44_Section_L _Exhibit_L-8_Sample_Short_Assessment.pdf

TQMC HT9402-15-R-0005 Section L, Exhibit L-8

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

Cranial Helmets and Other Orthotic Treatment of Plagiocephaly

(Short HTA)

February 4, 2013

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

TABLE OF CONTENTS

PURPOSE OF TECHNOLOGY

EXECUTIVE SUMMARY

Response to Key Questions

Evidence-Based Conclusions

Other Considerations

Policy Guidance

MEDICAL BACKGROUND

POLICY CONTEXT

DESCRIPTION

EVALUATION OF CLINICAL RESEARCH EVIDENCE

Search Strategy and Study Selection Criteria

Literature Review

GUIDELINES/REGULATORY STATUS/COVERAGE POLICIES

Published Technology Assessments and Systematic Reviews

Practice Guidelines

Regulatory Status

Coverage Policies

RELATED ISSUES

Cost

Economic Evaluations

Social, Legal, or Ethical Issues

ONGOING OR FUTURE RESEARCH

REFERENCES

APPENDICES

APPENDIX I. Literature Search Strategy

APPENDIX II. Evidence Quality Assessment

APPENDIX III. Evidence Tables

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

PURPOSE OF TECHNOLOGY

Cranial orthotic devices are used to redirect growth of the skull bones and reduce cranial asymmetry in infants who have nonsynostotic positional plagiocephaly.

EXECUTIVE SUMMARY

Cranial orthotic devices are used in infants for the treatment of positional plagiocephaly, deformation of the head that results from external pressure applied to the soft infant skull. The deformity can begin in utero but most commonly is associated with infants sleeping or lying on their backs, especially if the head is turned in the same direction for long periods of time. If detected during the first few months of life, frequent repositioning of the baby’s head combined with prone positioning during waking hours can correct the condition in the majority of children. In some babies, congenital muscular torticollis, or weakness of the neck muscles, can be a predisposing factor for development of positional plagiocephaly. For these children, physical therapy and massage to lengthen the neck muscles may be required in addition to repositioning. Neurodevelopmental delay may also contribute to a positional preference, and thus increase the likelihood that an infant will develop positional plagiocephaly. If the cranial asymmetry is not detected early or if repositioning therapy is unsuccessful, then cranial orthotic devices, such as adjustable head bands or plastic helmets, can be used to gradually mold the infant’s skull back into place over a period of weeks or several months. Surgical correction is rarely indicated for babies with positional plagiocephaly and would only be considered if a severe asymmetry remained uncorrected in a child older than 1 year of age.

Key Questions

The TRICARE MANAGEMENT AUTHORITY (TMA) has requested that this report address these issues:

Whether reliable evidence exists that untreated positional plagiocephaly results in vision, hearing, or respiratory impairments, or that treating plagiocephaly corrects or improves a bodily function, and whether the safety and efficacy have been proven to be equal or superior to conventional therapies.

Therefore, this report addresses the following key questions:

• Do cranial orthotic devices correct deformity; reduce the need for corrective surgery; or reduce the adverse effects of plagiocephaly on physiological function, overall function, or quality of life?

• How does the efficacy of cranial orthotic devices compare with that of other plagiocephaly treatment methods?

• Are any safety issues associated with cranial orthotic devices?

Response to Key Questions

Evidence evaluated for this report was obtained primarily from a search of the PubMed and Embase databases.

The literature search identified 16 peer-reviewed studies assessing the efficacy of cranial orthotics for the

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures treatment of positional plagiocephaly, including 13 prospective and 3 retrospective studies. Study sample sizes ranged from 27 to 346 patients who had confirmed nonsynostotic plagiocephaly. Cranial orthotics were typically worn for 4 to 7 months, although shorter treatment periods were reported in a few of the articles. To measure cosmetic outcome, cranial dimensions were obtained using calipers or laser devices or by assessment of reconstructions of the head derived from three-dimensional photographs. One of the studies evaluated the effect of cranial orthotic therapy on neurodevelopmental outcomes, and one study provided long-term follow-up to evaluate the impact of plagiocephaly treatment on function and psychosocial development. However, no studies evaluated the effect on physiological outcomes. None of the studies directly compared the different types of orthotic devices. Several of the studies reported on outcomes in patients treated with orthotic devices compared with those treated with repositioning therapy.

Correction of Cranial Asymmetry

A total of 10 uncontrolled studies evaluated the effect of cranial orthotics on reducing asymmetry in infants with positional plagiocephaly, including nine prospective studies and one retrospective study. Overall, the study results suggest that cranial orthotic devices significantly improve cranial asymmetry in infants. The duration and success of treatment were dependent on the age at initiation of treatment. Younger patients achieved more rapid success and more pronounced improvement than older patients. To achieve treatment success, cranial orthotics were worn for a significantly longer duration in older infants compared with younger infants. None of the studies explicitly evaluated the impact of cranial orthotic therapy on the incidence of surgical correction. Surgery is generally not indicated for positional plagiocephaly, although one older study reported surgical correction of some severely affected infants.

