Assessing calvarial vault constriction associated with helmet therapy in deformational plagiocephaly.

OBJECTIVE Deformational plagiocephaly and/or brachycephaly (DPB) is a cranial flattening frequently treated in pediatric craniofacial centers. The standard of care for DPB involves patient positioning or helmet therapy. Orthotic therapy successfully reduces cranial asymmetry, but there is concern over whether the orthotics have the potential to restrict cranial growth. Previous research addressing helmet safety was limited by lack of volume measurements and serial data. The purpose of this study was to directly compare head growth data in patients with DPB between those who underwent helmet therapy and those who received repositioning therapy. METHODS This retrospective cohort study analyzed pre- and posttherapy 3D photographs of 57 patients with DPB who had helmet therapy and a control group of 57 patients with DPB who underwent repositioning therapy. The authors determined the change in cranial vault volume and cranial circumference between each patient's photographs using 3D photogrammetry. They also computed a cubic volume calculated by multiplying anterior-posterior diameter, biparietal diameter, and height. Linear regressions were used to quantify effects of age and therapy type on these quantities. RESULTS A comparison of the following variables between the two groups yielded nonsignificant results: age at the beginning (p = 0.861) and end (p = 0.539) of therapy, therapy duration (p = 0.161), and the ratio of males to females (p = 0.689). There was no significant difference between patients who underwent helmeting versus positioning therapy with respect to change in either volume calculation or head circumference z-score (p ≥ 0.545). Pretherapy photograph age was a significant predictor of cranial growth (p ≤ 0.001), but therapy type was not predictive of the change in the study measurements (p ≤ 0.210). CONCLUSIONS The authors found no evidence that helmet therapy was associated with cranial constriction in the study population of patients with DPB. These results strengthen previous research supporting helmet safety and should allow health care providers and families to choose the appropriate therapy without concern for potential negative effects on cranial growth.

Use of a Midliner Positioning System for Prevention of Dolichocephaly in Preterm Infants.

PURPOSE: The purpose of this study was to determine effectiveness of a midliner positioning system (MPS, Tortle Midliner) for preventing dolichocephaly. METHODS: This was a nonrandomized, prospective study of 30 premature infants (study cohort, SC) using an MPS compared with a retrospective study cohort (RSC) of 65 infants who received standard of care intervention. RESULTS: RSC baseline cranial index (CI) of 80% and final CI of 77% significantly decreased over an average 5.5 weeks (P < .0001). The SC baseline CI and the final CI were both 79% over an average 5.7 weeks, indicating no significant difference between CI measures (P = .6). Gestational age, birth weight, reflux, time on continuous positive airway pressure, and time in a supine position were not associated with dolichocephaly. CONCLUSIONS: The SC developed less cranial molding (ie, had greater CI), compared with the RSC. A larger randomized study is needed to recommend routine use of MPS for prevention and/or treatment of cranial molding in premature infants.

One hundred consecutive endoscopic repairs of sagittal craniosynostosis: an evolution in care.

OBJECTIVE Endoscope-assisted repair of sagittal craniosynostosis was adopted at St. Louis Children's Hospital in 2006. This study examines the first 100 cases and reviews the outcomes and evolution of patient care protocols at our institution. METHODS The authors performed a retrospective chart review of the first 100 consecutive endoscopic repairs of sagittal craniosynostosis between 2006 and 2014. The data associated with length of hospital stay, blood loss, transfusion rates, operative times, cephalic indices (CIs), complications, and cranial remolding orthosis were reviewed. Measurements were taken from available preoperative and 1-year postoperative 3D reconstructed CT scans. RESULTS The patients' mean age at surgery was 3.3 ± 1.1 months. Of the 100 patients, 30 were female and 70 were male. The following perioperative data were noted. The mean operative time (± SD) was 77.1 ± 22.2 minutes, the mean estimated blood loss was 34.0 ± 34.8 ml, and the mean length of stay was 1.1 ± 0.4 days; 9% of patients required transfusions; and the mean pre- and postoperative CI values were 69.1 ± 3.8 and 77.7 ± 4.2, respectively. Conversion to open technique was required in 1 case due to presence of a large emissary vein that was difficult to control endoscopically. The mean duration of helmet therapy was 8.0 ± 2.9 months. Parietal osteotomies were eventually excluded from the procedure. CONCLUSIONS The clinical outcomes and improvements in CI seen in our population are similar to those seen at other high-volume centers. Since the inception of endoscope-assisted repair at our institution, the patient care protocol has undergone several significant changes. We have been able to remove less cranium using our "narrow-vertex" suturectomy technique without affecting patient safety or outcome. Patient compliance with helmet therapy and collaborative care with the orthotists remain the most essential aspects of a successful outcome.

