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Fetal ascites associated with ABO incompatibility: Case report and review of the literature
CASE REPORTS
Fetal ascites associated with ABO incompatibility: Case report and review of the literature Robert J. Stiller, MD," Robert Herzlinger, MD, b Stacey Siegel, DO, ~ and John C.G. Whetham, MD" Bridgeport, Connecticut We report a pregnancy complicated by anti-B isoimmunization that resulted in fetal ascites, anemia, hepatomegaly, and polyhydramnios. A previous pregnancy in the same patient was complicated by neonatal ABO incompatibility. A review of the obstetric literature suggests that ABO incompatibility may cause severe fetal anemia, especially in patients with type O blood or a previous history of ABO incompatibility of the newborn. (Am J Obstet Gynecol 1996;175:1371-2.)
Key words: ABO incompatibility, fetal ascites, fetal anemia, isoimmunization
ABO incompatibility is a frequent cause of neonatal hemolytic disease in newborns with type A or B blood born to mothers with type O blood. It has rarely been reported to cause significant fetal disease. We report a case of anti-B isoimmunization manifesting with fetal ascites, anemia, hepatomegaly, and polyhydramnios in a pregnancy of a mother with type O blood. Her previous pregnancy was complicated by neonatal ABO incompatibility. A review of the literature suggests that maternal type O blood and previous neonatal ABO incompatibility may be risk factors for severe ABO incompatibility in the fetus. Case report The patient, a 32-year-old woman (gravida 2, para 1-00-1), had type O, Rh-positive blood. She was referred to our High-Risk Pregnancy Unit at 35 weeks' gestation with spontaneous rupture of membranes. The pregnancy had been uncomplicated until 2 days before admission, when fundal size appeared greater than expected and an ultrasonographic examination revealed fetal ascites and polyhydramnios. Results of a previous ultrasonographic examination performed at 23 weeks' gestation had been reported as normal.
From the Section of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology,~ and the Section of Neonatology, Department of Pediatrics,b Bridgeport Hospital. Received for publication March 7, 1996; revised April 16, 1996; acceptedJune 10, 1996. Reprint requests: Robert,[. Stilleg, MD, Section of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Bridgeport Hospital, 267 Grant Street, Bridgeport, CT 06610. Copyright © 1996 by Mosby-Year Book, Inc. 0002-9378/96 $5.00+ 0 6/1/75729
Her obstetric history was significant for a cesarean section for fetal distress with delivery of a 3175 gm female infant who had Apgar scores of 9 and 9 at 1 and 5 minutes. On physical examination, the infant was found to have hepatomegaly. Laboratory studies of the newborn revealed type B, Rh-positive blood; a hematocrit value of 32%; a reticulocyte count of 46%; and a positive direct Coombs' test. The peak total bilirubin concentration was 5.5 mg/dl, with a direct bilirubin concentration of 2.6 mg/dl. A diagnosis of ABO incompatibility was made, and the infant was treated conservatively. Phototherapy or exchange transfusion was not required, and the infant was discharged on the fourth day of life. At the time of admission for the current pregnancy, repeated ultrasonographic examination confirmed fetal ascites and polyhydramnios. No pleural or pericardial effusions were observed. No obvious ultrasonographic findings could explain the fetal ascites. Results of the fetal anatomic survey, including evaluation of cardiac structures, appeared normal. The fetal heart rate was 140 beats/rain. Ultrasonographic examination of the placenta did not reveal any placental masses. Initial laboratory studies confirmed type O, Rh-positive blood with a negative antibody screen. The patient denied any recent medication use, trauma, infections, or a family history of anemia. The Kleihauer-Betke test result was negative. Serologic tests for toxoplasmosis were immunoglobulin G (IgG) positive and immunoglobulin M (IgM) negative. Serologic tests for cytomegalovirus and parvovirus B19 were IgG and IgM negative, and the serologic test for syphilis was nonreactive. Because of the previous cesarean delivery, unfavorable cervix, and uncertain fetal status, delivery was performed by cesarean section. A 3070 gm female infant was delivered; she had Apgar scores of 8 and 9 at 1 and 5 minutes. Neonatal examinadon found a grossly distended abdomen and respiratory difficulty. Fetal ascites and hepatomegaly also were detected. Paracentesis was performed with the removal of 1371
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Novmeber 1996 AmJ ObstetGynecol
Stiller et al.
