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Cyst of the fetal choroid plexus: A normal variant?
Antibody to endotoxin and postoperative infection
Volume 160 Number 2
5. Ziegler Ej, McCutchan jA, Fierer j, et al. Treatment of gram-negative bacteremia and shock with human antiserum to a mutant Escherichia coli. N Engl j Med 1982; 307:20: 1225-30. 6. Baumgartner jD, Glauser MP, McCutchan jA, Siegler EJ. Prevention of gram-negative shock and death in surgical
patients by antibody to core glycolipid. Lancet 1985;2:5966. 7. Lachman E, Pitsoe SB, Gaffin SL. Antilipopolysaccharide immunotherapy in the management of septic shock of obstetric and gynaecological origin. Lancet 1984; 1:981-3.
Cyst of the fetal choroid plexus: A normal variant? Beryl R. Benacerraf, MD, and Lane A_ Laboda, RDMS Boston, Massachusetts Cysts of the choroid plexus have been identified ultrasonographically in second-trimester fetuses and usually have regressed by 24 weeks' gestation. Chorioid plexus cysts have been linked with trisomy 18, and this possible association has prompted a review of our experience. Choroid plexus cysts were seen ultrasonographically in 38 consecutive fetuses between 15 and 28 weeks' gestation. Of these, 30 underwent repeat ultrasonograms after 24 weeks' gestation and showed complete resolution of the cysts. In 10 of the 38 fetuses, amniocentesis yielded normal karyotypes. A total of 36 patients were delivered of normal neonates at term. One patient was delivered of a normal neonate prematurely, at 34 weeks' gestation with a good outcome. Another fetus was delivered at 36 weeks' gestation because of late onset of nonimmune hydrops, which resolved without sequelae. No association between trisomy 18 and choroid plexus cysts was identified in this series. (AM J OasTET GYNECOL 1989;160:319-21.)
Key words: Fetus, ultrasonography, choroid plexus Cysts of the choroid plexus of the lateral ventricles are commonly found during an autopsy and are usually < 1 cm in size.' These cysts have been reported in both the neonate and the fetus. When first seen in the second-trimester fetus, they commonly regress by 22 to 24 weeks' gestation!" Although most reports suggest that choroid plexus cysts are benign and represent an incidental finding; 5 others have found an association between this finding and trisomy 18. 67 Because a correlation of this sort would alter guidelines for parental counseling and obstetric management, we reviewed our experience over 5 years, during which time we identified 38 fetuses with choroid plexus cysts between 16 and 28 weeks' gestation.
Material and methods Over 5 years 38 consecutive fetuses were found to have choroid plexus cysts as the only abnormality found during a prenatal ultrasonogram between 15 and 28 weeks' gestation. Ultrasonograms were performed with ACUSON 128 scanner (ACUSON, Mountainview,
From the Departments of Obstetncs and Gynecology and Radiology, Brigham and Women's Hospital, Harvard MedIcal School. ReceIVed for publication March 21, 1988; revLSed August 1, 1988,accepted August 2, 1988. Repnnt requests: Beryl R. Benacerraf, MD, 333 Longwood Ave., Boston, MA 02115.
Calif.) with a 3.5 MHz transducer. The presence of choroid plexus cysts was documented at the time of the initial scan, and a repeat ultrasonogram was requested as a follow-up. Each participant'S age at initial diagnosis, time of repeat scan (if done), karyotype (if available), and followup of her neonate were obtained by review of records and follow-up by telephone of each patient with her referring physician.
Results There were 38 consecutive fetuses that had one or multiple choroid plexus cysts as the only abnormality at the time of ultrasonography, and the gestational ages ranged between 16 and 28 weeks. A total of 14 fetuses were seen beween 16 and 18.4 weeks' gestation and 22 fetuses were seen between 18.5 and 21 weeks' gestation. There were two fetuses with choroid plexus cysts seen at 25 and 27 weeks' gestation, respectively. Follow-up ultrasonograms were obtained at 24 or more weeks' gestation for 30 of the 38 fetuses. In each of these cases the choroid plexus cysts were no longer visible. The indications for the initial ultrasonograms included dating or discrepancy in size and dates for 17 patients; amniocentesis for 10 patients; and sets of twins for two patients (only one twin in each set had choroid plexus cysts). Three fetuses were scanned for elevated maternal serum u-fetoprotein levels and one each was 319
320
Benacerraf and Laboda
Fig. 1. Transverse view through fetal head at 20 weeks' gestation shows unilateral choroid plexus cyst (arrow). Cyst was gone at time of repeat ultrasonogram 1 month later.
