Skip to main content
SpringerLink
Log in

Fetal MRI of cloacal exstrophy

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Background

Prenatal ultrasonographic (US) diagnosis of cloacal exstrophy (CE) is challenging.

Objective

To define the fetal MRI findings in CE.

Materials and methods

We performed a retrospective review of eight patients with CE. Imaging was performed between 22 weeks and 36 weeks of gestation with US in four and MRI in eight fetuses. Abdominal wall, gastrointestinal/genitourinary, and spine and limb abnormalities detected were compared with postnatal evaluation.

Results

US failed to display CE in one of the four fetuses. Fetal MRI confirmed CE in all eight fetuses by demonstrating absence of a normal bladder and lack of meconium-filled rectum/colon, associated with protuberant pelvic contour and omphalocele. These findings correlated postnatally with CE, atretic hindgut and omphalocele. One fetus had imaging before rupture of the cloacal membrane, showing a protruding pelvic cyst. Absent bladder was noted in the remaining seven fetuses. Confirmed skin-covered spinal defects were noted in seven fetuses, low conus/tethered cord in one and clubfoot in three. Six fetuses had renal anomalies, two had hydrocolpos and one had ambiguous genitalia.

Conclusion

Fetal MRI provides a confident diagnosis of CE when a normal bladder is not identified, there is a protuberant abdominopelvic contour and there is absence of meconium-filled rectum and colon. Genitourinary and spinal malformations are common associations.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11

Similar content being viewed by others

References

  1. Hendren WH (1996) Urogenital sinus and cloacal malformations. Semin Pediatr Surg 5:72–79

    PubMed  CAS  Google Scholar 

  2. Carey JC, Greenbaum B, Hall BD (1978) The OEIS complex (omphalocele, exstrophy, imperforate anus, spinal defects). Birth Defects Orig Artic Ser 14:253–263

    PubMed  CAS  Google Scholar 

  3. Martinez-Frias ML, Bermejo E, Rodriguez-Pinilla E et al (2001) Exstrophy of the cloaca and exstrophy of the bladder: two different expressions of a primary developmental field defect. Am J Med Genet 99:261–269

    Article  PubMed  CAS  Google Scholar 

  4. Ebert AK, Reutter H, Ludwig M et al (2009) The exstrophy-epispadias complex. Orphanet J Rare Dis 4:23

    Article  PubMed  Google Scholar 

  5. Bohring A (2002) OEIS complex, VATER, and the ongoing difficulties in terminology and delineation. Am J Med Genet 107:72–76

    Article  PubMed  Google Scholar 

  6. Carey JC (2001) Exstrophy of the cloaca and the OEIS complex: one and the same. Am J Med Genet 99:270

    Article  PubMed  CAS  Google Scholar 

  7. Austin PF, Homsy YL, Gearhart JP et al (1998) The prenatal diagnosis of cloacal exstrophy. J Urol 160:1179–1181

    Article  PubMed  CAS  Google Scholar 

  8. Wu JL, Fang KH, Yeh GP et al (2004) Using color Doppler sonography to identify the perivesical umbilical arteries: a useful method in the prenatal diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex. J Ultrasound Med 23:1211–1215

    PubMed  Google Scholar 

  9. Gobbi D, Fascetti Leon F, Tregnaghi A et al (2008) Early prenatal diagnosis of cloacal exstrophy with fetal magnetic resonance imaging. Fetal Diagn Ther 24:437–439

    Article  PubMed  CAS  Google Scholar 

  10. Caire JT, Ramus RM, Magee KP et al (2003) MRI of fetal genitourinary anomalies. AJR Am J Roentgenol 181:1381–1385

    Article  PubMed  Google Scholar 

  11. Chauvin NA, Epelman M, Victoria T et al (2012) Complex genitourinary abnormalities on fetal MRI: imaging findings and approach to diagnosis. AJR Am J Roentgenol 199:W222–W231

    Article  PubMed  Google Scholar 

  12. Chen CP, Chang TY, Liu YP et al (2008) Prenatal 3-dimensional sonographic and MRI findings in omphalocele-exstrophy-imperforate anus-spinal defects complex. J Clin Ultrasound 36:308–311

    Article  PubMed  Google Scholar 

  13. Chen CP (2007) Syndromes and disorders associated with omphalocele (II): OEIS complex and Pentalogy of Cantrell. Taiwan J Obstet Gynecol 46:103–110

    Article  PubMed  CAS  Google Scholar 

  14. Phillips TM (2011) Spectrum of cloacal exstrophy. Semin Pediatr Surg 20:113–118

    Article  PubMed  Google Scholar 

  15. Pinette MG, Pan YQ, Pinette SG et al (1996) Prenatal diagnosis of fetal bladder and cloacal exstrophy by ultrasound. A report of three cases. J Reprod Med 41:132–134

