Congenital Lymphedema Research Articles

Hereditary Lymphedema in Hereford Cattle

Congenital lymphedema is described in Hereford cattle. The disease was characterized by edema of the hind limbs, sometimes forelimbs, tail, and prepuce. Lymphatic system lesions were hypoplasia and aplasia of lymph vessels and prescapular, iliofemoral, and popliteal lymph nodes. Test matings demonstrated the transmission of the disease by an autosomal dominant trait with variable expressivity and incomplete penetrance.

Cryptococcal Cellulitis in Congenital Lymphedema

A 27-year-old woman with intestinal lymphangiectasia and congenital lymphedema developed cryptococcal cellulitis as a result of her underlying depressed cell-mediated immune responsiveness. Prompt therapy with amphotericin B and 5-fluorocytosine resulted in an excellent outcome. The authors believe that the increasing population of patients with depression of cell-mediated immune response will lead to an increase in the frequency with which cryptococcal cellulitis is seen and emphasize that proper evaluation of a patient with presumptive cellulitis includes consideration of fungal, as well as bacterial, etiology.

Whole-body lymphangioscintigraphy: preferred method for initial assessment of the peripheral lymphatic system.

In 20 patients with congenital and acquired lymphedema in either upper or lower extremities and in four patients without extremity edema, human serum albumin labeled with technetium-99m was injected intradermally into a digital web space of the hand or foot. With a digital gamma camera that permitted a "sweep" of the torso, serial extremity and whole-body lymphagioscintigraphy (LAS) of the peripheral lymphatic system was performed. In 11 patients with acquired lymphedema, a well-defined obstructive pattern was seen, characterized by discrete peripheral lymphatic trunks, delayed or absent depiction of regional nodes, and delayed but extensive soft-tissue tracer extravasation. Five of nine patients with congenital lymphedema showed hypoplasia characterized by poorly defined lymphatic trunks, delayed depiction of regional nodes, and early and extensive extravasation of tracer. The other four patients showed aplasia, with absence of trunks, no depiction of nodes, and little or no tracer extravasation. LAS is technically simple to perform and requires no special training. Radiation exposure is minuscule, and the procedure is safe and without apparent side effects. For these reasons, whole-body LAS should be the preferred method for the initial assessment of congenital or acquired lymphedema.

Role of Tensor Fascia Lata Musculocutaneous Flap in Lymphedema of the Lower Extremity and External Genitalia

The tensor fascia lata (TFL) musculocutaneous flap has been used to cover raw areas resulting after block dissection of fungating cancerous glands in the groin. Postoperative lymphorrhea, a frequent serious problem after groin block dissection, was not seen. It was inferred that the TFL musculocutaneous flap absorbed lymph. This observation was used in treating patients with lymphedema of the lower extremity and genitalia. Groin block dissection was performed in 12 patients with tubercular and filarial lymphedema. By performing a groin block dissection, lymph was allowed to escape in the area of dissection and the TFL musculocutaneous flap absorbed the lymph. As a result a consistent reduction in the size and edema of the affected areas was noted. Wrinkling and suppleness of skin was seen in each patient within the first week. In 1 patient with congenital lymphedema, edema was restricted to the leg and foot. In this patient, groin block dissection was not performed. One end of the TFL musculocutaneous flap was transferred to the calf, and in second stage the proximal end with the whole flap was transferred to the back of the thigh and popliteal fossa. The TFL musculocutaneous flap here acted as a bridge between the area of congenital lymphedema (leg) and the area with normal lymphatic drainage (thigh).

Cutis Verticis Gyrata and Chromosomal Abnormalities

sir .—In the article by Felding and Feingold, 1 published in the March 1988 issue of AJDC , the last sentence seems to imply that there are no chormosomal abnormalities observed in cases of cutis verticis gyrata (CVG). We recently treated a patient with Turner's syndrome (karyotype: 45,X) who exhibited such an anomaly. Patient Report .—The patient was born of two drug-addicted parents and had a birth weight of 2350 g (less than the third percentile), a length of 45 cm (less then the third percentile), Apgar scores of 7 and 8 at 1 and 5 minutes, respectively, and congenital lymphedema. When we saw her at 2½ years of age, her height was 80.7 cm (—2 SDs), she weighed 10.6 kg (third percentile), and her head circumference was 47 cm (tenth percentile). The patient had low-set, posteriorly angulated ears, downward-slanted palpebral fissures, epicanthal folds, a short webbed neck, a low

Angiosarcoma and Late Manifestation of Lymphangiokeratoma in a Patient with Congenital Lymphedema

Angiosarcoma and Late Manifestation of Lymphangiokeratoma in a Patient with Congenital Lymphedema

Congenital lymphedema of the genitalia. (Spanish)

Congenital lymphedema of the genitalia. (Spanish)

Squamous carcinoma of the foot arising in association with longstanding verrucous hyperplasia in a patient with congenital lymphedema

Squamous carcinoma of the foot arising in association with longstanding verrucous hyperplasia in a patient with congenital lymphedema

Pediatric microsurgery.