Februarv 1989 Am.J Obstet Gynew1
Fig. 3. Transverse view through choroid plexi (arrows) at 25 weeks' gestation shows there is no cyst. This fetus had cysts diagnosed at 17 weeks' gestation and returned for this follow-up.
of normal neonates as determined by physical examination at birth. One pregnancy resulted in premature delivery, at 34 weeks' gestation, of a normal neonate, who did well after a brief stay in the special care nursery. One fetus developed nonimmune hydrops late in pregnancy and was delivered at 36 weeks' gestation. The nonimmune hydrops was successfully treated in the special-care nursery and the cause was unknown. It was thought that the choroid plexus cyst seen in this fetus at 16 weeks' gestation (which was gone by 22.5 weeks' gestation) was unrelated to the nonimmune hydrops that developed in the third trimester. The infant is alive and well.
Comment
Fig. 2. Bilateral choroid plexus cysts (arrows) at 16 weeks' gestation seen during scan done before amniocentesis because of advanced maternal age. Karyotype was normal.
scanned for a previous child with a neural tube defect, a low serum a-fetoprotein level, abdominal pain, diabetes, referral from level 1 facility, and a molar pregnancy. Amniocentesis was performed on 10 of the 38 fetuses and yielded normal karyotypes. There were 22 fetuses that had unilateral choroid plexus cysts (two of which had more than one cyst), and 16 fetuses that had bilateral cysts (Figs. 1 to 3). A total of 36 pregnancies resulted in term delivery
Small choroid plexus cysts are occasionally seen in second-trimester fetuses, in both our experience and that of others."-' They usually regress by 24 weeks' gestation and are easily distinguished from hemorrhages or choroid plexus papillomas, which are usually echogenic. s The pathogenesis of these choroid plexus cysts is unclear but thought to represent filling in of neuroepithelial folds with cerebral spinal fluid and debris, which gives the impression of a cyst. 9 Asymptomatic cysts of the choroid plexus are found in 57% of autopsy cases and seem to occur in the fetus, the neonate, and the elderly.I-3 Increasing resolution of ultrasonographic equipment has probably made it possible to visualize these small cysts. Choroid plexus cysts rarely can be sufficiently large in the neonate to become symptmnatic.'" II
Cyst of fetal choroid plexus
Volume 160 Number 2
Recently several cases of choroid plexus cysts have been seen associated with trisomy 18, and these reports prompted the review of our experience reported here. 6 • 7 Our results differed from the findings of Nicolaides et al. 6 because all of our 38 fetuses with secondtrimester choroid plexus cysts had normal newborn examination findings at birth. Normal chromosomes were documented in 10 and the other 28 had no stigmata of aneuploidy at birth, although chromosome studies were not done. There was one premature birth with good outcome at 34 weeks' gestation, and one fetus was delivered at 36 weeks' gestation with an excellent recovery from idiopathic nonimmune hydrops that had developed in the third trimester. Although an occasional fetus with choroid plexus cysts may have trisomy 18, as previously reported, we suggest that the association of choroid plexus cysts with trisomy 18 described by Nicolaides et al. 6 may not apply to all populations or may be overestimated. In our experience most of these cysts are benign and lack apparent clinical sequelae. Trisomy 18 often involves obvious ultrasonographic abnormalities such as clubfeet, abnormal hands and forearms, and anomalies of the face, anterior abdominal wall, and diaphragmatic hernia. 12 The previous association of choroid plexus cysts with trisomy 18 would suggest that a thorough ultrasonographic examination of the fetus is warranted when a choroid plexus cyst is discovered ultrasonographically; however, on the basis of our sample the vast majority of fetuses with choroid plexus cysts appear to develop normally. It is difficult to draw conclusions or to counsel patients when a choroid plexus cyst is encountered on a prenatal ultrasonogram. Whether amniocentesis should be recommended to these patients is contro-
