Lumbopelvic stabilization with external fixator in a patient with lumbosacral agenesis

Abstract

A case of caudal regression syndrome in which rehabilitation was obtained by lumbopelvic distraction and stabilization with external fixation Orthofix Ò is presented. The objective of the study is to describe the benefit of spine external fixator in caudal regression syndrome. Caudal regression syndrome is a rare and sporadic neural defect of distal spinal segments affecting the development of the spinal cord. It is characterized by vertebropelvic instability and essentially manifests as neurological deficit in the lower limbs and absence of bladder and bowel control. Pluridisciplinary management of this affection is complex. The clinical presentation and treatment of caudal regression syndromes are reviewed. A case of a boy for whom physical rehabilitation was obtained with external fixation Orthofix Ò is presented. Lumbopelvic stabilization was performed with autograft and allograft bone. A spine distraction was performed with external fixation with pedicular screws in L1 and L2 and in the pelvis bone. An elongation of 19 mm was obtained in 35 days. The external fixator was removed after 4 months and a spine cast was applied during 8 months. This treatment resulted in lumbopelvic nonunion of very low mobility. Lumbopelvic stability and the disappearance of pain were achieved. Sitting position was stable without hand support. At 14 years of follow-up, his condition is stable with possible deambulation at home with crutches. Lumbopelvic arthrodesis in caudal regression syndrome is difficult to obtain. The use of spine external fixator, however, allows trunk elongation and can achieve a stable sitting and upright position.

Sacral agenesis combined with spinopelvic dissociation

“…According to the limited number of studies on spinopelvic stability, we found that bone nonunion had a relatively high incidence. [ 5 , 6 , 8 ] Yazici et al reported 3 cases with lumbosacral agenesis that underwent posterior lumbopelvic instrumentation and fixation with autogenic anterior tibial cortical structural graft for laminopelvic bridging and demineralized bone matrix for augmentation of osteoinduction. [ 7 ] Successful fusion was achieved for all 3 patients, which allowed for sitting.…”

Urine retention as the first presentation of congenital absence of the sacrum

“…[ 16 , 17 ] The main treatment for sacral agenesis is correction of deformities and orthopaedic intervention. [ 14 , 18 , 19 ] Moreover for neurogenic bladder, the current treatment methods include medications, neuromodulation, botulinum toxin A injection, surgical procedures, issue engineering, stem cell transplantation, and gene therapy, besides, Traditional Chinese Medicine such as acupuncture. A review demonstrated that acupuncture could have a beneficial effect on neurological function recovery.…”

Atypical caudal regression syndrome with agenesis of lumbar spine and presence of sacrum – case report and literature review

“…9,13 Only few of them consider quality of life of these patients underlining the importance of stable sitting position without hand support. 5 To achieve this goal, knee and hip joint contractures may require (or not) surgical intervention, depending on the patient. 15 Spino-pelvic instability is another problem causing declined torso position.…”

“…4 Currently, absence of a sacrum and partial absence of one or more vertebrae cephalad is classified as caudal regression syndrome. 5 Despite great variety of defects in this syndrome, spinal malformations are always accompanied by anomalies of the viscera, especially of urogenital system. 4,5 It is often associated with neurogenic bladder, which may lead to increased risk of renal impairment caused by recurring urinary tract infections and urinary retention.…”

“…5 Despite great variety of defects in this syndrome, spinal malformations are always accompanied by anomalies of the viscera, especially of urogenital system. 4,5 It is often associated with neurogenic bladder, which may lead to increased risk of renal impairment caused by recurring urinary tract infections and urinary retention. 6,7 Neurological deficits of various severity are often present.…”