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Horizon BCBSNJ
Uniform Medical Policy ManualSection:D M E
Policy Number:008
Effective Date: 01/21/2020
Original Policy Date:06/22/2001
Last Review Date:08/13/2019
Date Published to Web: 10/18/2019
Subject:
Adjustable Cranial Orthoses for Positional Plagiocephaly and Craniosynostoses

Description:
_______________________________________________________________________________________

IMPORTANT NOTE:

The purpose of this policy is to provide general information applicable to the administration of health benefits that Horizon Blue Cross Blue Shield of New Jersey and Horizon Healthcare of New Jersey, Inc. (collectively “Horizon BCBSNJ”) insures or administers. If the member’s contract benefits differ from the medical policy, the contract prevails. Although a service, supply or procedure may be medically necessary, it may be subject to limitations and/or exclusions under a member’s benefit plan. If a service, supply or procedure is not covered and the member proceeds to obtain the service, supply or procedure, the member may be responsible for the cost. Decisions regarding treatment and treatment plans are the responsibility of the physician. This policy is not intended to direct the course of clinical care a physician provides to a member, and it does not replace a physician’s independent professional clinical judgment or duty to exercise special knowledge and skill in the treatment of Horizon BCBSNJ members. Horizon BCBSNJ is not responsible for, does not provide, and does not hold itself out as a provider of medical care. The physician remains responsible for the quality and type of health care services provided to a Horizon BCBSNJ member.

Horizon BCBSNJ medical policies do not constitute medical advice, authorization, certification, approval, explanation of benefits, offer of coverage, contract or guarantee of payment.

__________________________________________________________________________________________________________________________

Cranial orthoses involve an adjustable helmet or band that progressively molds the shape of the infant cranium by applying corrective forces to prominences while leaving room for growth in the adjacent flattened areas. A cranial orthotic device may be used to treat postsurgical synostosis or positional plagiocephaly in pediatric patients.

PopulationsInterventionsComparatorsOutcomes
Individuals:

•  With open or endoscopic surgery for craniosynostosis

 

Interventions of interest are:

•  Postoperative cranial orthosis

 

 

Comparators of interest are:

•  Cranial vault remodeling without a cranial orthosis

Relevant outcomes include:

•  Change in disease status

•  Morbid events

•  Functional outcomes

•  Quality of life

•  Treatment-related morbidity

Individuals:

•  With positional plagiocephaly

 

Interventions of interest are:

•  Cranial orthosis

 

 

Comparators of interest are:

•  Positioning therapy

 

 

Relevant outcomes include:

•  Change in disease status

•  Morbid events

•  Functional outcomes

•  Quality of life

•  Treatment-related morbidity


BACKGROUND

Craniosynostoses

An asymmetrically shaped head may be synostotic or nonsynostotic. Synostosis, defined as premature closure of the sutures of the cranium, may result in functional deficits secondary to increased intracranial pressure in an abnormally or asymmetrically shaped cranium. The type and degree of craniofacial deformity depend on the type of synostosis. The most common isscaphocephaly, a narrowed and elongated head resulting from synostosis of the sagittal suture. Trigonocephaly, in contrast, is a premature fusion of the metopic suture and results in a triangular shape of the forehead. Unilateral synostosis of the coronal suture results in an asymmetric distortion of the forehead called plagiocephaly and fusion of both coronal sutures results in brachycephaly. Combinations of these deformities may also occur.

Treatment

Synostotic deformities associated with functional deficits are addressed by surgical remodeling of the cranial vault. The remodeling (reshaping) is accomplished by opening and expanding the abnormally fused bone.

In a review of the treatment of craniosynostosis, Persing (2008) indicated that premature fusion of one or more cranial vault sutures occurs in approximately one in 2500 births.1, Of these craniosynostoses, asymmetric deformities involving the cranial vault and base (e.g., unilateral coronal synostosis) will have a higher rate of postoperative deformity, which would require additional surgical treatment. Persing (2008) suggested that use of cranial orthoses postoperatively may serve two functions: (1) they protect the brain in areas of large bony defects, and (2) they may remodel the asymmetries in skull shape, particularly when the bone segments are more mobile.

Plagiocephaly

Plagiocephaly without synostosis, also called positional or deformational plagiocephaly, can be secondary to various environmental factors including, but not limited to, premature birth, restrictive intrauterine environment, birth trauma, torticollis, cervical anomalies, and sleeping position. Positional plagiocephaly typically consists of right or left occipital flattening with the advancement of the ipsilateral ear and ipsilateral frontal bone protrusion, resulting in visible facial asymmetry. Occipital flattening may be self-perpetuating in that once it occurs, it may be increasingly difficult for the infant to turn and sleep on the other side. Bottle feeding, a low proportion of "tummy time" while awake, multiple gestations, and slow achievement of motor milestones may contribute to positional plagiocephaly. The incidence of plagiocephaly has increased rapidly in recent years; this is believed to be a result of the "Back to Sleep" campaign recommended by the American Academy of Pediatrics, in which a supine sleeping position is recommended to reduce the risk of sudden infant death syndrome. It has been suggested that increasing awareness of identified risk factors and early implementation of good practices will reduce the development of deformational plagiocephaly.

