Perioperative management for craniosynostosis repair in a pediatric population: a narrative review

Introduction

Background

Craniosynostosis is a congenital condition characterized by the premature fusion of one or more cranial sutures, disrupting normal skull and brain growth. The early closure results in restricted bone growth perpendicular to the affected suture, while compensatory growth occurs along unaffected suture sites (1-3). This condition affects approximately 1 in 2,500 live births and may present in either isolated (nonsyndromic) or syndromic forms (1). Craniosynostosis can involve a single or multiple sutures, such as the sagittal, coronal, metopic, or lambdoid sutures, and leads to abnormal skull morphology with potential sequelae of raised intracranial pressure (ICP), cognitive and neurodevelopmental impairment, and psychological distress (1-3).

Nonsyndromic craniosynostosis, which accounts for 80% of cases, typically involves a single suture without associated craniofacial or systemic anomalies (3,4). In contrast, syndromic craniosynostosis arises from a genetic mutation or inherited condition and often involves multisystem anomalies, midface hypoplasia and limb abnormalities (5-7). Potential growth and development complications include impaired cognitive functions, craniofacial and limb deformities, breathing difficulties, vision problems, hearing loss, and feeding issues (8).

Another common complication is increased ICP due to hydrocephalus, craniocerebral disproportion, and abnormalities in cerebral venous drainage which require treatment at an early age prior to permanent neurologic changes and while the skull bones are soft and easier to reshape (7). Surgical correction is the primary treatment approach, typically undertaken early in life to prevent complications and promote normal cranial growth (7,8). Surgical correction generally occurs at 3–12 months of age (9,10). Perioperative management in this young population presents unique challenges. A multidisciplinary approach involving neurosurgeons, craniofacial surgeons, anesthesiologists and developmental specialists is essential to optimize outcomes and ensure proper skull growth, neurological function and overall quality of life.

Rationale and knowledge gap

Despite advances in surgical and anesthetic techniques, craniosynostosis repair still presents unique challenges, including significant risks related to perioperative management, securing and maintaining a potentially hyperreactive airway, intravenous access, temperature dysregulation, and excessive blood loss (1,5,6). Literature highlighting the anesthetic concerns associated with craniosynostosis patients remains limited (1,5,6). While emerging minimally invasive surgical techniques have shown promise in reducing morbidity, open procedures remain the gold standard for many cases (10,11). Additionally, there is no clear consensus on optimal perioperative anesthetic strategies for mitigating these risks (1,6). As with any anesthesia preparation, it is important to recognize the potential anesthetic difficulties in advance and develop strategies to prevent or treat them prior to their occurrence.

Objective

The authors aim to highlight the key perioperative considerations for managing children undergoing craniosynostosis repair to guide both surgeons and anesthesiologists. This review focuses on: (I) how perioperative risks (e.g., airway compromise, blood loss) can be reduced in pediatric craniosynostosis repair; (II) the comparative outcomes of open versus endoscopic techniques; and (III) which anesthetic strategies best optimize neuroprotection and recovery. The clinical need stems from rising surgical volumes and variability in practice, underscoring the demand for standardized, evidence-based protocols. We present this article in accordance with the Narrative Review reporting checklist (available at https://joma.amegroups.com/article/view/10.21037/joma-25-12/rc).

Methods

This narrative review was conducted through a literature search of PubMed, Google Scholar, and Cochrane Library databases from January 1999 to December 2024. Our search strategy employed a combination of Medical Subject Headings (MeSH) terms and keywords, including “cranial vault reconstruction”, “cranial vault repair”, “cranioplasty”, “anesthetic management”, “pediatric”, “children”, and “infants”. Boolean operators (AND, OR) were used to combine search terms effectively (Table 1).

Two independent reviewers (B.M. and H.S.) screened all identified articles through a two-stage screening process. First, titles and abstracts were evaluated against predetermined inclusion criteria: (I) studies involving pediatric patients (0–18 years) undergoing craniosynostosis repair; (II) articles reporting anesthetic or perioperative outcomes; and (III) publication in peer-reviewed journals. Exclusion criteria included non-English publications and studies lacking relevant outcome measures.

