A 21-year-old male presented with elephantiasis in the left lower limb secondary to primary congenital lymphedema due to agenesis of lymphatic vessels in the left hemibody.
It is diagnosed in early childhood due to the observation of the increase of the perimeter of the extremity in relation to the contralateral one, beginning at this moment physical measures for the local control of the pathology.
In puberty, the patient progresses to elephantiasis with important functional limitation that prevents him from walking, which makes it necessary to start antibiotic therapy at least once a year for lymphedema.
In a period of 3 years, the minimum perimeter of 102 cm finally becomes increased, reaching 180 cm in the last year, when there is an increase in the frequency of the episodes of lymphadenitis.
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The radiological evaluation by lymphography showed total absence of lymphatic drainage in the left lower limb and difficulty for lymphatic drainage in the left upper limb.
Computed tomography (CT) shows hypertrophy of the subcutaneous tissue.
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Once the advanced clinical and radiological state of the disease has been confirmed and in the absence of clinical response to conservative measures, it is proposed to perform excisional surgery according to the Charles procedure.
In the first surgical time, we performed a complete excision of the skin and subcutaneous cellular tissue in all its thickness, respecting the muscle fascia, from the ankle to the root of the thigh and covering through self-injectors of the same meshed partial thickness.
After 20 days of hospitalization and cures, we performed a second intervention to cover the residual scattered bloody areas in 3% of total body surface.
We obtained the grafts of the right thigh, which were applied 1:1.5 meshes.
In one week, at 31 days of admission, the patient was discharged with the pre-selected grafts in most cases, remaining residual areas scattered on a surface below that.
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The evolution of the treated area after hospital discharge was very favorable, with resolution of the residual bloody areas in 3 weeks.
We started pre-therapy one month after surgery and physiotherapy was maintained at all times.
However, at 3 months we observed a progressive increase in volume on the dorsum of the left foot, as well as small skin erosions due to friction of the pressotherapy garments with topical cure, which evolved favorably.
Seven months after the first surgery, a new surgical procedure was performed to treat residual lymphedema on the dorsum of the foot.
Similarly, we will perform according to the Charles procedure complete excision of the skin and subcutaneous tissue of the dorsum of the left foot, obtaining partial mesh of the deep fascia, and coverage of the bloody area by means of self-injection 1: thickness.
In 6 days the grafts were preformed and stable and the patient was discharged.
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After 3 years of outpatient follow-up at intervals of approximately 2 months, the erratic appearance of irritating raw areas persists, with non-purulent exudates, which are controlled with topical curing and non-contrast medication.
The patient has a functional limb with dimensions very similar to the contralateral healthy one and without new episodes of lymphangitis to date.
He continues to do pre-therapy, rehabilitation and hygienic measures.
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At 4 years of follow-up, the appearance is very good, the cutaneous manifestation remains unchanged, equal to or even lower than the healthy limb. The quality of the grafts is acceptable, with fibrotic tissue causing chronic fibrosis and irritation.
In the thigh there is no lymphorrhea, so the injected area of the thigh presents a better appearance than the grafts of alopecia.
