Evaluation of the Developmental Assessment for Intervention Manual (DAIM) for developmental screening in high-risk infants at 12 months of corrected age.

Early detection of developmental delay provides opportunities for early intervention. The Developmental Assessment for Intervention Manual (DAIM) is the simplified screening tool for detection of developmental delay in Thai young infants implemented country-wide by the Ministry of Public Health since 2015. Unlike standard assessment tool, DAIM can be obtained by lay staff or parents and takes less time. We aimed to evaluate the accuracy of the DAIM for detection of developmental delay among high-risk infants. This single center prospective study was conducted from May, 2017 to December, 2021. The infants with corrected aged of 12 months with history of birth asphyxia or low birth weight (<2500 g) who visited a tertiary hospital for developmental assessment were invited to participate the study. The four developmental domains (gross motor, fine motor, receptive language, and expressive language) were assessed by Bayley-III and followed by DAIM in the same visit. The 126 infants were enrolled, 50% were male. Using Bayley-III score < 7 as reference, the sensitivity for gross motor domain, fine motor domain, receptive language and expressive language were 83.3%, 57.1%, 55% and 19.2%, respectively. Specificity for each domain was 75.4%, 88.2%, 74.5% and 92%. Positive predictive value for each domain was 26.3%, 22.2%, 28.9% and 38.5%. Negative predictive value (NPV) for each domain was 97.7%, 97.2%, 89.8% and 81.4%. With high sensitivity and NPV of DAIM in motor domain, but low in expressive language domains, using DAIM alone in high-risk infants can detect motor delay well but will miss many infants with expressive language delaye.

Children Treated for Nonsyndromic Craniosynostosis Exhibit Average Adaptive Behavior Skills with Only Minor Shortcomings.

BACKGROUND: Adaptive behavior skills are important when assessing cognitive functions related to daily life; however, few studies have assessed these skills in patients treated for nonsyndromic craniosynostosis. In this study, the authors assessed the adaptive behavior skills of children treated for craniosynostosis and examined whether their outcomes are related to surgical technique. METHODS: The Adaptive Behavior Assessment System, 2nd Edition, parent report was used for children (age, 7 to 16 years) treated for sagittal (n = 41), metopic (n = 24), and other rare synostoses (n = 8). Background data, including intelligence quotient, were controlled for confounders. RESULTS: All evaluated children treated for craniosynostosis were estimated as lower in all aspects of adaptive behavior skills (full-scale, conceptual, social, and practical composites; effect size, 0.36 to 0.44) as compared with norms. The sagittal group showed shortcomings in social composite (effect size, 0.48) and subscales measuring self-care and self-direction, although no difference was observed between spring-assisted surgery and pi-plasty regarding outcomes of adaptive behavior skills. In addition, children treated for metopic synostosis showed results indicating shortcomings with adaptive behavior according to the full-scale, conceptual, and social composites (effect size, 0.53 to 0.61) relative to norms. Furthermore, attrition analysis revealed no significant differences between responders (rate, 80.2 percent) and nonresponders. CONCLUSION: These results found that children treated for craniosynostosis display average adaptive behavior skills, and that the two surgical techniques used to treat sagittal synostosis did not differ in their behavioral outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Neurodevelopmental outcomes after neonatal caffeine therapy.

Improving the adverse neurodevelopmental outcomes associated with prematurity is a priority. In the large international Caffeine for Apnea of Prematurity trial, caffeine improved survival without neurodevelopmental disability at 18 months and demonstrated long term safety up to 11 years. Caffeine is an adenosine receptor antagonist with effects on the brain, lung and other systems. The benefits of caffeine may be primary neuroprotection or reduction of risk factors for impairment, especially bronchopulmonary dysplasia. The effects of caffeine vary with age and dose. Animal data show risks of loss of neuronal protection from hypoxia. Treatment with earlier and higher dose caffeine may be beneficial but concerns remain.

Is autism driven by epilepsy in infants with Tuberous Sclerosis Complex?

