DISCUSSION

The Philippine Heart Center is a specialized hospital that caters to cardiovascular patients, one of its department is vascular medicine within it is the lymphedema clinic. Our vascular adult cardiologists serve as the lymphedema specialist. The diagnosis is primarily made based on history and physical examination. Typical clinical features such as persistent edema of more than three months, minimal response to elevation or medications, presence of skin changes, and asymmetric limb measurements are sought. ³ Additional imaging of lymphoscintigraphy is warranted to diagnose in the early stage of the disease or if it is unresponsive to treatment. A deep venous thrombosis screening using ultrasound is also done to exclude its presence.¹ The average age of patients seen belongs to the elderly group (60.3) years old, with a female predominance. The positive association for age and increase incidence in women is probably due to the comorbidities or diseases that linked or have caused the lymphedema.⁵ Most patients are classified under secondary since it is composed of more common diseases compared to primary which are due to some rare genetic or syndromic entities.⁵ The stage of severity seen is mostly from moderate to severe (IIA-III) and only a few were mild (I). This is probably due to the symptoms, physical and psychological turmoil experienced by the patients that led to consultation and treatment.¹⁰ The most affected site is bilateral lower extremities which is the same with other studies,^{8 , 9} a higher gravitational pull may be contributory to lymphedema and further stasis.²¹ A unilateral affection of the left and right legs compared to arms shows only a slight difference. The affectation of the arms is less prevalent probably due to a greater number of lymph nodes, preferential usage, and its proximity to the thoracic duct for drainage. Also, no bilateral upper extremity affectation was documented. Our lymphatic therapist mostly performed unilaterally over bilateral decompressive lymphatic therapy depending on the localization. A DLT is the standard of care and further treatment is based on the outcome and recurrence of lymphedema. Monitoring of outcome can be measured using a circumferential tape measure and devices such as tonometry and bioimpedance.²⁵ The cost incurred was based on the number of sessions and the type of treatment rendered. A unilateral DLT cost about P2,200 while a bilateral DLT a little higher P3,500. The cost does not include the transportation fee, nor the additional treatment cost of the primary disease or the complications of lymphedema such as recurrent skin infections.¹⁰ However certain discounts are made for senior and service patients. A total of P1,722,000 with an average of P17,753 per patient was incurred. The referral of patients mainly came from the private compared to the service section. The lack of awareness and exposure from the cardiology fellow in training and other physicians may be a contributing factor to this observation aside from the affordability of the treatment.

The patients with primary lymphedema included only a total of 8. It affects an estimated 1 in 100,000 people.⁵ The average age of patients seen belongs to the young adult 38.5 years old with no gender predilection.⁵ The diagnosis is suspected when the onset of edema occurs at a young age with no apparent cause. A culprit genetic mutation is usually not investigated, due to a lack of resources in our setting. A family history together with possible physical examination pointing to a syndrome provides valuable clues. The lymphoscintigraphy showing lymphatic hypoplasia with no or delayed lymph drainage confirms the diagnosis. Two patients were diagnosed with congenital lymphedema or Milroy’s disease. It is a rare autosomal dominant condition presenting with usually unilateral lower extremity lymphedema, up slanting toenails, papilloma, and prominent leg veins. ¹⁶ Five patients or 62.5% of primary lymphedema were diagnosed with praecox or Meigs disease. It is the most common form of primary lymphedema accounting for 65-80% of cases.⁵ It usually presents as unilateral leg swelling and females are more affected suggesting a hormonal effect on its pathogenesis.¹⁷ Among the five one is diagnosed with Klippel-Trenaunay syndrome. It is diagnosed clinically with the presence of port wine stains, unilateral leg hypertrophy of the bone, and soft tissue.¹⁸ Only one was diagnosed with lymphedema tarda presenting with painless, progressive swelling of the right leg during his 4th decade of life, similar to a case report.¹⁹ The cost of treatment incurred was an average of P10,100 the lowest among all groups. This observation may be due to the improvement of lymphatic drainage over time seen in some of the young patients as reported in a case report.²⁰

The majority of patients seen have secondary lymphedema. The average age belongs to the elderly group with a female predominance. The gender predilection can be explained with the association of the etiology of secondary lymphedema. The most common is cancer-related secondary to breast and gynecologic malignancies. It occurs due to radical surgery, node dissection, and radiotherapy as observed with other cancer treatment complications of prostate, colon, skin, and liposarcoma.⁵ The most studied is breast cancer in which a cumulative incidence of 13.5% after two years, 30.2% after five years, and 41.1% after 10 years post-treatment are observed.²¹ The second most common is phlebolymphedema, an institutional bias that may be at play since chronic venous insufficiency (CVI) is a common disease treated by a vascular adult cardiologist. In CVI a loss of valvular function of the lower extremities may result from age-related, occult, or previous deep venous thrombosis (DVT) and surgery. The incidence is approximately 6% to 7% at 50 years of age, 20% at age 70 with a slight female preponderance possibly due to estrogen effect. It has the highest average cost incurred at P23,032 among all groups which are probably due to factors aggravating the condition such as heart failure, cirrhosis, or nephropathy.²²

Although lymphatic filariasis is the most common cause of secondary lymphedema in the world⁵ only one out three infectious related was seen since it is not endemic in our region²³ the remaining were due to bacterial lymphangitis. Minor etiologies with no known general incidence except for few observational studies are lipolymohedema it is an abnormal asymmetric fat distribution that leads to lymphedema if left untreated. It almost exclusively occurs in females during puberty or pregnancy.²⁴ Obesity-induced due to lymphatic dysfunction is said to occur when the BMI threshold reaches between 53-59.¹⁶ Lastly, one iatrogenic cause occurred due to the injection of collagen in the buttocks and thighs for an aesthetic purpose which seldomly occurs. In terms of financial burden countries from the US and Europe assists thru statutory health insurances and takes into account direct and indirect costs.^{26, 27} In our institution, patients use their out-of-pocket money and grants obtained from the government.

CONCLUSION

Lymphedema is a chronic and recurrent disease that carries a substantial cost to patients. Its exact incidence, treatment outcomes, and quality of life among Filipinos are yet to be explored. A clinical profile will be the first step to raise awareness and to identify the different subset of populations for prevention, support, and treatment.