Ingle Sachin B, Hinge Ingle Chitra R
Sachin B Ingle, Department of Pathology, MIMSR Medical College, Latur, Maharashtra 4132512, India.
World J Clin Cases. 2014 Oct 16;2(10):528-33. doi: 10.12998/wjcc.v2.i10.528.
Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.
原发性特发性肠淋巴管扩张症是一种罕见疾病，其特征是在黏膜、黏膜下层或浆膜下层存在扩张的淋巴管，导致蛋白丢失性肠病。此病最常累及儿童，通常在3岁前确诊，但在成人中也很少见。下肢双侧凹陷性水肿是主要临床表现，类似全身性疾病，给临床医生将其与其他常见全身性疾病（如充血性心力衰竭、肾病综合征、蛋白质能量营养不良等）区分开来带来了真正的诊断难题。胶囊内镜检查可作出诊断，它能定位病变，但无法获取活检样本。因此，近来双气囊小肠镜检查与活检相结合可作为有效的诊断工具，以做出正确诊断。患者对由低长链甘油三酯和高蛋白组成并补充中链甘油三酯的饮食反应显著。所以早期诊断对于预防与疾病或治疗相关的不良并发症以获得准确的病理诊断很重要。
