Pediatric brainstem cavernous malformations: 2-center experience in 40 children

View More View Less
  • 1 Department of Neurosurgery, Clinical Neuroscience Center, University Hospital Zurich, Switzerland;
  • | 2 University of Zurich, Switzerland;
  • | 3 Division of Pediatric Neurosurgery, University Children’s Hospital Zurich, Switzerland;
  • | 4 Department of Pediatric Neurosurgery, APHP, Hôpital Necker, Paris, France;
  • | 5 Université de Paris, France;
  • | 6 Department of Neurosurgery, Kantonsspital St. Gallen, Switzerland; and
  • | 7 Department of Neurosurgery, Hôpital Pierre Zobda Quitman, CHU de Fort de France, Université des Antilles, Fort de France, Martinique
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
USD  $45.00
USD  $515.00
USD  $612.00
Print or Print + Online Sign in

OBJECTIVE

Brainstem cavernous malformations (BSCMs) are relatively uncommon, low-flow vascular lesions in children. Given the paucity of data, guidelines regarding the clinical management of BSCMs in children are lacking and the surgical indication is most commonly based on an individual surgeon’s judgment and experience. The goal in this study was to evaluate the clinical behavior of BSCMs in childhood and the long-term outcome in children managed conservatively and surgically.

METHODS

This was an observational, retrospective study including all children with BSCMs who were followed at 2 institutions between 2008 and 2020.

RESULTS

The study population consisted of 40 children (27 boys, 67.5%) with a mean age of 11.4 years. Twenty-three children (57.5%) were managed conservatively, whereas 17 children (42.5%) underwent resection of BSCMs. An aggressive clinical course was observed in 13 children (32.5%), who experienced multiple hemorrhages with a progressive pattern of neurological decline. Multiple BSCMs were observed in 8 patients, of whom 3 patients presented with a complex of multiple tightly attached BSCMs and posed a significant therapeutic challenge. The overall long-term outcome was favorable (modified Rankin Scale [mRS] scores 0–2) in 36 patients (90%), whereas an unfavorable outcome (mRS scores 3 and 4) was seen in 4 children (10%). An mRS score of 5 or 6 was not observed. The mean (± SD) follow-up was 88.0 (± 92.6) months.

CONCLUSIONS

The clinical course of BSCMs in children is highly variable, with benign lesions on the one hand and highly aggressive lesions with repetitive hemorrhages on the other. Given the greater life expectancy and the known higher functional recovery in children, surgical treatment should be considered early in young patients presenting with surgically accessible lesions and an aggressive clinical course, and it should be performed in a high-volume center.

ABBREVIATIONS

BSCM = brainstem cavernous malformation; CCM = cerebral cavernous malformation; CN = cranial nerve; DVA = developmental venous anomaly; FCCM = familial CCM; ICP = intracranial pressure; mRS = modified Rankin Scale.

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

USD  $515.00

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

USD  $612.00
USD  $515.00
USD  $612.00
  • 1

    Gross BA, Du R. Cerebral cavernous malformations: natural history and clinical management. Expert Rev Neurother. 2015;15(7):771777.

  • 2

    Prolo LM, Jin MC, Loven T, et al. Recurrence of cavernous malformations after surgery in childhood. J Neurosurg Pediatr. 2020;26(2):179188.

  • 3

    Braga BP, Costa LB Jr, Lemos S, Vilela MD. Cavernous malformations of the brainstem in infants. Report of two cases and review of the literature. J Neurosurg. 2006;104(6 Suppl):429433.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Goyal P, Mangla R, Gupta S, et al. Pediatric congenital cerebrovascular anomalies. J Neuroimaging. 2019;29(2):165181.

  • 5

    Garcia RM, Ivan ME, Lawton MT. Brainstem cavernous malformations: surgical results in 104 patients and a proposed grading system to predict neurological outcomes. Neurosurgery. 2015;76(3):265278.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6

    Nathal E, Patiño-Rodriguez HM, Arauz A, et al. Risk factors for unfavorable outcomes in surgically treated brainstem cavernous malformations. World Neurosurg. 2018;111:e478e484.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Porter RW, Detwiler PW, Spetzler RF, et al. Cavernous malformations of the brainstem: experience with 100 patients. J Neurosurg. 1999;90(1):5058.

  • 8

    Gross BA, Du R. Hemorrhage from cerebral cavernous malformations: a systematic pooled analysis. J Neurosurg. 2017;126(4):10791087.