Regulatory Status

Multiple cranial orthoses (helmets) have been cleared for marketing by the U.S. Food and Drug Administration through the 510(k) process and are intended to apply passive pressure to prominent regions of an infant's cranium to improve cranial symmetry and/or shape in infants from three to 18 months of age. Multiple marketed devices are labeled for use in children with moderate to severe nonsynostotic positional plagiocephaly, including infants with plagiocephalic- and brachycephatic-shaped heads. Food and Drug Administration product code: MVA.


Related Policies

  • None

Policy:
[INFORMATIONAL NOTE: Pursuant to the New Jersey State Mandate on Orthotic and Prosthetic Appliances (N.J.S.A. 17:48E-35.30, effective April 11, 2008), health benefit plans that provide benefits for orthotic and prosthetic devices are required to provide coverage for expenses incurred in obtaining orthotic and prosthetic appliances from any licensed orthotist, prosthetist, or any certified pedorthist if determined medically necessary by the physician. Please note that with respect to cranial orthosis for plagiocephaly, it is automatically eligible for coverage under the mandate. ASO/ASC/Self-Funded/Self-Insured accounts are exempt from these State mandated requirements but may choose to include these benefits at their discretion. For those self-insured accounts which opted not to adopt the mandate, the medical necessity criteria as specified below must be applied.

For Medicare Advantage, please refer to the Medicare Coverage Section below for coverage guidance.]

1. Use of an adjustable cranial orthosis is considered medically necessary following cranial vault remodeling surgery for synostosis.

2. Use of an adjustable cranial orthosis for synostosis in the absence of cranial vault remodeling surgery is not considered medically necessary.

3. Use of an adjustable cranial orthosis as a treatment of persistent plagiocephaly or brachycephaly without synostosis is considered medically necessary when all of the following conditions have been met:
    • the member is between 3 and 18 months old;
    • documented failure of conservative therapy (repositioning and physical therapy) of at least 2 months duration; AND
    • the member has a cephalic index that is at least two standard deviations above or below the mean for the appropriate gender and age.
4. Use of an adjustable cranial orthosis is not considered medically necessary for all other indications not outlined above.


Medicare Coverage:
HCPCS code S1040 is an invalid code per CMS, and therefore, is noncovered. There is no National Coverage Determination (NCD) for Adjustable Cranial Orthoses for Positional Plagiocephaly and Craniosynostoses. In the absence of an NCD, coverage decisions are left to the discretion of Local Medicare Carriers. Noridian Healthcare Services, LLC, the Local DME Medicare Carrier for jurisdiction JA, has not issued a determination for this service. Therefore, Medicare Advantage Products will follow the Horizon BCBSNJ Medical Policy.


Policy Guidelines: (Information to guide medical necessity determination based on the criteria contained within the policy statements above.)

Procedures are considered medically necessary if there is a significant physical functional impairment, and the procedure can be reasonably expected to improve the physical functional impairment (i.e., improve health outcomes). In this policy, procedures are considered reconstructive when intended to address a significant variation from normal related to accidental injury, disease, trauma, treatment of a disease, or congenital defect. Not all benefit contracts include benefits for reconstructive services as defined herein.

Assessment of plagiocephaly in research studies may be based on anthropomorphic measures of the head, using anatomic and bony landmarks. Although, there is no accepted minimum objective level of asymmetry for a plagiocephaly diagnosis there are definitions that have been adopted by convention.

Brachiocephaly: Shortened front to back dimension of the skull that results from premature fusion of the coronal suture

Cranial base: Asymmetry of the cranial base is measured from the subnasal point (midline under the nose) to the tragus (the cartilaginous projection in front of the external auditory canal

Cephalic index: The cephalic index, which describes a ratio of the maximum width to the head length expressed as a percentage, is used to assess abnormal head shapes without asymmetry. The maximum width is measured between the most lateral points of the head located in the parietal region (i.e., euryon). The head length is measured from the most prominent point in the median sagittal plane between the supraorbital ridges (i.e., glabella) to the most prominent posterior point of the occiput (i.e., the opisthocranion), expressed as a percentage. The cephalic index can then be compared to normative measures for age and gender. See Table 1 (as developed by AAOP 2004).

Cranial Vault Asymmetry: is assessed by measuring from the frontozygomaticus point (identified by palpation of the suture line above the upper outer corner of the orbit) to the euryon, defined as the most lateral point on the head located in the parietal region.

