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Numerous classification systems of nonsyndromic sagittal craniosynostosis (NSC) are applied but none has gained a wide acceptance, since each classification is focused on distinct aspects. The aim of the study was to assess the accuracy of 4 classifications of NSC discussed in the literature by defining the associations among the classifications, individual features (sex, age, cranial index), and objective morphologic criteria (frontal bossing, retrocoronal constriction, sagittal ridge, and occipital bulleting). The study was conducted on anonymized thin-cut CT scans of 133 children with NSC 1 to 12 months old (mean age 5.42 mo). The type of cranial dysmorphology was assessed using 4 classification systems, focusing on skull shape, pattern of sagittal suture closure (Heuzé classification), deformation of skull vault (Sakamoto classification), and a single-dominant feature (David classification). Each patient was also independently investigated for the presence of morphologic criteria. A multivariate analysis was performed to explore the relations among the classifications and assess their accuracy. In the analyzed cohort sphenocephaly (38.3%), CFF type by Heuzé (30.8%), type I by Sakamoto (72.9%), and a central type by David (42.9%) were dominant findings. Regarding the morphologic criteria, frontal bossing was observed the most frequently (91.7%). The age of patients and cranial index differed significantly among the shapes of skull and David classifications ( P <0.001). The shape-based system showed the strongest correlation with other classifications and with measurable variables. Other classifications have much in common and some overlap, but none of them constitutes a standalone system to define all aspects of cranial dysmorphology in NSC., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Mutaz B. Habal, MD.)

