Hydroceph in 3 rd trimester itp


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Hydroceph in 3 rd trimester itp

  1. 1. vol25_no6_jum_745-814_online.q 5/17/06 1:25 PM Page 777 Case Report Fetal Hydrocephalus in a Pregnancy Complicated by Idiopathic Thrombocytopenic Purpura Min Woo Kim, MD, Hyung Min Choi, MD, PhD I diopathic thrombocytopenic purpura (ITP) is an autoimmune disorder in which the platelets of patients are destroyed by autoantibodies. It commonly affects young women, and obstetricians frequently come across these patients. There have been concerns about whether vaginal delivery may predispose the fetus to intracranial hemorrhage, and it has led many obstetricians to use cesarean delivery to prevent intracranial bleeding. The incidence of intracranial hemorrhage, however, in neonates born to the mother with ITP has been reported to be less than 1%. Moreover, intrauterine intracranial hemorrhage regardless of mechanical stress at delivery seems to be very rare. We report a pregnancy complicated by ITP in which intrauterine intracranial hemorrhage was diagnosed prenatally in the third trimester. Case Report Abbreviations ITP, idiopathic thrombocytopenic purpura Received January 9, 2006, from the Department of Obstetrics and Gynecology, Ilsanpaik Hospital, College of Medicine, Inje University, Gyeonggi-do, Korea. Revision requested January 12, 2006. Revised manuscript accepted for publication January 26, 2006. Address correspondence to Hyung Min Choi, MD, PhD, Department of Obstetrics and Gynecology, Inje University Ilsanpaik Hospital, Daehwa-dong, Ilsangu, Goyang-si, Gyeonggi-do 411-706, Korea. E-mail: hhyae97@ilsanpaik.ac.kr A 34-year-old woman, gravida 3, para 1, was referred at a gestational age of 33 weeks. The referral was based on her medical history of ITP which was diagnosed 6 years , before this pregnancy. Her laboratory data showed a platelet count of 2 × 109/L (normal range in a pregnant woman, 150–450 × 109/L) with a normal coagulation status. Immunologic examination findings were negative for antiplatelet antibody. On targeted ultrasonographic examination performed at a gestational age of 33 weeks 4 days, there was an intracerebral hypoechoic lesion adjacent to the lateral ventricle, which was a strong allegation for intracerebral hemorrhage (Figure 1). Measurement of the transverse diameter of the ventricular atrium showed a normal atrial width of 0.92 cm (normal atrial width, <1.0 cm) (Figure 2). On follow-up examination at a gestational age of 37 weeks 2 days, fetal hydrocephalus was newly developed (Figure 3). After immunoglobulin therapy, her platelet count was elevated to 8 × 109/L. After the transfu- © 2006 by the American Institute of Ultrasound in Medicine • J Ultrasound Med 2006; 25:777–780 • 0278-4297/06/$3.50
  2. 2. vol25_no6_jum_745-814_online.q 5/17/06 1:25 PM Page 778 Fetal Hydrocephalus With Idiopathic Thrombocytopenic Purpura Figure 1. Transverse axial sonogram at a gestational age of 33 weeks 4 days. An intracerebral hypoechoic lesion (arrow) is shown adjacent to the lateral ventricle. sion of 12 U of platelet concentrate, the pregnancy was terminated by cesarean delivery under general anesthesia. A female neonate weighing 2540 g was delivered with Apgar scores of 7 at 1 minute and 8 at 5 minutes. The neonate had moderate physical activity and crying. Head circumference was 34.5 cm (75th–90th percentile). The fontanel was soft and flat, and there was no evidence of caput or molding on the head. Multiple bruises were noted on the face and scalp, and petechiae were present on the whole body skin. The palate was perforated (cleft palate), and petechiae were also noted Figure 2. Transverse axial sonogram at 33 weeks 4 days (transventricular view). The transverse diameter of the ventricular atrium has a normal atrial width. 