Plagiocephaly: Flattening of the skull on the back or one side of the head. Sagittal suture: Skull joint that separates the left and right halves of the skull.

Table PG1. Cephalic Index

Sex
Age
-2SD
-1SD
Mean
+1SD
+2SD
Male16 days to 6 months
63.7
68.7
73.7
78.7
83.7
Male6 to 12 months
64.8
71.4
78.0
84.6
91.2
Female16 days to 6 months
63.9
68.6
73.3
78.0
82.7
Female6 to 12 months
69.5
74.0
78.5
83.0
87.5


[RATIONALE: This policy was created in 2001 and has been updated regularly with searches of the MEDLINE database. The most recent literature update was performed through June 20, 2019.

Evidence reviews assess the clinical evidence to determine whether the use of technology improves the net health outcome. Broadly defined, health outcomes are the length of life, quality of life (QOL), and ability to function-including benefits and harms. Every clinical condition has specific outcomes that are important to patients and managing the course of that condition. Validated outcome measures are necessary to ascertain whether a condition improves or worsens; and whether the magnitude of that change is clinically significant. The net health outcome is a balance of benefits and harms.

To assess whether the evidence is sufficient to draw conclusions about the net health outcome of technology, two domains are examined: the relevance, and quality and credibility. To be relevant, studies must represent one or more intended clinical use of the technology in the intended population and compare an effective and appropriate alternative at a comparable intensity. For some conditions, the alternative will be supportive care or surveillance. The quality and credibility of the evidence depend on study design and conduct, minimizing bias and confounding that can generate incorrect findings. The randomized controlled trial is preferred to assess efficacy; however, in some circumstances, nonrandomized studies may be adequate. Randomized controlled trials are rarely large enough or long enough to capture less common adverse events and long-term effects. Other types of studies can be used for these purposes and to assess generalizability to broader clinical populations and settings of clinical practice.

This review was informed by a TEC Assessment (1999) that concluded the evidence on adjustable cranial orthoses as a treatment of positional plagiocephaly was insufficient to permit conclusions.2,

Cranial Orthoses for Craniosynostosis

Clinical Context and Test Purpose

The purpose of postoperative cranial orthosis is to provide a treatment option that is an alternative to or an improvement on existing therapies, such as cranial vault remodeling without a cranial orthosis, in patients with open or endoscopic surgery for craniosynostosis.

The question addressed in this policy is: Does the use of an adjustable cranial orthosis improve the net health outcome in infants who have undergone open or endoscopic surgery for craniosynostosis?

The following PICOs were used to select literature to inform this review.

Patients

The relevant population of interest are individuals with open or endoscopic surgery for craniosynostosis.

Interventions

The therapy being considered is postoperative cranial orthosis.

Comparators

Comparators of interest include cranial vault remodeling without a cranial orthosis. Treatments for craniosynostosis include surgeries such as strip sagittal craniectomy, frontal-orbital advancement, and frontal-occipital reversal.

Outcomes

The general outcomes of interest are a change in disease status, morbid events, functional outcomes, QOL, and treatment-related morbidity. The existing literature evaluating postoperative cranial orthosis as a treatment for open or endoscopic surgery for craniosynostosis has varying lengths of follow-up, ranging from 13 to 25 months. While studies described below all reported at least one outcome of interest, longer follow-up was necessary to fully observe outcomes. Therefore, 12 to 24 months of follow-up is considered appropriate to demonstrate efficacy. Patients with open or endoscopic surgery for craniosynostosis are actively managed by neurosurgeons, plastic surgeons, and primary care providers in an inpatient clinical setting.

Study Selection Criteria

Methodologically credible studies were selected using the following principles:

    a. To assess efficacy outcomes, comparative controlled prospective trials were sought, with a preference for RCTs;
    b. In the absence of such trials, comparative observational studies were sought, with a preference for prospective studies.
    c. To assess long-term outcomes and adverse events, single-arm studies that capture longer periods of follow-up and/or larger populations were sought.
    d. Studies with duplicative or overlapping populations were excluded.

Early literature consisted of a few case series that described the use of cranial orthoses following either open or endoscopically assisted surgery for craniosynostosis. For example, Kaufman et al (2004) reported on 12 children who used a cranial orthosis for 1 year after extended strip craniectomy.3, They found that the orthoses improved Cephalic Index score (100 times the ratio of cranial biparietal diameterand occipitofrontal diameter) more than a similar type of surgery without an orthosis reported elsewhere. The Cephalic Index score improved by 4 (range, 67-71) from baseline to 1 year in studies using surgery alone but improved by 10 (range, 65-75) with combined treatment (Cephalic Index normal range, 75-90). Stevens et al (2007) reported on a study that evaluated 22 patients from a single institution, on the effect of postoperative remolding orthoses following total cranial vault remodeling.4, The children's ages at the time of surgery ranged from 4 to 16 months (average age, 7.5 months). For the 15 (68%) of 22 children treated who completed helmet use and were not lost to follow-up, helmets were wornan average of 134 days. Summary analyses were not provided, because each patient case differed by location of fused suture, extent, and duration of the fusion, and surgical methods used.