Correction of Adverse Effects of Cranial Asymmetry

Only one study evaluated non-cosmetic outcome measures of plagiocephaly treatments. To ascertain the effect of plagiocephaly treatment on subsequent cognitive development, school performance, language skills, and societal function, a retrospective survey of children was performed. The children had a median age of 9 years when the survey was conducted. Plagiocephaly treatment methods achieved good cosmetic outcome, and fewer than 10% of children represented by the survey were aware of a head deformity or had been teased about head shape.

Nevertheless, the children in the study had a higher prevalence of developmental delays than seen in the average population. The study was underpowered to identify outcome differences between plagiocephaly treatment methods, and, thus, the authors did not provide separate data for repositioning plus orthotics versus repositioning alone. The survey response rate was poor (23%); therefore, study bias cannot be excluded.

Efficacy of Cranial Orthotics Compared with Alternative Treatments

A total of six studies compared the efficacy of cranial orthotics with active repositioning treatment, including four prospective studies and two retrospective studies. While both repositioning methods and cranial orthotic therapy were generally able to reduce cranial asymmetry satisfactorily, orthotic devices achieved success more rapidly and/or to a somewhat greater extent than repositioning methods in some studies. However, follow-up did not extend beyond the treatment period. The results of one study suggested that younger patients achieved more rapid success than older patients, and that patients treated with helmets improved more quickly than patients

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures treated with repositioning. In a prospective study of 114 infants, although 98% of the infants had satisfactory improvement in plagiocephaly, those treated with cranial orthotics had significantly better posterior cranial asymmetry than those treated with repositioning methods. In another study, infants in both treatment groups had equivalent reductions in cranial vault asymmetry; however, those in the cranial orthotic group had significantly greater improvements in cranial asymmetry than those treated by repositioning methods. In one retrospective study, there were no significant differences in treatment outcomes between the two groups, although the duration of treatment was one third shorter in the orthotic group than in the repositioning group. In a prospective study, > 30% of patients who were treated by repositioning methods showed worse rather than improved asymmetry at the follow-up assessment.

Safety of Cranial Orthotics

In general, cranial orthotics are safe devices and most complications do not require permanent discontinuation of treatment. Of the 16 reviewed studies, complications of cranial orthotic treatment were reported by only 1 study group. The complications consisted of skin injury from malfitting helmets, skin reactions to helmet materials, and occasional heat rash. A retrospective institutional database analysis was identified that reported complications associated with cranial orthotic devices in a group of 410 patients who received treatment for positional plagiocephaly. Complications occurred in 22% of patients and were classified into 5 different categories: pressure sores (11% of patients), ethanol erythema (6% of patients), skin infection (1% of patients), bacterial abscess (0.2% of patients), and malfitting helmet (6% of patients). There was no relationship between severity of deformity and the incidence of complications. Most complications of cranial orthotic device treatment can be avoided by thorough instructions to the parents.

Quality of the Evidence

The quality of the individual studies ranged from very poor to fair. There were no randomized controlled trials.

The noncomparative studies consisted of before-and-after assessments, which are of limited validity for a condition that has the potential to improve or resolve spontaneously. In the comparison studies, treatment method was often dictated by the parent and in some studies the two treatment groups were not equal in size.

Furthermore, comparative studies did not follow patients long enough to determine whether differences in outcome might diminish as children grew. In the medical community, there is a lack of consensus about what constitutes the severity of plagiocephaly as well as what constitutes treatment success. This led to variability in outcome measures in the reviewed studies. Thus, the evidence pertaining to the correction of deformity is of low quality. There was no direct evidence that cranial helmets reduce the need for corrective surgery, although this may be inferred from the positive effects on asymmetry. Evidence regarding reduction in the adverse effects of plagiocephaly on physiological function, overall function, or quality of life was insufficient (one survey of very poor quality).

Evidence-Based Conclusions

There is low-quality evidence that cranial orthotic devices can reduce or eliminate the cranial asymmetry associated with positional plagiocephaly when therapy is initiated before 12 to 18 months of age, and that there are few adverse effects. There is also some evidence that older age at initiation of treatment is associated with

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures less improvement in cranial asymmetry. Comparative studies indicated that repositioning can also reduce cranial deformity, although repositioning may take longer than cranial orthotic therapy and may not provide the same degree of correction over the short term in some infants. There was no evidence from the available studies that cranial orthotic therapy improved health outcomes or reduced the risk of neurodevelopmental delay or other problems, such as dental malocclusion.

Other Considerations

• Aetna, Humana, and UnitedHealthcare cover cranial orthotic treatment of moderate to severe positional plagiocephaly in children less than 18 months of age who have not responded adequately to a 2-month trial of conservative treatment.

• According to the American Academy of Pediatrics (AAP), there is currently no evidence that cranial orthotics work better than repositioning measures for infants with mild or moderate cranial deformity.

For patients with severe plagiocephaly, the best response for helmet treatment occurs in children age 4 to 12 months due to the malleability of the infant skull.

