The cranial orthosis, either a helmet or a band, can progressively mold the shape of the cranium.  This document addresses the use of the adjustable band or helmet as a post-operative treatment of craniosynostosis or as non-operative treatment for non-synostotic plagiocephaly (asymmetrically shaped head) and brachycephaly (abnormally shaped head; shortened in antero-posterior dimension without asymmetry) in infants.

Medically Necessary:  In this document, 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. 

Reconstructive: In this document, 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.  NOTE:  Not all benefit contracts include benefits for reconstructive services as defined by this document.  Benefit language supersedes this document.

Medically Necessary:

The use of an adjustable cranial orthosis is considered medically necessary in the post-operative management of infants following endoscopic repair of craniosynostosis.

Not Medically Necessary:

The use of an adjustable cranial orthosis is considered not medically necessary for all non-synostotic plagiocephaly related indications.

Reconstructive:

The use of an adjustable cranial orthosis as a treatment for moderate to severe non-synostotic plagiocephaly may be considered reconstructive when ALL of the following criteria are met:

1. The infant is at least 3 months of age but not greater than 18 months of age; AND
2. Marked asymmetry has not been substantially improved following conservative therapy of at least 2 months duration with cranial repositioning therapy with or without physical therapy. Note: Due to the mobility of infants greater than 4 months of age, repositioning therapy is not effective and thus, a trial of repositioning is not indicated; AND
3. Asymmetry of the cranial base as documented by any of the following:
   • Skull Base Asymmetry: At least 6 mm right/left discrepancy measured subnasally to the tip of the tragus (cartilaginous projection of the auricle at the front of the ear); or
   • Cranial Vault Asymmetry: At least a 8 mm right/left discrepancy, measured from the frontozygomaticus point (identified by palpation of the suture line above the upper outer corner of the orbit) to the contralateral euryon, defined as the most lateral point on the head located in the parietal region; or
   • Asymmetry of the orbitotragial distances, as documented by at least a 4 mm right/left asymmetry measured from the lateral aspect of orbit to tip of ipsilateral tragus.
4. The custom molded orthotic is designed to fit a child's head from 2-4 months. A second helmet or band may be required if the asymmetry has not resolved or significantly improved after 2-4 months.

Investigational and Not Medically Necessary:

The use of an adjustable cranial orthosis is considered investigational and not medically necessary as a treatment of brachycephaly.

The use of an adjustable cranial orthosis is considered investigational and not medically necessary as part of the post-operative management of craniosynostosis when an open surgical procedure is performed.

Plagiocephalic Cranial Asymmetry
Multiple case series have demonstrated that the adjustable cranial orthosis can reshape non-synostotic plagiocephalic cranial asymmetries. These asymmetries may involve the cranial base, the cranial vault and the orbitotragial distance. The evaluation of the medical necessity of the adjustable cranial orthosis for cranial asymmetries requires data that support 1) that non-synostotic plagiocephaly is associated with a physical functional impairment and 2) that dynamic orthotic plagiocephaly results in an improvement in the physical functional impairment. Examples of physical functional impairments include learning disabilities, ocular dysfunction and jaw malocclusion. To date, there is inadequate data to support that plagiocephaly causes or contributes to any type of physical functional impairment. For example, Miller and colleagues (2000) examined the long-term development outcomes in patients with deformational plagiocephaly in a case series of 181 children with positional plagiocephaly; families were invited to participate in a telephone interview regarding developmental outcomes. A total of 63 of the 181 contacted families agreed to participate in the interview; 39.7% of the children had received special help in primary school. Given the approximate 33% participation rate in this survey, no conclusions can be drawn. In addition, 27 of the 63 respondents had used helmet therapy as part of the treatment. Gupta and colleagues (2003) reported on the ophthalmologic findings in 93 patients with deformational plagiocephaly; 24% had unilateral or bilateral astigmatism compared with 19% prevalence in the normal population. This study did not indicate whether the participants did or did not undergo helmet therapy. Balan and colleagues (2002) examined auditory event-related potentials in 10 infants with deformational plagiocephaly compared to 15 sex- and age-matched controls. The infants with plagiocephaly exhibited smaller amplitudes in response. Again, this study did not indicate whether the participants had used helmet therapy. In summary, no controlled trials have documented functional impairments in infants with plagiocephaly and whether or not these impairments are improved with helmet therapy. Therefore, the treatment of non-synostotic plagiocephaly using the adjustable band is considered not medically necessary.