Background: The prevalence of trigonocephaly has increased worldwide over the past 2 decades. Early identification and appropriate treatment are critical. The aim of this study is to evaluate the outcomes and the effect of metopic suture excision, perisutural frontal bone shave, and bilateral pericranial flap method on the shape of the forehead after surgical correction in infants with moderate trigonocephaly., Methods: The present study was performed as a cross-sectional study on 40 infants of 3 to 12 months old with trigonocephalus who underwent metopic suture excision and pericardial flap surgery in Mofid Pediatric Hospital from 2016 to 2022. The definitive diagnosis of patients' trigonocephaly was made based on clinical signs and computed tomography scan findings by a plastic surgeon., Results: Overall in 40 patients operated by this technique, 23 (57.5%) of cases were males, and 17 (42.5%) were females. The mean age of patients was 7.86 ± 2.22 months. Hospital stay was 2 to 4 days (mean: 3 d), intensive care unit admission was in 33 cases for 24 hours, and no intensive care unit admission for 7 cases. Blood was transfused during surgery for 25 patients, and 15 patients did not require blood transfusion use. Results were evaluated in 6 to 12 months after surgery by 3 independent plastic surgeons, with pre and postoperative photos. Satisfaction with the results of forehead shape was excellent for 60% of patients, good for 37.5%, and moderate for 2.5%. Only one female patient had a recurrence after the surgery., Conclusion: This study showed that the pericranial flap method after full metopic suture excision and frontal shave was very effective in the treatment of infants with moderate trigonocephaly., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Jacobs syndrome is a rare trisomy (47, XYY) found in ~1 in 1000 male children associated with infertility, autism spectrum disorders, macrocephaly, hypertelorism, tall stature, and macroorchidism. Diagnosis is often delayed due to relatively subtle phenotypic changes. Craniosynostosis, a fusion of the cranial sutures, has been described in ~1 in 2000 live births, of which 25% are related to a diagnosed syndrome with the most common being Apert and Crouzon. Craniosynostosis does not have a known association with Jacobs syndrome and no prior cases have been reported. This case report seeks to describe the presentation and treatment of a patient with Jacobs syndrome and metopic craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Purpose: The purpose of this study was to characterize a novel type of calvarial thickening and provide objective measurements of skull thickness and calvarial suture morphology in patients with bronchopulmonary dysplasia., Methods: Infants with severe bronchopulmonary dysplasia who also had undergone computed tomography (CT) scans were identified from the neonatal chronic lung disease program database. Thickness analysis was performed using Materialise Mimics., Results: The chronic lung disease team treated 319 patients during the study interval of which, 58 patients (18.2%) had head CT available. Twenty-eight (48.3%) were found to have calvarial thickening. The rate of premature suture closure in the study population was 36.2% (21 of 58 patients), with 50.0% of affected cohort having evidence of premature suture closure on the first CT scan. Multivariate logistic regression identified 2 risk factors, requiring invasive ventilation at 6 months of age and fraction of inspired oxygen requirement at 6 months of age. Increased head circumference at birth protected against the development of calvarial thickening., Conclusions: We have described a novel subset of patients with chronic lung disease of prematurity who have calvarial thickening with remarkably high rates of premature closure of cranial sutures. The exact etiology of the association is unknown. In this patient population with radiographic evidence of premature suture closure, operative decision should be made after considering unequivocal evidence of elevated intracranial pressure or dysmorphology and balanced against the risk of the procedure., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Primary craniosynostosis is a congenital craniofacial disorder in which cranial sutures prematurely close. Iatrogenic secondary stenosis is abnormal cranial suture closure caused by surgical manipulation of the suture. In contrast, idiopathic secondary stenosis develops in a suture that did not undergo surgical manipulation. The objective of this systematic review was to consolidate and characterize the incidence, classification, and management of idiopathic secondary stenosis in the literature., Methods: Literature from PubMed, Web Of Science, and EMBASE from 1970 to March 2022 was reviewed. The following information was extracted for individual patients: incidence of idiopathic secondary stenosis, index primary craniosynostosis, primary surgical correction, presenting signs of secondary stenosis, management, and further complications., Results: Seventeen articles detailing 1181 patients were included. Ninety-one developed idiopathic secondary stenosis (7.7%). Only 3 of these patients were syndromic. The most common index craniosynostosis was sagittal synostosis (83.5%). The most common suture undergoing idiopathic secondary stenosis was the coronal suture (91.2%). Patients presented at a median age of 24 months. The most common presenting sign was a radiologic finding (85.7%), although some patients presented with headache or head deformity. Only 2 patients, both syndromic, had complications following surgical correction of secondary stenosis., Conclusions: Idiopathic secondary stenosis is a rare, long-term complication following index surgical repair of craniosynostosis. It can occur following any surgical technique. It most commonly affects the coronal suture but can affect any of the sutures, including pansynostosis. Surgical correction is curative in nonsyndromic patients., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Smaller operative exposures associated with suturectomy for craniosynostosis may result in difficulties visualizing the prematurely fused suture during surgery. The authors report cases of suturectomy for lambdoid and metopic craniosynostosis in which neuronavigation or frameless stereotaxy was used to assist with incision planning and intraoperative localization of the fused suture. In both cases, neuronavigation integrated easily and safely into established workflows and was associated with complete suture release. To our knowledge, this is the first report of applying this noninvasive technology, which does not require cranial pinning or rigid fixation, to suturectomy, and the authors demonstrate its use as an adjunct, especially for surgeons beginning in practice. Larger studies are needed to determine if neuronavigation in suturectomy is associated with a clinically significant reduction in blood loss or operative time or an increase in the rate of complete suturectomy., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Unilateral lambdoid synostosis is the rarest form of single-suture craniosynostosis. Although various surgical approaches have been described, cranial vault remodeling remains the predominant approach. To aid in surgical planning, preoperative virtual surgical modeling using a patient's presenting computed tomography scan can be used to increase reconstructive precision and to reduce operative time. Presented is a 7-month-old male with unilateral lambdoid synostosis who underwent medically modeled cranial vault reconstruction., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Spring-assisted cranioplasty (SAC) for the treatment of craniosynostosis uses internal springs to produce dynamic changes in cranial shape over several months before its removal. The purpose of this study was to report the first Egyptian experiences with SAC in the treatment of children with sagittal synostosis and evaluate the preliminary outcome. A total of 17 consecutive patients with scaphocephaly underwent SAC with a midline osteotomy along the fused sagittal suture and insertion of 3 springs with bayonet-shaped ends across the opened suture. Operative time, blood transfusion requirements and length of ICU, total hospital stay, and complications graded according to Oxford protocol classification were recorded. Spring removal was performed once re-ossification of the cranial defect occurred. All patients successfully underwent SAC without significant complications. The mean age at surgery was 6.8 months. The mean time of the spring insertion surgery was 63 minutes (SD 9.7). Blood transfusion was needed in less than half of the patients (41.2%).The mean duration of hospital stay was 3.2 days. The mean timing of spring removal was 5.5 months (SD 0.4). The mean time of the second surgery (spring removal) was 22.8 minutes (SD 3.6). In conclusion, SAC can easily be incorporated into the treatment armamentarium of craniofacial surgeons. The technique offers a safe and minimally invasive option for the treatment of sagittal craniosynostosis with the benefit of limited dural undermining, minimal blood loss, operative time, anesthetic time, ICU stay, and hospital stay., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Introduction: After treating a child with familial sagittal craniosynostosis, clinocephaly, and bilateral parietomastoid/posterior squamosal suture fusion, the authors wondered if major-suture synostosis and clinocephaly were associated with abnormal fusion of minor lateral calvarial sutures., Methods: The authors reviewed all preoperative volume-rendered head computed tomography reconstructions performed for craniosynostosis at their institution from 2010 through 2014 and determined whether the sphenoparietal, squamosal, and parietomastoid sutures were open, partially fused, or fused. The authors determined whether any sutures were abnormally fused based upon a previous study from their center, in which abnormal fusion was defined as either 1 of 3 abnormal fusion patterns or abnormally-early fusion. The authors then determined the rate of abnormal fusion of these sutures and whether abnormal fusion was associated with (1) major-suture craniosynostosis, (2) type of craniosynostosis (sutures involved; single-suture versus multisuture; syndromic versus nonsyndromic), and (3) clinocephaly., Results: In 97 included children, minor lateral sutures were abnormally fused in 8, or 8.2%, which was significantly higher than in children without craniosynostosis from our earlier study. Abnormal minor lateral suture fusion was not associated with the type of single-suture synostosis or with multisuture synostosis but was associated with syndromic synostosis. Four of 8 children with abnormal minor lateral suture fusion had multisuture synostosis and 6 had syndromic synostosis. Lateral sutures were abnormally fused in 1 of 4 subjects with clinocephaly, which was not significant., Conclusion: Abnormal minor lateral calvarial suture fusion is significantly associated with major-suture craniosynostosis, especially syndromic synostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Background: Sagittal craniosynostosis may present with complete or partial fusion of the sagittal suture, but relationships between degree of sagittal suture fusion and head shape are currently poorly described. The aim of this study was to characterize sagittal suture fusion patterns and determine associations with head shape in a cohort of patients with nonsyndromic sagittal craniosynostosis., Methods: Patients with nonsyndromic sagittal craniosynostosis at a tertiary care center with available computed tomography imaging were included in this study. The anterior and posterior distances of sagittal suture patency were measured along 3-dimensional parietal bones. Degree of sagittal suture fusion was compared to head shape characteristics, including cephalic index (CI), frontal bossing, and occipital bulleting., Results: Ninety patients (69 male) were included in this retrospective study. The sagittal suture was on average 85.6±20.1% fused, and 45 (50.0%) patients demonstrated complete fusion of the sagittal suture. CI was associated with increased degree of fusion for the anterior one-half (ρ=0.26, P =0.033) and anterior one-third (ρ=0.30, P =0.012) of the sagittal suture. Complete fusion of the anterior one-third of the sagittal suture predicted higher CI (β=13.86, SE=6.99, z =-0.25, P =0.047). Total degree of sagittal suture fusion was not predictive of CI or head shape in any analysis., Conclusions: Decreased fusion of the anterior one-third of the sagittal suture, but not total suture, may paradoxically predict increased severity of scaphocephaly as quantified by CI in nonsyndromic sagittal craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: This paper aims to describe a new technique of 1-piece frontoorbital osteotomy with a small supraorbital burr hole, which resulted in improved safety during osteotomy of the anterior skull base. An 11 × 4 mm burr hole was created at the center of the orbit above the superior orbital rim using an "eyebrow key-window approach." Extradural dissection from the nasion to the medial side of the sphenoid ridge was performed through this hole, and dissection of the lateral side of the sphenoid ridge was completed from the edge of the coronal osteotomy. Osteotomy along the zygomaticofrontal and sphenofrontal sutures were performed using an oscillating saw with secure protection of the dura. Between December 2019 and June 2020, 3 patients with unilateral coronal craniosynostosis underwent 1-piece frontoorbital advancement with the eyebrow key-window approach. The median age was 11 months (8-15 months), median operation time was 249 minutes (244-255 minutes), and the average blood transfusion was 10.8 ml/kg (0-18 ml/kg). No complications were observed, and the technique was associated with improved safety of osteotomy around the anterior skull base for 1-piece frontoorbital advancement., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: Endoscopic suturectomy is a minimally invasive surgical treatment for single-suture craniosynostosis in children between 1 and 4 months of age. This study sought to characterize the role played by diagnostic imaging in facilitating early surgical management with endoscopic suturectomy. The authors also characterized the overall diagnostic utility of imaging in patients assessed for abnormal head shape at their institution, regardless of surgical status. A retrospective cohort of children diagnosed with singlesuture synostosis undergoing