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THE EUROPEAN JOURNAL OF
lymphology
and related problems
VOLUME 29 • No. 75 • 2017
INDEXED IN EXCERPTA MEDICA
A RARE CLINICAL MANIFESTATION OF CONGENITAL LYMPHATIC DYSPLASIA: THE EFFICACY OF COMPLEX DECONGESTIVE THERAPY IN A YOUNG CHILD
Letter to editor
GÖKHAN ÇAG˘ LAYAN 1, PıNAR BORMAN 1 , HÜLYA YAVUZ 1, ÖZGE SOYER 2, HÜLYA DEMIR 2
Hacettepe University, Faculty of Medicine
1 Department of Physical and Rehabilitation Medicine 2 Department of Pediatrics
Corresponding author: Gökhan Çag˘ layan
Hacettepe University Hospital
Department of Physical and Rehabilitation Medicine Sıhhiye, Ankara, Turkey
Fax number: +90 312 310 5769
ABSTRACT
Authors report a child of congenital lymphedema presenting with lymphedema of right face, bilateral arms and with severe intestinal lymphectasia. In this case they aimed to indicate the short term effects of manual lymphatic drainage and multilayer bandaging in the right extremity. Although the regression of abdominal distention seems to be due to the parenteral nutrition with specific diet, they believe that kinesio taping may make even a little contribution.
Keyword: Congenital Lymphatic Dysplasia, Complex Decongestive Therapy, Intestinal Lymphectasia, Parenteral Nutrition.
INTRODUCTION
Dear Sir,
We report a child of congenital lymphedema presenting with lymphedema of right face, bilateral arms and with severe intestinal lymphectasia. A sixteen- month-old female child was consulted to our department of physical and rehabilitation medicine for the swelling of right face, arms (more noticeable in the right side) and abdomen (Figure 1). She had edema from birth on her right and left hands and arms, right side of face and diffuse edema in abdominal region. Her complaints had increased for the last four months and she had diarrhea for a long time. Her family’s past medical history was unremarkable. In her physical examination she had distended abdomen and tachpneic respiration besides. Her diffuse edema was non pitting type. The scintigraphic examination was revealed as; no lymphatic drainage at the right upper extremity (severe lymphatic hypoplasia or agenesia), and delayed lymphatic drainage at the left upper extremity, and normal lymphatics at the lower extremities. These findings were concordant with primary congenital lymphatic dysplasia. Also she
THE EUROPEAN JOURNAL OF LYMPHOLOGY - Vol. XXIX - Nr. 75 - 2017
Fig. 1
had ascites in the abdomen and pericardial effusion in computed tomography and echocardiography. She had no cardiac or renal failure. In her upper gastrointestinal endoscopic examination there was severe intestinal lymphectasia. She had paracentesis, pericardardial drainage and was performed total patenteral nutrition, following the proper diagnosis. After her general condition was stabilized, we started complex decongestive therapy for the lymphedema in the right upper extremity which was more prominent. Manuel lymphatic drainage and multilayered banding (with relatively low tension) techniques were applied daily, for a duration of three weeks. In addition kinesiotaping was performed for the abdominal lymphedema. In the follow up period, her measurements for edema and abdominal distention were improved
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