Pharyngeal Arches, Chapter 1: Normal Development and Derivatives.

The pharyngeal arches form the cornerstone of the complex anatomy of the face and neck. These embryologic structures are the foundation of face and neck development, and anomalous growth can result in craniofacial abnormalities. Surgeons who manage head and neck pathology and pathoanatomy will invariably encounter conditions associated with aberrant pharyngeal arch anatomy, and a thorough understanding of the normal and pathological development of these important structures is paramount to accurate diagnosis and treatment. This manuscript is the first of a three-part educational series that addressed the pharyngeal/branchial arch embryology, development, nomenclature, and normal anatomy (Part I), pathologic anomalies of ear and neck derived from abnormal development of the arches (Part II), and different types of orofacial clefts, including Tessier clefts (Part III).

Sickle Cell Disease Association with Premature Suture Fusion in Young Children.

Sickle cell disease (SCD) leads to the formation of an atypical hemoglobin tetramer with reduced capacity to carry oxygen. Although correlation between SCD and craniosynostosis (CS) has been mentioned, these are mostly small series or case reports. This article aimed to study any correlation between these entities in a large pediatric population. Methods: We retrospectively reviewed head CT scans of SCD patients from 0 to 8 years of age who required a CT for issues unrelated to their head shape between 2012 and 2020. We excluded patients with known history of CS or any CS-related syndrome, hydrocephalus, shunt placement, history of cranial surgery, or any reported cerebral or cranial shape abnormality. Results: Ninety-four CT scans were analyzed. The mean age at imaging was 4.48 ± 2.30 years. CS prevalence in this cohort was 19.1%. Analysis between independent variables and patients with +CS showed that SCD-associated vasculopathy, first-degree relatives with SCD, and the use of folic acid had a statistically significant association with CS development. Conclusions: Approximately 20% of pediatric patients with SCD developed CS. This association was higher in those patients who had a family history of SCD, used folic acid, and had SCD-associated vasculopathy. While the clinical impact of these findings needs more extensive study, centers that manage patients with SCD should be aware of the relatively high concordance of these diagnoses, vigilantly monitor head shape and growth parameters, and understand the potential risks associated with unidentified or untreated CS.