Papilledema in unicoronal synostosis: a rare finding

View More View Less
  • 1 Departments of Plastic and Reconstructive Surgery,
  • | 2 Neurosurgery, and
  • | 3 Ophthalmology, Sophia Children’s Hospital, Erasmus University Medical Center, Rotterdam, The Netherlands
Restricted access

Purchase Now

USD  $45.00

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

USD  $505.00

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

USD  $600.00
Print or Print + Online

OBJECTIVE

Unicoronal synostosis results in frontal plagiocephaly and is preferably treated before the patient is 1 year of age to prevent intracranial hypertension (ICH). However, data on the prevalence of ICH in these patients is currently lacking. This study aimed to establish the prevalence of preoperative and postoperative signs of ICH in a large cohort of patients with unicoronal synostosis and to test whether there is a correlation between papilledema and occipitofrontal head circumference (OFC) curve stagnation in unicoronal synostosis.

METHODS

The authors included all patients with unicoronal synostosis treated before 2 years of age at a single center between 2003 and 2013. The presence of ICH was evaluated by routine fundoscopy. The OFC growth curve was analyzed for deflection and in relationship to signs of ICH.

RESULTS

In total, 104 patients were included in this study, 84 (81%) of whom were considered to have nonsyndromic unicoronal synostosis. Preoperatively, none of the patients had papilledema as determined by fundoscopy (mean age at surgery 11 months). Postoperatively, 5% of patients with syndromic synostosis and 3% of those with nonsyndromic synostosis had papilledema, and this was confirmed by optical coherence tomography. Raised intracranial pressure was confirmed in 1 patient with syndromic unicoronal synostosis. Six of 78 patients had OFC stagnation, which was not significantly correlated to papilledema (p = 0.22). One child with syndromic unicoronal synostosis required repeated surgery for ICH (0.96%).

CONCLUSIONS

Papilledema was not found in patients with unicoronal synostosis when they underwent surgery before the age of 1 year and was also very rare during follow-up. There was no relationship between papilledema and OFC stagnation.

ABBREVIATIONS

FOAR = frontoorbital advancement and remodeling; ICH = intracranial hypertension; ICP = intracranial pressure; OCT = optical coherence tomography; OFC = occipitofrontal head circumference; TRT = total retinal thickness.

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

USD  $505.00

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

USD  $600.00

Contributor Notes

Correspondence Stephanie D. C. van de Beeten: Sophia Children’s Hospital, Erasmus University Medical Center, Rotterdam, The Netherlands. s.vandebeeten@erasmusmc.nl.

INCLUDE WHEN CITING Published online May 17, 2019; DOI: 10.3171/2019.3.PEDS18624.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

  • 1

    Alford J, Derderian CA, Smartt JM Jr: Surgical treatment of nonsyndromic unicoronal craniosynostosis. J Craniofac Surg 29:11991207, 2018

  • 2

    Buda FB, Reed JC, Rabe EF: Skull volume in infants. Methodology, normal values, and application. Am J Dis Child 129:11711174, 1975

  • 3

    Cetas JS, Nasseri M, Saedi T, Kuang AA, Selden NR: Delayed intracranial hypertension after cranial vault remodeling for nonsyndromic single-suture synostosis. J Neurosurg Pediatr 11:661666, 2013

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Cornelissen MJ, Loudon SE, van Doorn FE, Muller RP, van Veelen MC, Mathijssen IM: Very low prevalence of intracranial hypertension in trigonocephaly. Plast Reconstr Surg 139:97e104e, 2017

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5

    Denis D, Genitori L, Bolufer A, Lena G, Saracco JB, Choux M: Refractive error and ocular motility in plagiocephaly. Childs Nerv Syst 10:210216, 1994

  • 6

    Eley KA, Johnson D, Wilkie AO, Jayamohan J, Richards P, Wall SA: Raised intracranial pressure is frequent in untreated nonsyndromic unicoronal synostosis and does not correlate with severity of phenotypic features. Plast Reconstr Surg 130:690e697e, 2012

