Long-term ophthalmological outcomes in patients with anterior plagiocephaly managed with a fronto-orbital advancement procedure.

BACKGROUND: Anterior plagiocephaly results from early fusion of a unilateral coronal cranial suture, which affects 1/10,000 infants at birth and can be associated with ophthalmological complications. The study aimed to evaluate the frequency of oculomotor disorders and refractive errors in children with non-syndromic anterior plagiocephaly. METHODS: Patients with anterior plagiocephaly undergoing surgery using a fronto-orbital advancement technique were included in this retrospective study between 2011 and 2017. The following data were collected: cycloplegic refraction in diopters (D), best-corrected visual acuity, manifest strabismus in primary position, ocular motility, head tilt in primary position, slit-lamp and fundus examination. The refractive errors were determined by autorefraction after cycloplegia with cyclopentolate. RESULTS: Among the sixteen patients included, hyperopia >2.5D was found in 10 patients, astigmatism >1D in 10 patients and anisometropia >1D in 7 patients. Astigmatism was contralateral to the synostosis in 7 cases. In total, significant refractive errors were found in 92.9%. Amblyopia was found in 33.3% patients. Strabismus was found in 11 patients, most frequently combined horizontal and vertical, of which 4 required strabismus surgery. The most frequent vertical deviation was ipsilateral hypertropia in 54.5%. We found superior oblique muscle limitation in 3 patients. Optic disc pallor was found in 2 patients. CONCLUSION: In the years following fronto-orbital advancement for non-syndromic anterior plagiocephaly, refractive errors and oculomotor disorders are frequently encountered, mainly contralateral astigmatism and strabismus with both horizontal deviation and ispilateral hypertropia.

Extraocular muscle positions in anterior plagiocephaly: V-pattern strabismus explained using geometric mophometrics.

INTRODUCTION: Ophthalmological involvement in anterior plagiocephaly (AP) due to unicoronal synostosis (UCS) raises management challenges. Two abnormalities of the extraocular muscles (EOM) are commonly reported in UCS without objective quantification: (1) excyclorotation of the eye and (2) malposition of the trochlea of the superior oblique muscle. Here we aimed to assess the positions of the EOM in AP, using geometric morphometrics based on MRI data. MATERIALS AND METHODS: Patient files were listed using Dr WareHouse, a dedicated big data search engine. We included all patients with AP managed between 2013 and 2018, with an available digital preoperative MRI. MRIs from age-matched controls without craniofacial conditions were also included. We defined 13 orbital and skull base landmarks in order to model the 3D position of the EOM. Cephalometric analyses and geometric morphometrics with Procrustes superimposition and principal component analysis were used with the aim of defining specific EOM anomalies in UCS. RESULTS: We included 15 preoperative and 7 postoperative MRIs from patients with UCS and 24 MRIs from age-matched controls. Cephalometric analyses, Procrustes superimposition and distance computations showed a significant shape difference for the position of the trochlea of the superior oblique muscle and an excyclorotation of the EOM. CONCLUSIONS: Our results confirm that UCS-associated anomalies of the superior oblique muscle function are associated with malposition of its trochlea in the roof of the orbit. This clinical anomaly supports the importance of MRI imaging in the surgical management of strabismus in patients with UCS.

A New Operative One-Wing Technique to Correct Fronto-Orbital Region in Unilateral Nonsyndromic Plagiocephaly.

Anterior plagiocephaly is a premature closing of unilateral coronal suture. This premature suture fusion causes a series of cranial asymmetry and alteration of the fronto-orbital region.The authors described a new surgical technique to correct the facial asymmetry that reduces the operative time and the possible complications.In a period between 2013and 2016, 12 children affected by nonsyndromic anterior plagiocephaly were treated with this new procedure.Clinical, cranial development, and absence of postoperative complication demonstrated that one-wing surgical bone correction is a useful and innovative technique.

Defining and Correcting Asymmetry in Isolated Unilateral Frontosphenoidal Synostosis: Differences in Orbital Shape, Facial Scoliosis, and Skullbase Twist Compared to Unilateral Coronal Synostosis.

