Despite the clinical importance of identifying increased intracranial pressure (IIP) in children with craniosynostosis (CS), its presence is often uncertain due to limited utilization of invasive measurement methods, inconclusive clinical evaluations, and its variability depending on the CS phenotype. Hence, prevalence reports are highly variable. We previously developed a computational method to identify pediatric chronic IIP of diverse etiology based on subtle cranial thickness and density anomalies quantified from computed tomography (CT) scans. In this study, we evaluate cranial signs of IIP in a large dataset of presurgical CT scans of patients with diverse phenotypes of CS and its prevalence.

We quantified local cranial thickness and density in the CT scans of 417 patients with diverse phenotypes of CS (age 0-2 y). We used a normative reference of cranial development to quantify cranial bone anomalies in each phenotypic group and compared them with 48 patients with chronic IIP unrelated to CS. We then studied the risk of IIP and its prevalence in each phenotypic group of CS.

Patients with CS presented significant calvarial thickening and bone density decrease compared with normative patients (P < 0.001). Similar findings were found in patients with chronic IIP unrelated to CS (P > 0.23). Presurgical signs of IIP were more prevalent in patients with Apert syndrome (>74% patients) and nonsyndromic patients with coronal involvement (>30%) compared with other phenotypes (>18%).

Computational evaluation of routinely acquired presurgical CT scans can potentially support the evaluation of IIP in patients with CS.

Craniosynostosis is a congenital skull deformity that impacts development and quality of life of children if left untreated. This study aimed to evaluate literature regarding presentation, treatment, and outcomes of craniosynostosis in Africa.

A systematic review of the literature using PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar databases was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

Fourteen retrospective/prospective studies with 620 patients and 14 case reports involving 27 cases (8 countries) were included. In 12 articles, 56.6% of patients (317/560) were males, with a mean age of 2.4 years (confidence interval [CI]: 1.1-3.7). Abnormal head shape was the most reported presentation in 77.8% of cases (332/427, 8 articles). Syndromic craniosynostosis was seen in 25.2% (CI: 13.7%-36.6%). Common phenotypes were trigonocephaly in 31.5% (CI: 3.6%-59.4%), anterior plagiocephaly in 23.2% (CI: 5.1%-41.3%), and scaphocephaly in 22.1% (CI: 13.5%-30.8%). Five hundred seventy eight patients, 99.5% (CI: 99.0%-100.0%), underwent surgical treatment. Vault remodeling was performed in 72.9% patients (CI: 47.4%-98.6%). Postoperative complications included cerebrospinal fluid leaks 5.4% (CI: 0.0%-11.6%) and surgical site infections 4.5% (CI: 0.0%-10.8%). Follow-up ranged between 0.2 and 40.9 months; 95.6% of cases (CI: 90.1%-100.0%) exhibited improved deformity and neurological deficits at last follow-up. The mortality rate was 3.1% (CI: 0.0%-6.9%, 2 articles).

Few studies on craniosynostosis in Africa highlight the need for more research. Treatment with open techniques yields few complications and a low mortality rate. Early diagnosis and collaborative data reporting will enhance understanding of its burden and variations across Africa.

Children with syndromic craniosynostosis require multiple cranial expansion procedures. The purpose of this study was to determine how many expansions are typically performed through maturity, to assess complication rates, and to identify trends that might reduce the burden of care.

A retrospective chart review was conducted of all consecutive patients undergoing cranial vault enlargement procedures for syndromic craniosynostosis performed by a single surgeon.

Of 444 patients treated with Apert or Crouzon/Pfeiffer syndromes, 348 had complete long-term records. The mean age at last follow-up was 16.9 years. Initial cranial expansions were performed at a significantly younger age for those beginning care at outside centers: 7.7 months versus 25.7 months in Dallas; 26.4% had ventriculoperitoneal shunts (syndrome-specific incidences: Apert syndrome, 16%; Crouzon syndrome, 27%; and Pfeiffer syndrome, 54%). Of 498 procedures, 80% were anterior, 18% were posterior, and 2% were midvaults. The overall complication rate was 2%, with no differences found between anterior and posterior procedures. Those treated solely in Dallas underwent a mean of 1.9 expansions. Children with Pfeiffer syndrome underwent more expansions (2.7) than those with Apert or Crouzon syndrome. Factors correlating with more expansions included earlier surgical interventions, use of distraction, ventriculoperitoneal shunts, and treatment begun at outside centers (3.1 procedures).

