Skip to main content
SpringerLink
Log in

Prenatal MRI evaluation of limb–body wall complex

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

Abstract

Background

The sonographic (US) features of limb–body wall complex have been well documented; however the literature regarding the findings on MRI in limb–body wall complex is scant.

Objective

To characterize the prenatal MRI features of limb–body wall complex.

Materials and methods

We performed a retrospective review of all MRI scans of fetuses diagnosed with limb–body wall complex at our institution from 2001 to 2011. Fetuses without correlating US scans or follow-up information were excluded. Three pediatric radiologists blinded to the specific US findings reviewed the prenatal MRIs. Images were evaluated for the organ location and attachment, the body part affected, characterization of the body wall defect, and spinal, limb and umbilical cord abnormalities.

Results

Ten subjects met inclusion criteria. MRI was able to detect and characterize the body part affected and associated abnormalities. All fetuses had ventral wall defects, a small thorax and herniated liver and bowel. The kidneys were extracorporeal in three cases. The extruded organs were attached to the placenta or the uterine wall in all cases. Abnormal spinal curvatures of various degrees of severity were present in all cases. Eight cases had a short, uncoiled cord. Limb anomalies were present in 6 of the 10 cases.

Conclusion

We illustrate the common fetal MRI findings of limb–body wall complex. The prenatal diagnosis of limb–body wall complex and the differentiation of this defect from treatable abdominal wall defects are crucial to providing appropriate guidance for patient counseling and management.

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

Similar content being viewed by others

References

  1. Higginbottom MC, Jones KL, Hall BD et al (1979) The amniotic band disruption complex: timing of amniotic rupture and variable spectra of consequent defects. J Pediatr 95:544–549

    Article  PubMed  CAS  Google Scholar 

  2. Kalousek DK, Bamforth S (1988) Amnion rupture sequence in previable fetuses. Am J Med Genet 31:63–73

    Article  PubMed  CAS  Google Scholar 

  3. Smrcek JM, Germer U, Krokowski M et al (2003) Prenatal ultrasound diagnosis and management of body stalk anomaly: analysis of nine singleton and two multiple pregnancies. Ultrasound Obstet Gynecol 21:322–328

    Article  PubMed  CAS  Google Scholar 

  4. Hunter AG, Seaver LH, Stevenson RE (2011) Limb–body wall defect. Is there a defensible hypothesis and can it explain all the associated anomalies? Am J Med Genet A 155:2045–2059

    Article  Google Scholar 

  5. Van Allen MI, Curry C, Gallagher L (1987) Limb body wall complex: I. Pathogenesis. Am J Med Genet 28:529–548

    Article  PubMed  Google Scholar 

  6. Daskalakis G, Sebire NJ, Jurkovic D et al (1997) Body stalk anomaly at 10–14 weeks of gestation. Ultrasound Obstet Gynecol 10:416–418

    Article  PubMed  CAS  Google Scholar 

  7. Chen CP, Lin CJ, Chang TY et al (2007) Second-trimester diagnosis of limb–body wall complex with literature review of pathogenesis. Genet Couns 18:105–112

    PubMed  CAS  Google Scholar 

  8. Goldstein I, Winn HN, Hobbins JC (1989) Prenatal diagnostic criteria for body stalk anomaly. Am J Perinatol 6:84–85

    Article  PubMed  CAS  Google Scholar 

  9. Shalev E, Eliyahu S, Battino S et al (1995) First trimester transvaginal sonographic diagnosis of body stalk anomaly (correction of anatomy). J Ultrasound Med 14:641–642

    PubMed  CAS  Google Scholar 

  10. Costa ML, Couto E, Furlan E et al (2012) Body stalk anomaly: adverse maternal outcomes in a series of 21 cases. Prenat Diagn 32:264–267

    Article  PubMed  Google Scholar 

  11. Mann L, Ferguson-Smith MA, Desai M et al (1984) Prenatal assessment of anterior abdominal wall defects and their prognosis. Prenat Diagn 4:427–435

    Article  PubMed  CAS  Google Scholar 

  12. Russo R, Vecchione R (1996) Limb body wall complex: craniofacial defects as a distinctive factor. Birth Defects Orig Artic Ser 30:157–164

    PubMed  CAS  Google Scholar 

  13. Negishi H, Yaegashi M, Kato EH et al (1998) Prenatal diagnosis of limb–body wall complex. J Reprod Med 43:659–664

    PubMed  CAS  Google Scholar 

  14. Murphy A, Platt LD (2011) First-trimester diagnosis of body stalk anomaly using 2- and 3-dimensional sonography. J Ultrasound Med 30:1739–1743

    PubMed  Google Scholar 

  15. Luehr B, Lipsett J, Quinlivan JA (2002) Limb–body wall complex: a case series. J Matern Fetal Neonatal Med 12:132–137

    PubMed  CAS  Google Scholar 

  16. Levy R, Lacombe D, Rougier Y et al (2007) Limb body wall complex and amniotic band sequence in sibs. Am J Med Genet A 143:2682–2687

    Article  Google Scholar 

  17. Colpaert C, Bogers J, Hertveldt K et al (2000) Limb–body wall complex: 4 new cases illustrating the importance of examining placenta and umbilical cord. Pathol Res Pract 196:783–790

    Article  PubMed  CAS  Google Scholar 

  18. Luebke HJ, Reiser CA, Pauli RM (1990) Fetal disruptions: assessment of frequency, heterogeneity, and embryologic mechanisms in a population referred to a community-based stillbirth assessment program. Am J Med Genet 36:56–72

    Article  PubMed  CAS  Google Scholar 

  19. Pumberger W, Schaller A, Bernaschek G (2001) Limb–body wall complex: a compound anomaly pattern in body-wall defects. Pediatr Surg Int 17:486–490