INTRODUCTION: Isolated frontosphenoidal synostosis (FS) is a rare cause of fronto-orbital plagiocephaly that can be challenging to distinguish from isolated unicoronal synostosis (UC). The purpose of this paper is to analyze differences in fronto-orbital dysmorphology between the 2 conditions, to describe approaches for surgical correction, and to report surgical outcomes between FS and UC patients in a casecontrol fashion. METHODS: Patients treated for craniosynostosis over a 12-year period at our institution were retrospectively evaluated under institutional review board approval. Frontosphenoidal synostosis patients who underwent bilateral fronto-orbital correction of anterior plagiocephaly with minimum 2-year follow-up, adequate pre-, and minimum 2-year postoperative computed tomography scans were included in the case-control portion of the study. These patients were randomly age-matched to UC patients meeting the same inclusion criteria. Preoperative and postoperative orbital shape and volumetric analysis was performed using Mimics software. RESULTS: Twelve FS patients were treated during the study period. Seven of these patients met casecontrol inclusion criteria with average follow-up of 47.5 months. The characteristic FS orbit was a relatively wide, short, and shallow trapezoid, while the characteristic UC orbit was a relatively narrow, tall, and deep parallelogram. Frontosphenoidal synostosis orbits were significantly wider, shorter, shallower, and smaller than UC orbits. Surgical correction tailored to the differential dysmorphologies resulted in statistical equalization of these differences between affected and contralateral control orbits at follow-up, with the exception of UC orbital width, which remained significantly narrower than unaffected contralateral control. One patient in each group required cranioplasty for skull defects at follow-up, while no patient underwent surgical readvancement. CONCLUSIONS: Frontosphenoidal synostosis and UC orbital shape differ significantly, and can be normalized using fronto-orbital advancement tailored to the distinct orbital dysmorphologies of these 2 groups.

[A clinical analysis of 101 infants with plagiocephaly].

OBJECTIVE: To investigate the possible causes of plagiocephaly in infants and the therapeutic effect of postural correction training on plagiocephaly. METHODS: A total of 101 infants who were diagnosed with plagiocephaly were enrolled. According to the age at diagnosis, these infants were divided into 1-4 month group (31 infants), 5-8 month group (40 infants), and 9-12 month group (30 infants). The possible causes of plagiocephaly were analyzed in three groups. The cranial vault asymmetry index (CVAI) before and after postural correction training was compared in three groups. RESULTS: Of the 101 infants, 89 (88.1%) had a sleeping posture in the supine position, and there was no significant difference in the percentage of infants with such posture between the three groups. Compared with the 5-8 month group and the 9-12 month group, the 1-4 month group had significantly higher rate of preterm birth, incidence rate of adverse perinatal factors, and incidence rate of congenital muscular torticollis. The three groups showed a significant decrease in CVAI 3 months after postural correction training (P<0.001). Compared with the 5-8 month group and the 9-12 month group, the 1-4 month group had a significantly greater change in CVAI after postural correction training (P<0.001). CONCLUSIONS: The sleeping posture in the supine position may be associated with the development of plagiocephaly. Adverse perinatal factors, preterm birth, and congenital muscular torticollis as possible causes of plagiocephaly are commonly seen in early infancy. Postural correction training has a significant effect in improving plagiocephaly, especially in early infancy.

Plagiocephaly due to Frontosphenoidal Suture Synostosis: Report of 2 Cases and Literature Review.

BACKGROUND/AIMS: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. METHODS: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. RESULTS: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. CONCLUSION: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.

Impact of severity and therapy onset on helmet therapy in positional plagiocephaly.

Although helmet therapy is a widely established method in the treatment of positional plagiocephaly, therapeutic regimens remain contentious, especially regarding starting age. Hence, this study investigated the impact of starting age and severity on the effectiveness of helmet therapy. A total of 213 pediatric patients treated for positional plagiocephaly with an orthotic device were enrolled in this study. Pre- and post-treatment calvarial asymmetry was measured according to the Cranial Vault Asymmetry Index (CVAI) using 3D-Photogrammetry. Patients were classified by age at which treatment was started: Group 1 was comprised of patients younger than 24 weeks (n = 82); Group 2, those aged 24-32 weeks (n = 75); Group 3, those aged >32 weeks (n = 56). Additionally, groups were categorized by severity (mild: CVAI 3-7%; moderate: CVAI 7-12%; severe: CVAI > 12%). Mean initial CVAI was 9.8%, which reduced to 5.4% after helmet treatment. Group 1 (<24 weeks) showed the highest absolute and relative rate of correction. Within the groups, severity correlated positively with relative and absolute reduction of the asymmetry. A significant difference in the reduction of the CVAI depending on age was only seen in moderate and severe cases of plagiocephaly- but not in mild plagiocephaly. The present study confirms the effectiveness of helmet therapy for positional plagiocephaly. The use of an orthotic device is an appropriate treatment option particularly in infants with severe plagiocephaly and a start of helmet therapy before the age of 6 month is advisable.

