Effect of head orthoses on skull deformities in positional plagiocephaly: Evaluation of a 3-dimensional approach.

PURPOSE: The positional non-synostotic plagiocephaly represents a cranial asymmetry affecting all 3 dimensions. The aim of this study was to evaluate volumetric indices to assess the efficiency in improving non-synostotic cranial asymmetries in treatment with head orthoses. MATERIAL AND METHODS: A total of 96 infants were included in this observational retrospective study. The cohort was further divided into subgroups according to age of helmet supply (younger/older than 7.5 months) and duration of therapy (less/more than 150 days). With 3-dimensional photogrammetry data sets, the skull volume was separated into quadrants and set in relation to each other to create an Anterior Cranial Asymmetry Index (ACAI) and a Posterior Cranial Asymmetry Index (PCAI) as 3-dimensional parameters. RESULTS: Treatment with head orthoses led to a significant reduction of ACAI (p < 0.0001) and PCAI (p = 0.001). Cranial asymmetry was more severe in the occipital region and significantly improved mainly during the first 75 days with a 40.08% decrease of PCAI value in the short-term therapy in the younger treatment subgroup (p = 0.003). CONCLUSIONS: The introduced parameters sufficiently reproduce the improvement of asymmetry during helmet therapy, following the trend of already established parameters. Asymmetry was significantly improved in the occiput region, and helmet therapy was highly effective in younger infants and in the early treatment period.

Anterior Unilateral Plagiocephaly in Patient with Alagille Syndrome: Case Report.

BACKGROUND: The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon, Pfeiffer, Apert, or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, have shown an association with alterations in the development of cranial sutures, resulting in serious cosmetic defects and neurologic disorders. CASE DESCRIPTION: We report an exceptional case of a 30-month-old girl, a nephroblastoma survivor diagnosed with Alagille syndrome, who was referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the patient's age, we offered aggressive surgical treatment performing a new forehead harvested from the skull vertex with orbital rim reconstruction. CONCLUSIONS: Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only 3 cases have been described in the literature.

Head shape at age 36 months among children with and without a history of positional skull deformation.

OBJECTIVE In this study, the authors examined head shape through age 36 months for children with and without a history of positional plagiocephaly and/or brachycephaly (PPB). METHODS Infants with PPB (cases) were identified through a craniofacial clinic at the time of diagnosis. Infants without diagnosed PPB were identified through a participant registry. Clinician ratings of 3D cranial images were used to confirm the presence or absence of PPB. The cohort included 235 case infants (diagnosed PPB, confirmed with 3D imaging), 167 unaffected controls (no diagnosed PPB, no deformation detected), and 70 affected controls (no diagnosed PPB, discernible skull deformation). Participants were seen in infancy (age 7 months, on average) and again at ages 18 and 36 months. At each visit, automated 3D measures of skull deformation quantified posterior flattening and generated an absolute asymmetry score. The authors also used automated 2D measures to approximate overall asymmetry (approximate oblique cranial length ratio) and calculate the cephalic index. They used linear regression to compare cases to unaffected controls and to compare affected versus unaffected controls on all measures. They also calculated the proportion of children in each group with "persistent PPB," defined as one or more head shape measures above the 95th percentile relative to unaffected controls at 36 months. RESULTS Head shape became more rounded and symmetric for children with and without PPB, particularly between infancy and age 18 months. However, children with PPB continued to show greater skull deformation and asymmetry than unaffected controls at age 36 months. These differences were large in magnitude, ranging from 1 to 2 standard deviations (SDs), and in most (85.6%) of the cases, there was evidence of persistent PPB at 36 months. Similarly, although differences were more modest (i.e., 0.26-0.94 SD), affected controls continued to exhibit skull deformation on most measures relative to unaffected controls and approximately 30% had persistent PPB. Within the case group, head shape at 36 months was similar for untreated patients with PPB and for those who received helmet treatment and for patients with and without a history of torticollis. CONCLUSIONS Although head shape continues to improve, children with a history of skull deformation in infancy continue to exhibit measureable cranial flattening and asymmetry through age 36 months.