either primary endoscopic suturectomy or open calvarial reconstruction at the authors' institution from 1998 to 2018 was first reviewed. Of 132 surgical patients, 53 underwent endoscopic suturectomy and 79 underwent open repair. There was no difference in the proportion of endoscopic and open surgery patients imaged preoperatively before (24.5% versus 35.4%; P = 0.24) or after (28.3% versus 25.3%; P = 0.84) craniofacial assessment. Stratifying by historical epoch (1998-2010 versus 2011-2018), there was also no difference found between preoperative imaging rates (63.6% versus 56.4%; P = 0.35). In another cohort of 175 patients assessed for abnormal head shape, 26.9% were imaged to rule out craniosynostosis. Positive diagnostic imaging rates were recorded for suspected unicoronal (100%), metopic (87.5%), lambdoidal (75.0%), sagittal (63.5%), multisuture (50%), and bicoronal (0%) synostosis. The authors conclude that the use of diagnostic imaging at their institution has not increased despite higher utilization of endoscopic suturectomy and need for expedient identification of surgical candidates.However, their results suggest that imaging may play a greater diagnostic role for suspected bicoronal, sagittal, and multi-sutural synostosis among sutural subtypes of synostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: The posterior intraoccipital synchondrosis (PIOS) is a cartilaginous division separating the exoccipital and supraoccipital bones, allowing for flexibility of the cranial base at birth and which later ossifies in adolescence. The authors report a case of right PIOS synostosis that resembled right lambdoid synostosis, with left occipital bossing in a healthy, six-month-old female with an unremarkable birth history and no antecedent trauma. An initial referral was made from the pediatrician to oncology because of a concern over the presence and growth of a possible mass at the left occiput. Over 8 months, this mass grew and became more firm, accompanied by left occipital bossing and right occipital flattening. A computed tomography was obtained, which demonstrated the fusion of the PIOS, prompting a referral to plastic surgery. Cranial vault remodeling with switch cranioplasty was performed at age 14 months, complicated only by a superficial infection along the suture line. There are exceedingly few reports of PIOS synostosis, with occipital osteodiastasis related to birth trauma as the only prior explanation for this condition. With no birth trauma and delayed onset, our case likely represents idiopathic PIOS synostosis. With the many similarities in presentation, the surgical approaches commonly used for lambdoid synostosis, particularly switch cranioplasty, are suitable solutions to PIO synchondrosis with optimal functional and aesthetic outcomes., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: Scaphocephaly is the morphological consequence of premature sagittal suture fusion. Morphologic and morphometric studies on the cranial vault in scaphocephaly flourish in the literature. However, few studies are available on the cranial base in a scaphocephalic population, let alone the morphometry of its fossae. Therefore, this study aimed to analyze and compare the morphometry of the anterior, middle, and posterior cranial fossae (ACF, MCF, and PCF) in patients with scaphocephaly.The length and width of the ACF, MCF, and PCF were measured using fixed anatomical landmarks on the two-dimensional computed tomography scans of 24 consecutive patients diagnosed with isolated sagittal synostosis between 2014 and 2020, and 14 controls.A comparison of the results between patients with scaphocephaly and the controls showed that the ACF and PCF lengths increased significantly ( P = 0.041 and P = 0.018) in patients with scaphocephaly. No differences in the MCF lengths were observed ( P = 0.278; 0.774). When compared by the degree of severity, the ACF and PCF lengths were significantly increased ( analysis of variance [ANOVAI, P = 0.033; post-hoc, P = 0.013 and ANOVA, P = 0.015; post-hoc, P = 0.036) in scaphocephalic patients within the severe group as opposed to the control group.The morphometric data obtained indicate a preponderance of deformity in the ACF and PCF with elongation along the anteroposterior plane (lengths) in scaphocephalic patients. Minimal changes were observed in the transverse plane (widths) in scaphocephaly versus controls. This data could aid craniofacial surgeons in understanding the affectation of the cranial fossae and influencing the decision on the most suitable method of corrective modality., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Objective: Historically, studies have shown that cranial remolding therapy improves surgical correction and protects against regression for patients with sagittal suture craniosynostosis. This study aimed to define the most responsive cranial height for measuring cephalic index (CI) following cranial remolding therapy for infants with sagittal suture craniosynostosis., Methods: The authors performed a retrospective analysis of data between January 2018 and August 2019. The outcomes measured were CI-3 (level of glabella) through CI-7 (superior to eurions), where each value was defined as the width at levels 3 through 7 divided by the length at level 3. Differences between baseline- and post-treatment measurements were assessed using a 5 × 2 repeated measures analysis of variance., Results: Data from thirty-four patients (19 males, 15 females, and mean age 2.79 months) were analyzed. Mean treatment duration was 4.59 ± 1.86 months. There was a significant increase between baseline and posttreatment measurements (baseline: 72.60% ± 0.70%, post: 76.30% ± 0.80%; F1,33 = 27.74, P < 0.001). The interaction effect for CI level ∗ baseline-post was also significant (F1.43,47.16 = 6.75, P = 0.006). Post hoc analyses revealed the posttreatment measures were significantly greater than baseline measures at every CI level. The magnitude of the measured differences systematically decreased from CI-7 to CI-3, with a greater effect size at the most superior level (ie CI-7) of 0.961 compared to 0.778 at the traditional level (ie CI-3)., Conclusions: The CI measurement at level 7 demonstrated the greatest responsiveness to treatment, whereas the traditional CI measurement taken at level 3, the current standard, proved the least responsive., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Background: Minimally-invasive endoscopic strip-craniectomy (or suturectomy) for the repair of craniosynostosis combined with postoperative cranial orthotic molding has been widely adopted in the past 2 decades, proving itself as a safe and effective procedure. Over time the authors transitioned from performing an endoscopic strip-craniectomy, to performing the same surgery without the endoscope. The authors here describe our technique and compare its results to those published in the literature for endoscopic suturectomies., Methods: A retrospective chart review was performed for patients with nonsyndromic craniosynostosis who underwent minimally-invasive nonendoscopic suturectomy between 2019 and 2020 at our institution., Results: Thirteen patients (11 males; 2 females) were operated including 5 Metopic, 5 Sagittal, 2 coronal, and 1 lambdoid craniosynostosis. The average age at surgery was 4.35 months. The average length of surgery was 71 minutes. Averaged intraoperative estimated blood loss was 31.54 mL. Eleven patients received a blood transfusion (most before performing the skin incision) with a mean amount of 94.62 mL of blood transfused during surgery. The mean hemoglobin at discharge was 10.38 mg/dL. There was only 1 intraoperative mild complication. The mean intrahospital length of stay was 1.77 days with no postoperative complications noted. All patients initiated remodeling orthotic treatment following surgery. Long-term follow-up scans were available for 8 patients (5 metopic, 2 sagittal, and 1 lambdoid) with an average follow-up of 9 months. In all cases, there was a significant improvement in the skull width at the synostosis location as well as in the skull proportions and symmetry. The above outcomes are similar to those published in the literature for endoscope-assisted strip-craniectomies., Conclusions: Suturectomies assisted with cranial orthosis remodeling for the treatment of all types of nonsyndromic craniosynostosis can be performed without an endoscope while maintaining minimal-invasiveness, good surgical results, and low complication rates., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Abstract: Sagittal craniosynostosis results in scaphocephaly from abnormal fusion of the sagittal suture. Traditionally, craniosynostosis presents at birth and is diagnosed within the first year of life. The authors report a patient with development of sagittal craniosynostosis after birth, which we term postnatal sagittal craniosynostosis. This is a rare occurrence in which management considerations are critical but are not well discussed. A 3-year-old boy presented with concerns of a metopic ridge. Workup revealed metopic ridging and an open sagittal suture. The patient later developed signs of increased intracranial pressure and repeat computed tomography scan 14 months later identified a newly fused sagittal suture. The patient underwent open posterior cranial vault expansion, resulting in resolution of symptoms. Postnatal sagittal craniosynostosis is a rare condition and should be considered in otherwise unexplained increases in increased intracranial pressure among pediatric patients. Open posterior cranial vault expansion represents a safe and effective method to treat this condition., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Background: Various surgical methods are used for early treatment of nonsyndromic sagittal craniosynostosis. The craniofacial centers in Uppsala and Helsinki fundamentally both use the H-Craniectomy: Renier's technique. However, the Helsinki group systematically adds coronal suturectomies to prevent secondary coronal synostosis and posterior barrel staves to address posterior bulleting. The effects of these additions in early treatment of sagittal craniosynostosis are currently unknown., Methods: Thirty-six patients from Uppsala and 27 patients from Helsinki were included in the study. Clinical data and computed tomography scans were retrieved for all patients., Results: The Helsinki patients had a smaller preoperative Cranial index (CI) (65 vs 72) and a smaller preoperative width (10.1 vs 11.2). There was no difference in postoperative CI, corresponding to a difference in change in CI. Regression analysis indicated that the larger change in CI in the Helsinki group was mainly due to a lower preoperative CI allowing for a larger normalization. The Helsinki patients had less growth in length (1.5 vs 2.1 cm) and more growth in width (2.3 vs 1.9 cm). There were no differences in head circumference or surgical complications. Secondary coronal synostosis was present in 43% of the Uppsala group at 3 years of age, while calvarial defects located at sites of previous coronal suturectomies and posterior barrel staving were seen in the Helsinki group 1 year postoperatively., Conclusions: Lower preoperative CI appears to be the main factor in determining the amount of normalization in CI. Prophylactic coronal suturectomies do not seem to benefit preservation of coronal growth function since the modification correlates to less sagittal growth and more growth in width., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Abstract: Combined sagittal and metopic craniosynostosis is an uncommon condition that may result in a wide spectrum of cranial morphologies. This occurs as a result of the compound effects of both prematurely fused sutures. The authors present an incidence of sagittal craniosynostosis with a specific altered head morphology, and delayed diagnosis that the authors believe occurred due to structural changes associated with concurrent metopic suture synostosis This patient underscores the limitations of relying on classic diagnostic patterns and highlights the critical role of noninvasive imaging in the diagnosis of craniosynostosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Background: It is essential to localize the central sulcus in patients with lesions within or nearby the sensorial and/or motor cortex. The coronal suture is a valuable bony landmark in neurosurgical practice; it could be used to localize the central sulcus. There are scarce amount of literature about normal values of the distance between the central sulcus and the coronal suture. In the present study, the authors aimed to learn normative values of the distance between the central sulcus and the coronal suture in a patient sample representing Turkish population. The authors also aimed to look for any difference in values according to sex and age., Methods: The authors retrospectively reviewed a prospectively collected database. Patients were evaluated on cranial computed tomography (CT) reformatted in 3 planes (axial, coronal, and sagittal). Intracranial and extracranial pathologies were scanned. If there was no pathology, the reviewed CT scan was added up to the database. The coronal suture and the central sulcus were identified at the midline location on axial and sagittal view CT images. Vertical distance between coronal suture and central sulcus was measured., Results: Mean distance of the central sulcus to the coronal suture was 47.5 ± 7.6 mm (range = 26.2-67.3 mm)., Conclusions: Identifying the central sulcus relative to the coronal suture is essential to preserve the primary motor and/or sensory cortices in neurosurgical procedures. The distance of the central sulcus to the coronal suture is approximately 4.7 cm in adult patients from Turkey, which did not differ according to age or sex., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Abstract: Accessory cranial sutures have been described in the literature and are most commonly associated with the parietal bone. These sutures are typically identified incidentally and there have been no reported cases of accessory cranial sutures leading to abnormal head shape.The authors present the case of a 3-month-old patient with multiple congenital anomalies and an accessory parietal suture leading to abnormal head shape. The patient was successfully treated with cranial orthotic therapy. To our knowledge, this is the first reported case of an accessory cranial suture leading to abnormal head shape., Competing Interests: The authors report no conflicts of interest, (Copyright © 2020 by Mutaz B. Habal, MD.)

