Scaphocephaly Patients Research Articles

Evaluating mechanical benefit of wedge osteotomies in endoscopic surgery for sagittal synostosis using patient-specific 3D-printed models.

Endoscopically assisted sagittal strip craniotomy with subsequent cranial orthosis is a frequently used surgical approach for non-syndromic sagittal synostosis. Originally, this technique involved a wide sagittal strip craniectomy with bilateral wedge osteotomies. More recent studies suggest omitting wedge osteotomies, achieving similar outcomes. The controversy surrounding wedge osteotomies and our efforts to refine our technique led us to create models and evaluate the mechanical impact of wedge osteotomies. We conducted a 3D-print study involving preoperative CT scans of non-syndromic scaphocephaly patients undergoing minimally invasive-assisted remodelation (MEAR) surgery. The sagittal strip collected during surgery underwent thickness measurement, along with a 3-point bending test. These results were used to determine printing parameters for accurately replicating the skull model. Model testing simulated gravitational forces during the postoperative course and assessed lateral expansion under various wedge osteotomy conditions. The median sagittal strip thickness was 2.00mm (range 1.35-3.46mm) and significantly positively correlated (p = 0.037) with the median force (21.05 N) of the 3-point bending test. Model testing involving 40 models demonstrated that biparietal wedge osteotomies significantly reduced the force required for lateral bone shift, with a trend up to 5-cm-long cuts (p = 0.007). Additional cuts beyond this length or adding the occipital cut did not provide further significant advantage (p = 0.1643; p = 9.6381). Biparietal wedge osteotomies reduce the force needed for lateral expansion, provide circumstances for accelerated head shape correction, and potentially reduce the duration of cranial orthosis therapy.

A Photo Score For Aesthetic Outcome In Sagittal Synostosis: An ERN CRANIO Collaboration.

European Reference Network (ERN) CRANIO is focused on optimizing care for patients with rare or complex craniofacial anomalies, including craniosynostosis and/or rare ear, nose, and throat disorders. The main goal of ERN CRANIO is to collect uniform data on treatment outcomes for multicenter comparison. We aimed to develop a reproducible and reliable suture-specific photo score that can be used for cross-center comparison of phenotypical severity of sagittal synostosis and aesthetic outcome of treatment. We conducted a retrospective study among nonsyndromic sagittal synostosis patients aged <19 years. We included preoperative and postoperative photo sets from 6 ERN CRANIO centers. Photo sets included bird's eye, lateral, and anterior-posterior views. The sagittal synostosis photo score was discussed in the working group, and consensus was obtained on its contents. Interrater agreement was assessed with weighted Fleiss' Kappa and intraclass correlation coefficients.The photo score consisted of frontal bossing, elongated skull, biparietal narrowness, temporal hollowing, vertex line depression, occipital bullet, and overall phenotype. Each item was scored as normal, mild, moderate, or severe. Results from 36 scaphocephaly patients scored by 20 raters showed kappa values ranging from 0.38 [95% bootstrap CI: 0.31, 0.45] for biparietal narrowness to 0.56 [95% bootstrap CI: 0.47, 0.64] for frontal bossing. Agreement was highest for the sum score of individual items [intraclass correlation coefficients agreement 0.69 [95% CI: 0.57, 0.82]. This is the first large-scale multicenter study in which experts investigated a photo score to assess the severity of sagittal synostosis phenotypical characteristics. Agreement on phenotypical characteristics was suboptimal (fair-moderate agreement) and highest for the summed score of individual photo score items (substantial agreement), indicating that although experts interpret phenotypical characteristics differently, there is consensus on overall phenotypical severity.

Calvarial vault remodeling including pterional decompression for the treatment of nonsyndromic scaphocephaly patients: technical note and retrospective case series.

