International survey on the management of lumbosacral cutaneous stigmata in infants with suspected occult spinal dysraphism

View More View Less
  • 1 Department of Neurosurgery, Brown University, Providence, Rhode Island;
  • | 2 Department of Neurosurgery, University of Colorado, Denver; and
  • | 3 Department of Neurosurgery, Children’s Hospital Colorado, Aurora, Colorado
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
Print or Print + Online

OBJECTIVE

Occult spinal dysraphism (OSD) is a common pediatric neurosurgical diagnosis rife with controversy surrounding both the screening of asymptomatic infants and the threshold to offer a prophylactic detethering operation. The authors sought to clarify international practice patterns with a survey of pediatric neurosurgeons.

METHODS

A survey asked pediatric neurosurgeons whether they would perform imaging in patients with a variety of cutaneous stigmata associated with OSD and whether they would offer prophylactic detethering surgery for asymptomatic patients with a variety of imaging findings on the OSD spectrum.

RESULTS

Completed surveys were received from 141 pediatric neurosurgeons. Broad consensus was demonstrated on the need for obtaining images in sample patients with more severe stigmata ranging from large lipoma with a skin appendage to focal dysplastic skin in the lumbar midline. Ninety percent of respondents would perform MRI for these patients. In contrast, for patients with a low-sacral dimple, flat hemangioma, and symmetric (Y-shaped) splaying of the intergluteal cleft, opinion on the need for imaging varied considerably (between 57% and 89% recommended imaging). Respondents differed on the type of imaging that they would perform, with 31% to 38% recommending ultrasound screening. The responses reflected less consensus on when to offer surgery to patients with simple spinal tethering (low-lying conus medullaris and fatty filum terminale). Both a lower level of the conus and increased thickness of the filum terminale affected decision-making.

CONCLUSIONS

The results of this survey showed significant consensus on the recommendation for screening imaging in patients with more dramatic cutaneous stigmata, although these stigmata are the rarest. A significant variance in opinions was reflected in the recommendation for imaging of the most common cutaneous stigmata. Consensus was also lacking on which lesions deserve prophylactic detethering surgery. Significant equipoise exists for future study of screening imaging and of surgical decision-making in patients with asymptomatic OSD and associated cutaneous stigmata.

ABBREVIATIONS

FFT = fibrolipoma of the filum terminale; OSD = occult spinal dysraphism; REDCap = Research Electronic Data Capture.

Supplementary Materials

    • Supplemental Data (PDF 1,265 KB)

Illustration from Soleman et al. (pp 544–552). Copyright Lucille Solomon.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $515.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
  • 1

    Hoffman HJ, Hendrick EB, Humphreys RP. The tethered spinal cord: its protean manifestations, diagnosis and surgical correction. Childs Brain. 1976;2(3):145155.

    • Search Google Scholar
    • Export Citation
  • 2

    Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurg Focus. 2007;23(2):E2.

  • 3

    Tani S, Yamada S, Knighton RS. Extensibility of the lumbar and sacral cord. Pathophysiology of the tethered spinal cord in cats. J Neurosurg. 1987;66(1):116123.

    • Search Google Scholar
    • Export Citation
  • 4

    Tuite GF, Thompson DNP, Austin PF, Bauer SB. Evaluation and management of tethered cord syndrome in occult spinal dysraphism: recommendations from the international children’s continence society. Neurourol Urodyn. 2018;37(3):890903.

    • Search Google Scholar
    • Export Citation
  • 5

    Chern JJ, Aksut B, Kirkman JL, Shoja MM, Tubbs RS, et al. The accuracy of abnormal lumbar sonography findings in detecting occult spinal dysraphism: a comparison with magnetic resonance imaging. J Neurosurg Pediatr. 2012;10(2):150153.

    • Search Google Scholar
    • Export Citation
  • 6

    Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol. 2004;140(9):11091115.

    • Search Google Scholar
    • Export Citation
  • 7

    Henriques JG, Pianetti G, Henriques KS, Costa P, Gusmão S. Minor skin lesions as markers of occult spinal dysraphisms—prospective study. Surg Neurol. 2005;63(suppl 1):S8S12.

    • Search Google Scholar
    • Export Citation
  • 8

    Kucera JN, Coley I, O’Hara S, Kosnik EJ, Coley BD. The simple sacral dimple: diagnostic yield of ultrasound in neonates. Pediatr Radiol. 2015;45(2):211216.

    • Search Google Scholar
    • Export Citation
  • 9

    McGovern M, Mulligan S, Carney O, Wall D, Moylett E. Ultrasound investigation of sacral dimples and other stigmata of spinal dysraphism. Arch Dis Child. 2013;98(10):784786.

    • Search Google Scholar
    • Export Citation
  • 10

    O’Neill BR, Gallegos D, Herron A, et al. Use of MRI to detect occult spinal dysraphism in infants. J Neurosurg Pediatr. 2017;19(2):217226.