Abstract: Rapid maxillary expansion (RME) is an effective orthopedic procedure to correct maxillary transversal deficiency in young patients. In cases of sutural closure, surgically assisted RME is used to reduce the resistance to sutural disjunction. Foundation of a diagnostic relationship between maturation indices and the expected skeletal response to expansion can enable clinicians to identify the need for surgically assisted RME prospectively. The primary aim of this study was to assess the correlation between the amount of achieved skeletal expansion with midpalatal suture density on computed tomography images, and the secondary aim was to assess the correlation between the amount of achieved skeletal expansion with the recently suggested midpalatal suture maturation indicators: cervical vertebral maturation (CVS), midpalatal suture maturation stage (MPSM) and midpalatal suture density ratio. Forty Digital Imaging and Communications in Medicine data from the archived computed tomography scans of 20 patients (mean age: 15.55 years) before RME (T1) and after 3-month retention period (T2) were analyzed. The tested maturity indicators did not show statistically significant correlation between the skeletal effect proportions. However, clinically and radiologically effective RME was seen in MPSMs A, B, and C and also cervical vertebra stages CVS5 and CVS6. More research is needed to determine a diagnostic predictor for the skeletal effects of maxillary expansion treatment modalities., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Abstract: Craniosynostosis, a deformity of the skull caused by premature fusion of ≥1 cranial sutures, is treated surgically via endoscopic approaches or cranial vault remodeling. Postoperative infection is rare. Management of postoperative surgical site infections often involves culture-directed intravenous antibiotics and debridement, with removal of osteomyelitic bone and hardware in refractory cases. Removal of autologous bone in a pediatric patient presents a reconstructive challenge, as alloplastic options are not optimal in a growing child, especially in the setting of infection. Moreover, infants and small children have limited autologous bone options for reconstruction. We present our case of a young child who developed an infectious complication following cranial vault remodeling. The patient's demographic information, clinical presentation and postoperative course, radiologic features, surgical interventions, and treatment outcomes were reviewed. In our case, autologous osteomyelitic bone underwent tissue processing to eradicate the infection and complete skull reconstruction using the patient's own processed autologous bone was performed in a delayed fashion. The patient is now 1 year postoperative with no recurrence of infection. We present this case as a novel technique to eradicate infection in autologous bone, allowing for delayed autologous cranial reconstruction., (Copyright © 2020 by Mutaz B. Habal, MD.)