Squamosal Suture Synostosis: Incidence, Associations, and Implications for Treatment.

Squamosal suture craniosynostosis is thought to be a relatively rare entity. In the authors' experience, it is underreported in imaging examinations and the existing literature. The authors sought to determine the incidence of squamosal synostosis, whether it is increasing in frequency, and its relationship with synostosis of the major calvarial sutures.Patients undergoing computed tomography imaging for suspected craniosynostosis over a 15-year period were reviewed by a plastic surgeon and pediatric neuroradiologist. Patients with synostosis of the squamosal sutures were identified and involvement of additional sutures, gender, and the presence of a known syndromic diagnosis were recorded. Patients greater than 4 years of age or those with prior craniofacial surgery were excluded.One hundred twenty-five patients met inclusion criteria, 26 of whom had squamosal suture synostosis (26/125, 20.8%). Squamosal synostosis was found in isolation in 3 patients (3/26, 11.5%), with 1 additional major suture in 10 patients (10/26, 38.5%), and ≥2 major sutures in 13 patients (13/26, 50%). Squamosal synostosis was more common in patients with a syndromic diagnosis (11/26 syndromic, 15/99 nonsyndromic, P < 0.001). Eleven of 26 patients with squamosal synostosis were identified in the radiology report (42.3%).Craniosynostosis of the squamosal suture is much more common than previously reported and can contribute to abnormal head shape in isolation, or in combination with major sutures. Squamosal suture synostosis is underdiagnosed clinically and radiologically, although insufficient evidence exists to determine if its true incidence is increasing.

Diagnostic Yield of Routine Skull Radiographs in Infants With Deformational Plagiocephaly.

OBJECTIVE: Differentiating synostotic and nonsynostotic plagiocephaly can be challenging, and many providers routinely obtain screening skull radiographs when evaluating an infant with plagiocephaly. However, the diagnostic yield of radiographs has not been studied in this clinical setting. DESIGN: This study was a retrospective chart review. SETTING: The study took place in a tertiary care center. PATIENTS: We retrospectively reviewed the records of all patients referred to Children's Medical Center (Dallas, TX) between the years 2010 to 2012 with a diagnosis of plagiocephaly. After an initial evaluation, skull radiographs were obtained to rule out craniosynostosis. We reviewed clinical and demographic data and radiographic findings to determine the diagnostic yield of routine screening radiographs in infants presenting with plagiocephaly. RESULTS: There were 1219 patients in total, and 1213 of these patients received screening four-view conventional skull radiographs. Six had computed tomography without prior radiographs. Of the patients in the skull radiograph group, 24% (289 of 1213) had abnormal radiographic findings, and 7.6% of this group (22 of 289) had findings that were indicative of craniosynostosis. Of these 22 patients, 12 obtained follow-up studies, and only three patients (0.2% of skull group) had true craniosynostosis. In comparison, 50% (three of six) in the group of patients who underwent computed tomography without prior conventional screening radiographs had true craniosynostosis. CONCLUSIONS: Routine screening skull radiographs have a low diagnostic yield in differentiating between synostotic and nonsynostotic plagiocephaly in patients presenting to a tertiary care deformational plagiocephaly clinic. Considering the costs and radiation exposure, the benefit of the routine use of skull radiographs in patients with deformational plagiocephaly is questionable.

Nasal soft-tissue and vault deviation in unicoronal synostosis.