• The Canadian Paediatric Society (CPS) recommends repositioning therapy for patients who are < 4 months of age and have mild to moderate asymmetry and recommends cranial molding therapy for patients with severe asymmetry who are < 8 months of age.

• The literature reviewed for this report referenced no longitudinal studies designed to demonstrate a plausible cause-and-effect relationship between plagiocephaly and health or developmental problems.

• A recent study found a low prevalence of plagiocephaly in a group of teens born during the era when parents were encouraged to place their infants on their backs for sleeping, which suggests that children may outgrow cranial asymmetry without intervention.

• Studies that might address gaps in the evidence, such as the impact of cranial orthotics on health outcomes or risk of developmental delay, do not appear imminent.

Policy Guidance

There is some evidence that cranial orthotic devices are effective in reducing skull asymmetry associated with positional plagiocephaly, especially in infants who are 4 to 12 months of age, and compared with repositioning therapy may provide more complete and/or more rapid correction of the asymmetry . However, there is no evidence that cranial orthotic therapy improves health outcomes or reduces the risk of future health problems, such as neurodevelopmental delay, nor is there evidence that nonsynostotic occipital plagiocephaly causes health problems. Therefore, since the TRICARE benefit excludes treatments that are expected primarily to improve physical appearance or restore form, but that do not correct or materially improve a bodily function, continuation of the noncoverage policy for cranial orthotic therapy is recommended.

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

MEDICAL BACKGROUND

Positional plagiocephaly, also called benign positional molding, lambdoid plagiocephaly, deformational plagiocephaly, and occipital or posterior plagiocephaly, is a deformity of the skull in which a portion of the head, usually the occiput, or back part of the head, is flattened due to increased pressure on that portion of the skull.

The forehead and the cheek on the affected side may also be somewhat prominent, and the ear on the affected side may be pushed forward compared with the opposing ear. The effects of positional plagiocephaly on health and developmental outcomes are uncertain. Ocular and dental problems, as well as facial deformity, are thought to be potential consequences from the asymmetrical growth of the cranium and there is a significant association between plagiocephaly and developmental delays. However, it remains to be determined whether head deformity is the cause or result of developmental delays (Rekate, 1998; Persing et al., 2003; Collett et al., 2005;

McGarry et al., 2008; Collett et al., 2011; Looman and Flannery, 2012).

There are a number of predisposing factors for development of plagiocephaly, including premature birth, firstborn birth order, intrauterine positioning, breech presentation, length of labor, twin pregnancy, and a supine sleeping position. Another cause is congenital muscular torticollis, in which a shortened neck muscle on one side causes the infant to keep the head in one position. The back-sleeping position, currently recommended to reduce the risk of sudden infant death syndrome (SIDS), has been associated with an increased frequency of positional plagiocephaly due to the pressure of a firm mattress against the back of the head. The incidence of deformational plagiocephaly is estimated to occur in < 1% to 48% of healthy infants under 1 year of age (Littlefield et al., 1999;

Mulliken et al., 1999; Miller and Clarren, 2000; Boere-Boonekamp et al., 2001; Littlefield et al., 2002; Peitsch et al., 2002; Biggs, 2003; Persing et al., 2003; Losee et al., 2007; Bialocerkowski et al., 2008; McKinney et al., 2008; Xia et al., 2008; McKinney et al., 2009; Laughlin et al., 2011; Looman and Flannery, 2012).

Positional plagiocephaly must be differentiated from craniosynostosis, a condition in which one or more of the sutures of an infant’s skull close prematurely, resulting in a misshapen head that becomes progressively more deformed as the child grows. True craniosynostosis occurs much less frequently than positional plagiocephaly and is currently estimated to affect < 3 infants per 100,000 births. Craniosynostosis usually requires surgical treatment to reshape the bone in the affected area, while surgical treatment is only rarely indicated for positional plagiocephaly. In the past, many infants with cranial asymmetry were incorrectly diagnosed with craniosynostosis, and neurosurgeons and craniofacial surgeons have only recently begun to recognize that most of these individuals do not require surgery, especially if the condition is diagnosed during the first year of life. The diagnostic criteria for positional plagiocephaly include a unilateral flattening of the occipital area, ipsilateral frontal and parietal bossing, cheekbone prominence, and an anterior ear displacement ipsilateral to the flattened occiput forming a parallelogram when observing the top of the child’s head. Radiographs and computed tomography (CT) scans can be used to aid the diagnosis, although the images must be interpreted with caution since open cranial sutures may appear to be fused when they overlap due to compression of the skull on one side (Huang et al., 1996; Pople et al., 1996; Persing et al., 2003; van Vlimmeren et al., 2007; Looman and Flannery, 2012).

