تورم رجلي اليمنى

ابنتي العزيزة؛ أهلا ومرحبا بك على مجانين.
مرضك الذي ذكرت يا ابنتي هو مرض نادر يصيب الأوعية الليمفاوية Milroy's disease غالبا فيكون عددها قليل في الأطراف، ودائما ما تتعرض تلك الأطراف للالتهابات المستمرة مثل (مرض الحمرة)، وأذكر أن واحدا من أساتذة الجراحة منذ أكثر من عشرين عاما أخبرنا بأن هناك بعض العمليات الجراحية التي تُجرى لتصريف السائل الليمفاوي في الطرف المنتفخ ولكنها غير منتشرة كثيرا في العالم، واضطررت يا بنيتي أن أرجع للإنترنت كي أنعش ذاكرتي القديمة في علم الجراحة والذي توقفت عن دراسته منذ عشرين عاما بالتمام والكمال، وفي مثل هذه الأيام تماما كنت أذاكر وأراجع دروس الجراحة استعدادا للامتحان النهائي لبكالوريوس الطب والجراحة بالإسكندرية عام 1987 ويا لها من أيام صعبة وقاسية!!، المهم المعلومات على الإنترنت غزيرة، اخترت لك وللمهتمين بالموضوع بعضا منها:

Swollen Legs
Legs swell for a variety of reasons, not all of them medical. Mild swelling of the ankles and feet occurs in many people who stand a great deal, during hot spells or after long-haul flights. It is made worse by sloppy footwear and lack of exercise. Such swelling usually resolves overnight after the feet have been elevated to heart level.

Constant swelling of the ankles may represent a disorder in circulation, assuming it is not secondary to trauma or to known heart or kidney disease. The two most common circulatory abnormalities leading to swollen legs are venous disease and lymphatic disease.

The former may occur as a result of damage to the veins in the superficial or deep venous systems, as an inherited condition or secondary to deep venous thrombosis. Damage to superficial veins is usually curable. Deep venous damage is treatable, but if excessive usually requires lifelong management and support.

Lymphoedema caused by lymphatic disease is uncommon in the UK. When it occurs, it is often due to inherent damage to the lymphatic vessels. Obstruction of the vessels by lymph node damage is rare, and inherited familial lymphoedema (Millroy's disease) even more so. Leg swelling in lymphoedema is often exacerbated by repeated bouts of skin infections (cellulitis) or fungal infection (particularly Athlete's foot).

Treatment is usually non-surgical and is directed at controlling the swelling.
Milroy's Syndrome
Milroy's Syndrome is an old term used to describe hereditary congenital lymphedema. It is a congenital familial primary lymphedema which results from vertical autosomal inheritance of a single gene. The gene has been identified as VEGFR3. The condition usually presents itself at birth with the swelling of one or even both legs.

If the condition is unilateral (single leg), the other leg may continue in the latency stage for years before expressing itself. The same is indicated for arm lymphedema.

It is the rarest of the inherited lymphedema, accounting for approximately 2% of hereditary lymphedemas.

Hereditary lymphedema was first described by Nonne in 1891, however in 1892 Dr. William F. Milroy described a missionary who had returned from work in India who had swollen legs his entire life. His mother likewise was afflicted with the same condition. Milroy had also, previously studied the 250 year history of a family and had been able to identify 22 persons with this condition through 6 generations. He was also able to pinpoint when the condition entered the family through a marriage in 1768.
Diagnosis
Basic diagnosis can be made by the fact that swelling (generally of the legs) presents at birth and there is a family history of similar swelling. Currently the most precise diagnosis can be made by a lymphoscintigraphy test. In this test a radioactive substance is injected into the limb and is traced on a computer screen. Through this method the exact location of the lymphatic blockages can be identified.
Etiology
The cause of Milroy's Syndrome is a break in the VEGFR3 gene.
Complications
The usual complications involved with the condition include fibrosis of the limb tissues, cellulitis (and or lymphangitis and erysipelas infections). Other complications made include involvement of the genitalia, pain, skin conditions and in very rare situations lymphangiosarcoma, inflammation and various defects, including distichiasis, extradural cysts, vertebral anomalies, cerebrovascular malformations, yellow nails.
Treatment
Decongestive therapy is the most widely accepted form of treatment. There is no cure for Milroy's but the condition can be managed by early diagnosis and treatment. Other treatments involved will focus on the complications such as infections, pain and associated skin problems.
Other therapies used may include compression pump therapy and surgical procedures.
Prognosis
Long term prognosis is excellent is the condition is identified early and treatment begins so after the diagnosis is made.
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Stages of Milroy's Syndrome (Lymphedema)
LATENCY STAGE
Lymphatic transport capacity is reduced
No visible/palpable edema
Subjective complaints are possible

STAGE I
(Reversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting edema
Reduces with elevation (no fibrosis)

STAGE II
(Spontaneously Irreversible Lymphedema)
Accumulation of protein rich edema fluid
Pitting becomes progressively more difficult
Connective tissue proliferation (fibrosis)

STAGE III
(Lymphostatic Elephantiasis)
Accumulation of protein rich edema fluid
Non pitting
Fibrosis and sclerosis (severe induration)
Skin changes (papillomas, hyperkeratosis, etc.)
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Fibrosis in Milroy's Syndrome
Long standing lymphedema causes a condition known as fibrosis. As the fluid continually collects in a limb, it becomes hard and dense. With each stage of lymphedema there is also a change in the tissue texture of a limb.

With stage one the tissue is still much like normal tissue, its just satiated with fluid. As the swelling continues and as he fluid changes to that protein-rich fluid referred to a lymphorrea, you enter into stage two. In this stage, the tissue become very similar to a grape (best image I can think of). Already it is becoming much more difficult for antibiotics to reach bacteria and it becomes less response to the decongestive therapy.