321
versial. Our data indicate the yield of abnormal karyotype is low, particularly if the rest of the ultrasonographic examination of the fetus is normal. Our series is drawn from 5 years of observation; however, because the actual number of cases is small, reports from other laboratories with regard to choroid plexus cysts will help define clearer guidelines for counseling patients and for obstetric management. REFERENCES I. Findlay JW. The choroid plexuses of the lateral ventricles of the brain, their histology, normal and pathological (in relation specially to insanity). Brain 1899;22:161. 2. Fakhry J, Schechter A, Tenner MS, et al. Cysts of the choroid plexus in neonates: documentation and review of the literature. J Ultrasound Med 1985;4:561. 3. Friday RO, Schwartz DB, Tuffti GA. Spontaneous intrauterine resolution of intraventricular cystic masses. J Ultrasound Med 1985;4:385. 4. Chudleigh P, Pearce J, Campbell S. The prenatal diagnosis of transient cysts of the fetal choroid plexus. Pre nat Diagn 1984;4:135. 5. Benacerraf BR. Asymptomatic cysts of the choroid plexus in the second trimester. J Ultrasound Med 1987;6:475. 6. Nicolaides KH. Rodek CH, Gosden CM. Rapid karyotypmg in non-lethal fetal malformation. Lancet 1986; I :283. 7. Bundy AL, Saltzman DH, Pober B, et al. Antenatal so no graphic findings in trisomy 18. J Ultrasound Med 1986;5:361. 8. Cappe IP, Lam AH. Ultrasound in the diagnosis of choroid plexus papilloma. JCU 1985; 13: 121. 9. Shuangshoti S, Roberts MP, Netsky MG. Neuroepithelial (colloid) cysts: pathogenesis and relation to choroid plexus and ependyma. Arch Path 1965;80:214. 10. Giorgi C. Symptomatic cyst of the choroid plexus of the lateral ventricle. Neurosurgery 1979;5:53. II. Neblett CR, Robertson JW. Symptomatic cysts of the telencephalic choroid plexus. J Neurol Neurosurg Psychiatry 1971 ;34:324. 12. Benacerraf BR, Miller WA, Frigoletto FD. Sonographic detection of fetuses with trisomy 13 and 18: accuracy and limitations. AM J OBSTET GYNECOL 1988; 158:404.
Volume 160 Number 2
5. Ziegler Ej, McCutchan jA, Fierer j, et al. Treatment of gram-negative bacteremia and shock with human antiserum to a mutant Escherichia coli. N Engl j Med 1982; 307:20: 1225-30. 6. Baumgartner jD, Glauser MP, McCutchan jA, Siegler EJ. Prevention of gram-negative shock and death in surgical
patients by antibody to core glycolipid. Lancet 1985;2:5966. 7. Lachman E, Pitsoe SB, Gaffin SL. Antilipopolysaccharide immunotherapy in the management of septic shock of obstetric and gynaecological origin. Lancet 1984; 1:981-3.
Cyst of the fetal choroid plexus: A normal variant? Beryl R. Benacerraf, MD, and Lane A_ Laboda, RDMS Boston, Massachusetts Cysts of the choroid plexus have been identified ultrasonographically in second-trimester fetuses and usually have regressed by 24 weeks' gestation. Chorioid plexus cysts have been linked with trisomy 18, and this possible association has prompted a review of our experience. Choroid plexus cysts were seen ultrasonographically in 38 consecutive fetuses between 15 and 28 weeks' gestation. Of these, 30 underwent repeat ultrasonograms after 24 weeks' gestation and showed complete resolution of the cysts. In 10 of the 38 fetuses, amniocentesis yielded normal karyotypes. A total of 36 patients were delivered of normal neonates at term. One patient was delivered of a normal neonate prematurely, at 34 weeks' gestation with a good outcome. Another fetus was delivered at 36 weeks' gestation because of late onset of nonimmune hydrops, which resolved without sequelae. No association between trisomy 18 and choroid plexus cysts was identified in this series. (AM J OasTET GYNECOL 1989;160:319-21.)
