Papilledema in unicoronal synostosis: a rare finding

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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.
Article Information

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.

© AANS, except where prohibited by US copyright law.

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