Lymphatic Obstruction Research Articles

Results and functional outcomes of en-bloc resection and vascular reconstruction in extremity musculoskeletal tumors

ObjectivesThis paper aims to evaluate the extremity function and vascular outcome after limb-sparing surgery for extremity musculoskeletal tumors invading vascular structure required reconstruction. MethodsOf the 507 patients with musculoskeletal tumors, who underwent surgery between 2004 and 2007, 17 (3,3%) patients with major vessel involvement were included in the study. The mean age was 37.8 ± 14.5, with a female/male ratio of 8/9. Thirteen (76.4%) patients had Stage IIb disease, and 2 (11,7%) patients had Stage III disease. In 2 (11,7%) patients have locally aggressive tumor that had Stage 3. Fifteen (88.2%) of the cases involved lower extremity, whilst 2 (11.8%) of them involved upper extremity. An arterial reconstruction was carried out in all patients. Wide tumor resection and endoprosthetic reconstruction were performed in 6 (35.2%) patients. Other 11 (65.8%) patients were treated with wide resection and soft tissue reconstruction. Postoperative data included; perioperative morbidities such as bleeding, infection, graft thrombosis, rupture, metastatic local recurrence and mortality. Ankle brachial index (ABI) and color-flow-duplex-scan (CFDS) were done at the final follow-up of the study, in order to prove the efficacy of reconstruction. Functional outcome was evaluated with International Society of Limb Salvage (ISOLS) criteria. ResultsThe mean follow-up was of 39 months (range 3–120). Perioperative complications were arterial graft thrombosis occurred in 3 (17.6%) patients treated acutely with thrombectomy, uncontrolled deep wound infection occurred in 2 patients whom extremities were amputated.The most frequent complication after surgery was limb edema according to possibly venous and lymphatic obstruction, staged as C1, C2 and C3 disease was established in 6 patients (two patients in each group), and 1 patient was classified as C6 disease. Three (17.6%) patients had local recurrence (1/3 patient died and 2/3 (11.7%) patients underwent transfemoral amputation). At the last follow-up, 9 (52.9%) patients were alive without evidence of disease, 8 (47.1%) patients were died due to primary disease. There were 8 (47.1%) patients alive with an intact limb. Although functional outcome scores were satisfactory, emotional acceptance scores were low. The limb salvage probability was 74.0%. ConclusionLimb-sparing oncological surgery in musculoskeletal tumors with vascular invasion provides a satisfactory limb function, which may lead to an improved life quality. Arterial reconstruction has a high rate of patency in the long term. The surgeon should be aware of early perioperative complication related to vascular reconstruction and infection that effect on the rate of extremity survival. Level of evidenceLevel IV, Therapeutic study

Acoustic Radiation Force Impulse Elastography: Tissue Stiffness Measurement in Limb Lymphedema.

Purpose To evaluate the feasibility of cutaneous and subcutaneous limb tissue elasticity measurement in participants with limb lymphedema by using acoustic radiation force impulse (ARFI) elastography. Materials and Methods From July 2015 to June 2017, ARFI elastography was performed in 64 participants with lymphedema (seven men and 57 women; age range, 23-85 years) by using a US system. Tissue stiffness quantification with shear-wave velocity (SWV) was obtained in the cutaneous and subcutaneous limb tissues. Lymphoscintigraphy was the reference standard. Results SWV was significantly higher in limbs with lymphatic obstruction than in unaffected limbs (cutaneous tissue: 2.75 m/sec vs 1.74 m/sec, respectively; subcutaneous tissue: 1.90 m/sec vs 1.35 m/sec, respectively; P < .001). SWV was significantly different among limbs without lymphatic drainage obstruction, with partial obstruction, and with total obstruction (cutaneous tissue: 1.74 m/sec vs 2.75 m/sec vs 2.77 m/sec; subcutaneous tissue: 1.35 m/sec vs 1.90 m/sec vs 1.90 m/sec, respectively; P < .001). By using a cut-off value of 2.10 m/sec and 1.43 m/sec for cutaneous and subcutaneous tissue, respectively, sensitivity was 83.1% (59 of 71) and 80.3% (57 of 71), and specificity was 86.0% (49 of 57) and 70.2% (40 of 57) for manifestation of lymphatic obstruction. The corresponding areas under the receiver operating characteristic curve were 0.91 and 0.83, respectively. Conclusion Acoustic radiation force impulse elastography showed that cutaneous and subcutaneous tissues are stiffer in lymphedematous limbs than in unaffected limbs. Acoustic radiation force impulse elastography is a feasible imaging modality for noninvasive tissue stiffness quantification in limb lymphedema. © RSNA, 2018 Online supplemental material is available for this article.