    • Search Google Scholar
    • Export Citation
  • 11

    Tubbs RS, Wellons JC III, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg. 2004;100(2)(Suppl Pediatrics):8689.

    • Search Google Scholar
    • Export Citation
  • 12

    Weprin BE, Oakes WJ. Coccygeal pits. Pediatrics. 2000;105(5):E69.

  • 13

    Ponger P, Ben-Sira L, Beni-Adani L, Steinbok P, Constantini S. International survey on the management of skin stigmata and suspected tethered cord. Childs Nerv Syst. 2010;26(12):17191725.

    • Search Google Scholar
    • Export Citation
  • 14

    Steinbok P, Garton HJ, Gupta N. Occult tethered cord syndrome: a survey of practice patterns. J Neurosurg. 2006;104(5)(suppl):309313.

    • Search Google Scholar
    • Export Citation
  • 15

    Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde JG. Research electronic data capture (REDCap)—a metadata-driven methodology and workflow process for providing translational research informatics support. J Biomed Inform. 2009;42(2):377381.

    • Search Google Scholar
    • Export Citation
  • 16

    Ben-Sira L, Ponger P, Miller E, Beni-Adani L, Constantini S. Low-risk lumbar skin stigmata in infants: the role of ultrasound screening. J Pediatr. 2009;155(6):864869.

    • Search Google Scholar
    • Export Citation
  • 17

    Bulsara KR, Zomorodi AR, Enterline DS, George TM. The value of magnetic resonance imaging in the evaluation of fatty filum terminale. Neurosurgery. 2004;54(2):375380.

    • Search Google Scholar
    • Export Citation
  • 18

    Harada A, Nishiyama K, Yoshimura J, Sano M, Fujii Y. Intraspinal lesions associated with sacrococcygeal dimples. J Neurosurg Pediatr. 2014;14(1):8186.

    • Search Google Scholar
    • Export Citation
  • 19

    Paul M, Goodyear H, Rodrigues D, Loffeld A. Early recognition of occult spinal dysraphism through cutaneous stigmata. Arch Dis Child Educ Pract Ed. 2015;100(5):252280,280.

    • Search Google Scholar
    • Export Citation
  • 20

    Kriss VM, Desai NS. Occult spinal dysraphism in neonates: assessment of high-risk cutaneous stigmata on sonography. AJR Am J Roentgenol. 1998;171(6):16871692.

    • Search Google Scholar
    • Export Citation
  • 21

    Robinson AJ, Russell S, Rimmer S. The value of ultrasonic examination of the lumbar spine in infants with specific reference to cutaneous markers of occult spinal dysraphism. Clin Radiol. 2005;60(1):7277.

    • Search Google Scholar
    • Export Citation
  • 22

    Lendon RG, Emery JL. Forking of the central canal in the equinal cord of children. J Anat. 1970;106(Pt 3):499505.

  • 23

    McLendon RE, Oakes WJ, Heinz ER, Yeates AE, Burger PC. Adipose tissue in the filum terminale: a computed tomographic finding that may indicate tethering of the spinal cord. Neurosurgery. 1988;22(5):873876.

    • Search Google Scholar
    • Export Citation
  • 24

    Al-Omari MH, Eloqayli HM, Qudseih HM, Al-Shinag MK. Isolated lipoma of filum terminale in adults: MRI findings and clinical correlation. J Med Imaging Radiat Oncol. 2011;55(3):286290.

    • Search Google Scholar
    • Export Citation
  • 25

    Brown E, Matthes JC, Bazan C III, Jinkins JR. Prevalence of incidental intraspinal lipoma of the lumbosacral spine as determined by MRI. Spine (Phila Pa 1976).1994;19(7):833836.

    • Search Google Scholar
    • Export Citation
  • 26

    Cools MJ, Al-Holou WN, Stetler WR Jr, Wilson TJ, Muraszko KM, et al. Filum terminale lipomas: imaging prevalence, natural history, and conus position. J Neurosurg Pediatr. 2014;13(5):559567.

    • Search Google Scholar
    • Export Citation
  • 27

    Kesler H, Dias MS, Kalapos P. Termination of the normal conus medullaris in children: a whole-spine magnetic resonance imaging study. Neurosurg Focus. 2007;23(2):E7.

    • Search Google Scholar
    • Export Citation
  • 28

    Day EL, Proctor MR, Scott RM. Surgical volume of simple tethered spinal cord releases: review of a large pediatric neurosurgical service experience. J Neurosurg Pediatr. 2020;26(1):6064.

    • Search Google Scholar
    • Export Citation
  • 29

    Harris PA, Taylor R, Minor BL, et al. The REDCap consortium: Building an international community of software partners. J Biomed Inform. 2019;95:103208.

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 142 142 131
Full Text Views 43 43 33
PDF Downloads 64 64 50
EPUB Downloads 0 0 0