For potentially eligible studies, full-text articles were obtained and reviewed in detail. Discrepancies between reviewers were resolved through discussion and, when necessary, consultation with a third author (C.S.). Data extraction was performed using a standardized form capturing: study design, patient demographics, surgical techniques, anesthetic protocols, outcome measures, and complications.

We note the inherent limitations of narrative synthesis in addressing potential publication bias and study heterogeneity. All search strategies, inclusion decisions, and data extraction forms were documented to ensure reproducibility (Table 2).

Perioperative management of craniosynostosis repair

Surgical management

Craniosynostosis treatment can begin as early as 6 weeks of age and may necessitate multiple procedures to achieve optimal outcomes. Syndromic cases, often involving multiple organ systems, present an elevated risk of perioperative complications, potentially necessitating a more conservative approach to anesthetic management and surgical planning (12-15).

Surgical correction remains the primary treatment for treating craniosynostosis, with an individualized approach dictated by the number of sutures involved, the severity of clinical presentation, and patient-specific anatomical and clinical factors (7,8,12-15). Early intervention is typically preferred to facilitate normal brain growth and cognitive development (9,10). Several surgical techniques are employed, ranging from extended strip craniectomy, total vault reconstruction, minimally invasive endoscopic surgery, spring-assisted cranioplasty, fronto-orbital remodeling, and posterior calvaria vault expansion (14,16,17).

Cranial vault remodeling is a highly invasive procedure requiring extensive removal and reconfiguration of the cranial vault to create an optimal environment for cerebral expansion and skull development (12,18). This approach, however, carries a significant risk of severe complications and a higher likelihood of reoperation due to the formation of neosutures, which may contribute to secondary craniosynostosis (19,20).

In contrast, endoscopic surgery, also known as endoscopic strip craniectomy or endoscopic release surgery, utilizes an endoscope to excise small segments of bone from the fused suture (21,22). This procedure is typically followed by postoperative helmet therapy to guide cranial molding (21-25). Both approaches carry inherent risks to the infant, but regardless of approach, perioperative management of anesthesia is important.

Preoperative considerations

Surgical intervention for craniosynostosis is most commonly performed by 12 months or earlier, as early correction is critical to accommodate ongoing brain growth (7-10). Operating at a younger age offers the advantage of softer, more malleable bone, allowing natural cranial expansion to contribute to postoperative remodeling (1,26). Neonates, however, present a heightened surgical risk due to lower blood volume, increasing the potential for significant blood loss (26,27). Additionally, there is a risk of reoperation due to craniocerebral disproportion (28). Delaying surgery until later infancy may facilitate safer anesthetic management but introduces technical challenges, as increased ossification of the cranial bones complicates reconstruction and developmental delays may have already occurred (29,30). At the same time, due to the minimally invasive nature of endoscopic strip craniectomy, there are less risks regarding blood loss during the surgery as well, but the patient is younger and has less total blood volume, so there are different risks and benefits (29,30).

Nutrition

Despite its critical role in perioperative management, research on nutrition in infants with craniosynostosis remains limited (31,32). Adequate nutrition during the neonatal and infant periods is essential for optimal craniofacial development (31,33). Studies have demonstrated that food consistency can have a lasting impact on skull bone formation, while maternal undernutrition during lactation is also associated with reduced skull size (33,34). Malnutrition is also associated with higher American Society of Anesthesiologists (ASA) scores, underscoring its significance in perioperative risk assessment (35).

In these patients, malnutrition alters anesthetic drug pharmacokinetics by affecting volume of distribution, protein binding, metabolism, and elimination, necessitating dose adjustments to mitigate adverse effects (35). Additionally, it increases intraoperative risks (e.g., hypotension, cardiac/respiratory complications) and worsens postoperative outcomes, including infections, delayed wound healing, and higher mortality (35). Certain syndromic cases, such as Apert syndrome, frequently require specialized dietetic intervention and enteral supplementation to support appropriate growth and development (36).

