Nisan 2008
Intestinal lenfanjiektazi, olgu sunumu
Intestinal lymphangiectasia, case report
- Ana Sayfa
- Sayılar
- Nisan 2008
- Intestinal lenfanjiektazi, olgu sunumu...
Özet
Intestinal lenfanjiektazi, çocuklarda ödem ve asite yol açan önemli bir protein kaybettiren enteropati nedenidir. Primer olarak lenfatiklerin konjenital obstrüksiyon defektine bagli ya da konjestif kalp yetmezligi, retroperitoneal fibrozis, pankreatit, tümöral infiltrasyon, Behçet hastaligi gibi hastaliklara ikincil olarak gelisirler. Gastrointestinal kanal yoluyla lenfatik sivi kaybinin oldugu hastalikta, ödem ve diyare önde gelen klinik yakinma olup, lenfopeni ve immun yetmezlik, önemli morbidite nedenlerindendir. Tedavi, ikincil lenfanjiektazilerde altta yatan etkene yöneliktir. Primer lenfanjiektazilerde ise öncelikli tedavi seçenegi, “tibbi tedavi” olup, “yerine koymadestek tedavileri” ve bir somatostatin analogu olan “octreotid” önemli seçeneklerdir. Tüm intestinal lenfanjiektazilerde ilk seçenek dietin düzenlenmesidir. Sag bacakta sislik yakinmasi ile basvuran ve tibbi tedavi seçeneklerine yanit vermemesi üzerine parsiyel jejunektomi yapilan, primer lenfanjiektazi tanili 5 yasinda bir erkek hasta sunulmustur. Makroskopik olarak jejunum segmentinin sarimsi bir renk aldigi, mukozal yüzeyin silöz bir materyalle kapli oldugu görülmüstür. Mukoza ve submukozada içlerinden süt benzeri sivi gelen, çaplari yer yer 0,1-0,2 cm’ye ulasan kistik lezyonlar izlenmistir. Mikroskopik incelemede genislemis lenfatiklerin mukoza ve submukoza yanisira seroza ve lenf nodlarinda da bulundugu dikkati çekmistir. Operasyon sonrasi bacaktaki sisligi gerileyen ancak takipte protein kaybina bagli bulgulari yeniden ortaya çikan olgu, primer intestinal lenfanjiektazideki cerrahi deneyimimizi paylasmak amaciyla ameliyat materyalindeki demonstratif makroskopik ve mikroskopik özellikleri esliginde sunulmustur.
Abstract
Intestinal lymphangiectasia is an important cause of protein losing enteropathy in children, resulting in severe oedema and ascite. It occurs primarily due to congenital obstruction defect of lymphatics or secondary to diseases like congestive heart failure, retroperitoneal fibrosis, pancreatitis, tumoral infiltration, or Behcet’s disease. Because of the loss of chylous fluid into the gut lumen, oedema and diarrhea are the leading clinical symptoms. Immun deficiency and lymphopenia, are the major causes of morbidity. Treatment modalities depend on underlying disease in secondary intestinal lymphangiectasia while replacement therapies and octreotid are choosen in primary cases. Dietary modifications are the first treatment of choices in all forms of the disease. We present a 5 year-old boy who was attended with swelling in his right leg, diagnosed as primary lymphangiectasia on further investigation and treated with partial jejunectomy as the condition was refractory to all standard medical therapies. Macroscopically, the jejenum segment was yellowish in color and the mucosa was covered by chili. Both mucosa and submucosa showed cystic lesions measuring 0.1-0.2 cm in diameter. In microscopic examination, the ectatic lymph vessels were also observed in serosa and lymph nodes. Postoperatively, his leg swelling was resolved, but symptoms due to protein loss were recurred in a years time. The case was presented to share our surgical experience in a case of primary intestinal lymphangiectasia along with the demonstrative macroscopic and microscopic findings in resected specimen.