The Incidence of Complications Associated With Molding Helmet Therapy: An Avoidable Risk in the Treatment of Positional Head Deformities?

Molding helmet therapy using an individual head orthosis presents a widely accepted treatment option for children with positional head deformities; however, studies addressing the incidence of complications during helmet therapy are rare. The current study evaluates the incidence of complications in 205 children with positional head deformity undergoing molding helmet therapy. Children were classified according to the severity of their deformity as presented by the Cranial Vault Asymmetry Index (CVAI) and the Cephalic Index (CI). Fifty-nine (28.8%) of our patients presented a moderate and 146 (71.2%) a severe form of a positional head deformity. Of these children, 166 (81.0%) were diagnosed for plagiocephaly, 19 (9.3%) were brachycephalic, and 20 (9.7%) showed a combination of plagiocephaly and brachycephaly. Overall, 54 children (26.3%) showed minor complications during their helmet molding including pressure sores (13.7%), ethanol erythema (2.9%), skin erosions/skin infections (4.3%), or deficient fitting (5.4%). Children with a combination of plagiocephaly and brachycephaly (n = 20) showed the highest risk for complications, which was significantly higher compared with children with plagiocephaly (50% vs 22.3%; P = 0.012). Irrespective of the type of positional head deformity, no statistical difference was revealed between the moderate and the severe form. Minor complications are a relatively frequent event during helmet molding therapy. Especially children with a combination of plagiocephaly-brachycephaly are at high risk for complications. A reduction of this rate might be reached by a close follow-up for a short period between helmet manufacturing adjustments.

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.

Frontosphenoid synostosis: an unusual cause of anterior plagiocephaly.

Nonpositional anterior plagiocephaly results commonly from unilateral coronal craniosynostosis. We present 2 patients of a rare cause of anterior plagiocephaly known as frontosphenoid synostosis. This condition is characterized by the absence of a harlequin eye (or the harlequin sign on computed tomography), which is usually present in unilateral coronal synostosis. We also observed no reduction in the ear-eye distance, which can distinguish it from coronal craniosynostosis.

The inter-rater and intra-rater reliability of a modified "severity scale for assessment of plagiocephaly" among physical therapists.

Infants with congenital muscular torticollis (CMT) are at risk of developing skull asymmetry. The aim of this study was to investigate the inter-rater and intra-rater reliability of a modified "severity scale for assessment of plagiocephaly" among physical therapists (PT). Thirty-nine members of a network of PTs working with infants with CMT participated in the study. Photos of infants were used in this study. They were taken from above (superior view) to estimate posterior flattening and forehead asymmetry, and from the front (anterior view) to estimate neck involvement and facial asymmetry. The photos were coded and sent to the PTs together with instructions and a protocol for estimation. A second estimate was carried out with the same photos in a different order. The PTs also answered questions concerning their experience of CMT and the scale used. The participants had worked as PTs for an average of 20 years, and with CMT for an average of 7 years. The inter-rater reliability kappa was 0.71, and the intra-rater reliability was mean 0.68. Intra-rater reliability was significantly higher for years of experience and for years working with CMT. There was no significant impact on reliability in relation to how many infants the PTs usually examine yearly, whether they found the scale easy to use, or how much experience they had using it. In conclusion, the modified "severity scale for assessment of plagiocephaly" has satisfying statistical agreement. Reliability is affected by the number of years of experience of the PTs.

[Plagiocephaly resulted from frontosphenoidal synostosis and squamosal suture synostosis: a report of five cases].

OBJECTIVE: To investigate the diagnosis and mapagement of plagiocephaly resulted from For frontosphenoidal synostosis, frontosphenoidal synostosis and squamosal suture synostosis. METHODS: orthotic nolding therapy should be selected for patients under 3-month-old. If it can' t make effect after treatment for 3 months, transcranial surgery should be adopted with the horizontal advancement of orbitofrontal bar at the affected side. Patients with squamosal suture synostosis should receive prohotic molding From 2005 to 2012, five patients were therapy for six month, remolding the cap every month. RESULTS: treated with satisfactory result, including 3 patients with frontosphenoidal synostosis who received surgery and 2 patiens with squamosal suture synostosis who receive orthotic molding therapy only. CONCLUSIONS: Few patients with plagiocephaly are caused by frontosphenoidal synostosis and squamosal suture synostosis. The orthotic molding therapy should be selected at an early time, but mostly, surgery may be needed for

The prevalence of cobedding and SIDS-related child care practices in twins.