Gebelikte, Rekürren Bartolin Apsesine Bağlı Gelişen Vulva Ödemi

Vulvar edema appears as a manifestation in many diseases rather than (being) a disease. It does not have a specific etiology; it can appear as a result of allergic, inflammatory and neurological diseases affecting vascular and/or lymphatic system, or trauma. In its etiology there are herpes infections, Bartholin abscess, lymphogranuloma venereum, Crohn's disease, Behcet's disease, which are of infection origin. Vulvar edema in pregnancy is a rare clinical condition that often appears at the third trimester, is due to lymphatic obstruction and toxemia of pregnancy. In this paper we present a 25-year-old female patient who was primipara, developing edema of the vulva arising from recurrent Bartholin abscess at the second trimester of pregnancy. After the examinations, vulvar edema was attributed to Bartholin abscess caused by bacterial colonization due to traumatic coitus.

Imaging lymphatics in human normal and lymphedema limbs-Usefulness of various modalities for evaluation of lymph and edema fluid flow pathways and dynamics.

The human lymphatic system morphology and function still remain largely unknown to clinicians and biologists. How does the lymphatic vascular system look like in comparison to the blood transport system, how does lymph flow, where does capillary filtrate accumulate in cases with lymphatic obstruction caused by inflammation, trauma, and cancer therapy, remain as basic questions. Visualization of the lymphatic pathways and dynamics of lymph flow, and in cases of obstruction, the localization of the capillary filtrate/edema fluid accumulation becomes indispensable. The contemporary methods only partly meet these requirements. Since the early 1950s of the 20th century only few specific clinical methods of imaging of limb lymphatics are being used in human clinic. Each of the applied modalities provides different images due to different physical chemistry and distribution of tracer, methods used for its detection in tissues, their sensitivity and specificity and clinical type of lymph vessel pathology. Here, the advantages and disadvantages of the most commonly used 3 methods of imaging: the iodinated oil X-ray, isotopic, and fluorescent lympangiographies are presented. The study is based on retrospective and recent collections of lymphangiograms from large cohorts of patients. Imaging of lymph nodes has not been included as it is requiring different interpretation compared with vessels. Composite evaluation of X-ray, isotopic, and fluorescent lymphographic images or, as it is practiced now the isotope and indocyanine green near infrared lymphographies, provide most clinically important information. Special attention was directed at methods enabling early diagnosis of imminent lymphedema especially in cases with cancer therapy-related lymphedema. Groups of typical images obtained with the 3 methods are presented.

