Lumbopelvic Stabilization Outcome in Renshaw Type IV Sacral Agenesis: A Case Report and Review of the Literature

Siamak Shabani; Maziar Malekzadeh; Masoud Moazzami; Mohammad Zarei

doi:10.18502/jost.v11i3.19409

Siamak Shabani Spine Fellowship, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Maziar Malekzadeh Spine Fellowship, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Masoud Moazzami Spine Fellowship, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Mohammad Zarei Associate Professor, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/jost.v11i3.19409

Keywords: Spinal Fusion; Sacral Agenesis; Caudal Regression Syndrome; Spinopelvic Fixation

Abstract

Background: Caudal regression syndrome (CRS), also known as sacral agenesis (SA), is a rare congenital disorder characterized by malformations of the caudal spine, lower limbs, urogenital, and anorectal systems. While the exact etiology remains unknown, a strong association with maternal diabetes mellitus (DM) has been observed.

Case Report: This retrospective study includes two patients diagnosed with Renshaw type IV CRS who underwent spinopelvic fusion. Both patients presented with back pain, severe thoracolumbar (TL) kyphotic deformity, and difficulty in sitting. Postoperative outcomes demonstrated improved sitting ability, enhanced mobilization, increased self-esteem, and better overall quality of life.

Conclusion: Orthopedic, neurological, and visceral anomalies are prevalent in patients with SA. Spinopelvic instability in type IV CRS significantly impairs sitting and mobilization. Surgical fusion can facilitate sitting and improve functional outcomes and cosmesis.