Page 24 - VOL 26 N. 72 - 2015

June 5, 2015 - Auditoire C. Roux
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CHYLOUS REFLUX AND PRIMARY LYMPHEDEMA IN PEDIATRICS
PAPENDIECK C.M., BARBOSA L.
Angiopediatria Foundation, Buenos Aires (AR)
Introduction: Edema resulting from a dysfunction of the lymphatic system, that is because hyperplasia or hypoplasia, segmental agenesia and or 14 different possible dysplasias of the lymphatic system, is a reason of primary lymphedema. These dysfunctions are the result of dysembriogenesis, hamartomatosis and at last, vascular anomalies. One of the causes may be a chylous reflux. Chyle is formed exclusively at the mucosal level of the small gut. Its flow, in pediatrics, is difficult to study with clear images, between the intestinal level and the Pecquet Cystern. Even more when it is associated with a simultaneous free collection of chyle. This condition of the chyle lymph hemi-circuit is a complex issue in pediatrics, it is orphan, but more frequent than expected..We analyze 9 pediatric patients with combined vascular malformations, primary lymphedema and chyloperitoneum and or other collections.
Material: Considered where 9 pediatric patients, age range 2-6 years, with Waldmann´s disease, Klippel Trenaunay syndrome, CLOVEs Syndrome, Proteus Syndrome,, Gorham Stout and Haferkamp Syndrome and Primary Lymphedema alone. In all of them, we chose an inguinal lymph node surgical approach in the context of their treatment with vascular rehabilitation and diet. The identification of a transvascular and or transnodal chylous reflux justified a therapeutical lympho venous anastomosis. An eventual exudative enteropathy loses protein at the mucosal level of the small gut. Or it does not absorb them. This means a positive fecal alpha 1 anti trypsin test (Van de Kamer) hypo proteinemia and in general malnutrition to a important or lesser degree, depending of the extension of the lesion. If there is no chyle formation, no chylous reflux is possible. If chyle is formed and coexists with shyloperitoneum or other collections, it means that there is chylous reflux, backflow or chylous leak. Backflow means that the chyle returns to the place of its formation. Chylo peritoneum or its equivalent in other cavities,in pediatrics, is traumatic, or the result of a rupture of a lympho vascular malformation. Reflux of chyle on retroperitoneal systemic lymph vessels, is a diverted flow of chyle on vessels with a incompetent valvular system, due to hypertension or malformation., and equally through lymph nodes. Interpreting a trans lympho nodal diverted flow is complex. These mechanisms may causes primary lymphedema, with the significance of a primary blockage of the system, or simply due to diverted flow of chyle through systemic lymphatic vessels on the alffected limb. To explain the inverted flux of chyle through a lymph node, is a task. If this occure before the Pecquet Cystern, the reflux is of pure chyle: if the Cystern is blocked, the backflow is with mixed lymph, chyle and systemic lymph. In both cases, a surgical approach is necessary, in the context of complex vascular rehabilitation and specific Diet.. The use of valves (eg Degni, Denver, Le Veen and others) is not possible, because its diameter and it is a transient solution. A specific Diet, cannot be held for a long time, as it would lead to malnutrition, but it immediately restrain the chyle formation...
Conclusion: Chyle refllux or chyle collections because malformations of the lymphatic system is a infrequent disorder in pediatrics. Possible in syndromes with vascular malformations , combined or not. This disorder is a reason to indicate surgery. Among them, resection of the compromised area of the small intestine, and or an inguinal-only lymphovenous shunt in the context of a specific treatment for primary lymphedema, like complex vascular rehabilitation and for a short but necessary time, diet.
CLINICAL MANIFESTATIONS OF LIPEDEMA FROM A PROSPECTIVE COHORT OF PATIENTS
I. FORNER-CORDERO
Spain
Lipedema is a misdiagnosed and poorly investigated disorder with only few publications in this field.
Aim: To describe the prevalence of signs and symptoms of the patients with lipedema to set diagnostic criteria.
Methods: Prospective cohort study of patients with lipedema. Female patients were included if they present with bilateral lower limb (LL) enlargement and at least 3 symptoms of lipedema: spontaneous or provoked pain, easy bruising, family history of lipedema, absence of LL injuries, absence of Stemmer sign, symmetrical LL involvement, no swollen feet; and consent to participate.
Results: We included 93 patients. The median age was 50.7 years (range: 17.8-80.7), the mean age at the onset of symptoms was 21.9 years (+13,5), related more frequently to puberty in 46.2%. The time elapsed until diagnosis was 25.8 years (+15.8).
Body mass Index (BMI) was 31.1 (95%CI: 29.6-32.5); it was normal or underweight in 20.7%. All the patients had bilateral and symmetrical involvement; 86% disproportion with upper part of the body; 90.3% spare feet; 92.5% pain; 88.2% bruising; 82.8% absence of Stemmer sign; 83.9% had vascular spiders; 91.4% were worse at night; pitting was positive in 15.1%. Coldness sensation was reported by 36.8% of the patients. Most of the patients reported a positive family history of lipedema (82.8%).
The most frequent type of lipedema was Type 3 (from hip-to-ankles) 62.4%. The stages of lipedema were well distributed between stages I to III (I: 34.8%; II: 33.7; III: 26.1%). Concerning pain, 37.5% was neuropathic and 55.7% nociceptive somatic; in 51.2% it was mild and in 47.6% moderate. Subjective symptoms were recorded in an Analogic Visual Scale. Pain: 6.0 (95%CI:5.4-6.7); heaviness: 6.6 (95%CI: 6.1-7.1); numbness: 3.2 (95%CI: 2.5-3.8) The disproportion between upper and lower part of the body decreased with the age and BMI: the waist-to-hip index increased with age (coefficient β=0.03; p<0.0001) and BMI (coefficient β=0.03; p=0.007).
Conclusion: Most important manifestations of lipedema were bilateral involvement of LL, pain, spare feet, bruising, and disproportion between upper and lower part of the body. These features could be used as clinical diagnostic criteria of lipedema.
THE EUROPEAN JOURNAL OF LYMPHOLOGY - Vol. XXVI - Nr. 72 - 2015
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