The initial treatment for positional plagiocephaly involves repositioning the infant so that there is no pressure on the flattened region of the skull. This is started as soon as the condition is diagnosed and is most successful in babies younger than 6 months of age. Frequent supervised prone positioning can assist the infant in developing upper body strength. To reduce the tendency to consistently turn the head to the same side, infants with

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures torticollis receive additional treatment and physical therapy, including neck muscle massage, stretching exercises, and, in rare cases, surgery. If improvement of the deformity is not apparent after several months of repositioning or if the asymmetry is severe, an orthotic device may be used to apply pressure to prominent regions of the infant’s skull to redirect growth in the flattened regions. The types of orthotic devices used for this condition include: (1) a custom-made molding helmet that allows for normal growth of the brain while preventing further distortion of the prominent areas of the cranium; and (2) a headband, which is based on a concept similar to the helmet but is open on the top and applies more pressure to redirect skull growth. The timely use of cranial orthotics may allow an infant’s head shape to alter sufficiently to avoid the need for surgery. In very few instances, the deformity is severe and persistent and requires reconstructive surgery for correction, which is usually performed between 12 and 18 months of age (Rekate, 1998; Persing et al., 2003; Collett et al., 2005;

Robinson and Proctor, 2009; Laughlin et al., 2011).

POLICY CONTEXT

TRICARE currently covers the Dynamic Orthotic Cranioplasty (DOC) Band-PostOp devices for adjunctive use for infants from 3 to 18 months of age whose synostosis has been surgically corrected but who still have moderate-to-severe cranial deformities, including plagiocephalic-, brachycephalic-, and scaphocephalic-shaped heads.

Current policy excludes coverage of cranial orthosis or cranial molding helmets for the treatment of nonsynostotic positional plagiocephaly (deformational plagiocephaly, plagiocephaly without synostosis) or for the treatment of craniosynostosis before surgery. However, TRICARE is being petitioned to provide coverage for cranial helmets to treat positional plagiocephaly.

DESCRIPTION

Cranial orthotic devices are constructed by first making a plaster mold of the patient’s skull. The mold is then used to create a plastic model, which is custom-fitted with inserts to provide gentle pressure to the areas of maximal protuberance and redirect growth to the flattened areas of the skull. The formation of the plaster mold and fitting of the device can be accomplished without sedation or anesthesia. The orthosis is worn continuously, and the parents are instructed to check the scalp frequently for signs of irritation or abrasion. The headband type of orthosis fits more tightly than the molding helmets and must be adjusted by trained personnel every 1 to 2 weeks.

The response to treatment is monitored by anthropometric measurements of the cranium and clinical assessment, with radiographic or computed tomography (CT) imaging, if needed. Cranial orthotic therapy typically is started at 5 to 6 months of age and continues for an average of 4 months, although the length of treatment varies with the severity of the asymmetry and the response to the orthotic device. Physical therapy and neck-stretching exercises are also performed if congenital muscular torticollis is present (Rekate, 1998; Loveday and deChapain, 2001; Aliberti et al., 2002; Losee et el., 2007; Bialocerkowski et al., 2008).

EVALUATION OF CLINICAL RESEARCH EVIDENCE

Search Strategy and Study Selection Criteria

Evidence evaluated for this report was obtained primarily from a search of the PubMed and Embase databases spanning 1966 to January 2013. Search terms included (cranial orthosis OR helmet) AND plagiocephaly. Studies

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures were selected for detailed review if they were published in the peer-reviewed literature in English-language journals. Studies of cranial orthotic device use after surgical correction of plagiocephaly and those that only reported qualitative or subjective measures of efficacy were excluded. Studies of cranial cups, which are foam devices designed to cradle the newborn’s head during sleep, were also excluded.

Literature Review

Description of Studies

The literature search identified 16 peer-reviewed studies assessing the efficacy of cranial orthotics for the treatment of positional plagiocephaly, including 13 prospective studies and 3 retrospective studies. Details of the reviewed studies are presented in Appendix III. Study sample sizes ranged from 27 to 346 patients. The average age of the patients was 6.5 months and the ages typically ranged from 3 to 12 months. Information on study inclusion criteria was sparse; however, all of the studies required that the patients have nonsynostotic plagiocephaly. The severity of the study subject’s plagiocephaly was rarely reported. Cranial orthotics were typically worn for 4 to 7 months, although shorter treatment periods were reported in a few of the articles. To measure cosmetic outcome, cranial dimensions were obtained using calipers or laser devices or by assessment of reconstructions of the head derived from three-dimensional photographs. The majority of the studies evaluated the cosmetic effect of orthotic devices. Outcome measures for correction of cranial asymmetry included cranial vault asymmetry (CVA), cranial vault asymmetry index (CVAI), and cranial (or cephalic) index (CI). The CI is the ratio of the head width to head length. The CVA measurement is the diagonal length of the head minus the opposite diagonal length. The CVAI is utilized instead of, or in addition to, the CVA because it accounts for variation in infant head size. Only one of the studies evaluated the effect of cranial orthotic therapy on neurodevelopmental outcomes (Shamji et al., 2012) and only one study provided long-term follow-up to evaluate the impact of plagiocephaly on function or psychosocial development (Shamji et al., 2012). None of the studies directly compared the different types of orthotic devices. Several of the studies reported on outcomes in patients treated with orthotic devices compared with those treated with repositioning therapy.

