Prior Authorization Review Panel MCO Policy …...in this initial evaluation are skull base...

Prior Authorization Review Panel MCO Policy Submission

A separate copy of this form must accompany each policy submitted for review. Policies submitted without this form will not be considered for review.

Plan: Aetna Better Health Submission Date:09/01/2019

Policy Number: 0379 Effective Date: Revision Date: 06/13/2017

Policy Name: Cranial Remodeling

Type of Submission – Check all that apply:

New Policy Revised Policy* Annual Review – No Revisions Statewide PDL

*All revisions to the policy must be highlighted using track changes throughout the document.

Please provide any clarifying information for the policy below:

CPB 0379 Cranial Remodeling

Clinical content was last revised on 06/13/2017 . Additional non-clinical updates were made by Corporate since the last PARP submission, as documented below.

Update History since the last PARP Submission:

07/16/2019-This CPB has been updated with additional background information and references.

Name of Authorized Individual (Please type or print):

Dr. Bernard Lewin, M.D.

Signature of Authorized Individual:

Revised July 22, 2019

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(https://www.aetna.com/)

Cranial Remodeling

Clinical Policy Bulletins Medical Clinical Policy Bulletins

Policy History

Last Revi

ew

07/16/2019

Effective: 02/01/2000

Next Review:

04/10/2020

Review History

Definitions

Additional Information

Number: 0379

Policy *Please see amendment for Pennsylvania Medicaid at the end of this CPB.

I. Aetna considers cranial remodeling bands (or helmets) as medically

necessary orthoses for treatment of moderate-to-severe positional head

deformities associated with premature birth, restrictive intra-uterine

positioning, cervical abnormalities, birth trauma, torticollis (shortening of

the sternocleidomastoid muscle) and sleeping positions in children when

banding is initiated at 3 to 12 months of age and the following conditions

are met:

A. A 2-month trial of conservative therapy consisting of re-positioning the

child's head such that the child lies opposite to the preferred position,

has failed to improve the deformity and is judged to be unlikely to do so,

and

B. One of the following must be met:

1. Anthropometric data (measurements used to evaluate abnormal

head shape by measuring the distance in mm from one pre

designated point on the face or skull to another, comparing the right

and left sides) verifies that a moderate-to-severe plagiocephaly is

documented by a physician experienced in such measurement. Proprietary

http://www.aetna.com/cpb/medical/data/300_399/0379.html 08/28/2019

http://www.aetna.com/cpb/medical/data/300_399/0379.html

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(Note: These measurements are generally obtained by the orthotist

fitting the band or helmet). The most significant measurements used

in this initial evaluation are skull base asymmetry, cranial vault

asymmetry, orbitotragial depth, and cephalic index.

DIAGRAM:

A difference of asymmetry greater than 6 mm between anthropometric

measurements (see diagram above) in any of the anthropometric data in the first

column of the following table warrants coverage of a trial of orthotic banding to

correct the craniofacial deformity:

Anthropometric Measurement

Orbitotragial

2. For brachycephaly evaluation, a cephalic index of 2 standard

deviations (SDs) below mean (head narrow for its length) or 2 SDs

above mean (head wide for its length) warrants coverage of a trial of

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orthotic banding to correct the craniofacial deformity in a child after

4 months of age and before 12 months of age. (Note: These

measurements are generally obtained by the orthotist fitting the

band or helmet).

Head width

(eu - eu)

Cephalic index = Head width (eu - eu) x 100

Head length (g - op)

64.8 71.4 78.0 84.6 91.2

3. Infants who develop significant plagiocephaly secondary to a constant

head position required for long-term hyperalimentation who do not

respond to simple changing of the catheter location allowing the head to

be re-positioned.

4. Members with excess frontal bossing secondary to sagittal synostosis

5. Premature infants with dolichocephalic head shape who have developed

a mis-shapen head secondary to sustained head position.

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A second cranial remodeling band or helmet is considered medically necessary for

children who met the aforementioned criteria at the initiation of therapy if the

asymmetry has not resolved or significantly improved after 2 to 4 months such that

the severity of head deformity indicates another orthosis and the orthosis becomes

ill-fittin g a fte r a tte m p ts to a d ju st a n d le a ve s little o r n o ro o m fo r n e w

growth. A second orthosis may be medically necessary to prevent

regression of head shape in very young infants (8 months or younger)

who met the aforementioned criteria at the initiation of therapy, who

have outgrown the initial orthosis, and have not developed midline head

control, rolling, or sitting. Note: remodeling bands (or helmets) are

contraindicated and considered not medically necessary after 2 years of

age).

II. Aetna considers the use of a cranial remodeling band (or

helmet) cosmetic for persons not meeting the afore-mentioned criteria.

III. Aetna considers use of a cranial remodeling band (or helmet) medically

necessary for infants with synostotic plagiocephaly to correct continued

asymmetry following surgery (i.e., a trial of conservative therapy is not

needed when the cranial remodeling band is used following surgery for

synostotic plagiocephaly).

Aetna considers the use of a cranial remodeling band or helmet without

surgery to correct asymmetry in infants with synostotic plagiocephaly as

experimental and investigational; craniosynostosis that is not surgically

corrected is a contraindication to use of cranial remodeling bands or

helmets.

Aetna considers pre-operative molding helmet therapy for the treatment of

sagittal craniosynostosis experimental and investigational because the

effectiveness of this approach has not been established.

IV. Aetna considers the use of sleep positioning wrap for the treatment of

infants with positional head shape deformities experimental and

investigational because its effectiveness has not been established.

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V. Aetna considers intra-operative indocyanine green angiography to evaluate

scalp perfusion during cranial vault remodeling in infants experimental and

investigational because the effectiveness of this approach has not been

established.

VI. Aetna considers distraction osteogenesis medically necessary for syndromic

craniosynostosis -- Apert, Crouzon, and Pfeiffer syndromes (see

CPB 0549 - Distraction Osteogenesis for Craniofacial Defects

(../500_599/0549.html)

Note: Aetna considers cranial removeling helmets and bands contraindicated and

not medically necessary in unshunted or uncontrolled hydrocephalus..

Background

Plagiocephaly (an asymmetrical head shape) is most often the result of an infant

spending extended periods of time on their back, typically during sleep.