95 ml of ascitic fluid. Laboratory studies of the newborn revealed a hemoglobin level of 9.0 gm/dl, hematocrit value of 26.7%, white blood cell count of 21,000 cells/L, platelet count of 107 x 109 cells/L, and reticulocyte level of 26% of total erythrocytes. The direct Coombs' antibody test result was strongly positive. Chloroform eluti0n of the newborn red blood cells revealed anti-B IgG. An antibody titer specific for anti-B was determined for maternal serum and revealed IgG anti-B antibodies at a titer of 1 : 131,200. Evaluation for congenital infection was performed. The ophthalmologic examination result was normal, toxoplasmosis IgM antibodies were not found, and urine culture did not detect cytomegalovirus. The total bilirubin concentration peaked at 12.9 mg/dl, with a direct bilirubin level of 7 mg/dl, The infant required phototherapy and one transfusion for anemia but ultimately did well and was discharged home on the sixth day of life. Comment
ABO incompatibility is a common hematologic problem in newborns. Approximately 20 % to 25 % of pregnancies are at risk for ABO incompatibility, with mothers having anti-A or anti-B antibodies and their infants having the respective antigens. Newborns with the disease may have no symptoms or may have symptoms of hyperbilirubinemia, anemia, or cholestasis. Mild ABO incompatibility occurs in approximately 1 of 150 births, and severe disease is seen in approximately 1 of 3000 births. ABO incompatibility rarely results in significant fetal disease for several reasons. The A and B antigens are not well expressed on fetal red blood cells. Cells other than fetal red blood cells possess the A or B antigen and provide additional antibody-binding sites. Most anti-A and anfi-B antibodies are of the IgM type and do not cross the placenta. However, some patients with type O blood may preferentially produce anti-A and anti-B autoantibodies of the IgG type, which can cross the placenta. 1 Three cases of ABO incompatibility associated with hydrops fetalis have been reported in the recent literature. Gilja and Shah 2 reported a pregnancy in a 27-yearold woman, gravida 6, para 4, with type O, Rh-positive blood. At 32 weeks' gestation the fetus was found to have hydrops fetalis with fetal ascites and anasarca and was delivered. Laboratory assessment of the newborn re-
vealed a hematocrit value of 30%, reticulocyte concentradon of 20%, and bilirubin concentration of 3.5 mg/dl. The cord blood was direct Coombs' test positive, and anti-A IgG was eluted from the newborn cells. Serologic studies of maternal blood revealed an IgG anti-A antibody with a titer of 1 : 4000. In her previous pregnancy severe ABO incompatibility in the newborn required phototherapy and exchange transfusions. Scherer et al.1 reported a case of anti-B sensitization with hydrops fetalis at 34 weeks. In that case a 30-year-old woman, gravida 4, para 1-0-2-1, with type O, Rh-positive blood was delivered of a newborn with type B blood who had a cord hemoglobin level of 6.6 g m / d l and Apgar scores of 3 and 6 at 1 and 5 minutes. Specific maternal anti-B IgG titer was 1:65,536. The infant subsequently required multiple exchange transfusions but ultimately did well. The woman's previous pregnancy was not described. Miller and Petrie 3 reported a case of a 38-yearold woman, gravida 5, para 4, with type O, Rh-positive blood who was delivered of a 3250 gm, hydropic, stillborn infant with type A blood. The neonatal autopsy was unremarkable other than the finding of marked erythroblastosis. The maternal antibody screen was negative, but the maternal anti-A IgG titer was 1 : 1024. In two of her previous pregnancies this woman was delivered of infants with type A blood who required treatment for ABO incompatibility, which consisted of six neonatal exchange transfusions for the second infant and phototherapy for the third. Maternal type O blood and a history of previous ABO incompatibility appear to be risk factors for severe fetal presentations of ABO incompatibility. In these patients determination of maternal anti-A and anti-B IgG titers should be included in the evaluation of unexplained fetal ascites or hydrops.
REFERENCES
1. Sherer DM, AbramowiczJS,Ryan RM, Sheils LA, Blumberg N, WoodsJR. Severe fetal hydrops resulting from ABO incompatibility.AmJ Obstet Gynecol 1991;78:897-9. 2. Gilja BK, Shah VP. Hydrops fetalis due to ABO incompatibility. Clin Pediatr 1988;27:210-2. 3. Miller DF, Petrie SJ. Fetal erythroblastosis fetalis secondary to ABO incompatibility. Obstet Gynecol 1963;22:773-7.