Children with syndromic craniosynostosis treated at a single center underwent an average of 2 skull expansion procedures with a 2% complication rate. Calvarial distraction, ventriculoperitoneal shunts, and earlier surgical interventions were all associated with higher numbers of expansion procedures. Further research is needed to determine whether these correlations are causal.

Therapeutic, IV.

Craniosynostosis is a common diagnosis requiring early referral to a pediatric plastic surgeon; however, disparities in healthcare may influence presentation timing and affect treatment options and outcomes. This study aimed to explore sociodemographic factors contributing to delay in craniosynostosis surgical consultation.

A retrospective cohort study of 694 California-based craniosynostosis patients at a tertiary children's hospital was performed from 2006 to 2023. State-specific Area Deprivation Index (ADI) and distance to the hospital were calculated using ZIP codes. Multivariate linear and logistic regressions considered race, insurance type, syndromic status, suture type, and ZIP code-based socioeconomic factors.

Median age of presentation was 4.5 [interquartile range: 2.6-7.6] months with racial/ethnic breakdown of Hispanic/Latinx (41.2%), White (23.6%), Asian (3.7%), Black/African American (2.0%), or other/unreported (29.5%) with 58.4% having public insurance and an average distance to the hospital of 48.3 km. Median ADI was 5.4 [interquartile range: 4.0-7.1]. By linear regression, public insurance (P < 0.001) and higher ADI decile (P < 0.001) independently contributed to an older age of presentation. Patients with public insurance (odds ratio 1.90; P = 0.002) were more likely to present after 4 months of age.

Patients who had public insurance or resided in more disadvantaged areas presented later for craniosynostosis surgical consultation. Eliminating disparities in these populations ensures more equitable access to surgical options and can improve patient outcomes.

Previous studies have sought to analyze risk factors associated with craniosynostosis and while syndromic craniosynostosis is often linked to genetic mutations, the factors impacting nonsyndromic cases are less investigated. The aim of current meta-analysis is to evaluate the relationship between ethnicity and suture type in nonsyndromic craniosynostosis patients.

The search term "craniosynostosis [Title/Abstract] AND (race [Title/Abstract] OR ethnicity [Title/Abstract])) NOT (syndrome [Title/Abstract])" was used to search the PubMed, Cochrane, and MEDLINE databases. Analyses were conducted separately for each racial and ethnic group for each suture type cohort. Odds ratios were conducted for each suture cohort and confounders were adjusted using linear mixed-effect models. Because of the homogeneity of the populations and categorical nature of the classification, binary logistic regression was run on aggregate data.

The literature search yielded 165 articles. After reviewing titles, abstracts, and manuscript contents of these articles, 5 studies were ultimately included in a meta-analysis. Studies with missing data for a particular cohort or variable were excluded from the respective analysis. Hispanic children had higher odds of sagittal suture involvement (OR: 1.53, P <0.001), whereas Asian had coronal suture (OR: 2.47, P <0.001). Both Asian and African American children had significantly lower odds of sagittal suture involvement (OR: 0.50, P <0.001 and OR: 0.7, P =0.04, respectively).

The relationship between ethnicity and craniosynostosis has been suggested as a risk factor, but without definitive conclusion. Present meta-analysis findings demonstrated association between ethnicity and suture type, however further research with larger scale and geographically varied data is warranted.