Plagiocephaly can also occur as a feature of other disorders (e.g., craniofacial

disorders, torticollis, cervical anomalies) and is categorized as either positional or

synostotic (premature union of cranial sutures). Although 1 in 300 infants exhibit

variable degrees of plagiocephaly, true sutural synostosis, which interferes with

cranium development and may cause increased intra-cranial pressure, occurs in

only 0.4 to 1 per 1,000 live births.

Positional plagiocephaly is treated conservatively and many cases do not require

any treatment as the condition may resolve spontaneously when the infant begins

to sit up. When the deformity is moderate or severe and a trial of re-positioning the

infant has failed, a pediatric neurologist, neurosurgeon or other appropriate

specialist in craniofacial deformities may prescribe a cranial remodeling band to

remodel the misshapen head.

Cranial orthotics are designed to improve plagiocephaly without synostosis or

deformational plagiocephaly, which is a condition found in infants whose heads

show an asymmetrical flattening caused by uneven external pressures on the skull.

There are two types of cranial orthotics: cranial bands and soft-shell helmets.

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The headband or helmet is custom made and custom fitted to the infant’s head and

is designed to actively guide the growth of the skull to a more normal shape.

Orthotic management of plagiocephaly without synostosis is usually initiated

between three and 18 months of age and continues for an average of four to six

months. Both helmets and cranial bands are recommended to be worn 23 hours

per day with an hour off for exercises and skin care.

Examples of brands of cranial remodeling bands and helmets include the DOC

BAND®, Gillette Children's Craniocap, and the STARband™ Cranial Headband.

Average treatment time with the cranial remodeling band or helmet is 4.5 months.

A systematic evidence review of cranial orthosis treatment for infant deformational

plagiocephaly prepared for the UK National Health Services (NHS QIS, 2007) found

no randomized controlled trials assessing the effectiveness of cranial orthoses for

the treatment of deformational plagiocephaly were identified. The assessment

stated that no evidence-based conclusions can be reached on the effectiveness of

cranial orthoses due to the limited methodological quality of the available trials.

"Further research in the form of a randomised controlled trial is needed to

determine the true effectiveness of cranial orthoses."

While infants with positional plagiocephaly may be treated with head positioning

and/or helmeting, the standard treatment for synostotic plagiocephaly

(asymmetrical head caused by premature closure of the cranial sutures) is surgery.

There is some evidence suggesting that a cranial remodeling band (or helmet) may

improve outcomes following surgery to treat synostotic plagiocephaly. Seymour-

Dempsey et al (2002) retrospectively reviewed the results of surgery alone (n = 6)

versus surgery and post-operative banding (n = 15) in treating children diagnosed

with sagittal synostosis. The investigators reported that correction toward a normal

cephalic index was seen in the banded group throughout the course of treatment,

while this trend was not present in the non-banded group.

Cranial rmodeling bands and helmets are contraindicated in infants older than 24

months. The skulls of these children have finished growing and no longer have the

pliability and plasticity necessary to create a change in shape.

In a randomized controlled trial, Hutchison et al (2010) examined the effectiveness

of the Safe T Sleep positioning wrap in infants with positional head shape

deformities. A total of 126 infants presenting to a plagiocephaly clinic were

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randomized to either positioning strategies or to positioning plus the use of a Safe T

Sleep™ positioning wrap. Head shape was measured using a digital photographic

technique, and neck function was assessed. They were followed-up at home 3, 6

and 12 months later. There was no difference in head shape outcomes for the 2

treatment groups after 12 months of follow-up, with 42 % of infants having head

shapes in the normal range by that time; 80 % of children showed good

improvement. Those that had poor improvement were more likely to have both

plagiocephaly and brachycephaly and to have presented later to clinic. The authors

concluded that most infants improved over the 12-month study period, although the

use of a sleep positioning wrap did not increase the rate of improvement.

Larsen (2004) stated that a second orthosis is rarely required but could be used in

very severe head deformations, unusual circumstances (illness-negated use or if

the child has serious health and/or positioning issues), or unusually high

expectations of the family. The author noted that criteria for determining a second

orthosis include the following:

Despite every effort, the orthosis becomes ill-fitting or leaves little or no

room for new growth;

If age and severity indicate another orthosis and parents are willing to

continue; and

If prescribed for use as a continued post-operative adjunct or for

preventative measures.

The American Academy of Orthotists and Prosthetists' draft consensus statement

on "Orthotic management of deformational plagiocephaly (AAOP, 2004) stated that

"very young infants who have not developed midline head control, rolling, or sitting,

may require a second orthosis to prevent regression of the head

shape". The AAOP stated that a second orthosis is rarely required but may be

used in cases of increased severity, extenuating circumstance (infant with multiple

health issues), or a very young infant (less than 3 months). Criteria for use of a

second orthosis include ill-fitting orthosis after multiple attempts to adjust,

age/severity indicators with a willingness to continue by the family, post-operative

adjunct/ preventative measures. The guideline also noted that termination of the

orthotic treatment program is recommended, without weaning, when head shape

falls within normal limits. If unresolved torticollis exists or if sleeping patterns are

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poor (same side as flatness), use is continued for an additional 2 to 4 weeks.

Furthermore, unshunted or uncontrolled hydrocephalus as well as craniosynostosis

are contraindications for cranial remolding orthosis.

Chan and colleagues (2013) noted that craniosynostosis results in characteristic

skull deformations. Correction of craniosynostosis has traditionally involved an

open cranial vault remodeling (CVR) procedure. A technique recently developed

endoscope-assisted craniectomy (EAC) repair in conjunction with a post-operative

molding helmet to guide cranial growth. Few studies compared these 2

approaches to the treatment of the various forms of craniosynostosis. These

investigators presented a single institution's experience with open CVR and EAC.

