Child's Nervous System

, Volume 33, Issue 7, pp 1113–1123 | Cite as

Fetal ventriculomegaly: Diagnosis, treatment, and future directions

  • Jared M. PisapiaEmail author
  • Saurabh Sinha
  • Deborah M. Zarnow
  • Mark P. Johnson
  • Gregory G. Heuer
Focus Session


Fetal ventriculomegaly (VM) refers to the enlargement of the cerebral ventricles in utero. It is associated with the postnatal diagnosis of hydrocephalus. VM is clinically diagnosed on ultrasound and is defined as an atrial diameter greater than 10 mm. Because of the anatomic detailed seen with advanced imaging, VM is often further characterized by fetal magnetic resonance imaging (MRI). Fetal VM is a heterogeneous condition with various etiologies and a wide range of neurodevelopmental outcomes. These outcomes are heavily dependent on the presence or absence of associated anomalies and the direct cause of the ventriculomegaly rather than on the absolute degree of VM. In this review article, we discuss diagnosis, work-up, counseling, and management strategies as they relate to fetal VM. We then describe imaging-based research efforts aimed at using prenatal data to predict postnatal outcome. Finally, we review the early experience with fetal therapy such as in utero shunting, as well as the advances in prenatal diagnosis and fetal surgery that may begin to address the limitations of previous therapeutic efforts.


Fetal ventriculomegaly Hydrocephalus Fetus Fetal magnetic resonance imaging 



Atrial diameter


Aqueductal stenosis


Cerebrospinal fluid


Magnetic resonance imaging




Ventriculo-amniotic fluid





We thank Eo Trueblood, MA, lead medical illustrator at The Children’s Hospital of Philadelphia, for his creation of Figs. 1 and 2.

Compliance with ethical standards

Funding source

Pediatric Hydrocephalus Foundation Research Grant.

Financial disclosure

The authors have no financial relationships relevant to this article to disclose.

Conflict of interest

The authors declare that they have no conflicts of interest.