Correction of Cranial Asymmetry

A total of 10 uncontrolled studies evaluated the effect of cranial orthotic devices on cranial asymmetry in infants with positional plagiocephaly, including 9 prospective studies (Littlefield et al., 1998; Kelly et al., 1999; St. John et al., 2002; Bruner et al., 2004; Thompson et al., 2009; Schaaf et al., 2010; Kluba et al., 2011; Kluba et al., 2012;

Seruya et al., 2013) and 1 retrospective study (Yoo et al., 2012). Overall, the study results suggest that cranial orthotic devices significantly improve cranial asymmetry in infants. The duration and success of treatment were dependent on the age at initiation of treatment. Younger patients achieved more rapid success and more pronounced improvement than older patients. In a prospective study of 175 infants, age > 8.2 months at treatment initiation was a significant predictor of a poorer outcome (Thompson et al., 2009). In another study, initiation of treatment at age > 6 months was associated with significantly less improvement in CVA and CVAI than treatment at a younger age (Kluba et al., 2011). To achieve treatment success in older infants, cranial orthotics were worn for a significantly longer duration compared with younger infants (Kluba et al., 2011; Seruya et al., 2013).

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

Correction of Adverse Effects of Cranial Asymmetry

To ascertain the effect of plagiocephaly treatment on subsequent cognitive development, school performance, language skills, and societal function as well as cosmetic outcome, a retrospective survey of parents was performed (Shamji et al., 2012). The children had a median age of 9 years when the survey was conducted, and 18% of children whose parents responded were felt to have developmental delay before treatment of plagiocephaly. Plagiocephaly treatment methods had achieved good cosmetic outcome; according to parent respondents, fewer than 10% of children were aware of a head deformity or had been teased about head shape.

Nevertheless, at the time of the survey, the children had a higher prevalence of developmental delays than seen in the average population. The survey response rate was poor (23%); therefore, study bias cannot be excluded.

Data for children who had and had not been treated with cranial orthotics were not presented separately, and the study did not measure increases or declines in developmental delay.

Efficacy of Cranial Orthotics Compared with Alternative Treatments

A total of five studies, including four prospective studies (Argenta et al., 1996; Mulliken et al., 1999; Plank et al., 2006; Lipira et al., 2010) and one retrospective study (Loveday and deChapain, 2001), compared the efficacy of cranial orthotics with that of active repositioning treatment. While both repositioning methods and cranial orthotic therapy were able to reduce cranial asymmetry, orthotic devices achieved success more rapidly and to a greater extent than repositioning methods in some studies. The results of one study (Argenta et al., 1996) suggested that younger patients achieved more rapid success than older patients, and that patients treated with helmets improved more quickly than patients treated with repositioning. In a prospective study of 114 infants, although 98% of the infants had satisfactory improvement in plagiocephaly, those treated with cranial orthotics had significantly better posterior cranial asymmetry than those treated with repositioning methods (Mulliken et al., 1999). Lipira et al. (2010) observed similar results in a study of 70 infants. Infants in both treatment groups had equivalent reductions in CVA; however, those in the cranial orthotic group had significantly greater improvements in mean asymmetry, maximum asymmetry, and region of greatest asymmetry than those treated by repositioning methods (Lipira et al., 2010). In one retrospective study, there were no significant differences in treatment outcomes between the two groups, but the time taken to treat positional plagiocephaly was one third shorter in the helmet group than in the repositioning group (Loveday and deChapain, 2001). In contrast, in one prospective study, > 30% of patients who were treated by repositioning methods showed worsening asymmetry at the follow-up assessment (Plank et al., 2006). Follow-up did not appear to extend beyond the treatment period.

Safety of Cranial Orthotics

In general, cranial orthotics are safe devices and most complications do not require permanent discontinuation of treatment. Of the 16 reviewed studies, complications of cranial orthotic treatment were reported only by a group in the Loveday and deChapain (2001) study. These complications consisted of skin injury from malfitting helmets, skin reactions to helmet materials, and occasional heat rash. A retrospective institutional database analysis was identified that reported complications of cranial orthotic treatment for 410 patients who had moderate or severe nonsynostotic plagiocephaly (Wilbrand et al., 2012). Complications occurred in 22% of patients and were classified into 5 different categories: pressure sores, ethanol erythema, skin infection, bacterial abscess, and malfitting helmet. There was no relationship between severity of deformity and the incidence of complications. Pressure

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures sores, which form in areas of tight contact between the helmet and the skin, occurred in 10.5% of patients.

Pressure sores were more frequent in brachycephalic children than children with plagiocephaly or those with a combination of plagiocephaly and brachycephaly. Approximately 6% of patients had malfitting orthotics.

Accumulation of cleaning fluids in the foam layer of the orthotic caused ethanol erythema in 6.3% of patients. Skin infection developed in 1.2% of patients and is typically caused by poor hygiene. One patient developed a bacterial abscess, which resulted from an untreated pressure sore. Most complications of cranial orthotic device treatment can be avoided by thorough instruction of the parents.