This study was a retrospective review of 57 patients who underwent

craniosynostosis repair by either the endoscope-assisted or open techniques; and

compared operating room times, blood loss, volume of transfused blood, length of

hospital stay, and overall costs. The endoscopic technique was performed on

younger children (4.7 months versus 10.6 months, p = 0.001), has shorter

operating room times (2 hours 13 minutes versus 5 hours 42 minutes, p = 0.001),

lower estimated blood loss (74.4 ml versus 280.2 ml, p = 0.001), less transfused

blood (90.6 ml versus 226.9 ml), shorter hospital stays (1.2 days versus 4.9 days, p

= 0.001), and decreased cost ($24,404 versus $42,744, p = 0.008) relative to the

traditional open approach. The authors concluded that issues with the endoscope-

assisted procedure primarily concerned the post-operative helmet regimen,

specifically patient compliance (17.1 % non-compliance rate) and minor skin

breakdown (5.7 %). The endoscope-assisted repair with post-operative helmet

molding therapy was a cost-effective procedure with less operative risk and minimal

post-operative morbidity. This was a valuable treatment option in younger patients

with compliant care-givers.

Vogel and associates (2014) stated that the surgical management of infants with

sagittal synostosis has traditionally relied on open CVR techniques; however,

minimally invasive technologies, including EAC repair followed by helmet therapy

(HT, EAC+HT), is increasingly used to treat various forms of craniosynostosis

during the 1st year of life. These researchers determined the costs associated with

EAC+HT in comparison with those for CVR. They performed a retrospective case-

control analysis of 21 children who had undergone CVR and 21 who had

undergone EAC+HT. Eligibility criteria included an age less than 1 year and at

least 1 year of clinical follow-up data. Financial and clinical records were reviewed

for data related to length of hospital stay and transfusion rates as well as costs

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associated with physician, hospital, and outpatient clinic visits. The average age of

patients who underwent CVR was 6.8 months compared with 3.1 months for those

who underwent EAC+HT. Patients who underwent EAC+HT most often required

the use of 2 helmets (76.5 %), infrequently required a 3ird helmet (13.3 %), and

averaged 1.8 clinic visits in the first 90 days after surgery. Endoscope-assisted

craniectomy plus HT was associated with shorter hospital stays (mean of 1.10

versus 4.67 days for CVR, p < 0.0001), a decreased rate of blood transfusions (9.5

% versus 100 % for CVR, p < 0.0001), and a decreased operative time (81.1

versus 165.8 minutes for CVR, p < 0.0001). The overall cost of EAC+HT,

accounting for hospital charges, professional and helmet fees, and clinic visits, was

also lower than that of CVR ($37,255.99 versus $56,990.46, respectively, p <

0.0001). The authors concluded that EAC+HT were a less costly surgical option for

patients than CVR. Furthermore, EAC+HT were associated with a lower utilization

of peri-operative resources. The authors stated that these findings suggested that

EAC+HT for infants with sagittal synostosis may be a cost-effective 1st-line surgical

option.

Hinchcliff et al (2013) stated that the current treatment of craniosynostosis is open

surgical excision of the prematurely fused suture and CVR. Due to the change in

skull morphology and the increase in volume, some tension on the skin flaps is

noted with closure. Although complete wound breakdown is rare, it can be a

devastating complication. These researchers presented their experience with the

use of the SPY imaging system (Lifecell Corporation, Branchburg, NJ) to visualize

and record blood flow within the flaps of a 1-year old patient with anterior

plagiocephaly. The authors concluded that intra-operative indocyanine green

angiography has the potential to be a significant advantage in such cases,

providing a safe and objective method to assess intra-operative scalp perfusion,

allowing the surgeon to take additional measures to ameliorate any ischemic

problems.

Xia et al (2008) reported on a systematic evidence review to compare molding

helmet therapy with head repositioning therapy for infants with deformational

plagiocephaly. The Cochrane Library and MEDLINE were searched using reported

terms. Electronic searches were conducted of ISI Web of Science, Science Direct.

Journals@Ovid and conference proceedings were screened. Studies that

compared molding helmet therapy with head repositioning therapy for otherwise

healthy infants with deformational plagiocephaly with or without torticollis were

eligible for inclusion. Infants had to have received no prior treatment. Reasons for

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exclusion of identified studies included insufficient information about recruitment of

samples and methods used to measure outcomes. The review assessed treatment

success. Included studies compared molding with repositioning with and without

physiotherapy or neck stretching. In most studies, the duration of treatment ranged

from three to five months. All infants were under 12 months when treatment started;

in most studies treatment started at five to eight months. Two reviewers

independently selected studies. Seven cohort studies were included (n = 881). The

number of children in each treatment group ranged from 10 to 176. Five

prospective, one retrospective and one study with a prospective repositioning group

and a retrospective molding group were included. All studies included consecutive

infants. Flaws included allocation based on physician or patient preference, cross-

over from repositioning to molding, inadequate details of co-interventions, lack of

reporting of masked outcome assessment, molding offered to older or more

severely affected infants and a high drop-out rate. Five studies with comparable

data reported that success rates were higher in infants treated with molding

compared to repositioning therapy. Of the other two studies, the average treatment

time for reposition was much greater than the duration of molding time and the

other did not use the same anatomical landmarks to assess outcomes in both

groups. The only study (n=335) for which the author felt able to calculate the

magnitude to treatment effect reported that treatment success was significantly

more common in the molding compared to the repositioning group; RR 1.3 (95 %

confidence interval (CI): 1.2 to 1.4); NNT 5 (95 % CI: 4 to 7). Reasons for exclusion

of other studies included inadequate data or information about treatments,

significant measurement bias and recruitment only of children who failed

repositioning. The authors concluded that there was considerable evidence that

molding therapy may be more effective at reducing skull asymmetry than

repositioning therapy in infants with deformational plagiocephaly. However, studies

were potentially biased and more research was required.

A critique of this systemic review stated that Xia et al’s conclusions that there was

considerable evidence appeared inconsistent with the subsequent statement about

potential biases in the included studies and a more cautious initial statement would

appear to have been more appropriate (CRD, 2009).

Taylor et al (2015) reported long-term aesthetic outcomes with fronto-orbital

advancement and CVR in treating unicoronal synostosis over a 35-year period.