OBJECTIVE: To evaluate the relationship between age at seizure onset and neurodevelopmental outcome at age 24 months in infants with TSC, as well as the effect on neurodevelopmental outcome of early versus conventional treatment of epileptic seizures with vigabatrin (80-150 mg/kg/day). METHODS: Infants with TSC, aged ≤4 months and without previous seizures were enrolled in a prospective study and closely followed with monthly video EEG and serial standardized neurodevelopmental testing (Bayley Scales of Infant Development and Autism Diagnostic Observation Schedule). RESULTS: Eighty infants were enrolled. At the age of 24 months testing identified risk of Autism Spectrum Disorder (ASD) in 24/80 children (30.0%), and developmental delay (DD) in 26/80 (32.5%). Children with epilepsy (51/80; 63.8%) had a higher risk of ASD (P = 0.02) and DD (P = 0.001). Overall, no child presented with moderate or severe DD at 24 months (developmental quotient < 55). In 20% of children abnormal developmental trajectories were detected before the onset of seizures. Furthermore, 21% of all children with risk of ASD at 24 months had not developed seizures at that timepoint. There was no significant difference between early and conventional treatment with respect to rate of risk of ASD (P = 0.8) or DD (P = 0.9) at 24 months. INTERPRETATION: This study confirms a relationship between epilepsy and risk of ASD/DD. However, in this combined randomized/open label study, early treatment with vigabatrin did not alter the risk of ASD or DD at age 2 years.

Radiographic Severity of Metopic Craniosynostosis Correlates with Long-Term Neurocognitive Outcomes.

BACKGROUND: Reports of neurodevelopmental delays in adolescents with metopic craniosynostosis have ranged from 15 to 61 percent. Previously, event-related potentials have correlated preoperative radiographic severity with language deficiencies in infancy. This study sought to characterize neurocognitive testing at cranial maturity and correlate outcomes to preoperative radiographic severity. METHODS: Patients diagnosed with metopic craniosynostosis who underwent surgical correction in infancy completed a neurodevelopmental battery evaluating age-normalized intelligence quotient, academic achievement, and visuomotor integration. Data were stratified by preoperative endocranial bifrontal angle (moderate, >124 degrees; severe, <124 degrees). Multiple variable regression was used to control measured intelligence and achievement for age at surgery, age at testing, parental education, and income. Significance was set at p < 0.05. RESULTS: Twenty patients completed neurodevelopmental testing. Mean intelligence quotient was 111.7 ± 13 and academic achievement was similar to national averages (word reading, 53.4 percent; reading comprehension, 53.4 percent; reading composite, 53.5 percent; spelling, 44 percent; and math, 52.9 percent). Radiographic measurements revealed 36 percent of patients with moderate phenotype and 64 percent with severe. Patients with severe phenotypes had lower intelligence quotient measures and scored more poorly in every academic measure tested. Word reading (113 versus 95; p = 0.035) and reading composite (109 versus 98; p = 0.014) reached significance. CONCLUSIONS: Overall, cranial mature patients with metopic craniosynostosis had above average intelligence quotient and academic achievement near the national mean. Long-term neurocognitive function was correlated to preoperative radiographic severity in metopic craniosynostosis, with more severe cases performing worse. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Avaliação do desenvolvimento motor infantil e sua associação com a vulnerabilidade social / Evaluación del desarrollo motor infantil y su asociación con la vulnerabilidad social / Evaluation of child motor development and its association with social vulnerability

RESUMO O objetivo deste estudo foi avaliar o desenvolvimento motor de crianças de quatro a 17 meses e investigar sua associação com fatores de risco sociodemográficos. Estudo transversal, descritivo, composto por crianças de quatro a 17 meses provenientes da unidade de internação pediátrica de um hospital público de Porto Alegre (RS), clinicamente estáveis e com alta breve prevista. Para a avaliação dos fatores de risco sociodemográficos foi utilizado um questionário elaborado pelas pesquisadoras, que abordou fatores biológicos, sociais e ambientais. Para a avaliação do desenvolvimento motor foi utilizada a Alberta Infant Motor Scale na versão traduzida, adaptada e validada para a população brasileira. Para a análise estatística foi utilizado o teste t de Student e o teste qui-quadrado, com nível de significância de 5% (p≤0,05). De um total de 110 crianças avaliadas, o desempenho motor se mostrou aquém do esperado em mais da metade delas (63,6%, n=70). Houve associação estatisticamente significativa entre o desenvolvimento motor e vacinas atrasadas (p=0,005), convivência com tabagistas em casa (p=0,047) e recebimento de benefício socioeconômico (p=0,036). Conclui-se que esses fatores sociais podem estar associados a fatores de risco ao desenvolvimento motor de crianças de quatro a 17 meses.

