Retroperitoneal lymphangiectasia: a great clinical masquerade

Retroperitoneal lymphangiectasia is a very rare lymphatic disorder characterized by abnormal proliferation of lymphatics. We present series of 3 cases of retroperitoneal lymphangiectasia which are diagnosed in our institute with the help of Ultrasonography (USG), Computed Tomography (CT) and Magnetic resonance imaging (MRI) of abdomen and pelvis with unusual clinical presentation. We include clinical features and imaging findings of this disorder with its pathogenesis and diagnosis. Two of the cases were clinically masquerading as hernia and one case was mimicking varicocele. Thorough clinical examination and USG, colour Doppler, CT and MRI are extremely helpful imaging investigation that aid in differentiating these lesions from hernia/ varicocele and the cross sectional imaging like CT and MRI can  depict the anatomical extent of the disease.

High frequency ultrasound in diagnosis of children with primary intestinal lymphangiectasia / 中国医学影像技术

Objective: To explore the diagnostic value of high-frequency ultrasound in children with primary intestinal lymphangiectasia (PIL). Methods: Totally 20 children with clinically diagnosed PIL were retrospectively analyzed. All 20 children underwent abdominal high-frequency ultrasound,13 of them underwent pathologic examinations. The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of ultrasound in diagnosis of PIL were calculated. Results: The accuracy, sensitivity, specificity, positive predictive value and negative predictive value of high-frequency ultrasound in diagnosis of PIL was 80.00% (16/20), 92.31% (12/13), 57.14% (4/7), 80.00% (12/15) and 80.00% (4/5), respectively. Conclusion: High frequency ultrasound can accurately detect PIL from children with thickened intestinal wall.

Successful treatment of conjunctival lymphangiectasia accompanied by corneal dellen using a high-frequency radiowave electrosurgical device

A 62-year-old man presented with a 1-month history of right eye pain unresponsive to artificial tears and topical steroids. At presentation, bullous yellowish fluid collection was noted in the nasal conjunctiva. Corneal thinning and opacity were observed at the 3 o'clock position of the cornea. High-frequency radiowave ablation and biopsy were performed at the affected area. Conjunctival lymphangiectasia was confirmed by excisional biopsy. An improvement in the degree of corneal dellen and chemosis was evident 1 week after ablation. Use of a high-frequency radiowave electrosurgical device may be a simple and effective treatment option for symptomatic conjunctival lymphangiectasia.

Linfangiectasia intestinal primaria: primer reporte de caso en Perú / Primary intestinal lymphangiectasia: first case report in Peru

La linfangiectasia intestinal primaria es una entidad clínica poco común de etiología desconocida. La edad típica de presentación de esta enfermedad es durante los 3 primeros años de vida, pero también se han reportado casos en adultos. Posee sintomatología variable, pero la manifestación clínica principal es el edema, puede presentarse también diarrea y pérdida de peso. La pérdida de fluido linfático en el tracto gastointestinal conlleva también a hipoproteinemia y linfopenia. El diagnóstico se establece en base a la clínica, a los estudios de laboratorio, al estudio endoscópico y se confirma con la evaluación histológica de la biopsia realizada. El manejo se da mediante una dieta rica en proteínas, baja en grasas y triglicéridos de cadena media. A continuación, se presenta el caso de un paciente varón de 1 año de edad que presenta edema generalizado, con predominio de miembros inferiores, y diarrea. Los exámenes de laboratorio muestran la presencia de hipoproteinemia marcada. Posteriormente, se realiza una endoscopía digestiva alta y una biopsia duodenal. El estudio histológico confirma el diagnóstico de linfangiectasia intestinal primaria. El paciente recibe el tratamiento establecido para esta enfermedad y, finalmente es dado de alta.