Abstract: Craniosynostosis is a condition where the cranial sutures are early fused. Sagittal suture synostosis is found to be the most prevalent. Many techniques ranging from simple suture excision to wide suturectomy have been developed for treating this condition. While repeated fusion of previously excised involved sutures is common, neosuture formation has been identified in many recent reports after craniosynostosis surgery. In this case report, the authors present a finding of the neosuture formation in a patient presented with sagittal craniosynostosis after wide suturectomy with total cranial vault remodeling so that the pathologically fused suture can be reversed., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Competing Interests: The authors have no conflicts of interest to disclose.

Abstract: Secondary Coronal Synostosis (SCS) in patients operated for non-syndromic Sagittal Craniosynostosis is a postoperative phenomenon with unclear implications. The aim of this study was to investigate whether SCS is a negative or a benign occurrence in the postoperative course. The authors hypothesized that SCS is related to reduced cranial growth and intracranial hypertension. Thirty-one patients operated for SC at an early age with the H-craniectomy technique were included in the study. Associations between SCS and cranial shape, growth, and signs of intracranial hypertension were analyzed. Intracranial volume distribution was assessed by measuring partial intracranial volumes defined by skull base landmarks. A total of 12/31 patients developed SCS during the postoperative course. The presence of SCS was associated with a higher prevalence of gyral impressions and a larger normalization of Cranial Index due to less growth in the anteroposterior plane. The SCS group had a smaller postoperative intracranial volume due to less posterior intracranial volume as well as less growth in head circumference. Whether this is a growth restriction caused by the SCS or a secondary effect of less primary brain growth remains to be determined. However, the correlation between SCS, less cranial growth and gyral impressions does imply that SCS should be taken into consideration during clinical follow-up as a potentially adverse event., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Background: Craniosynostosis treatment modalities have changed over time. These have included open calvarial remodeling, suturectomy with helmet molding, hand-powered distraction devices, and spring-mediated distraction. Implantable springs were first described for their use in treatment of craniosynostosis in 1998 (Lauritzen et al, Plast Reconstr Surg 121;2008:545-554). They have been used for the correction of craniosynostosis involving single and multiple sutures and have been placed through both endoscopic and open approaches. Their use for correction of lambdoid synostosis has been previously only described using an open approach (Arnaud et al, Child Nerv Syst 28;2012:1545-1549). The senior author has performed spring-mediated distraction for treating unilambdoid craniosynostosis using an endoscopic approach, which is described below and has not previously been reported by other authors., Methods: A retrospective analysis of our series of endoscopic unilambdoid synostosis repairs is included in this article. Patients were analyzed based on patient characteristics, operative details, and outcomes. The operation commences by approaching the lambdoid suture endoscopically through a 2- to 3-cm incision lateral to the lambdoid suture. Burr holes are placed on either side of the suture and a suturectomy is performed. Springs are bent preoperatively to a predetermined force. Two springs are placed across the suturectomy defect and the skin is closed. The patient is monitored for improvement in head shape and cranial X-rays are performed to measure the degree of distraction., Results: Seven patients underwent endoscopic spring-mediated distraction for unilambdoid craniosynostosis. The average age at the time of operation was 9.4 months. The median force of each spring placed was 7.0 N. The median length of hospital stay after spring placement was 2 days. Springs were removed at 5.6 months on average. Five patients had X-rays immediately after placement and prior to removal. Each spring expanded an average distance of 15.3 mm. There were no surgical complications. Three patients had both preoperative and postoperative computed tomography scans available. The angle of the cranial base, calculated by comparing foramen magnum to cribriform plate angles, improved 5.8° (12.3 preoperatively to 6.6 postoperatively)., Conclusion: Endoscopic spring-mediated distraction is a safe and effective method of treatment for unilambdoid craniosynostosis. The series represents the largest experience with this technique. The approach can be considered in all patients with unilambdoid synostosis given the efficacious improvement in vault remodeling, low patient morbidity, short operating time, and minimal inpatient stay.

Sagittal craniosynostosis is the most common of all craniosynostoses. Patients with sagittal craniosynostosis exhibit a typical cranial appearance, including scaphocephaly, and an elongated head, with a fused and ridged sagittal suture. Moreover, some recent reports described atypical sagittal craniosynostosis accompanied by autism, speech delay, and hyperactivity. This condition, known as delayed-onset craniosynostosis, is rarely reported, given that it is difficult to determine if the condition is congenital or has a delayed-onset.This report describes the clinical course and treatment of 2 brothers with atypical sagittal synostosis. The shapes of their heads were not indicative of scaphocephaly and the younger brother exhibited delayed-onset sagittal synostosis. Their father and paternal grandmother exhibited similar cranial morphologies. Therefore, we hypothesized the involvement of a familial factor in the etiology of atypical sagittal synostosis in these patients.

Object: Distraction osteogenesis (DO) may allow for maximal volumetric expansion in the posterior vault (PV) by overcoming viscoelastic forces of overlying soft tissues. Little evidence exists regarding surgical planning and anticipated 3D volumetric changes pre- and post-operatively. We aim to study the volumetric changes occurring in PV distraction in lambdoid craniosynostosis., Methods: From 2007 to 2019, a single institution retrospective review revealed 232 craniosynostosis patients. Fourteen demonstrated lambdoid synostosis (6%), and of those, 11 patients were included in the study due to treatment with PVDO or representative sample. Six patients had unilateral synostosis and 5 had bilateral synostosis. Imaging protocol for PVDO patients included preoperative head CT within 1 month of surgery and 8 weeks following distraction cessation with weekly skull plain films. 3D volumetric analyses were performed on pre and postoperative head CT using 3D Slicer software., Results: Posterior fossa volume (PFV) increased by 38.7% and foramen magnum area increased by 26.9% postoperatively. Unilateral lambdoid craniosynostosis patients had greater increases in PFV versus bilateral lambdoid craniosynostosis patients (63.5% versus 8.9%, P = 0.007). Osteotomy to the asterion was more effective in increasing PFV versus osteotomy to foramen magnum (P = 0.050). Placement of distractor in the inferior third of the lambdoid suture is more effective in increasing PFV versus placement in the middle or top third of the suture (P = 0.041)., Conclusions: Highest volumetric increases are seen in unilateral lambdoid synostosis. Extending osteotomy beyond the asterion is not necessary for maximal PV volumetric gain. Placement of distractor in the inferior third of the suture leads to maximal PV volumetric gains.

Purpose: How different from "normal" are the cranial base and vault of infants with nonsyndromic, single-suture sagittal synostosis (NSSS)? This study quantitatively addresses this question utilizing computed tomography (CT) analytic technology., Method: Head CT scans of infants with NSSS and normocephalic controls were analyzed using Mimics to calculate craniometric angles, distances, and segmented volumes. Craniometric measurements and asymmetry indices were compared between NSSS and control groups using linear regressions controlling for age. Ratios of anterior-, middle-, and posterior-to-total cranial vault volume were compared between groups using beta regressions controlling for age., Results: Seventeen patients with NSSS and 19 controls were identified. Cranial index and interoccipital angle were significantly smaller in NSSS compared with controls (P = 0.003 and <0.001, respectively). Right-but not left-external acoustic meatus angle and internal acoustic meatus-to-midline distance were significantly greater in NSSS than in controls (P = 0.021 and 0.016, respectively). NSSS patients and controls did not significantly differ in any asymmetry indices, except for the articular fossa angle asymmetry index (P = 0.016). Anterior vault volume proportion was greater in NSSS relative to controls (proportion ratio = 1.63, P < 0.001). NSSS trended toward a smaller posterior vault volume proportion (P = 0.068) yet did not differ in middle vault volume proportion compared with controls., Conclusion: In this small study, patients with nonsyndromic, single-suture sagittal craniosynostosis had relatively similar cranial base measurements, and larger anterior vault volumes, when compared with controls. Further work is needed to confirm the possibility of rightward asymmetry of the anterior cranial base.

Background: Craniosynostosis isolated to the frontosphenoidal suture is an extremely rare entity, distinct from other types of synostotic anterior plagiocephaly, from the embryologic, phenotypic and endocranial morphology viewpoints. Embryologically, the sphenoid bone has two origins, which morphogenetically represent 2 distinct subunits. Depending on the region involved, unique craniofacial features involving the forehead, temporal region and orbit will be demonstrated. A case of frontosphenoidal suture synostosis depicting these features is presented. In addition, a literature review was performed and a treatment algorithm is proposed.