Nevertheless, the adjustable cranial orthosis may be considered reconstructive for plagiocephalic cranial asymmetries, based on available data that the therapy can indeed reshape the cranium to a more normal contour. To determine if the service is reconstructive, the following issues are relevant.

1.  What degree of asymmetry would warrant correction?

Studies of helmet therapy typically describe 3 different types of asymmetry; asymmetry of the cranial base, cranial vault, and orbitotragial distance. All 3 result in visible facial asymmetry. The degree of asymmetry that would warrant correction is not well addressed in the published medical literature. However, one can examine the degree of asymmetry reported in case series of infants treated with an adjustable cranial orthosis. The following table presents the mean pretreatment asymmetries reported in large case series.

* In this report, the asymmetry was measured from the tragus to the frontozygomatic point instead of the excanthion

The study by Moss and colleagues (1997) was the only article that attempted to distinguish degrees of asymmetry. Mild to moderate asymmetry was defined as 12 mm or less, while moderate to severe asymmetry was defined as greater than 12 mm. Treatment with helmet therapy was offered to those with moderate to severe asymmetry, while the rest were treated with repositioning therapy.

2.     What is the optimal timing of helmet therapy? 

Again, this issue is not specifically addressed in the literature, but some providers would consider helmet therapy only after a failure of an initial trial of repositioning. For example, Pollack et al. (1997) recommended a 2- to 3-month trial of repositioning therapy. In their series of 69 children, 39 failed repositioning therapy and thus were subsequently treated with helmet therapy. However, some providers may suggest an earlier intervention of helmet therapy may be warranted in older children. For example, helmet therapy may be increasingly less effective in older children as the synostoses begin to close. Therefore, requiring a 2 to 3 month trial of repositioning therapy in children 6 to 9 months old may limit the effectiveness of a subsequent trial of helmet therapy. Furthermore, repositioning therapy may be less effective in older children who are increasingly mobile and do not maintain a single sleeping position. The American Academy of Pediatrics (AAP), in their 2003 clinical report: Prevention and Management of Positional Skull Deformities in Infants, stated that:

The best response for helmets occurs in the age range of 4 to 12 months because of the greater malleability of the young infant skull bone and the normalizing effect of the rapid growth of the brain. There is less modification of the cranial configuration when used after 12 months of age. The use of helmets and other related devices seems to be beneficial primarily when there has been a lack of response to mechanical adjustments and exercises. In most situations, an improvement to repositioning and neck exercise is seen over a 2- to 3-month period if these measures are instituted as soon as the condition is recognized.

3.  What is the stopping point of therapy?

This issue is not well addressed in the published literature. Presumably the stopping point is when symmetry is achieved, however even the non-synostotic skull will have some degree of minor asymmetry, which would be considered within normal limits. The issue may arise when providers request a second device for ongoing correction of asymmetry. The manufacturer suggests that helmets are designed for approximately 2-4 months of use, after which point a child might "outgrow" the device. If symmetry has not been achieved within this time frame, a provider may request a second device. The manufacturer of the DOC Band^® (Cranial Technologies, Inc., Tempe, AZ) estimates that this occurs in about 20% of cases. 

Brachycephaly
Brachycephaly describes a head that although symmetric, is abnormally shaped (flattened), and is assessed using the cranial index (CI). Medical dictionaries and anthropologic sources define brachycephaly as a cranial index (width divided by length x 100%) greater than 81%. A literature search did not identify any randomized prospective comparative clinical trials which establish the efficacy of an adjustable cranial orthosis as a treatment of brachycephaly. 

In a retrospective chart-review study, Teichgraeber et al (2004) evaluated treatment outcomes in groups of children with positional brachycephaly and plagiocephaly and concluded that the use of a cranial orthotic device was effective for both groups, but that more children in the plagiocephaly group were normalized after treatment. In this study infants were treated with either repositioning (n=132) or with the DOC Band (n=292). Of the 292 treated with a molding orthotic, 64 were brachycephalic and 228 had plagiocephaly. Of 64 patients with brachycephaly treated with banding, 33 met specific inclusion criteria:  charted diagnosis of brachycephaly, age 12 months or less, and complete anthropomorphic measurements recorded in the record. In the brachycephalic group, significant improvement occurred in the CI (P<0.01) after treatment with the DOC Band but infants were described as still significantly different from age and sex adjusted norms. Only one child in this group normalized to within 1 standard deviation (SD) of the norm by the end of treatment. In this study, cranial orthotic treatment was reported as more effective in treating posterior plagiocephaly than brachycephaly, but specific data with benchmark norms were not provided. The limitations of this study included its retrospective design and the lack of reporting of comparative data from the group treated with positioning alone.

