2025 ICD-10-CM code Q87.4
(Active) Effective Date: N/A Revision Date: N/A Congenital malformations - Other specified congenital malformation syndromes affecting multiple systems Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99) Feed
Marfan syndrome is a genetic disorder affecting connective tissue.
Medical necessity for procedures and monitoring related to Marfan syndrome is established by the presence of the condition and the associated risk of life-threatening complications, primarily those affecting the cardiovascular system.Regular echocardiograms and other cardiovascular imaging studies are essential for early detection and management of aortic dilation and other cardiovascular problems.
Diagnosis and management of Marfan syndrome involves genetic counseling, regular cardiovascular monitoring (including echocardiograms and aortic imaging), ophthalmological evaluations, and management of associated symptoms.A multidisciplinary approach, including cardiologists, ophthalmologists, and geneticists, is often necessary.
- Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- Q87 Other specified congenital malformation syndromes affecting multiple systems
In simple words: Marfan syndrome is a genetic condition that affects how the body grows. People with this condition tend to be tall and have long limbs.It can also cause problems with their eyes and heart.Doctors regularly monitor individuals with Marfan syndrome for heart complications.
Marfan syndrome (Q87.4) is an inherited connective tissue disorder characterized by abnormalities in the skeleton, eyes, and cardiovascular system.Skeletal features include long limbs, tall stature, and joint hypermobility. Ocular manifestations can include ectopia lentis (lens dislocation). Cardiovascular involvement is a significant concern and may include aortic dilation or dissection, mitral valve prolapse, and other heart abnormalities.The severity and specific manifestations vary widely among individuals.
Example 1: A 20-year-old patient presents with tall stature, long fingers, and a family history of Marfan syndrome.Echocardiography reveals mild aortic dilation.The diagnosis of Marfan syndrome (Q87.4) is made, and regular cardiac monitoring is recommended., A 5-year-old child is diagnosed with ectopia lentis (lens dislocation) and skeletal abnormalities consistent with Marfan syndrome.Further evaluation is needed to assess for cardiovascular involvement., A 40-year-old patient experiences sudden chest pain.Imaging studies reveal an acute aortic dissection. The patient's medical history includes a diagnosis of Marfan syndrome (Q87.4) in childhood, and the aortic dissection is directly attributed to this underlying condition.
Detailed family history, physical examination findings (including anthropometric measurements, skeletal features, and ophthalmological examination), echocardiography, and genetic testing (if available) are crucial for accurate diagnosis and management of Marfan syndrome.Documentation should clearly describe all associated manifestations.
** Marfan syndrome is a complex disorder with a wide spectrum of severity.Accurate coding requires careful documentation of all associated findings to ensure appropriate reimbursement and patient care.
- Payment Status: Active
- Specialties:Cardiology, Genetics, Ophthalmology
- Place of Service:Office, Hospital (Inpatient and Outpatient), Genetics Clinic