These investigators performed a retrospective review on patients with isolated

unicoronal synostosis from 1977 to 2012. Demographic, pre-operative phenotypic,

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and long-term aesthetic outcomes data were analyzed with chi-squared and

Fisher's exact test for categorical data and Wilcoxon rank-sum and Kruskal-Wallis

rank for continuous data. A total of 238 patients were treated; 207 met inclusion

criteria. None underwent secondary intervention for intracranial pressure. At

definitive intervention, there were 96 (55 %) Whitaker class I patients, 11 (6 %)

class II, 62 (35 %) class III, and 6 (3 %) class IV. Nasal root deviation and occipital

bossing each conferred an increased risk of Whitaker class III/IV [odds ratios (OR),

4.4 (1.4 to 13.9), p = 0.011; OR, 2.6 (1.0 to 6. 8), p = 0.049]. Patients who

underwent bilateral CVR with extended unilateral bandeau were less likely Whitaker

class III/IV at latest follow-up compared with those undergoing strictly unilateral

procedures [OR, 0.2 (0.1 to 0.7), p = 0.011]. Over-correction resulted in decreased

risk of temporal hollowing [OR, 0.3 (0.1 to 1.0), p = 0.05]. Patients with 5 years or

more of follow-up were more likely to develop supraorbital retrusion [OR, 7.2 (2.2 to

23.4), p = 0.001] and temporal hollowing [OR, 3.7 (1.5 to 9.6), p = 0.006] and have

Whitaker class III/IV outcomes [OR, 4.9 (1.8 to 12.8), p = 0.001]. The authors

concluded that traditional fronto-orbital advancement and CVR appears to mitigate

risk of intracranial pressure but may lead to aesthetic shortcomings as patients

mature, namely fronto-orbital retrusion and temporal hollowing.

Van Wijk et al (2014) reported on the results of the first randomized controlled trial

of helmet therapy in infants with positional skull deformation. The trial determined

the effectiveness of helmet therapy for positional skull deformation compared with

the natural course of the condition in infants aged 5-6 months. The investigators

performed a pragmatic, single blinded, randomized controlled trial (HEADS, HElmet

therapy Assessment in Deformed Skulls) nested in a prospective cohort study in 29

pediatric physiotherapy practices; helmet therapy was administered at four

specialized centers. Study participants were 84 infants aged 5 to 6 months with

moderate to severe skull deformation, who were born after 36 weeks of gestation

and had no muscular torticollis, craniosynostosis, or dysmorphic features.

Participants were randomly assigned to helmet therapy (n = 42) or to natural course

of the condition (n=42) according to a randomization plan with blocks of eight. Six

months of helmet therapy compared with the natural course of skull deformation. In

both trial arms parents were asked to avoid any (additional) treatment for the skull

deformation. The primary outcome was change in skull shape from baseline to 24

months of age assessed using plagiocephalometry (anthropometric measurement

instrument). Change scores for plagiocephaly (oblique diameter difference index)

and brachycephaly (cranioproportional index) were each included in an analysis of

covariance, using baseline values as the covariate. Secondary outcomes were ear

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deviation, facial asymmetry, occipital lift, and motor development in the infant,

quality of life (infant and parent measures), and parental satisfaction and anxiety.

Baseline measurements were performed in infants aged between 5 and 6 months,

with follow-up measurements at 8, 12, and 24 months. Primary outcome

assessment at 24 months was blinded. The change score for both plagiocephaly

and brachycephaly was equal between the helmet therapy and natural course

groups, with a mean difference of -0.2 (95 % CI:-1.6 to 1.2, p = 0.80) and 0.2 (-1.7

to 2.2, p = 0.81), respectively. Full recovery was achieved in 10 of 39 (26 %)

participants in the helmet therapy group and 9 of 40 (23 %) participants in the

natural course group (odds ratio 1.2, 95 % CI:0.4 to 3.3, p = 0.74). All parents

reported one or more side effects. The investigators concluded, based on the

equal effectiveness of helmet therapy and skull deformation following its natural

course, high prevalence of side effects, and high costs associated with helmet

therapy, we discourage the use of a helmet as a standard treatment for healthy

infants with moderate to severe skull deformation.

An UpToDate review on “Overview of craniosynostosis” (Buchanan and Hollier,

2015) states that “In most cases, positional plagiocephaly can be treated by change

in positioning. A custom-fitted helmet designed to relieve pressure on the flattened

side is often used in severe cases (which are rare). However, a single-blind trial

has found no difference in outcomes, including change in skull shape

(plagiocephaly or brachycephaly) and full recovery, at two years of age in 84 infants

with moderate to severe positional skull deformation who were randomly assigned

to helmet therapy or to no therapy (natural course of the condition). In addition, a

number of adverse effects were reported with helmet use, including skin irritation

and parental difficulty in cuddling the infant. The trial had several limitations,

including the 21 percent participation rate and exclusion of the most severe cases

of positional flattening. Until further larger randomized trials that include patients

with more severe positional plagiocephaly/brachycephaly are performed, we will

continue to suggest helmet therapy for patients with severe or recalcitrant positional

flattening”.

Utria and colleagues (2016) noted that due to the changing properties of the infant

skull, there is still no clear consensus on the ideal time to surgically intervene in

cases of non-syndromic craniosynostosis (NSC). These investigators shed light on

how patient age at the time of surgery may affect surgical outcomes and the

subsequent need for reoperation. They performed a retrospective cohort review for

patients with NSC who underwent primary cranial vault remodeling between 1990

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and 2013. Patients' demographic and clinical characteristics and surgical

interventions were recorded. Post-operative outcomes were assessed by assigning

each procedure to a Whitaker category. Multi-variate logistic regression analysis

was performed to determine the relationship between age at surgery and need for

minor (Whitaker I or II) versus major (Whitaker III or IV) re-operation. Odds ratios

for Whitaker category by age at surgery were assigned. A total of 413 unique

patients underwent cranial vault remodeling procedures for NSC during the study

period. Multi-variate logistic regression demonstrated increased odds of requiring

major surgical revisions (Whitaker III or IV) in patients younger than 6 months of

age (OR 2.49, 95 % CI: 1.05 to 5.93), and increased odds of requiring minimal

surgical revisions (Whitaker I or II) in patients older than 6 months of age (OR 2.72,

95 % CI: 1.16 to 6.41). The authors concluded that timing, as a proxy for the

changing properties of the infant skull, is an important factor to consider when

planning vault reconstruction in NSC. They stated that the data presented in this

study demonstrated that patients operated on before 6 months of age had

increased odds of requiring major surgical revisions.