Primary intestinal lymphangiectasia is a rare clinical condition of unknown etiology. The common age of presentation is during the first 3 years of life, but cases in adults have also been reported. It has a variable symptomatology, but the main clinical manifestation is edema, also diarrhea and weight loss can occur. The loss of lymph fluid into the gastrointestinal tract also leads to hypoproteinemia and lymphopenia. Diagnosis is based on clinical manifestations, laboratory and endoscopic findings, and is confirmed on histopathological examination of biopsy. The main treatment is a protein rich, low in fat and medium chain triglyceride diet. We present the case of a 1-year-old male patient who presents with generalized edema, predominantly in lower limbs, and diarrhea. Laboratory findings show the presence of marked hypoproteinemia. Then an endoscopy and a duodenal biopsy are performed, and the histopathological study confirms the diagnosis of primary intestinal lymphangiectasia. The patient is treated and after a satisfactory evolution, is discharged.

Linfangiectasia pulmonar congénita unilateral en un neonato prematuro / Unilateral congenital pulmonary lymphangiectasia in a preterm infant

La linfangiectasia pulmonar congénita (LPC) unilateral es una enfermedad extremadamente rara de los vasos linfáticos pulmonares. OBJETIVO: presentar un caso de LPC en un recién nacido prematuro. CASO CLÍNICO: recién nacido masculino, prematuro, con insuficiencia respiratoria severa a las 2 horas de vida extrauterina, recibió tratamiento con surfactante exógeno, catecolaminas y ventilación de alta frecuencia oscilatoria (VAFO). La tomografía axial computarizada (TAC) de tórax reveló bulas y atrapamiento de aire de pulmón izquierdo, el estudio histopatológico describió dilatación quística de los canales linfáticos broncoalveolares. Se diagnosticó LPC unilateral secundaria. La evolución clínica hasta los 19 meses de edad fue normal y la TAC de tórax mostró escasas bulas enfisematosas. CONCLUSIONES: La LPC debe ser uno de los diagnósticos diferenciales en neonatos con dificultad respiratoria inexplicable. El pronóstico dependerá del tipo de LPC y de la afectación pulmonar.

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Primary intestinal lymphangiectasia in children / 中华实用儿科临床杂志

With the progress of clinical understanding and examination methods,the incidence of primary intestinal lymphangiectasia in children is gradually increased,congenital lymphatic malformation is the most common pathogen,protein-losing enteropathy is the main clinical feature.In addition to endoscopic and histologic examination,lymphatic radionuclide imaging,has become the common method in diagnosis,especially suitable for infants and young children.The management of lymphangiectasia include:a low-fat diet associated with supplementary medium chain three acyl glycerol,symptomatic treatment,medicine and surgery.Emphasis should be put on long-term management and follow-up work.

Clinical analysis of intestinal lymphangiectasia in 47 children / 中华儿科杂志

Objective@#To analyze the clinical manifestations, diagnosis, treatment and prognosis of intestinal lymphangiectasia (IL) in children in order to improve the skills of diagnosis and treatment of IL.@*Method@#Clinical manifestations, laboratory findings, gastroscopic findings, histopathological examinations and lymphatic radionuclide imaging assessments were analyzed retrospectively among 47 IL patients who were hospitalized in the Gastroenterology Department of Beijing Children's Hospital Affiliated to Capital Medical University from June 2007 to December 2015. All patients were followed up by telephone. According to the various causes, the patients were divided into the primary intestinal lymphangiectasia (PIL) group and secondary IL group, and their clinical manifestations were compared by t test, Rank sum test or Chi-square test.@*Result@#In 47 IL patients, there were 38 children (81%) younger than 3 years old. There were 43 PIL patients (91%) and 4 secondary IL patients (9%). Between PIL and secondary IL, there were statistical differences in serum albumin (t=-3.950, P<0.005) , globulin(t=-2.850, P=0.007), age of onset(U=27.000, P=0.024), age at diagnosis(U=29.000, P=0.030) and course of disease(U=26.500, P=0.023), whereas there were no statistical differences in lymphocyte count, IgG, lymphatic radionuclide imaging, histopathology and gender(all P>0.05). Edema (44 cases, 94%), diarrhea (42 cases, 89%), accompanied with infection (35 cases, 74%) and ascites (30 cases, 64%) were the main clinical manifestations. In 47 IL patients, 45 patients were done gastroscopy and histopathological examinations, and there were 31 patients' histopathological examinations(69%) were positive. Forty patients were done lymphatic radionuclide imaging, and there was evidence of protein losing from gut via lymphatic radionuclide imaging in 39 patients(98%). Among 47 patients, 35 patients (74%) were followed up, 32 patients had good prognosis, 2 patient failed to show evidence of improvement, 1 patient died and no patient experienced a relapse till the end of the follow-up. In 35 patients, 28 patients were treated with medium chain triglycerides (MCT) dietary therapy, 26 patients showed improvement in symptoms, and 2 patients had no improvement. Among 35 patients with follow-up, there were 6 patients received surgical treatment, and their symptoms were improved.@*Conclusion@#PIL are the majority of IL in children younger than 3 years old. The main clinical manifestations are edema, diarrhea, accompanied with infection and ascites. For the patients without the evidence of lymphangiectasia from duodenum histopathological examination, further consideration of lymphatic radionuclide imaging, clinical manifestations, and laboratory studies are needed to make a final diagnosis. MCT dietary therapy is the cornerstone of IL medical management.