Neurofibromatosis-associated Malabsorption

We report on a case of a young woman who presented with anemia secondary to malabsorption in the setting of neurofibromatosis with intestinal involvement. A 25-year-old woman presented with symptomatic iron deficiency anemia. There was no history of menorrhagia or pregnancy and she was eating a regular diet. For the prior 18 months, she suffered from diarrhea and weight loss. Her family history was significant for both her mother and brother having neurofibromatosis type 1. She, herself, had been previously diagnosed with neurofibromatosis based on café au lait spots. Her exam was significant for several café au lait spots, a 2.5cm neurofibroma on the chest, a firm yet non-tender abdomen, and a body mass index of 17.3. Labs revealed a microcytic anemia of 6.4g/dL with an associated ferritin of 2.7ng/mL. Further laboratory testing revealed a low serum copper of 29μg/dL, hypoalbuminemia of 2.0g/dL, and a low 25-OH vitamin D at 6.1ng/mL. Thiamine, vitamin E, vitamin B12, folate and IgA tissue transglutaminase were normal. A CT scan showed multiple segments of severely thickened jejunal and ileal loops. Some areas contained polypoid luminal masses. A conglomeration of low-density masses was noted throughout the small bowel mesentery (Figure 1). The terminal ileum and proximal duodenum were not involved. A subsequent diagnostic push enteroscopy revealed markedly dilated lacteals in the jejunum (Figure 2). Jejunal biopsies confirmed diffuse mucosal involvement by neurofibroma with associated dilated lacteals (Figure 3). This led to a diagnosis of small bowel neurofibromatosis-associated malabsorption. Neurofibromatosis type 1 is an autosomal dominant condition associated with gastrointestinal tumors in up to 25% of cases.1 Neurofibromatosis causing lymphatic obstruction of the extremities with resultant lymphedema is well reported. Neurofibromatosis involving the small bowel may present with occult bleeding, intussusception, or constipation.3-5 In the case described here, infiltration of the mesentery as well as the small bowel with neurofibromatosis resulted in dilated lacteals, associated hypoalbuminemia, as well as multiple vitamin deficiencies. The patient's anemia and copper improved with parenteral iron and copper supplementation, but her hypoalbuminemia and diarrhea persisted. Because of extensive small bowel involvement, surgical resection was deferred and she was referred to a nutritionist for dietary manipulation.Figure: CT demonstrating segments of thickened jejunal and ileal loops, polypoid luminal masses. and a conglomeration of low-density masses throughout the small bowel mesentery.Figure: Push enteroscopy revealing markedly dilated lacteals in the jejunum.Figure: Jejunal biopsies showing diffuse neurofibroma with dilated lacteals.

P3-052 - One patient who presented with a return current disorder due to lymphatic obstruction after using RAM+PTX

P3-052 - One patient who presented with a return current disorder due to lymphatic obstruction after using RAM+PTX

Improvement of the efficacy of vascularized lymph node transfer for lower-extremity lymphedema via a prefabricated lympho-venous shunt through lymphaticovenular anastomosis between the efferent lymphatic vessel and small vein in the elevated vascularized lymph node.

Following vascularized lymph node (VLN) transfer (VLNT), the VLN may be at a risk for sclerosis because of efferent lymphatic vessel obstruction. We developed a new technique to prevent VLN sclerosis via a prefabricated lympho-venous (LV) shunt. This study compared the treatment outcomes of single VLNT with prefabricated LV shunt, conventional multiple VLNTs, and conventional single VLNT. Overall, 47 limbs of 45 patients that underwent VLNT for lower-extremity lymphedema (LEL) in late clinical stage II/III were divided into 3 groups: conventional single VLNT group (control; 21 limbs where 4 had primary LEL), multiple VLNTs group (13 limbs where 3 had primary LEL), and prefabricated LV shunt group (13 limbs where 4 had primary LEL). In the prefabricated LV shunt group, lymphaticovenular anastomosis between the efferent lymphatic vessel and small vein in the elevated VLN were performed simultaneously with VLNT. Although venous thrombosis at the anastomosis site was observed in 1 case, it was salvaged by re-anastomosis, and all VLNs survived. No other complications were observed. The LEL index significantly improved in the prefabricated LV shunt group compared with that in the control group (28.0 ± 1.7 vs 20.9 ± 1.5, P = 0.02). In the prefabricated LV shunt group, all VLNs survived functionally, and the average size of the transferred lymph nodes was significantly larger than that of the control group (5.7 ± 0.1 vs 4.3 ± 0.2 mm, P < 0.01). Prefabricated LV shunt may improve the efficacy of VLNT.

