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Surya Sri Krishna Gour, Mohit Agrawal and Dattaraj Sawarkar

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Marie-Lise C. van Veelen, Dalibor Mihajlović, Ruben Dammers, Hester Lingsma, Leon N. A. van Adrichem and Irene M. J. Mathijssen

OBJECT

Various techniques to correct sagittal synostosis have been described. The authors of this study assess the results of 2 techniques for late complete cranial remodeling and test the hypothesis that adding a widening bridge would improve outcome.

METHODS

In this retrospective study, the authors evaluated patients with nonsyndromic sagittal synostosis—those who underwent frontobiparietal remodeling (FBR) and those who underwent modified FBR (MFBR) involving the introduction of a bony bridge to increase the width of the skull. Outcomes for both groups are described in terms of the aesthetic results assessed on photographs and any changes in the cranial index (CI) and head circumference over time, the presence of papilledema, and complaints of headache. The effect of the surgical technique on CI and head circumference over time was assessed using linear regression analysis, with adjustment for preoperative CI and head circumference.

RESULTS

Sixty-nine patients with isolated sagittal synostosis were included in this study: 35 underwent MFBR and 34 underwent the original technique of FBR. The mean follow-up period was 7 years. In the 1st year after surgery, mean CI improved by 9% in the FBR group and by 12% in the MFBR group. One year after surgery, CI in the MFBR group was on average 4.7% higher than that in the FBR group (p < 0.001). During follow-up, CI decreased in both groups; however, at all time points CI was significantly higher in the MFBR group than in the FBR group. The impact of surgical technique on CI was less important than the impact of preoperative CI (R2= 0.26 vs 0.54), and this applied at all time points during follow-up. Head circumference declined during follow-up in both groups. It was influenced by preoperative head circumference, but not by surgical technique. Aesthetic outcome, prevalence of headache (42%), and papilledema (7%) were comparable in both groups.

CONCLUSIONS

Adding a widening bridge to late complete remodeling significantly improved CI and helped to prevent CI from decreasing in the long term. This addition did not affect the head circumference growth curve. Despite a mean head circumference remaining at +1 SD, patients continued to develop papilledema postoperatively (7%).

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Martijn J. Cornelissen, Robbin de Goederen, Priya Doerga, Iris Cuperus, Marie-Lise van Veelen, Maarten Lequin, Paul Govaert, Irene M. J. Mathijssen, Jeroen Dudink and Robert C. Tasker

OBJECTIVE

In addition to craniocerebral disproportion, other factors, such as Chiari malformation type I, obstructive sleep apnea, and venous outflow obstruction, are considered to have a role in the occurrence of intracranial hypertension in craniosynostosis. This pilot study examined cerebral venous flow velocity to better characterize the complex intracranial venous physiology of craniosynostosis.

METHODS

The authors performed a prospective cohort study of craniosynostosis patients (n = 34) referred to a single national (tertiary) craniofacial unit. Controls (n = 28) consisted of children who were referred to the unit’s outpatient clinic and did not have craniosynostosis. Transfontanelle ultrasound scans with venous Doppler flow velocity assessment were performed at the first outpatient clinic visit and after each surgery, if applicable. Mean venous blood flow velocities of the internal cerebral vein (ICVv) and the superior sagittal sinus (SSSv) were recorded and blood flow waveform was scored.

RESULTS

Preoperatively, SSSv was decreased in craniosynostosis patients compared with controls (7.57 vs 11.31 cm/sec, p = 0.009). ICVv did not differ significantly between patients and controls. Postoperatively, SSSv increased significantly (7.99 vs 10.66 cm/sec, p = 0.023). Blood flow waveform analyses did not differ significantly between patients and controls.

CONCLUSIONS

Premature closure of cranial sutures was associated with decreased SSSv but not ICVv; indicating an effect on the superficial rather than deep venous drainage. Further Doppler ultrasound studies are needed to test the hypothesis that at an early stage of craniosynostosis pathology SSSv, but not pulsatility, is abnormal, and that abnormality in both SSSv and the superficial venous waveform reflect a more advanced stage of evolution in suture closure.

