Positional Skull Deformities

Abstract

Background: Especially in the first 6 months of life, skull deformities manifesting as a uni- or bilateral flattening of the occiput often give rise to questions of differential diagnosis and potential treatment. In this review, the authors summarize the current understanding of risk factors for this condition, and the current state of the relevant diagnostic assessment and options for treatment.

Methods: The recommendations given in this selective review of the literature are based on current studies and on existing guidelines on the prevention of sudden infant death, the recommendations of the German Society for Pediatric Neurology (Deutsche Gesellschaft für Neuropädiatrie), and the American guidelines on the treatment of positional plagiocephaly in infancy.

Results: Pre-, peri-, and postnatal risk factors can contribute to the development of positional skull deformities. These deformities can be diagnosed and classified on the basis of their clinical features, supplemented in unclear cases by ultrasonography of the cranial sutures. The putative relationship between positional skull deformities and developmental delay is currently debated. The main preventive and therapeutic measure is parent education to foster correct positioning habits (turning of the infant to the less favored side; prone positioning on occasion when awake) and beneficial stimulation of the infant (to promote lying on the less favored side). If the range of motion of the head is limited, physiotherapy is an effective additional measure. In severe or refractory cases, a skull orthosis (splint) may be useful.

Conclusion: The parents of children with positional skull deformities should be comprehensively informed about the necessary preventive and therapeutic measures. Treatment should be initiated early and provided in graded fashion, according to the degree of severity of the problem. Parental concern about the deformity should not be allowed to lead to a rejection of the reasonable recommendation for a supine sleeping position.

Figure 1

Classification of positional plagiocephaly and brachycephaly according to Argenta (20)

Figure 2

a) Schematic depiction of cephalometric measurements (see also eFigure 1). The solid line shows the measurement of the cranial vault asymmetry (CVA) according to Moss and Mortenson et al. (e5, e6), based on the difference between the largest and smallest diagonal diameter. The dotted line shows the measurement of the cranial vault asymmetry index (CVAI) according to Loveday et al. (e7), based on two diagonals that are both angled at 30° to the mid-sagittal plane. b–f) Stereophotogrammetric images (top view) with differing cranial vault asymmetry (CVA). Even though the image cannot visualize all clinical signs, compensatory prominence of the forehead and compensatory widening of the skull with increasing degrees of severity are clearly recognizable.

Figure 3

Child wearing cranial remolding orthosis, Arrows show space for growth

eFigure 1

Cephalometric measurement of the diagonal diameter by means of pelvimetry

eFigure 2

Clinical distinction between right-sided positional deformational plagiocephaly (DP) and right-sided unilateral lambdoid suture synostosis (LS) Left: DP – top view shows parallelogram-like shift; back view shows normal shaped head Right: LS – top view shows trapezoid shaped head; back view shows parallelogram shaped head