Program Operations Manual System (POMS)
TN 62 (10-23)
COMPASSIONATE ALLOWANCES INFORMATION
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CONGENITAL LYMPHEDEMA
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ALTERNATE NAMES
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Congenital Hereditary Lymphedema; Congenital Primary Lymphedema; Milroy Disease; Primary
Lymphedema;
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DESCRIPTION
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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. 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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing:
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Biopsy reports documenting lymphatic channel anaplasia (loss of the mature or specialized
features of a cell or tissue);
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CT, MRI, or nuclear medicine scans; and
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Doppler ultrasonography is used to evaluate flow in the lymphatic and venous systems.
Physical findings: Physical findings of this condition may include:
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Lymphatic pathway dysplasia (abnormal development of the lymphatic vessels that transports
lymphatic fluid);
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Aplasia (congenital absence of a limb, organ or other body part);
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Hypoplasia (incomplete development of an organ or part);
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Heavy, swollen lower or upper limbs that may be shorter than unaffected limbs, or
may have digital anomalies; and
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Skin changes such as tightness, thickening, discoloration, or hardening; there may
be signs of infection.
ICD-9:
757.0
ICD-10: Q82.0
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Doppler ultrasound reports documenting abnormal flow in lymphatic and venous systems;
and
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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101.18
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Listing level severity must be documented.
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Equals
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101.21
108.09
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Listing level severity must be documented.
Chronic conditions of the skin or mucous membranes.
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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