RESUMEN El presente estudio tuvo el objetivo de evaluar el desarrollo motor de niños de 4 a 17 meses de edad e investigar su asociación con factores de riesgo sociodemográficos. Es un estudio transversal, descriptivo, en el cual participaron niños de 4 a 17 meses de la unidad de hospitalización pediátrica de un hospital público en Porto Alegre (Brasil), clínicamente estables y con la espera de recibir el alta pronto. Para la evaluación de los factores de riesgo sociodemográficos, se utilizó un cuestionario desarrollado por los investigadores, que abordó los factores biológicos, sociales y ambientales. Para la evaluación del desarrollo motor, se utilizó la Alberta Infant Motor Scale en la versión traducida, adaptada y validada para la población brasileña. En el análisis estadístico, se aplicaron la prueba t de Student y la prueba chi-cuadrado, con un nivel de significación del 5% (p≤0,05). De 110 niños evaluados, más de la mitad de ellos (63,6%, n=70) tuvieron rendimiento motor inferior a lo esperado. Hubo una asociación estadísticamente significativa entre el desarrollo motor y las vacunas tardías (p=0,005), la convivencia con fumadores en el hogar (p=0,047) y el recibimiento de beneficios socioeconómicos (p=0,036). Se concluye que estos factores sociales pueden estar asociados con factores de riesgo para el desarrollo motor de niños de 4 a 17 meses.

ABSTRACT This study aimed to evaluate the motor development of children aged four to 17 months and investigate its association with sociodemographic risk factors. This is a cross-sectional descriptive study conducted with clinically stable children aged four to 17 months from the pediatric inpatient unit of a public hospital in Porto Alegre, RS, and whose hospital discharge would happen soon. For the evaluation of sociodemographic risk factors, a questionnaire developed by the researchers was used which addressed biological, social and environmental factors. The Alberta Infant Motor Scale (AIMS), in its version translated, adapted and validated to Brazilian Portuguese, was used in the evaluation of motor development. In statistical analysis, Student's t-test and Chi-square test were used with significance level of 5% (p≤0.05) for all tests. From a total of 110 evaluated children, motor performance was lower than expected in more than half of them (63.6%, n=70). Motor development presented statistically significant associations with delayed vaccines (p=0.005), cohabitation with smokers (p=0.047), and receiving socioeconomic benefits (p=0.036). In conclusion, social factors such as delayed vaccines, cohabitation with smokers and receiving socioeconomic benefits may be associated with risk factors related to motor development of children aged four months to 17 months old.

SMAD6 Genotype Predicts Neurodevelopment in Nonsyndromic Craniosynostosis.

BACKGROUND: De novo or rare transmitted mutations in the SMAD6 gene affect 7 percent of midline nonsyndromic synostosis patients. This study aimed to determine the neurocognitive sequelae of SMAD6 synostosis. METHODS: Nonsyndromic synostosis patients 6 years or older with SMAD6 mutations and non-SMAD6 nonsyndromic synostosis controls were recruited. All patients completed a double-blinded neurodevelopmental battery (i.e., Wechsler Fundamentals, Wechsler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental test), and parents/guardians completed behavioral surveys (Behavior Rating Inventory of Executive Function and Behavior Rating System for Children). RESULTS: Twenty-eight patients participated: 10 known SMAD6 patients (average age, 10 years; 1 female; eight metopic and two sagittal; nine treated with cranial vault remodeling and one treated with strip craniectomy) and 18 non-SMAD6 controls (age, 9.5 years; three female; 12 metopic and six sagittal; 17 treated with cranial vault remodeling and one treated with strip craniectomy). There were no differences between any demographics. Testing age, surgical age, parental education, and household income correlated with cognition (p < 0.05). After controlling for these factors, SMAD6 patients performed worse on numerical operations (p = 0.046), performance intelligence quotient (p = 0.018), full-scale intelligence quotient (p = 0.010), and motor coordination (p = 0.043) compared to age/race/gender/synostosis/operation-matched controls. On behavioral surveys, SMAD6 patients scored worse on 14 assessments, including aggression, communication, and behavior. CONCLUSIONS: This prospective double-blinded study revealed that neuropsychiatric development of nonsyndromic synostosis may be under genetic control. SMAD6 mutations led to poorer mathematics, performance intelligence quotient, full-scale intelligence quotient, and motor coordination, even after controlling for exogenous factors. Genetic testing may be critical for advocating early adjunctive neurodevelopmental therapy. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Risk and protective factors for child development: An observational South African birth cohort.