Summary and Quality of the Evidence:

The results of the reviewed studies indicated that repositioning can be an effective treatment for positional plagiocephaly, and if therapy is initiated before 6 to 8 months of age. None of the studies explicitly evaluated the impact of cranial orthotic therapy on the incidence of surgical correction. Surgery is generally not indicated for positional plagiocephaly, although one older study reported surgical correction of some severely affected infants.

Only one of the available studies evaluated the impact of cranial orthotic devices on neurodevelopmental outcomes and psychosocial development, and this study was of very poor quality; therefore, the results are questionable. Although ocular and dental problems have been reported in older individuals who have uncorrected plagiocephaly, the magnitude and significance of these long-term sequelae have not been addressed, and none of the selected studies assessed the effectiveness of orthotic devices in preventing such problems.

If fitted properly and enlarged with an infant’s growth, cranial orthotic devices are considered safe. The complications reported in the literature were generally minor and involved pressure sores, skin rashes, and skin infection. Most complications are considered to be preventable if the helmet is properly fitted and the parents are thoroughly educated on the use and care of orthotic helmets.

The quality of individual studies ranged from very poor to fair. There were no randomized controlled trials. The noncomparative studies consisted of before-and-after assessments, which are of limited validity for a condition that has the potential to spontaneously resolve. In the comparison studies, treatment method was often dictated by the parent and in some studies the two treatment groups were not equal in size. Furthermore, comparative studies did not follow patients long enough to determine whether differences in outcome might diminish as children grew. In the medical community, there is a lack of consensus about what constitutes severity of plagiocephaly and also what constitutes treatment success. This led to variability in outcome measures in the reviewed studies. Thus, the evidence pertaining to correction of deformity is of low quality. There was no direct evidence that cranial helmets reduce the need for corrective surgery, although this may be inferred from the positive effects on asymmetry. Evidence regarding reduction in any adverse effects of plagiocephaly on physiological function, overall function, or quality of life was insufficient (one very poor quality survey).

There is low-quality evidence that cranial orthotic devices can reduce or eliminate the cranial asymmetry associated with positional plagiocephaly when therapy is initiated before 12 to 18 months of age, and that there are few adverse effects. There is also some evidence that older age at initiation of treatment is associated with poorer outcome. Comparative studies indicated that repositioning can also reduce cranial deformity, although repositioning may take longer than cranial orthotic therapy and may not provide the same degree of correction over the short term in some infants. None of the reviewed studies addressed the impact of cranial orthotic

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures therapy on need for surgical correction of severe deformities and only one, poor-quality study evaluated the effect of orthotic use on health outcomes.

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

GUIDELINES/REGULATORY STATUS/COVERAGE POLICIES

Websites for the following sources were searched: Centre for Reviews and Dissemination, National Guidelines Clearinghouse, Veterans Administration Department of Defense Clinical Practice Guidelines United States, National Institute for Health and Clinical Excellence (NICE), American Academy of Pediatrics (AAP), American Society of Craniofacial Surgery, American Association of Neurological Surgeons, American Cleft Palate-Craniofacial Society, American Society of Maxillofacial Surgeons, American Association of Pediatric Plastic Surgeons, American Association of Pediatric Neurosurgeons, Food and Drug Administration (FDA), the Centers for Medicare & Medicaid Services (CMS), and selected private payers.

Published Technology Assessments and Systematic Reviews

There were no technology assessments or systematic reviews of cranial orthotic devices identified in the available literature.

Practice Guidelines

American Academy of Pediatrics (AAP): The AAP issued a clinical report in 2011 regarding the prevention and management of positional skull deformities in infants. This report recommended preventive counseling to parents during the newborn period, including instruction on alternating the newborn’s laying position while sleeping and having the infant spend at least 30 to 60 minutes daily in the prone position while awake. If deformational plagiocephaly is diagnosed, the AAP recommends mechanical adjustments, including positioning, so that the rounded side of the head is placed against the mattress and a change in the layout of the room to cause a child to look away from the flattened side of the head to see parents or others in their room. For torticollis, neck motion exercises are recommended. If these therapies prove unsuccessful, the AAP recommends visiting a neurosurgeon to ensure a proper diagnosis and direct subsequent management. According to the AAP, there is currently no evidence that cranial orthotics work better than repositioning measures for infants with mild or moderate cranial deformity. For patients with severe plagiocephaly, the best response for helmet treatment occurs in children age 4 to 12 months due to the malleability of the infant skull. Surgery is only indicated when the deformities are severe and resistant to nonsurgical interventions (Laughlin et al., 2011).

Canadian Paediatric Society (CPS): The CPS issued a practice point in 2011 that included recommendations for the prevention, diagnosis, and treatment of positional plagiocephaly (Cummings, 2011). Based on a review of the published studies, CPS recommends repositioning therapy for patients who are < 4 months of age and have mild-to-moderate asymmetry. CPS recommends cranial molding therapy for patients with severe asymmetry who are < 8 months of age. However, this recommendation was not well supported by the available evidence at the time of publication. CPS made the following recommendations:

• Plagiocephaly may be prevented by encouraging the infant to lie on each side in the supine position.