Fetal ascites associated with ABO incompatibility: Case report and review of the literature Robert J. Stiller, MD," Robert Herzlinger, MD, b Stacey Siegel, DO, ~ and John C.G. Whetham, MD" Bridgeport, Connecticut We report a pregnancy complicated by anti-B isoimmunization that resulted in fetal ascites, anemia, hepatomegaly, and polyhydramnios. A previous pregnancy in the same patient was complicated by neonatal ABO incompatibility. A review of the obstetric literature suggests that ABO incompatibility may cause severe fetal anemia, especially in patients with type O blood or a previous history of ABO incompatibility of the newborn. (Am J Obstet Gynecol 1996;175:1371-2.)
Key words: ABO incompatibility, fetal ascites, fetal anemia, isoimmunization
ABO incompatibility is a frequent cause of neonatal hemolytic disease in newborns with type A or B blood born to mothers with type O blood. It has rarely been reported to cause significant fetal disease. We report a case of anti-B isoimmunization manifesting with fetal ascites, anemia, hepatomegaly, and polyhydramnios in a pregnancy of a mother with type O blood. Her previous pregnancy was complicated by neonatal ABO incompatibility. A review of the literature suggests that maternal type O blood and previous neonatal ABO incompatibility may be risk factors for severe ABO incompatibility in the fetus. Case report The patient, a 32-year-old woman (gravida 2, para 1-00-1), had type O, Rh-positive blood. She was referred to our High-Risk Pregnancy Unit at 35 weeks' gestation with spontaneous rupture of membranes. The pregnancy had been uncomplicated until 2 days before admission, when fundal size appeared greater than expected and an ultrasonographic examination revealed fetal ascites and polyhydramnios. Results of a previous ultrasonographic examination performed at 23 weeks' gestation had been reported as normal.
From the Section of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology,~ and the Section of Neonatology, Department of Pediatrics,b Bridgeport Hospital. Received for publication March 7, 1996; revised April 16, 1996; acceptedJune 10, 1996. Reprint requests: Robert,[. Stilleg, MD, Section of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Bridgeport Hospital, 267 Grant Street, Bridgeport, CT 06610. Copyright © 1996 by Mosby-Year Book, Inc. 0002-9378/96 $5.00+ 0 6/1/75729
Her obstetric history was significant for a cesarean section for fetal distress with delivery of a 3175 gm female infant who had Apgar scores of 9 and 9 at 1 and 5 minutes. On physical examination, the infant was found to have hepatomegaly. Laboratory studies of the newborn revealed type B, Rh-positive blood; a hematocrit value of 32%; a reticulocyte count of 46%; and a positive direct Coombs' test. The peak total bilirubin concentration was 5.5 mg/dl, with a direct bilirubin concentration of 2.6 mg/dl. A diagnosis of ABO incompatibility was made, and the infant was treated conservatively. Phototherapy or exchange transfusion was not required, and the infant was discharged on the fourth day of life. At the time of admission for the current pregnancy, repeated ultrasonographic examination confirmed fetal ascites and polyhydramnios. No pleural or pericardial effusions were observed. No obvious ultrasonographic findings could explain the fetal ascites. Results of the fetal anatomic survey, including evaluation of cardiac structures, appeared normal. The fetal heart rate was 140 beats/rain. Ultrasonographic examination of the placenta did not reveal any placental masses. Initial laboratory studies confirmed type O, Rh-positive blood with a negative antibody screen. The patient denied any recent medication use, trauma, infections, or a family history of anemia. The Kleihauer-Betke test result was negative. Serologic tests for toxoplasmosis were immunoglobulin G (IgG) positive and immunoglobulin M (IgM) negative. Serologic tests for cytomegalovirus and parvovirus B19 were IgG and IgM negative, and the serologic test for syphilis was nonreactive. Because of the previous cesarean delivery, unfavorable cervix, and uncertain fetal status, delivery was performed by cesarean section. A 3070 gm female infant was delivered; she had Apgar scores of 8 and 9 at 1 and 5 minutes. Neonatal examinadon found a grossly distended abdomen and respiratory difficulty. Fetal ascites and hepatomegaly also were detected. Paracentesis was performed with the removal of 1371
1372
Novmeber 1996 AmJ ObstetGynecol
Stiller et al.
