Clinical and Radiological Characterization of an Infant with Caudal Regression Syndrome Type III

Caudal regression syndrome is a rare disorder of developmental failure of lumbosacral vertebra and corresponding spinal cord during notochord formation. The severity varies from absent coccyx to complete absence of lumbosacral vertebra and caudal spinal cord. Both genetic and environmental factors are believed to play roles in aetiopathogenesis of caudal regression. The authors report a two-month-old child born to a diabetic mother, in whom the diagnosis of caudal regression syndrome type III was confirmed based on clinical and radiological characteristics. The child was managed by the multidisciplinary team to continue supportive care and screen and monitor for long-term complications. The long-term prognosis for mobility was less favourable given the presence of bilateral hip dysplasia and involvement of lumbar vertebra in addition to sacral agenesis.

Download Full-text

Long-term urologic outcome in patients with caudal regression syndrome, compared with meningomyelocele and spinal cord lipoma

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2007.10.036 ◽

2008 ◽

Vol 43 (3) ◽

pp. 530-533 ◽

Cited By ~ 21

Author(s):

Michele Torre ◽

Piero Buffa ◽

Vincenzo Jasonni ◽

Armando Cama

Keyword(s):

Spinal Cord ◽

Caudal Regression Syndrome ◽

Caudal Regression ◽

Cord Lipoma

Download Full-text

Surgical Correction of Spinopelvic Instability in Children With Caudal Regression Syndrome

Global Spine Journal ◽

10.1177/2192568218779984 ◽

2018 ◽

Vol 9 (3) ◽

pp. 260-265 ◽

Cited By ~ 2

Author(s):

Sergei Vissarionov ◽

Josh E. Schroder ◽

Dmitrii Kokushin ◽

Vladislav Murashko ◽

Sergei Belianchikov ◽

...

Keyword(s):

Fusion Rate ◽

Surgical Correction ◽

Caudal Regression Syndrome ◽

Lumbosacral Region ◽

Institutional Review ◽

Pedicular Screw ◽

Caudal Regression ◽

Short And Long Term

Study Design: Retrospective cohort. Objective: To analyze the outcome of surgical correction of children with caudal regression syndrome. Methods: The study included 12 patients aged 1.5 to 9 years with caudal regression syndrome. In order determine the type of caudal regression, the Renshaw Classification was used. The surgery included correction and stabilization of the kyphotic deformity at the unstable lumbosacral region, with reconstruction of the sagittal balance using a bony block constructed from allograft. Short- and long-term outcomes were evaluated. The study was approved by the local institutional review board. Results: Children with types III and IV caudal regression syndrome underwent spinal-pelvic fusion, with 100% fusion rate, which allows sufficient stabilization of the lumbopelvic segment permitting patient mobilization and standing in type III patients. There were 5 complications needing additional care. Conclusion: Multilevel pedicular screw fixation in combination with spinopelvic fusion with cortical allografts allows reconstruction of the sagittal alignment with solid bony fusion improving the quality of life for these patients.

Download Full-text

Tetanus Toxin in Dissociated Spinal Cord Cultures: Long-Term Characterization of Form and Action

Journal of Neurochemistry ◽

10.1111/j.1471-4159.1986.tb00700.x ◽

2006 ◽

Vol 47 (3) ◽

pp. 930-937 ◽

Cited By ~ 48

Author(s):

William H. Habig ◽

Hans Bigalke ◽

Gregory K. Bergey ◽

Elaine A. Neale ◽

M. Carolyn Hardegree ◽

...

Keyword(s):

Spinal Cord ◽

Tetanus Toxin

Download Full-text

Long-Term Characterization of Axon Regeneration and Matrix Changes Using Multiple Channel Bridges for Spinal Cord Regeneration

Tissue Engineering Part A ◽

10.1089/ten.tea.2013.0111 ◽

2014 ◽

Vol 20 (5-6) ◽

pp. 1027-1037 ◽

Cited By ~ 20

Author(s):

Hannah M. Tuinstra ◽

Daniel J. Margul ◽

Ashley G. Goodman ◽

Ryan M. Boehler ◽

Samantha J. Holland ◽

...