  1. 1.
    Edwards JH (1958) Congenital malformations of the central nervous system in Scotland. Br J Prev Soc Med 12:115–130PubMedPubMedCentralGoogle Scholar
  2. 2.
    Laurence KM, Carter CO, David PA (1968) Major central nervous system malformations in South Wales. I. Incidence, local variations and geographical factors. Br J Prev Soc Med 22:146–160PubMedPubMedCentralGoogle Scholar
  3. 3.
    Almog B, Gamzu R, Achiron R et al (2003) Fetal lateral ventricular width: what should be its upper limit? A prospective cohort study and reanalysis of the current and previous data. J Ultrasound Med 22:39–43CrossRefPubMedGoogle Scholar
  4. 4.
    Cardoza JD, Goldstein RB, Filly RA (1988) Exclusion of fetal ventriculomegaly with a single measurement: the width of the lateral ventricular atrium. Radiology 169:711–714. doi: 10.1148/radiology.169.3.3055034 CrossRefPubMedGoogle Scholar
  5. 5.
    Goynumer G, Yayla M, Arisoy R, Turkmen O (2014) The criterion value of fetal cerebral lateral ventricular atrium width for diagnosis of ventriculomegaly. Clin Exp Obstet Gynecol 41:67–71PubMedGoogle Scholar
  6. 6.
    McKechnie L, Vasudevan C, Levene M (2012) Neonatal outcome of congenital ventriculomegaly. Semin Fetal Neonatal Med 17:301–307. doi: 10.1016/j.siny.2012.06.001 CrossRefPubMedGoogle Scholar
  7. 7.
    Griffiths PD, Reeves MJ, Morris JE et al (2010) A prospective study of fetuses with isolated ventriculomegaly investigated by antenatal sonography and in utero MR imaging. AJNR Am J Neuroradiol 31:106–111. doi: 10.3174/ajnr.A1767 CrossRefPubMedGoogle Scholar
  8. 8.
    Norton M (2016) Fetal cerebral ventriculomegaly. In: UpToDateGoogle Scholar
  9. 9.
    Bruner JP, Davis G, Tulipan N (2006) Intrauterine shunt for obstructive hydrocephalus—still not ready. Fetal Diagn Ther 21:532–539. doi: 10.1159/000095668 CrossRefPubMedGoogle Scholar
  10. 10.
    Wang K-C, Lee JY, Kim S-K et al (2011) Fetal ventriculomegaly: postnatal management. Childs Nerv Syst 27:1571–1573. doi: 10.1007/s00381-011-1556-0 CrossRefPubMedGoogle Scholar
  11. 11.
    Von Koch CS, Gupta N, Sutton LN, Sun PP (2003) In utero surgery for hydrocephalus. Childs Nerv Syst 19:574–586. doi: 10.1007/s00381-003-0775-4 CrossRefPubMedGoogle Scholar
  12. 12.
    Pilu G, Reece EA, Goldstein I et al (1989) Sonographic evaluation of the normal developmental anatomy of the fetal cerebral ventricles: II. The atria. Obstet Gynecol 73:250–256PubMedGoogle Scholar
  13. 13.
    Melchiorre K, Bhide A, Gika AD et al (2009) Counseling in isolated mild fetal ventriculomegaly. Ultrasound Obstet Gynecol 34:212–224. doi: 10.1002/uog.7307 CrossRefPubMedGoogle Scholar
  14. 14.
    Sadan S, Malinger G, Schweiger A et al (2007) Neuropsychological outcome of children with asymmetric ventricles or unilateral mild ventriculomegaly identified in utero. BJOG 114:596–602. doi: 10.1111/j.1471-0528.2007.01301.x CrossRefPubMedGoogle Scholar
  15. 15.
    Atad-Rapoport M, Schweiger A, Lev D et al (2015) Neuropsychological follow-up at school age of children with asymmetric ventricles or unilateral ventriculomegaly identified in utero. BJOG 122:932–938. doi: 10.1111/1471-0528.12976 CrossRefPubMedGoogle Scholar
  16. 16.
    Durfee SM, Kim FM, Benson CB (2001) Postnatal outcome of fetuses with the prenatal diagnosis of asymmetric hydrocephalus. J Ultrasound Med 20:263–268CrossRefPubMedGoogle Scholar
  17. 17.
    Gaglioti P, Oberto M, Todros T (2009) The significance of fetal ventriculomegaly: etiology, short- and long-term outcomes. Prenat Diagn 29:381–388. doi: 10.1002/pd.2195 CrossRefPubMedGoogle Scholar
  18. 18.
    Kandula T, Fahey M, Chalmers R et al (2015) Isolated ventriculomegaly on prenatal ultrasound: what does fetal MRI add? J Med Imaging Radiat Oncol 59:154–162. doi: 10.1111/1754-9485.12287 CrossRefPubMedGoogle Scholar