778 on it. Laboratory data showed a platelet count of 1 × 109/L (normal range in a neonate, 150–350 × 109/L) with a prolonged prothrombin time. Immunologic examination findings were negative for antiplatelet antibody and platelet-associated immunoglobulin G. On brain ultrasonography, moderate to severe hydrocephalus was noted, and there was no evidence of intraventricular hemorrhage (Figure 4). Computed tomography revealed diffuse ischemic and encephalomalatic changes in both hemispheres and severe hydrocephalus (Figure 5). The neonate had been treated with intravenous immunoglobulin therapy for 2 days (1 g ⋅ kg–1 ⋅ d–1). Because the platelet count remained low during hospitalization, and the compression of brain parenchyma due to hydrocephalus was not remarkable, extraventricular drainage was not performed. The infant was discharged on the 25th day. Since then, we have been following her in the outpatient department. Her platelet count was first normalized to 163 × 109/L on the 90th day, and the last data showed a platelet count of 151 × 109/L on the 114th day. Neurologic signs such as delay in eye contact and frequent vomiting subsequently developed, but the infant was still alive. Discussion Idiopathic thrombocytopenic purpura is an autoimmune disorder in which platelets are destroyed in the reticuloendothelial system by the antibody of the immunoglobulin G group against platelet surface antigen. It affects about 5% of the population and often develops in young women. The incidence of ITP in pregnant women has been reported as approximately 1 to 2 per 1000 pregnancies.1,2 The autoantibody can cross the placenta and induce neonatal thrombocytopenia. The risk of severe thrombocytopenia in neonates born to mothers with ITP seems to be very rare. The incidence of severe thrombocytopenia, defined as a platelet count at birth of less than 20 × 109/L, ranges from 1% to 5%.3 The diagnosis of ITP is based on the exclusion of other causes of thrombocytopenia. The presence of antiplatelet antibodies is regarded as a hallmark of ITP, but antigen-specific assays detect platelet-associated and plasma autoantibodies in about 50% to 75% of patients.4 The varJ Ultrasound Med 2006; 25:777–780
  3. 3. vol25_no6_jum_745-814_online.q 5/17/06 1:25 PM Page 779 Kim and Choi ious types of assays proved to be poor in sensitivity, specificity, and reproducibility.5 In addition, the antibody can also be seen in various other diseases, such as systemic lupus erythematosus and human immunodeficiency virus infection. As a result, the presence of the autoantibody cannot be used as a diagnostic criterion yet. In our case, maternal immune assay results were negative for the antiplatelet antibody. The purpose of management in pregnant women with ITP is to reduce the hemorrhagic complications by restoring hematologic profiles rather than to normalize the platelet count. The treatment options are corticosteroid therapy, intravenous immunoglobulin or anti-RhD, and plasmapheresis. To our knowledge, however, there have been no prospective trials guiding the treatment of pregnant patients with this disorder. In our case, the maternal platelet count was not sufficiently elevated after corticosteroid, immunoglobulin, and anti-RhD (WinRho) therapy. The fetus with severe thrombocytopenia is at increased risk of hemorrhage, including intracranial hemorrhage, which usually occurs in association with labor and delivery. Also, there are controversies with regard to the mode of delivery in pregnancies with ITP Some reports . have shown that neonatal intracranial hemorrhage are unrelated to the mode of delivery, which should be based on obstetric considerations only.6–8 In general, obstetricians prefer cesarean delivery to lower the risk of intracranial hemorrhage. Some interventions have been used to determine the fetal blood count, including cordocentesis, scalp sampling, and umbilical cord sampling. Some authors, however, emphasized the risk of these procedures, including doubts of reliability, and did not recommend performing these prenatal interventions.9,10 Recently, with advances in techniques and procedures, in utero therapies as well as diagnostic procedures have been reported to show promising success rates. In a review of the literature, Cook et al11 reported that the incidence rates of intracranial hemorrhage among neonates with severe thrombocytopenia (<50 × 109/L) were 4% after cesarean delivery and 5% after vaginal delivery. In our case, it is suspected that the intracranial hemorrhage caused the hydrocephalus. The mechanism by which intracranial hemorrhage J Ultrasound Med 2006; 25:777–780 Figure 3. Transverse axial sonogram at 37 weeks 2 days (transventricular view). Note that bilateral hydrocephalus has newly developed (arrow). led to hydrocephalus has been suggested (or suspected) to be that arachnoid granulations were obstructed