KEY POINTS Parietal foramina are symmetric oval calvarial defects located near the parietal eminences, separated by a narrow bridge of bone. Diagnosis is aided by palpation, radiography, antenatal sonography, and magnetic resonance imaging. Parietal foramina can occur in isolation or as part of multiple congenital anomaly syndromes with autosomal dominant inheritance with variable expression and intrafamilial variability observed. Parietal foramina can manifest as cranium bifidum in…

KEY POINTS The anterior fontanel on rare occasions ossifies and forms a bony plate. An anterior frontal bone can be seen in normal infants as a variant or in association with craniosynostosis. The anterior fontanelle is an integral part of the pediatric exam, and any abnormalities such as delayed closure can be associated with various pathologic conditions. Diagnosis is important to differentiate it from craniosynostosis or…

KEY POINTS Vertex craniotabes is characterized by diminished mineralization in the superior portions of the parietal bones, caused by prolonged forceful pressure on vertex during birth. The affected region of the calvarium feels soft to palpation and may exhibit a “ping-pong” sensation on compression. The sides of the calvarium and other skeletal regions are typically unaffected, helping to differentiate it from generalized mineralization problems. The condition…

KEY POINTS Vertex birth molding is the bony adjustments within the cranial vault and soft tissue swelling due to external fetal head compression during delivery. Factors such as fetal head position and size, gestational age, maternal pelvic shape, and uterine contractions influence the degree of molding. During vertex molding, the frontal and occipital bones slide under the parietal bones, elongating the occipitofrontal diameter and reducing the…

KEY POINTS Multiple sutural synostosis can result from a profound degree of prenatal head constraint or genetic mutations in genes associated with a syndromic form of craniosynostosis. Differential diagnosis is important considering the natural history and prognosis of different syndromes. Elevated intracranial pressure is more common in multiple sutural synostosis compared to single suture synostosis. Symptoms include a cloverleaf-shaped head, increased intracranial pressure signs, optic atrophy,…

KEY POINTS Lambdoidal craniosynostosis is often an isolated anomaly but can be associated with muscular torticollis or an abnormal fetal lie/birth presentation. Differential diagnosis includes nonsynostotic deformational posterior plagiocephaly, synostotic anterior plagiocephaly, and other forms of craniosynostosis. Unilateral lambdoidal craniosynostosis results in the protrusion of the ipsilateral mastoid bone, palpable sutural ridging, occipital flattening, downward displacement of the auricle, and trapezoidal cranial asymmetry. Early recognition and…

KEY POINTS The metopic suture is the first cranial suture to close and normally closes by 8 to 9 months. Mild degrees of metopic ridging occur frequently at birth, but unless there is progressive distortion of the orbits, it usually resolves. Metopic synostosis is characterized by trigonocephaly, lateral supraorbital retrusion, and hypotelorism. Trigonocephaly can occur as an isolated anomaly or as part of a syndrome or…

KEY POINTS Coronal craniosynostosis may occur as an isolated finding or as part of several genetic disorders. Coronal craniosynostosis can be unilateral or bilateral and can be caused by early descent of the fetal head into a constraining position, or aberrant fetal lie, or constraint within a bicornuate uterus. Late gestational constraint may cause an appearance similar to coronal synostosis at birth, but spontaneous restitution occurs…

KEY POINTS Sagittal craniosynostosis limits lateral cranial expansion, with progressive frontal and/or occipital prominence and ridging along the mid-posterior portion of the skull. Sagittal synostosis can result from fetal head constraint secondary to factors such as twining, oligohydramnios, or early descent of the fetal head into the maternal pelvis with fetal head entrapment, resulting in biparietal constraint. Various surgical techniques have been used to correct sagittal…

KEY POINTS Differentiating between craniosynostosis and primary cranial deformation is critical to determining the proper mode of treatment. It is important for pediatric and primary care providers to know the normal range and timing of fontanel and cranial suture closure. Craniosynostosis can occur due to prenatal limitation of normal growth stretch across a suture during late fetal life or a deficit in brain growth, among other…