Key words: Fetus, ultrasonography, choroid plexus Cysts of the choroid plexus of the lateral ventricles are commonly found during an autopsy and are usually < 1 cm in size.' These cysts have been reported in both the neonate and the fetus. When first seen in the second-trimester fetus, they commonly regress by 22 to 24 weeks' gestation!" Although most reports suggest that choroid plexus cysts are benign and represent an incidental finding; 5 others have found an association between this finding and trisomy 18. 67 Because a correlation of this sort would alter guidelines for parental counseling and obstetric management, we reviewed our experience over 5 years, during which time we identified 38 fetuses with choroid plexus cysts between 16 and 28 weeks' gestation.
Material and methods Over 5 years 38 consecutive fetuses were found to have choroid plexus cysts as the only abnormality found during a prenatal ultrasonogram between 15 and 28 weeks' gestation. Ultrasonograms were performed with ACUSON 128 scanner (ACUSON, Mountainview,
From the Departments of Obstetncs and Gynecology and Radiology, Brigham and Women's Hospital, Harvard MedIcal School. ReceIVed for publication March 21, 1988; revLSed August 1, 1988,accepted August 2, 1988. Repnnt requests: Beryl R. Benacerraf, MD, 333 Longwood Ave., Boston, MA 02115.
Calif.) with a 3.5 MHz transducer. The presence of choroid plexus cysts was documented at the time of the initial scan, and a repeat ultrasonogram was requested as a follow-up. Each participant'S age at initial diagnosis, time of repeat scan (if done), karyotype (if available), and followup of her neonate were obtained by review of records and follow-up by telephone of each patient with her referring physician.
Results There were 38 consecutive fetuses that had one or multiple choroid plexus cysts as the only abnormality at the time of ultrasonography, and the gestational ages ranged between 16 and 28 weeks. A total of 14 fetuses were seen beween 16 and 18.4 weeks' gestation and 22 fetuses were seen between 18.5 and 21 weeks' gestation. There were two fetuses with choroid plexus cysts seen at 25 and 27 weeks' gestation, respectively. Follow-up ultrasonograms were obtained at 24 or more weeks' gestation for 30 of the 38 fetuses. In each of these cases the choroid plexus cysts were no longer visible. The indications for the initial ultrasonograms included dating or discrepancy in size and dates for 17 patients; amniocentesis for 10 patients; and sets of twins for two patients (only one twin in each set had choroid plexus cysts). Three fetuses were scanned for elevated maternal serum u-fetoprotein levels and one each was 319
320
Benacerraf and Laboda
Fig. 1. Transverse view through fetal head at 20 weeks' gestation shows unilateral choroid plexus cyst (arrow). Cyst was gone at time of repeat ultrasonogram 1 month later.
Februarv 1989 Am.J Obstet Gynew1
Fig. 3. Transverse view through choroid plexi (arrows) at 25 weeks' gestation shows there is no cyst. This fetus had cysts diagnosed at 17 weeks' gestation and returned for this follow-up.
of normal neonates as determined by physical examination at birth. One pregnancy resulted in premature delivery, at 34 weeks' gestation, of a normal neonate, who did well after a brief stay in the special care nursery. One fetus developed nonimmune hydrops late in pregnancy and was delivered at 36 weeks' gestation. The nonimmune hydrops was successfully treated in the special-care nursery and the cause was unknown. It was thought that the choroid plexus cyst seen in this fetus at 16 weeks' gestation (which was gone by 22.5 weeks' gestation) was unrelated to the nonimmune hydrops that developed in the third trimester. The infant is alive and well.
Comment
Fig. 2. Bilateral choroid plexus cysts (arrows) at 16 weeks' gestation seen during scan done before amniocentesis because of advanced maternal age. Karyotype was normal.