At present, there is no high-level evidence regarding nutrition in craniosynostosis patients; further research is warranted to better inform clinical practice in this area. The current studies are limited to case reports, literature reviews, and prospective studies on non-human subjects.

Preoperative education

Preoperative education is necessary to set patient and family expectations regarding surgical and postoperative outcomes, yet remains an underemphasized component of surgical planning for craniosynostosis patients (37). Given the complexity of these procedures, ensuring that families have a thorough understanding of the surgical process, anticipated outcomes, and potential risks is paramount (37). Effective preoperative discussions not only establish realistic expectations but also contribute to improved psychological well-being (37,38). Studies have demonstrated that extensive education enhances patient satisfaction, reduces anxiety, and improves overall quality of care (38). This aspect of perioperative management remains relatively understudied in the context of craniosynostosis surgery, and further research is needed to optimize educational strategies and enhance patient and family preparedness. Overall, preoperative education represents a critically understudied area with substantial potential for improving outcomes.

Intraoperative management

The management of craniosynostosis repair requires meticulous multidisciplinary planning to address the unique challenges presented by young infants, particularly those with abnormal cranial morphology. These cases often involve complex airway anatomy that can complicate mask ventilation, intubation, and postoperative airway management. Significant intraoperative blood loss is a frequent concern due to the rich vascularity of the infant skull and the extensive bony dissection required, with the risk magnified in smaller patients who have proportionally lower circulating blood volumes. In syndromic patients, additional challenges arise from multisystem involvement such as cardiac anomalies, respiratory compromise, and airway malformations (38-41). Effective preoperative assessment, coordinated intraoperative management, and vigilant postoperative monitoring are essential to optimize outcomes in this vulnerable population.

Intraoperative anesthesia

Standardized anesthetic protocols have been described in the literature to optimize perioperative management in craniosynostosis surgery (42). Dave et al. reported a regimen involving intravenous midazolam (0.05 mg/kg) as sedative premedication, followed by glycopyrrolate (4 µg/kg) and fentanyl (2 µg/kg) before induction in patients with an anticipated uncomplicated airway (43). For patients with difficult airways, anesthesia induction was achieved with inhaled sevoflurane, followed by atracurium administration via mask ventilation (43). In syndromic craniosynostosis cases where conventional intubation proved challenging, the Frova intubation introducer facilitated successful endotracheal intubation (43).

Anesthesia was maintained using a closed-circuit system delivering a mixture of air, oxygen, and sevoflurane, supplemented with dexmedetomidine at 0.5 µg/kg/h (43). Perioperative analgesia consisted of intermittent fentanyl boluses (1 µg/kg/h) and paracetamol (15 mg/kg), ensuring adequate pain control while minimizing opioid-related respiratory depression (43).

The available literature on intraoperative anesthesia for craniosynostosis spans from high-level narrative reviews to center-specific observational studies, underscoring the need for higher-quality, prospective research to establish standardized, evidence-based anesthetic protocols for this high-risk pediatric population.

Airway management

Airway management in craniosynostosis repair is particularly challenging, especially in syndromic cases such as Apert, Crouzon, and Pfeiffer syndromes (1,44). These patients frequently exhibit craniofacial anomalies, including midface hypoplasia, choanal stenosis, and tracheal abnormalities, which contribute to difficulties in mask ventilation, intubation, and postoperative airway maintenance (1,44,45). Nearly 20% of craniofacial patients require tracheotomy, with syndromic cases exhibiting the highest rates—up to 48% in conditions such as Crouzon, Pfeiffer, and Apert (46). The high prevalence of obstructive sleep apnea (OSA) in these patients, as demonstrated by Inverso et al. and Cielo et al., is largely attributed to midface hypoplasia (47,48). Furthermore, research by Lesciotto et al. reported that craniosynostosis patients have reduced airway volumes, further complicating perioperative respiratory management (49).