Surgical techniques to correct scaphocephaly often rely on the implantation of foreign material and/or postoperative helmet therapy and possibly result in minimal correction of frontal bossing. Moreover, foreign material and helmet therapy are associated with extra medical care and financial costs. Frontal bossing is perceived as a prominent, disfiguring feature of scaphocephaly. Herein, authors present the results of a total cranial vault remodeling technique that corrects scaphocephaly features without relying on foreign material or postoperative helmet therapy. It includes frontal release and pterional decompression, which aim to correct frontal bossing. All patients who had been operated on for isolated scaphocephaly at a single institution between January 2011 and December 2020 were included in this retrospective review. Operation time, transfusion volume, hospital stay, complications, cephalic index (CI), and bossing angle (BA) were analyzed. Sixty-five patients with nonsyndromic scaphocephaly were included in this analysis. Imaging to calculate the CI and BA preoperatively, immediately postoperatively, and 1 year postoperatively was available in 22 and 20 patients, respectively. The mean CI increased from 69.2% preoperatively to 74.6% postoperatively and 75.5% 1 year postoperatively. The mean BA decreased from 114.5° preoperatively to 111.6° postoperatively and 108.9° 1 year postoperatively. The mean operating time was 2 hours 4 minutes, and the median blood transfusion volume was 100 ml. There were no major complications or reoperations. The described total cranial vault remodeling technique is a safe procedure that mitigates total treatment burden, as no helmet therapy or implantation of foreign material is needed. It is effective in correcting CI and results in significant frontal bossing correction. The latter is attributed to a distinctive feature of the technique: frontal release and pterional decompression.

Longitudinal Three-Dimensional Follow-Up and Secondary Treatment Aspects after Endoscopic and Open Scaphocephaly Surgery.

This retrospective cohort study evaluated the longitudinal three-dimensional cranial shape developments and the secondary treatment aspects after endoscopically assisted craniosynostosis surgery (EACS) with helmet therapy and open cranial vault reconstruction (OCVR) for scaphocephaly. Longitudinally collected three-dimensional photographs from scaphocephaly patients and healthy infants were evaluated. Three-dimensional cranial shape measurements and growth maps were compared between the groups over time. Secondary treatment aspects were compared for the treatment groups. Both surgical techniques showed their strongest changes directly after surgery, with mean parietal three-dimensional growths up to 10 mm. At age 24 months, comparison of head shapes showed mean three-dimensional differences less than ±2 mm, with OCVR resulting in a lower vertex and longer cranial length when compared with EACS. At 48 months of age, no measurements were significantly different between treatment groups. Only the total head volume was somewhat larger in the male EACS group at age 48 months ( P = 0.046). Blood loss in EACS (mean, 18 mL; range, 0 to 160 mL) was lower than in OCVR (mean, 100 mL; range, 15 to 300 mL; P < 0.001). Median length of stay after surgery was shorter for EACS (mean, 2 days; range, 1 to 5 days) compared with OCVR (mean, 5 days; range, 3 to 8 days; P < 0.001). The authors conclude that EACS for scaphocephaly shows equal craniometric results at age 48 months and has a better surgery profile compared with OCVR. Early diagnostics and referral for suspected scaphocephaly to allow EACS is therefore recommended. Therapeutic, III.

Statistical shape analyses of corpus callosum changes at preoperative and postoperative scaphocephaly patients.

Scaphocephaly is the premature closure of the sagittal suture. The treatment strategies mainly focus on correcting the shape of the head, but there are very limited studies examining changes in brain structure. This study aimed to investigate shape differences in the shape of corpus callosum regarding the pre-treatment and post-treatment term at scaphocephaly patients. Cranium shape data were collected from the two-dimensional digital images. The generalized Procrustes analysis was used to obtain mean shapes in the pre- and postoperative phases. The shape deformation of the corpus callosum from the pre- to postoperative phases was evaluated using the thin plate spline method. There is an enlargement of the splenium part of corpus callosum in the late group. In the early group, corpus callosum genu and body enlargement were observed in the postoperative period compared to the preoperative period, followed by a narrowing of the isthmus region. This study showed structural deformations in the corpus callosum in scaphocephaly patients using head shape with the landmark-based geometric morphometric method by taking into consideration the topographic distribution. An enlargement at the splenium part of corpus callosum exposes after the cranial vault expansion depending on time.