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KEY POINTS Brachycephaly is defined as a shortened, wide head, often caused by constant supine positioning during infancy. Infants should be placed on their backs for sleep but on their stomachs during awake time under adult supervision to encourage neck rotation and motor skills. Routine use of repositioning and tummy time during the first 6 weeks and thereafter should prevent brachycephaly. The “Back to Sleep” campaign…

KEY POINTS Sudden infant death syndrome (SIDS) has a multifactorial and heterogeneous etiology with a triple-risk hypothesis proposed: (1) exogenous/environmental stressor, (2) early infancy, and (3) an underlying vulnerability or genetic susceptibility. Metabolic conditions have historically been thought to be a substantial proportion of SIDS cases, but many are now detected early through newborn screening programs. Infant sleeping position has emerged as a major risk factor…

KEY POINTS Deformational plagiocephaly is most common in first born, large babies, breech presentation, and multiple-gestation infants. Factors associated with asymmetric head deformation include a constraining intrauterine environment, clavicular fracture, cervical/vertebral abnormalities, and incomplete bone mineralization. Nonsynostotic , deformational , and positional have been used to distinguish plagiocephaly without synostosis. Deformational plagiocephaly is not associated with premature closure of cranial sutures. The diagnosis can be challenging…

KEY POINTS Congenital muscular torticollis (CMT) must be distinguished from acquired torticollis and underlying structural torticollis (from cervical vertebral anomalies). It is most often caused by a congenital asymmetry in the length and/or strength of the sternocleidomastoid (SCM) muscles. CMT may be caused by early fetal head descent, an abnormal fetal position, venous occlusion, and/or trauma to the muscle during difficult deliveries. Other postnatal causes of…

KEY POINTS Congenital compression of the chin can limit jaw growth before birth and result in asymmetric jaw retrusion. An asymmetric mandible can be secondary to an altered cranial base or a condylar abnormality secondary to genetic or environmental factors. Skin redundancy under the chin suggests pressure-induced growth restriction of the jaw. Asymmetric mandibular growth deficiency is often associated with muscular torticollis and can indicate associated…

KEY POINTS Congenital ear deformities are common, with an incidence as high as 58% of all newborns. Congenital ear anomalies are divided into malformations and deformations. Malformations involve chondro-cutaneous defects, while deformations involve mechanically distorted architectural anomalies of the newborn’s pinna. Crumpled and distorted ears can be secondary to late gestational deformational forces. Apparent ear enlargement on the side opposite the muscular torticollis is common. Many…

KEY POINTS Nasal deformities can result from constraint or a birth-related trauma in newborns. Nasal septum deviations must be distinguished from deformities of the whole nose. Spontaneous correction of nasal deformities can occur within the first 3 months of life. Nasal obstruction in infants may require intervention, especially if it leads to significant respiratory distress and oxygen desaturation. Failure to recognize and treat dislocation may lead…

KEY POINTS External fetal constraint rarely causes persistent scoliosis but can result in infantile idiopathic scoliosis that responds to physical therapy. Congenital scoliosis is caused by a failure of vertebral segmentation, which may lead to progressive spinal deformity with growth. Neuromuscular scoliosis is caused by central nervous system dysfunction, peripheral neuromuscular dysfunction, or combined sensory and motor dysfunction. Idiopathic scoliosis is divided into infantile (in children…

KEY POINTS Pectus excavatum and pectus carinatum are the most common abnormalities of the thorax. The incidence of pectus excavatum is between 1 and 8 per 1000 persons, and pectus carinatum is two to four times less frequent than pectus excavatum. Pectus excavatum is thought to result from overgrowth of costal cartilages, which becomes more apparent during the period of rapid skeletal growth in early adolescence.…