BACKGROUND: Approximately 250 million (43%) children under the age of 5 years in low- and middle-income countries (LMICs) are failing to meet their developmental potential. Risk factors are recognised to contribute to this loss of human potential. Expanding understanding of the risks that lead to poor outcomes and which protective factors contribute to resilience in children may be critical to improving disparities. METHODS AND FINDINGS: The Drakenstein Child Health Study is a population-based birth cohort in the Western Cape, South Africa. Pregnant women were enrolled between 20 and 28 weeks' gestation from two community clinics from 2012 to 2015; sociodemographic and psychosocial data were collected antenatally. Mothers and children were followed through birth until 2 years of age. Developmental assessments were conducted by trained assessors blinded to background, using the Bayley-III Scales of Infant and Toddler Development (BSID-III), validated for use in South Africa, at 24 months of age. The study assessed all available children at 24 months; however, some children were not able to attend, because of loss to follow-up or unavailability of a caregiver or child at the correct age. Of 1,143 live births, 1,002 were in follow-up at 24 months, and a total of 734 children (73%) had developmental assessments, of which 354 (48.2%) were girls. This sample was characterised by low household employment (n = 183; 24.9%) and household income (n = 287; 39.1% earning 1 domain affected, and 75 (10.2%) had delay in all domains. Bivariate and multivariable analyses revealed several factors that were associated with developmental outcomes. These included protective factors (maternal education, higher birth weight, and socioeconomic status) and risk factors (maternal anaemia in pregnancy, depression or lifetime intimate partner violence, and maternal HIV infection). Boys consistently performed worse than girls (in cognition [ß = -0.74; 95% CI -1.46 to -0.03, p = 0.042], receptive language [ß = -1.10; 95% CI -1.70 to -0.49, p < 0.001], expressive language [ß = -1.65; 95% CI -2.46 to -0.84, p < 0.001], and fine motor [ß = -0.70; 95% CI -1.20 to -0.20, p = 0.006] scales). There was evidence that child sex interacted with risk and protective factors including birth weight, maternal anaemia in pregnancy, and socioeconomic factors. Important limitations of the study include attrition of sample from birth to assessment age and missing data in some exposure areas from those assessed. CONCLUSIONS: This study provides reliable developmental data from a sub-Saharan African setting in a well-characterised sample of mother-child dyads. Our findings highlight not only the important protective effects of maternal education, birth weight, and socioeconomic status for developmental outcomes but also sex differences in developmental outcomes and key risk and protective factors for each group.

Laparoscopic ligation of a congenital extrahepatic portosystemic shunt for children with hyperammonemia: a single-institution experience.

PURPOSE: A congenital extrahepatic portosystemic shunt (CEPS) associated with hyperammonemia requires occlusion of the shunt vessels. We evaluated the effectiveness and safety of laparoscopic ligation of a CEPS in children with hyperammonemia. METHODS: The subjects of this retrospective study were seven children with hyperammonemia who underwent laparoscopic ligation of a CEPS. Their median age was 5.2 years (range 1-16 years). Before the laparoscopic procedure, a catheter was inserted through the femoral vein and placed in the portal vein via the shunt vessel. The shunt vessel was dissected and taped laparoscopically. After measuring the portal vein pressure under temporal occlusion, the shunt vessels were ligated. RESULTS: The types of shunts according to location were patent ductus venosus (n = 2), splenorenal shunt (n = 2), gastrorenal shunt (n = 2), and superior mesenteric vein-inferior vena cava shunt (n = 1). Laparoscopic ligation of the shunt vessel was completed uneventfully in all patients. The median portal vein pressure was 19 mmHg after ligation. The median preoperative blood ammonia level was 94 µg/dL (range 71-259 µg/dL), which decreased after ligation in all patients. There was no incidence of postoperative liver failure. CONCLUSION: Laparoscopic ligation of a CEPS is safe and effective for children with hyperammonemia.

Building New York State Centers of Excellence in Children's Environmental Health: A Replicable Model in a Time of Uncertainty.

Public health programs may be seriously affected in periods of federal retrenchment. During these times, state-based strategies provide an alternate pathway for advancing public health.A 12-year campaign to secure state support for a network of Centers of Excellence in Children's Environmental Health (CEH) promoting health of children across New York State is described. It was driven by rising rates of asthma, birth defects, developmental disorders, and other noncommunicable diseases in children; growing evidence associating hazardous environmental exposures with these conditions; and recognition that federal resources in CEH are insufficient.Critical campaign elements were (1) formation of a statewide coalition of academic health centers, health care providers, public health officials, community advocates, and other stakeholders; (2) bipartisan collaborations with legislative champions and government leaders; (3) assessment of the burden of developmental disorders and noncommunicable diseases associated with environmental exposures among children; (4) maps documenting the presence of environmental hazards in every county statewide; (5) iterative charting of a changing political landscape; and (6) persistence. The 2017 award of a 5-year, $10 million contract to establish Centers of Excellence in CEH demonstrates the value of this statewide strategy.