Apart from listening to the cry of a healthy newborn, it is the declaration by the attending paediatrician in the labour room that the child is normal which brings utmost joy to parents. The global incidence of children born with congenital anomalies has been reported to be 3%-6% with more than 90% of these occurring in low- and middle-income group countries. The exact percentages/total numbers of children requiring surgical treatment cannot be estimated for several reasons. These children are operated under several surgical disciplines, viz, paediatric-, plastic reconstructive, neuro-, cardiothoracic-, orthopaedic surgery etc. These conditions may be life-threatening, e.g., trachea-oesophageal fistula, critical pulmonary stenosis, etc. and require immediate surgical intervention. Some, e.g., hydrocephalus, may need intervention as soon as the patient is fit for surgery. Some, e.g., patent ductus arteriosus need 'wait and watch' policy up to a certain age in the hope of spontaneous recovery. Another extremely important category is that of patients where the operative intervention is done based on their age. Almost all the congenital anomalies coming under care of a plastic surgeon are operated as elective surgery (many as multiple stages of correction) at appropriate ages. There are advantages and disadvantages of intervention at different ages. In this article, we present a review of optimal timings, along with reasoning, for surgery of many of the common congenital anomalies which are treated by plastic surgeons. Obstetricians, paediatricians and general practitioners/family physicians, who most often are the first ones to come across such children, must know to guide the parents appropriately and convincingly impress upon the them as to why their child should not be operated immediately and also the consequences of too soon or too late.

Study Design: A Case Report. Objective: Craniosynostosis is a craniofacial condition defined by premature fusion of at least one cranial suture. Resynostosis or secondary craniosynostosis of a previously patent adjacent suture following primary repair is a relatively common complication. While studies have assessed the rates of secondary craniosynostosis and subsequent reoperation, extremely limited data regarding reoperation techniques is available. Methods: We present a unique case of a pediatric patient with sagittal craniosynostosis who previously underwent a modified pi procedure and later developed resynostosis of the sagittal suture and secondary synostosis of the bicoronal sutures. We subsequently performed total cranial vault reconstruction with virtual surgical planning (VSP). Results: At his 31-month postoperative follow-up, he displayed normal head shape and denied any clinical signs of elevated intracranial pressures with a normal ophthalmological exam. Conclusions: The reoperation was successful with no significant postoperative complications noted. Performing geometric expansion with VSP to manage fusion of a previously open suture following primary treatment of sagittal synostosis should be considered within the armamentarium of operative options.

Considerable controversy exists around the optimal age on which to operate for craniosynostosis. This study aims to use data from the American College of Surgeons National Surgical Quality Improvement Program-Pediatric to assess the impact of operative age on hospital stay and outcomes. After excluding patients who underwent endoscopic cranial vault remodeling, a total of 3292 patients met inclusion criteria in the National Surgical Quality Improvement Program-Pediatric between 2012 and 2019. Median age at surgery was 300 days (interquartile range: 204-494). Patients between 0 and 6 months underwent the highest proportion of complex cranial vault remodeling, Current Procedural Terminology 61,558 ( n =44, 7.7%) and Current Procedural Terminology 61,559 ( n =317, 55.1%). White blood cell counts peaked in the 12 to 18 months group, and were lowest in the 24± months group. Hematocrit was lowest in the 0 to 6 months group and rose steadily to the 24± months group; the inverse pattern was found in platelet concentration, which was highest in the youngest patients and lowest in the oldest. Prothrombin time, international normalized ratio, and partial thromboplastin time were relatively consistent across all age groups. Younger patients had significantly shorter operating room times, which increased with patient age ( P <0.001). Younger patients also had significantly shorter length of stay ( P =0.009), though length of stay peaked between 12 and 18 months. There was a significantly lower rate of surgical site infection in younger patients, which occurred in 0.7% of patients 0 to 12 months and 1.0 to 3.0% in patients over 12 to 24± months. There was no significant difference in the average number of transfusions required in any age group ( P =0.961).

Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome. This study examined the impact of reoperation on neurocognitive outcome in children with nonsyndromic sagittal craniosynostosis using comprehensive neurocognitive testing.

Forty-seven school-age children (age 5-16 years) with nonsyndromic sagittal craniosynostosis who underwent whole-vault cranioplasty were included in this analysis. Participants were administered a battery of standardized neuropsychological testing to measure neurocognitive outcomes.