  19. 19.
    Hannon T, Tennant PWG, Rankin J, Robson SC (2012) Epidemiology, natural history, progression, and postnatal outcome of severe fetal ventriculomegaly. Obstet Gynecol 120:1345–1353. doi: 10.1097/AOG.0b013e3182732b53 CrossRefPubMedGoogle Scholar
  20. 20.
    Gaglioti P, Danelon D, Bontempo S et al (2005) Fetal cerebral ventriculomegaly: outcome in 176 cases. Ultrasound Obstet Gynecol 25:372–377. doi: 10.1002/uog.1857 CrossRefPubMedGoogle Scholar
  21. 21.
    Sethna F, Tennant PWG, Rankin J, Robson SC (2011) Prevalence, natural history, and clinical outcome of mild to moderate ventriculomegaly. Obstet Gynecol 117:867–876. doi: 10.1097/AOG.0b013e3182117471 CrossRefPubMedGoogle Scholar
  22. 22.
    Pagani G, Thilaganathan B, Prefumo F (2014) Neurodevelopmental outcome in isolated mild fetal ventriculomegaly: systematic review and meta-analysis. Ultrasound Obstet Gynecol 44:254–260. doi: 10.1002/uog.13364 CrossRefPubMedGoogle Scholar
  23. 23.
    Cardoen L, De Catte L, Demaerel P et al (2011) The role of magnetic resonance imaging in the diagnostic work-up of fetal ventriculomegaly. Facts Views Vis ObGyn 3:159–163PubMedPubMedCentralGoogle Scholar
  24. 24.
    Pier DB, Levine D, Kataoka ML et al (2011) Magnetic resonance volumetric assessments of brains in fetuses with ventriculomegaly correlated to outcomes. J Ultrasound Med 30:595–603CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Parazzini C, Righini A, Doneda C et al (2012) Is fetal magnetic resonance imaging indicated when ultrasound isolated mild ventriculomegaly is present in pregnancies with no risk factors? Prenat Diagn 32:752–757. doi: 10.1002/pd.3896 CrossRefPubMedGoogle Scholar
  26. 26.
    Benacerraf BR, Shipp TD, Bromley B, Levine D (2007) What does magnetic resonance imaging add to the prenatal sonographic diagnosis of ventriculomegaly? J Ultrasound Med 26:1513–1522CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Salomon LJ, Ouahba J, Delezoide A-L et al (2006) Third-trimester fetal MRI in isolated 10- to 12-mm ventriculomegaly: is it worth it? BJOG 113:942–947. doi: 10.1111/j.1471-0528.2006.01003.x CrossRefPubMedGoogle Scholar
  28. 28.
    Manganaro L, Savelli S, Francioso A et al (2009) Role of fetal MRI in the diagnosis of cerebral ventriculomegaly assessed by ultrasonography. Radiol Med 114:1013–1023. doi: 10.1007/s11547-009-0434-2 CrossRefPubMedGoogle Scholar
  29. 29.
    Morris JE, Rickard S, Paley MNJ et al (2007) The value of in-utero magnetic resonance imaging in ultrasound diagnosed foetal isolated cerebral ventriculomegaly. Clin Radiol 62:140–144. doi: 10.1016/j.crad.2006.06.016 CrossRefPubMedGoogle Scholar
  30. 30.
    Kelly EN, Allen VM, Seaward G et al (2001) Mild ventriculomegaly in the fetus, natural history, associated findings and outcome of isolated mild ventriculomegaly: a literature review. Prenat Diagn 21:697–700CrossRefPubMedGoogle Scholar
  31. 31.
    Vergani P, Locatelli A, Strobelt N et al (1998) Clinical outcome of mild fetal ventriculomegaly. Am J Obstet Gynecol 178:218–222CrossRefPubMedGoogle Scholar
  32. 32.
    Parilla BV, Endres LK, Dinsmoor MJ, Curran L (2006) In utero progression of mild fetal ventriculomegaly. Int J Gynaecol Obstet 93:106–109. doi: 10.1016/j.ijgo.2006.01.026 CrossRefPubMedGoogle Scholar
  33. 33.
    Melchiorre K, Liberati M, Celentano C et al (2009) Neurological outcome following isolated 10-12 mm fetal ventriculomegaly. Arch Dis Child Fetal Neonatal Ed 94:F311–F312. doi: 10.1136/adc.2007.134312 CrossRefPubMedGoogle Scholar
  34. 34.