A study by Graham et al (2005) compared the effect of repositioning versus helmet therapy on CI in infants referred for brachycephaly. This study collected longitudinal data on 193 infants referred and treated for abnormal head shapes at a single institution between 1997 and 2001. The CI was compared before and after treatment with either repositioning or helmet therapy. In a subgroup of infants (n=92) with severe brachycephaly (CI ≥ 90%), the authors concluded that although both groups (repositioning and orthotic) improved, repositioning was less effective than cranial orthotic therapy based on reduction in CI (2.5% vs 5.3%). The limitations of this study include a lack of randomized design, baseline differences in initial mean age and cephalic index and differences in mean duration of therapy between the two treatment groups.

Craniosynostosis
Craniosynostosis is a cranial deformity resulting from the premature closure of cranial sutures. It affects 1 in 2100 children, and of those, 40%-60% involve the sagittal suture, 20%-30% involve the coronal suture, and 10% the metopic suture. Lambdoid synostosis is the rarest. Researchers do not have a clear understanding of the genetic influences or developmental pathology that predisposes cranial sutures to premature closure (Barringer, 2004). Surgical techniques for correction of craniosynostosis involve an open or an endoscopic approach.

In the open procedure the deformed bone is removed through an ear to ear incision over the top of the scalp. Reshaping the head is accomplished by bone replacement secured with dissolving plates and screws. A small study by Seymour-Dempsey et al (2002) compared the operative outcomes of patients treated with and without cranial banding following surgery for craniosynostosis. This small, non-randomized, retrospective study included 21 children with sagittal craniosynostosis treated surgically between 1994 and 2001. Six children were treated with surgery alone and 15 were treated with surgery and postoperative cranial banding with the dynamic orthotic cranioplasty (DOC Band). The investigators recorded anthropomorphic measurements pre-operatively, post-surgery, and post-orthotic treatment. They found that the postoperative cephalic indices (CI), when compared with preoperative CI, improved in both groups. While surgical improvement was seen in both groups, the orthotic group demonstrated a continued correction toward a more normal CI not seen in the non-orthotic group. The authors concluded that the use of an orthosis maintains the initial surgical correction and promotes more normal cranial growth patterns. Based on this small retrospective analysis, the authors recommend the use of cranial orthoses as an adjunct to surgery for sagittal synostosis. Kaufman et al (2004) reported a small (n=12) case-series comparing outcomes of an open craniectomy for sagittal synostosis utilizing a postoperative cranial orthotic. In this group, immediate and 1-year postoperative CTs did not reveal a statistically significant improvement in CI, (preoperative CI, 65 ± 3.4; range, 58 to 70; post treatment CI, 74 ± 4.3; range, 68 to 80) however, visually, the head shape was improved. The results of this study yielded similar results when compared to historic outcomes without the use of cranial orthotics postoperatively. 

The endoscopic approach is a minimally invasive procedure removing deformed bone through an endoscope via 2 small incisions. However the endoscopic approach cannot accommodate application of fixation devices to maintain reshaping postoperatively. In this case, postoperative cranial banding is frequently used to maintain reshaping. While only a few published, uncontrolled case series document the use of postoperative cranial orthoses as an adjunct to surgery for craniosynostosis, these investigators propose that postoperative cranial orthoses are a valuable tool in enhancing the surgical outcome (Jimenez, 2002; Jimenez, 2007; Jimenez, 2010; Murad, 2005; Cohen, 2004).

Plagiocephaly, which refers to an asymmetrically shaped head, can be subdivided into synostotic and non-synostotic types.

Synostotic plagiocephaly or craniosynostosis describes an asymmetrically shaped head due to premature closure of the sutures of the cranium. Craniosynostosis may require surgery to reopen the closed sutures. Surgery can be performed by an open or endoscopic technique, depending upon the type and extent of the synostosis. The open approach requires an incision and may involve removal, reshaping or replacing the deformed cranial bone. For this extensive surgery, dissolving plates and screws are used to maintain the reshaped cranium post operatively. The endoscopic procedure is a minimally invasive technique where bone segments are removed, releasing the fusion. Since no plates or screws are inserted, cranial orthotics have been proposed to maintain the surgical correction postoperatively.