Jimenez et al (2002, 2007, 2012) reported on routine use of helmets for 12 months following endoscopically assisted surgery for craniosynostosis in 256 consecutive children.5,6,7, Anthropomorphic measurements at 3, 6, 9, and 12 months after surgery showed continued improvement in symmetry in most patients. Jimenez and Barone (2010) reported on the treatment of 21 infants with multiple-suture (nonsyndromic) craniosynostosis with endoscopically assisted craniectomies and postoperative cranial orthoses.8, Helmet therapy lasted an average of 11 months (range, 10-12 months). The decision to discontinue therapy was based on the child reaching the 12-month postoperative mark or 18 months of age. After the first year postsurgery, patients were followed annually or biannually (range, 3-135 months). The mean preoperative Cephalic Index score was 98. The postoperative Cephalic Index score (>1 year) was 83, a 15% decrease from baseline.

Since these initial reports, literature updates have identified a larger series describing endoscopically assisted strip craniectomy and postoperative helmet therapy for craniosynostosis. They include a series of 97 children with nonsyndromic single-suture synostosis reported by Gociman et al (2012) and a series of 73 children reported by Honeycutt (2014).9,10, Honeycutt (2014)asserted that because head-shape correction occurs slowly after surgery, helmet therapy is as important as the surgery to remove the abnormal suture.

Shah et al (2011) prospectively collected outcomes from endoscopically assisted vs open repair of sagittal craniosynostosis in 89 children treated between 2003 and 2010.11, The endoscopic procedure was offered starting in 2006 and has become the most commonly performed approach. The 42 patients treated with open-vault reconstruction had a mean age at surgery of 6.8 months and a mean follow-up of 25 months. Mean age of the 47 endoscopically treated patients at surgery was 3.6 months and a mean follow-up was 13 months. Of the 29 endoscopically treated patients who completed helmet therapy, the mean duration for helmet therapy was 8.7 months. Noncompliance with helmet therapy has also been reported in a substantial proportion of patients.12,

Section Summary: Cranial Orthoses for Craniosynostosis

The evidence on the efficacy of cranial orthoses following endoscopically assisted or open cranial vault remodeling surgery for craniosynostosis is limited and includes only case series. In the postoperative period after craniosynostosis repair, the role of cranial orthoses is to continue remodeling the skull after surgery. Functional impairments are related to craniosynostosis, including the potential for increased intracranial pressure and the risk of harm from additional surgery when severe deformity has not been corrected. This indirect evidence is considered sufficient to suggest an improvement in health outcomes with postsurgical use of cranial orthosis for craniosynostosis.

Cranial Orthoses for Positional Plagiocephaly

Clinical Context and Test Purpose

The purpose of cranial orthosis is to provide a treatment option that is an alternative to or an improvement on existing therapies, such as positioning therapy, in patients with positional plagiocephaly.

The question addressed in this policy is: Does the use of an adjustable cranial orthosis improve the net health outcome in infants who have positional plagiocephaly?


The following PICOs were used to select literature to inform this review.

Patients

The relevant population of interest are individuals with positional plagiocephaly. Some increase in the prevalence of positional plagiocephaly may be related to the change in recommended sleep practice (back to sleep) to prevent sudden infant death syndrome.

Interventions

The therapy being considered is cranial orthosis.Custom-fitted cranial orthoses are designed to be worn 23 hours a day for several months.

Comparators

Comparators of interest include positioning therapy. Treatment for positional plagiocephaly includes head repositioning and helmet therapy. It is estimated that about two-thirds of plagiocephaly cases may auto-correct spontaneously after regular changes in sleeping position or following physical therapy aimed at correcting neck muscle imbalance. A cranial orthotic device is usually requested after a trial of repositioning fails to correct the asymmetry, or if the child is too immobile for repositioning.

Outcomes

The general outcomes of interest are a change in disease status, morbid events, functional outcomes, QOL, and treatment-related morbidity. Guideline-related systematic reviews reported a mean duration of cranial orthotic as four-six months depending on the age of the patient with longer-term outcome assessments reported at two years. Patients with positional plagiocephaly are managed by neurologists, pediatricians and other primary care providers in an outpatient clinical setting.

Study Selection Criteria

Methodologically credible studies were selected using the following principles:

    a. To assess efficacy outcomes, comparative controlled prospective trials were sought, with a preference for RCTs;
    b. In the absence of such trials, comparative observational studies were sought, with a preference for prospective studies.
    c. To assess long-term outcomes and adverse events, single-arm studies that capture longer periods of follow-up and/or larger populations were sought.
    d. Studies with duplicative or overlapping populations were excluded.

