Surgical and Radiologic Anatomy

, Volume 35, Issue 8, pp 749–754 | Cite as

Horseshoe adrenal gland: a rare congenital anomaly

  • Noah DitkofskyEmail author
  • Nori Bradley
  • Paul Jeon
Anatomic Variations


A solitary adrenal gland spanning the midline has been alternatively described as “Horseshoe” or “Butterfly” and is a rare congenital abnormality almost exclusively associated with neonates. There have been 65 reported cases in fetuses and infants, based on ultrasound and autopsy findings. Horseshoe adrenal gland is associated with several congenital anomalies, such as asplenia (52 %), neural tube defects (37 %), renal abnormalities (29 %), and diaphragmatic defects (1 %). Recently, Feldman and colleagues [2] reported the first case of an adult male with horseshoe adrenal gland and posterior midline diaphragmatic anomaly found incidentally with CT imaging. We present a second case of asymptomatic adult horseshoe adrenal gland with posterior midline diaphragmatic hernia in a 51-year-old woman discovered on CT imaging. In our patient these abnormalities were also associated with a 9th thoracic butterfly vertebrae and a unilateral region of underdevelopment of the paraspinal musculature at the level of the diaphragmatic defect. To our knowledge, this is the first time this abnormality has been documented with MRI.


Horseshoe adrenal gland Midline adrenal gland Butterfly adrenal gland Fused adrenal gland Midline anomaly Diaphragmatic defect 


Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    De Graaf R-J, Matricali B, Hamburger HL (1982) Butterfly vertebra. Clin Neurol Neurosurg 84(3):163–169PubMedCrossRefGoogle Scholar
  2. 2.
    Feldmann EJ, Berko NS, Jain VR, Haramati LB (2009) A man with horseshoe adrenal gland and associated midline posterior diaphragmatic deficit. J Comput Assist Tomogr 33:717–720PubMedCrossRefGoogle Scholar
  3. 3.
    Gilbert-Barness E, Debich-Spicer D, Cohen MM et al (2001) Evidence for the “midline” hypothesis in associated defects in laterality formation and multiple midline anomalies. Am J Med Gen 101:382–387CrossRefGoogle Scholar
  4. 4.
    Gosemann J-H, Doi T, Kutasy B et al (2012) Pax3 gene expression is not altered during diaphragmatic development in nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg 47:1067–1071PubMedCrossRefGoogle Scholar
  5. 5.
    Kardong K (2011) Vertebrates: comparative anatomy, function, evolution, 6th edn. McGraw-Hill Publishing, USAGoogle Scholar
  6. 6.
    Klatt EC, Pysher TJ, Pavlova Z (1998) Adrenal fusion. Pediatr Dev Pathol 1:475–479PubMedCrossRefGoogle Scholar
  7. 7.
    Lang D, Powell SK, Plummer RS et al (2007) PAX genes: roles in development, pathophysiology, and cancer. Biochem Pharm 73:1–14PubMedCrossRefGoogle Scholar
  8. 8.
    Laufer E, Kesper D, Vortkamp A et al (2012) Sonic hedgehog signaling during adrenal development. Mol Cell Endocr 351:19–27CrossRefGoogle Scholar
  9. 9.
    Moore KL (1977) The developing human: clinically oriented embryology, 2nd edn. W.B Saunders, USAGoogle Scholar
  10. 10.
    Nelms BL, Labosky PA (2010) Transcriptional control of neural crest development. Morgan & Claypool Life Sciences, USAGoogle Scholar
  11. 11.
    Skandalakis JE, Colborn GL, Weidman TA et al (2004) Adrenal (suprarenal) glands. In: Skandalakis JE, Colburn GL, Weidman TA, Foster RS Jr, Kingsworth AN, Skandalakis LJ, Skandalakis PN, Mirilas PS (eds) Skandalakis’ surgical anatomythe embryologic and anatomic basis of modern surgery. McGraw-Hill Professional Publishing, USAGoogle Scholar
  12. 12.
    Strouse PJ, Haller JO, Berden WE et al (2002) Horseshoe adrenal gland in association with asplenia: presentation of six new cases and review of the literature. Pediatr Radiol 32:778–782PubMedCrossRefGoogle Scholar
  13. 13.
    Wehr R, Gruss P (1996) Pax and vertebrate development. Int J Dev Biol 40:369–377PubMedGoogle Scholar
  14. 14.
    Williamson KA, Hever AM, Rainger J et al (2006) Mutations in SOX2 cause anopthalmia-esophageal-genital (AEG) syndrome. Hum Mol Gen 15:1413–1422PubMedCrossRefGoogle Scholar
  15. 15.
    Zhou H-M, Wang J, Rogers R et al (2008) Lineage-specific responses to reduced embryonic Pax3 expression levels. Dev Biol 315:369–382PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag France 2013

Authors and Affiliations

  1. 1.Department of Diagnostic Imaging Health Sciences CenterMemorial University of NewfoundlandSt. John’sCanada
  2. 2.Division of General Surgery, Vancouver General HospitalUniversity of British ColumbiaVancouverCanada

Personalised recommendations