95 ml of ascitic fluid. Laboratory studies of the newborn revealed a hemoglobin level of 9.0 gm/dl, hematocrit value of 26.7%, white blood cell count of 21,000 cells/L, platelet count of 107 x 109 cells/L, and reticulocyte level of 26% of total erythrocytes. The direct Coombs' antibody test result was strongly positive. Chloroform eluti0n of the newborn red blood cells revealed anti-B IgG. An antibody titer specific for anti-B was determined for maternal serum and revealed IgG anti-B antibodies at a titer of 1 : 131,200. Evaluation for congenital infection was performed. The ophthalmologic examination result was normal, toxoplasmosis IgM antibodies were not found, and urine culture did not detect cytomegalovirus. The total bilirubin concentration peaked at 12.9 mg/dl, with a direct bilirubin level of 7 mg/dl, The infant required phototherapy and one transfusion for anemia but ultimately did well and was discharged home on the sixth day of life. Comment
ABO incompatibility is a common hematologic problem in newborns. Approximately 20 % to 25 % of pregnancies are at risk for ABO incompatibility, with mothers having anti-A or anti-B antibodies and their infants having the respective antigens. Newborns with the disease may have no symptoms or may have symptoms of hyperbilirubinemia, anemia, or cholestasis. Mild ABO incompatibility occurs in approximately 1 of 150 births, and severe disease is seen in approximately 1 of 3000 births. ABO incompatibility rarely results in significant fetal disease for several reasons. The A and B antigens are not well expressed on fetal red blood cells. Cells other than fetal red blood cells possess the A or B antigen and provide additional antibody-binding sites. Most anti-A and anfi-B antibodies are of the IgM type and do not cross the placenta. However, some patients with type O blood may preferentially produce anti-A and anti-B autoantibodies of the IgG type, which can cross the placenta. 1 Three cases of ABO incompatibility associated with hydrops fetalis have been reported in the recent literature. Gilja and Shah 2 reported a pregnancy in a 27-yearold woman, gravida 6, para 4, with type O, Rh-positive blood. At 32 weeks' gestation the fetus was found to have hydrops fetalis with fetal ascites and anasarca and was delivered. Laboratory assessment of the newborn re-
vealed a hematocrit value of 30%, reticulocyte concentradon of 20%, and bilirubin concentration of 3.5 mg/dl. The cord blood was direct Coombs' test positive, and anti-A IgG was eluted from the newborn cells. Serologic studies of maternal blood revealed an IgG anti-A antibody with a titer of 1 : 4000. In her previous pregnancy severe ABO incompatibility in the newborn required phototherapy and exchange transfusions. Scherer et al.1 reported a case of anti-B sensitization with hydrops fetalis at 34 weeks. In that case a 30-year-old woman, gravida 4, para 1-0-2-1, with type O, Rh-positive blood was delivered of a newborn with type B blood who had a cord hemoglobin level of 6.6 g m / d l and Apgar scores of 3 and 6 at 1 and 5 minutes. Specific maternal anti-B IgG titer was 1:65,536. The infant subsequently required multiple exchange transfusions but ultimately did well. The woman's previous pregnancy was not described. Miller and Petrie 3 reported a case of a 38-yearold woman, gravida 5, para 4, with type O, Rh-positive blood who was delivered of a 3250 gm, hydropic, stillborn infant with type A blood. The neonatal autopsy was unremarkable other than the finding of marked erythroblastosis. The maternal antibody screen was negative, but the maternal anti-A IgG titer was 1 : 1024. In two of her previous pregnancies this woman was delivered of infants with type A blood who required treatment for ABO incompatibility, which consisted of six neonatal exchange transfusions for the second infant and phototherapy for the third. Maternal type O blood and a history of previous ABO incompatibility appear to be risk factors for severe fetal presentations of ABO incompatibility. In these patients determination of maternal anti-A and anti-B IgG titers should be included in the evaluation of unexplained fetal ascites or hydrops.
REFERENCES
1. Sherer DM, AbramowiczJS,Ryan RM, Sheils LA, Blumberg N, WoodsJR. Severe fetal hydrops resulting from ABO incompatibility.AmJ Obstet Gynecol 1991;78:897-9. 2. Gilja BK, Shah VP. Hydrops fetalis due to ABO incompatibility. Clin Pediatr 1988;27:210-2. 3. Miller DF, Petrie SJ. Fetal erythroblastosis fetalis secondary to ABO incompatibility. Obstet Gynecol 1963;22:773-7.