[Guide to clinical practice for the diagnosis, treatment and rehabilitation of non-syndromic craniosynostosis on 3 levels of care]. / Guía de práctica clínica para el diagnóstico, tratamiento y rehabilitación de craneosinostosis no sindrómica en los 3 niveles de atención.

INTRODUCTION: Craniosynostosis is a congenital anomaly resulting from the premature fusion of the cranial sutures changing growth patterns of the skull. METHODOLOGY: Focus, scope, target population and clinical questions to be solved were defined. A systematic search for evidence in different databases (Medline, Embase, KoreaMed, Cochrane Library and the website of the World Health Organization) in stages was performed: clinical practice guidelines; systematic reviews, and clinical trials and observational studies; using MeSH, Decs and corresponding free terms, unrestricted language or temporality. Risk of bias was evaluated using appropriate tools (AMSTAR, Risk of Bias or STROBE). The quality of evidence was graduated using the GRADE system. Modified Delphi Panel technique was used to assign the recommendation's strength and direction, as well as the degree of agreement with it, taking into account the quality of evidence, balance between risks and benefits of interventions, values and preferences of patients and availability of resources. RESULTS: There were 3,712 documents obtained by search algorithms; selecting 29 documents for inclusion in the qualitative synthesis. Due to heterogeneity between studies, it was not possible to perform meta-analysis. CONCLUSIONS: We issued 7 recommendations and 8 good practice points, which will serve as support for decision-making in the comprehensive care of patients with craniosynostosis.

Early diagnosis and treatment of children with skull deformations. The challenge of modern medicine.

Skull deformations affect approximately 45% of newborn babies, the most common ones being: plagiocephaly, brachycephaly and scaphocephaly. The first symptoms can be observed 4 to 8 weeks after birth. The causes of skull deformation in newborns can be divided into congenital ones and those acquired after birth. An increase in the incidence of acquired head deformations can be attributed to the "BACK TO SLEEP" campaign, carried out in 1992 by the American Academy of Pediatrics (AAP), which was aimed to reduce the frequency of sudden infant death syndrome (SIDS) by placing babies to sleep in the supine position. By the year 2000, the number of SIDS incidents had been significantly lowered, however, it seems that this improvement was achieved at the cost of an increased number of head deformations [3, 4, 5, 34]. Skull deformations, if left untreated, may have consequences for the future. Plagiocephalic deformations may be associated with delayed intellectual and motor development [2]. Early recognition of the condition and the appropriate classification of each skull deformation are crucial for the success of the treatment [8]. Treatment choice depends on the etiology of the problem and its severity, as well as on the age of the infant. Available options include training for the parents/caregivers, physical therapy, custom head orthosis and surgical intervention.

Long-term developmental follow-up in children with nonsyndromic craniosynostosis.