    Beeghly M, Ware J, Soul J et al (2010) Neurodevelopmental outcome of fetuses referred for ventriculomegaly. Ultrasound Obstet Gynecol 35:405–416. doi: 10.1002/uog.7554 CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Kutuk MS, Ozgun MT, Uludag S et al (2013) Postnatal outcome of isolated, nonprogressive, mild borderline fetal ventriculomegaly. Childs Nerv Syst 29:803–808. doi: 10.1007/s00381-013-2020-0 CrossRefPubMedGoogle Scholar
  36. 36.
    Devaseelan P, Cardwell C, Bell B, Ong S (2010) Prognosis of isolated mild to moderate fetal cerebral ventriculomegaly: a systematic review. J Perinat Med 38:401–409. doi: 10.1515/JPM.2010.048 CrossRefPubMedGoogle Scholar
  37. 37.
    Laskin MD, Kingdom J, Toi A et al (2005) Perinatal and neurodevelopmental outcome with isolated fetal ventriculomegaly: a systematic review. J Matern Fetal Neonatal Med 18:289–298. doi: 10.1080/14767050500329775 CrossRefPubMedGoogle Scholar
  38. 38.
    Breeze ACG, Alexander PMA, Murdoch EM et al (2007) Obstetric and neonatal outcomes in severe fetal ventriculomegaly. Prenat Diagn 27:124–129. doi: 10.1002/pd.1624 CrossRefPubMedGoogle Scholar
  39. 39.
    Graham E, Duhl A, Ural S et al (2001) The degree of antenatal ventriculomegaly is related to pediatric neurological morbidity. J Matern Fetal Med 10:258–263CrossRefPubMedGoogle Scholar
  40. 40.
    Baffero GM, Crovetto F, Fabietti I et al (2015) Prenatal ultrasound predictors of postnatal major cerebral abnormalities in fetuses with apparently isolated mild ventriculomegaly. Prenat Diagn 35:783–788. doi: 10.1002/pd.4607 CrossRefPubMedGoogle Scholar
  41. 41.
    Lee CS, Hong SH, Wang K-C et al (2006) Fetal ventriculomegaly: prognosis in cases in which prenatal neurosurgical consultation was sought. J Neurosurg 105:265–270. doi: 10.3171/ped.2006.105.4.265 PubMedGoogle Scholar
  42. 42.
    McGirt MJ, Leveque J-C, Wellons JC 3rd et al (2002) Cerebrospinal fluid shunt survival and etiology of failures: a seven-year institutional experience. Pediatr Neurosurg 36:248–255CrossRefPubMedGoogle Scholar
  43. 43.
    Varadi V, Toth Z, Torok O, Papp Z (1988) Heterogeneity and recurrence risk for congenital hydrocephalus (ventriculomegaly): a prospective study. Am J Med Genet 29:305–310. doi: 10.1002/ajmg.1320290209 CrossRefPubMedGoogle Scholar
  44. 44.
    Haratz KK, Nardozza LMM, de Oliveira PS et al (2011) Morphological evaluation of lateral ventricles of fetuses with ventriculomegaly by three-dimensional ultrasonography and magnetic resonance imaging: correlation with etiology. Arch Gynecol Obstet 284:331–336. doi: 10.1007/s00404-010-1666-z CrossRefPubMedGoogle Scholar
  45. 45.
    Gezer NS, Guleryuz H, Gezer C et al (2015) The prognostic role of prenatal MRI volumetric assessment in fetuses with isolated ventriculomegaly. Turk J Pediatr 57:266–271PubMedGoogle Scholar
  46. 46.
    Li Y, Estroff JA, Mehta TS et al (2011) Ultrasound and MRI of fetuses with ventriculomegaly: can cortical development be used to predict postnatal outcome? AJR Am J Roentgenol 196:1457–1467. doi: 10.2214/AJR.10.5422 CrossRefPubMedPubMedCentralGoogle Scholar
  47. 47.
    Hankinson TC, Vanaman M, Kan P et al (2009) Correlation between ventriculomegaly on prenatal magnetic resonance imaging and the need for postnatal ventricular shunt placement. J Neurosurg Pediatr 3:365–370. doi: 10.3171/2009.1.PEDS08328 CrossRefPubMedGoogle Scholar
  48. 48.
    Michejda M, Hodgen GD (1981) In utero diagnosis and treatment of non-human primate fetal skeletal anomalies. I. Hydrocephalus. JAMA 246:1093–1097CrossRefPubMedGoogle Scholar
  49. 49.
    Nakayama DK, Harrison MR, Berger MS et al (1983) Correction of congenital hydrocephalus in utero I. The model: intracisternal kaolin produces hydrocephalus in fetal lambs and rhesus monkeys. J Pediatr Surg 18:331–338CrossRefPubMedGoogle Scholar
  50. 50.
    Glick PL, Harrison MR, Halks-Miller M et al (1984) Correction of congenital hydrocephalus in utero II: efficacy of in utero shunting. J Pediatr Surg 19:870–881CrossRefPubMedGoogle Scholar