BACKGROUND: Unicoronal synostosis (UCS) results in nasal root deviation toward the fused side of the face, resulting in an apparent nasal dorsal deviation to the non-fused side. The impact of the altered radix position on the osteocartilaginous vault and nasal soft tissue has not been analyzed. The purpose of this study is to morphometrically assess the nasal structure and deviation in UCS. We hypothesize the proximal etiology exerts an impact on the distal nasal form, compared to controls. METHODS: Demographic data were tabulated and computed tomographic information recorded. Three-dimensional reconstruction was created and analyzed digitally (using Surgi Case). Morphometric landmarks were determined and used to perform measurements on the nasal soft tissue and osseous skull surface to evaluate nasal deviation within a midsaggital plane (MSP). RESULTS: Forty three-dimensional CT scans of 20 UCS patients and 20 control subjects were analyzed. The deviation angle of the nose to the non-fused side was 6.6 ± 2.9° in the bony layer. In the soft-tissue layer, the deviation angle of the nasal dorsum line to the non-fused side was 5.4 ± 3.4°. The tip of the nose showed a significant deviation to the non-fused side (2.2 ± 1.2 mm). Paired landmarks (alares, inferior lateral nostril bases) related to the MSP showed a greater distance on the non-fused side. Paired landmarks related to an intrinsic nasal midline (Nsup-ANS; tip-columella line (TCL)) did not show any significant differences. CONCLUSION: UCS confers osteocartilaginous and soft-tissue nasal deviation, with the distal nose toward the non-fused side. The nasal root inclination underpins this asymmetry across the midsaggital reference plane. However, the nose in isolation exhibits balanced side-side proportions.

The assessment of relationship between the skull base development and the severity of frontal plagiocephaly after bilateral fronto-orbital advancement in the early life.

PURPOSE: The deformation of the skull base in patients with unilateral frontal plagiocephaly (UFP) is well known, but the mechanism is not still clear. We analyzed the skull base in the patients with UFP who underwent fronto-orbital advancement (FOA) in the early life during the last decade. METHODS: We assessed the treatment results and outcome of FOA performed in six patients, four girls and two boys younger than 2 years, in the last decade. Also, the basal cranium's angles were measured by 3D reconstruction images on computed tomography (CT) scan. RESULTS: The mean patients' age at FOAs was 11 months. Two cases were classified as grade 2A, two cases as grade 2B, and two cases as grade 3 (the classification of Di Rocco and Velardi). The ethmoidal axis was deviated a mean of 8.2° to the affected side. The mean angle between the petrosal pyramids and the midline (anterior-petrosal-sagittal angle, APSA) was 75.3° on the affected side and 66.2° on the normal side. The mean difference of APSA was 9.2°. On the follow-up CT images 5 years after surgery, the deviations of the ethmoidal axis clearly decreased, 5.7°, but the differences of APSA did not change, 8.8°. CONCLUSIONS: The midline distortion of anterior skull base should be considered to be spontaneously corrected during the follow-up periods in patients with all types of UFP who underwent FOA, unlike posterior skull base in the patients with grades 2B and 3 classification.

Wormian bones in a general paediatric population.

UNLABELLED: Wormian bones are small bones that are often found within the sutures and fontanelles of the skull. When a child presents an unexplained fracture or fracture(s), osteogenesis imperfecta is usually suggested when an "abnormally high number" of fractures are seen. PURPOSE: To assess the frequency, number, and topography of wormian bones in a "normal" paediatric population. MATERIALS AND METHODS: In a population aged from 0 to 3 years, we retrospectively analysed 605 CT brain scans carried out for a range of indications, excluding cases in which there was a suspicion of constitutional bone disease. RESULTS: In our population, wormian bones were found in 53% of children (n=320): 43% of the children had between one and three (n=260), 10% had four or more (n=60), and 6% had five or more (n=40). There was no significant relationship between the number of wormian bones and the various indications that had led to the CT scan being carried out. Wormian bones in the lambdoid suture were found in by far the greatest numbers. CONCLUSION: Wormian bones are common and can sometimes be numerous without necessarily pointing to osteogenesis imperfecta, since 10% of the children in our study had at least four.

Value of high-resolution ultrasound in the differential diagnosis of scaphocephaly and occipital plagiocephaly.