AbstractThe contemporary status of microsurgical techniques in different pediatric surgical categories is summarized. Employment of microsurgical techniques permits autografts of jejunum to be used for esophageal replacement and vascularized bone grafts to be used for bridging defects in long bones. The same techniques improve overall results in creating vascular access for hemodialysis. Combined with temporary nephrectomy, extracorporeal hypothermic organ preservation, and autotransplantation, microsurgery makes possible repair of injured kidneys, partial nephrectomy for bilateral Wilm's tumor, and correction of renal vascular hypertension.Microsurgical techniques facilitate correction of hypospadia, thereby reducing the complication of fistulas. These methods make possible the reimplantation of an accidentally amputated extremity or a traumatically amputated testicle or penis. Vas deferens injured during herniorrhaphy can be repaired and intra‐abdominal testes can be relocated. Improved drainage for congenital lymphedema of the extremities and varicocele are possible. Remnants of hilar bile ducts can be more accurately identified in children with biliary atresia and may thereby improve the results of Kasai portoenterostomy operations.With the smaller size of organ structures in the pediatric age group, microsurgical techniques play an especially prominent increasing role in this particular population.

Congenital lymphoedema and its complications in pregnancy and the puerperium

Congenital lymphoedema and its complications in pregnancy and the puerperium

The dermal lymphatics in lymphoedema visualized by indirect lymphography.

Iotasul, a new water-soluble contrast medium, is very suitable for subepidermal infusion (indirect lymphography) for the demonstration of small (initial) dermal lymphatics in the macro-range (precollectors). By this method normal lymph vessels can be demonstrated in the healthy skin of hands and feet which are so fine that they cannot be punctured for direct lymphography. We studied thirty-eight patients with various forms of lymphoedema by indirect lymphography. In patients with congenital lymphoedema (particularly Milroy's disease) the initial dermal lymphatics in the lymphoedematous region were absent but large lymph collectors were present. Hyperplasia of dermal lymphatics in the form of an extremely fine net was seen in most patients with lymphoedema praecox and tardum. In some forms of primary lymphoedema the defect may be in the initial dermal lymphatics. In secondary lymphoedema, the changes in the peripheral lymphatics (mainly distension and congestion) are apparently a consequence of the primary lesion in the lymph nodes and large collectors. Indirect lymphography might be useful for the early diagnosis of mild lymphoedema and for excluding patients with hypoplasia of initial lymph vessels from surgery.

Congenital lymphedema of the penis: A method of reconstruction

Congenital lymphedema of the genitalia has profound physical and psychological consequences for the growing child. Extensive resection of this tissue and reconstruction by skin grafting offers a less than satisfactory cosmetic result. Over the past year we have employed a method of total excision of the lymphedematous tissue of the penile shaft with cosmetic reconstruction without skin grafting. A circumferential incision was made 5-10 mm from the coronal sulcus and deepened to the level of Buck's fascia. The skin and subcutaneous tissue were then completely dissected away from the penis. The skin was everted and all of the abnormal lymphedematous tissue excised up to the dermal skin margin. The skin was then tailored to the size of the penile shaft and reapproximated. This method has been employed in two patients with the advantages of (1) shorter hospitalization, (2) lack of morbidity associated with the skin donor site, and (3) satisfactory cosmetic results.

Surgical management of congenital lymphedema in infants and children.

Of 67 children and infants with lymphedema, 28 had the congenital type. Congenital lymphedema appears during the first few weeks of life, frequently involves more than one extremity, and enlarges at a slower rate than general body growth. The swelling usually becomes less pronounced with age, and no specific therapy is required in two thirds of the patients. Seven of the 28 children had swelling of the upper extremities and a generalized lymphangiopathy syndrome. Subcutaneous lymphangiectomy was performed on ten of 28 patients who had moderate to severe swelling. Those with hand and arm involvement were particularly benefited; however, operations on the dorsum of the foot produced hypertrophic scars in one third of the cases. The operation is deferred until after age 2 years to permit optimal technical repair and to identify those patients whose conditions will improve spontaneously.

