Looking at appearance of urine before performing a renal biopsy in nephrotic syndrome.

Chyluria results from an abnormal connection between lymphatic bed and urinary tract, causing lymph leakage into the urine. The clinical picture often begins with the appearance of cloudy, milky urines accompanied by monolateral flank pain, malnutrition, weight loss and weakness. We report a case of chyluria that occurred in a young woman who was referred to our unit for nephrotic-range proteinuria. Before performing a renal biopsy, we found that urine analysis demonstrated a massive lipiduria. Therefore, we collected urine samples from each kidney with a selective ureteral catheterization, demonstrating a monolateral source of lipids and proteins. We suspended the renal biopsy and performed a lymphography that showed an inherited lymphangioma on the left lumbar lymphatic bed. Sclerosing solution instillation, renal pedicle lymphatic disconnection or laser therapy are invasive therapeutical options that may cause severe adverse effects. Instead of these procedures, a conservative therapy based on a low-fat diet supplemented with medium-chain triglycerides was chosen. This dietetic schedule was followed by complete resolution of proteinuria and lipiduria. The patient progressively gained body weight and improved quality of life. No relapses were observed after 3 years of follow-up. This case emphasizes the possible role of a noninvasive therapeutical option for patients with chyluria.

Infected giant lymphangioma circumscriptum treated with a combination therapy.

Lymphangioma circumscriptum is an uncommon benign disorder of skin and subcutaneous tissues characterized by dilated lymphatic channels. It is an uncommon vascular tumor and it rarely becomes infected. We report a 20-year-old man who had an infected giant tumor in his left thigh. After an extensive resection and radiofrequency energy therapy, he recovered well with an acceptable cosmetic result. We followed up the patient for 2 years without any recurrence.

Lymphatic malformations: a proposed management algorithm.

OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

Adult intussusception due to lymphangioma of the colon.

We present herein a case report of adult intussusception due to lymphangioma of the colon. On May 27, 2000, a 39-year-old woman with right lower abdominal pain was admitted to our hospital. Preoperative imaging studies, by ultrasonography, computed tomography (CT), and barium enema examination, showed right colon intussusception with a multilocular cystic tumor as a leading point. Emergency operation was performed. During the operation, normograde ileocecal intussusception with a 9 x 6-cm soft submucosal tumor of the cecum was recognized. Ileocecal resection was performed, and the patient's postoperative course was uneventful. Pathological diagnosis of the resected specimen was a cystic type lymphangioma of the cecum. Recently, lymphangioma of the colon has been diagnosed more frequently by colonoscopy and endoscopic ultrasonography. However, only a few cases of colon intussusception due to lymphangioma have been reported in the literature to date. Large lymphangioma of the colon is rare, but it should be taken into consideration that it is one of the organic lesions that causes adult intussusception.

A clinical evaluation of lymphangioma of the large intestine: a case presentation of lymphangioma of the descending colon and a review of 279 Japanese cases.

With the development and widespread use of colonoscopy, lymphangioma of the large intestine has recently been reported frequently. This paper presents some findings from a review of 279 cases of this disease in Japan, including a typical case that we encountered. A 69-year-old female was diagnosed as having lymphangioma of the descending colon based on the findings of a barium enema and a colonoscopy, and the lesion was successfully removed by an endoscopic resection. In the published reports, the etiology of this disease is not clear yet but the age at onset range shows a tendency toward a higher incidence in comparatively older patients and the male-to-female ratio indicates a higher incidence in males. If there is no complication, endoscopic treatment seems to be the preferable procedure for this disease.

Lymphangioma of the colon: a case report and review of the Japanese literature.

Intraabdominal lymphangiomas are rare, although these lesions can occur in the mesentery, omentum, retroperitoneum, or gastrointestinal tract wall. Here we report a case of lymphangioma of the transverse colon and review the other cases reported in the Japanese literature. Our patient presented with lower abdominal pain and barium enema revealed a filling defect in the transverse colon. Colonoscopy disclosed a submucosal tumor, which changed shape with alteration the patient's position and showed the cushion sign. This lesion was covered with normal-looking mucosa. A correct diagnosis was made from these findings. Knowledge of these endoscopic features may help physicians to provide appropriate diagnosis and treatment of colonic lymphangioma.

Percutaneous sclerotherapy of lymphangiomas.

PURPOSE: The authors report their experience with percutaneous image-guided sclerotherapy for treatment of unresectable lymphangiomas. MATERIALS AND METHODS: Five patients with unresectable lymphangiomas of the pelvis (n = 2), neck (n = 1), abdomen (n = 1), or leg (n = 1) were treated at two medical centers with sclerotherapy, with use of doxycycline as the sclerosant. Computed tomography was used to guide the procedures, with supplemental lymphoscintigraphy, ultrasound, and magnetic resonance imaging used as needed. RESULTS: Symptomatic relief of lymphedema, lymphorrhea, or a decrease in size of lymphatic pools was achieved, to varying degrees, in the five patients. Follow-up is ongoing, and further sclerotherapy may be indicated as the clinical course dictates. CONCLUSION: Percutaneous sclerotherapy with doxycycline is safe and appears effective for palliative treatment of unresectable lymphangiomas on the basis of our initial clinical experience.