Value of CT lymphangiography combined with direct lymphangiography in diagnosing primary intestinal lymphangiectasia / 中华放射学杂志

Objective To investigate the clinical value of CT lymphangiography (CTL) combined with direct lymphangiography (DLG) in primary intestinal lymphangiectasia (PIL). Methods Sixteen patients diagnosed as PIL with intestinal enteroscopy were recruited in this retrospective study. All the patients were performed DLG and CTL one week before exploratory laparotomy. Subjective assessment in DLG included weak lymphatic fluid drainage, lymphangiectasia, lymphatic reflux, fistula and thoracic outlet reflux or obstruction. While for CTL combined with DLG, the intestinal and extra-intestinal lesions were evaluated, including lymph node, edema, lymphangiectasia and abnormal distribution, fistula, and lymphangiomatosis. All the diagnosis was compared with intestinal endoscopy results. Results For DLG, 16 weak lymphatic fluid drainages, 9 lymphangiectasia, 1 fistula with abdomen and 14 thoracic outlets weak lymphatic fluid drainage or obstruction were found. For DLG combined with CTL, 16 intestinal lumens dilatation and 14 circumferential intestinal thickening were found in intestinal lesions. While for extra-intestinal lesions, the imaging features included edematous findings (12 in mesentery, 7 ascites only, 2 hydrothorax and ascites, and 3 pericardial, thoracic and abdominal effusions), abdominal lymph nodes (6 cases), lymphangiectasia and abnormal distributions (14 cases), fistulas (lymph-intestinal luminal fistula in 4 cases, and lymph-abdominal fistula in 3 cases), lymphangiomatosis (3 cases), and thoracic duct outlet dysfunction and reflux (14 cases).The number of cases diagnosed as intestinal lymphangiectasia, intestinal luminal lymph exudation and lymph fistula were 16, 10 and 6 with intestinal endoscopy, while the number were 11, 0, and 4 with CTL combination with DLG. Conclusion Combination of CTL with DLG is valuable in the diagnosis of PIL.

Primary chylous reflux syndrome Ⅰ : a case report / 中华皮肤科杂志

An 18-year-old woman was admitted to the hospital for swelling of the right lower limb and vulva with milky-white vesicles for 3 years.Skin examination revealed swollen and enlarged right lower limb and labium,numerous clustered or scattered pinhead-to millet-sized whitish thick-walled vesicles over the right labium majus,bilateral labium minus,vaginal orifice,and right thigh,with milky-white chylous fluid draining from the vagina.Magnetic resonance imaging revealed obvious lymphangiectasia in the lower abdominal cavity and right side of the pelvis,dilation and distortion of lymphatic vessels in the skin and subcutaneous tissue of the right inner thigh,the vaginal wall and at the medial side of the right labium majus,but no abnormality in the uterus.Histopathological examination of the milky-white vesicles showed cystic dilation of lymphatic vessels in the superficial dermis,and fibrous hyperplasia of the middle and lower dermis.A diagnosis of primary chylous reflux syndrome Ⅰ was made.