In plagiocephaly without synostosis, the sutures remain open. 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. Brachycephaly refers to a head shape that is not asymmetric but is disproportionately short. The incidence of plagiocephaly and brachycephaly has increased rapidly in recent years as a result of the "Back to Sleep" campaign initiated in 1992 by the American Academy of Pediatrics (AAP), in which a supine sleeping position is recommended to reduce the risk of sudden infant death syndrome (SIDS). It is estimated that one of every 60 neonates may have some degree of plagiocephaly or brachycephaly. Positional plagiocephaly typically consists of right or left occipital flattening with advancement of the ipsilateral ear and prominence of the ipsilateral frontal region, 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. Assessment of plagiocephaly and brachycephaly are based on anthropomorphic measures of the head, using anatomical and bony landmarks.

There are 3 basic options for treating non-synostotic plagiocephaly and brachycephaly; no therapy, repositioning therapy, and adjustable band. Repositioning therapy includes supervised "tummy time," or placement of the child in a half supine position with a towel or blanket roll behind the shoulder to position the occiput away from the flat side. Physical therapy may also be recommended, particularly if there is shortening or tightening of the sternocleidomastoid muscle. The adjustable band involves use of a custom-molded orthotic, either a helmet or band that can progressively mold the shape of the cranium by applying corrective forces to the frontal and occipital prominences while leaving room for growth in the adjacent flattened areas. Treatment is typically initiated around 4 to 6 months of age, frequently after a prior trial of repositioning therapy, and continues for an average of 4 to 5 months. Both helmets and cranial bands are recommended for wear 23 hours per day, with 1 hour off for skin care and hygiene.

Asymmetry of 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)

Asymmetry of 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

Asymmetry of Orbitotragial depth: asymmetry of the orbitotragial depth is measured from the exocanthion (outer corner of the eye fissure where the eyelids meet) to the tragus (the cartilaginous projection in front of the external auditory canal)

Brachycephaly: describes a head shape that is symmetric and disproportionately wide, (width ÷ length x 100%) ≥ 81%

Cranial Index: the cranial 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 ophisthocranion), expressed as a percentage. The cranial index can then be compared to normative measures

Craniosynostosis: a congenital deformity of the infant skull that occurs when the fibrous joints between the bones of the skull (called cranial sutures) close prematurely

Non-synostotic plagiocephaly: a condition where an infant's head becomes deformed due to external forces; in non-synostotic plagiocephaly the joints between the skull bone plates (sutures) remain open, allowing non-surgical correction; this condition is also known as positional plagiocephaly

Orthotic cranioplasty: a method to correct non-synostotic plagiocephaly through the wearing of a custom-fitted helmet or head band which places constant gentle pressure on the infant's head to assume a more natural skull shape

Plagiocephaly: a condition characterized by an abnormal head shape

The following codes for treatments and procedures applicable to this document are included below for informational purposes.  Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy.  Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

When Services may be Medically Necessary when criteria are met: 

When services are Not Medically Necessary:
For the procedure codes listed above, for the following diagnoses 

When Services may be Reconstructive, when criteria are met:
For the procedure codes listed above, services may be considered Reconstructive when criteria are met for non-synostotic plagiocephaly 

When Services are Investigational and Not Medically Necessary:
For the procedure codes listed above, for the following diagnosis code when specified as brachycephaly, or when criteria are not met for craniosynostosis.

Peer Reviewed Publications:

