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Fetal ventriculomegaly: Diagnosis, treatment, and future directions

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Abstract

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.

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Abbreviations

AD:

Atrial diameter

AS:

Aqueductal stenosis

CSF:

Cerebrospinal fluid

MRI:

Magnetic resonance imaging

US:

Ultrasound

VA:

Ventriculo-amniotic fluid

VM:

Ventriculomegaly

References

  1. Edwards JH (1958) Congenital malformations of the central nervous system in Scotland. Br J Prev Soc Med 12:115–130

    CAS  PubMed  PubMed Central  Google Scholar 

  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 22:146–160

    CAS  PubMed  PubMed Central  Google Scholar 

  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 22:39–43

    Article  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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 41:67–71

    CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  8. Norton M (2016) Fetal cerebral ventriculomegaly. In: UpToDate

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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 73:250–256

    CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  16. Durfee SM, Kim FM, Benson CB (2001) Postnatal outcome of fetuses with the prenatal diagnosis of asymmetric hydrocephalus. J Ultrasound Med 20:263–268

    Article  CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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 3:159–163

    CAS  PubMed  PubMed Central  Google Scholar 

  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 30:595–603

    Article  PubMed  PubMed Central  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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 26:1513–1522

    Article  PubMed  PubMed Central  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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 21:697–700

    Article  CAS  PubMed  Google Scholar 

  31. Vergani P, Locatelli A, Strobelt N et al (1998) Clinical outcome of mild fetal ventriculomegaly. Am J Obstet Gynecol 178:218–222

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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 10:258–263

    Article  CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    PubMed  Google Scholar 

  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 36:248–255

    Article  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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 57:266–271

    PubMed  Google Scholar 

  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

    Article  PubMed  PubMed Central  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  48. Michejda M, Hodgen GD (1981) In utero diagnosis and treatment of non-human primate fetal skeletal anomalies. I. Hydrocephalus. JAMA 246:1093–1097

    Article  CAS  PubMed  Google Scholar 

  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 18:331–338

    Article  CAS  PubMed  Google Scholar 

  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 19:870–881

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  52. Emery SP, Greene S, Hogge WA (2015) Fetal therapy for isolated aqueductal stenosis. Fetal Diagn Ther 38:81–85. doi:10.1159/000382015

    Article  PubMed  Google Scholar 

  53. Birnholz JC, Frigoletto FD (1981) Antenatal treatment of hydrocephalus. N Engl J Med 304:1021–1023. doi:10.1056/NEJM198104233041706

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  55. Harrison MR, Filly RA, Golbus MS et al (1982) Fetal treatment 1982. N Engl J Med 307:1651–1652. doi:10.1056/NEJM198212233072623

    Article  CAS  PubMed  Google Scholar 

  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

    Article  CAS  PubMed  Google Scholar 

  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):1–4

  58. Sutton LN, Sun P, Adzick NS (2001) Fetal neurosurgery. Neurosurgery 48:124

    CAS  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  PubMed Central  Google Scholar 

  64. Heuer GG, Adzick NS, Sutton LN (2015) Fetal myelomeningocele closure: technical considerations. Fetal Diagn Ther 37:166–171. doi:10.1159/000363182

    Article  PubMed  Google Scholar 

  65. Davis GH (2003) Fetal hydrocephalus. Clin Perinatol 30:531–539

    Article  PubMed  Google Scholar 

  66. Frigoletto FDJ, Birnholz JC, Greene MF (1982) Antenatal treatment of hydrocephalus by ventriculoamniotic shunting. JAMA 248:2496–2497

    Article  PubMed  Google Scholar 

  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

    Article  PubMed  Google Scholar 

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Acknowledgements

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

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Authors and Affiliations

  1. Department of Neurosurgery, University of Pennsylvania, 3400 Spruce Street, 3rd Floor Silverstein Pavilion, Philadelphia, PA, 19104, USA

    Jared M. Pisapia, Saurabh Sinha & Gregory G. Heuer

  2. Division of Neuroradiology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Deborah M. Zarnow

  3. The Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Deborah M. Zarnow, Mark P. Johnson & Gregory G. Heuer

  4. Division of Neurosurgery, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Gregory G. Heuer

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  1. Jared M. Pisapia
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  2. Saurabh Sinha
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Correspondence to Jared M. Pisapia.

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Pediatric Hydrocephalus Foundation Research Grant.

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Pisapia, J.M., Sinha, S., Zarnow, D.M. et al. Fetal ventriculomegaly: Diagnosis, treatment, and future directions. Childs Nerv Syst 33, 1113–1123 (2017). https://doi.org/10.1007/s00381-017-3441-y

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