Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Plagiocephaly/brachycephaly.
Treatment:  Custom-Made Cranial Helmet, Pre-service.

The insurer denied the  Custom-Made Cranial Helmet, Pre-service.  The denial is overturned. 

The patient is a female infant twin A. She was born full term via vaginal delivery. She has a medical history significant for gastroesophageal reflux, left torticollis, and subsequent plagiocephaly/brachycephaly. Her family has been actively repositioning (more than three months), and she has been receiving weekly physical therapy without significant improvement.

Per the physical therapy notes, the patient is not able to hold her head in proper alignment and is unable to use both upper extremities equally for reaching and grasping skills. She underwent evaluation for a DOC (Dynamic Orthotic Cranioplasty) band. History revealed low lying in utero positioning as twin A. Head shape evaluation revealed right plagiocephaly and brachycephaly right greater than left, head tilt to the left with head turning to the right, right occipital flattening, left frontal flattening, right anterior ear and orbit shift, left eye fissure narrowed, and head height right greater than left. Anthropometrics revealed cephalic index 93.9 (4.4 standard deviations from the mean) and 8 mm (millimeters) cranial vault asymmetry, representing severe brachycephaly with some component of plagiocephaly. A DOC (Dynamic Orthotic Cranioplasty) band was recommended, possibly with need for a second band.

Yes, the DOC (Dynamic Orthotic Cranioplasty) band is medically necessary and appropriate for this infant.

"Positional plagiocephaly" and "deformational brachycephaly" are broad terms used to encompass abnormal head shape due to a wide variety of etiologies. Prenatal causes include resting of the fetal head against a hard surface (such as the mother's pelvis or the limb of a sibling in a multiple gestation pregnancy) for a prolonged period of time. Postnatal causes are more common and include congenital torticollis, vertebral anomalies, neurologic impairment, or forced sleep position (e.g. the AAP's [American Academy of Pediatrics] "Back to Sleep" recommendations [6]). Presentation ranges from mild to severe. Mild and even moderate cases may respond to mechanical adjustments and exercises. However, there are some cases that are severe and refractory to repositioning.  Although rare, some cases are severe enough to require surgery for correction. Although positional head deformity is generally not a life-threatening condition, up to 10 percent of infants may have permanent deformity with subsequent complications.

The current standard of care for treatment of mild head shape abnormality includes repositioning, physical therapy, and increased time in the prone position. Most physicians currently still recommend cranial orthoses for their infant patients with moderate to severe skull deformity because research in the long term shows distinct benefit from helmeting compared to repositioning.

Graham et al. (1) evaluated 176 infants treated with repositioning, 159 treated with helmets, and 37 treated first with repositioning followed by helmet therapy when initial treatment failed. The authors compared reductions in diagonal difference (DD, cranial vault asymmetry) between repositioning and cranial orthotic therapy. They found that "orthotic therapy was more effective than repositioning (61% decrease versus 52% decrease in DD [diagonal difference]), and early orthosis was significantly more effective than later orthosis (65% decrease versus 51% decrease in DD [diagonal difference])." They concluded, "When physical therapy and repositioning fail to treat or prevent plagiocephaly and there is more than 1 cm [centimeter] difference between the two cranial diagonal differences at six months, orthotic therapy is effective in correcting such asymmetry. Delays in initiating corrective treatment until later infancy may lead to incomplete or ineffective correction even if orthotic therapy is initiated, so early diagnosis and treatment is essential."

Another study was published using a longitudinal cohort design, following 100 infants with deformational plagiocephaly and/or brachycephaly, comparing helmeting with repositioning. These infants were followed up to ten years. Their data suggest, "Infants will have more improvement in head shape with a helmet than with repositioning" (3). In terms of brachycephaly, Graham et al. (3) recommended, "for those infants who do not make progress with repositioning and tummy-time, with severe persistent brachycephaly (CI [confidence interval] > 90%) at age 5 months, use of an orthotic helmet will correct the brachycephaly and any associated plagiocephaly." For infants with extreme occipital flattening, prolonged treatment may be necessary.

Of more concern at this point comes from a study by Collett et al. (5), in which school-aged children that had history of moderate to severe positional plagiocephaly and/or brachycephaly were found to score lower on cognitive and academic measures compared to controls without abnormal head shape or those with mild head shape abnormality. Using head shape as a marker, these authors suggest ongoing developmental assessment of infants with more severe head shape abnormalities. Further, there are ongoing long-term concerns with untreated plagiocephaly/brachycephaly in terms of ability to fit eyeglasses, jaw alignment, and psychological impact.

Finally, the Congress of Neurological Surgeons and the AANS/CNS (American Association of Neurological Surgeons/Central Nervous System) Joint Section on Pediatric Neurosurgery have posited the following in recently published guidelines: "Helmet therapy is recommended for infants with persistent moderate to severe plagiocephaly after a course of conservative treatment (repositioning and/or physical therapy)" (4).

This female infant has severe brachycephaly compounded by plagiocephaly complicating left torticollis and gastroesophageal reflux. Her family has been repositioning for months, and she has been receiving physical therapy without significant benefit. At this point, it is negatively impacting her development as described in the physical therapy notes. The requested DOC (Dynamic Orthotic Cranioplasty) band is medically necessary and appropriate, with the possibility of a second DOC (Dynamic Orthotic Cranioplasty) band in the future.