Preoperative imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), is essential for evaluating airway anatomy and anticipating potential difficulties (50,51). 3D reconstructions aid in identifying tracheal abnormalities and the degree of midface retrusion, enabling more strategic airway planning (50,51). Given these anatomical complexities, a carefully tailored strategy is essential for induction, intubation, and postoperative airway care.

Intraoperative, securing the airway demands a well-planned approach incorporating advanced techniques to mitigate risk (1,46). Inhalational induction with sevoflurane is often preferred in pediatric patients with anticipated difficult airways until a secure airway is achieved (52,53). Video laryngoscopy and fiberoptic intubation have been shown to significantly improve intubation success rates in pediatric patients with challenging airways, as demonstrated in a multicenter study by Burjek et al. (53). In severe cases involving airway obstruction or tracheal anomalies, such as cartilaginous tracheal sleeves, tracheostomy may be necessary to ensure adequate ventilation and reduce perioperative complications (54). Additionally, meticulous attention to patient positioning, including head elevation and maintenance of neutral neck alignment, is essential to prevent jugular venous obstruction and mitigate the risk of increased ICP during the procedure (1,51).

Postoperative airway management is equally critical, particularly for syndromic patients or those with preexisting OSA (55). In high-risk cases, delayed extubation or prolonged postoperative ventilation may be required to maintain airway stability (56). Factors such as prolonged operative duration, significant fluid shifts, extensive blood transfusion, prolonged prone positioning, and preoperative airway compromise can necessitate delayed extubation (1,57,58). Continuous monitoring for signs of airway obstruction, including stridor and oxygen desaturation, is essential to prevent respiratory complications (59). In patients with either an endotracheal tube or a tracheostomy, meticulous postoperative care is paramount to avoid complications such as tube dislodgement or infection at the tracheostomy site.

The literature on airway management in craniosynostosis encompasses a mix of contemporary cohort studies, narrative reviews, and foundational observational work, with overall evidence quality ranging from moderate to low. Collectively, these works provide important clinical insight, but the predominance of observational designs and the scarcity of craniosynostosis-specific prospective trials limit the strength of current recommendations.

Blood loss

Craniosynostosis repair, particularly open cranial vault reconstruction, is associated with a substantial risk of blood loss, necessitating an encompassing and multimodal blood management strategy (60). Various interventions have been explored to mitigate intraoperative hemorrhage and reduce the need for transfusion, including the use of erythropoietin, cell salvage technology, and antifibrinolytics such as tranexamic acid (61,62).

The magnitude of intraoperative blood loss is influenced by multiple factors, including patient age, body weight, head-to-body size ratio, and procedural complexity- parameters that are particularly relevant in syndromic cases where surgical interventions are often more extensive (63,64). A study by Meyer et al. highlighted the challenges associated with perioperative transfusion, reporting that only 48% of cases achieved optimal transfusion levels, while 32% experienced overtransfusion (65). Furthermore, transfusion-related complications, such as metabolic disturbances (hypoglycemia, hyperkalemia, and hypokalemia), infectious risks, and prolonged hospitalization, underscore the need for judicious blood conservation strategies (66).

Tranexamic acid, an antifibrinolytic agent, has emerged as a critical component in perioperative hemostatic management, demonstrating a significant reduction in intraoperative blood loss without a discernible difference between high and low dose regimens (67,68). Additionally, intraoperative cell salvage, which enables the reinfusion of autologous red blood cells, has been shown to decrease reliance on allogeneic blood transfusions, with studies indicating that only 30% of patients receiving intraoperative cell salvage ultimately require donor blood (69). Erythropoietin therapy has similarly proven beneficial in augmenting preoperative hemoglobin levels and minimizing transfusion requirements postoperatively (70).