    PubMed  CAS  Google Scholar 

  16. Gearhart JP, Ben-Chaim J, Jeffs RD et al (1995) Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 85:961–964

    Article  PubMed  CAS  Google Scholar 

  17. Yiee J, Wilcox D (2008) Abnormalities of the fetal bladder. Semin Fetal Neonatal Med 13:164–170

    Article  PubMed  Google Scholar 

  18. Wilcox DT, Chitty LS (2001) Non-visualisations of the fetal bladder: aetiology and management. Prenat Diagn 21:977–983

    Article  PubMed  CAS  Google Scholar 

  19. Langer JC, Brennan B, Lappalainen RE et al (1992) Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane. J Pediatr Surg 27:1352–1355

    Article  PubMed  CAS  Google Scholar 

  20. Emanuel PG, Garcia GI, Angtuaco TL (1995) Prenatal detection of anterior abdominal wall defects with US. Radiographics 15:517–530

    PubMed  CAS  Google Scholar 

  21. Chen CP (2008) Syndromes, disorders and maternal risk factors associated with neural tube defects (III). Taiwan J Obstet Gynecol 47:131–140

    Article  PubMed  Google Scholar 

  22. Carr MC, Benacerraf BR, Mandell J (1994) Prenatal diagnosis of an XY fetus with aphallia and cloacal exstrophy variant. J Ultrasound Med 13:323–325

    PubMed  CAS  Google Scholar 

  23. Soffer SZ, Rosen NG, Hong AR et al (2000) Cloacal exstrophy: a unified management plan. J Pediatr Surg 35:932–937

    Article  PubMed  CAS  Google Scholar 

  24. Marvin T (2007) Cloacal exstrophy: a case study. Neonatal Netw 26:21–30

    Article  PubMed  Google Scholar 

  25. Tiblad E, Wilson RD, Carr M et al (2008) OEIS sequence – a rare congenital anomaly with prenatal evaluation and postnatal outcome in six cases. Prenat Diagn 28:141–147

    Article  PubMed  Google Scholar 

  26. Hamada H, Takano K, Shiina H et al (1999) New ultrasonographic criterion for the prenatal diagnosis of cloacal exstrophy: elephant trunk-like image. J Urol 162:2123–2124

    Article  PubMed  CAS  Google Scholar 

  27. Richards DS, Langham MR Jr, Mahaffey SM (1992) The prenatal ultrasonographic diagnosis of cloacal exstrophy. J Ultrasound Med 11:507–510

    PubMed  CAS  Google Scholar 

  28. Keppler-Noreuil K, Gorton S, Foo F et al (2007) Prenatal ascertainment of OEIS complex/cloacal exstrophy – 15 new cases and literature review. Am J Med Genet A 143A:2122–2128

    Article  PubMed  Google Scholar 

  29. Kutzner DK, Wilson WG, Hogge WA (1988) OEIS complex (cloacal exstrophy): prenatal diagnosis in the second trimester. Prenat Diagn 8:247–253

    Article  PubMed  CAS  Google Scholar 

  30. Hyun SJ (2006) Cloacal exstrophy. Neonatal Netw 25:101–115

    Article  PubMed  Google Scholar 

  31. Saguintaah M, Couture A, Veyrac C et al (2002) MRI of the fetal gastrointestinal tract. Pediatr Radiol 32:395–404

    Article  PubMed  Google Scholar 

  32. Brugger PC, Prayer D (2006) Fetal abdominal magnetic resonance imaging. Eur J Radiol 57:278–293

    Article  PubMed  Google Scholar 

  33. Hsieh K, O’Loughlin MT, Ferrer FA (2005) Bladder exstrophy and phenotypic gender determination on fetal magnetic resonance imaging. Urology 65:998–999

    Article  PubMed  Google Scholar 

  34. Calvo-Garcia MA, Kline-Fath BM, Levitt MA et al (2011) Fetal MRI clues to diagnose cloacal malformations. Pediatr Radiol 41:1117–1128

    Article  PubMed  Google Scholar 

Download references

Conflicts of interest

None

Author information

Authors and Affiliations

  1. Department of Radiology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229-3039, USA

    Maria A. Calvo-Garcia, Beth M. Kline-Fath & Arnold C. Merrow

  2. Department of Radiology, Children’s National Medical Center, Washington, DC, USA

    Eva I. Rubio

  3. Department of Radiology, Nemours Children’s Hospital, Orlando, FL, USA

    Carolina V. Guimaraes

  4. Pediatric Surgery and Fetal Care Center of Cincinnati, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Foong-Yen Lim

Authors
  1. Maria A. Calvo-Garcia
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Beth M. Kline-Fath
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Eva I. Rubio
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Arnold C. Merrow
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Carolina V. Guimaraes
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Foong-Yen Lim
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maria A. Calvo-Garcia.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Calvo-Garcia, M.A., Kline-Fath, B.M., Rubio, E.I. et al. Fetal MRI of cloacal exstrophy. Pediatr Radiol 43, 593–604 (2013). https://doi.org/10.1007/s00247-012-2571-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-012-2571-3

Keywords

Search

Navigation