Laplace-Beltrami Refined Shape Regression Applied to Neck Reconstruction for Craniosynostosis Patients

Abstract This contribution is part of a project concerning the creation of an artificial dataset comprising 3D head scans of craniosynostosis patients for a deep-learning-based classification. To conform to real data, both head and neck are required in the 3D scans. However, during patient recording, the neck is often covered by medical staff. Simply pasting an arbitrary neck leaves large gaps in the 3D mesh. We therefore use a publicly available statistical shape model (SSM) for neck reconstruction. However, most SSMs of the head are constructed using healthy subjects, so the full head reconstruction loses the craniosynostosis-specific head shape. We propose a method to recover the neck while keeping the pathological head shape intact. We propose a Laplace- Beltrami-based refinement step to deform the posterior mean shape of the full head model towards the pathological head. The artificial neck is created using the publicly available Liverpool-York-Model. We apply our method to construct artificial necks for head scans of 50 scaphocephaly patients. Our method reduces mean vertex correspondence error by approximately 1.3 mm compared to the ordinary posterior mean shape, preserves the pathological head shape, and creates a continuous transition between neck and head. The presented method showed good results for reconstructing a plausible neck to craniosynostosis patients. Easily generalized it might also be applicable to other pathological shapes.

Combining deep learning with 3D stereophotogrammetry for craniosynostosis diagnosis

Craniosynostosis is a condition in which cranial sutures fuse prematurely, causing problems in normal brain and skull growth in infants. To limit the extent of cosmetic and functional problems, swift diagnosis is needed. The goal of this study is to investigate if a deep learning algorithm is capable of correctly classifying the head shape of infants as either healthy controls, or as one of the following three craniosynostosis subtypes; scaphocephaly, trigonocephaly or anterior plagiocephaly. In order to acquire cranial shape data, 3D stereophotographs were made during routine pre-operative appointments of scaphocephaly (n = 76), trigonocephaly (n = 40) and anterior plagiocephaly (n = 27) patients. 3D Stereophotographs of healthy infants (n = 53) were made between the age of 3–6 months. The cranial shape data was sampled and a deep learning network was used to classify the cranial shape data as either: healthy control, scaphocephaly patient, trigonocephaly patient or anterior plagiocephaly patient. For the training and testing of the deep learning network, a stratified tenfold cross validation was used. During testing 195 out of 196 3D stereophotographs (99.5%) were correctly classified. This study shows that trained deep learning algorithms, based on 3D stereophotographs, can discriminate between craniosynostosis subtypes and healthy controls with high accuracy.

Statistical Shape Analyses of Patients with Scaphocephaly After Surgical Remodeling.

To investigate the differences in the head shapes of scaphocephaly patients during the pre- and postoperative phases. Cranium shape data were collected from the two-dimensional digital images. The generalised Procrustes analysis was used to obtain mean shapes in the pre- and postoperative phases. The shape deformation of the cranium from the pre- to postoperative phases was evaluated using the thin plate spline method. There was no significant cranial shape difference in the pre and postoperative phases. The high-level deformations from the pre- to postoperative phase were seen in thin plate spline graphs. The highest deformation was observed at the biparietal dimension. In this study, we showed that the structural deformation of the cranium was correlated with sagittal craniosynostosis. The present study also shows that pre- and postoperative head shapes of patients with scaphocephaly can be compared using head shape with the landmark-based geometric morphometric method by taking into consideration the topographic distribution.

Perinatal complications in patients with unisutural craniosynostosis: An international multicentre retrospective cohort study

PurposeCraniosynostosis may lead to hampered fetal head molding and birth complications. To study the interaction between single suture craniosynostosis and delivery complications, an international, multicentre, retrospective cohort study was performed. Materials and methodsAll infants born between 2006 and 2012 in the Netherlands and Sweden with sagittal or metopic suture synostosis were included. All births were included as a reference population. The primary outcome measure was rate of medically assisted labor. The secondary outcomes included method of conception, term of birth and fetal position. ResultsWe included 152 trigonocephaly patients, 272 scaphocephaly patients and 1.954.141 controls. A higher rate of assisted reproductive technology (ART) was found in patients with trigonocephaly (13%) and scaphocephaly (7%) compared to controls (3%, p < 0.001). Scaphocephaly resulted in more postterm births (8% vs 4%, p < 0.001). Trigonocephaly patients showed more preterm births (11% vs 6%, p < 0.001), breech position was more frequent (10% vs 4%, p = 0.003) and labor was more often induced. Rate of assisted delivery, including cesarean section, was significantly higher in both patient groups. ConclusionsScaphocephaly leads to more postterm births and an increased rate of cesarean sections. Trigonocephaly is related to ART, and in addition higher rates of breech position and cesarean section are found. Prenatal detection of single suture craniosynostosis could improve perinatal care.

