Purpose: Patients with lumbosacral agenesis characteristically sit on their iliac wings with their torsos bent forward, which yields an increase in intra-abdominal pressure and, subsequently, negative effects on their diaphragm. The sacrum is not available as an anchor point for instrumentation. Dunn-McCarthy rods or Galveston fixations cannot be performed due to these limited anatomic properties. On the other hand, the absence of necessary bone mass for fusion anteriorly limits the fusion interventions to the posterior. Therefore, a secure and rigid fixation is essential to preclude the need for an external support. There are limited publications discussing different techniques due to the relatively rare incidence of the disease. We report the clinical and radiological results of a new technique applied to three patients in which previously recommended methods are modified.
Methods: Two 6-year-old female patients and one 5-year-old male patient with lumbosacral agenesis underwent posterior lumbopelvic instrumentation and fusion. Together with standard pedicle screw spinal instrumentation, pelvic fixation is obtained with a combination of supero-inferior directed rod and/or screw to overcome deforming forces created at the flexion-extension pivot points of the lumbopelvic junction. Autogenic anterior tibial cortical structural graft is used for laminopelvic bridging, and demineralized bone matrix is used for the augmentation of osteoinduction. A single leg hip spica is applied for 4 months to protect the fixation.
Results: Total correction yielded an aligned spine with a posture that allows for sitting on the ischial spines for all three patients. Solid fusion was observed to maintain this correction at the final follow-up.
Conclusion: The use of new-generation pediatric spinal instrumentation systems with a new technique without knee disarticulation provides a safe and effective fixation and fusion in lumbosacral agenesis.
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http://dx.doi.org/10.1007/s11832-010-0322-2 | DOI Listing |
Pediatr Neurol
August 2024
Department of Radiology, National Institute of Child Health, Karachi, Pakistan. Electronic address:
J Pediatr Urol
August 2024
Department of Pediatric Surgery, Chengdu Women's and Children's Central Hospital, School of Medicine, University of Electronic Science and Technology of China, Chengdu, 611731, China. Electronic address:
Neurochirurgie
September 2024
Neurosurgery Department, University Hospital of Caen, France. Electronic address:
Introduction: Anterior sacral meningocele is a rare congenital disorder, occurring isolated or in syndromic disease.
Case Report: A 15-year-old patient who complained of abdominal pain and urinary dysfunction was managed surgically. Imaging diagnosed a giant presacral meningocele and agenesis of the coccyx.
J Pediatr Urol
June 2024
Department of Urology, Institute of Urology and Nephrology, Kuala Lumpur Hospital, Jalan Pahang, 50586 Kuala Lumpur, Malaysia. Electronic address:
Introduction: Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect CIC compliance and to determine if CIC compliance affected short-term urological outcomes among patients in Malaysia.
Study Design: 50 patients aged 2-18 years who perform CIC were included in this cohort study.
Childs Nerv Syst
June 2024
Royal Orthopaedic Hospital, Birmingham, West Midlands, UK.
Introduction: Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made perinatally, and prognostic discussions become an important aspect of parental counselling. This study engaged SA sufferers and their caregivers to obtain objective, long-term patient reported outcome data.
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