Multidetector CT for Evaluation of the Extrapleural Space.

The extrapleural space (EPS) is an anatomic space at the periphery of the chest that can be involved in a number of disease processes. This space lies between the inner surface of the ribs and the parietal pleura and contains adipose tissue, loose connective tissue, lymph nodes, vessels, endothoracic fascia, and the innermost intercostal muscle. It is often overlooked on cross-sectional imaging studies and almost invariably overlooked on conventional radiographic studies. At conventional radiography, the EPS occasionally can be seen when there is extrapleural fat proliferation, which might be confused with pleural thickening or pleural effusion. Knowledge of the normal anatomy of the EPS depicted at computed tomography (CT) and of the relationship of the EPS with parenchymal, pleural, and chest wall processes is key to the detection of extrapleural abnormalities. Disease entities that most commonly affect the EPS include chronic inflammatory disorders, infection, trauma, and neoplasms. Chronic inflammatory conditions and infectious processes of the lungs and pleurae induce adipocyte proliferation adjacent to the inflamed tissue, resulting in increased extrapleural fat. Chest wall trauma with extrapleural hematoma formation causes characteristic CT findings that enable differentiation of the extrapleural hematoma from hemothorax and warrant a different treatment approach. Extrapleural air is commonly seen in patients with pneumomediastinum and should be distinguished from pneumothorax because it requires a different treatment approach. Intrathoracic neoplasms can cause an increase in the attenuation of normal extrapleural fat owing to pleural inflammation, lymphatic obstruction, lymphangitic spread, or direct invasion by tumor. The normal and pathologic appearances of the EPS, as depicted at thoracic CT, and the differential diagnosis of findings in the EPS are reviewed. ©RSNA, 2017.

Value of CT lymphangiography combined with direct lymphangiography in diagnosing primary intestinal lymphangiectasia

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. Key words: Lymphangiectasis, intestinal; Tomography,X-ray computed; Lymphography; Endoscopy, digestive system

Management of refractory chylous ascites with peritoneovenous shunts.

The purpose of this study was to define outcome of treatments of refractory chylous ascites using peritoneovenous shunts (PVSs). Clinical data of patients with refractory chylous ascites treated with PVSs between 1992 and 2015 were retrospectively reviewed. The primary end point was clinical benefit, defined as cured, improved, or poor results; secondary end points were complications and reinterventions. Seventeen patients (eight female [47%]; median age, 47years [range, 19-78years]) with refractory chylous ascites were studied. This group represented 6% of 284 patients treated for chylous ascites during the study period. The etiology was primary lymphangiectasia in 10 patients (59%) and secondary chylous ascites due to previous surgery, lymphatic obstruction with associated portal hypertension, or malignant tumor in 7 (41%). Eleven patients were treated with LeVeen shunts and six with Denver shunts. Thirty-day mortality, morbidity, and reintervention rates were 5.9%, 18%, and 12%, respectively. Reintervention rate at 6months was 9.1% with LeVeen shunt, significantly lower than 100% with Denver shunt (P= .001). During a mean follow-up of 5.1years (range, 17 days-22.7years), 7 of 11 patients with LeVeen shunt and all 6 patients with Denver shunt required shunt replacement. Median duration of patency was 215days (range, 2 days-9.0years) of a total of 25 LeVeen shunts placed in 11 patients and 44days (range, 6-91days) of 20 Denver shunts placed in 6 patients. At last follow-up, patency of the LeVeen shunt was 36% (4/11); symptoms improved in 64% of the patients (7/11). Patency rate of Denver shunts was 33% (2/6), and symptoms improved in 33% (2/6). Treatment of refractory chylous ascites continues to be a major challenge. The only currently available PVS, the Denver shunt, had a median patency period of<2months; it required frequent replacements and resulted in intermittent short-term clinical benefit in one-third of the patients. Improvements in technology to design new shunts, to develop new therapies, or to adopt new techniques to treat chylous ascites are urgently needed.

