Topical Tacrolimus 0.1% for treatment of cutaneous microcystic lymphatic malformations.

Microcystic lymphatic malformations as described in the international literature form a subgroup of low-flow congenital vascular malformations (VM) resulting from irregular embryological development. Microcystic lesions normally manifest as an accumulation of lymph- and blood-filled vesicles that, when externalized, cause skin maceration with consequent pain and potential infection resulting in the impairment of the patient's quality of life. There is no consensus on a standardized algorithm nor clear guidelines for successful treatment of this type of lymphatic malformation, and treatment options employed often result in ambivalent and transient outcomes with a high rate of recurrence. The topical formulation of tacrolimus is a well-known FDAapproved anti-T cell agent that was recently identified as a potent activator of ALK1, which is involved in several processes and functions including angiogenesis. We investigated if topical administration of tacrolimus may be an effective therapy for directly targeting cutaneous microcystic lymphatic malformations as a complement to systemic treatment. The study enrolled four patients with cutaneous microcystic lymphatic malformations: three male (ages: 13,15,18) and one female (age: 30). Two of the patients presented lesions on their backs, one patient on the left hand and one on the left lower limb. All four patients received treatment with topical tacrolimus 0.1% twice a day for 10 weeks on a previously selected area for application. Weekly clinical follow-ups were conducted along with close physician-patient contact. All patients displayed a satisfactory response after treatment. Lymphorrhea and bleeding were stopped in all cases and the esthetic aspect of lesions improved in two patients. To date, all patients presented no clinically significant changes to the size or extension of the lesion. Topical tacrolimus treatment is a promising and reasonable option for microcystic lymphatic malformations. Our results encourage further exploration in larger populations with the consideration that it is a safe and effective alternative or complementary therapy to systemic treatment.

Imaging of lymphatic dysplasia in Noonan syndrome: Case studies and historical atlas.

To determine the historical use and utility of various lymphatic imaging modalities in Noonan syndrome (NS) patients, we performed a comprehensive literature review by collecting the published medical imaging of NS lymphatic dysplasias. We correlated imaging findings with clinical phenotypes and treatment. Our analysis of lymphatic imaging modalities provides an algorithmic approach to imaging and patient care across the spectrum of NS developmental defects. A total of 54 NS cases have been published since 1975. Using the observations reported in 15 reviewed publications, an association was made between disruptions in central lymphatic flow and poor clinical presentations/outcomes in NS patients.

In Memoriam Prof. Prof. h.c. Dr. med. Michael Földi, January 10, 1920 - October 20, 2018 "Father of Lymphology".

In Memoriam: With deep sadness the world of Lymphology learned of the death of Prof. Prof. h.c. Dr. med. Michael Földi, a ground breaking pioneer of modern Lymphology. Words alone will never fully describe or capture the breadth and depth of Michael's contribution to our lymphatic knowledge and the legacy he has left for us all.

A new combined operative technique using crossed inguinal lymphatic rescue for pediatric patients with mixed lymphatic and venous malformations.

Truncular venous malformations and acquired functional or anatomical venous occlusions (or sub-occlusions) can be the cause of secondary lymphedema and even the cause of primary lymphedema when they are associated with lymphatic malformations (lymphangiodysplasia - LAD I, lymphadenodysplasia - LAD II, or a combination of both) in pediatric patients. This understanding recognizes the shared and successive embryogenesis of both systems. These conditions can exhibit hypertension in the venous pedicles intended for lymph-venous anastomosis, and this finding would be a formal contraindication to the procedure. However, this hypertension is a rarely considered condition and is not commonly identified. As a technique to solve this problem, we have combined Nielubowicz, Olszewski, Campisi, and Palma's proposals and created a lymph-venous anastomosis from the side with lymphedema and venous hypertension (lymphatic donor and venous recipient) with an internal suprapubic saphenous venous bridge (from the normal side to the lymphedematous side with venous hypertension) to enable a crossed inguinal lymphatic/venous rescue. We believe this newly synthesized approach will allow better clinical care of pediatric patients with complex and combined lymphatic-venous malformations and is worthy of further investigation.

A salvage surgical solution for recurrent lymphangioma of the prepuce.

