Appeal type: Medical Necessity
Decision: Overturned Decision of Health Plan

Nature of Statutory Criteria/Case Summary:  The enrollees parent has requested authorization and reimbursement for pediatric day health care and private duty nursing/nursing care at home.  The record indicates the enrollee is diagnosed with Koolen de Vries Syndrome after genetic testing was done and revealed KANLS1 mutation. The enrollee had been feeding poorly after delivery and magnetic resonance imaging (MRI) of the brain showed dysplastic corpus callosum. She spent approximately four weeks in the Neonatal Intensive Care Unit prior to discharge home, related to her feeding issues. She subsequently underwent gastrostomy tube placement and takes 40% of nutritional needs by mouth, with the rest being delivered via gastrostomy (G) tube. She has hypotonia, eustachian tube dysfunction, tracheomalacia, reflux, silent aspiration, audio neuropathy and developmental delay.  She has inspiratory stridor that exacerbates when crying. She has hearing loss, gross motor, fine motor, and language delays. She is followed by Neurology, Genetics, Ophthalmology, Otolaryngology, and Gastroenterology. She receives physical and occupational therapy, and home health private duty nursing care. Gastroenterology records indicate the enrollee has a history of constipation and decreased appetite. She takes Enfamil Gentlease formula by mouth and by tube daily. G tube feedings are administered over approximately 25 minutes. She tolerates some solids by mouth. Weight has not been progressing as desired. The documentation outlines a plan to increase feeding volume. She takes seven feeds per day and the volume of each feed is increased. The enrollee has a history of silent aspiration of thin liquids, which has resolved according to a  swallow study. Documentation from the pediatrician describes multiple encounters for upper respiratory symptoms, ear infections, and bronchiolitis.  Nursing documentation states that nurses are to prepare and administer formula (by mouth and by gastrostomy tube); auscultate the lungs at the beginning of each shift; provide gentle exercises to improve range of motion and facilitate physical therapy in the setting of hypotonia; provide gastrostomy care related to feeding; and to monitor the skin for integrity. According to the medical literature, the chromosome 17q21.31 deletion syndrome is a genomic disorder characterized by highly distinctive facial features, moderate-to-severe intellectual disability, hypotonia and friendly behavior. Review of the record indicates the enrollee is still vulnerable from a physical and developmental standpoint. Her hypotonia (a component of her genetic syndrome) and gross motor delays place her at risk of aspiration, the presence of which, cannot be precluded by a single swallow study. Chronic pulmonary aspiration, a type of lung injury caused by intermittent or persistent contamination of the airways with oral and/or gastric content, occurs when the normal airway protective mechanisms are impaired, bypassed, or overwhelmed. The consequences of injuries may include bronchospasm, atelectasis, pulmonary edema, pneumonia, and/or bronchiectasis. Further, most major complications of children following PEG placement occur within the first year following placement. Infants with technology dependence, including those requiring nutritional supports, are candidates for home nursing. Hospitalized children with complex chronic conditions, which includes technology dependence, who were discharged to home health care experienced fewer short-term re-admissions and subsequent hospitalizations versus those not receiving home health care. This childs medical complexity and young age warrant close monitoring with pediatric day health care and private duty nursing/ nursing care at home.Final Result: The reviewer determined that the service at issue is medically necessary for treatment of the enrollees condition. Credentials/Qualifications: The physician reviewer is board certified in Pediatric Gastroenterology and is actively practicing. The reviewer is an expert in the treatment of the enrollees medical condition and knowledgeable about the proposed treatment through recent or current actual clinical experience treating those with the same or similar medical condition.