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2025 ICD-10-CM code F84.2

Rett syndrome is a rare neurodevelopmental disorder primarily affecting girls, characterized by developmental regression, loss of motor skills and speech, and distinctive hand movements.

Coding guidelines for Rett Syndrome require accurate documentation supporting the diagnosis based on clinical findings and genetic testing.The code should reflect the patient's current clinical status.

Modifiers may be applied to procedural codes used in the management of Rett syndrome based on the circumstances of service delivery. Consult the appropriate coding guidelines and payer policies for specific modifier rules.

Medical necessity for services related to Rett syndrome is supported by the presence of diagnostic criteria, the severity of symptoms impacting daily functioning, and the need for interventions to improve quality of life and manage complications. Documentation should demonstrate the need for specific services based on the patient's clinical presentation and response to treatment.

The clinical responsibility for Rett syndrome management involves a multidisciplinary approach including pediatricians, neurologists, geneticists, therapists (physical, occupational, speech), and other specialists as needed to address the various symptoms and complications.

IMPORTANT:No alternate codes are specifically mentioned in the provided data, however,consideration should be given to related codes if other symptoms are present (e.g., codes for autism spectrum disorder, intellectual disability, or seizure disorders).

In simple words: Rett syndrome is a rare brain disorder mostly affecting girls.Babies seem normal at first, but later lose skills like talking and using their hands. They may develop repetitive hand movements and have trouble walking. There's no cure, but treatments help manage symptoms.

Rett syndrome (RTT) is a rare, severe, X-linked neurodevelopmental disorder predominantly affecting females.It's characterized by an initial period of seemingly normal development (6-18 months), followed by a rapid regression in language and motor abilities. This regression includes a partial or complete loss of purposeful hand skills, loss of spoken language, gait abnormalities, and the development of stereotypic hand movements (e.g., hand-wringing, clapping, tapping).Other common features include deceleration of head growth, severe intellectual disability, seizures (60-80%), breathing abnormalities (apnea, hyperventilation), autistic features, and gastrointestinal problems.The disorder progresses through distinct stages: early onset, rapid deterioration, plateau, and late motor deterioration, with significant variability in symptom severity and progression rate. The etiology is primarily linked to pathogenic variants in the MECP2 gene (methyl CpG-binding protein 2) on the X chromosome. Diagnosis is based on clinical criteria and genetic testing to identify MECP2 mutations.

Example 1: A 2-year-old girl presents with developmental regression, loss of purposeful hand use, and the onset of hand-wringing.Genetic testing confirms an MECP2 mutation, leading to a diagnosis of Rett syndrome.Treatment involves physical and occupational therapy, speech therapy, and monitoring for seizures., A 10-year-old girl with Rett syndrome experiences increased irritability and breathing difficulties.Her care team adjusts her medication regimen and implements strategies to manage her respiratory symptoms., An adult woman with Rett syndrome requires ongoing support with activities of daily living (ADLs), including assistance with feeding and mobility.She also receives regular assessments to monitor her condition and address any emerging needs.

Detailed developmental history, including milestones achieved and lost; description of hand movements; assessment of communication and language skills; documentation of gait abnormalities; neurological examination findings; genetic test results;results of any other relevant investigations (e.g., EEG, MRI);treatment plan and progress notes.

** The severity and progression of Rett syndrome vary significantly among individuals.Comprehensive documentation is crucial for accurate coding and reimbursement.Always refer to the latest ICD-10-CM coding guidelines and payer-specific requirements.

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