriol estimation in pregnancy is discussed. (AM J OBSTET GVNECOL 1994; 171 :566-7.)

Key words: Fetal growth retardation, steroid sulfatase deficiency, urinary estriol

We recently reported a pregnancy affected by placen­tal sulfatase deficiency with unexplained intrauterine fetal death and delivery by cesarean section. I This patient had a previous affected pregnancy. Her son was of low birth weight, and congenital icthyosis developed at the age of 9 months. The third, unaffected preg­nancy in the same patient is described here, in associa­tion with fetal growth retardation.

The patient conceived shortly after her affected preg­nancy. Ultrasonographic scanning suggested a structur­ally normal male fetus. At 30 weeks, the 24-hour urinary estriol level was low (40 mmol, normal 42 to 245 mmol)

From the Department of Obstetrics and Gynecology, Liandough HospItal. ReceIVed for publicatIOn September 9, 1993; reVISed September 29. 1993; accepted March 3, 1994. Repnnt requests: Karl A. Johansen. MD, Department of Ob,tetncs and Gynaecology, Llandough HospItal, Penarth. CardIff, Umted Kmgdom CF64 2XX. Copyright © 1994 by Mosby-Year Book, Inc. 0002-9378/94 $3.00 + 0 6/1/55622

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but placental sulfatase deficiency was excluded by a normal urinary l6a-hydroxydehydroepiandrosterone sulfate (16a-hydroxy-DHAS) value (16 mmol, normal < 20 mmol). Similar low values for both hormones were reported at 32 and 34 weeks. Subsequently retarded fetal growth was diagnosed clinically and ultrasono­graphically. At 37 weeks fetal distress necessitated de­livery by cesarean section of a live male infant weighing 2.35 kg, who has developed normally. The low maternal urinary estriol level remains unexplained, but placental sulfatase deficiency is unlikely because the urinary l6a­hydroxy-DHAS value was normaF

In the absence of anencephaly or other congenital or maternal factors additional causes oflow estriol levels in pregnancy are placental dysfunction and primary con­genital adrenal hypoplasia. The former seems probable because the fetus was growth-retarded without neonatal evidence of adrenal hypofunction. It is noteworthy that low maternal urinary estriol levels were identified be­fore biophysical impairment was evident. Therefore we