Development and assessment of competency-based neurotrauma course curriculum for international neurosurgery residents and neurosurgeons.

OBJECTIVE: Traumatic brain injuries (TBIs) are a significant disease burden worldwide. It is imperative to improve neurosurgeons' training during and after their medical residency with appropriate neurotrauma competencies. Unfortunately, the development of these competencies during neurosurgeons' careers and in daily practice is very heterogeneous. This article aimed to describe the development and evaluation of a competency-based international course curriculum designed to address a broad spectrum of needs for taking care of patients with neurotrauma with basic and advanced interventions in different scenarios around the world. METHODS: A committee of 5 academic neurosurgeons was involved in the task of building this course curriculum. The process started with the identification of the problems to be addressed and the subsequent performance needed. After this, competencies were defined. In the final phase, educational activities were designed to achieve the intended learning outcomes. In the end, the entire process resulted in competency and outcomes-based education strategy, including a definition of all learning activities and learning outcomes (curriculum), that can be integrated with a faculty development process, including training. Further development was completed by 4 additional academic neurosurgeons supported by a curriculum developer specialist and a project manager. After the development of the course curriculum, template programs were developed with core and optional content defined for implementation and evaluation. RESULTS: The content of the course curriculum is divided into essentials and advanced concepts and interventions in neurotrauma care. A mixed sample of 1583 neurosurgeons and neurosurgery residents attending 36 continuing medical education activities in 30 different cities around the world evaluated the course. The average satisfaction was 97%. The average usefulness score was 4.2, according to the Likert scale. CONCLUSIONS: An international competency-based course curriculum is an option for creating a well-accepted neurotrauma educational process designed to address a broad spectrum of needs that a neurotrauma practitioner faces during the basic and advanced care of patients in different regions of the world. This process may also be applied to other areas of the neurosurgical knowledge spectrum. Moreover, this process allows worldwide standardization of knowledge requirements and competencies, such that training may be better benchmarked between countries regardless of their income level.

Pfeiffer Syndrome Type 3 and Prune Belly Anomaly in a Female: Case Report and Review.

Background: Pfeiffer syndrome (PS) is an autosomal dominant entity characterized by craniosynostosis, broad thumbs, and preaxially deviated great toes. It is classified in three types depending on the severity. Type 1: Mild to moderate severity, Type 2: Severe presentation with cloverleaf skull, and Type 3: Severe craniosynostosis with prominent ocular proptosis. Association of Pfeiffer syndrome (PS) types 2 and 3 with "prune belly" anomaly has been reported in two non-related patients, one PS type 2 and one PS type 3. Case Report: we report the second case of PS type 3 in a female neonate with "prune belly" anomaly and prenatal exposure to Parvovirus B19. Conclusions: We suggest that the "prune belly" anomaly and others abdominal wall defects as omphalocele and scar-type defects may be included as a feature in PS type 2 and 3.

Pediatric Tonsil Cancer: A National and Institutional Perspective.

OBJECTIVE: To evaluate childhood and adolescent tonsil cancer incidence and to identify the clinical characteristics indicative of those patients who would benefit from urgent operative intervention. STUDY DESIGN: The Surveillance, Epidemiology and End Results 18 database, inclusive of national cancer statistics from 1973 to 2013, provided quantitative tonsil cancer incidence data. An institutional retrospective chart review of pediatric patients diagnosed with tonsil malignancy from January 2013 to January 2017 identified supplementary qualitative clinical presentation information. RESULTS: The Surveillance, Epidemiology and End Results 18 database included 138 pediatric patients with tonsil cancer with an age-adjusted incidence rate of 0.021/100 000 patients per year. The majority of cases were unilateral (79.7%), and there was both a male and Caucasian predominance. Non-Hodgkin lymphoma (84.1%) was the most common malignancy, of which Burkitt lymphoma (31.1%), diffuse large B-cell lymphoma (26.8%), and follicular lymphoma (10.1%) were the most common subtypes. Five tonsillar malignancy patients were identified upon institutional chart review. The majority likewise had non-Hodgkin lymphoma and all shared a history of rapid tonsillar enlargement over ≤12 weeks. Significant tonsillar asymmetry was present in 4 patients. Four patients additionally exhibited prominent cervical lymphadenopathy. CONCLUSIONS: Pediatric tonsil cancer is rare, with non-Hodgkin lymphoma accounting for the majority of pediatric tonsillar malignancies. A high index of suspicion is appropriate in children who present with relatively rapid tonsil enlargement, tonsillar asymmetry characterized by a difference in tonsillar size of ≥2 degrees on the Brodsky scale, or concurrent prominent cervical lymphadenopathy.

Preferential associated anomalies in 818 cases of microtia in South America.

The etiology of microtia remains unknown in most cases. The identification of patterns of associated anomalies (i.e., other anomalies that occur with a given congenital anomaly in a higher than expected frequency), is a methodology that has been used for research into the etiology of birth defects. We conducted a study based on cases of microtia that were diagnosed from more than 5 million live (LB)- and stillbirths (SB) examined in hospitals participating in ECLAMC (Latin American Collaborative Study of Congenital Malformations) between 1967 and 2009. We identified 818 LB and SB with microtia and at least one additional non-related major congenital anomaly (cases) and 15,969 LB and SB with two or more unrelated major congenital anomalies except microtia (controls). A logistic regression analysis was performed to identify the congenital anomalies preferentially associated with microtia. Preferential associations were observed for 10 congenital anomalies, most of them in the craniofacial region, including facial asymmetry, choanal atresia, and eyelid colobomata. The analysis by type of microtia showed that for anomalies such as cleft lip and palate, macrostomia, and limb reduction defects, the frequency increased with the severity of the microtia. In contrast, for other anomalies the frequency tended to be the same across all types of microtia. Based on these results we will integrate data on the developmental pathways related to preferentially associated congenital anomalies for future studies investigating the etiology of microtia.