Isolated Xanthogranuloma of Pelvico-Ureteric Junction: is this Beginning of Xanthogranulomatous Pyelonephritis? – A Case Report

Xanthogranulomatous Pyelonephritis (XGP) is a rare condition characterised by chronic granulomatous inflammation of renal tissue [1,2]. This can be due to obstruction, urinary tract infection and renal calculi [1-5]. The exact pathophysiology and progression of this condition is poorly understood, but altered immune response, changes in lipid metabolism, disturbances in leukocyte function, lymphatic obstruction, venous occlusion/haemorrhage, malnutrition, arterial insufficiency and necrosis of pericalyceal fat [2,4]. Its management with surgical intervention in the form of nephrectomy, partial or complete, is the most definitive management option of this condition [1]. We report the case of a woman with isolated xanthogranuloma obstructing the pelviureteric junction. We propose this as the initial pathophysiological process of Xanthogranuloma pyelonephritis.

Effectiveness of Preoperative Lymphoscintigraphy for the Detection of Cervical Lymph Node Metastasis in Patients with Oral Squamous Cell Carcinoma.

Background:Oral squamous cell carcinoma (OSCC) is one of the most common malignant tumor. OSCC is the malignancy of squamous epithelium of oral cavity, which is the sixth most common malignancy reported worldwide and one with highest mortality rate among all malignancies.Aims:The aims of this study is to assess the diagnostic performance of lymphoscintigraphy (LSG) for the detection of cervical lymph node metastasis in patients with OSCC.Materials and Methods:This was a prospective study done in Oral and Maxillofacial Surgery Department and National Institute of Nuclear Medicine and Allied Science, Bangabandhu Sheikh Mujib Medical University during July 2015–June 2016. Thirty-six patients with OSCC were included in this study. Radioisotope technique was used in the detection of cervical metastases in patients with histologically proven OSCC. Patients were assessed by LSG after diagnosis of OSCC, and then, it was compared with postoperative histopathology report.Results:Lymphoscintigraphically out of 36 patients, 23 had lymphatic channel obstruction where histologically 20 patients had lymph node metastasis. There were 20 true positive cases, 13 true negative cases, and 3 false positive cases but no false negative case was found. The test of validity result reveals that sensitivity 100.0%, specificity 81.25%, accuracy 96.66%, positive predictive value 86.96%, and negative predictive value 100.0%.Conclusion:LSG for the detection of cervical lymph node metastasis has an important role for the management of OSCC. It is also cost-effective and decreases the morbidity.

An Infectious Pig Model of Crohn's Disease.

Over the last several years, we have demonstrated that intestinal lymphangitis and lymphatic obstruction are fundamental lesions in Crohn's disease, for which no therapy is currently available. There is an infectious enteritis of young pigs that offers an opportunity to understand how the lymphangitis of CD may have been initiated. The pathology of chlamydial enteritis was described earlier, from 1987 to 2009. Tissue blocks and hematoxylin and eosin-stained slides from Chlamydia suis-inoculated young pigs were provided by D. Rogers and F. Guscetti. Experimental animals, gnotobiotic and conventional, had been autopsied 4, 7, and 10 days postinoculation. Serial sections of intestine were immunostained with a Chlamydia trachomatis antibody, which cross-reacted with C. suis antigen, and compared with hematoxylin and eosin preparations. Immunohistochemistry revealed antigen in villous epithelial cells of jejunum and ileum and in the endothelium of lacteals and lymphatics by day 4. This was accompanied by lymphatic endothelial necrosis, lymphangitis, and inflammatory lymphatic obstruction, through several layers of the affected intestinal segments, days 4 through 10. Although the original authors documented lesions to define the porcine disease, here the author characterizes the lymphangitis as a model for understanding Crohn's disease and suggests a chlamydial origin for the latter.