Behavioral and neurodevelopmental outcome of children after maternal allopurinol administration during suspected fetal hypoxia: 5-year follow up of the ALLO-trial.

OBJECTIVE: To evaluate the long-term neurodevelopmental and behavioral outcome of antenatal allopurinol treatment during suspected fetal hypoxia. STUDY DESIGN: We studied children born from women who participated in a randomized double-blind placebo controlled multicenter study (ALLO-trial). Labouring women in whom the fetus was suspected to have fetal hypoxia were randomly allocated to receive allopurinol or placebo. At 5 years of age, the children were assessed with 2 parent reported questionnaires, the Ages and Stages Questionnaire (ASQ) and the Child Behavior Checklist (CBCL). A child was marked abnormal for ASQ if it scored below 2 standard deviation under the normative mean of a reference population in at least one domain. For CBCL, a score above the cut-off value (95th percentile for narrowband scale, 85th percentile for broadband scale) in at least one scale was marked as abnormal. RESULTS: We obtained data from 138 out of the original 222 mildly asphyxiated children included in the ALLO-trial (response rate 62%, allopurinol n = 73, placebo n = 65). At 5 years of age, the number of children that scored abnormal on the ASQ were 11 (15.1%) in the allopurinol group versus 11 (9.2%) in the placebo group (relative risk (RR) 1.64, 95% confidence interval (CI): 0.64 to 4.17, p = 0.30). On CBCL 21 children (30.4%) scored abnormal in de allopurinol group versus 12 children (20.0%) in the placebo group (RR 1.52, 95% CI: 0.82 to 2.83, p = 0.18). CONCLUSION: We found no proof that allopurinol administered to labouring women with suspected fetal hypoxia improved long-term developmental and behavioral outcome. These findings are limited due to the fact that the study was potentially underpowered. TRIAL REGISTRATION: NCT00189007 Dutch Trial Register NTR1383.

Is Less Noise, Light and Parental/Caregiver Stress in the Neonatal Intensive Care Unit Better for Neonates?

In utero sensory stimuli and interaction with the environment strongly influence early phases of fetal and infant development. Extremely premature infants are subjected to noxious procedures and routine monitoring, in addition to exposure to excessive light and noise, which disturb the natural sleep cycle and induce stress. Non-invasive ventilation, measures to prevent sepsis, and human milk feeding improve short-term and long-term neurodevelopmental outcomes in premature infants. To preserve brain function, and to improve quality of life and long-term neurodevelopmental outcomes, the focus now is on the neonatal intensive care unit (NICU) environment and its impact on the infant during hospital stay. The objectives of this write-up are to understand the effects of environmental factors, including lighting and noise in the NICU, on sensory development of the infant, the need to decrease parental and caregiver stress, and to review existing literature, local policies and recommendations.

Impact of the Neonatal Resuscitation Program-Recommended Low Oxygen Strategy on Outcomes of Infants Born Preterm.

OBJECTIVE: To evaluate the impact of the Neonatal Resuscitation Program (NRP)-recommended low oxygen strategy (LOX) on neonatal morbidities, mortality, and neurodevelopmental outcomes in neonates born preterm. STUDY DESIGN: In March 2011, Parkland Hospital changed from a high oxygen strategy (HOX) of resuscitation with initial 100% oxygen and targeting 85%-94% oxygen saturation for delivery room resuscitation to a LOX with initial 21% oxygen and titrating oxygen to meet NRP-recommended transitional target saturations. Neonates ≤28 weeks' gestational age born between August 2009 and April 2012 were identified. In this retrospective, observational study, neonates exposed to LOX vs HOX were compared for short-term morbidity, mortality, and long-term neurodevelopmental outcomes. Regression analysis was performed to control for confounding variables. RESULTS: Of 199 neonates, 110 were resuscitated with HOX and 89 with LOX. Compared with HOX, neonates exposed to LOX had lower oxygen exposure in the delivery room (5.2 ± 1.5 vs 7.8 ± 2.8 [∑FiO2 × time min], P < .01), spent fewer days on oxygen (30 [5, 54] vs 46 [11, 82], P = .01), and had lower odds of developing bronchopulmonary dysplasia (aOR 0.4 [0.2, 0.9]). There was no difference in mortality (17 [20%] vs 20 [18%]), but neonates exposed to LOX had greater motor composite scores on Bayley Scales of Infant and Toddler Development-Third edition assessment (91 [85, 97] vs 88 [76, 94], P < .01). CONCLUSION: The NRP-recommended LOX strategy was associated with improved respiratory morbidities and neurodevelopmental outcomes with no increase in mortality. Prospective trials to confirm the optimal oxygen strategy for the resuscitation of neonates born preterm are needed.