Longitudinal, three-dimensional analysis of head shape in children with and without deformational plagiocephaly or brachycephaly.

OBJECTIVES: To assess 3-dimensional (3D) changes in head shape in infancy and at age 18 months in children with and without plagiocephaly or brachycephaly. STUDY DESIGN: Using a longitudinal design, we evaluated head shape using 3D surface imaging. We compared the head shapes of children with (1) diagnosed deformational plagiocephaly or brachycephaly (cases; n=233); (2) unaffected controls, with no evidence of dysmorphology (n=167); and (3) affected controls, who despite having no previous diagnosis demonstrated skull dysmorphology on 3D surface imaging (n=70). RESULTS: Cases had greater skull flattening and asymmetry than unaffected controls at both time points, as did controls with skull dysmorphology. In all groups, head shapes became less flat and more symmetric over time. Among cases, symmetry improved slightly more for those who received orthotic treatment. CONCLUSIONS: Although head shape improves over time for children with deformational plagiocephaly or brachycephaly, skull dysmorphology persists relative to unaffected controls. Further research is needed to clarify the extent to which these differences are detectable to clinicians and lay observers.

New severity indices for quantifying single-suture metopic craniosynostosis.

OBJECTIVE: To describe novel severity indices with which to quantify severity of trigonocephaly malformation in children diagnosed with isolated metopic synostosis. METHODS: Computed tomographic scans of the cranium were obtained from 38 infants diagnosed with isolated metopic synostosis and 53 age-matched control patients. Volumetric reformations of the cranium were used to trace two-dimensional planes defined by the cranium-base plane and well-defined brain landmarks. For each patient, novel trigonocephaly severity indices (TSI) were computed from outline cranium shapes on each of these planes. The metopic severity index based on measurements of interlandmark distances was also computed and a receiver operating characteristic analysis used to compare the accuracy of classification based on TSIs versus that based on the metopic severity index. RESULTS: The proposed TSIs are a sensitive measure of trigonocephaly malformation that can provide a classification accuracy of 96% with a specificity of 95%, in contrast with 82% of the metopic severity index at the same specificity level. CONCLUSIONS: We completed exploratory analysis of outline-based severity measurements computed from computed tomographic image planes of the cranium. These TSIs enable quantitative analysis of cranium features in isolated metopic synostosis that may not be accurately detected by analytic tools derived from a sparse set of traditional interlandmark and semilandmark distances.

Maternal and infant characteristics associated with prone and lateral infant sleep positioning in Washington state, 1996-2002.

OBJECTIVE: To identify factors predictive of either lateral or prone infant sleep positioning. STUDY DESIGN: We used data for 11340 mother-infant pairs from the Pregnancy Risk Assessment Monitoring System for infants born in Washington State, 1996 to 2002. We used predictive modeling to identify statistically significant (P < .05) predictors of lateral and prone sleep positioning. RESULTS: Factors associated with both high-risk sleep positions included infant's year of birth, maternal race and ethnicity, maternal county of residence, and maternal parity. Mother's being US-born (versus foreign-born) and male infant sex were predictive only of prone sleep positioning. Having Medicaid as primary insurance, receipt of government benefits, low infant gestational age, and low birth weight were predictive only of lateral sleep positioning. CONCLUSIONS: Factors predictive of either high-risk sleep position should be considered when devising public health intervention strategies for the prevention of SIDS.

The morphogenesis of wormian bones: a study of craniosynostosis and purposeful cranial deformation.

Wormian bones are accessory bones that occur within cranial suture and fontanelles, most commonly within the posterior sutures. They occur more frequently in disorders that have reduced cranial ossification, hypotonia or decreased movement, thereby resulting in deformational brachycephaly. The frequency and location of wormian bones varies with the type and severity of cranial deformation practiced by ancient cultures. We considered the hypothesis that the pathogenesis of wormian bones may be due to environmental variations in dural strain within open sutures and fontanelles. In order to explore this further, we measured the cephalic index (CI) in 20 purposefully deformed pre-Columbian skulls: 10 from Chichen Itza, Mexico, and 10 from Ancon, Peru, as well as 20 anatomically normal skulls used for medical school anatomy classes. We tested for a direct correlation between the CI and the number of wormian bones in skulls with varying degrees of brachycephalic cranial deformation and found no significant correlation. When the CI was grouped into three categories (normal (CI < 81), brachycephalic (CI 81-93), and severely brachycephalic (CI > 93)) there was a trend toward increasing number of wormian bones as the skull became more brachycephalic (P = 0.039). A second part or our study tabulated the frequency and location of large wormian bones (greater than 1 cm) in 3-dimentional computerized tomography (3D-CT) scans from 207 cases of craniosynostosis and compared these data with published data on 485 normal dry skulls from a manuscript on wormian bones by Parker in 1905. Among cases of craniosynostosis, large wormian bones were significantly more frequent (117 out of 207 3D-CT scans) than in dry skulls (131 out of 485). There was a 3.5 greater odds of developing a wormian bone with premature suture closure (P < 0.001). Midline synostosis, specifically metopic or sagittal synostosis, has more wormian bones in the midline, whereas unilateral lambdoidal or coronal synostosis more often had wormian bones on the contralateral side. Taken together, these data suggest that wormian bones may arise as a consequence of mechanical factors that spread sutures apart and affect dural strain within sutures and fontanelles.