Keyword(s):

Spinal Cord ◽

Axon Regeneration ◽

Spinal Cord Regeneration ◽

Multiple Channel

Download Full-text

Spinal Cord Stimulation for Complex Regional Pain Syndrome Type I: A Prospective Cohort Study With Long-Term Follow-Up

Neuromodulation Technology at the Neural Interface ◽

10.1111/ner.12024 ◽

2013 ◽

Vol 16 (6) ◽

pp. 523-529 ◽

Cited By ~ 58

Author(s):

José W. Geurts ◽

Helwin Smits ◽

Marius A. Kemler ◽

Florian Brunner ◽

Alfons G. H. Kessels ◽

...

Keyword(s):

Spinal Cord ◽

Cohort Study ◽

Spinal Cord Stimulation ◽

Prospective Cohort ◽

Pain Syndrome ◽

Type I ◽

Syndrome Type ◽

Long Term Follow Up

Download Full-text

Clinico-radiologic Findings in Group II Caudal Regression Syndrome

Journal of Clinical Imaging Science ◽

10.4103/2156-7514.114214 ◽

2013 ◽

Vol 3 ◽

pp. 26 ◽

Cited By ~ 4

Author(s):

Pankaj Sharma ◽

Sheo Kumar ◽

Awdesh Jaiswal

Keyword(s):

Spinal Cord ◽

Case Report ◽

Congenital Abnormality ◽

Caudal Regression Syndrome ◽

Radiologic Findings ◽

Caudal Regression ◽

Rare Congenital Abnormality ◽

Group 2 ◽

Spinal Cord Function ◽

Sacral Spine

Caudal regression syndrome (CRS) is a rare congenital abnormality in which a segment of the lumbo-sacral spine and spinal cord fails to develop. The severity of the morphologic derangement inversely correlates with residual spinal cord function. We present a case report of a 10-year-old girl with Group 2 CRS, to emphasize clinical and radiologic findings in this rare abnormality.

Download Full-text

SURGICAL TREATMENT OF SPINAL PATHOLOGY IN CHILDREN WITH CAUDAL REGRESSION SYNDROME: ANALYSIS OF LONG-TERM OUTCOMES

Hirurgiâ pozvonočnika ◽

10.14531/ss2015.4.36-41 ◽

2015 ◽

pp. 36-41

Author(s):

Sergey Vissarionov ◽

◽

Dmitrii Kokushin ◽

Vladislav Murashko ◽

Sergey Belyanchikov ◽

...

Keyword(s):

Surgical Treatment ◽

Caudal Regression Syndrome ◽

Long Term Outcomes ◽

Caudal Regression ◽

Syndrome Analysis

Download Full-text

Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected from a Syndrome of Caudal Regression

10.20944/preprints201611.0133.v1 ◽

2016 ◽

Author(s):

Jesús Devesa ◽

Alba Alonso ◽

Natalia López ◽

José García ◽

Carlos Israel Puell ◽

...

Keyword(s):

Spinal Cord ◽

Growth Hormone ◽

Congenital Abnormality ◽

Plantar Flexion ◽

Blood Analysis ◽

Caudal Regression Syndrome ◽

Gh Treatment ◽

Caudal Regression ◽

And Migration ◽

The Brain

Caudal regression syndrome (CRS) is a congenital abnormality characterized by an incomplete development of the spinal cord (SC) and other abnormalities. We studied a 9-months old CRS child presenting: interruption of SC at L2-L3 level, sacral agenesis, lack of innervation of the inferior limbs (flaccid paraplegia) and neurogenic bladder and bowel. Given the effects of growth hormone (GH) on the proliferation, differentiation and migration of neural stem cells (NSCs), we treated him with GH and rehabilitation, trying to induce the recovery of main sequelae. GMFM-88 test score was 12.31%. After a blood analysis, GH treatment (0.3 mg/day, 5 days/week, 3 months and then 15 days without GH) and rehabilitation commenced. This protocol was followed during 5 years, being the last GH dose 1 mg/day. Blood analysis and physical exams were performed every 3 months initially and every 6 months later. Six months after commencing the treatment GMFM-88 score increased to 39.48%. Responses to sensitive stimuli appeared in most of the territories explored; 18 months later sensitive innervation was complete and the patient moved any muscle over the knees and controlled his sphincters. Three years later he walked with the help of canes, there was plantar flexion and GMFM-88 score was 78.48%. In summary, GH plus rehabilitation may be useful for innervating distal territories, below the level of the incomplete spinal cord in CRS. Most likely, GH acts on ependymal SC NSCs, as the hormone does in the neurogenic niches in the brain.

Download Full-text