Subgaleal Drain Placement After Primary Cranioplasty in Craniosynostosis

Tong and associates (2015) stated that there is no published data addressing the

use of post-operative subgaleal drains in patients undergoing primary cranioplasty

for craniosynostosis. These investigators conducted a retrospective chart review in

this population of patients, comparing outcomes of those who received post-

operative drains with those who did not. They hypothesized that the subgaleal

drains can significantly reduce post-operative facial edema and decrease the length

of hospital stay. These researchers conducted a retrospective chart review of all

patients undergoing primary cranioplasty for craniosynostosis with subgaleal drain

placement (May 2010 to March 2012). A comparison group without drain

placement was matched appropriately to establish a comparison of outcomes. The

authors examined if subgaleal drainage led to improvement in post-operative facial

edema, reduced length of hospital stay, post-operative changes in hematocrit (Hct),

and complication rates. Of the 50 patients in this cohort, 25 patients had received

subgaleal drains. The mean length of stay was 2.4 versus 3.5 days for the

respective drained and un-drained cohorts (p =0.03). There was no significant

difference in the mean decline in Hct between drained and un-drained patients, with

the mean Hct drop of 4.8 % versus 5.0 %, respectively (p =0.83). Post-operative

seroma formation developed in 3 un-drained patients (17 %) versus none in the

drained cohort (0 %). Although subjective, drained patients were observed to

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achieve quicker resolution of facial swelling and earlier recovery of eye opening.

The authors concluded that there is clinical benefit in subgaleal drain placement as

earlier resolution of post-operative facial edema and a significantly shortened length

of hospital stay was found among the drained cohort. They stated that future

studies warrant prospective clinical trials to establish the safety and effectiveness of

using subgaleal drains in cranial remodeling procedures of craniosynostosis.

Distraction Osteogenesis for Surgical Treatment of Craniosynostosis

Mundinger and colleagues (2016) stated that distraction osteogenesis has been

proposed as an alternative to cranial remodeling surgery for craniosynostosis, but

technique descriptions and outcome analyses are limited to small case series.

These researchers summarized operative characteristics and outcomes of

distraction osteogenesis and presented data comparing distraction osteogenesis to

cranial remodeling surgery. They performed a systematic review of the literature;

descriptive analysis, operative technical data, outcomes, or post-operative

complications of distraction osteogenesis for craniosynostosis were included. A

total of 1,325 citations were reviewed, yielding 53 articles and 880 children who

underwent distraction osteogenesis for craniosynostosis. Distraction plates were

used in 754 patients (86 %), whereas springs were used for the remaining 126

patients (14 %). Standard and spring distraction osteogenesis was reported to

successfully treat the primary condition 98 % of the time. Suboptimal results were

reported in 11 patients (1.3 %), and minor complications were reported in 19.5 % of

cases (n = 172). Major complications were rare, occurring in 3.5 % of cases (n =

31), and included 2 reported deaths. Absolute operative times and blood loss were

marginally greater for cranial remodeling surgery cases, but the differences were

not statistically significant. The authors concluded that distraction osteogenesis is

an effective cranial vault remodeling technique for treating craniosynostosis. No

statistical differences were found with respect to operative time, blood loss, need

for transfusion, or intensive care unit resources compared with cranial remodeling

surgery. Moreover, they stated that outcome studies with longer follow-up periods

specifically investigating cost, relapse, and reoperation rates are needed to

effectively compare this treatment modality as an alternative to cranial remodeling

surgery. (Level of Evidence = 4)

Pre-Operative Molding Helmet Therapy for the Treatment of Sagittal Craniosynostosis

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Swanson and colleagues (2016) stated that there is no clear consensus for the

optimal treatment of sagittal craniosynostosis; however, recent studies suggested

that improved neurocognitive outcomes may be obtained when surgical intervention

imparts active cranial expansion or remodeling and is performed before 6 months of

age. The authors considered spring-mediated cranioplasty (SMC) to optimally

address these imperatives, and this was an investigation of how helmet orthoses

before or after SMC affect aesthetic outcomes. The authors retrospectively

evaluated patients treated with SMC and adjunct helmeting for sagittal synostosis.

Patients were stratified into 4 cohorts based on helmet usage: (i) pre-op, (ii) post-

op, (iii) both, and (iv) neither. The cephalic index was used to assess head shape

changes and outcomes; 26 patients met inclusion criteria: 6 (23 %) had pre-op, 11

(42 %) had post-op, 4 (15 %) had pre-op and post-op, and 5 (19 %) had no

helmeting. Average age at surgery was 3.6 months. Overall, cephalic index

improved from a mean 69.8 to 77.9 during an average 7-month course of care.

Mean pre-operative change in cephalic index showed greater improvement with pre-

op helmet (1.3) versus not (0.0), (p = 0.029), despite similar initial cephalic index in

these cohorts (70.4 and 69.6 respectively, p = 0.69). Nonetheless, all patient cohorts

regardless of helmeting status achieved similar final cephalic indices (range of 76.4 to

80.4; p = 0.72). The authors concluded that pre-operative molding helmet therapy led

to improved cephalic index at the time of spring-mediated cranioplasty. However, they

noted that this benefit did not necessarily translate into overall improved cephalic

index after surgery and in follow-up, calling into question the benefits of molding

helmet therapy in this setting.

Post-Operative Intensive Care Unit Care Following Cranial Vault Remodeling for Sagittal Synostosis

Wolfswinkel and colleagues (2017) stated that of U.S. craniofacial and

neurosurgeons, 94 % routinely admit patients to the intensive care unit (ICU)

following cranial vault remodeling for correction of sagittal synostosis. These

investigators examined the outcomes and cost of direct ward admission following

primary cranial vault remodeling for sagittal synostosis. An institutional review board-

approved retrospective review was undertaken of the records of all patients who

underwent primary cranial vault remodeling for isolated sagittal craniosynostosis

from 2009 to 2015 at a single pediatric hospital. Patient demographics, peri-

operative course, and outcomes were recorded. A total of 110 patients met inclusion

criteria with absence of other major medical problems.