scanned for a previous child with a neural tube defect, a low serum a-fetoprotein level, abdominal pain, diabetes, referral from level 1 facility, and a molar pregnancy. Amniocentesis was performed on 10 of the 38 fetuses and yielded normal karyotypes. There were 22 fetuses that had unilateral choroid plexus cysts (two of which had more than one cyst), and 16 fetuses that had bilateral cysts (Figs. 1 to 3). A total of 36 pregnancies resulted in term delivery
Small choroid plexus cysts are occasionally seen in second-trimester fetuses, in both our experience and that of others."-' They usually regress by 24 weeks' gestation and are easily distinguished from hemorrhages or choroid plexus papillomas, which are usually echogenic. s The pathogenesis of these choroid plexus cysts is unclear but thought to represent filling in of neuroepithelial folds with cerebral spinal fluid and debris, which gives the impression of a cyst. 9 Asymptomatic cysts of the choroid plexus are found in 57% of autopsy cases and seem to occur in the fetus, the neonate, and the elderly.I-3 Increasing resolution of ultrasonographic equipment has probably made it possible to visualize these small cysts. Choroid plexus cysts rarely can be sufficiently large in the neonate to become symptmnatic.'" II
Cyst of fetal choroid plexus
Volume 160 Number 2
Recently several cases of choroid plexus cysts have been seen associated with trisomy 18, and these reports prompted the review of our experience reported here. 6 • 7 Our results differed from the findings of Nicolaides et al. 6 because all of our 38 fetuses with secondtrimester choroid plexus cysts had normal newborn examination findings at birth. Normal chromosomes were documented in 10 and the other 28 had no stigmata of aneuploidy at birth, although chromosome studies were not done. There was one premature birth with good outcome at 34 weeks' gestation, and one fetus was delivered at 36 weeks' gestation with an excellent recovery from idiopathic nonimmune hydrops that had developed in the third trimester. Although an occasional fetus with choroid plexus cysts may have trisomy 18, as previously reported, we suggest that the association of choroid plexus cysts with trisomy 18 described by Nicolaides et al. 6 may not apply to all populations or may be overestimated. In our experience most of these cysts are benign and lack apparent clinical sequelae. Trisomy 18 often involves obvious ultrasonographic abnormalities such as clubfeet, abnormal hands and forearms, and anomalies of the face, anterior abdominal wall, and diaphragmatic hernia. 12 The previous association of choroid plexus cysts with trisomy 18 would suggest that a thorough ultrasonographic examination of the fetus is warranted when a choroid plexus cyst is discovered ultrasonographically; however, on the basis of our sample the vast majority of fetuses with choroid plexus cysts appear to develop normally. It is difficult to draw conclusions or to counsel patients when a choroid plexus cyst is encountered on a prenatal ultrasonogram. Whether amniocentesis should be recommended to these patients is contro-
321
versial. Our data indicate the yield of abnormal karyotype is low, particularly if the rest of the ultrasonographic examination of the fetus is normal. Our series is drawn from 5 years of observation; however, because the actual number of cases is small, reports from other laboratories with regard to choroid plexus cysts will help define clearer guidelines for counseling patients and for obstetric management. REFERENCES I. Findlay JW. The choroid plexuses of the lateral ventricles of the brain, their histology, normal and pathological (in relation specially to insanity). Brain 1899;22:161. 2. Fakhry J, Schechter A, Tenner MS, et al. Cysts of the choroid plexus in neonates: documentation and review of the literature. J Ultrasound Med 1985;4:561. 3. Friday RO, Schwartz DB, Tuffti GA. Spontaneous intrauterine resolution of intraventricular cystic masses. J Ultrasound Med 1985;4:385. 4. Chudleigh P, Pearce J, Campbell S. The prenatal diagnosis of transient cysts of the fetal choroid plexus. Pre nat Diagn 1984;4:135. 5. Benacerraf BR. Asymptomatic cysts of the choroid plexus in the second trimester. J Ultrasound Med 1987;6:475. 6. Nicolaides KH. Rodek CH, Gosden CM. Rapid karyotypmg in non-lethal fetal malformation. Lancet 1986; I :283. 7. Bundy AL, Saltzman DH, Pober B, et al. Antenatal so no graphic findings in trisomy 18. J Ultrasound Med 1986;5:361. 8. Cappe IP, Lam AH. Ultrasound in the diagnosis of choroid plexus papilloma. JCU 1985; 13: 121. 9. Shuangshoti S, Roberts MP, Netsky MG. Neuroepithelial (colloid) cysts: pathogenesis and relation to choroid plexus and ependyma. Arch Path 1965;80:214. 10. Giorgi C. Symptomatic cyst of the choroid plexus of the lateral ventricle. Neurosurgery 1979;5:53. II. Neblett CR, Robertson JW. Symptomatic cysts of the telencephalic choroid plexus. J Neurol Neurosurg Psychiatry 1971 ;34:324. 12. Benacerraf BR, Miller WA, Frigoletto FD. Sonographic detection of fetuses with trisomy 13 and 18: accuracy and limitations. AM J OBSTET GYNECOL 1988; 158:404.