Infantile Spasms and Injuries of Prematurity: Short-Term Treatment-Based Response and Long-Term Outcomes.

The association of infantile spasms and periventricular leukomalacia and/or intraventricular hemorrhage is well documented. Data regarding early treatment-based and long-term outcomes are limited. A retrospective chart review identified children with infantile spasms born prematurely (<37 weeks) with diagnoses of periventricular leukomalacia and/or intraventricular hemorrhage. Thirteen children were included. Median gestational age was 30 weeks and age of onset of infantile spasms was 8 months. Nine children had intraventricular hemorrhage, 10 had periventricular leukomalacia, and 6 children had both. Twelve of 13 children had resolution of spasms. In responders, the successful medication was adrenocorticotropic hormone (ACTH) in 7, topiramate in 3, and vigabatrin in 2. Follow-up after a median of 7.1 years found that all patients had developmental delay but only 1 had refractory epilepsy. Standard therapies (ACTH and vigabatrin) appeared to be more effective than other treatments. Developmental delay is common in children with periventricular leukomalacia / intraventricular hemorrhage and infantile spasms, but refractory epilepsy might be less frequent.

An infant and mother with severe B12 deficiency: vitamin B12 status assessment should be determined in pregnant women with anaemia.

The vitamin B12 status of infants depends on maternal B12 status during pregnancy, and during lactation if breastfed. We present a 9-month-old girl who was admitted to the metabolic unit for assessment of developmental delay. She was exclusively breastfed and the introduction of solids at 5 months was unsuccessful. Investigations revealed pancytopenia, undetectable B12 and highly elevated methylmalonic acid and homocysteine. Methylmalonic acid and homocysteine normalised following B12 injections. Marked catch-up of developmental milestones was noted after treatment with B12. Investigations of parents showed normal B12 in the father and combined B12 and iron deficiency in the mother. Maternal B12 deficiency, most likely masked by iron deficiency, led to severe B12 deficiency in the infant. Exclusive breastfeeding and a subsequent failure to wean exacerbated the infant's B12 deficiency leading to developmental delay. This case highlights the need for development of guidelines for better assessment of B12 status during pregnancy.

The Management of Trigonoscaphocephaly as a Result of Combined Metopic and Sagittal Synostosis.

BACKGROUND: The combination of sagittal and metopic synostosis is rare, resulting in a scaphocephalic shape, but with an absence of frontal bossing and therefore varying degrees of trigonocephaly and occipital prominence. Treatment is primarily surgical, with a combination of procedures to address both the scaphocephaly and trigonocephaly required involving multiple operations. The authors discuss their experience of treating combined trigonoscaphocephaly in a single-stage procedure and propose a management strategy based on the severity of the presenting deformity. METHODS: The Oxford Craniofacial Unit database was searched from inception in October of 2004 to August of 2013 to identify all patients with combined sagittal and metopic synostosis. Case notes were then manually searched to identify those patients who had true trigonoscaphocephaly. RESULTS: Of 2856 patients in the authors' database, a total of nine were identified as having had true trigonoscaphocephaly. Seven of these patients underwent a combined single-stage procedure with an average cephalic index of 68.7 percent preoperatively and 80.3 percent postoperatively. CONCLUSIONS: Management of trigonoscaphocephaly has been traditionally performed by multiple, staged surgical procedures. The authors propose that it can instead be managed in a single surgical procedure, with the choice of procedure determined by the severity of the deformity. If the deformity is mild to moderate with no occipital bullet, a combined fronto-orbital advancement remodeling and subtotal calvarial remodeling can be performed; however, if there is an occipital bullet, the authors propose the combination of fronto-orbital advancement remodeling and total calvarial remodeling performed in one operation with the patient turned from prone to supine intraoperatively. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.