Linfangioma quístico retroperitoneal gigante: informe de un caso / Giant retroperitoneal cystic linfangioma: one case report

Se informa un caso de linfangioma quístico gigante retroperitoneal, diagnosticado y tratado en el Hospital Juárez de México; masculino de 38 años de edad, con manifestación clínica de ascitis y masa palpable en ambos cuadrantes derechos del abdomen. Datos de laboratorio de anemia normocítica normocrómica, hipoproteinemia hipocolesterolemia, los estudios radiológicos convencionales: colon por enema, serie gastroduodenal y urografía excretora, demuestran desplazamiento de estos órganos por masa tumoral retroperitoneal del lado derecho. En la laparotomía se encuentra tumor retroperitoneal multiquístico de 40 x 30 x 14 cm, se extirpa en su totalidad; el diagnóstico histopatológico es de linfangioma gigante quístico retroperitoneal. Durante 5 años de seguimiento cursa asintomático y sin recidiva del tumor. En conclusión los LQR son raros, en su mayoría asintomáticos; los métodos radiológicos convencionales ayudan a localizar el tumor en el retroperitoneo, pero no así su extirpe linfática. En la actualidad se diagnostican preoperatoriamente con el empleo del ultrasonido, linfografía, pero principalmente con el uso combinado de la TAC y aspiración percutánea con aguja fina. Su tratamiento es la extirpación total.

[Pneumatosis coli vs. deep cystic colitis]. / Pneumatosis coli vs. colitis quística profunda.

We present the case of a 63-year-old female who with no previous history presented with episodes of self-limited diarrhea without fever, general repercussions, abnormal elements or tenesmus. Biochemistry: no abnormality. Parasite and feces culture: negative. Colonoscopy: at 20 to 40 cm from the anal margin were found polypoid lesions covered with normal mucosa. Barium enema confirmed the presence of multiple lacunar defects and possible parietal gas. Biopsy: submucous cysts with foreign body type giant cells. Mixed infiltrate. The differential diagnosis was made between intestinal enterogenous cyst, lymphangioma, retractable mesenteritis and deep cystic colitis, concluding with pneumatosis coli that evolved favorably.

[Cystic lymphangiomas of the abdomen. CT and US findings]. / Linfangiomi cistici dell'addome. Rilievi TC e US.

Cystic lymphangiomas of the abdomen are an uncommon kind of hamartoma. Their diagnosis is often difficult and calls for an accurate differential diagnosis of this disease and such lesions as cystic pancreatic neoplasms, pseudocysts, hematomas, abscesses and urinomas. Eight cases of abdominal lymphangioma are reported, in patients ranging 35 to 68 years; all lesions were uni/multilocular containing serous fluid. Lymphangiomas were located in the retroperitoneal space (3 cases), in the mesenteric bed (3 cases), close to the left lumbar ureter (1 case), and on the left colon wall (1 case); the symptoms were little characteristic, mostly due to pressure on the adjacent organs. Both CT and US were able to detect and evaluate the lesions; in particular, CT provided also with exact topography. A small lymphangioma in the left colon was revealed only by barium enema. In 2 huge retroperitoneal lymphangiomas the diagnostic evaluation was improved by the use of CT-guided fine-needle biopsy; in patients with mesenteric lesions angiography was employed, also for surgical planning. Lymphography was never performed because CT and US proved the best diagnostic procedures. All patients underwent surgery, whose results confirmed the previous diagnosis, but for the patient with para-ureteral location the surgeon thought nephrectomy necessary.

[Cystic lymphangioma of the abdomen in an adult. Contribution of echography and computed x-ray tomography. Apropos of a case]. / Lymphangiome kystique de l'abdomen chez un adulte. Apport de l'échographie et de la tomodensitométrie. A propos d'un cas.

Authors present a case of cystic lymphangioma of the abdomen in an adult patient. Pre-operative investigations are based on ultrasound and computed tomography since plain films, excretory urograms and barium enema are less helpful. Opportunity of such a diagnosis in adult is not frequent.

Lymphangioma of the transverse colon treated by transendoscopic polypectomy: report of a case and review of literature.

This report concerns a 73-year-old man complaining of intermittent, dull, umbilical pain on one month's duration. Barium-enema examination and endoscopy revealed a hemispheric cystic tumor at the midportion of the transverse colon, and the presumptive diagnosis was cystic submucosal tumor of the colon. Transendoscopic polypectomy was performed and the bulk of the lesion was locally excised from the colonic mucosa. Histologic examination revealed cavernous lymphangioma originating from the submucosa.