Average age at operation was 6.7 months, with a mean follow-up of 19.8 months;

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98 patients (89 %) were admitted to a general ward for post-operative care,

whereas the remaining 12 (11 %) were admitted to the ICU for pre-operative or peri-

operative concerns. Among ward-admitted patients, there were 4 (3.6 %) minor

complications; however, there were no major adverse events (AEs), with none

necessitating ICU transfers from the ward and no mortalities. Average hospital stay

was 3.7 days. The institution's financial difference in cost of ICU stay

versus ward bed was $5,520 on average per bed per day. Omitting just 1 ICU post-

operative day stay for this patient cohort would reduce projected health care costs

by a total of $540,960 for the study period. The authors concluded that despite the

common practice of post-operative admission to the ICU following cranial vault

remodeling for sagittal craniosynostosis, they suggested that post-operative care be

considered on an individual basis, with only a small percentage requiring a higher

level of care.

Pre-Operative Erythropoietin Therapy

Fearon and Weinthal (2002) noted that the vast majority of infants and children

undergoing craniosynostosis correction receive a blood transfusion. The risks of

blood transfusion include, but are not limited to, acute hemolytic reactions

(approximately 1 of 250,000), human immunodeficiency virus (HIV; approximately 1

of 200,000), hepatitis B and C (approximately 1 of 30,000 each), and transfusion-

related lung injuries (approximately 1 of 5,000). In a prospective, single-blinded,

randomized study, these researchers examined the safety and efficacy of pre-

operative single weekly dosing with erythropoietin ([EPO]; epoetin alfa [Procrit]) in

reducing the rate of transfusion in infants and small children undergoing

craniosynostosis repair. A total of 29 patients (less than 8 years) undergoing

craniosynostosis repair were randomized into 2 groups: one received pre-operative

EPO (600 U/kg) weekly for 3 weeks, and the other served as a control. All care-

givers responsible for blood transfusions were blinded, and strict criteria for

transfusion were established. A pediatric hematologist monitored both groups, and

all patients received supplemental iron (4 mg/kg); 14 patients were randomized to

receive EPO, and 8 of these 14 patients (57 %) needed transfusion (mean age of

17 months; mean weight of 10.1 kg). Of the 6 patients not requiring transfusion, 3

were younger than 12 months old (mean of 6 months); 14 of 15 patients (93 %) in

the control group (mean age of 13 months; mean weight of 9.3 kg) needed a blood

transfusion during the study. The only control patient not requiring transfusion was

the eldest (5 years old). The difference between the 2 groups was statistically

significant (Fisher's exact test = 0.03). The control group showed no change in

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hemoglobin (Hb) levels from baseline to pre-operative levels, but the EPO group

increased their average Hb levels from 12.1 to 13.1 g/dL. There were no adverse

events (AEs) noted among children receiving EPO, nor were there any surgical

complications. The authors concluded that the pre-operative administration of EPO

significantly raised Hb levels and reduced the need for a blood transfusion with

craniosynostosis correction. They stated that more suggestions were made that

may further reduce the need for blood transfusions, and a cost-benefit analysis was

discussed.

Koh and Gries (2007) stated that craniosynostosis, premature closures of the skull

sutures, results in dysmorphic features if left untreated. Brain growth and cognitive

development may also be impacted. Craniosynostosis repair is usually performed

in young infants and has its peri-operative challenges. These investigators

provided background information regarding the different forms of craniosynostosis,

with an overview of associated anomalies, genetic influences, and their connection

with cognitive function. The authors also discussed the anesthetic considerations

for peri-operative management, including blood-loss management and strategies to

reduce homologous blood transfusions. These researchers noted that available

studies suggested that EPO may reduce the transfusion requirements, but could

not eliminate the need for transfusion.

Krajewski and colleagues (2008) stated that craniosynostotic correction typically

performed around infant physiologic nadir of Hb (approximately 3 to 6 months of

age) is associated with high transfusion rates of packed red blood cells (RBCs) and

other blood products. As a blood conserving strategy, these investigators studied

the use of recombinant human EPO or Procrit (to optimize pre-operative hematocrit

[Hct]); and Cell Saver (to recycle the slow, constant ooze of blood during the

prolonged case). Patients with craniosynostosis at UCLA from 2003 to 2005 were

divided into the study group (Procrit and Cell Saver) or the control group (n = 79).

The study group first received recombinant human EPO at 3 weeks, 2 weeks, and 1

week pre-operatively and then used Cell Saver intra-operatively. Outcomes were

based on morbidities and transfusion rate comparisons. The 2 groups were

comparable with regards to age (5.66 and 5.71 months), and operative times (3.11

versus 2.59 hours). In the study group there was a marked increase in pre-

operative Hct (56.2 %). The study group had significantly lower transfusions rates

(5 % versus 100 % control group) and lower volumes transfused than in the control

group (0.05 pediatric units versus 1.74 pediatric units). Additionally, of the 80 % of

patients in the study group who received Cell Saver blood at the end of the case,

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approximately 31 % would have needed a transfusion if the recycled blood were

unavailable. The authors concluded that these findings showed that for elective

craniosynostotic correction, successful blood conserving dual therapy with Procrit

and Cell Saver might be used to decrease transfusion rates and the need for any

blood products.

Vega and associates (2014) stated that children with craniosynostosis may require

cranial vault remodeling to prevent or relieve elevated intra-cranial pressure (ICP)

and to correct the underlying craniofacial abnormalities. The procedure is typically

associated with significant blood loss and high transfusion rates. The risks

associated with transfusions were well documented and included transmission of

infectious agents, bacterial contamination, acute hemolytic reactions, transfusion-

related lung injury, and transfusion-related immune modulation. These

investigators presented the Children's Hospital of Richmond (CHoR) protocol,

which was developed to reduce the rate of blood transfusion in infants undergoing

primary craniosynostosis repair. They performed a retrospective chart review of

pediatric patients treated between January 2003 and February 2012. The CHoR

protocol was instituted in November 2008, with the following 3 components: the use

of pre-operative EPO and iron therapy; the use of an intra-operative blood recycling

device: and acceptance of a lower level of Hb as a trigger for transfusion (less than

7 g/dL). Patients who underwent surgery prior to the protocol implementation

served as controls. A total of 60 children were included in the study, 32 of whom

were treated with the CHoR protocol. The control (C) and protocol (P) groups were

comparable with respect to patient age (7 versus 8.4 months, p = 0.145).