Compression in dermato-lymphangio-adenits

Not available

Aligned nanofibrillar collagen scaffolds – Guiding lymphangiogenesis for treatment of acquired lymphedema

Secondary lymphedema is a common disorder associated with acquired functional impairment of the lymphatic system. The goal of this study was to evaluate the therapeutic efficacy of aligned nanofibrillar collagen scaffolds (BioBridge) positioned across the area of lymphatic obstruction in guiding lymphatic regeneration. In a porcine model of acquired lymphedema, animals were treated with BioBridge scaffolds, alone or in conjunction with autologous lymph node transfer as a source of endogenous lymphatic growth factor. They were compared with a surgical control group and a second control group in which the implanted BioBridge was supplemented with exogenous vascular endothelial growth factor-C (VEGF-C). Three months after implantation, immunofluorescence staining of lymphatic vessels demonstrated a significant increase in lymphatic collectors within close proximity to the scaffolds. To quantify the functional impact of scaffold implantation, bioimpedance was used as an early indicator of extracellular fluid accumulation. In comparison to the levels prior to implantation, the bioimpedance ratio was significantly improved only in the experimental BioBridge recipients with or without lymph node transfer, suggesting restoration of functional lymphatic drainage. These results further correlated with quantifiable lymphatic collectors, as visualized by contrast-enhanced computed tomography. They demonstrate the therapeutic potential of BioBridge scaffolds in secondary lymphedema.

The Role of the Mesentery in Crohn's Disease: The Contributions of Nerves, Vessels, Lymphatics, and Fat to the Pathogenesis and Disease Course.

Crohn's disease (CD) is a complex gastrointestinal disorder involving multiple levels of cross talk between the immunological, neural, vascular, and endocrine systems. The current dominant theory in CD is based on the unidirectional axis of dysbiosis-innate immunity-adaptive immunity-mesentery-body system. Emerging clinical evidence strongly suggests that the axis be bidirectional. The morphologic and/or functional abnormalities in the mesenteric structures likely contribute to the disease progression of CD, to a less extent the disease initiation. In addition to adipocytes, mesentery contains nerves, blood vessels, lymphatics, stromal cells, and fibroblasts. By the secretion of adipokines that have endocrine functions, the mesenteric fat tissue exerts its activity in immunomodulation mainly through response to afferent signals, neuropeptides, and functional cytokines. Mesenteric nerves are involved in the pathogenesis and prognosis of CD mainly through neuropeptides. In addition to angiogenesis observed in CD, lymphatic obstruction, remodeling, and impaired contraction maybe a cause and consequence of CD. Lymphangiogenesis and angiogenesis play a concomitant role in the progress of chronic intestinal inflammation. Finally, the interaction between neuropeptides, adipokines, and vascular and lymphatic endothelia leads to adipose tissue remodeling, which makes the mesentery an active participator, not a bystander, in the disease initiation and precipitation CD. The identification of the role of mesentery, including the structure and function of mesenteric nerves, vessels, lymphatics, and fat, in the intestinal inflammation in CD has important implications in understanding its pathogenesis and clinical management.

Great tumour burden in the axilla may influence lymphatic drainage in breast cancer patients.

We investigated whether great tumour burden in the primary draining lymph node would lead to obstructed lymphatic flow in breast cancer patients. Breast cancer patients with false-negative sentinel lymph nodes (SLNs) were enrolled from January 2001 to March 2011, retrospectively. A further 45 breast cancer patients were recruited prospectively from December 2013 to November 2014. Carbon nanoparticles, a lymphatic tracer, were injected into the subareolar area 24h before surgery, followed by axillary lymph node dissection. In the SLN cohort, among the 28 false-negative cases, >50% showed great tumour burden in the axilla. In the carbon nanoparticles cohort, we found that cases with <3 nodes involved in the pathology had more lymph nodes stained by carbon nanoparticles than the subgroup with ≥3 involved nodes (P=0.003). Nodes stained with carbon nanoparticles showed smaller tumour burdens compared with unstained nodes (P<0.05). Furthermore, five cases showed metastatic nodes that were not stained with carbon nanoparticles, and all the lymph nodes that were free of metastasis were stained with carbon nanoparticles. Great tumour burden in the axilla might lead to lymphatic flow obstructions in clinical practice. Nevertheless, clinical trials are still needed to validate our findings.