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Robbin de Goederen, Iris E. Cuperus, Robert C. Tasker, Bianca K. den Ottelander, Marjolein H. G. Dremmen, Marie-Lise C. van Veelen, Jochem K. H. Spoor, Koen F. M. Joosten and Irene M. J. Mathijssen

OBJECTIVE

Intracranial hypertension is a major concern in children with syndromic craniosynostosis (sCS). Cerebral venous hypertension caused by cerebral venous outflow obstruction is believed to contribute to intracranial hypertension. The authors therefore hypothesized that cerebral venous volume would be increased in those children with sCS and intracranial hypertension.

METHODS

In a case series of 105 children with sCS, of whom 32 had intracranial hypertension, cerebral MRI techniques were used to quantify the volume of the superior sagittal sinus, straight sinus (StrS), and both transverse sinuses.

RESULTS

Linear regression showed that total cerebral venous volume increased by 580.8 mm3 per cm increase in occipitofrontal head circumference (p < 0.001). No significant difference was found between the intracranial hypertension group and the nonintracranial hypertension group (p = 0.470). Multivariate ANOVA showed increased StrS volume (as a proportion of total volume) in the intracranial hypertension group (8.5% vs 5.1% in the nonintracranial hypertension group, p < 0.001). Multivariate logistic regression showed that a 100-mm3 increase in StrS volume is associated with increased odds of having intracranial hypertension by 60% (OR 1.60, 95% CI 1.24–2.08).

CONCLUSIONS

Although intracranial hypertension was not associated with total cerebral venous volume increase, it was associated with an isolated increase in StrS volume. Hence, it is unlikely that general cerebral venous outflow obstruction is the mechanism of intracranial hypertension in sCS. Rather, these findings indicate either a central cerebral vulnerability to intracranial hypertension or a mechanism involving venous blood redistribution.

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Priya N. Doerga, Maarten H. Lequin, Marjolein H. G. Dremmen, Bianca K. den Ottelander, Katya A. L. Mauff, Matthias W. Wagner, Juan A. Hernandez-Tamames, Sarah L. Versnel, Koen F. M. Joosten, Marie-Lise C. van Veelen, Robert C. Tasker and Irene M. J. Mathijssen

OBJECTIVE

In comparison with the general population, children with syndromic craniosynostosis (sCS) have abnormal cerebral venous anatomy and are more likely to develop intracranial hypertension. To date, little is known about the postnatal development change in cerebral blood flow (CBF) in sCS. The aim of this study was to determine CBF in patients with sCS, and compare findings with control subjects.

METHODS

A prospective cohort study of patients with sCS using MRI and arterial spin labeling (ASL) determined regional CBF patterns in comparison with a convenience sample of control subjects with identical MRI/ASL assessments in whom the imaging showed no cerebral/neurological pathology. Patients with SCS and control subjects were stratified into four age categories and compared using CBF measurements from four brain lobes, the cerebellum, supratentorial cortex, and white matter. In a subgroup of patients with sCS the authors also compared longitudinal pre- to postoperative CBF changes.

RESULTS

Seventy-six patients with sCS (35 female [46.1%] and 41 male [53.9%]), with a mean age of 4.5 years (range 0.2–19.2 years), were compared with 86 control subjects (38 female [44.2%] and 48 male [55.8%]), with a mean age of 6.4 years (range 0.1–17.8 years). Untreated sCS patients < 1 year old had lower CBF than control subjects. In older age categories, CBF normalized to values observed in controls. Graphical analyses of CBF by age showed that the normally expected peak in CBF during childhood, noted at 4 years of age in control subjects, occurred at 5–6 years of age in patients with sCS. Patients with longitudinal pre- to postoperative CBF measurements showed significant increases in CBF after surgery.

CONCLUSIONS

Untreated patients with sCS < 1 year old have lower CBF than control subjects. Following vault expansion, and with age, CBF in these patients normalizes to that of control subjects, but the usual physiological peak in CBF in childhood occurs later than expected.