Prenatal ultrasound parameters in single-suture craniosynostosis

Introduction: Although single-suture craniosynostosis is diagnosed sporadically during pregnancy, timely referral is critical for its treatment. Additionally, craniosynostosis leads to increased maternofetal trauma during birth. In the Netherlands, 95% of pregnant women receive a standard ultrasound at around 20 weeks of gestation, potentially an ideal setting for detecting craniosynostosis prenatally. To enhance the prenatal detection of the metopic and the sagittal suture synostosis, we wished to identify new screening parameters.Materials and methods: We retrospectively analyzed data of the 20-week anomaly scan in trigonocephaly patients (n = 41), scaphocephaly patients (n = 41), and matched controls (n = 82). We measured six different cranial dimensions, including head circumference, biparietal diameter, and occipito-frontal diameter, defining the cephalic index as the ratio between biparietal and occipito-frontal diameter.Results: Prenatal biometric measurements did not differ significantly between trigonocephaly patients and controls. Although significantly lower in scaphocephaly patients (0.76 versus 0.79; p = .000), the cephalic index by itself is not appropriate for screening at 20 weeks of gestation. Longitudinal analysis suggests that a deflection in BPD curve is found in scaphocephaly patients, starting at 20 weeks of gestation.Conclusions: Prenatal biometric measurements do not differ significantly between trigonocephaly patients and controls. The CI is lower in scaphocephaly patients. A deflection in BPD curve should be followed by 3 D imaging of the cranial sutures.

Improving the Aesthetic Outcome in Scaphocephaly Correction: Hairline Lowering During Vault Remodeling Procedures.

The bossed forehead in patients with scaphocephaly often leads to a high hairline. A new technique to improve the aesthetic outcome of patients undergoing scaphocephaly correction is described. Sixteen patients with scaphocephaly and having a high hairline due to frontal bossing who underwent scaphocephaly correction by subtotal or total vault remodeling were analyzed. The median age at surgery was 18 months. The mean distance between the nasofrontal suture and the hairline was preoperatively 70 mm (range 58-91). An obvious lowering of the hairline could be achieved in all 16 patients. The mean postoperative distance nasofrontal suture to hairline was 59 mm (range 50-73). There were no complications associated with the technique. The hairline lowering technique is a useful addition to vault remodeling techniques and can improve the postoperative aesthetic appearance considerably. The authors recommend this technique in scaphocephaly patients, who present with a high hairline.

Cranial Compression Using Distractors in Reverse Fashion as an Alternative Method for Correcting Scaphocephaly in Older Patients

Background: Intraoperative calvarial contraction of conventional calvarial remodeling is more difficult in scaphocephaly patients who are 1 year or older in age. In our current study, gradual cranial compression with a distractor was used to correct scaphocephaly in this older age group and the surgical results were quantitatively analyzed. Methods: Gradual cranial compression was used to treat 7 sagittal craniosynostosis patients. The mean age was 20.9 months (range, 12-32 months) and the mean follow-up period was 65 months (range, 3-81 months). Computed tomography was used to calculate the cephalic index (CI; equal to the maximum width of the head/maximum length of the head ×100), and the ratios of these indices at 3 different time periods (before, immediately after, and at 1 year postoperatively) were evaluated. Results: An average cranial compression of 18 mm was found to be possible with an average CI increase from 67.9 to 73.5. The CI ratio at 1 year after surgery was found to be the highest, showing a 9.8% increase. Conclusion: Sagittal craniosynostosis patients are less easy to treat with conventional calvarial remodeling surgery if they are older than 1 year. Gradual cranial vault compression with distractors can be another option in these cases.

