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Saturday, May 27th, 2017
SESSION VI
Chairmen: M. Oberlin, J. Rößler, K. Johansson
INNOVATIVE THERAPIES FOR CHILDREN WITH LYMPHATIC MALFORMATIONS
JOCHEN RÖSSLER
Medical Center - University Freiburg, Center of Pediatrics and Adolescent Medicine, Germany
Lymphatic malformations (LM) are inborn abnormalities frequently diagnosed in the paediatric age group. Recently, somatic mutations in PI3KCA, a molecule of the mTOR signalling pathway have been identified in LM.
As mTOR inhibitors are available since several years for immunosuppressive therapy, the use of these drugs could now be an innovative approach for complicated LM that are not accessible to standard therapy such as sclerotherapy, surgery and conservative measures. Furthermore, complications of LM could be managed by this new strategy.
Lymph leakage is a severe complication of LM that can lead to protein loss and infections. We report on six patients that were treated with the mTOR inhibitor Sirolimus as an individual “ultima ratio” therapeutic approach. The patients had multiple pre-treatments such as surgery, laser- and sclerotherapy. First lymph leakage episodes started at the age of 2 months, 8 years, 12 years (2) and 14 years (2). Sirolimus was effective after one day (1), ten days (1), four weeks (4). Importantly, no toxicities were observed. Five patients are still on sirolimus for up to 3 years.
PROTOCOL OF LYMPHOEDEMA PREVENTION AFTER BREAST CANCER 3 YEARS OF FOLLOW-UP
M. CESTARI, S. AMATI, F. APPETECCHI, L. CURTI, M. DE MARCHI, DE REBOTTI, C. TOMASSI
O.U. Territorial Rehabilitation Domus Gratiae Center - NHS Umbria2 - Terni, Italy
Introduction: Four years ago, in the authors’ laboratory of lymphology, it was decided to focus the attention on patients without clinical evidence of oedema, at risk of developing lymphoedema in the homolateral arm after breast cancer treatment.
The authors planned a preventive protocol that highlighted the importance of primary prevention in order to avoid lymphoedema onset.
Materials and Methods: After join lymphologist and physiotherapist assessment consisting of clinical evaluation, a centimetrical/volumetrical measurement of compared arms, a lymphoscintigraphy request, a shoulder functionality evaluation, through Constant-Murley Shoulder Score, and BMI evaluation, patients were included in five different groups after the start-up of a rehabilitative project:
-Informative Group, which includes all patients (20 patients at a time), whose end-point is the information on lymphoedema and the preventive behavioural rules;
-Individual Rehabilitation Treatment which includes patients with limited shoulder functionality (Costant-Murley Shoulder Score: range of motion equal or less to 28 out of 40 points) who undergo specific treatment after physiatrician and physiotherapist assessment. After improvement the patient is included in the Physical Activity Group.
-Individual Lymphological Section which includes patients with positive lymphoscintigraphy exam (slower radiotracer flow with initial dermal back-flow) and which consists of the review of preventive behavioural rules and a prescription of standard flat sleeve for housework. After this Section the patient is included in the Physical Activity Group.
-Individual post-surgery problems treatment (breast oedema, scar with or without oedema, axillary web syndrome, mobilization of prosthesis). After the treatment the patient is included in the Physical Activity Group.
-Physical Activity Group (10 patients at a time), which includes a physiotherapist, and patients without limited shoulder functionality or with a range more than or equal to 30 out of 40 points as well as all patients from individual treatments. This Physical Activity Group consists of gymnastics, with mobilisation of all physical districts, breathing exercises, relaxation technique, with music-therapy, and stretching exercises; furthermore, the physiotherapist highlights the importance of physical activity, based upon the patients predisposition and problems, and patients confront their problems with physiotherapist and/or each other.
In cases of slower radiotracer flow, a follow-up of 6 months was required instead of 12 months in cases of normal lymphoscintigraphy.
Results: The preventive protocol has highlighted its usefulness in order to prevent lymphedema onset (9% of patients after 3 years of follow-up) and the physical-psychological well-being achieved through the early and holistic care.
Conclusions: In the authors’ laboratory of lymphology a protocol of lymphoedema prevention after breast cancer was planned, useful, in their opinion to prevent lymphoedema onset through the compliance of the patients, who must cooperate actively, and a rehabilitative team with early and holistic approach.
THE EUROPEAN JOURNAL OF LYMPHOLOGY - Vol. XXIX - Nr. 76 - 2017
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