Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Congenital cataract, intrauterine frowth
retartdation, torticollis, plagiocephaly
Treatment: DME - Cranial Orthosis
The insurer denied the DME - Cranial Orthosis.
The denial is overturned. 

The patient is a male with history of congenital cataract status post-surgical correction, intrauterine growth retardation and postnatal diagnosis of failure to thrive, and torticollis referred to physical therapy (PT).  The patient was referred to Orthotics and to Neurosurgery because of persistent abnormal head shape. In addition to PT, the patient's family was also practicing repositioning. The patient was subsequently diagnosed with severe plagiocephaly both clinically and with anthropometrics. 

On clinical exam, the patient was noted to have forehead bossing to the right, increased bilateral cranial vault height, right occipital flattening, and right anterior ear displacement. Cephalic index was 84.85% and cranial vault asymmetry was 16.00 millimeters (mm). A cranial remolding orthosis was recommended and prescribed. 

Yes, the proposed treatment with DME - Cranial Orthosis is medically necessary.

"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 "Back to Sleep" recommendations). 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. (1-3) Although positional head deformity is generally not a life-threatening condition, up to ten percent of infants may have permanent deformity with subsequent complications. (4)
 
Because plagiocephaly and brachycephaly are extremely variable in etiology and presentation, strict guidelines for management are not possible. In 2003, the American Academy of Pediatrics (AAP) published prevention and management guidelines for positional skull deformities in infants, written by the Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery. "Management of deformational plagiocephaly involves preventive counseling of parents, mechanical adjustments, and exercises ...Skull molding helmets are an option for patients with severe deformity or skull shape that is refractory to therapeutic physical adjustments and position changes. Surgery is rarely necessary but may be indicated in severe refractory cases of deformational plagiocephaly or in patients with craniosynostosis." (1)

More recently, new guidelines were proposed for treatment: "for infants less than three months of age, aggressive repositioning of the infant off the flattened occiput is recommended with neck exercises. Among infants between three to five months of age, orthotic cranioplasty may be indicated, and for children 5 to 18 months of age, orthotic cranioplasty is required. After 24 months of age, surgery by a paediatric neurosurgeon or craniofacial surgeon is required, as orthotic cranioplasty is no longer an effective option." (5) 

Graham et al (3) 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), and early orthosis was significantly more effective than later orthosis (65% decrease versus 51% decrease in DD)." They concluded that "when physical therapy and repositioning fail to treat or prevent plagiocephaly and there is more than 1 cm difference between the two cranial diagonal differences at 6 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." 

Finally, 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 that "infants will have more improvement in head shape with a helmet than with repositioning". (9) 

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. Further, the Congress of Neurological Surgeons and the AANS/CNS 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)." (10)

This male infant has severe plagiocephaly likely exacerbated by torticollis that has not responded to repositioning or physical therapy. Based on the current standard of care supported by peer-reviewed literature, health plan definitions of medically necessary treatments, and endorsed by the American Association of Neurological Surgeons, the Congress of Neurological Surgeons, and the American Academy of Pediatrics, cranial orthosis is medically necessary and appropriate for this infant with severe plagiocephaly.