Validation of cephalic index measurements in scaphocephaly

The cephalic index (CI) of the head can be measured manually using a caliper, the original technique, but it is also possible to determine it using skull X-ray, 2DCT and 3DCT images, 3D photo and with help of plagiocephalometry (PCM). In this study, the manual caliper determination is statistically compared with other measuring methods for scaphocephaly patients (n = 39). The CI mean differences for the most representative data are sequentially 3.74, 2.16, 1.09 and 0.97 for the 2DCT, PCM, 3D photo and 3DCT techniques. The CI 2DCT values show a significant difference (p < 0.01) in reference to CI manually, while the other techniques show a p > 0.05. The conclusions are that significantly different results are achieved when using 2DCT relative to the manual caliper determination. No significant difference is observed between the 3D techniques and the manual method.

Experience with craniosynostosis treatment using posterior cranial vault distraction osteogenesis

Background:Craniosynostosis compromises the cranial vault volume, severely impede growth, and may lead to increased intracranial pressure (ICP). Posterior cranial vault (PCV) distraction osteogenesis (DO) offers an excellent treatment opportunity for this condition. This article intends to describe the outcomes of PCV DO.Materials and Methods:Nine males and seven female children indicated for PCV DO were included in the study. The single vector distraction devices with quick-disconnect distraction rods, a type of miniaturized hardware, was used in all cases.Result:Seven of the 16 patients had a history of one or more prior cranioplasty. All reoperations in this series were performed for the indication of raised ICP including five of the scaphocephaly patients and the syndromic patients. Clinical signs of raised ICP were present in all patients with either measured raised intracranial pressure or those with clinical signs of raised ICP preoperatively. There was substantial decrease in the ICP postoperatively.Discussion:The outcomes of this study were encouraging. Placing the distractor stems as flat as possible against the outer layer of the cranial bone seems to be a very important maneuver. This keeps the distractor stem less proud and less likely to sustain future trauma. Removal of the distractor stems keeps the devices further away from the risk of later traumatic dislodgement. Moreover, miniaturized distractors allow precise control of the rate and the amount of distraction.

Papilledema in Isolated Single-Suture Craniosynostosis

The purpose of this retrospective study was to assess the prevalence of papilledema in patients with isolated craniosynostosis. Second, we wanted to assess if the presence of ventricular dilatation on computed tomography (CT) scan is a predictive factor for the occurrence of papilledema. We included 205 consecutive children with an isolated single-suture craniosynostosis, who had at least 1 fundus examination. Preoperative CT scans of the brain were examined for the presence of ventricular dilatation.Papilledema developed in 14 of 205 patients: 10 developed papilledema before surgery and 4 during the follow-up period. Ten of the patients with papilledema had a synostosis of the sagittal suture, and 4 of the metopic suture. Prevalence of papilledema in scaphocephaly was 9.7%, and in trigonocephaly, 5.6%. Based on evaluation of all CT scans, ventricular dilatation seemed not to be a predictive factor for papilledema in children with isolated craniosynostosis.The incidence of papilledema in almost 10% of scaphocephaly patients is remarkably higher than expected. Therefore, we recommend routine preoperative screening, especially for patients with scaphocephaly, but also for patients with trigonocephaly. Postoperative screening is recommended in all patients when there is any uncertainty.

Is Isolated Sagittal Synostosis an Isolated Condition?

Scaphocephaly is the most common type of craniosynostosis. In the medical literature, there is little information about the association of scaphocephaly and other congenital anomalies. To determine the prevalence of genetic anomalies in scaphocephaly patients, a retrospective review of the medical charts of 30 consecutive patients was performed. A questionnaire was sent to parents to evaluate demographics. The male-female ratio was 2:1, 22% of patients were born at less than 37 weeks gestation, and 4% of the patients were twins. Nineteen percent of mothers smoked during pregnancy, and 23% of the mothers consumed alcohol during pregnancy. Eighteen of the 30 patients visited the department of clinical genetics, whereas the other patients were examined by a pediatrician. Genetic analysis was performed on 8 patients. A genetic mutation on the FGFR-2 gene was found in 1 patient with Gorlin-Goltz syndrome. Except for the basal cell carcinomas and the sagittal synostoses, this patient had no other congenital anomalies. Two other patients had congenital malformations of the heart and kidneys. This study demonstrates the low incidence of associated congenital anomalies and scaphocephaly. Associated factors of isolated sagittal synostosis are preterm birth and male sex.