Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Abnormal Movements.
Treatment: Inpatient Hospital Stay.
The insurer denied the Inpatient Hospital Stay.
The determination is overturned.

The patient saw an outside child neurologist and was sent to the emergency room for urgent evaluation of abnormal movements. She presented with facial grimacing, eyelid fluttering, eye rolling, repetitive tongue movements, and involuntary limb movements. The movements subsided during sleep. Her parents described hyperactivity, clumsiness and staring spells without clear loss of consciousness. She had difficulty walking, speaking had become difficult, and she had incoordination when trying to eat or write.
Six weeks prior to admission, the patient had had two episodes of diarrhea and vomiting. Four weeks prior to admission she had fever to 103 degrees Fahrenheit (F), with a sore throat, nasal congestion, joint pain and a headache. She was seen by her pediatrician, who recommended acetaminophen.
The patient's past medical history was unremarkable. There was no family history of movement disorder or rheumatic fever. The patient lived with her parents and two siblings. Examination was significant for abnormal movements, as described above. No cardiac murmurs were heard, and no subcutaneous nodules were felt.
Video electroencephalogram (EEG) telemetry was started to capture the abnormal movements. No epileptiform activity was noted. The movements were involuntary physiological movements. An echocardiogram and electrocardiogram were normal. The antistreptolysin titer (ASO) was markedly elevated at 662, resulting in a diagnosis of probable acute rheumatic fever. Inpatient pediatric cardiology, infectious disease and neurology consults were obtained. The patient was started on amoxicillin, and discharged home with neurology and cardiology followup.
At issue in this review is whether inpatient stay was medically necessary for this patient.

The requested health service/treatment of inpatient stay was medically necessary for this patient. The patient met criteria for probable acute rheumatic fever. It was medically necessary for her to be admitted for a diagnostic workup including video EEG telemetry to capture the abnormal movements for differential diagnosis, and for a cardiac evaluation, including an echocardiogram and electrocardiogram. The abnormal movements were suspicious for Sydenham's chorea, seen in acute rheumatic fever, but the differential diagnosis included tics or an unusual form of epilepsy. Sudden death is occasionally seen in acute rheumatic fever, either due to acute heart failure or arrhythmia. Inpatient level of care was required.
Lubberdink et al [1] reported that "Isolated motor disturbances in the paediatric population are uncommon presentations to the emergency department. Choreiform movements have a broad differential diagnosis and may present insidiously with progressive worsening of asymmetric clumsiness, hypotonia and dysarthria. The incidence of Sydenham's chorea (SC) caused by acute rheumatic fever (ARF) is very rare in developed countries.".
Watemberg et al [2] describes the importance of video EEG telemetry in clarifying the diagnosis in children with the onset of paroxysmal movements. "Video recording was added to 137 of 666 routine studies. Mean patient age was 4.8 years.Adding video recording to routine EEG was helpful in 45% of cases referred for frequent paroxysmal events."
Khanna et al [3] notes that "Acute rheumatic fever (ARF) is an inflammatory reaction involving the joints, heart, and nervous system that occurs after a group A streptococcal infection. It typically presents as a febrile illness with clinical manifestations that could include arthritis, carditis, skin lesions, or abnormal movements. Of these, the cardiac manifestations of acute rheumatic fever are most concerning, as children may present in acute heart failure and may go on to develop valvular insufficiency or stenosis. Because this is a rare presentation to emergency departments in developed countries, it is crucial for clinicians to keep a broad differential when presented with clinical presentations suspicious for acute rheumatic fever."
Because of its rarity in the United States, neither the Academy of Pediatrics (AAP), American Heart Association (AHA) nor the Infectious Diseases Society of America have guidelines on the diagnosis, investigation and management of suspected acute rheumatic fever. Acute rheumatic fever remains common in the Aboriginal population of Australia, however. Rheumatic Heart Disease Australia has published detailed guidelines on the diagnosis, investigation and management of patients with suspected rheumatic fever [4]. The guidelines emphasize the need for hospital admission:
"Admit all patients suspected to have ARF
Determine the diagnosis
The diagnosis is determined based on
1) Understanding of epidemiological risk
2) History obtained from primary care staff and/or patient and their family
3) Clinical observation prior to anti-inflammatory treatment: use paracetamol (first line) during this time if required for fever or joint pain
4) Investigations
5) Follow up findings
The final diagnosis may not be clear until several months after the acute episode; e.g. if Jones criteria are not met for a diagnosis of definite ARF but a follow up echocardiogram confirms rheumatic valvular changes not visible at the outset, then the diagnosis shifts from possible or probable to definite ARF."
Inpatient hospital admission was medically necessary for this patient, who had probable rheumatic fever with Sydenham's chorea.