Children with strong positional preference may require more effort to lie on their other side.

• During awake time, infants should be placed in a prone position for 10 to 15 minutes at least 3 times daily.

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

• Evaluation of a child with plagiocephaly should include assessments for craniosynostosis, congenital torticollis, and cervical spine abnormalities.

• Repositioning therapy, plus physiotherapy as needed, is the treatment of choice for most children with mild-to-moderate positional plagiocephaly.

• Cranial molding therapy may be considered for children with severe asymmetry. There is insufficient evidence to support helmet therapy for infants with mild or moderate asymmetry.

Cincinnati Children's Hospital Medical Center (CCHMC): CCHMC prepares Best Evidence Statements that address key points of care for a target population. These statements are derived from a review of the literature and evaluation of the current practices at the time of their development. CCHMC recommends that care givers of infants receive education regarding methods to reduce the amount of time that infants spend in a supine position to decrease the incidence of plagiocephaly (CCHMC, 2012). In 2011, CCHMC prepared a Best Evidence Statement about the prognosis of development of infants with plagiocephaly and torticollis (CCHMC, 2011). CCHMC concluded that:

• Plagiocephaly alone is not a predictor of developmental delay.

• In very young infants (average age 22 weeks), developmental delay appears related to sleep position, muscle tone, activity level, male gender, and neck dysfunction.

Regulatory Status

Food and Drug Administration (FDA): There are dozens of manufacturers of FDA-approved cranial orthoses for the treatment of severe nonsynostotic positional plagiocephaly in infants 3 to 18 months of age. They are classified as Class II devices. The FDA requires special controls for these orthoses, including: prescription use; biocompatibility testing; and labeling (contraindications, warnings, precautions, adverse events, instructions for physicians and parents) (CDRH, 2013).

Coverage Policies

Centers for Medicare and Medicaid Services (CMS): No National Coverage Determination (NCD) for cranial orthotic devices for the treatment of positional plagiocephaly was identified on the CMS website (CMS, 2013). However, infants with positional plagiocephaly would not fall into the Medicare beneficiary population.

Selected Commercial Payer Coverage Policies:

Aetna: Aetna considers cranial remodeling bands (or helmets) as medically necessary orthoses for treatment of moderate to severe positional head deformities that result in documented asymmetry when banding is initiated at 4 to 12 months of age. The patients must have failed a 2-month trial of conservative therapy (Aetna, 2012).

CIGNA: CIGNA covers a custom-molded/fitted cranial orthotic device as medically necessary for the treatment of moderate-to-severe nonsynostotic positional plagiocephaly when all of the following criteria are met (CIGNA, 2012):

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures

• There is photographic evidence supporting moderate-to-severe nonsynostotic positional plagiocephaly.

• The child is one of the following:

– Age 3 to 5 months and has failed to respond to a 2-month trial of repositioning therapy.

– Age 6 to 18 months.

• There is documentation of either of the following criteria:

– Cephalic index ± at least two standard deviations from the mean for the appropriate sex/age.

– Asymmetry of ≥ 12 millimeters (mm) in one of the following measures: cranial vault, skull base, orbitotragial depth.

Humana: Humana covers cranial orthotic treatment for patients < 18 months of age with positional plagiocephaly that has not adequately responded to a 2-month trial of repositioning and/or physical therapy (Humana, 2012).

Regence Group: No coverage policy for cranial orthotics was identified on the Regence Group website (Regence Group, 2013).

UnitedHealthcare (UHC): UHC considers cranial orthotic devices to be reconstructive for the treatment of craniofacial asymmetry in infants 3 to 18 months of age with severe nonsynostotic positional plagiocephaly (UHC, 2012).

RELATED ISSUES

Health Implications of Positional Plagiocephaly

The issue of whether nonsynostotic skull deformities cause any physiological or functional problems, or merely present a cosmetic problem, is a matter of debate and active investigation.

Neurodevelopmental Delay

There is no definitive evidence that infants with positional plagiocephaly will suffer any neurological damage or that there are long-term effects on brain development, although some auditory and visual defects may be associated with severe cranial deformation (Balan et al., 2002; Siatkowski et al., 2005; Kennedy et al., 2009). One study showed that children with plagiocephaly had a mental developmental index that was significantly different from the standardized distribution, and, in the same study, more children were identified as being severely delayed than was expected (Panchal et al., 2001). Additionally, Panchal et al. (2001) reported significant differences in the psychomotor developmental index between infants with plagiocephaly and a standardized distribution. Infants with plagiocephaly were significantly more likely to fall into the mildly delayed or severely delayed range of functioning compared with the standardized distribution (Panchal et al., 2001). In a recent study, the Bayley Scales of Infant and Toddler Development (BSID) results of 225 toddlers with a history of plagiocephaly were compared with the results of 230 toddlers without plagiocephaly (Collett et al., 2011). Toddlers with a history of plagiocephaly scored clinically significantly lower than toddlers without plagiocephaly for all scales of the test, but the scores were still within the average range for the BSID. In addition, toddlers with a history of plagiocephaly were more likely than their peers to receive scores in the delayed range, according to the test norms. To study the effect of plagiocephaly on development, 126 children who received repositioning treatment

