ICD 10 Coding Alert

Use additional code for any associated intellectual disabilities.

If your psychiatrist specializes in treating patients with childhood developmental or disintegrative disorders, you’ll be relieved to know that a diagnosis of Rett’s syndrome can be very specifically reported using the ICD-10 system while you had to use a generalized diagnosis code in ICD-9.

ICD-9: When your clinician makes a diagnosis of Rett’s syndrome, you will have to report it with 330.8 (Other specified cerebral degenerations in childhood). You have to note that ICD-9 does not have a specific code to report this condition, and you have only a generalized code to report a diagnosis of Rett’s syndrome.

You use the same diagnosis code if the diagnosis is Alpers’ disease or gray-matter degeneration; Infantile necrotizing encephalomyelopathy; Leigh’s disease; or Sub-acute necrotizing encephalopathy or encephalomyelopathy.

Reminder: You also have to code any associated intellectual disabilities with a separate additional code to identify them.

ICD-10: Unlike ICD-9, which does not have a specific code to report a diagnosis of Rett’s syndrome, you have a code for this condition in ICD-10. So, when you begin using ICD-10, you can report a diagnosis of Rett’s syndrome using F84.2 (Rett’s syndrome).

Note, you have a very specific list of exclusions that you cannot report with F84.2. So, you do not report F84.2 if the diagnosis is Asperger’s syndrome (F84.5); Autistic disorder (F84.0); or other childhood disintegrative disorders that includes dementia infantilis, disintegrative psychosis, Heller’s syndrome, and symbiotic psychosis. You report these childhood disintegrative disorders using the ICD-10 code F84.3. As with ICD-9, you will use an additional code under ICD-10 to identify any associated medical condition and intellectual disabilities.

Focus on These Basics

Documentation spotlight: Your psychiatrist will arrive at a diagnosis of Rett’s syndrome based on a complete history and a complete evaluation of the patient. Your psychiatrist will perform a complete mental status examination, a complete psychiatric and medical history of the patient and family, and a review of systems; he or she will also order and interpret diagnostic tests, neurobehavioral tests, and other evaluation questionnaires.

Some of the findings that your clinician would most likely record in a patient with Rett’s syndrome will include lack of communication skills, lack of social interaction, speech abnormalities, autistic-like behavior, sleep disorders, seizures, breathing abnormalities, decelerated growth, stereotypical hand movements (like hand wringing, clapping or hand-to-mouth movements), and poor weight gain.

Tests: If your clinician suspects a diagnosis of Rett’s syndrome, he will ask for genetic tests, chromosomal studies, urinalysis, and other lab tests for serum lactate, amino acids, ammonia, and pyruvate.

In addition, your practitioner will also ask for other tests such as MRI, ECG, EEG, and electroretinography (ERG) to help differentiate the condition from other similar diagnoses and also to help confirm the diagnosis of Rett’s syndrome. Additionally, other tests, such as overnight pH study or barium swallow and polygraphic respiratory recordings will help confirm the diagnosis and rule out other conditions that may mimic Rett’s.

Your clinician might also ask for specialized neuropsychological tests and psychometric assessments, such as the Gilliam autistic rating scales and Children’s autism rating scales, to identify symptoms that are similar to autistic disorder in patients with Rett’s syndrome.

Example: Our psychiatrist recently reviewed a four-year-old female patient who was accompanied by her parents. The parents complained to our clinician that the child was not having normal development of her gross motor skills, and her communication skills also seemed to be not normal for her age. They complained that the child was often listless and would not respond or interact with her siblings or any other family members. They complained that the deterioration appeared to be very drastic over the past month or so, although her development was not the same as her other siblings. They also said that she often appeared to be holding her breath at times and would lose color due to this behavior.

Upon examination, our clinician noted abnormal hand movements (repetitive clapping of hands); lack of eye contact; reduced growth of head circumference; generalized lack of weight gain; lack of communication skills; and irritability.

Since the parents complained of breathing and feeding abnormalities along with lack of normal growth, our psychiatrist suspecting Rett’s sydnrome ordered genetic testing for the MECP2 gene and chromosomal studies for Angelman syndrome (chromosome 15) along with other laboratory tests.

He also ordered for EEG, ECG, EMG, ERG, and MRI tests. He also ordered an overnight pH study to check for gastroesophageal reflux and a polygraphic respiratory recording to test for signs of apnea and hyperventilation.

Our clinician also administered screening tests, such as the Gilliam autism rating scales and Children’s autism rating scales, to study social interaction skills, communication abilities, and stereotypical behavior.

Based on the patient history, assessment of the patient, the evaluation and interpretation of the screening tests, and evaluation of lab and diagnostic tests, our psychiatrist arrived at the diagnosis of Rett’s syndrome.

What to report: You will report the initial diagnostic evaluation that the psychiatrist provided with 90792 (Psychiatric diagnostic evaluation with medical services). Since there were communication difficulties during the evaluation of the child, you may also report +90785 (Interactive complexity [List separately in addition to the code for primary procedure]) with 90792. You report the diagnosis with 330.8 if you’re using ICD-9 codes and F84.2 if you are using ICD-10 codes.