Recombinant EPO effectively raised the mean pre-operative Hb level in the P group

(12 versus 9.7 g/dL, p < 0.001). Although adoption of more aggressive surgical

vault re-modeling in 2008 resulted in a higher estimated blood loss (EBL; 212

versus 114.5 ml, p = 0.004) and length of surgery (4 versus 2.8 hours, p < 0.001),

transfusion was performed in significantly fewer cases in the P group (56 % versus

96 %, p < 0.001). The mean length of stay (LOS) in the hospital was shorter for the

P group (2.6 versus 3.4 days, p < 0.001). The authors concluded that a protocol

that included pre-operative administration of recombinant EPO, intra-operative

autologous blood recycling, and accepting a lower transfusion trigger significantly

decreased transfusion utilization (p < 0.001). A decreased LOS (p < 0.001) was

observed, although the authors did not examine if composite transfusion

complication reductions led to better outcomes. These findings were confounded

by the combined use of pre-operative administration of recombinant EPO, intra-

operative autologous blood recycling, and accepting a lower transfusion trigger.

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White and co-workers (2015) noted that surgery for craniosynostosis is associated

with the potential for significant blood loss. Multiple technologies have been

introduced to reduce the volume of blood transfused. These are pre-operative

autologous donation; pre-operative EPO; intra-operative cell salvage (CS); acute

normo-volemic hemodilution; anti-fibrinolytic drugs such as tranexamic acid,

ε-aminocaproic acid, and aprotinin; fibrin sealants or fibrin glue; and post-operative

drain re-infusion. All comparative studies with a treatment group and a control

group were considered. There was a range of different study types from

randomized controlled trials (RCTs) to case series with historic controls. These

were intervention versus no intervention or a comparison of 2 interventions.

Studies were identified by searching Cochrane CENTRAL, Medline, and Embase;

manufacturer's Web sites; and bibliographies of relevant published articles. The

primary outcome measures were the number of allogeneic blood donor exposures,

the volume of allogeneic blood transfused, and the post-operative Hb or Hct levels.

A total of 696 studies were identified. After removal of duplicates and after

exclusion criteria were applied, 18 studies were included; 14 were case series with

controls and 4 were RCTs. The authors concluded that the production of high-

quality evidence on the interventions to minimize blood loss and transfusion in

children undergoing surgery for craniosynostosis was difficult. Most of the literature

was non-randomized and non-comparative. Several areas remain unaddressed;

EPO and tranexamic acid were comparatively well studied; CS, acute normo-

volemic hemodilution, and aprotinin were less so. There was only 1 comparative

study on the use of fibrin glue and drain re-infusion, with no studies on pre-

operative autologous donation and ε-aminocaproic acid. Tranexamic acid was

clinically effective in reducing allogeneic blood transfusion. There was some

evidence that CS and EPO may be clinically effective. None of the interventions

studied was shown to be cost-effective because of lack of evidence.

Mathijssen (2015) stated that the “Guideline for care of patients with the diagnoses

of craniosynostosis” was developed by a national working group with

representatives of 11 matrix societies of specialties and the national patients’

society. All medical aspects of care for non-syndromic and syndromic

craniosynostosis were included, as well as the social and psychologic impact for

the patient and their parents. Managerial aspects were incorporated as well, such

as organizing a timely referral to the craniofacial center, requirements for a

dedicated craniofacial center, and centralization of this specialized care. The

conclusions and recommendations within this document were founded on the

available literature, with a grading of the level of evidence, thereby highlighting the

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areas of care that are in need of high-quality research. The development of this

guideline was made possible by an educational grant of the Dutch Order of Medical

Specialists. This guideline stated that “Administration of EPO preceding the

intervention, as well as collecting autologous blood for autotransfusion are advised

against”.

Peuntel-Espel and associates (2016) noted that in craniofacial centers where

resources are limited, planning takes an even more important role, where potential

pitfalls and complications must be extensively discussed by all team members

previous to the operation. In addition to a central and an arterial line, blood

transfusion products must be available and planned based on the patient's

estimated blood volume and accurate blood loss monitoring. The room

temperature should be kept warm, and if this is difficult, a warming device (e.g.,

bear hugger [3M, USA]) must be used. In addition, the use of a tumescent

infiltration containing triamcinolone, ropivacaine, adrenaline, and hyaluronidase on

the subgaleal plane, both at the incision site and on the regions where dissection is

going to be performed, can be of invaluable help in providing hemostasis, analgesia

and reducing swelling in these patients. In places where, devices such as cell

saving, or medications like EPO or tranexamic acid are not available, the treating

surgeons must be extremely alert of silent bleeders.

Aljaaly and colleagues (2017) stated that pediatric craniosynostosis surgery is

associated with significant blood loss often requiring allogenic blood transfusion

(ABT). These researchers examined the clinical effectiveness of pre-operative

EPO administration in pediatric craniosynostosis surgery in reducing transfusion

requirements. They carried out a systematic review and meta-analysis of the

literature for studies published in English language between 1946 and 2015.

Inclusion criteria included original studies in the pediatric population (0 to 8 years of

age) involving pre-operative use of EPO in craniofacial procedures with quantitative

reporting of peri-operative blood transfusion. Extracted data included

demographics, EBL, Hb, Hct, number of patients transfused, and amount of ABT.

A total of 4 studies met the inclusion criteria with a total of 117 patients. Patients

were divided into 2 groups: EPO versus control. No statistical differences were

found in the demographics between the 2 groups. Mean pre-operative Hct level

was higher in the EPO group compared with control (43 % versus 35 %). The

percentage of patients who required ABT and the volume of transfused blood were

less in the EPO group (54 % versus 98 % and 84 versus 283 ml, respectively).

Meta-analysis of 3 comparable studies showed a lower proportion of patients who

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needed blood transfusion in the EPO group. The authors concluded that the

present meta-analysis demonstrated that pre-operative administration of EPO in

pediatric craniosynostosis surgery decreased the proportion of patients requiring

ABT. In addition, the volume of transfusion was reduced in patients who received

EPO. Moreover, these investigators stated that future randomized studies are

needed to establish the cost-effectiveness of routine pre-operative EPO

administration in craniosynostosis surgery.