Background: Patients with Crouzon syndrome develop various types of anatomic deformities due to different forms of craniosynostosis, yet they have similar craniofacial characteristics. However, exact homology is not evident. Different pathology then may be best treated by different forms of surgical technique. Therefore, precise classification of Crouzon syndrome, based on individual patterns of cranial suture involvement is needed., Methods: Ninety-five computed tomography (CT) scans (Crouzon, n = 33; control, n = 62) were included in this study. All the CT scans are divided into 4 types based on premature closure of sutures: class I = coronal and lambdoidal synostosis; class II = sagittal synostosis; class III = pansynostosis; and class IV = "Others." The CT scan anatomy was measured by Materialise software., Results: The class III, pansynostosis, is the most prevalent (63.6%). The classes I, III, and IV of Crouzon have significantly shortened entire anteroposterior cranial base length, with the shortest base length in class III. The external cranial measurements in class I show primarily a decreased posterior facial skeleton, while the class III presented with holistic facial skeleton reduction. Class II has the least severe craniofacial malformations, while class III had the most severe., Conclusion: The morphology of patients with Crouzon syndrome is not identical in both cranial base and facial characteristics, especially when they associated with different subtypes of cranial suture synostosis. The classification of Crouzon syndrome proposed in this study, summarizes the differences among each subgroup of craniosynostosis suture involvement, which, theoretically, may ultimately influence both the timing and type of surgical intervention.

Nonsyndromic craniosynostosis results in premature fusion of cranial sutures and is rarely found in the context of other craniofacial abnormalities. Here the authors present the case of a 3-month-old male infant with a rare presentation of sagittal craniosynostosis and concomitant calcified cephalohematoma repaired by endoscopic-assisted sagittal strip craniectomy with good cosmetic and functional outcomes. The authors discuss the advantages of endoscopic repair of craniosynostosis when found in the presence of a cephalohematoma and the need for further research to investigate a possible causal relationship between these 2 pathologies.

Objective: Severe hydrocephalic macrocephaly can cause significant morbidity in infants due to positioning difficulties, skin breakdown, and poor cosmesis. Many surgeons over the past decades have described a variety of surgical techniques of reduction cranioplasty. In this article, the authors describe a novel technique for skull reduction cranioplasty with modified bilateral Pi craniectomy., Methods: Anterior coronal and posterior lambdoid bone cuts are performed to extend inferiorly toward the sqamous bone. Last bone cuts are made connecting the parasagittal burr holes bilaterally, thus isolating and de-roofing of the sagittal suture. Bilateral peninsular bone flaps are reduced medially aiming to approximate the upper borders of both flaps. Edges of frontal and occipital bone can be resected and shaved to achieve smooth round contour of the reconstruct. The final reconstruct can be fixated with metal meshes., Results: In our technique, modified bilateral Pi craniectomy is simple and effective reduction cranioplasty technique, as the technique does not require bone graft resection and reconstruction of the grafts making the time of the surgery short with decreased possible complications. Also, the amount of bone resection, bone bending, and shaving of the bone edges can be controlled and adjusted during the. An important issue is that this technique avoids posterior skull reconstruction which is more risky, as many patients are bed ridden with secondary compressed and flat occiput., Conclusions: Modified bilateral Pi craniectomy is a simple and effective technique for cranial vault reduction, especially in flat-occiput cases. Good understanding of the structural abnormality and the pathophysiological mechanisms of the possible complications is very important for performing proper surgical reconstruction.

Introduction: Proper temporalis resuspension following craniotomy or craniectomy is necessary to prevent significant temporofacial deformity. Several methods of temporalis reconstruction have been described with varying success; currently there are no reports of suture anchor utilization., Methods: A patient is presented displaying successful temporalis resuspension using suture anchor fixation. An incision was made in the temporal hair-bearing scalp to access and lengthen the retracted temporalis under direct visualization. Stab incisions were then made in the scalp to expose the superior temporal line, where suture anchors were placed for muscle fixation. After confirming appropriate vectors for muscle resuspension, 1 suture arm was passed through a subgaleal tunnel to capture the temporalis and the other was fixated to the temporoparietal skull. The suture anchors were secured and the incisions were closed in layers., Results: A 36-year-old female with history of decompressive craniectomy for hemorrhagic stroke presented with significant temporal hollowing. Her temporalis was retracted with a noticeable defect on frontal view and bulging over the zygomatic arch. The patient underwent temporalis resuspension as described with durable correction resembling her premorbid state. The buried suture anchors were nonpalpable., Conclusion: Temporal hollowing after craniotomy is a difficult contour deformity to correct. In the presented patient, reconstruction with temporalis elevation and suture anchor resuspension was found to be an effective technique with excellent cosmetic outcome. The efficiency of suture anchor placement, postoperative maintenance of muscle tension, and nonpalpable profile of the buried suture anchors suggest comparable efficacy to plate fixation and suture-only techniques.

Background: The morphologic effect of squamosal synostosis has not been clarified, due to its low occurrence rate, and its inclination to be combined with premature closure of other major sutures, especially in syndromic synostosis. This study's goal is to explore the morphological influence of squamosal synostosis in both syndromic and nonsyndromic craniosynostosis patients., Methods: Twenty-five computed tomography (CT) scans from nonsyndromic squamosal synostosis (NSS), Crouzon syndrome with squamosal synostosis (CSS), Crouzon syndrome without squamosal synostosis, and normal controls were included. Three-dimensional (3D) cephalometry, entire cranial volume and cranial fossa volume were measured using Materialise software., Results: The entire cranial volume of all groups was similar. The NSS developed a gradual reduction in the severity of reduced segmental volume from the anterior cranial fossa, to the middle cranial fossa, and to the unaffected volume of posterior cranial fossa. The CSS developed the most severe volume reduction of middle cranial fossa (12608.30 ± 2408.61 mm vs 26077.96 ± 4465.74 mm, 52%), with proportionate volume reduction of the anterior (7312.21 ± 2435.97 mm vs 10520.63 ± 2400.43 mm, 30%) and posterior cranial fossa (33487.29 ± 5598.93 mm vs 48325.04 ± 14700.44 mm, 31%). The NSS developed a narrower anterior cranial fossa, a clockwise rotated Frankfort horizontal plane, and a retracted chin (pogonion)., Conclusion: Squamosal synostosis may alter the development of cranial fossa volume, especially the volume of middle cranial fossa. However, the development of cranial base, and related facial features are determined largely by major vault sutures and associated syndromic conditions, rather than the squamosal suture alone., Level of Evidence: II.

The formation of the cranial sutures, in utero, occurs when the ossification of the skull bones reaches predestined positions around gestational week 15 to 20. Craniosynostosis, and the consequent skull shape deformities, is treated with surgery including osteotomies of the fused sutures. The occasional appearance of a new suture in the osteotomy lines has previously been described as sporadic events. In this retrospective study, a 4-year consecutive series of osteotomies combined with springs for craniosynostosis were systematically analysed regarding the appearance of neosutures. In total, 84 patients were included and in 16 patients (19%) a new radiologically normal suture appeared in a part of the suture that was completely closed preoperatively. Additionally, in 7 patients (8%) a new suture appeared in a part of the suture that had a discernible suture prior to surgery.In conclusion, in this consecutive and well-defined patient cohort operated for craniosynostosis, the formation of a neosuture is not a rare, and speculatively not a random, event. The appearance of a new suture long after the normal time period for suture formation in utero indicates that the craniosynostosis may just as well be caused by disturbed formation of the suture as actual premature closure.

