Segmental spinal dysgenesis: insights from three consecutive cases and a review of the literature

Authors

Ross-Jordon S. Elliott, Department of Neurological Surgery, George Washington University Hospital, Washington, DC, USA.
Jayanidhi Kedda, Department of Neurological Surgery, George Washington University Hospital, Washington, DC, USA.
Tiffany N. Phan, Division of Neurosurgery, Children's National Hospital, 111 Michigan Avenue NW, Washington, DC, 20010, USA.
Matthew E. Oetgen, Department of Orthopaedic Surgery, Children's National Hospital, Washington, DC, USA.
Chima O. Oluigbo, Division of Neurosurgery, Children's National Hospital, 111 Michigan Avenue NW, Washington, DC, 20010, USA. COluigbo@cnmc.org.

Document Type

Journal Article

Publication Date

9-1-2023

Journal

Spine deformity

Volume

11

Issue

5

DOI

10.1007/s43390-023-00684-z

Keywords

Congenital spinal abnormality; Segmental spinal dysgenesis; Spinal cord; Surgery

Abstract

OBJECTIVE: Segmental spinal dysgenesis (SSD) is a rare congenital spinal abnormality affecting the thoracic and lumbar region of the spine, as well as the spinal cord of neonates and infants. The purpose of the study was to analyze our institution's surgical case series to provide insights into our best practices to contribute to SSD management principles, while conducting a comprehensive literature review. METHODS: Following institutional review board approval, a retrospective review of SSD surgical cases was examined to observe clinical findings, radiographic findings, management, surgical intervention, and outcomes. Keywords in the comprehensive literature review included SSD, congenital spinal dysgenesis, congenital spinal stenosis, spinal aplasia, and surgery. RESULTS: Three cases underwent successful surgical management with either improvement or maintenance of neurological baseline. Patients were diagnosed at an average age of 2.7 months, while surgical intervention averaged at 40.3 months with fecal incontinence, neurogenic bladders, spinal cord compression, clubfoot, and concerns for worsening spinal deformity as surgical indicators. The average time for follow-up was 33.7 months and no complications were reported. CONCLUSIONS: Operative management for SSD is a clinically complex decision that requires multidisciplinary input and care. Patients should be observed at neurological baseline and receive intervention at the appropriate time to allow sufficient growth for functioning without permitting drastic disease progression. Consideration of patient size and spinal instrumentation are significant towards surgical success.

APA Citation

Elliott, Ross-Jordon S.; Kedda, Jayanidhi; Phan, Tiffany N.; Oetgen, Matthew E.; and Oluigbo, Chima O., "Segmental spinal dysgenesis: insights from three consecutive cases and a review of the literature" (2023). GW Authored Works. Paper 3496.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/3496

Department

Neurological Surgery