The adoption of minimally invasive approaches, such as endoscopic-assisted craniectomy, has been associated with significantly reduced perioperative blood loss and lower transfusion rates compared to traditional open techniques (71,72). Dave et al. demonstrated that administering tranexamic acid at an initial dose of 10 mg/kg, followed by continuous infusion at 1 mg/kg/h, effectively minimizes intraoperative hemorrhage while optimizing postoperative hemoglobin levels (43).

Overall, the available literature on perioperative blood management in craniosynostosis surgery is composed primarily of moderate- to low-quality studies, with a few higher-quality analyses. Collectively, this body of work supports pragmatic perioperative strategies, yet the absence of large, multicenter randomized controlled trials and standardized protocols limits the certainty of recommendations. Future research should focus on adequately powered trials examining tranexamic acid dosing strategies, preoperative erythropoietin and iron optimization, the role of cell saver and viscoelastic-guided transfusion, and the development of standardized enhanced recovery pathways, with stratified analyses by syndrome status, age, and weight, as well as attention to safety, cost-effectiveness, and long-term neurodevelopmental outcomes.

ICP management

Elevated ICP is a well-documented concern in craniosynostosis, particularly in syndromic cases, where the prevalence of increased ICP has been reported in 30–40% of patients, according to Deopujari et al., and in up to 58% of cases with preoperative ICP exceeding 10 mmHg, as documented by Makoshi et al. (73,74). The broader literature indicates that one to two-thirds of children with craniosynostosis experience elevated ICP, which, if left unmanaged, may result in cerebral ischemia, brain herniation, and long-term neurocognitive deficits (75-84). These risks necessitate vigilant intraoperative monitoring and targeting perioperative interventions to optimize cerebral perfusion (85).

The pathophysiology of elevated ICP in craniosynostosis is multifactorial, involving craniocerebral disproportion, impaired venous drainage, and potential hydrocephalus, challenges especially pronounced in syndromic variants of the condition (86). Studies have demonstrated that surgical intervention effectively alleviates elevated ICP, with Makoshi et al. reporting a 64% reduction in mean ICP following endoscopic-assisted craniectomy in infants under 1 year of age, with preoperative levels decreasing from 12.7 to 2.9 mmHg postoperatively (74).

Effective intraoperative ICP management hinges on a multimodal approach. Immediate stabilization measures include maintaining head elevation at 15–30 degrees to promote venous drainage, ensuring optimal oxygenation and ventilation, and preserving cerebral perfusion pressure (CPP) through meticulous hemodynamic control (85). Osmotic agents, such as intravenous mannitol (0.25–1 g/kg) or hypertonic saline (3% saline, 2–5 mL/kg), are commonly utilized to mitigate cerebral edema and transiently lower ICP (85). Additionally, controlled hyperventilation [targeting a partial pressure of carbon dioxide (PaCO₂) range of 35–40 mmHg] may provide temporary ICP reduction by inducing cerebral vasoconstriction, though its use must be carefully titrated to prevent adverse effects on cerebral oxygenation (85).

Sedation and analgesia play a crucial role in preventing agitation-induced ICP surges, while early initiation of invasive ICP monitoring, via intraparenchymal sensors or ventricular catheters, allows for real-time assessment and targeted therapeutic interventions (85). Given the unique physiological considerations in pediatric patients, including lower baseline blood pressure and reduced circulating blood volume, ICP management strategies must be carefully adapted to avoid complications such as hypotension and electrolyte imbalances (85).

Recent literature on ICP monitoring in craniosynostosis includes both conceptual overviews and procedure-specific observational data. Collectively, these studies strengthen the rationale for incorporating ICP monitoring into the perioperative management of craniosynostosis, though larger, multicenter trials are needed to establish standardized monitoring protocols and to define postoperative ICP thresholds predictive of long-term neurodevelopmental outcomes.

Extubation

Extubation in pediatric patients adheres to fundamental anesthetic principles similar to those in adults; however, the unique anatomical and physiological characteristics of infants introduce distinct challenges (87,88). The inability of this population to effectively communicate distress, coupled with a propensity for airway obstruction, hypoventilation, and peri-extubation respiratory compromise, necessitates a nuanced approach to extubation planning (87,88).