Thirteen of the 47 participants underwent reoperation (27.7%); 11 out of the 13 reoperations were minor revisions while 2 reoperations were cranioplasties. Reoperation rate was not statistically different between patients who had earlier surgery (at age ≤6 months) versus later surgery (at age >6 months) (P > 0.05). Nonreoperated patients who had only one later-in-life surgery did not perform statistically better than reoperated patients on any outcome measure of neurocognitive function, including IQ, academic achievement, visuomotor integration, executive function, and behavior. Comparing reoperated earlier surgery patients with nonreoperated later surgery patients, reoperated earlier surgery patients had higher full-scale and verbal IQ (P < 0.05), scored higher on word reading, reading comprehension, spelling, numerical operations, and visuomotor integration (P < 0.05), and had fewer indicators of suspected learning disabilities (P < 0.01) compared to nonreoperated later surgery patients.

Reoperation rate after whole vault cranioplasty was 27.7%, with few cases of repeat cranioplasty (4.2% of all patients). Reoperation was not associated with worse neurocognitive outcome. Reoperated earlier surgery patients in fact performed better in IQ, academic achievement and visuomotor integration when compared to nonreoperated later surgery patients.

Posterior cranial vault distraction is an important modality in the management of craniosynostosis. This surgical technique increases intracranial volume and improves cranial aesthetics. A single procedure is often inadequate in patients with complex multisuture craniosynostosis, as some will go on to develop intracranial hypertension despite the operation. Considering the negative effects of intracranial hypertension, some patients may warrant 2 planned distractions to prevent this scenario from ever occurring. Three patients with complex multiple-suture synostosis and severe intracranial volume restriction (occipital frontal head circumferences [OFCs] <1st percentile) were treated with 2 planned serial posterior cranial vault distractions at the institution between 2013 and 2018. Demographics, intraoperative data, and postoperative distraction data were collected. The OFC was recorded pre- and postdistraction, at 3- and 6-month follow-up appointments. Patients had a corrected average age of 18 weeks at the time of their initial procedure. There was an average of 38 weeks between the end of consolidation and the time for their 2nd distraction procedure. There was an average age of 79 weeks at the time of the 2nd procedure. All patients had a substantial increase in OFC and improvement of the posterior calvarium shape. The average increase in OFC was 5.2 cm after first distraction and 4.3 cm after 2nd distraction. No postoperative complications were encountered. Planned serial posterior cranial vault distraction is a safe and effective strategy for increasing intracranial volume, improving aesthetic appearance, and preventing the consequences of intracranial hypertension in patients with multisuture craniosynostosis and severe intracranial volume restriction.

Craniosynostosis is the premature fusion of 1 or more sutures that normally separate the bony plates of an infant's skull and occurs in about 1 in 2,000 to 2,500 live births. Primary or congenital craniosynostoses represent the majority of cases and consist of single-suture and multisuture synostoses. Multisuture synostoses are typically associated with distinct craniofacial syndromes, including Muenke syndrome, Apert syndrome, Crouzon syndrome, and Pfeiffer syndrome, and are thus categorized under syndromic craniosynostoses. Secondary causes of craniosynostoses include metabolic or hematologic disorders that affect bone metabolism and typically present much later than primary synostoses. The severity of the deformity and the presence of increased intracranial pressure dictate the need for early surgical intervention, prompting the importance of early recognition and timely referral. Infants with craniosynostosis are also at increased risk for neurodevelopmental impairment and thus require close follow-up and monitoring. The early recognition and referral of craniosynostosis is imperative for the optimization of management and minimization of potential neurologic impairments that may develop.

Surgical site infection (SSI) after open cranial vault reconstruction (CVR), while relatively uncommon, has received little attention in the literature to date. Here, the authors report our institution's experience with the perioperative management of infectious complications following CVR for craniosynostosis and present the first systematic review of the literature on this topic.