UNLABELLED: Sudden unexplained and unexpected death in twins has not been well studied, particularly in relation to the common practice of sleeping twin infants together in the same cot (cobedding). Postal surveys of 109 mothers of twins were undertaken when the infants were 6 weeks, 4 months, and 8 months of age to determine the prevalence of cobedding, infant care practices related to sudden infant death syndrome (SIDS), and the mother's knowledge of SIDS risk factors. SIDS risk factor knowledge was similar to an earlier survey of singletons in 2005. At 6 weeks, 76% of infants usually slept on the back; this changed to 86% at 4 months and 73% at 8 months. At 6 weeks, 52% usually cobedded with their twin all or part of the time. At 4 and 8 months, this had reduced to 31% and 10%, respectively. Side-by-side cobedding configurations were predominant at 6 weeks, with feet-to-feet configurations increasing as the infants grew older. Breastfeeding was reported in 83%, 61% and 31% of infants at 6 weeks, 4 months and 8 months, respectively. Pacifier use was reported in 38%, 50% and 33% at the respective surveys. As the age of the twins increased, mothers were significantly more likely to be concerned about deformational plagiocephaly in Twin 1 than in Twin 2. CONCLUSION: Despite a lack of evidence of the risks or benefits of cobedding, this practice was very popular, especially in the younger infants. More research is needed to establish the safety of cobedding in relation to the risk of SIDS.

Equipo cubano interdisciplinario de cirugía craneofacial en pediatría: Resultados de un quinquenio / Interdisciplinary Cuban staff of craniofacial surgery in Pediatrics: Results from a five-year period

Las deformidades craneofaciales se deben en su mayoría a alteraciones del crecimiento y desarrollo, traumatismos y neoplasias. Con el objetivo de describir el tratamiento quirúrgico integral de estas, se realizó un estudio descriptivo transversal en 46 pacientes tratados por el Equipo Intertidisciplinario de Cirugía Craneofacial del Hospital Pediátrico Universitario Juan M. Márquez en el periodo comprendido entre mayo de 2003 a marzo de 2007. La deformidad más frecuente fue la craneosinostosis (57 por ciento), específicamente la plagiocefalia (17 por ciento). La mayoría de los pacientes fueron de piel blanca (70 por ciento) y se intervinieron entre el 1ro.y 7mo.años de vida (57 por ciento), con una media de 6,3 años. En general no hubo predilección por sexo. Se emplearon 18 diferentes técnicas quirúrgicas. Se presentaron cinco complicaciones durante el posoperatorio (11 por ciento), una defunción (2,2 por ciento), una oftalmoplejía (2,2 por ciento), dos salidas de líquido cefalorraquídeo (4,4 por ciento) y una desaturación por depresión del centro respiratorio (2,2 por ciento). Excepto la oftalmoplejía hubo resolución de las complicaciones en un intervalo de 15 días. Los resultados reportados por este equipo, único de su tipo en el país, son relevantes al compararlos con la literatura internacional

Most of the craniofacial deformities are due to alterations of growth, development, traumata and neoplasms. To describe the integral surgical treatment of above deformities, a cross-sectional and descriptive study was conducted in 46 patients treated by the interdisciplinary staff of craniofacial surgery from the Juan Manuel Márquez University Children Hospital from May, 2003 to March, 2007. The more frequent deformity was the craniosynostosis (57 percent), specifically the plagiocephaly (17 percent). Most of patients were of white race (70 percent) and were operated don between the first and the seventh year of life (57 percent) for a mean of 6,3 years. Generally there wasn't sex predominance. A total of 18 different surgical techniques were used. There were 5 complications during the postoperative period (11 percent), a decease (2.2 percent), a ophthalmoplegia (2.2 percent), two cerebrospinal fluid leakage (4.4 percent) and a desaturation by depression of respiratory center (2.2 percent). With the exception of ophthalmoplegia there was a resolution of complications in a 15 days interval. Results reported by this unique Cuban staff are outstanding compared with international literature