Recurrent scrotal edema in a patient with radiation enteritis: A case report.

Since its introduction as an alternative treatment technique, radiotherapy has been increasingly used as the medical treatment of choice for patients with malignant tumors. However, radiotherapy is associated with a number of common, well-described side effects, which may compromise the quality of life of the patients. Scrotal edema is an infrequent complication in patients who undergo pelvic irradiation, which is suspected to be due to lymphatic obstruction. An extensive literature search found no previous case report describing this complication in patients receiving pelvic radiotherapy. Herein, we present a case of recurrent scrotal edema in a 59-year-old man with prostate cancer and radiation enteritis. Conservative therapy was applied and was successful in relieving the symptoms. To the best of our knowledge, this is the first case report of scrotal edema in a patient with radiation enteritis.

Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature.

Acquired lymphangiectasia of the vulva (ALV) is a rare condition thought to be secondary to pelvic lymphatic obstruction. Although benign, this entity often occurs after previous malignancy and can be confused with conditions such as genital warts. We sought to clarify the clinicopathologic features of ALV by studying affected patients from our institution and from the existing literature. We reviewed retrospectively the clinical and histopathologic characteristics of patients with ALV from our institution and performed an extensive literature search to identify all published cases of ALV. Eleven patients with ALV were identified from our institution. Three patients had Crohn disease, three had malignancies involving the pelvic region (rectal adenocarcinoma, cervical carcinoma, endometrial carcinoma), and three (two with pelvic malignancy) had previous radiation therapy. One patient had Proteus syndrome. Of 67 distinct cases of ALV identified in the literature, 41 (61%) had a history of malignancy. In any acquired or clinically unusual lymphatic process in the skin and soft tissues, a lesion or obstructive process of the draining lymphatics should be investigated.

Primary Intestinal Lymphangiectasia and its Association With Generalized Lymphatic Anomaly

Background: Lymph is a fluid originating in the interstitial spaces of the body that contains cells, proteins, particles, chylomicrons, and sometimes bacteria. Objectives: The aim of the present study is to demonstrate that primary intestinal lymphangiectasia (PIL) results from a disruption of lymphatic circulation, thus corresponding to a secondary rather than a primary event in the context of generalized lymphatic anomaly. Materials and Methods: In this case series and record review, an analysis of intestinal lymphatic involvement was performed on patients diagnosed with PIL between 1965 and 2013. Of the 21 patients included in the study, 10 had been diagnosed before 5 years of age (1 prenatal), 8 between 5 and 18 years of age, and 3 while older than 18 years of age. The follow-up period varied between 1 and 34 years. Clinical data, blood and fecal parameters, imaging studies, endoscopy results, biopsy analyses, treatment details, and outcome information were collected from medical records. Endoscopy, histological studies, magnetic resonance imaging, and lymphoscintigraphy were performed on all patients. Dynamic intranodal lymphangiography was performed on 8 patients. Results: Central lymphatic channel obstruction was identified in 12 patients (57%). Associated lymphatic malformation (LM) was present in 16, diarrhea in 10, chylothorax in 11, chylous ascites in 10, pericardial effusion in 6, coagulopathy in 3, and osteolysis in 7. Conclusions: We consider intestinal lymphangiectasia not as an entity in itself, but as a consequence of lymphatic flow impairment in the thoracic duct, producing chylous reflux into the intestinal lymphatics.