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  4. Spine duplication or split notochord syndrome – case report and literature review

Spine duplication or split notochord syndrome – case report and literature review

The Journal of Spinal Cord Medicine, 2020 · DOI: 10.1080/10790268.2018.1547531 · Published: January 1, 2020

PediatricsSpinal DisordersResearch Methodology & Design

Simple Explanation

Spine duplication, also known as split notochord syndrome, is a rare condition involving abnormalities in the spine, sometimes accompanied by other defects. This study presents the case of a boy with this syndrome, followed from birth to adulthood, describing his medical journey and outcomes. The patient had multiple congenital defects, including abdominal wall and cloacal issues, along with spine abnormalities. He underwent several surgeries and rehabilitation to address these issues, with a focus on enabling him to walk. Despite the severity of his condition, the patient was able to walk independently with a prosthesis by the age of 18. This case highlights that even with significant spine duplication and associated issues, a reasonable quality of life and mobility are possible.

Study Duration
Followed from birth to 18 years
Participants
One male infant
Evidence Level
Level 4: Case Report and Literature Review

Key Findings

  • 1
    The patient presented with defects of the abdominal wall and cloacal exstrophy, a transposed anus, and split bony elements of the spine.
  • 2
    Imaging revealed split vertebrae starting at L2, with two vertebrae and spinal canals at each level downward from L3. The spinal cord divided into two 'semicords' at L1.
  • 3
    Despite long duplication and double sacrum, walking was a feasible option with prosthesis.

Research Summary

This case report describes a unique instance of split notochord syndrome in a male, followed from birth to 18 years, detailing various congenital defects and surgical interventions. The patient presented with a complex array of anomalies, including spine duplication, gastrointestinal, and urogenital defects, necessitating multiple surgeries and extensive rehabilitation. Despite the severity of the condition, the patient achieved independent walking with a prosthesis, underscoring the potential for a reasonable quality of life even with extensive spine duplication.

Practical Implications

Prenatal Screening

If spine duplication is suspected prenatally, referral to a tertiary hospital is crucial to assess for potentially life-threatening visceral anomalies.

Early Intervention

Early surgical intervention and comprehensive rehabilitation are essential for maximizing the patient's potential for mobility and quality of life.

Prognosis Indicators

The extent of visceral anomalies and the nature of sacral bone involvement may serve as indicators for predicting neurological outcomes and walking ability.

Study Limitations

  • 1
    Single case report limits generalizability.
  • 2
    Difficulty drawing conclusions due to the variety of spine duplication types.
  • 3
    Further research needed with data collected from published case reports.

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