Simple craniosynostosis is a cranial disease characterized by the premature closure of the cranial sutures, it develops during the first years of life and affects 1 in every 2000 to 2500 births worldwide (1). The cranial growth alteration occurs as parallel flattening to the compromised sutured with compensatory bulging in a perpendicular vector. Currently, The Suturectomy is the gold validated surgical treatment, that besides the dynamic Cranioplasties of multiples revolutions allows the design of bone flaps and therefore the correction of the secondary deformities caused by the synostosis. This multicenter descriptive study assessed a 20 series of cases (6 Plagiocephaly, 13 Scaphocephaly, 1 Brachycephaly) obtained in Cali, Colombia, that underwent surgery between January of 2014 and December of 2017, applying a Suturectomy surgery with additional telescoping of multiple revolution cranial osteotomies. The authors observe no clinical complications in the recruited patients regarding postoperative period of the described surgical technique (1, 90, and 180 days), thereby obtaining excellent outcomes on the maintained suture distraction focused on the assessment of the 3D reconstruction computed tomography scans.

Background: Minimally invasive approaches for the treatment of single-suture craniosynostosis are sometimes touted as equivalent to cranial vault reconstruction. While techniques for sagittal synostosis have been reviewed previously, evidence regarding open and less invasive surgical techniques for metopic, coronal, and lambdoid synostosis has yet to be reviewed., Methods: Systematic searches were performed using Embase.com and PubMed. Included studies reported short- or long-term outcomes, compared at least 2 standard techniques, discussed single-suture coronal, metopic, or lambdoid craniosynostosis, and enrolled at least 20 study participants. Two authors screened titles and abstracts, and also performed full text review and data extraction. Given heterogeneous outcomes, qualitative synthesis was performed after data extraction., Results: The search strategy yielded 2348 articles. Of these, 313 were removed as duplicates, and 1935 were excluded during title/abstract review. After full text review of 100 articles, 19 were selected for data extraction. The heterogeneity of outcomes precluded meta-analysis and required qualitative synthesis. While short-term outcomes indicated decreased morbidity of minimally invasive techniques, only 2 articles presented long-term reoperation rates. One study reported higher reoperation rates in the less invasive technique, and the second reported no reoperations in the median follow-up period of 33 months., Conclusion: Studies comparing long-term outcomes between different surgical techniques for single-suture craniosynostosis remain deficient. The development of standardized outcome measures is essential, and prospective, multicenter studies are necessary to assess the long-term efficacy of these procedures.

Background: Bilateral coronal craniosynostosis is the premature fusion of both coronal sutures. Traditionally, this condition is treated by frontal-orbital advancement (FOA). Endoscopic strip craniectomy with cranial orthotic therapy, which has gained popularity in treating single suture craniosynostosis, has recently been adapted for the treatment of bicoronal synostosis. There have been few studies documenting the outcomes of this treatment. The objective of this study is to compare the morphological outcomes of endoscopic strip craniectomy and FOA in patients with bicoronal synostosis., Methods: A retrospective case series was done on 24 patients with bilateral coronal synostosis treated with endoscopic strip craniectomy or FOA at 2 institutions. Patients with preoperative and 1-year postoperative computed tomography scans were included. Multiple measures of cranial shape and size were assessed: cephalic index, turricephaly index, basofrontal angle, circumference Z-score, and cranial vault volume Z-score., Results: The 2 groups were similar statistically in terms of morphology at preoperative scan. There were no statistical differences between the FOA group and endoscopic group at postoperative scan in any of the morphologic outcomes measured. Cranial vault volume Z-scores increased postoperatively in both the endoscopic (P < 0.001) and FOA (P = 0.034) groups., Conclusions: One year after repair there were no significant morphological difference between patients with bicoronal synostosis treated with the endoscopic approach and those treated by FOA.

The aim of the surgery for craniosynostosis is to release increased intracranial pressure and to normalize cranial shape. The procedure has developed so far in Japan, from a simple strip craniectomy before 1960s through a total calvarial remodeling after 1970s and later methods of 1990s, such as distraction and its modifications applying to the posterior cranium. Since a distracter needs certain hardness and thickness of the bone, the surgery has to be stood by until 5 to 6 months of age. Modified Jimenez procedure, as a latest option, were applied for very early years of 10 patients with several types of craniosynostosis, in our team in the past 3 years. The experience was small with short follow-up; the results are all good in those volume change and shape. The procedure proved to be another alternative especially prior to posterior distraction of syndromic patients in terms of positive and expedited control in an earlier surgical management.

Background: Previously, the authors developed a new method of distraction osteogenesis for the treatment of craniosynostosis, multidirectional cranial distraction osteogenesis (MCDO). The purpose of this study is to review the authors' experience of MCDO for remodeling of the anterior cranium in the patients of craniosynostosis., Methods: Forty-five patients with craniosynostosis underwent MCDO for anterior cranial remodeling from 2003 to 2017. The ages of the patients ranged from 8 to 72 months (mean: 23.9 months, median: 13 months). The involved sutures included the bicoronal suture in 14 patients, unicoronal suture in 4 patients, sagittal suture in 14 patients, metopic suture in 2 patient, and multiple sutures in 10 patients., Results: An improvement of in cranial shape was achieved in 40 patients. In 1 patient, intraoperative massive bleeding forced us finishing the procedure before completing the planned osteotomy. Transient cerebrospinal fluid leakage occurred in 3 patients during the activation phase but recovered with conservative therapy. The mean blood transfusion was 25.5 mL/kg. The phase of activation ranged from 7 to 17 days (mean, 10.6 days) and the consolidation period ranged from 16 to 67 days (mean, 32.4 days). Loosening of traction pins occurred in 7 patients, resulting in undercorrection in 2 patients, and loosening of anchor pins occurred in 19 patients, resulting in acceleration of removal of the devices in 7 patients., Conclusions: Multidirectional cranial distraction osteogenesis was effective and has several advantages over the conventional procedures. Therefore, the authors conclude that MCDO will be a useful alternative for all phenotypes of craniosynostosis.

Treatment of unicoronal craniosynostosis is a surgically challenging problem, due to the involvement of coronal suture and cranial base, with complex asymmetries of the calvarium and orbit. Several techniques for correction have been described, including surgical bony remodeling, early strip craniotomy with orthotic helmet remodeling and distraction. Current distraction devices provide unidirectional forces and have had very limited success. Nitinol is a shape memory alloy that can be programmed to the shape of a patient-specific anatomy by means of thermal treatment.In this work, a methodology to produce a nitinol patient-specific distractor is presented: computer tomography images of a 16-month-old patient with unicoronal craniosynostosis were processed to create a 3-dimensional model of his skull and define the ideal shape postsurgery. A mesh was produced from a nitinol sheet, formed to the ideal skull shape and heat treated to be malleable at room temperature. The mesh was afterward deformed to be attached to a rapid prototyped plastic skull, replica of the patient initial anatomy. The mesh/skull construct was placed in hot water to activate the mesh shape memory property: the deformed plastic skull was computed tomography scanned for comparison of its shape with the initial anatomy and with the desired shape, showing that the nitinol mesh had been able to distract the plastic skull to a shape close to the desired one.The shape-memory properties of nitinol allow for the design and production of patient-specific devices able to deliver complex, preprogrammable shape changes.