Positional Plagiocephaly and Anthropometric Outcomes

Results from a pragmatic multicenter, single-blinded, randomized controlled trial, HElmet therapy Assessment in Deformed Skulls, were reported in 2014.13, The trial included 84 infants ages 5 to 6 months with moderate-to-severe skull deformation (oblique diameter difference index ≥108% or cranioproportional index ≥95%) who were randomized to cranial orthoses for 6 months or to the natural course (observation). It should be noted that 3% of infants recruited were excluded from the trial due to very severe deformation (oblique diameter difference index >113% or cranioproportional index >104%). Of the 42 infants randomized to a cranial orthosis, 10 (23%) wore a cranial orthosis until 12 months of age. Parents of 10 infants discontinued treatment before 12 months due to adverse events. The primary outcome (change score for plagiocephaly [oblique diameter difference index] and brachycephaly [cranioproportional index] at 24 months) was similar for the 2 groups. Full recovery was reported for 26% of children in the orthoses group and 23% of children in the observation arm (odds ratio, 1.2; 95% confidence interval, 0.4 to 3.3; p=0.74).

A systematic review by McGarry et al (2008) described 9 publications involving the use of cranial orthoses.14, More than half of the studies were retrospective cohorts; none was randomized. For studies comparing orthoses with active counter positioning, one reported greater decreases in posterior cranial asymmetry (from 12 to 0.6 mm) than treatment of infants using repositioning alone (from 12 to 10 mm); other studies found faster, but ultimately similar, reductions in asymmetry with helmets.15,16, Another 2008 systematic review identified 7 cohort studies meeting selection criteria.17, In most studies, physicians offered (and parents elected) the method of treatment, resulting in a bias toward older infants and greater deformity in the molding groups. One study (2005) included 159 infants with molding therapy and 176 treated with repositioning and physical therapy.18, Molding therapy was recommended for infants older than six months with more severe deformity, and repositioning was recommended for infants four months or younger. Both treatments were offered for infants between 4 and 6 months of age, although anthropomorphic measurements indicated that molding therapy was effective in 93% of infants, while repositioning was effective in 79% of infants. In this review, the relative risk was 1.3 favoring molding therapy. A prospective longitudinal study by Kluba et al (2014) evaluated 128 infants treated with or without a helmet; authors found that, although children treated with a helmet had more severe asymmetry originally, they showed significantly more improvement (68% vs 31%).19, In a study of 1050 infants, Couture et al (2013) reported on the successful use of off-the-shelf helmet therapy.20, Infants with an Argenta classification type I (minimal deformity) were treated with repositioning while infants with an Argenta severity rating of II to V were treated with a helmet. Correction (overall rate, 81.6%) took longer in patients with an Argenta severity of III, IV, and V compared with Argenta type II, but was not significantly affected by age.

Positional Plagiocephaly and Functional Outcomes

Since the publication of the TEC Assessment (1999), few studies have examined the association between positional plagiocephaly and functional impairments. Some, such as that by Fowler et al (2008), found no difference in the neurologic profile, posture, or behavior of 49 infants with positional plagiocephaly compared with 50 age-matched concurrent controls.21,

Other studies have compared developmental outcomes in children using positional plagiocephaly with normative values. Panchal et al (2001) reported that scores from a standardized measure of mental and psychomotor development differed significantly from the expected standardized distribution, with 8.7% of children categorized as severely delayed on the Mental Development Index compared with the expected 2.5%.22, A study by Miller and Clarren (2000) obtained responses on long-term developmental outcomes in 63 of 181 children asked to participate in this study.23, Results were limited by the lack of concurrent controls and potential self-selection population bias. In addition, these studies did not evaluate the possible causal relation for the observed association. For example, children with preexisting development delays or weakness might be at a higher risk for plagiocephaly if they were more apt to lie in one position for extended periods of time.

The effect of treatment for positional plagiocephaly on health outcomes has also been investigated. For example, Shamij et al (2012) surveyed parents of 80 children treated for positional plagiocephaly to assess the cosmetic outcome, school performance, language skills, cognitive development, and societal function.24, Analysis indicated that the children of respondents were representative of the total pool. Positional therapy was applied in all children, while 36% also used helmet therapy. At a median follow-up of 9 years, a normal head appearance was reported in 75% of cases. Compared with right-sided deformation, left-sided plagiocephaly was associated with a need for special education classes (27% vs 10%), fine motor delay (41% vs 22%), and speech delay (36% vs 16%).

