Papilledema in unicoronal synostosis: a rare finding

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>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%).</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>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.</jats:p></jats:sec>

Prevalence of Ocular Anomalies in Craniosynostosis: A Systematic Review and Meta-Analysis

“…In the anatomic ophthalmic domain, papilledema was the most reported anomaly in both non-syndromic 1% (n = 333; 95% CI 0-18) [18,[20][21][22] and syndromic craniosynostosis 18% (n = 474; 95% [18,20,21,[25][26][27][28][29][30][31][32][33][34]. In non-syndromic craniosynostosis, only one study reported papilledema in lambdoid craniosynostosis, with a prevalence of 67% (n = 9) [19].…”

“…All anatomical ophthalmic anomalies in non-syndromic craniosynostosis are sented in Table 2. Compared to syndromic craniosynostosis papilledema was less c mon in non-syndromic craniosynostosis, with a prevalence of 6% (n = 333; 95% CI 0 [18][19][20][21][22]. It had the highest prevalence in lambdoid craniosynostosis 67% (n = 9) repo Abbreviations: RS = Retrospective case study; CS = case-series; PS = prospective study; CFS = Craniofrontonasal dysplasia.…”

“…All anatomical ophthalmic anomalies in syndromic craniosynostosis are presented in Table 3. In syndromic craniosynostosis, papilledema was the most reported ophthalmic anomaly with a prevalence of 18% (n = 474; 95% CI 11-26) [18,20,21,[25][26][27][28][29][30][31][32][33][34]. It was most prevalent in Crouzon syndrome, with a prevalence of 34% (n = 177; 95% CI [20,21,[25][26][27]29,30,34], followed by Apert syndrome, 9% (n = 59; 95% CI 1-22) [20,21,26,29,31,34].…”

“…Three studies did not define the demographics and were rated as high risk [24,36,37]. The included studies had a good representative population of the target population, most studies only evaluated syndromic or non-syndromic craniosynostosis, however some of these studies evaluated them together [18,20,21,25,47]. This often meant that the study population was larger, and therefore the prevalence's were stated differently than if you compared each group with itself.…”

Updated Guideline on Treatment and Management of Craniosynostosis

“…Regarding unicoronal synostosis, a recent study by Van de Beeten found papilledema in 1 patient out of 89 (1.1%) in the postoperative follow-up trajectory for which no surgery was necessary. 41 Given this very low prevalence, screening in children with non-syndromic unicoronal synostosis does not seem to be of any value.…”