Meige Trofoedema: A form of primary lymphedema

The lymphatic system consists of small non-contractile lymph vessels which collect fl uid from the interstitial space and carry it to the major contractile lymphatic vessels with valves that maintain a unidirectional fl ow to the lymph nodes and is responsible for returning proteins, lipids and water from the interstitium to the venous system near the junction of the subclavian vein and internal jugular vein on both sides. Lymphedema would be the result of the failure of transport with the further accumulation of fl uid rich in protein at the interstitium, especially in limbs. Primary lymphedema, which affects 1.15 / 100000 children (especially pre-pubescent girls), not always clinically evident at birth, is a genetic disorder that represents many different subsets of phenotypes: congenital or congenital lymphangiectasia, congenital trofoedema family or Meige, congenital amniotic band and essence or idiopathic The most common form of presentation is due to congenital absence or abnormality of the lymphatic tissue caused by a genetic mutation of the genes responsible for the development of lymphatic vessels that is characterized by the diffi culty of draining lymph vessels. This genetic alteration may be sporadic or inherited. Case Report Meige Trofoedema: A form of primary lymphedema Carlos Al Sanchez Salguero* Professor of Pediatric, Paediatric Department, Medicine School of Cádiz University Hospital, Puerto Real, Spain *Address for Correspondence: Carlos Al Sanchez Salguero, Professor of Pediatric, Paediatric Department, Medicine School of Cádiz University Hospital ,Puerto Real, Spain, Tel: 0034956480912; Email: libraygeminis@hotmail.com Submitted: 12 April 2018 Approved: 23 April 2018 Published: 24 April 2018 Copyright: 2018 Salguero CAS. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited


Case Report
We report the case of a 10-year-old male patient was admitted to the Pediatric Service of our Hospital to present a picture of edema of the right foot 3 months and had not referred pain, functional disability or impairment of ambulation, and fever, initially disappearing with rest and worsens with exercise to become permanent in recent weeks. Had been previously assessed by the trauma that ruled bone disease and disorders of the talar joint.
On arrival at our department with a scan weight 46 kg (P90-97), height 140 cm. (P 50) with a Body Mass Index of 23.47 (P 95-97). Good condition. Precast skin well without rash or petechiae. Pasty oral mucosa. Normal oropharynx. Normal otoscopy. Neck with good mobility. Cardiac: pure tones and rhythm, without murmurs auscultated. Respiratory: symmetrical air entry bilaterally with no pathological sounds auscultated. Abdomen soft, pitting, no masses or organ enlargement. No tenderness. Blumberg negative. Renal Puñopercusión negative. Normal peristalsis. Male genitalia with testes in normoconformados bag. Neurological system: no meningeal signs or neck stiffness, sensory lucid, normal muscle strength and tone in all limbs, normal pedis patellar re lexes, no focal neurologic signs.
Edema is noted on the back of the right foot with the presence of pressing 2 + fovea. No cyanosis of the ingers or acral parts. Pedal pulses present but weak in tone. Blood pressure 120/85 mmHg (P 75) (Figures 1 and 2). Diameter measurements were made of left and right upper limb with a difference of 0.7 mm, and measure the diameter of the lower limbs with a difference of 2cm.
After the initial assessment data were collected personal medical history in which the patient had a proper vaccination schedule, no food or drug allergies, and family background in the presence of a sibling diagnosed with benign familial hematuria.
Study was conducted urine sediment with presence of microhematuria, mucopolysaccharides negative, negative urine culture and renal function in the school with diuresis of 600 cc/day with normal parameters. Hormones TSH, ACTH, cortisol and GH normal. Negative antinuclear Ab.
Among the imaging tests and chest X-ray showed normal members; Echocardiography no pathological indings, renal ultrasonography: normal with no signs of obstructive uropathy or renal stones, Arterial Doppler ultrasound of the right leg: no sonographic evidence of arterial insuf iciency, or signi icant arterial stenosis, absence of deep vein thrombosis or signs of venous valvular insuf iciency. Faced with the possibility of lymphedema diagnosis was performed indirect isotopic lymphoscintigraphy of both lower limbs after intradermal injection of 99mTc Nano colloid digital, which is appreciated and preserved normal migration wide left limb, whereas in the right lower limb showed a decreased radiocolloid lymphatic migration and slowed (Figure 3).  After initial evaluation by the Pediatric Surgical Services and Rehabilitation measures recommended to the patient conservative therapy with physical exercises that allow adequate drainage of lymph accumulated in the distal leg, direct massage on the affected limb and gradual compression stockings.

Discussion
Fischer hypothesized that the rate of skin low originated from the intrinsic contraction segments deep lymphatic collectors are transmitted by micro lymphatic surface through deep channels incompetent.
The normal function of the lymphatic system is to return proteins, lipids and water from the interstitium to the intravascular space. Lymphedema is the result of the failure of this transport with subsequent accumulation of lymphatic luid, especially in the extremities [1,2]. The clinical presentation appears as a swelling in the foot or ankle of a limb increases with heat, fatigue, exercise progressively less and less reducible, until it inally stabilizes. But always keeps a high standard and is the absence of pain. Lymphedema can be primary or secondary. Primary lymphedema is subdivided congenital lymphedema, lymphedema early or late depending on the age of onset. These are often sporadic, with no family history and almost always affects one lower extremity [3][4][5].
High hydrostatic pressures in the arterial capillary force the protein out into the interstitial luid, causing an increased interstitial oncotic pressure [6]. About 90% of the liquid returns to the circulation through the venous capillaries. The remaining 10% is composed of high molecular weight protein and its corresponding oncotic associated water, which lows into the lymph capillaries [7]. In the pathological state of capillary lymphatic transport capacity is reduced [8][9][10].
Although it is uncommon to use test for the diagnosis, some methods are used to con irm the diagnosis, to determine the residual lymphatic function, set preferences for treatment and to evaluate therapy. Lymphangiography was the gold standard for evaluating the lymphatic system for many years, although its use was abandoned because it caused in lammatory reactions in the endothelium of lymphatic vessels, with potential to cause scarring, atrophy, and obstruction of the lumen. It has now been replaced by less invasive techniques such as lymphoscintigraphy or lymphoscintigraphy, which is used to de ine the anatomy and patency, evaluate the dynamics of low and reverse low and determine the severity of the obstruction [11,12]. Computerized Tomography scan is used in cases of suspected malignancy, as are the tests that give more information [14]. One of the main differential diagnosis is the lipedema caused by subcutaneous fat deposition associated with edema, which is characteristic of females, bilateral and symmetrical foot minimum commitment.
Therapeutic measures should be initially conservative avoiding any engagement factor of the skin, tight clothes and excessive heat, must limit physical activity violent or repetitive movements to be appropriate swimming with a water temperature exceeding 28º C and exercises isotonic (walking, biking, swimming).
There must be a manual lymphatic drainage. The movements must be distal to proximal, soft and slow to less affected areas, with daily or weekly sessions and ending with a bandage bandages low extensibility. External compression is done with measures such as compression bandage bandages used multilayer rigid, non-adhesive elastic bandages [15], including compression therapy, vacuum pump and graduated compression boots (good outcome in patients with incipient lymphedema unorganized or Grade I) and compression devices [16]. The use of diuretics is not indicated and surgery is usually palliative therapy although one of the most innovative techniques being used are autologous transplantation of lymphoid tissue [17].

Conclusion
Dilatation of lymphatic capillaries is a rare entity in the daily paediatric clinic, however in situations of lower limb edema we must always take this diagnosis into account in order not to delay the start of effective treatment, especially when we speak children's.