Section Summary: Cranial Orthoses for Positional Plagiocephaly

Results from the HElmet therapy Assessment in Deformed Skulls trial have suggested that, in a practice setting, the effectiveness of cranial orthoses may not differ from the natural course of development for infants with moderate to severe plagiocephaly and brachycephaly. However, the validity of these results is limited by the low percentage of infants who wore the cranial orthoses for the duration of the trial and the relatively low percentage of infants who achieved recovery in either group. In addition, the efficacy of cranial orthoses in infants with very severe plagiocephaly was not addressed. A few reports have assessed the association between positional plagiocephaly and functional impairments. The largest controlled study found no difference in function between infants with plagiocephaly and age-matched concurrent controls. While some series have suggested an association between plagiocephaly and developmental delay, they lacked controls and did not evaluate the possible causal relation to observed association. Results of a study on right-sided vs left-sided plagiocephaly suggested an association between left-sided and functional performance but these results have not been confirmed. During the 2019 update for this policy, professional society clinical input was sought with a response that acknowledged the evidence limitations but an endorsement of current professional guidelines.

Summary of Evidence

For individuals who have open or endoscopic surgery for craniosynostosis who receive a postoperative cranial orthosis, the evidence includes case series. The relevant outcomes are a change in disease status, morbid events, functional outcomes, QOL, and treatment-related morbidity. Overall, the evidence on the efficacy of cranial orthoses following endoscopic-assisted or open cranial vault remodeling surgery for craniosynostosis is limited. However, functional impairments are related to craniosynostosis, and there is a risk of harm from additional surgery when severe deformity has not been corrected. Because cranial orthoses can facilitate remodeling, use of a cranial orthosis is likely to improve outcomes after cranial vault remodeling for synostosis. The evidence is sufficient to determine that the technology results in a meaningful improvement in the net health outcome.

For individuals who have positional plagiocephaly who receive a cranial orthosis, the evidence includes a comparative study and case series. The relevant outcomes are a change in disease status, morbid events, functional outcomes, QOL, and treatment-related morbidity. Overall, evidence on an association between positional plagiocephaly and health outcomes is limited. The largest controlled study found no difference in function between infants with plagiocephaly and age-matched concurrent controls. Taking into consideration the limited number of publications over the past decade and the low likelihood of development of high-level evidence from controlled studies, the scientific literature  is limited in support of an effect of deformational plagiocephaly on functional health outcomes. The evidence is insufficient to determine the effects of the technology on health outcomes. However, during the 2019 update for this policy, professional society clinical input was sought with a response that acknowledged the evidence limitations but an endorsement of current professional guidelines.

SUPPLEMENTALINFORMATION

Clinical Input From Physician Specialty Societies and Academic Medical Centers

While the various physician specialty societies and academic medical centers may collaborate with and make recommendations during this process, through the provision of appropriate reviewers, input received does not represent an endorsement or position statement by the physician specialty societies or academic medical centers, unless otherwise noted.

In response to requests, input was received from 3 physician specialty societies (4 reviews) and 2 academic medical centers while this policy was under review in 2008. Input was mixed about whether the use of helmets or adjustable banding for treatment of plagiocephaly or brachycephaly without synostosis should be considered medically necessary or not medically necessary. Input agreed that cranial orthoses may be indicated following cranial vault surgery.

Practice Guidelines and Position Statements

The Congress of Neurological Surgeons and the Section on Pediatric Neurosurgery(2016) published a joint evidence-based guideline on the role of cranial molding orthosis therapy for patients with positional plagiocephaly.25,26,The guideline was endorsed by the Joint Guidelines Committee of the American Association of Neurological Surgeons and the Congress of Neurological Surgeons and American Academy of Pediatrics (AAP).

The guideline provided level II recommendations (uncertain clinical certainty) on the use of helmet therapy "for infants with persistent moderate to severe plagiocephaly after a course of conservative treatment (repositioning and/or physical therapy)" and "for infants with moderate to severe plagiocephaly presenting at an advanced age." The recommendations were based on a randomized controlled trial, five prospective comparative studies, and nine retrospective comparative studies (all rated as class II evidence).

National Institute of Neurological Disorders and Stroke

The National Institute of Neurological Disorders and Stroke (2017) has stated that "Treatment for craniosynostosis generally consists of surgery to improve the symmetry and appearance of the head and to relieve pressure on the brain and the cranial nerves [although] for some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head."27,

National Health Service Quality Improvement

Scotland's National Health Service Quality Improvement (2007) issued an evidence note on the use of cranial orthosis treatment for infant deformational plagiocephaly.28, No evidence-based conclusions could be reached due to the limited methodologic quality of available trials.

American Academy of Pediatrics

The AAP (2011) revised its 2003 policy on the prevention and management of positional skull deformities in infants.29,30,The AAP indicated that in most cases, the diagnosis and successful management of deformational plagiocephaly can be assumed by the pediatrician or primary health care clinician and that mechanical methods if performed early in life, may prevent further skull deformity and may reverse existing deformity. In most cases, improvement is seen over a 2- to 3-month period with repositioning and neck exercises, especially if these measures are instituted as soon as the condition is recognized. The AAP indicated that use of helmets and related devices seems to be beneficial primarily when there has been a lack of response to mechanical adjustments and exercises, and the best response to helmets occurs in the age range of 4 to 12 months of age.