Evaluation of infants with craniosynostosis for surgical intervention, as opposed to conservative management, remains a challenge within the field of craniofacial surgery. Studies have consistently demonstrated that surgical repair of craniosynostosis is ideally performed between 3 and 12 months of age. As such, there is limited data regarding neurocognitive development in infants who initially present with uncorrected craniosynostosis after 12 months of age. Moreover, the impact of cranial vault surgery on neurocognitive development at all ages remains under investigation. A prospective, nonrandomized study was performed. All children with nonsyndromic craniosynostosis who presented for initial evaluation after 12 months of age were enrolled. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) was utilized to assess pre- and postoperative cognitive development and comparisons were made to normative values. Developmental delay is defined as scoring < 85. Five infants, average age 26 months (13-43 months) at initial presentation, underwent cranial vault remodeling and developmental testing. Fused cranial sutures involved: metopic (n = 4), and right coronal (n = 1). Cognitive testing demonstrated that 4 of 5 infants (80%) were developmentally delayed at presentation (scores: 60, 70, 72, and 80), and 1 infant was within normal limits (score: 100). Postoperative testing was performed between 2 and 12 months postoperatively. Universal improvement was observed in infants who were delayed prior to surgery (80, 80, 75, and 90, respectively). The infant who was not delayed prior to surgery remained within normal limits after surgery. This study demonstrates an association between cranial vault surgery and cognitive improvement in infants presenting late with developmental delay.

Unilateral anterior plagiocephaly is most commonly the result of deformational plagiocephaly or unilateral coronal synostosis, a premature fusion of the frontoparietal suture. However, other sutures within the coronal ring have been implicated in producing anterior cranial asymmetries. These fusions can occur in isolation or in concert with adjacent sutures. The frontosphenoidal suture is one such suture within the coronal ring that has been involved both concomitantly with and independently of frontoparietal suture fusion. Although isolated frontosphenoidal synostosis has been presented previously in the literature, these reports include patients with fusion of the extraorbital portion of the frontosphenoidal suture. This clinical report presents the first clearly documented patient of isolated frontosphenoidal synostosis that occurs entirely within the intraorbital region.The patient presented to Plastic Surgery Clinic at 3 months of age with left frontal flattening, supraorbital rim retrusion, and temporal bulging that was noted soon after birth. Computed tomography analysis revealed an isolated fusion of the greater and lesser wings of the sphenoid bone to the frontal bone on the left side. The patient had no family history of cranial anomalies and genetic testing was negative for mutations. The infant was treated with a cranial orthotic for 3 months, underwent open fronto-orbital advancement and cranial vault remodeling at 6 months, and continued wearing a cranial orthotic for another 4.5 months. Following surgical and orthotic treatment, the patient achieved a satisfactory result.

Deformities of the cranium in patients with nonsyndromic single-suture synostosis occur because of growth restriction at fused sutures and growth over compensation at normal sutures. Traditional surgery includes ostectomies of the synostotic suture to release these restricted areas and osteotomies to enable immediate cranial remodeling. In the process of reshaping the cranium, traditional approaches usually involve obliteration of both the normal functioning suture and the pathologic suture. The directive growth approach (DGA) is a new, simpler, more natural way to repair deformities caused by single-suture cranial synostosis. The DGA works by reversing the original deforming forces by temporarily restricting growth in areas of over compensation and forcing growth in areas of previous synostotic restriction. Most importantly, it preserves a normal functioning suture to allow for improved future cranial growth. Eighteen consecutive nonsyndromic patients with unilateral coronal synostosis were used to illustrate the efficacy of the DGA. Ten patients who underwent DGA treatment were compared with a control group of 8 patients treated with traditional frontal orbital advancement. Postoperative three-dimensional computed tomography (CT) comparison measurements were taken, including bilateral vertical and transverse orbital dimensions, lateral orbital rim to external auditory canal, and forehead measurements from the superior aspect of the orbital rim to the pituitary fossa. The traditional treatment group showed absence of the coronal sutures bilaterally on long-term CT scans. The DGA group showed normal coronal sutures on the unaffected sides. Postoperative CT measurements showed no statistical difference between the 2 techniques (P < 0.05).

Aim: To compare bone regeneration in the critical-sized bone defects grafted with demineralized bone matrix, platelet-rich fibrin, and hyaluronic acid in rats., Materials and Methods: Forty Wistar Albino rats allocated into 4 experimental groups: platelet-rich fibrin (PRF) group, noncross-linked hyaluronic acid gel (HA) group, demineralized bone matrix in putty form (DBM) group, and control group. A critical-sized defect of 8 mm was formed involving the sagittal suture for each rat under anesthetic induction. All animals were sacrificed at 21st day after surgery and histomorphometric parameters of total horizontal length (THL) and total vertical length (TVL) of newly produced bone and longest bone trabecula (LBT) were measured in the histologic slides. The difference between experimental groups for these parameters was analyzed., Results: There was statistically significant difference in THL and LBT but not in TVL. Total horizontal length was significantly increased in DBM group compared with control and HA groups (P < 0.05). There was also statistically significant increase in THL in PRF group compared with control group (P < 0.05). Longest bone trabecula significantly increased in DBM group compared with HA group (P < 0.05). There was also statistically significant increase in HA group compared with control group (P < 0.05) and LBT significantly increased in PRF group compared with HA group (P < 0.05)., Conclusion: The sole usage of HA does not effectively increase bone regeneration when compared with DBM and PRF. The DBM and PRF do not have superiority to each other in the bone regeneration while they are superior to HA.

This report presents a case of a 4-year-old girl suffering from multiple-suture nonsyndromic craniosynostosis. The patient is characterized with fronto-orbital malformation and poor visual acuity. The preoperational three-dimensional computed tomography scan showed premature closure of the sagittal suture, bilateral coronal, and lambdoidal suture. The virtual surgical planning was performed before operation for the reposition of bone segments and communication with the patient's family. The navigational plate for reshaping was made based on the surgical plan with three-dimensional printed model. Both visual acuity and the fronto-orbital morphology were improved at 1 year follow-up. The authors believed that multiple-suture nonsyndromic craniosynostosis should be treated as early as possible to protect the brain and eyes. It can improve the safety and precision of the treatment using the virtual surgical planning and prefabricated surgical template.

Background: Asymmetric multisutural craniosynostosis (AMC) is characterized by fusion of a midline suture combined with unilateral fusion of at least 1 nonmidline suture. Due to its rarity, complexity, and high rate of reoperation, the purpose of this study is to evaluate outcomes of our staged approach to AMC., Methods: Patients treated for craniosynostosis between January 2004 and December 2013 were identified retrospectively. Only patients with AMC and a minimum follow-up of 2 years were included. The 3-staged algorithm includes: extended strip craniectomy of fused sutures; postoperative helmet molding; and fronto-orbital advancement versus a touch-up procedure. Morphologic, aesthetic, and functional outcomes were evaluated., Results: Nine patients (6.8% of cases) were treated for AMC (mean follow-up: 4.1 years). Sixty-seven percent of patients (6 of 9) demonstrated signs of elevated intracranial pressure. Patients were divided into 2 groups: "Group A" included patients treated according to the staged algorithm (n = 5); "Group B" included those treated by traditional techniques (n = 4). Group A underwent their first calvarial vault procedure earlier than those from Group B (2.7 vs. 13.2 months; P < 0.02). Postoperatively, no Group A patients had developmental delay, signs of elevated intracranial pressure, or reoperation. Three of 5 patients (60%) were rated Whitaker Classification II and the others (40%) rated Whitaker Classification III., Conclusions: Asymmetric multisutural synostosis results in a complex and evolving deformity involving the entire craniofacial complex. However, when asymmetric multisutural synostosis is approached in stages with early intervening helmet therapy, acceptable functional and appearance-related outcomes can be obtained with minimal complications., Level of Evidence: III.