An analysis of seven cases of intestinal lymphangiectasia complicated by portal vein hypertension / 中华消化杂志

Objective To investigate the incidence,diagnosis,treatment and prognosis of abnormal lymphatic vessels in intestinal lymphangiectasia (IL) patients complicated by portal vein hypertension.Methods General information,clinical manifestations,laboratory tests,endoscopy and pathological examination,the results of direct and nuclide lymphangiography,treatment and prognosis of seven IL patients complicated by portal vein hypertension were retrospective analyzed.Results Among seven IL patients complicated by portal vein hypertension,there were five cases with abnormal lymphatic vessels and two cases were merely secondary to portal vein hypertension.The main symptoms were edema,abdominal distension and diarrhea.The results of laboratory tests mainly were lymphocytopenia (0.3× 109 to 1.7 × 109/L),hypoalbuminemia (16.0 to 27.8g/L) and hypoglobulinemia (13.2 to 17.7 g/L).Under endoscope,mucosal edema,chronic inflammatory reaction,intestinal villi spot and patched white nodular changes and polyp-like changes appeared.For treatment,the low-fat with medium-chain triglyceride diet and symptomatic support was the basic treatment.The condition could be relieved by surgical remission of abnormal lymphatic vessels or partial small-bowel resection.If the etiological factors could not be removed,the therapeutic effect was poor in the patients merely secondary to portal vein hypertension.Conclusion The suspicious IL patients complicated by portal vein hypertension need further examination to explore whether abnormal lymphatic vessels existed,and to provide evidence for surgical intervention.

Histopathologic Characteristics of Conjunctivochalasis

PURPOSE: In this study we investigated the histopathologic characteristics of conjunctivochalasis and its association with lymphangiectasis using impression cytology and conjunctival excisional biopsy in patients with conjunctivochalasis. METHODS: This prospective study included 14 outpatients diagnosed with conjunctivochalasis, and who had excisional biopsies performed between March 1, 2012 and November 30, 2012. Preoperative slit-lamp examination, dry eye diagnostic test and impression cytology were also performed. For histological analysis, conjunctival sections were stained with hematoxylin and eosin (H&E), Verhoeff-van Gieson (VVG) elastic staining, and monoclonal antibody D2-40, a lymphatic endothelial marker. RESULTS: Tear break-up time and Schirmer's value were decreased in patients with conjunctivochalasis. Impression cytology showed a decrease in goblet cell density and an increase in nucleoplasmic/cytoplasmic ratio. The patients' conjunctival tissues exhibited an infiltration increase of chronic inflammatory cells on H&E stain, and a decrease in collagen density with degeneration of elastic fibers on VVG stain compared to tissues from the control subjects. D2-40 immunohistochemistry revealed the dilated subconjunctival lymphatics in patients with conjunctivochalasis. CONCLUSIONS: This study suggests that the characteristic histopathological features of conjunctivochalasis include dilated lymphatic vessels as well as decreased goblet cell and collagen densities with degeneration of elastic fibers.

Malformación linfangiomatosa intestinal con tejido pancreático ectópico: a propósito de un caso y revisión de la literatura / Intestinal lymphangiomatosis with ectopic pancreatic tissue: a case report and literature review

Intestinal lymphangiomatosis is a rare malformation typically affecting children. Its etiology is unknown, although several hypotheses have been proposed. We present a case of intestinal lymphangiomatosis with ectopic pancreatic tissue of the mesentery, an association not previously described in literature. We emphasize the fact that this entity has sometimes been associated with other intra-abdominal malformations, even conditions undescribed in scientific literature. It must be considered the first differential diagnosis for pediatric patients with radiological evidence of mesenteric cystic lesions, subsequently confirmed by pathological examination.

La malformación linfangiomatosa intestinal es una entidad poco frecuente, típica de la edad pediátrica, cuya etiopatogenia es desconocida, aunque se barajan varias hipótesis. Presentamos un caso de linfangiomatosis intestinal con tejido pancreático ectópico mesentérico, una asociación no descrita anteriormente en la literatura. Destacamos la importancia de conocer que esta entidad, en ocasiones, se asocia a otro tipo de malformaciones intrabdominales, incluso no descritas previamente en la literatura. Se debe plantear como el primer diagnóstico diferencial durante la edad pediátrica, ante el hallazgo radiológico de lesiones quísticas mesentéricas, confirmando posteriormente mediante un estudio anatomopatológico de las lesiones.