Extubation failure, though relatively infrequent, carries significant morbidity. Weatherall et al. observed a 5% extubation failure rate in pediatric patients, most of whom were extubated directly to anesthesia face masks without adjunctive support (e.g., oropharyngeal or nasopharyngeal airways) (89). The principal causes of failure included oxygen desaturation, airway obstruction, and hypoventilation, frequently requiring reintubation (89). Risk stratification is crucial, as identified by Baisch et al., who identified age below 6.5 months, extended surgical duration, and syndromic cases with craniofacial anomalies as significant predictors of extubation failure, with an overall failure rate of 4.1% (90-92). Similarly, Jagannathan et al. highlighted that children younger than 18.5 months or weighing less than 10 kg face an increased risk of extubation failure, reinforcing the importance of patient-specific extubation strategies (93).

Peri-extubation pharmacologic interventions have demonstrated efficacy in improving outcomes (94). Parajuli et al. found that corticosteroids administration within 24 hours prior to extubation reduced post-extubation complications, likely by mitigating airway inflammation and edema (94). Given the implications of extubation failure, including prolonged mechanical ventilation, increased intensive care requirements, and heightened perioperative morbidity-meticulous planning, patient-specific risk assessment, and multimodal airway management strategies are essential to optimizing peri-extubation outcomes in this vulnerable population (91).

The current literature on pediatric extubation comprises a mix of randomized trials, multicenter observational studies, single-center cohorts, and narrative reviews, with overall evidence quality ranging from moderate to low. Collectively, these studies inform best practices for pediatric extubation, but craniosynostosis-specific evidence remains sparse, particularly regarding syndromic airway challenges. Future research should focus on prospective, craniofacial-specific protocols, standardized definitions of extubation failure, and longer-term follow-up assessing respiratory, airway, and neurodevelopmental outcomes.

Antibiotics

The judicious use of perioperative antibiotics is essential in infection prevention in craniosynostosis repair, balancing the reduction of surgical site infections (SSIs) against preventing antibiotic resistance (95,96). Current evidence supports the efficacy of a single-dose antibiotic prophylaxis regimen in the prevention of infections while avoiding the risks associated with prolonged antibiotic exposure (96).

Holle et al. demonstrated that a single intraoperative dose of cefuroxime (50 mg/kg intravenous) administered at anesthesia induction, in conjunction with tranexamic acid, was non-inferior to extended prophylactic regimens involving multiple postoperative doses in terms of SSI rates and wound colonization (96). Their findings challenge the necessity of prolonged prophylaxis, which traditionally involved an initial intraoperative dose of cefuroxime followed by 24–48-hour postoperative regimen of 33 mg/kg intravenous three times daily (96). Given the absence of significant differences in infection rates between single-dose and prolonged regimens, a streamlined prophylactic approach offers a more judicious balance of efficacy and antimicrobial stewardship (96).

Similarly, Esposito et al. advocate for a cefazolin-based regimen (30 mg/kg intravenous, maximum 2 g), administered 30 minutes preoperatively, with an additional intraoperative dose for surgeries exceeding 4 hours (97). In cases of methicillin-resistant Staphylococcus aureus (MRSA) or methicillin-susceptible Staphylococcus aureus (MSSA) colonization—where preoperative decolonization is unfeasible due to surgical urgency—a dual antibiotic regimen incorporating cefazolin (30 mg/kg intravenous) and vancomycin (15 mg/kg intravenous, maximum 2 g) is recommended to ensure optimal antimicrobial coverage (97).

The literature on perioperative antibiotic prophylaxis in pediatric surgery, including craniosynostosis repair, is limited in both quantity and quality, with most evidence derived from observational data, small single-center studies, or expert consensus. Taken together, these studies support streamlined, stewardship-conscious prophylactic strategies, but the paucity of randomized controlled trials—particularly in craniosynostosis surgery—limits the certainty of recommendations. Future research should focus on multicenter, procedure-specific trials to define the optimal spectrum, timing, and duration of prophylaxis, with particular attention to high-risk subgroups such as syndromic craniosynostosis.