    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

    Article  PubMed  CAS  Google Scholar 

  21. Mathai AM, Menezes RG, Kumar S et al (2009) A fetal autopsy case of body stalk anomaly. Leg Med 11:241–244

    Article  Google Scholar 

  22. Sahinoglu Z, Uludogan M, Arik H et al (2007) Prenatal ultrasonographical features of limb body wall complex: a review of etiopathogenesis and a new classification. Fetal Pediatr Pathol 26:135–151

    Article  PubMed  Google Scholar 

  23. Tang TT, Oechler HW, Hinke DH et al (1991) Limb body–wall complex in association with sirenomelia sequence. Am J Med Genet 41:21–25

    Article  PubMed  CAS  Google Scholar 

  24. Van Allen MI, Curry C, Walden CE et al (1987) Limb–body wall complex: II. Limb and spine defects. Am J Med Genet 28:549–565

    Article  PubMed  Google Scholar 

  25. Fukumasu H, Imanaka M, Matsumoto M et al (1993) Significance of fetography in prenatal diagnosis of limb–body wall complex (LBWC). Int J Gynaecol Obstet 43:317–321

    Article  PubMed  CAS  Google Scholar 

  26. Chikkannaiah P, Dhumale H, Kangle R et al (2013) Limb body wall complex: a rare anomaly. J Lab Physicians 5:65–67

    Article  PubMed  PubMed Central  Google Scholar 

  27. Chen C-P, Cheng S-J, Lin Y-H et al (2005) Prenatal imaging of limb–body wall complex by magnetic resonance imaging. Prenat Diagn 25:521–523

    Article  PubMed  CAS  Google Scholar 

  28. De Silva MV, Senanayake H, Siriwardana KD (2001) Body stalk anomaly. Ceylon Med J 46:68

    PubMed  Google Scholar 

  29. Kanamori Y, Hashizume K, Sugiyama M et al (2007) Long-term survival of a baby with body stalk anomaly: report of a case. Surg Today 37:30–33

    Article  PubMed  Google Scholar 

  30. Morrow RJ, Whittle MJ, McNay MB et al (1993) Prenatal diagnosis and management of anterior abdominal wall defects in the west of Scotland. Prenat Diagn 13:111–115

    Article  PubMed  CAS  Google Scholar 

  31. Forrester MB, Merz RD (1999) Epidemiology of abdominal wall defects, Hawaii, 1986–1997. Teratology 60:117–123

    Article  PubMed  CAS  Google Scholar 

  32. Torpin R (1965) Amniochorionic mesoblastic fibrous strings and amniotic bands: associated constricting fetal malformations or fetal death. Am J Obstet Gynecol 91:65–75

    PubMed  CAS  Google Scholar 

  33. Herva R, Karkinen-Jääskeläinen M (1984) Amniotic adhesion malformation syndrome: fetal and placental pathology. Teratology 29:11–19

    Article  PubMed  CAS  Google Scholar 

  34. Duhamel B (1963) Embryology of exomphalos and allied malformations. Arch Dis Child 38:142–147

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  35. Hartwig NG, Vermeij-Keers C, De Vries HE et al (1989) Limb body wall malformation complex: an embryologic etiology? Hum Pathol 20:1071–1077

    Article  PubMed  CAS  Google Scholar 

  36. Deruelle P, Hay R, Subtil D et al (2000) [Antenatal diagnosis of limb body wall complex]. J Gynecol Obstet Biol Reprod 29:385–391

    CAS  Google Scholar 

  37. Lockwood CJ, Scioscia AL, Hobbins JC (1986) Congenital absence of the umbilical cord resulting from maldevelopment of embryonic body folding. Am J Obstet Gynecol 155:1049–1051

    Article  PubMed  CAS  Google Scholar 

  38. Kalish RB, Hunter T, Sharma G et al (2003) Clinical significance of the umbilical cord twist. Am J Obstet Gynecol 189:736–739

    Article  PubMed  Google Scholar 

  39. Ente G, Penzer PH (1991) The umbilical cord: normal parameters. J R Soc Health 111:138–140

    Article  PubMed  CAS  Google Scholar 

  40. Naeye RL (1985) Umbilical cord length: clinical significance. J Pediatr 107:278–281

    Article  PubMed  CAS  Google Scholar 

  41. Miller ME, Higginbottom M, Smith DW (1981) Short umbilical cord: its origin and relevance. Pediatrics 67:618–621

    PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This work received the Caffey Award for Best Scientific Exhibit, Society for Pediatric Radiology, 55th Annual Meeting & Postgraduate Course 2012, San Francisco, CA.

Conflicts of interest

None

Author information

Authors and Affiliations

  1. Department of Radiology, The Children’s Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Elisa Aguirre-Pascual, Monica Epelman, Ann M. Johnson, Nancy A. Chauvin, Beverly G. Coleman & Teresa Victoria

  2. Department of Radiology, Hospital Universitario Doce de Octubre, Madrid, Spain

    Elisa Aguirre-Pascual

  3. Department of Medical Imaging, Nemours Children’s Hospital, 13535 Nemours Parkway, Orlando, 32827, FL, USA

    Monica Epelman

Authors
  1. Elisa Aguirre-Pascual
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Monica Epelman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Ann M. Johnson
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Nancy A. Chauvin
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Beverly G. Coleman
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Teresa Victoria
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Monica Epelman.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Aguirre-Pascual, E., Epelman, M., Johnson, A.M. et al. Prenatal MRI evaluation of limb–body wall complex. Pediatr Radiol 44, 1412–1420 (2014). https://doi.org/10.1007/s00247-014-3026-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-014-3026-9

Keywords

Search

Navigation