A clinical analysis of 61 cases of protein-losing enteropathy / 中华内科杂志

Objective To increase the understanding in protein-losing enteropathy (PLE).Methods Sixty-one PLE patients were enrolled in the study and the clinical characteristics, complicated disease, diagnosis and treatment were analyzed. Results The age of the patients was 16-77 (40±15)years, and the gender ratio was 35:26 (female: male). The main clinical manifestations were bilateral lower limb edema in 51 cases, ascites in 41 cases, bilateral pleural effusion in 23 cases, pericardial effusion in 13cases, abdominal pain in 16 cases and diarrhea in 33 cases. The prominent abnormality in laboratory examinations was hypoalbuminemia. The underlying diseases include systemic lupus erythematosus (SLE) in 28 cases, intestinal lymphangiectasia in 12 cases, hepatic cirrhosis in 5 cases, heart diseases in 5 cases,Crohn's disease in 3 cases, membranous nephropathy in 2 cases, Budd-Chiari syndrome in 1 case. Four cases happened after abdominal operation and 1 case after radiation therapy of gastric cardia cancer. Thirtyseven cases were diagnosed by 99Tcm-labelled human serum albumin scintigraphy and 24 cases were diagnosed clinically. Treatment was focused on underlying diseases. The clinical manifestations in 21 cases of SLE improved after SLE was controlled. In 2 cases of intestinal lymphangiectasia and one with Crohn's disease, the clinical manifestations improved after surgery. The other patients had no improvement.Conclusions PLE was not uncommon in clinical practice. Its predominant characteristics were severe hypoalbuminemia, edema and dropsy of serous cavity. PLE can complicate other diseases such as SLE,intestinal lymphangiectasia. Treatment should be focused on primary disease.

Doença celíaca associada à tireoidite de Hashimoto e síndrome de Noonan / Celiac disease associated with Hashimoto's thyroiditis and Noonan syndrome

OBJETIVO: Relatar o caso clínico de uma criança portadora de doença celíaca, tireoidite de Hashimoto e síndrome de Noonan. DESCRIÇÃO DE CASO: Menina de dez anos e seis meses, branca, apresentando história de diarreia líquida há cinco meses e "aumento da barriga". Ao exame, mostrava peso de 20.580g (p<3), estatura de 114cm (p<3), hidratada, descorada 2+/4+ e consciente. Presença de fácies triangular, com hipertelorismo ocular aparente, posição antimongoloide das fendas palpebrais, orelhas em abano de baixa implantação, micrognatia, pescoço curto e pectus excavatum. O abdome mostrava-se globoso, flácido, indolor, com hérnia umbilical, fígado a 2cm do rebordo costal direito, linfedema em membro superior direito e edema de membros inferiores. Nos exames subsidiários, havia anemia microcítica e hipocrômica, déficit de proteínas totais, tireoidite de Hashimoto e atraso de cinco anos na idade óssea. Na ultrassonografia abdominal, as alças intestinais estavam levemente dilatadas. Devido ao linfedema e à diarreia crônica, a hipótese inicial foi de linfangiectasia intestinal, confirmada pela biópsia jejunal, que ainda mostrou padrão compatível de doença celíaca. O cariótipo foi 46XX com diagnóstico clínico de síndrome de Noonan. COMENTÁRIOS: As doenças autoimunes se associam; no caso apresentado, a doença celíaca se associou à tireoidite de Hashimoto, possivelmente pela presença de antígenos do sistema HLA. Já a associação de doença celíaca à síndrome de Noonan é muito rara, sendo este o terceiro relato na literatura.