Limitations

This narrative review appraises the perioperative anesthetic considerations in craniosynostosis repair, addressing an area that remains underrepresented in the literature. While it consolidates evidence across multiple domains, including blood management, ICP control, extubation strategies, and antibiotic prophylaxis, the findings are inherently constrained by the paucity of high-level evidence specific to anesthetic interventions. Existing studies often lack granularity in anesthetic outcomes (e.g., opioid-sparing effects, neurocognitive impacts) and disproportionately focus on surgical metrics. Small sample sizes, especially for syndromic cases, limit generalizability. Few studies address long-term follow-up, leaving gaps in understanding developmental trajectories post-repair.

The primary strength of this review lies in its integration of available data to generate pragmatic, evidence-informed guidance for anesthetic management in pediatric craniosynostosis surgery. By synthesizing findings from disparate sources, it delineates key anesthetic considerations and offers clinical insights into the mitigation of perioperative complications. However, the reliance on smaller-scale studies, expert consensus, and retrospective analyses limits the generalizability of certain recommendations.

To advance the field, further large-scale, prospective studies are required to establish standardized anesthetic protocols tailored to this high-risk patient population. Future research should focus on augmenting preoperative hemoglobin levels, optimizing blood conservation techniques, refining ICP management strategies, and evaluating novel peri-extubation and infection control interventions. A multidisciplinary, evidence-based approach will be essential to improving outcomes and enhancing the safety and efficacy of anesthetic management in craniosynostosis repair.

Conclusions

The perioperative management of craniosynostosis repair remains a complex and evolving challenge, particularly in syndromic cases where multisystem comorbidities and intricate craniofacial anomalies amplify surgical and anesthetic risks. While significant progress has been made in recent years—including the adoption of minimally invasive techniques, refined blood conservation strategies, and improved airway management protocols—critical gaps persist in standardizing care and optimizing long-term outcomes. Surgical innovations such as endoscopic-assisted techniques and spring-mediated cranioplasty have reduced operative trauma, blood loss, and hospital stays, particularly in nonsyndromic cases, though open cranial vault remodeling remains the gold standard for complex or multisutural synostosis, necessitating further research to refine patient selection criteria.

Anesthetic care has seen notable advances through the integration of tranexamic acid, cell salvage, and preoperative erythropoietin to mitigate transfusion-associated risks, while advanced airway tools like video laryngoscopy and fiberoptic intubation have improved safety in syndromic patients with difficult anatomy. The development of multidisciplinary care protocols has further enhanced perioperative monitoring, particularly for ICP management and extubation strategies. However, several unresolved challenges remain, including the lack of consensus on transfusion thresholds, antifibrinolytic dosing, and optimal ICP monitoring techniques. There is also a pressing need for evidence-based guidelines tailored to the distinct needs of syndromic versus nonsyndromic patients.

Future research must prioritize multicenter studies to address critical gaps in long-term neurodevelopmental outcomes, comparative surgical approaches, and equitable access to care. There remains a need for high-quality evidence guiding preoperative hemoglobin optimization, effective blood-sparing methods, and standardized ICP management protocols. Key investigations should focus on optimizing anesthesia (e.g., dexmedetomidine), validating monitoring technologies, and establishing standardized protocols through collaborative, data-driven research. By integrating translational science with patient-centered outcomes, we can advance personalized care models that improve both perioperative safety and long-term quality of life for craniosynostosis patients worldwide.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the Narrative Review reporting checklist. Available at https://joma.amegroups.com/article/view/10.21037/joma-25-12/rc

Peer Review File: Available at https://joma.amegroups.com/article/view/10.21037/joma-25-12/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://joma.amegroups.com/article/view/10.21037/joma-25-12/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

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