Stricker and co-workers (2017) noted that the Pediatric Craniofacial Collaborative

Group established the Pediatric Craniofacial Surgery Perioperative Registry to

elucidate practices and outcomes in children with craniosynostosis undergoing

complex cranial vault reconstruction (CCVR) and inform quality improvement

efforts. These researchers determined peri-operative management, outcomes, and

complications in children undergoing CCVR across North America and to delineate

salient features of current practices. A total of 31 institutions contributed data from

June 2012 to September 2015. Data extracted included demographics, peri-

operative management, LOS, laboratory results, and blood management

techniques employed. Complications and outlier events were described.

Outcomes analyzed included total blood donor exposures, intra-operative and peri-

operative transfusion volumes, and LOS outcomes. A total of 1,223 cases were

analyzed: 935 children aged less than or equal to 24 months and 288 children aged

more than 24 months; 95 % of children aged less than or equal to 24 months and

79 % of children aged more than 24 months received at least 1 transfusion. There

were no deaths. Notable complications included cardiac arrest, post-operative

seizures, unplanned post-operative mechanical ventilation, large-volume

transfusion, and unplanned second surgeries. Utilization of blood conservation

techniques was highly variable. The authors presented a comprehensive

description of peri-operative management, outcomes, and complications from a

large group of North American children undergoing CCVR. These investigators

stated that transfusion remained the rule for the vast majority of patients. The

occurrence of numerous significant complications together with large variability in

peri-operative management and outcomes suggested targets for improvement. In

particular, these researchers stated that synthetic EPO has been studied as a

means to increase the amount of blood loss that can be safely tolerated in infants

undergoing craniofacial surgery. Despite potential benefits, only 3 patients in their

dataset received pre-operative EPO. It appeared that cost, inconvenience, and

concerns for complications have all but eliminated the use of EPO in CCVR.

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An UpToDate review on “Red blood cell transfusion in infants and children:

Indications” (Teruya, 2019) states that “Certain orthopedic surgeries are often

associated with significant blood loss requiring RBC transfusion. This is particularly

true of spinal fusion surgery for scoliosis, which can be associated with massive

blood loss. The severity of blood loss correlates with the number of spinal levels

fused. Several techniques have been used to reduce the transfusion rates in

pediatric and adult patients undergoing spinal fusion. These include preoperative

autologous blood donation, intraoperative blood salvage, and antifibrinolytics (e.g.,

tranexamic acid and epsilon-aminocaproic acid). Other surgeries that occasionally

require multiple units of RBCs include certain plastic surgery and surgical oncology

procedures. In particular, surgical correction of craniosynostosis almost always

requires RBC transfusion. Measures that have reduced RBC allogeneic

transfusions include the preoperative use of erythropoietin and perioperative blood

salvage. However, in one report, acute normovolemic hemodilution in surgery for

the repair of craniosynostosis did not change the need for transfusion and the

volume of transfused RBC”.

Furthermore, an UpToDate review on “Overview of craniosynostosis” (Buchanan

and Hollier, 2019) does not mention the use of EPO as a management tool.

CPT Codes / HCPCS Codes / ICD-10 Codes

Information in the [brackets] below has been added for clarification purposes. Codes requiring a 7th character are represented by "+":

Page 22 of 29

CPT codes covered if selection criteria are met:

20690 Application of a uniplane (pins or wires in 1 plane), unilateral, external

fixation system

20692 Application of a multiplane (pins or wires in more than 1 plane),

unilateral, external fixation system (eg, Ilizarov, Monticelli type)

20693 Adjustment or revision of external fixation system requiring anesthesia

(eg, new pin[s] or wire[s] and/or new ring[s] or bar[s])

20694 Removal, under anesthesia, of external fixation system

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Code Code Description

20696 Application of multiplane (pins or wires in more than 1 plane), unilateral,

external fixation with stereotactic computer-assisted adjustment (eg,

spatial frame), including imaging; initial and subsequent alignment(s),

assessment(s), and computation(s) of adjustment schedule(s)

20697 Application of multiplane (pins or wires in more than 1 plane), unilateral,

external fixation with stereotactic computer-assisted adjustment (eg,

spatial frame), including imaging; exchange (ie, removal and

replacement) of strut, each

CPT codes not covered for indications listed in the CPB:

92240 Indocyanine-green angiography (includes imaging) with interpretation

and report

92242 Fluorescein angiography and indocyanine-green angiography (includes

multiframe imaging) performed at the same patient encounter with

interpretation and report, unilateral or bilateral

Other CPT codes related to the CPB:

97763 Orthotic(s)/prosthetic(s) management and/or training, upper extremity

(ies), lower extremity(ies), and/or trunk, subsequent orthotic(s)/prosthetic

(s) encounter, each 15 minutes

HCPCS codes covered if selection criteria are met:

D5924 Cranial prosthesis

L0112 Cranial cervical orthosis, congenital torticollis type, with or without soft

interface material, adjustable range of motion joint, custom fabricated

L0113 Cranial cervical orthotic, torticollis type, with or without joint, with or

without soft interface material, prefabricated, includes fitting and

adjustment

S1040 Cranial remolding orthosis, pediatric, rigid, with soft interface material,

custom fabricated, includes fitting and adjustment(s)

HCPCS codes not covered for indications listed in the CPB:

C9733 Non-ophthalmic fluorescent vascular angiography

ICD-10 codes covered if selection criteria are met:

Q67.2 Dolichocephaly

Q67.3 Plagiocephaly

Q67.4 Other congenital deformities of skull, face and jaw (see criteria)

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Q75.0

Q75.1

ICD-10 codes not covered for indications listed in the CPB (not all inclusive):

Page 24 of 29

G91.0

G91.1

G91.2

Q05.0 - Q05.4

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Copyright Aetna Inc. All rights reserved. Clinical Policy Bulletins are developed by Aetna to assist in administering plan

benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial,

general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care

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AETNA BETTER HEALTH® OF PENNSYLVANIA

Amendment to Aetna Clinical Policy Bulletin Number: 0379 Cranial

Remodeling

There are no amendments for Medicaid.

www.aetnabetterhealth.com/pennsylvania updated 07/16/2019

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http://www.aetnabetterhealth.com/pennsylvania

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