A syndrome of congenital lymphedema of the upper extremity and associated systemic lymphatic malformations: Eric W. Fonkalsrud. Surg Gynecol Obstet 145:228–234 (August), 1977

A syndrome of congenital lymphedema of the upper extremity and associated systemic lymphatic malformations: Eric W. Fonkalsrud. Surg Gynecol Obstet 145:228–234 (August), 1977

Surgical Treatment of Lymphedema — A Reappraisal

The clinical picture of lymphedema and its classification have been briefly reviewed with a discussion of surgical treatment both historical and current. It has been pointed out that the primary mode of treatment of congenital or secondary lymphedema of the extremities is intensive medical therapy and that surgical intervention is warranted in only rare and unusual circumstances. The gamut of surgical procedures attempted have been reviewed and it is this author's contention that the only procedure of limited, worthwhile value is a combination of the Kondoleon and the Thompson procedures. The Kondoleon phase of the procedure debulks the extremity in stages and removes the deep fascia. The Thompson form of the procedure advances a deepithelialized dermal flap into the deep lymphatical venous system. It has not been conclusively proven whether this dermal flap is of significant physiologic benefit, but I feel it is worth adding this to the Kondoleon procedure so that in time, we will have an opportunity to evaluate the value of this phase of the procedure.

LYMPHEDEMA

In a retrospective study of primary lymphedema, we found that the age of onset ranged from 0 to 16 years; sex did not appear to be a factor; and treatment was preventive rather than surgical. Thirteen patients were available for follow-up studies, the time interval being at least 6 years. The degree of swelling at the time of the follow-up examination was defined as mild, moderate, or severe. In patients with mild and moderate swelling the edema was not progressive and surgical treatment was not indicated. Severe swelling appeared to be associated with congenital lymphedema and with repeated infection. Pressure-support treatment can be effective in the reduction of swelling or in the prevention of further swelling, if applied constantly over a long period of time. The authors discuss the histological similarity between lymphedema and lymphagioma, and present an anatomical classification for the two entities.

Lymphangiosarcoma Arising in Congenital Lymphedema

An 85-year-old woman with congenital lymphedema of the right upper extremity developed a small purplish papule on the forearm, which was the first clinical evidence of lymphangiosarcoma. The lesion grew rapidly and became necrotic and ulcerated. The patient experienced severe pain. A mid-arm amputation was performed. Microscopical examination of the amputated limb showed widespread histological involvement of skin, muscle, and subcutaneous tissue by lymphangiosarcoma. The need for continuous monitoring of patients with both primary and secondary lymphedema is emphasized, since early diagnosis of the developing lymphangiosarcoma and rapid surgical intervention provide the best prognosis for survival.

Lymphangiosarcoma and Congenital Lymphedema of the Extremity

Lymphangiosarcoma is known to be associated with chronic lymphedema of an extremity, especially when it is secondary to radical mastectomy. Only rarely has this malignant neoplasm been secondary to chronic lymphedema without the previous occurrence of a malignant neoplasm. Our patient had lymphangiosarcoma secondary to congenital lymphedema, which is a very rare occurrence. Clinical, microscopic, histochemical, and ultrastructural studies were performed. Multiple early biopsy specimens of this patient's lesion showed no definite neoplasm. If an area of ulceration occurs within a chronically lymphedematous extremity, multiple deep biopsies should be performed; one or two normal biopsy findings do not rule out the presence of malignant disease. The prognosis in this malignant condition is poor, but the best chance of long-term survival is prompt diagnosis and radical surgery.

Congenital lymphoedema (Milroy's disease). A report of 10 cases in a family.

Ten cases of congenital and hereditary lymphoedema (Milroy’s disease) in a family of 19 members in 5 generations have been described. The literature is briefly reviewed.

Surgical management of congenital malformations of the lymphatic system

Lymphangiomas, cystic hygromas, lymphan-giectasis, and congenital lymphedema are believed to represent different forms of congenital lymphatic obstructions. Two or more of these anomalies may be present in the same patient. Eightynine children with congenital malformations of the lymphatic system have been treated at the UCLA Hospital during the past fifteen years. Spontaneous regression of lymphatic malformations is uncommon, and growth is likely to occur in proportion to general body growth. Malignant degeneration is very uncommon. Operative management is recommended to remove the bulk of the lesion in most children and is most successful in localized hygromas and lymphedema. Repeated resection may be necessary to control lymphangiomas. Partial resection and diuretics have been of some value in treating intestinal lymphangiectasis.