Secondary to the increase in deformational plagiocephaly a growing number of infants with cranial deformity present to craniofacial teams. Computed tomography (CT) is diagnostic, but uses ionizing radiation. The purpose of this study was to evaluate ultrasound as a screening test for the patency of cranial sutures in scaphocephaly and occipital plagiocephaly. The cranial sutures of 54 infants with this cranial deformity were assessed by ultrasound. Sutures were read as patent or fused if a hypoechoic gap could or could not be demonstrated between the hyperechoic clavarial bones, respectively. Seven children suffered from true craniosynostosis of either the sagittal or the lambdoid suture. In five cases the ultrasound findings were diagnostic for a fused suture, in two cases the results were inconclusive. Forty-seven infants presented with deformational plagiocephaly. Ultrasound examination demonstrated patent sutures in 45 cases and was inconclusive in two cases. Sonography of the cranial sutures is a good screening tool to distinguish fused from patent cranial sutures in scaphocephaly and occipital plagiocephaly and avoids the radiation exposure associated with CT examinations.

Validation of optical three-dimensional plagiocephalometry by computed tomography, direct measurement, and indirect measurements using thermoplastic bands.

The aim of the study was to validate optical three-dimensional surface imaging for plagiocephalometry by comparing it with established direct and indirect plagiocephalometric measurements. From head models with symmetric and asymmetric shapes, a number of distances were determined for plagiocephalometry. Data were acquired by optical three-dimensional surface imaging, direct and indirect measurements, and computed tomography. Data acquisition was repeated 10 times with each method. In 10 patients with positional plagiocephaly, optical three-dimensional surface data were acquired, and thermoplastic strips were used to reproduce the largest circumferences of the patients' heads. All measurements were carried out by the same observer. The measurements for the head models were used to assess the reproducibility of the different measurement techniques and to check if there were statistically significant differences between them. The patient measurements were also used to determine if there were statistically significant differences between the different techniques in clinical use. None of the plagiocephalometric distances showed statistically significant differences when the 4 different methods were compared on the head models (P > 0.05). Comparison of plagiocephalometric distances obtained from optical three-dimensional imaging and from thermoplastic strips in clinical use showed also no significant difference (P > 0.05). The results of the current study reveal that optical three-dimensional imaging is a relevant alternative to other established techniques in plagiocephalometry that should be tested in larger patient series.

Vertical position of the orbits in nonsyndromic plagiocephaly in childhood and its relation to vertical strabismus.

PURPOSE: To determine the existence of a correlation between the vertical angle of strabismus and the vertical angle between the orbital axes in nonsyndromic plagiocephaly in childhood. METHODS: Patients were included when diagnosed with plagiocephaly. Orthoptic measurements showed a vertical strabismus and three-dimensional computed tomographic (CT) imaging of the skull was available. Patients were excluded if plagiocephaly was part of a syndrome or if any surgical intervention had taken place before our measurements. Three-dimensional CT imaging was used to calculate the vertical angle between the orbital axes in 3 reference planes (VAO) perpendicular to a line of reference through the lower borders of the maxilla (VAOmax), both auditory canals (VAOaud), and the lower points of the external occipital protuberances (VAOocc). RESULTS: Fourteen patients were included (mean age, 14 mo). Three-dimensional CT measurements showed a mean (SD) VAOmax of 1.70 (2.31) degrees, VAOaud of -1.54 (1.46) degrees, and VAOocc of -2.06 (4.29) degrees (a negative value indicates that the eye on the affected side was situated lower in the head). The mean vertical angle of strabismus was -2.39 (4.69) degrees in gaze toward the affected side, 3.66 (3.77) degrees in gaze ahead, and 8.14 (5.63) degrees in gaze toward the nonaffected side. The Pearson test showed no significant correlations. CONCLUSIONS: The clinical observation that vertical strabismus in adult plagiocephaly is correlated with the vertical angle of the orbital axes could not be confirmed in young children.