by the breakdown products from the hemorrhage. Neurodevelopmental outcome is thought to be poor. With regard to neonatal prognosis, Boynton et al12 reported outcomes of 50 preterm neonates with posthemorrhagic hydrocephalus. The mortality rate was 7%; seizures developed in 38% of the patients; and limitations in motor function developed in 49%.12 There have been some reports about hydrocephalus caused by alloimmune thrombocytopenia, but hydrocephalus occurring after Figure 4. Brain sonogram on the day of birth. Moderate to severe hydrocephalus is shown, which is symmetric and compresses the brain parenchyma. There is no sign of intracerebral or intraventricular hemorrhage. 779
  4. 4. vol25_no6_jum_745-814_online.q 5/17/06 1:25 PM Page 780 Fetal Hydrocephalus With Idiopathic Thrombocytopenic Purpura References 1. 2. Warner MN, Moore JC, Warkentin TE, Santos AV, Kelton JG. A prospective study of protein-specific assays used to investigate idiopathic thrombocytopenic purpura. Br J Haematol 1999; 104:442–447. 5. Cines DB, Blanchette VS. Immune thrombocytopenic purpura. N Engl J Med 2002; 346:995–1008. 6. George JN, El-Harake MA, Raskob GE. Chronic idiopathic thrombocytopenic purpura. N Engl J Med 1994; 331: 1207–1211. 7. Letsky EA, Greaves M. Guidelines on the investigation and management of thrombocytopenia in pregnancy and neonatal alloimmune thrombocytopenia. Br J Haematol 1996; 95:21–26. 8. 780 Kelton JG. Idiopathic thrombocytopenic purpura complicating pregnancy. Blood Rev 2000; 16:43–46. 4. intracranial hemorrhage in fetuses of mothers with ITP has rarely been reported. In 1985, Morales and Stroup13 reported 1 case of intracranial hemorrhage in utero that developed in a neonate with isoimmune thrombocytopenia and commented that a similar condition can arise from maternal ITP Only 1 case, reported by . Tampakoudis et al,14 demonstrated a similar condition. They described a fetal intracranial hemorrhage that was first diagnosed at the beginning of the third trimester, possibly secondary to maternal ITP Similar to our case, no . sign of hemorrhage other than hydrocephalus was found in the neonatal evaluation after cesarean delivery. The neonate died 2 months after birth.14 In the absence of other neonatal diagnostic assay results, whether neonatal thrombocytopenia is attributable only to the maternal ITP is unclear. However, it can be an important cause of intracranial hemorrhage and subsequent hydrocephalus that spontaneously occur in a pregnancy complicated by ITP In addition, because the . infant in this case lived, we believe that our case may present important information about the prognosis of this condition. Gil KK, Kelton JG. Management of idiopathic thrombocytopenic purpura in pregnancy. Semin Hematol 2000; 37:275–289. 3. Figure 5. Computed tomogram taken on the second postnatal day shows severe hydrocephalus and diffuse ischemic and encephalomalatic changes in both hemispheres. al-Mofada SM, Osman ME, Kides E, al-Momen AK, al Herbish AS, al-Mobaireek K. Risk of thrombocytopenia in the infants of mothers with idiopathic thrombocytopenia. Am J Perinatal 1994; 11:423–426. Schwartz KA. Gestational thrombocytopenia and immune thrombocytopenias in pregnancy. Hematol Oncol Clin North Am 2000; 14:1101–1116. 9. Burrows RF, Kelton JG. Pregnancy in patients with idiopathic thrombocytopenic purpura: assessing the risks for the infant at delivery. Obstet Gynecol Surv 1993; 48:781– 788. 10. Silver RM, Branch W, Scott JR. Maternal thrombocytopenia in pregnancy: time for a reassessment. Am J Obstet Gynecol 1995; 173:479–482. 11. Cook RL, Miller RC, Katz VL, Cefalo RC. Immune thrombocytopenic purpura in pregnancy: a reappraisal of management. Obstet Gynecol 1991; 78:578–583. 12. Boynton BR, Boynton CA, Merritt TA, Vaucher YE, James HE, Bejar RF. Ventriculoperitoneal shunts in low birth weight infants with intracranial hemorrhage: neurodevelopmental outcome. Neurosurgery 1986; 18:141–145. 13. Morales WJ, Stroup M. Intracranial hemorrhage in utero due to isoimmune neonatal thrombocytopenia. Obstet Gynecol 1985; 65(suppl):20S–21S. 14. Tampakoudis P, Bili H, Lazaridis E, Anastasiadou E, Andreou A, Mantalenakis S. Prenatal diagnosis of intracranial hemorrhage secondary to maternal idiopathic thrombocytopenic purpura: a case report. Am J Perinatol 1995; 12: 268–270. J Ultrasound Med 2006; 25:777–780