Van der woude syndrome with associated scaphocephaly: a rare case report

The purpose of this case is to provide a descriptive report of a rare combination of Van der Woude syndrome, recurrent in three generations of the same family, in a female patient with bilateral cleft lip and palate associated with a sagittal craniosynostosis (scaphocephaly).

Minimally Invasive Non-Endoscopic Correction for Sagittal Craniosynostosis in Children Aged Below 6 Months

AIM: Object of this study is to determine effectiveness of this procedure used in this study which is minimally invasive non endoscopic correction for sagittal craniosynostosis in children below 6 months. METHODS: A prospective study was done in Neurosurgery children unit, Abu Elreash Japanese children hospital, Cairo university, Egypt, for patients with non syndromatic sagittal craniosynostosis. The variables analyzed; OFD, BPD, CI pre. & postoperative, age of patients at time of surgery, sex, duration of surgery and length of hospital stay. RESULTS: Our results shows statistically significant difference between pre-operative with post-operative after 2 days, post-operative follow-up, and follow-up after 1 year for OFD, BPD and CI. CONCLUSIONS: Internal helmet technique used in correction of sagittal synostosis in children below 6 months old giving good cosmetic results with the following advantages, (1) short surgery time, (2) small wound, (3) no much blood loss, (4) immediate good cosmetic result, (5) no need to use external devices which reduces the cost, family load, and child suffering, and (6) no need for long-term follow-up.

Predicting and comparing three corrective techniques for sagittal craniosynostosis

Sagittal synostosis is the most occurring form of craniosynostosis, resulting in calvarial deformation and possible long-term neurocognitive deficits. Several surgical techniques have been developed to correct these issues. Debates as to the most optimal approach are still ongoing. Finite element method is a computational tool that’s shown to assist with the management of craniosynostosis. The aim of this study was to compare and predict the outcomes of three reconstruction methods for sagittal craniosynostosis. Here, a generic finite element model was developed based on a patient at 4 months of age and was virtually reconstructed under all three different techniques. Calvarial growth was simulated to predict the skull morphology and the impact of different reconstruction techniques on the brain growth up to 60 months of age. Predicted morphology was then compared with in vivo and literature data. Our results show a promising resemblance to morphological outcomes at follow up. Morphological characteristics between considered techniques were also captured in our predictions. Pressure outcomes across the brain highlight the potential impact that different techniques have on growth. This study lays the foundation for further investigation into additional reconstructive techniques for sagittal synostosis with the long-term vision of optimizing the management of craniosynostosis.

Rate of craniometric change following suture release in patients with metopic and sagittal craniosynostosis

OBJECTIVE Craniosynostosis is a congenital disorder resulting from the premature fusion of cranial sutures in the infant skull. This condition results in significant cosmetic deformity and can impede neurodevelopment, if left untreated. Currently, rates of craniometric change following minimally invasive surgery have only been examined for sagittal craniosynostosis. A better understanding of postoperative skull adaptations in other craniosynostosis subtypes is needed to objectively categorize surgical outcomes and guide length of cranial orthosis therapy. METHODS Eleven patients with sagittal and 8 with metopic craniosynostosis treated using endoscopic strip craniectomy and postoperative helmet orthoses were retrospectively reviewed. Using semiautomated image analysis of top-down orthogonal 2D photographs, the following craniometrics were recorded before surgery and at postoperative visits: cephalic index (CI), cranial vault asymmetry index (CVAI), anterior arc angle (AAA), posterior arc angle (PAA), anterior-middle width ratio (AMWR), anterior-posterior width ratio (APWR), left-right height ratio (LRHR), sagittal Hu moment (Sag-Hu), and brachycephaly Hu moment (Brachy-Hu). These craniometrics were then normalized to photograph-based measurements of normocephalic patients and the rates of change between metopic and sagittal craniosynostoses were compared. RESULTS Patients with sagittal craniosynostosis exhibited significantly lower CI, lower PAA, higher AMWR, higher APWR, lower Sag-Hu, and higher Brachy-Hu preoperatively compared to patients with normocephalic craniosynostosis. Patients with metopic craniosynostosis exhibited lower AAA and AMWR preoperatively compared to normocephalic subjects. Sagittal and metopic patients had a rapid initial change in normalized CI or AAA, respectively. Craniometric rates of change that significantly differed between metopic and sagittal patients were found in AAA (p < 0.001), AMWR (p < 0.001), and APWR (p < 0.0001). Metopic patients had a prolonged AAA change with a significantly different rate of change up to 6 months postoperatively (median at 3 months = 0.027 normalized units/day, median at 6 months = 0.017 normalized units/day, and median at > 6 months = 0.007 normalized units/day), while sagittal CI rate of change at these time points was not significantly different. CONCLUSIONS Patients with metopic craniosynostosis have a prolonged rate of change compared to patients with sagittal craniosynostosis and may benefit from longer helmet use and extended postoperative follow-up. Categorizing craniometric changes for other craniosynostosis subtypes will be important for evaluating current treatment guidelines.