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 7

    Florisson JM, van Veelen ML, Bannink N, van Adrichem LN, van der Meulen JJ, Bartels MC, et al. : Papilledema in isolated single-suture craniosynostosis: prevalence and predictive factors. J Craniofac Surg 21:2024, 2010

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Goodrich JT: Skull base growth in craniosynostosis. Childs Nerv Syst 21:871879, 2005

  • 9

    Hilling DE, Mathijssen IM, Mulder PG, Vaandrager JM: Long-term aesthetic results of frontoorbital correction for frontal plagiocephaly. J Neurosurg 105 (1 Suppl):2125, 2006

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Jimenez DF, Barone CM: Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative cranial orthosis therapy: 16-year experience. J Neurosurg Pediatr 12:207219, 2013

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

    MacKinnon S, Proctor MR, Rogers GF, Meara JG, Whitecross S, Dagi LR: Improving ophthalmic outcomes in children with unilateral coronal synostosis by treatment with endoscopic strip craniectomy and helmet therapy rather than fronto-orbital advancement. J AAPOS 17:259265, 2013

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

    Mathijssen I, Arnaud E, Lajeunie E, Marchac D, Renier D: Postoperative cognitive outcome for synostotic frontal plagiocephaly. J Neurosurg 105 (1 Suppl):1620, 2006

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Renier D, Lajeunie E, Arnaud E, Marchac D: Management of craniosynostoses. Childs Nerv Syst 16:645658, 2000

  • 14

    Renier D, Sainte-Rose C, Marchac D, Hirsch JF: Intracranial pressure in craniostenosis. J Neurosurg 57:370377, 1982

  • 15

    Rijken BF, den Ottelander BK, van Veelen ML, Lequin MH, Mathijssen IM: The occipitofrontal circumference: reliable prediction of the intracranial volume in children with syndromic and complex craniosynostosis. Neurosurg Focus 38(5):E9, 2015

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16

    Spruijt B, Joosten KF, Driessen C, Rizopoulos D, Naus NC, van der Schroeff MP, et al. : Algorithm for the management of intracranial hypertension in children with syndromic craniosynostosis. Plast Reconstr Surg 136:331340, 2015

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

    Taylor JA, Paliga JT, Wes AM, Tahiri Y, Goldstein JA, Whitaker LA, et al. : A critical evaluation of long-term aesthetic outcomes of fronto-orbital advancement and cranial vault remodeling in nonsyndromic unicoronal craniosynostosis. Plast Reconstr Surg 135:220231, 2015

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

    Thompson DN, Malcolm GP, Jones BM, Harkness WJ, Hayward RD: Intracranial pressure in single-suture craniosynostosis. Pediatr Neurosurg 22:235240, 1995

  • 19

    Tuite GF, Chong WK, Evanson J, Narita A, Taylor D, Harkness WF, et al. : The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery 38:272278, 1996

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20

    van Veelen MLC, Eelkman Rooda OH, de Jong T, Dammers R, van Adrichem LN, Mathijssen IM: Results of early surgery for sagittal suture synostosis: long-term follow-up and the occurrence of raised intracranial pressure. Childs Nerv Syst 29:9971005, 2013

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 21

    van Veelen MLC, Kamst N, Touw C, Mauff K, Versnel S, Dammers R, et al. : Minimally invasive, spring-assisted correction of sagittal suture synostosis: technique, outcome, and complications in 83 cases. Plast Reconstr Surg 141:423433, 2018

    • Search Google Scholar
    • Export Citation
  • 22

    van Veelen MLC, Mihajlović D, Dammers R, Lingsma H, van Adrichem LN, Mathijssen IM: Frontobiparietal remodeling with or without a widening bridge for sagittal synostosis: comparison of 2 cohorts for aesthetic and functional outcome. J Neurosurg Pediatr 16:8693, 2015

    • Crossref
    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 786 185 3
Full Text Views 104 18 0
PDF Downloads 106 10 0
EPUB Downloads 0 0 0