The authors performed a retrospective chart review for patients with syndromic and nonsyndromic craniosynostosis who underwent open CVR between 1990 and 2015 at a single institution to determine predictors of SSIs and a systematic review of studies that evaluated for SSI after CVR to ascertain the pooled incidence of SSI, common infectious organisms, and prophylactic antibiotic protocols.

Out of 548 primary and 163 secondary CVR cases at our institution, 6 primary reconstruction patients (1.09%) and 9 secondary reconstruction patients (5.52%) developed an SSI requiring extended hospital stay or readmission (P <0.001); overall infection rate was 2.11%. Streptococcus and Staphylococcus species were the most common organisms isolated. On multivariate analysis, syndromic status conferred more than 7 times greater odds of SSI (OR 7.7, P = 0.023). Pooled analysis of the literature yielded an overall SSI rate of 1.05% to 2.01%. In contrast to our institutional findings, the most common organisms reported were Candida species and Pseudomonas aeruginosa. The most common prophylactic antibiotic protocol was a first-generation cephalosporin for 24 to 72 hours post-operatively.

Patients undergoing secondary reconstruction have higher infection rates after CVR, and syndromic status is an important predictor of infection when controlling for other patient factors. Our literature review reveals nosocomial organisms to be the most commonly reported source of infection, though this is contrary to our institutional findings of skin flora being most common. Antibiotic prophylaxis varies institutionally.

The timing of operation for trigonocephaly is recommended before the age of 1 year. To evaluate the outcome of a consecutive series operated in a single centre, the current study was performed. The study cohort comprised a retrospective single-centre series of 20 patients with trigonocephaly operated before the age of 12 months. Intra- and postoperative clinical data, as well as aesthetic outcome measured by two-dimensional asymmetry index (2D AI) and correction of the curvature on lateral and pictures from above were evaluated. Furthermore, to evaluate postoperative aesthetic outcome, a new classification (Grades I-IV) was proposed. The mean age of the cohort was 7.9 months. 2D AI changed from 0.049 pre- to 0.03 postoperatively (P=0.165). The correction of the curvature in lateral pictures yielded a significant change on postoperative pictures (P=0.002) as well as on pictures from above. Using the proposed classification, 16/20 (80%) patients had a postoperative outcome of Grade I, 4/20 (20%) Grade II. In patients operated before the age of 12 months an excellent (Grade I, AI <0.03) or good aesthetic (Grade II, adequate parents' or surgeon's opinion) result could be reached in all patients.

Better understanding the incidence and patterns of elevated intracranial pressure (ICP) in patients with craniosynostosis may facilitate more timely intervention to alter neurocognitive outcomes. Spectral-domain optical coherence tomography (OCT) of the retina can non-invasively diagnose elevated ICP, and has demonstrated high sensitivity and specificity among patients with craniosynostosis. This study sought to characterize patterns of elevated ICP among patients with craniosynostosis.

Quantitative retinal parameters were prospectively assessed in both eyes of patients with craniosynostosis using spectral-domain OCT. Based on retinal OCT thresholds associated with elevated ICP (> 15 mmHg), subjects were assigned an OCT diagnosis of elevated or non-elevated ICP which was analyzed relative to clinical characteristics and craniosynostosis patterns.

Eighty subjects (aged 0.2-18 years) with craniosynostosis were enrolled; among these, 67 (84%) were nonsyndromic. OCT evaluation was performed at initial vault expansion in 56 (70%) patients. Among this subset, 27 (48%) patients had peri-papillary changes suggestive of elevated ICP, reflecting a 44% incidence in nonsyndromic and 83% in syndromic patients. The median age at initial vault expansion was higher among those with elevated ICP (11.1 months) than those without (7.8 months; p = 0.04.) Multi-suture synostosis was associated with changes consistent with elevated ICP in 9 (75%) patients compared with 18 (41%) with single suture synostosis (p = 0.05).

OCT of the retina produces a potentially sensitive indicator of ICP in craniosynostosis patients. Elevated ICP may be associated with number of involved sutures and older patient presentation, and refining appropriate "cutoffs" will be important as the technology becomes more widespread.