In a policy statement, the AAP (2011) indicated that consideration should be given to early referral of infants with plagiocephaly when it is evident that conservative measures have been ineffective, because orthotic devices may help avoid the need for surgery in some cases.31,The AAP also recommended placing infants on their backs for sleep with supervised "tummy time" for the prevention of plagiocephaly.

U.S. Preventive Services Task Force Recommendations

Not applicable.

Ongoing and Unpublished Clinical Trials

Some currently unpublished trials that might influence this review are listed in Table 1.

Table 1. Summary of Key Trials
NCT No.Trial Name
Planned Enrollment
Completion Date
Ongoing 
 
 
NCT02370901aCranial Orthotic Device Versus Repositioning Techniques for the Management of Plagiocephaly: the CRANIO Randomized Trial
226
Nov 2020
a Denotes industry-sponsored or cosponsored trial.

NCT: national clinical trial.]
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Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

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Index:
Adjustable Cranial Orthoses for Positional Plagiocephaly and Craniosynostoses
Cranial Orthosis for Positional Plagiocephaly and Craniosynostoses
Cranial Banding
Cranial Remodeling
CranioCap
Craniosynostoses, Cranial Orthosis for
DOC Band
Dynamic Orthotic Cranioplasty
Helmet Therapy
Non-Synostotic Plagiocephaly, Cranial Orthosis for
Plagiocephaly, Cranial Orthosis for
Positional Plagiocephaly, Cranial Orthosis for
Skull Molding Caps
STAR Band
Synostotic Plagiocephaly, Cranial Orthosis for

References:
1. Persing JA. MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg. Apr 2008;121(4 Suppl):1-11. PMID 18379381

2. Blue Cross and Blue Shield Association Technology Evaluation Center (TEC). Cranial Orthosis for Plagiocephaly without Synostosis. TEC Assessments. 1999;Volume 14:Tab 21.

3. Kaufman BA, Muszynski CA, Matthews A, et al. The circle of sagittal synostosis surgery. Semin Pediatr Neurol. Dec 2004;11(4):243-248. PMID 15828707

4. Stevens PM, Hollier LH, Stal S. Post-operative use of remoulding orthoses following cranial vault remodelling: a case series. Prosthet Orthot Int. Dec 2007;31(4):327-341. PMID 18050005

5. Jimenez DF, Barone CM, Cartwright CC, et al. Early management of craniosynostosis using endoscopic-assisted strip craniectomies and cranial orthotic molding therapy. Pediatrics. Jul 2002;110(1 Pt 1):97-104. PMID 12093953

6. Jimenez DF, Barone CM. Early treatment of anterior calvtimes craniosynostosis using endoscopic-assisted minimally invasive techniques. Childs Nerv Syst. Dec 2007;23(12):1411-1419. PMID 17899128

7. Jimenez DF, Barone CM. Endoscopic technique for sagittal synostosis. Childs Nerv Syst. Sep 2012;28(9):1333- 1339. PMID 22872245

8. Jimenez DF, Barone CM. Multiple-suture nonsyndromic craniosynostosis: early and effective management using endoscopic techniques. J Neurosurg Pediatr. Mar 2010;5(3):223-231. PMID 20192637

9. Gociman B, Marengo J, Ying J, et al. Minimally invasive strip craniectomy for sagittal synostosis. J Craniofac Surg. May 2012;23(3):825-828. PMID 22565892

10. Honeycutt JH. Endoscopic-assisted craniosynostosis surgery. Semin Plast Surg. Aug 2014;28(3):144-149. PMID 25210508

11. Shah MN, Kane AA, Petersen JD, et al. Endoscopically assisted versus open repair of sagittal craniosynostosis: the St. Louis Children's Hospital experience. J Neurosurg Pediatr. Aug 2011;8(2):165-170. PMID 21806358

12. Chan JW, Stewart CL, Stalder MW, et al. Endoscope-assisted versus open repair of craniosynostosis: a comparison of perioperative cost and risk. J Craniofac Surg. Jan 2013;24(1):170-174. PMID 23348279

13. van Wijk RM, van Vlimmeren LA, Groothuis-Oudshoorn CG, et al. Helmet therapy in infants with positional skull deformation: randomised controlled trial. BMJ. May 1 2014;348:g2741. PMID 24784879

14. McGarry A, Dixon MT, Greig RJ, et al. Head shape measurement standards and cranial orthoses in the treatment of infants with deformational plagiocephaly. Dev Med Child Neurol. Aug 2008;50(8):568-576. PMID 18754893