OBJECTIVE: To describe the clinical case of a child with celiac disease, Hashimoto's thyroiditis and Noonan syndrome. CASE DESCRIPTION: A Caucasian girl aged ten years and six months had liquid diarrhea for five months, and a "distended belly". At the physical exam: weight of 20,580g (p<3), length of 114cm (p<3), hydrated, anemic 2+/4+ and conscious. The patient presented triangular facies, apparent ocular hypertelorism, antimongoloid position of the palpebral fissures, ears with low implantation, micrognathia, short neck and pectus excavatum. The abdomen was globular, flaccid and painless; the liver was 2cm below the right costal margin. Lymphedema in right upper limb and lower limb edema was also noted. Laboratory exams showed microcytic and hypochromic anemia, deficit of total proteins, Hashimoto's thyroiditis and a 5-year delay in bone age. Abdominal ultrasonography showed the bowel slightly dilated. Due to lymphedema and chronic diarrhea, the initial hypothesis was intestinal lymphangiectasis, which was confirmed by a jejunal biopsy, which also showed celiac disease. The genetic evaluation revealed a 46XX karyotype and a clinical diagnosis of Noonan syndrome. COMMENTS: Different autoimmune diseases can be associated. In this case, the celiac disease and the Hashimoto's thyroiditis are possibly related to the presence of HLA system antigens. However, the association of the celiac disease with the Noonan syndrome is very rare, and this is the third report in the literature.

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

Protracted Diarrhea: Results of the Five-year Survey in a Tertiary Hospital in Korea

The syndrome of protracted diarrhea (PD) includes several diseases with diverse etiologies. This study was conducted to characterize the spectrum of causes, clinical manifestations, and the outcomes of PD. A retrospective analysis of the clinical and pathological findings was performed on 25 patients with diarrhea starting within the first 2 yr of life and a requirement of parenteral nutrition (PN). According to the intestinal histopathology, patients were classified into four groups: immune enteropathy (12 cases), lymphangiectasia (6 cases), epithelial dysplasia (5 cases), and unclassified (2 cases). All patients with epithelial dysplasia had earlier onset of diarrhea and longer duration of PN than those in the other groups. Three patients (12%) had an evidence of a familial condition. Five patients (three with microvillous inclusion disease and two with immune enteropathy) died. Sixteen patients recovered, and three (two with primary lymphangiectasia and one with microvillous inclusion disease) still had diarrhea. One patient underwent intestinal transplantation for tufting enteropathy. In conclusion, infants with PD should be referred to specialized centers where advanced diagnostic and therapeutic facilities are available, because histological analysis is critical for the diagnosis of PD, and PN or intestinal transplantation is the only therapeutic option in a subset of cases.

A Case of Pulmonary Lymphangiectasis

Pulmonary lymphangiectasis is relatively a rare disorder. This disorder can be divided into three groups. First, pulmonary abnormality is associated with lymphangiectasia in other viscera (especilly intestine) and extremities. In the second group, patients have a concomitant cardiac anomaly, which appears to be associated with obstructed pulmonary venous return. The third group comprises of patients whose lymphangicetasia is not associated with cardiac anomalies. This form is thought to result from abnormal development of the lung. In this case, a 6-year-old male who was previously diagnosed as lymphedema of extremities and intestinal lymphangiectasis, suffered from coughing and dyspnea repeatedly. High resolution computed tomography showed thickening of bronchovascular bundle and interlobular septa, pneumonic consolidation and pleural effusion. These findings were compatible with pulmonary lymphangiectasis. We report a case of pulmonary lymphangiectasis with brief review of literatures.

Parapelvic Renal Cyst (Pericalyceal Lymphangiectasis): A case report

Parapelvic renal cyst, also designated as pericalyceal lymphangiectasis, is an unusual lesion that is usually brought to light during surgery for ureteropelvic junction obstruction or recurrent pyelonephritis. Grossly, the renal pelvis is enveloped by a multilocular cystic mass filled with clear fluid. This lesion is confined to the peripelvic tissues and does not extend into the parenchyma, which, however, may show the effects of hydronephrosis or pyelonephritis. A 50-year-old man presented with hydronephrosis. An ultrasonography revealed hydronephro-sis of the left kidney. Intravenous pyelography and DMSA ("Tc-Dimercaptosuccinic acid) scan showed nonfunctioning kidney of the same side. Simple left nephrectomy was done. The renal pelvis was mildly dilated and a cyst was found buldging into the renal pelvis. The content was watery clear and the cyst was not connected to the renal pelvis or calyces. The cyst was round unilocular and lined by attenuated single layer of endothelial cells. The endothelial cells showed no reactivity to factor-VIII related antigen. With these findings, we concluded that this cystic lesion is basically lymphatic cyst and hydronephrosis was caused by the compression of pelvic out-flow of the kidney.