Incidence of Non-Syndromic and Syndromic Craniosynostosis in Sweden

AimsCraniosynostosis is a congenital condition characterised by premature fusion of one or more cranial sutures. The aim of this study was to analyse ophthalmic function before and after cranial surgery, in children with various types of non-syndromic craniosynostosis.MethodsChildren referred to Uppsala University Hospital for surgery of non-syndromic craniosynostosis were examined preoperatively. Visual acuity was measured with Preferential Looking tests or observation of fixation and following. Strabismus and eye motility were noted. Refraction was measured in cycloplegia and funduscopy was performed. Follow-up examinations were performed 6–12 months postoperatively at the children’s local hospitals.ResultsOne hundred twenty-two children with mean age 6.2 months were examined preoperatively. Refractive values were similar between the different subtypes of craniosynostosis, except for astigmatism anisometropia which was more common in unicoronal craniosynostosis. Strabismus was found in seven children, of which four had unicoronal craniosynostosis.Postoperatively, 113 children were examined, at mean age 15.9 months. The refractive values decreased, except for astigmatism and anisometropia in unicoronal craniosynostosis. Strabismus remained in unicoronal craniosynostosis. Two new cases with strabismus developed in unicoronal craniosynostosis and one in metopic, all operated with fronto-orbital techniques. No child had disc oedema or pale discs preoperatively or postoperatively.ConclusionOphthalmic dysfunctions were not frequent in children with sagittal craniosynostosis and preoperative ophthalmological evaluation may not be imperative. Children with unicoronal craniosynostosis had the highest prevalence of strabismus and anisometropia. Fronto-orbital techniques used to address skull deformity may be related to a higher prevalence of strabismus postoperatively.

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Anomalous venous drainage in a case of non-syndromic craniosynostosis

Child s Nervous System ◽

10.1007/s003810050052 ◽

1997 ◽

Vol 13 (2) ◽

pp. 97-100 ◽

Cited By ~ 26

Author(s):

P. J. Anderson ◽

William J. Harkness ◽

Wendy Taylor ◽

Barry M. Jones ◽

Richard D. Hayward

Keyword(s):

Venous Drainage ◽

Syndromic Craniosynostosis ◽

Anomalous Venous Drainage

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Are ultrasonography measurements of optic nerve sheath diameter an alternative to funduscopy in children with syndromic craniosynostosis?

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.6.peds10547 ◽

2011 ◽

Vol 8 (3) ◽

pp. 329-334 ◽

Cited By ~ 19

Author(s):

Caroline Driessen ◽

Natalja Bannink ◽

Maarten Lequin ◽

Marie-Lise C. van Veelen ◽

Nicole C. Naus ◽

...

Keyword(s):

Optic Nerve ◽

Optic Nerve Sheath Diameter ◽

Optic Nerve Sheath ◽

Predictive Values ◽

Syndromic Craniosynostosis ◽

Nerve Sheath ◽

Age Related ◽

The Mean ◽

A Prospective Study ◽

Ct Measurement

Object Children with syndromic or complex craniosynostosis are evaluated for increased intracranial pressure (ICP) using funduscopy to detect papilledema. However, papilledema is a late sign of increased ICP. Because papilledema might be preceded by an increase in optic nerve sheath (ONS) diameter, the authors conducted a prospective study to establish the validity and applicability of measuring the ONS using ultrasonography. Methods From January 2007 to December 2009, 175 bilateral ultrasonography ONS measurements were performed in 128 patients with syndromic or complex craniosynostosis during the daytime. The measurements were correlated with ONS diameter assessed on CT and simultaneous funduscopy, when available. Furthermore, results were compared by using thresholds for ONS diameters on ultrasonography that are available in the literature. Results The mean ONS diameter on ultrasonography was 3.1 ± 0.5 mm. The CT measurement was significantly correlated with the ultrasonography measurement (r = 0.41, p < 0.001). The mean ONS diameter in 38 eyes with papilledema was 3.3 ± 0.5 mm, compared with 3.1 ± 0.5 mm in the eyes of patients without papilledema (p = 0.039). Relative to the age-related thresholds, the ONS diameter was too large in 11 eyes (3%), particularly in patients with Crouzon syndrome. Compared with funduscopy, ultrasonography sensitivity was 11%, specificity was 97%, and positive and negative predictive values were 40% and 86%, respectively. Conclusions Ultrasonography is a valid and easy way of quantifying the ONS. Although the ONS diameter is larger in children with papilledema, it cannot be used as a daytime screening tool instead of funduscopy. The ONS diameter is possibly a more real-time indicator of ICP.

