TN 8 (11-12)
DI 23022.939 Congenital Lymphedema
COMPASSIONATE ALLOWANCE INFORMATION
Congenital Hereditary Lymphedema; Primary Lymphedema; Congenital Primary Lymphedema; Milroy disease
Lymphedema is swelling that is caused by the abnormal accumulation of lymph fluid in tissues, resulting from malfunctions in the lymphatic system or blockage of lymph vessels. Congenital Lymphedema is a rare type of primary lymphedema (non-acquired) occurring at birth. In most cases, lymphedema primarily affects the lower limbs (starting with the feet) but it can also affect the upper limbs. This disorder is characterized by lymphatic pathway dysplasia (abnormal development of the lymphatic vessels that transports lymphatic fluid), and may be associated with aplasia (congenital absence of a limb, organ or other body part) or hypoplasia (incomplete development of an organ or part). The exact cause of congenital lymphedema is unknown, but the disorder may be part of a congenital syndrome. Females are twice as likely as males to have congenital lymphedema.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: Biopsy reports documenting lymphatic channel anaplasia; CT, MRI, or nuclear medicine scans; Doppler ultrasonography is used to evaluate flow in the lymphatic and venous systems.
Physical findings: Heavy, swollen lower or upper limbs that may be shorter than unaffected limbs, or may have digital anomalies; Skin changes such as tightness, thickening, discoloration or hardening; there may be signs of infection.
ONSET AND PROGRESSION
This disorder occurs at birth or soon after and is most often bilateral. In some cases, the lymphedema may spontaneously improve. Other syndromic types of lymphedema have later onset, most often during puberty.
There is no cure for lymphedema. Treatment is symptomatic, and surgery is considered to be palliative. Infections are a major risk and require prompt initiation of antibiotics and wound care. Persons with lymphedema are treated with compression bandages and manual lymph drainage massage techniques to move the extra fluid to other parts of the body so that the body can excrete it.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation:
Suggested Listings for Evaluation:
101.02 A or B
Listing level severity must be documented.
Continuing surgical management must be documented.
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.