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures were assessed using the Ages and Stages Questionnaire at 3, 6, and 12 months after initiation of treatment (Hutchison et al., 2012). The children had an above average incidence of delays at each measurement, particularly in the gross motor domain. However, the incidence of delays declined and approached average values by the 12-month follow-up assessment, at a mean age of 17 months. In another study, the BSID was administered to 110 untreated plagiocephalic infants (Kordestani et al., 2006). The plagiocephalic infants had significantly different psychomotor and mental development indices than the standardized population and showed delays in both domains. Most notably was the absence of accelerated development. However, studies that show a link between positional plagiocephaly and developmental delay should be tempered by the knowledge that children born with congenital anomalies that cause developmental delay, as well as babies born prematurely, tend to be weaker and therefore may rest in one position for long periods of time unless someone moves their head to another position.

Thus, children who exhibit neurodevelopmental delay would be more likely to develop positional plagiocephaly (Panchal et al., 2001; Hutchinson et al., 2009; Collett et al., 2011). Although none of the clinical trials specifically addressed the effect of therapy with cranial orthotics on prevention of subsequent neurodevelopmental delay or on reducing neurodevelopmental delay, there is no evidence that the cranial deformity associated with positional plagiocephaly causes neurological or neurodevelopmental problems, although infants with neurodevelopmental delay may be more likely to exhibit positional plagiocephaly than normal infants.

Dental and Ocular Problems

In a review of the subject, Rekate (1998) cautions that many dental and ocular problems resulting from positional plagiocephaly may exist but may not have been documented due to failure to recognize an association with the primary asymmetry. The author also noted that, since the incidence of positional plagiocephaly has only increased over the past several years, many affected individuals have not yet reached adulthood. The results from some recent studies suggested that the skull asymmetry may cause growth deformities in other bones, such as the mandible, which may then lead to significant dental abnormalities and create difficulties with the temporomandibular joint (Kane et al., 1996a). Severe skull asymmetry can also cause deformity of the orbit, with the potential for ocular disturbances such as strabismus and abnormalities in ocular motility (Fredrick et al., 1993).

In addition to the effect on oral and ocular function, cranial asymmetry may have adverse psychosocial effects later in life, although this aspect of positional plagiocephaly has not been well examined. In a survey of 9-year-old children who had been treated for plagiocephaly as infants, 10% were aware of a head deformity and had been teased about their head shape (Shamji et al., 2012). This study was of poor quality, primarily because the survey had a 23% response rate; therefore, the results are questionable. It remains to be determined whether an abnormal head shape results in difficult peer relations or disturbances in self-image (Rekate, 1998).

Incidence of Positional Plagiocephaly

Numerous recent reports have documented a large increase in the numbers of infants diagnosed with nonsynostotic plagiocephaly over the past several years, with estimates of incidence ranging from 1 in 300 live births to 48% of live births. More accurate diagnostic techniques and increased recognition of characteristic clinical features of synostotic versus nonsynostotic deformities have led to better differentiation of these two conditions, and many cranial deformities once thought to be synostotic and to require surgical correction are now

Section L, Exhibit L-8 Sample Short Assessment, Evolving Practices, Devices, Medicines, Treatments and Procedures known to be positional plagiocephaly and are treated with more conservative measures (Huang et al., 1996;

Huang et al., 1998; Persing et al., 2003; Bialocerkowski et al., 2008; Xia et al., 2008; Robinson et al., 2009). In addition, a number of experts suggest that the increased incidence of positional plagiocephaly is directly related to the increased number of infants who are placed in the supine position for sleeping, following the recommendation of the AAP that infants should sleep on their backs in order to reduce the risk of sudden infant death syndrome (SIDS) (Kane et al., 1996b; Turk et al., 1996; Persing et al., 2003; Laughlin et al., 2011).

Several authors have documented that posterior plagiocephaly occurs more commonly in boys. Mulliken et al.

(1999) state that there is a 3:1 ratio of boys to girls in their population of infants referred for treatment, and Miller and Clarren (2000) also reported that more boys than girls had been diagnosed with plagiocephaly. Possible explanations include the larger size and faster growth of the head of the male, or a relative lack of flexibility in the male fetus that might lead to more deformational anomalies. The molded baby syndrome includes these abnormalities along with other distortions of the bony skeleton caused by intrauterine crowding and pressure (Kane et al., 1996b; Mulliken et al., 1999; Miller and Clarren, 2000; Boere-Boonekamp et al., 2001; Rubio et al., 2009).

The prevalence of positional plagiocephaly was determined in teens who were born between 1992 and 1998, which corresponds to the time when parents were encouraged to place their infants on their backs to sleep but prior to the Food and Drug Administration (FDA) approval of cranial orthotic devices (Roby et al., 2012).