At stage three, the tissue become similar to one of those old synthetic kitchen sponges, the ones that become rock hard when they are dry.

This is the very real serious side affect of stage three lymphedema. This type of tissue increases potential of persistent and very hard to treat cellulitis or lymphangitis.

The denseness of the limb prohibits antibiotics from reaching the infecting bacterium and it is often able to survive in pockets of fibrotic tissue. These pockets act as a septic foci and after antibiotic treatment is completed, the infections will reappear.
Generally at this stage it is going to take IV antibiotics to deal with any infection because oral antibiotics just are not able to penetrate this mass of hard tissue.

Also, as the fibrosis intensifies you become more susceptible to deep venous thrombosis (DVT) and other circulatory problems. You may also start to experience neuropathy as the pressure of this tissue compresses nerves within the limb.
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Decongestive Therapy and Milroy's Syndrome
Manual Lymphatic Drainage (MLD): is a unique, therapeutic method of stimulating the movement of fluids in the tissues. The gentle, rhythmic, pumping, massage movements follow the direction of lymph flow and produce rapid results. It assists the cutaneous lymphatics in picking up and removing not just fluids, but all the waste products, protein particles and debris from our system. It also is successful in breaking fibrosis and fibrotic areas of a lymphodemous limb.

This treatment was created and developed Danish therapists Dr. Emil Vodder and his wife, Estrid, in the 1930's and was introduced in Paris in 1936. They are also credit with creating a specialty of medicine called Lymphology.

First brought to North America in 1982, the school is located in
Victoria, British Columbia, Canada. Before it was introduced the standard treatment course in North American was either a surgery called debulking or the use of compression machines wherein the limb was literally squeezed by pneumatic air pressure.

Comprehensive Decongestive Therapy (CDT) is used primarily in the treatment of lymphedema and venous insufficiency edema. It is a combination of MLD, bandaging exercises and skin care. CDT may also involve breathing exercises, compressive garments and dietary measures. A frequent indication for CDT is lymphedema caused by irradiation or surgery due to cancer. It can relieve edema, fibrosis and the accompanying pain and discomfort.

Also known as Complete Decongestive Physiotherapy (CDP), this treatment therapy was pioneered in the United States by Dr. Robert Lerner.
وهذه هي بعض المواقع التي تتناول هذا الموضوع على الإنترنت:

Lymphedema Glossary
http://www.lymphedemapeople.com/forum/topic.asp?TOPIC_ID=247
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Acupuncture Treatment
http://www.lymphedemapeople.com/thesite/lymphedema_acupuncture_treatment.htm 
Benzopyrones Treatment
http://www.lymphedemapeople.com/thesite/lymphedema_benzopyrones_treatmen.htm 
Compression Pump Therapy
http://www.lymphedemapeople.com/thesite/lymphedema_compression_pump_ther.htm 
Diuretics Treatment
http://www.lymphedemapeople.com/thesite/lymphedema_diuretics_treatment.htm 
Endermologie Therapy
http://www.lymphedemapeople.com/thesite/lymphedema_and_endermologie_therapy.htm 
Kinesiology Therapy
http://www.lymphedemapeople.com/thesite/lymphedema_and_kinesiology_therapy.htm 
Laser Treatment - Sara's Experience
http://www.lymphedemapeople.com/thesite/lymphedema_laser_treatment_saras_experience.htm 
Liposuction Treatment
http://www.lymphedemapeople.com/thesite/lymphedema_liposuction_treatment.htm 
Reflexology Therapy
http://www.lymphedemapeople.com/thesite/lymphedema_and_reflexology_therapy.htm 
Lymphedema Surgeries
http://www.lymphedemapeople.com/thesite/lymphedema_surgeries.htm
Lymphedema Treatments are Poorly Utilized
http://www.lymphedemapeople.com/thesite/lymphedema_treatments_are_poorly_utilized.htm    
Lymphedema Treatment Programs Canada
http://www.lymphedemapeople.com/thesite/lymphedema_treatment_programs_canada.htm
Wholistic Treatment
http://www.lymphedemapeople.com/thesite/lymphedema_wholistic_treatment.htm
Microsurgeries
http://www.lymphedemapeople.com/thesite/lymphedema_and_microsurgery.htm
Homeopathy
http://www.lymphedemapeople.com/thesite/lymphedema_and_homeopathy.htm
Aromatherapy
http://www.lymphedemapeople.com/thesite/lymphedema_and_aromatherapy

وخلاصة هذا الكلام أن هذا المرض وراثي، وهناك خللا جينيا مسئولا عن حدوثه يسمى VEGFR3 gene وأن هناك ثلاثة مراحل لهذا المرض:
أولها تورم الطرف السفلي ولكنه ورم قابل للضغط، وآخرها هو تورم الطرف السفلي كله مع التليف التام للأوعية الليمفاوية وهنا نجد صعوبة كبيرة في الضغط على الجلد أمام قصبة الساق.

أما مراكز العلاج الطبي لهذا المرض النادر فتنتشر بين باريس وهولندا وبريتش كولومبيا بكندا وبالولايات المتحدة الأمريكية.
وهناك طرق كثيرة للعلاج ذكرت لك مواقعها، وأهم هذه الطرق هي:
مدرات البول والملح
البنزبيرون
مفتتات وشافطات الدهون
العلاج بالمضخات الضاغطة
العلاج بالليزر
الجراحة الميكروسكوبية
جراحات التجميل، وغيرهم

ابنتي الفاضلة "حسناء"؛
أتمنى أن أكون قد أجبتك عما يدور في ذهنك ويشغل بالك، وأرجو شفاؤك التام من كل متاعبك، وتابعينا بأخبارك.