15. Mulliken JB, Vander Woude DL, Hansen M, et al. Analysis of posterior plagiocephaly: deformational versus synostotic. Plast Reconstr Surg. Feb 1999;103(2):371-380. PMID 9950521

16. Loveday BP, de Chalain TB. Active counterpositioning or orthotic device to treat positional plagiocephaly? J Craniofac Surg. Jul 2001;12(4):308-313. PMID 11482615

17. Xia JJ, Kennedy KA, Teichgraeber JF, et al. Nonsurgical treatment of deformational plagiocephaly: a systematic review. Arch Pediatr Adolesc Med. Aug 2008;162(8):719-727. PMID 18678803

18. Graham JM, Jr., Gomez M, Halberg A, et al. Management of deformational plagiocephaly: repositioning versus orthotic therapy. J Pediatr. Feb 2005;146(2):258-262. PMID 15689920

19. Kluba S, Kraut W, Calgeer B, et al. Treatment of positional plagiocephaly--helmet or no helmet? J Craniomaxillofac Surg. Jul 2014;42(5):683-688. PMID 24238984

20. Couture DE, Crantford JC, Somasundaram A, et al. Efficacy of passive helmet therapy for deformational plagiocephaly: report of 1050 cases. Neurosurg Focus. Oct 2013;35(4):E4. PMID 24079783

21. Fowler EA, Becker DB, Pilgram TK, et al. Neurologic findings in infants with deformational plagiocephaly. J Child Neurol. Jul 2008;23(7):742-747. PMID 18344457

22. Panchal J, Amirsheybani H, Gurwitch R, et al. Neurodevelopment in children with single-suture craniosynostosis and plagiocephaly without synostosis. Plast Reconstr Surg. Nov 2001;108(6):1492-1498; discussion 1499-1500. PMID 11711916

23. Miller RI, Clarren SK. Long-term developmental outcomes in patients with deformational plagiocephaly. Pediatrics. Feb 2000;105(2):E26. PMID 10654986

24. Shamji MF, Fric-Shamji EC, Merchant P, et al. Cosmetic and cognitive outcomes of positional plagiocephaly treatment. Clin Invest Med. Oct 6 2012;35(5):E266. PMID 23043707

25. Tamber MS, Nikas D, Beier A, et al. The Role of Cranial Molding Orthosis (Helmet) Therapy. 2016; https://www.cns.org/guidelines/guidelines-management-patients-positional-plagiocephaly/Chapter_5. Accessed August 2, 2019.

26. Tamber MS, Nikas D, Beier A, et al. Guidelines: Congress of Neurological Surgeons systematic review and evidence-based guideline on the role of cranial molding orthosis (helmet) therapy for patients with positional plagiocephaly. Neurosurgery. Nov 2016;79(5):E632-E633. PMID 27759675

27. National Institute of Neurological Disorders and Stroke (NINDS). Craniosynostosis Information Page. 2017; https://www.ninds.nih.gov/Disorders/All-Disorders/Craniosynostosis-Information-Page. Accessed August 2, 2019.

28. NHS Quality Improvement Scotland. The use of cranial orthosis treatment for infant deformational plagiocephaly. Evidence Note No. 16. 2007; http://www.healthcareimprovementscotland.org/programmes/medicines_and_technologies/archived_evidence_n otes/evidence_note_16.aspx. Accessed August 2, 2019.

29. Persing J, James H, Swanson J, et al. Prevention and management of positional skull deformities in infants. American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery. Pediatrics. Jul 2003;112(1 Pt 1):199-202. PMID 12837890

30. Laughlin J, Luerssen TG, Dias MS, et al. Prevention and management of positional skull deformities in infants. Pediatrics. Dec 2011;128(6):1236-1241. PMID 22123884

31. Task Force on Sudden Infant Death Syndrome, Moon RY. SIDS and other sleep-related infant deaths: expansion of recommendations for a safe infant sleeping environment. Pediatrics. Nov 2011;128(5):1030-1039. PMID 22007004

Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)

CPT*

HCPCS

    S1040

* CPT only copyright 2019 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.

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Medical policies can be highly technical and are designed for use by the Horizon BCBSNJ professional staff in making coverage determinations. Members referring to this policy should discuss it with their treating physician, and should refer to their specific benefit plan for the terms, conditions, limitations and exclusions of their coverage.

The Horizon BCBSNJ Medical Policy Manual is proprietary. It is to be used only as authorized by Horizon BCBSNJ and its affiliates. The contents of this Medical Policy are not to be copied, reproduced or circulated to other parties without the express written consent of Horizon BCBSNJ. The contents of this Medical Policy may be updated or changed without notice, unless otherwise required by law and/or regulation. However, benefit determinations are made in the context of medical policies existing at the time of the decision and are not subject to later revision as the result of a change in medical policy

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