1. Balan P, Kushnerenko E, Sahlin P, et al. Auditory ERPs reveal brain dysfunction in infants with plagiocephaly. J Craniofac Surg. 2002; 13(4):520-525.
2. Barringer W. The use of postoperative cranial orthoses in the management of craniosynostosis. J Prosth and Orthotics. 2004; 16(45):56-58.
3. Cohen SR, Holmes RE, Ozgur BM, et al.  Fronto-orbital and cranial osteotomies with resorbable fixation using an endoscopic approach. Clin Plast Surg. 2004; 31(3):429-442, vi.
4. Graham J, Kreutzman J. Deformational Brachycephaly in Supine-Sleeping Infants. J Pediatr. 2005; 146(2):253-257.
5. Gupta PC, Foster J, Crowe S, et al. Ophthalmologic findings in patients with nonsyndromic plagiocephaly. J Craniofac Surg. 2003; 14(4):529-532.
6. Hutchison BL, Hutchison LA, Thompson JM, Mitchell EA. Plagiocephaly and brachycephaly in the first two years of life: a prospective cohort study. Pediatrics. 2004; 114(4):970-980.
7. Jimenez DF, Barone CM, Cartwright CC, Baker L. Early management of craniosynostosis using endoscopic-assisted strip craniectomies and cranial orthotic molding therapy. Pediatrics. 2002; 110(1):97-104.
8. Jimenez DF, Barone CM.  Early treatment of anterior calvarial craniosynostosis using endoscopic-assisted minimally invasive techniques. Childs Nerv Syst. 2007; 23(12):1411-1419. 
9. Jimenez DF, Barone CM. Multiple-suture nonsyndromic craniosynostosis: early and effective management using endoscopic techniques. J Neurosurg Pediatr. 2010; 5(3):223-231.
10. Kabbani H, Raghuveer TS. Craniosynostosis. Am Fam Physician. 2004; 69(12):2863-2870.
11. Kaufman BA, Muszynski CA, Matthews A, Etter N. The circle of sagittal synostosis surgery. Semin Pediatr Neurol. 2004; 11(4):243-248.
12. Kordestani RK, Patel S, Bard DE et al. Neurodevelopmental delays in children with deformational plagiocephaly. Plast Reconstr Surg 2006; 117(1):207-218
13. Miller RI, Clarren SK. Long-Term developmental outcomes in patients with deformational plagiocephaly. Pediatrics. 2000; 105(2):E26.
14. Moss, DS. Nonsurgical nonorthotic treatment of occipital plagiocephaly:  What is the natural history of the misshapen neonatal head? J Neurosurg. 1997; 87(5):667-670.
15. Murad GJ, Clayman M, Seagle MB, et al. Endoscopic-assisted repair of craniosynostosis. Neurosurg Focus. 2005; 19(6):E6.
16. Pollak IF, Losken W, Fasick P. Diagnosis and management of posterior plagiocephaly. Pediatrics. 1997; 99(2):180-185.
17. Pomatto J, Beals S, Joganic E. Preliminary results and new treatment protocol for cranial banding following endoscopic-assisted craniectomy for sagittal synostosis. J Craniofac Surg 2001;9:47–49.
18. Seymour-Dempsey K, Baumgartner JE, Teichgraeber JF, et al. Molding helmet therapy in the management of sagittal synostosis. J Craniofac Surg 2002;13(5):631-635.
19. Teichgraeber JF, Ault JK, et al. Deformational posterior plagiocephaly: diagnosis and treatment. Cleft Palate Craniofac J. 2002; 39(6):582-586.
20. Xia JJ, Kennedy KA, Teichgraeber JF, et al. Nonsurgical treatment of deformational plagiocephaly: a systematic review. Arch Pediatr Adolesc Med. 2008; 162(8):719-727.

Government Agency, Medical Society, and Other Authoritative Publications:

1. American Academy of Neurological Surgeons (AANS). Craniosynostosis and Craniofacial Disorders. Available at: http://www.neurosurgerytoday.org/what/patient_e/craniosynostosis.asp?ShowMenu=false&ShowPrint=false   Accessed on February 22, 2010.
2. American Academy of Neurological Surgeons (AANS). Positional Plagiocephaly. Available at: http://www.neurosurgerytoday.org/what/patient_e/positional_plagiocephaly.asp. Accessed on February 22, 2010.
3. National Institute of Neurological Disorders and Stroke (NINDS). Craniosynostosis Information Page. Available at:  http://www.ninds.nih.gov/disorders/craniosynostosis/craniosynostosis.htm. Accessed on February 22, 2010.
4. Institute for Clinical Systems Improvement (ICSI). Cranial orthoses for deformational plagiocephaly. ICSI Technology Assessment Reports. TA #082. Bloomington, MN: ICSI; March 2004. Available at: http://www.icsi.org/technology_assessment_reports_-_active/ta_cranial_orthoses_for_deformational_plagiocephaly.html. Accessed on February 22, 2010.
5. Persing J, James H, Swanson J, Kattwinkel J. American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery. Prevention and management of positional skull deformities in infants. Pediatrics. 2003; 112(1 Pt 1):199-202.

1. National Library of Medicine. Medical Encyclopedia. Cranial Sutures. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/002320.htm. Accessed on February 22, 2010.

Ballert Cranial Molding Helmet™
Clarren Helmet™
Cranial Shaping Helmet™
Cranial Solutions Orthosis CSO™
Cranial Symmetry System™
DOC Band^®
Hanger Cranial Band™
O & P Cranial Molding Helmet™
P.A.P. Orthosis™
Plagiocephalic Applied Pressure Orthosis ™
RHS Cranial helmet™
STARband™ Cranial Remolding Orthosis™
STARlight™ Cranial Remolding Orthosis™
Static Cranioplasty Orthosis™

The use of specific product names is illustrative only.  It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.