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Development of secondary unilateral coronal suture synostosis with a sagittal suture synostosis in a nonsyndromic patient

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.11.peds11320 ◽

2012 ◽

Vol 9 (2) ◽

pp. 116-118 ◽

Cited By ~ 2

Author(s):

Leonardo Rangel-Castilla ◽

Steven W. Hwang ◽

Andrew Jea ◽

William E. Whitehead ◽

Daniel J. Curry ◽

...

Keyword(s):

Surgical Intervention ◽

Early Stage ◽

Sagittal Synostosis ◽

Clinical Appearance ◽

Syndromic Craniosynostosis ◽

Intervention Failure ◽

Frontoorbital Advancement ◽

Coronal Synostosis ◽

Second Procedure

Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura. The disinhibition of these factors permits the sutures to then fuse at an early stage. The authors report on a patient who developed secondary unilateral coronal synostosis after the diagnosis of an isolated sagittal synostosis. The secondary synostosis was identified at the time of the initial surgical intervention and ultimately required a second procedure of a frontoorbital advancement. The clinical appearance of this phenomenon may be subtle, and surgeons should monitor for the presence of secondary synostosis during surgery as it may require intervention. Failure to identify the secondary synostosis may necessitate another surgery or result in a poor cosmetic outcome. The authors recommend close clinical follow-up for the short term in patients with isolated sagittal synostosis.

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Outcomes of endoscopic suturectomy with postoperative helmet therapy in bilateral coronal craniosynostosis

Journal of Neurosurgery Pediatrics ◽

10.3171/2016.2.peds15693 ◽

2016 ◽

Vol 18 (3) ◽

pp. 281-286 ◽

Cited By ~ 14

Author(s):

S. Alex Rottgers ◽

Subash Lohani ◽

Mark R. Proctor

Keyword(s):

Hospital Stay ◽

Head Circumference ◽

Major Complication ◽

Csf Leak ◽

Perioperative Morbidity ◽

Syndromic Craniosynostosis ◽

Duration Of Surgery ◽

Coronal Synostosis ◽

The Mean

OBJECTIVE Historically, bilateral frontoorbital advancement (FOA) has been the keystone for treatment of turribrachycephaly caused by bilateral coronal synostosis. Early endoscopic suturectomy has become a popular technique for treatment of single-suture synostosis, with acceptable results and minimal perioperative morbidity. Boston Children's Hospital has adopted this method of treating early-presenting cases of bilateral coronal synostosis. METHODS A retrospective review of patients with bilateral coronal craniosynostosis who were treated with endoscopic suturectomy between 2005 and 2012 was completed. Patients were operated on between 1 and 4 months of age. Hospital records were reviewed for perioperative morbidity, length of stay, head circumference and cephalic indices, and the need for further surgery. RESULTS Eighteen patients were identified, 8 males and 10 females, with a mean age at surgery of 2.6 months (range 1–4 months). Nine patients had syndromic craniosynostosis. The mean duration of surgery was 73.3 minutes (range 50–93 minutes). The mean blood loss was 40 ml (range 20–100 ml), and 2 patients needed a blood transfusion. The mean duration of hospital stay was 1.2 days (range 1–2 days). There was 1 major complication in the form of a CSF leak. The mean follow-up was 37 months (range 6–102 months). Eleven percent of nonsyndromic patients required a subsequent FOA; 55.6% of syndromic patients underwent FOA. The head circumference percentiles and cephalic indices improved significantly. CONCLUSIONS Early endoscopic suturectomy successfully treats the majority of patients with bilateral coronal synostosis, and affords a short procedure time, a brief hospital stay, and an expedited recovery. Close follow-up is needed to detect patients who will require a secondary FOA due to progressive suture fusion or resynostosis of the released coronal sutures.

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