  51. 51.
    Edwards MS, Harrison MR, Halks-Miller M et al (1984) Kaolin-induced congenital hydrocephalus in utero in fetal lambs and rhesus monkeys. J Neurosurg 60:115–122. doi: 10.3171/jns.1984.60.1.0115 CrossRefPubMedGoogle Scholar
  52. 52.
    Emery SP, Greene S, Hogge WA (2015) Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther 38:81–85. doi: 10.1159/000382015 CrossRefPubMedGoogle Scholar
  53. 53.
    Birnholz JC, Frigoletto FD (1981) Antenatal treatment of hydrocephalus. N Engl J Med 304:1021–1023. doi: 10.1056/NEJM198104233041706 CrossRefPubMedGoogle Scholar
  54. 54.
    Clewell WH, Johnson ML, Meier PR et al (1982) A surgical approach to the treatment of fetal hydrocephalus. N Engl J Med 306:1320–1325. doi: 10.1056/NEJM198206033062202 CrossRefPubMedGoogle Scholar
  55. 55.
    Harrison MR, Filly RA, Golbus MS et al (1982) Fetal treatment 1982. N Engl J Med 307:1651–1652. doi: 10.1056/NEJM198212233072623 CrossRefPubMedGoogle Scholar
  56. 56.
    Manning FA, Harrison MR, Rodeck C (1986) Catheter shunts for fetal hydronephrosis and hydrocephalus. Report of the international fetal surgery registry. N Engl J Med 315:336–340. doi: 10.1056/NEJM198607313150532 CrossRefPubMedGoogle Scholar
  57. 57.
    Al-Anazi AR (2010) In utero ventriculo-uterine shunt treatment for fetal hydrocephalus: preliminary study of Al-Anazi ventriculo-uterine shunt. Neurosurg Q 20(1):1–4Google Scholar
  58. 58.
    Sutton LN, Sun P, Adzick NS (2001) Fetal neurosurgery. Neurosurgery 48:124PubMedGoogle Scholar
  59. 59.
    Cavalheiro S, Moron AF, Zymberg ST, Dastoli P (2003) Fetal hydrocephalus—prenatal treatment. Childs Nerv Syst 19:561–573. doi: 10.1007/s00381-003-0772-7 CrossRefPubMedGoogle Scholar
  60. 60.
    Al-Anazi A, Al-Mejhim F, Al-Qahtani N (2008) In uteroventriculo-amniotic shunt for hydrocephalus. Childs Nerv Syst 24:193–195. doi: 10.1007/s00381-007-0451-1 CrossRefPubMedGoogle Scholar
  61. 61.
    Emery SP, Hogge WA, Hill LM (2015) Accuracy of prenatal diagnosis of isolated aqueductal stenosis. Prenat Diagn 35:319–324. doi: 10.1002/pd.4520 CrossRefPubMedGoogle Scholar
  62. 62.
    Chen Y, Emery SP, Maxey AP et al (2016) A novel low-profile ventriculoamniotic shunt for foetal aqueductal stenosis. J Med Eng Technol 40:186–198. doi: 10.3109/03091902.2016.1154617 CrossRefPubMedGoogle Scholar
  63. 63.
    Saadai P, Runyon T, Farmer DL (2011) Fetal neurosurgery: current state of the art. Future Neurol 6:165–171. doi: 10.2217/fnl.11.3 CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Heuer GG, Adzick NS, Sutton LN (2015) Fetal myelomeningocele closure: technical considerations. Fetal Diagn Ther 37:166–171. doi: 10.1159/000363182 CrossRefPubMedGoogle Scholar
  65. 65.
    Davis GH (2003) Fetal hydrocephalus. Clin Perinatol 30:531–539CrossRefPubMedGoogle Scholar
  66. 66.
    Frigoletto FDJ, Birnholz JC, Greene MF (1982) Antenatal treatment of hydrocephalus by ventriculoamniotic shunting. JAMA 248:2496–2497CrossRefPubMedGoogle Scholar
  67. 67.
    Kulkarni AV, Drake JM, Mallucci CL et al (2009) Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus. J Pediatr 155:254–9.e1. doi: 10.1016/j.jpeds.2009.02.048 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Jared M. Pisapia
    • 1
    Email author
  • Saurabh Sinha
    • 1
  • Deborah M. Zarnow
    • 2
    • 3
  • Mark P. Johnson
    • 3
  • Gregory G. Heuer
    • 1
    • 3
    • 4
  1. 1.Department of NeurosurgeryUniversity of PennsylvaniaPhiladelphiaUSA
  2. 2.Division of NeuroradiologyChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.The Center for Fetal Diagnosis and TreatmentChildren’s Hospital of PhiladelphiaPhiladelphiaUSA
  4. 4.Division of NeurosurgeryChildren’s Hospital of PhiladelphiaPhiladelphiaUSA

Personalised recommendations