Document Detail

Tethered spinal cord and VACTERL association.
MedLine Citation:
PMID:  17465385     Owner:  NLM     Status:  MEDLINE    
OBJECT: Vertebral defects, anal atresia, cardiovascular anomalies, tracheoesophageal fistulas (TEFs), renal anomalies, and limb defects (most often of the radius) are commonly associated and known collectively by the acronym VACTERL. The authors studied these nonrandomly associated birth defects to determine if a further relationship exists between VACTERL association and the presence of a tethered spinal cord (TSC). METHODS: From 2001 to 2004, 12 patients with VACTERL association who were treated operatively by a single pediatric surgeon underwent magnetic resonance (MR) imaging to evaluate the intraspinal abnormalities that may cause tethering. Three patients were excluded from the study due to complications after surgery for TEF. Coincidentally, these three patients did not have imperforate ani. In the remaining nine patients, seven had associated urogenital anomalies, and six of these also had high-type imperforate ani. Five of the six patients and the one patient with low-type imperforate anus and a urogenital anomaly were found to have TSCs. In the remaining two patients without urogenital anomalies there was a high-type imperforate anus without a TSC in one patient and a low-type imperforate anus with a TSC in the other. All seven patients with TSCs underwent successful untethering. The lesions contributing to TSC included terminal filum lipomas (TFLs) in five patients, an intramedullary ependymal cyst in one patient, and a lipo-meningomyelocele in another patient. CONCLUSIONS: The authors found that in patients with VACTERL association there was a high incidence of TSC (seven of nine patients) if an imperforate anus was present as one of the anomalies. In patients with VACTERL association and urogenital anomalies, the incidence of TSC was even higher (86%). Five of the seven cases of TSC in the present study were caused by a TFL, a lesion that can be easily and safely managed surgically. The authors conclude that MR imaging is essential for ruling out the possibility of a TSC in patients with VACTERL association combined with urogenital anomalies or an imperforate anus.
Meng-Fai Kuo; Yihsin Tsai; Wen-Ming Hsu; Ruei-Sheng Chen; Yong-Kwang Tu; Huei-Shyong Wang
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of neurosurgery     Volume:  106     ISSN:  0022-3085     ISO Abbreviation:  J. Neurosurg.     Publication Date:  2007 Mar 
Date Detail:
Created Date:  2007-04-30     Completed Date:  2007-05-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0253357     Medline TA:  J Neurosurg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  201-4     Citation Subset:  AIM; IM    
Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei.
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MeSH Terms
Abnormalities, Multiple*
Anus, Imperforate / complications*
Follow-Up Studies
Middle Aged
Neural Tube Defects / complications*,  surgery
Prospective Studies
Treatment Outcome
Urogenital Abnormalities / complications*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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