OBJECT: The aim of this study was to determine the distribution of Full Scale IQ (FSIQ) by type of craniosynostosis and to verify the finding that at long-term follow-up, verbal IQ (VIQ) is significantly higher than performance IQ (PIQ) in patients with single-suture sagittal synostosis (SS) despite falling within the "average" range for intelligence. Whether this also occurs in other types of craniosynostosis and whether surgery and sex are relevant were also determined. The relationship between age at time of surgery and later IQ was ascertained. METHODS: The data for 91 children with craniosynostosis (47 sagittal, 15 unicoronal, 13 metopic, 9 multisuture, and 7 bicoronal) were collected at their routine, 10 years of age IQ assessment (mean age 123.8 months). The patients included 61 males and 30 females; 62 patients had undergone surgery and 29 had not. RESULTS: The mean FSIQ for all types of craniosynostosis combined (96.2) fell within the average range for the general population. Some variation was evident across the different types of craniosynostosis: the SS group showed the highest FSIQs and a "normal" distribution of bandings; the other types had a higher proportion of FSIQs in the lower bandings. The data confirmed the finding that VIQ is greater than PIQ despite falling within the average range for intelligence, with a difference of 5.0 for all types of craniosynostosis combined (p = 0.001), 7.6 for the SS group (p = 0.001), and 6.9 for the unicoronal group (p = 0.029). This VIQ > PIQ effect was not found with multisuture craniosynostosis. The VIQ > PIQ discrepancy occurred regardless of whether the patient had undergone surgery and occurred more often in males than females. In the SS group and the bicoronal group, FSIQ (p = 0.036 and p = 0.046, respectively) and PIQ (p = 0.012 and p = 0.017, respectively), though not VIQ, were higher when surgery had been performed early. CONCLUSIONS: The study confirms that at long-term follow-up, although children with nonsyndromic craniosynostosis fall within the normal range for intelligence, there is a VIQ > PIQ discrepancy above what would be expected in the normal population, which may be indicative of more subtle difficulties in achievement. This discrepancy is affected by type of craniosynostosis, sex, and age at time of surgery.

Long-term outcomes in treatment of deformational plagiocephaly and brachycephaly using helmet therapy and repositioning: a longitudinal cohort study.

OBJECTIVES: Deformational plagiocephaly and/or brachycephaly (DPB) is a misshapen head presenting at birth or shortly thereafter, caused by extrinsic forces on an infant's malleable cranium. There are two treatment methods available for DPB: helmeting and repositioning. Little is known about the long-term outcomes of these two treatment options. The purpose of this study was to examine children who received helmeting or repositioning therapy for DPB as infants and compare the long-term head shape outcomes of the two groups. METHODS: A longitudinal cohort study design was used to evaluate change in head shape of the two groups. One hundred children (50 helmeted, 50 repositioned) were initially evaluated at 6 months or younger for DPB. Anthropometric skull measurements taken as infants before treatment were compared with measurements taken for this study. Inclusion criteria included initial clinic visit at age 6 months or younger, evaluation by the same practitioner, and current age 2-10 years. Cephalic index and cranial vault asymmetry were calculated based on caliper measurements. RESULTS: Data from 100 children were evaluated for this study. Significant differences between the treatment groups in the mean change in cephalic index (p = 0.003) and cranial vault asymmetry (p < 0.001) were found; the children that used helmet therapy demonstrated greater improvement. CONCLUSIONS: This is one of the larger published long-term outcome studies comparing children that used helmets and repositioning to treat their DPB as infants. The data suggest that infants will have more improvement in head shape with a helmet than with repositioning.

Diagnóstico, tratamiento y rehabilitación de craneosinostosis no sindrómica en los tres niveles de atención / Diagnosis, treatment and rehabilitation of nonsyndromic craniosynostosis in the three levels of care

Esta guía aborda aspectos relacionados con el diagnóstico, el tratamiento y la rehabilitación de la craneosinostosis no sindrómica en los tres niveles de atención

Interdisciplinary rehabilitation of a patient with Opitz C-syndrome and extensive craniofacial malformation.

A 24-year-old patient with C-syndrome and multiple congenital deformities was followed-up for his complete lifetime. The patient received extensive interdisciplinary rehabilitation of his complex somatic and craniofacial malformation. This article intends to show from the clinical point of view, how such patients can have profit from broad arrangements in specific periods of life, how far the rehabilitation can go in cases of good patients and parental compliance and how important an interdisciplinary cooperation is.

Torticollis and plagiocephaly in infancy: therapeutic strategies.

