Hereditary lymphedema
ICD-10 Q82.0 is a billable code used to indicate a diagnosis of hereditary lymphedema.
Hereditary lymphedema is a congenital condition characterized by the abnormal accumulation of lymphatic fluid due to malformations in the lymphatic system. This condition can manifest at birth or develop later in life, often affecting the limbs, but can also involve other areas of the body. The most common form, Milroy disease, is inherited in an autosomal dominant pattern and is caused by mutations in the FLT4 gene, which plays a crucial role in lymphatic vessel development. Clinically, patients may present with swelling, recurrent infections, and skin changes such as thickening or hyperpigmentation. Diagnosis is typically made through clinical evaluation, family history, and imaging studies to assess lymphatic function. Management may include compression therapy, physical therapy, and in some cases, surgical intervention to improve lymphatic drainage. Understanding the genetic basis and clinical implications of hereditary lymphedema is essential for effective treatment and counseling.
Pediatric documentation should include growth patterns, developmental milestones, and specific symptoms related to lymphedema.
A pediatric patient presenting with limb swelling and recurrent infections, requiring evaluation for hereditary lymphedema.
Consideration of the psychosocial impact on the child and family, as well as the need for multidisciplinary care.
Genetic documentation must include details of genetic testing, inheritance patterns, and family pedigree.
A family seeking genetic counseling after a diagnosis of hereditary lymphedema in a child, discussing risks for future pregnancies.
Understanding the implications of genetic mutations and the need for ongoing surveillance for associated conditions.
Used in conjunction with diagnosis of hereditary lymphedema to assess lymphatic function.
Document indications for lymphatic mapping and results.
Pediatric specialists may need to coordinate with surgical teams for comprehensive care.
Genetic testing is crucial for confirming the diagnosis of hereditary lymphedema, understanding the inheritance pattern, and guiding treatment options. It also helps in family planning and counseling for affected families.
