Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Chorioretinal Colobomas.
Treatment: Scleral Cover Shell and Ocular Conformer Fitting.
The insurer denied the Scleral Cover Shell and Ocular Conformer Fitting.
The denial is overturned.

The patient is a female with history of bilateral chorioretinal colobomas with cysts and microphthalmia of the left eye (OS) for whom an orbital conformer replacement (V2627/V2628) is the subject of review. 

Reportedly, the patient's ocular conformer was lost, and the provider states that this service is necessary for orbital bone growth for a patient with a severely micophthalmic blind eye.  

A note documents complaints that the patient's cover shell keeps falling out. Vision was questionable light perception right eye (OD) and questionable no light perception left eye (OS). The patient had developed serous retinal detachments, left eye greater than right eye (OS>OD) due to the colobomas. 

Coverage was previously denied as the plan states that coverage is not available for replacement of prosthetic devices that are lost or stolen.

Yes, the proposed treatment is medically necessary.

No, while the Health Plan followed its policy, it did not act reasonably, with sound medical judgment and in the best interest of this pediatric patient with congenital microphthalmia with cyst.

Congenital anophthalmia and microphthalmia are rare diseases that cause anomalous orbitofacial growth and significant visual morbidity. The absence of a normal-sized globe is associated with orbital bone and soft tissue retardation and hemifacial microsomia. The management of severe microphthalmia or anophthalmia involves stimulating orbitofacial growth.

Different techniques and materials have been used to expand the orbital volume, including acrylic conformers, spherical orbital implants, mucous grafts, dermis fat grafts, bone and muscle grafts, inflatable balloon devices, and hydrogel expanders. However, the growth of eyelids and orbital bones also require stimulation either concurrently or soon after addressing orbital volume expansion. Hence, the rehabilitation process should begin soon after birth. Parents often decline surgical management because their children are very young.

In cases where surgical management is refused, progressive expansion therapy with custom eye conformers followed by custom ocular prostheses is a reasonable option. Previous studies have reported that socket expansion with self-inflating expanders and custom-made eye conformers produce similar outcomes.

A conformer is a clear acrylic shell fitted to an anophthalmic socket or in the case of a microphthalmia socket before fitting an ocular prosthesis. It helps in the formation of fornices and helps expand the socket. In the case of congenital contracted sockets, the conformer size needs to be changed every six weeks to two months for size to expand the socket gradually. It is also used after an enucleation an evisceration of the eyeball over the implant inside the socket to hold the shape of the eye socket and allow the eyelids to blink over the shell without rubbing the suture line. The conformer shell holds the shape ready for the artificial eye.

Orbital expansion and lid fornices formation by using an eye conformer is effective, easy, and acceptable to parents. It is medically necessary per standard of care. Not replacing an eye conformer either because of loss or growth would not be in the best interest of this child.