Treatment of lymphangioma circumscriptum of the prepuce is often technically demanding. Many different medical and surgical approaches have been described over the years, none of which has proved completely successful. We describe a case in which skin deficiency was a problem after prior multiple excisions of lymphangiomatous tissue. We decided to bury the degloved shaft in the scrotum. After 6 months the penis was raised and reconstructed using scrotal skin; this achieved satisfactory skin coverage. In such cases piliferous follicles can easily be dealt with at a later stage by cosmetic treatment. We believe this technical approach may be a valid alternative to conventional procedures (i.e. grafts and flaps) especially when extensive resection makes skin coverage particularly difficult.

Lipoblastoma-lipoblastomatosis associated with unilateral limb hypertrophy: a case report in a newborn.

Lipoblastoma is a benign mesenchymal tumor of embryonic adipose tissue, uncommon in infancy. Multicentricity, absence of a capsule and histopathology best defines a lipoblastoma. Synonyms for this lesion are embryonic lipoma, fetal lipoma, lipoblastic tumor, and congenital lipomatoid tumor. Lipoblastoma is more common in males (approximately 80%), is usually located in the subcutaneous soft tissue (benign lipoblastoma) or in the deep interstitium (benign lipoblastomatosis), or sometimes in all corporeal segments. Primary treatment is complete excision. Relapse is between 14-25%, many of which are more "mature," and difficult to differentiate from lipoma. The differential diagnosis includes liposarcoma, which is rare under ten years. Radical excision in children is recommended with relapses, especially with lipoblastomatosis. Chromosomal markers help discriminate between liposarcoma and lipoblastoma.

Klippel-Trenaunay-Servelle syndrome in pediatrics.

The aim of this report is to explain the historical differences between Klippel-Trenaunay (KT), Klippel-Trenaunay-Weber (KTW), and Klippel-Trenaunay-Servelle (KTS) syndromes. Furthermore, an attempt will be made to describe the different causes, symptoms, and consequences of the pathology, largely as a consequence of venous hypertension. The significance of these syndromes within the pediatric population is highlighted.

Lymphatic dysplasia in paediatrics. A new classification.

A classification of dysplasias of the lymphatic system is proposed on the basis of the anatomical structures involved: lymph vessel-angiodysplasia, lymphadeno(nodal)-dysplasia and the summary of both lymphangio-adeno(nodal)dysplasias. The suggested terminology is: LAD I (Lymphangiodysplasias), LAD II (Lymphadeno(nodal)dysplasia) and LAAD (lymphadeno(nodal)-dysplasia). With this classification we may coherently group the malformations we already know, relate them to the big syndromes and to all the other angiodysplasias and also create the reference background for another chapter of lymphology: primary or idiopathic lymphedema.

Hodgkin's disease in childhood: therapy results in Argentina.

From 1940 to 1977, a total of 152 children aged 15 years or less with the histologic diagnosis of Hodgkin's disease were treated in Argentina. For analysis, these patients were placed into three periods which displayed the most outstanding changes in diagnostic workup and therapy. The periods are as follows: (1) 1940 to 1966 (43 children), (2) 1967 to 1972 (35 children), and (3) 1973 to 1977 (74 children). The patients were treated with extended field radiation therapy and followed by courses of cyclophosphamide-vinblastine-procarbazine-prednisone, with CCNU added (CCVPP) or not added (CVPP) in a randomized trial. The mean age of the whole group was 7.5 years (range 2 to 15 years) and there was a predominance of males (79%). Crude 6-year survival for the three periods were as follows: 1940 to 1966, 34%; 1967 to 1972, 50%; and 1973 to 1977, 79%. We conclude that (1) survival of Hodgkin's disease of childhood has shown a rather marked improvement during the last decade and this progress is probably due to the use of combined multidrug chemotherapy administered under collaborative controlled clinical trials; (2) preliminary evaluation of the results of CVPP and CCVPP therapy shows that the latter combination (CCVPP) neither increases the percent of patients achieving complete remission nor prolongs relapse-survival in Hodgkin's disease of childhood: and (3) all stages of childhood Hodgkin's disease can be successfully managed with multidrug chemotherapy alone.