European Spine Journal

, Volume 27, Supplement 3, pp 533–537 | Cite as

The etiology of congenital scoliosis: genetic vs. environmental—a report of three monozygotic twin cases

  • Woojin Cho
  • Nicholas Shepard
  • Vincent Arlet
Case Report



To describe the presence of congenital scoliosis in a genetically identical population as it relates to the possible genetic vs. environmental etiologic factors.


The authors describe three cases of congenital scoliosis in monozygotic twins. The first pair includes two 4-year-old girls presenting with mirror curves, one of whom had an associated stage I Chiari malformation. The second pair is a 4-year-old girl who presented with thoracic scoliosis, a T10–11 hemivertebra, and multilevel failure of segmentation in the lumbar spine whose identical sibling is unaffected. The third pair includes a 4-month-old boy with T9 and L4 hemivertebra whose brother is also unaffected.


All three cases were managed conservatively with observation and remained asymptomatic throughout the duration of follow-up. There were no associations with extraspinal deformities, although one patient presented with concomitant type I Chiari malformation.


The variable presentation of congenital scoliosis in a genetically unique population serves as testament to the complexity associated with its development, likely involving both environmental factors and a genetic predisposition.


Congenital scoliosis Monozygotic twins Chiari malformation Hemivertebra 


Compliance with ethical standards

Conflict of interest

None of the authors has any potential conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Orthopaedic SurgeryAlbert Einstein College of Medicine/Montefiore Medical CenterBronxUSA
  2. 2.NYU Langone Orthopedic HospitalNew YorkUSA
  3. 3.Department of Orthopaedic SurgeryPennsylvania HospitalPhiladelphiaUSA

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