BACKGROUND: Asymmetry in infancy is a diagnosis with a large spectrum of features, expressing an abnormal shape of parts of the body or unequal postures and movements, which might be structural and/or functional, with localized or generalized expression. PURPOSE: The purpose of the present study is to highlight different therapeutic aspects of the most occurring asymmetries in infancy: congenital muscular torticollis, positional torticollis and plagiocephaly, based on best evidence in current literature. RESULTS: A flow chart is presented showing different pathways in therapeutic strategies, such as physical therapy, orthotic devices (helmet treatment and Dynamic Orthotic Cranioplasty) and surgery. CONCLUSION: It is concluded that there are different views towards management on torticollis and plagiocephaly. A systematic therapeutic management to evaluate these asymmetries is indicated. The presented therapeutic flow chart might serve as a basis in order to achieve uniformity in therapeutic thinking and performance.

The circle of sagittal synostosis surgery.

Sagittal synostosis, the premature closure of the sagittal suture, accounts for more than 50% of all nonsyndromic single-suture synostoses. Although no detrimental neurologic effects can be directly attributed to the synostosis, a number of patients will have relatively increased intracranial pressure. Surgical correction for sagittal synostosis has evolved from simple removal of bone strips to extensive cranial remodeling, all in a attempt to achieve a normal head shape. The lack of outcome measures has limited the surgeon's ability to choose one surgical procedure over another. The use of a cranial helmet for passive molding of the head after surgery is effective when used with limited endoscopic surgery. We present the results of using an extended strip craniectomy combined with long-duration molding helmet therapy and compare it with other reported methods. The results suggest that passive molding with the helmet may have a greater effect on cranial shape than surgery.

Molding helmet therapy in the management of sagittal synostosis.

The current surgical treatment of sagittal craniosynostosis often fails to produce a normal cephalic index; however, recent reports of early endoscopic suture release and postoperative helmet therapy promise improved cranial symmetry. The role of helmet therapy is critical in this approach, but there are few reports on the use of helmet therapy after craniosynostosis surgery. The authors present a retrospective review comparing the results of surgery alone versus surgery and postoperative banding in treating children diagnosed with sagittal synostosis. Cephalic index (CI) measurements and the divergence of the CI from the norm (DFN) were used to compare the banded and the non-banded groups. Included in the study were 21 children with sagittal craniosynostosis of whom 6 were treated with surgery alone and 15 were treated with surgery and postoperative banding. Preoperatively, both groups were significantly different from the norm (non-banded, P < 0.05; banded, P < 0.01), while postoperatively they were not significantly different from the norm. Statistically significant changes were present in the CIs of the banded group in the preoperative-follow-up interval (P < 0.01), while no significant changes occurred in the non-banded group during this same interval. Statistically significant changes in the DFN occurred in the preoperative-postoperative and preoperative-follow-up intervals of the banded group; however, these changes were not significant in the non-banded group during the same intervals. Correction toward a normal CI was seen in the banded group throughout the course of treatment, while this trend was not present in the non-banded group. Therefore, molding helmet therapy maintains the operative correction obtained and promotes more normal cranial growth patterns.

Craniosynostosis and skull molding.

On February 20-23, 1997 in Scottsdale, Arizona, a symposium was held that was sponsored by the Plastic Surgery Educational Foundation, the American Society of Maxillofacial Surgeons, and the Joint Section on Pediatric Neurological Surgery of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons. The chairs of the meeting were Jeffrey C. Posnick and Harold L. Rekate. The symposium examined issues relating to craniosynostosis and skull molding. The program consisted of three parts. Day 1 focused on the basic concepts of craniosynostosis and skull molding. Day 2 focused on evaluation and treatment of craniosynostosis. Day 3 focused on the diagnosis and treatment of craniofacial syndromes. The symposium was significant because it brought craniofacial and pediatric neurosurgeons together for the first time at a combined meeting to discuss important aspects of craniosynostosis and skull molding. This article summarizes the presentations made at the meeting.

Rehabilitation aspects of Apert's syndrome.

Two children with the unusual condition known as Apert's syndrome are presented. Both have the typical manifestations of the syndrome: craniosynostosis, exorbitism, hypertelorism, maxillary hypoplasia, dental malocclusion, cleft palate, compound syndactyly of the hands and simple syndactyly of the feet. A review of the latest surgical procedures for correction of these defects is presented and the importance of early physical and social rehabilitation is stressed.