  • 9

    Kearns KN, Chen CJ, Tvrdik P, Park MS, Kalani MYS. Outcomes of surgery for brainstem cavernous malformations: a systematic review. Stroke. 2019;50(10):29642966.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Abla AA, Lekovic GP, Turner JD, de Oliveira JG, Porter R, Spetzler RF. Advances in the treatment and outcome of brainstem cavernous malformation surgery: a single-center case series of 300 surgically treated patients. Neurosurgery. 2011;68(2):403415.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Zaidi HA, Mooney MA, Levitt MR, Dru AB, Abla AA, Spetzler RF. Impact of timing of intervention among 397 consecutively treated brainstem cavernous malformations. Neurosurgery. 2017;81(4):620626.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Dukatz T, Sarnthein J, Sitter H, et al. Quality of life after brainstem cavernoma surgery in 71 patients. Neurosurgery. 2011;69(3):689695.

  • 13

    Abla AA, Lekovic GP, Garrett M, et al. Cavernous malformations of the brainstem presenting in childhood: surgical experience in 40 patients. Neurosurgery. 2010;67(6):15891599.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Bhardwaj RD, Auguste KI, Kulkarni AV, Dirks PB, Drake JM, Rutka JT. Management of pediatric brainstem cavernous malformations: experience over 20 years at the hospital for sick children. J Neurosurg Pediatr. 2009;4(5):458464.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Liu W, Liu R, Ma Z, Li C. Transcallosal anterior interforniceal approach for removal of superior midbrain cavernous malformations in children: a retrospective series of 10 cases in a single center. World Neurosurg. 2018;118:e188e194.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 16

    Lena G, Ternier J, Paz-Paredes A, Scavarda D. Central nervous system cavernomas in children. Article in French. Neurochirurgie. 2007;53(2-3 Pt 2):223237.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Sawarkar DP, Janmatti S, Kumar R, et al. Cavernous malformations of central nervous system in pediatric patients: our single-centered experience in 50 patients and review of literature. Childs Nerv Syst. 2017;33(9):15251538.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Li D, Hao SY, Tang J, et al. Clinical course of untreated pediatric brainstem cavernous malformations: hemorrhage risk and functional recovery. J Neurosurg Pediatr. 2014;13(5):471483.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19

    van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke. 1988;19(5):604607.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 20

    Morrison L, Akers A. Cerebral cavernous malformation, familial. In: Adam MP, Ardinger HH, Pagon RA, et al, eds. GeneReviews. University of Washington;2003.

    • Search Google Scholar
    • Export Citation
  • 21

    Yang Y, van Niftrik B, Ma X, et al. Analysis of safe entry zones into the brainstem. Neurosurg Rev. 2019;42(3):721729.

  • 22

    Yang Y, Velz J, Neidert MC, Bozinov O. Classification of brainstem lesion location. World Neurosurg. 2020;140:410412.

  • 23

    Yang Y, Velz J, Neidert MC, Lang W, Regli L, Bozinov O. The BSCM score: a guideline for surgical decision-making for brainstem cavernous malformations. Neurosurg Rev. Published online October 29, 2021.doi: 10.1007/s10143-021-01679-y

    • Search Google Scholar
    • Export Citation
  • 24

    Velz J, Vasella F, Yang Y, Neidert MC, Regli L, Bozinov O. Limited impact of serial follow-up imaging in clinically stable patients with brainstem cavernous malformations. Front Neurol. 2020;11:789.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 25

    Riant F, Bergametti F, Ayrignac X, Boulday G, Tournier-Lasserve E. Recent insights into cerebral cavernous malformations: the molecular genetics of CCM. FEBS J. 2010;277(5):10701075.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 26

    Cavalcanti DD, Kalani MYS, Martirosyan NL, Eales J, Spetzler RF, Preul MC. Cerebral cavernous malformations: from genes to proteins to disease. J Neurosurg. 2012;116(1):122132.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 27

    Labauge P, Laberge S, Brunereau L, Levy C, Tournier-Lasserve E. Hereditary cerebral cavernous angiomas: clinical and genetic features in 57 French families. Société Française de Neurochirurgie. Lancet. 1998;352(9144):18921897.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 28

    Velz J, Neidert MC, Yang Y, et al. Mortality in patients with brainstem cavernous malformations. Cerebrovasc Dis. 2021;50(5):574580.

  • 29

    Yang Y, Velz J, Neidert MC, Stienen MN, Regli L, Bozinov O. Natural history of brainstem cavernous malformations: on the variation in hemorrhage rates. World Neurosurg. 2022;157:e342e350.

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 1033 1033 251
Full Text Views 165 165 43
PDF Downloads 177 177 81
EPUB Downloads 0 0 0