Ultrasonographic features of intestinal lipogranulomatous lymphangitis in 10 dogs.

Intestinal lipogranulomatous lymphangitis (ILL) is a granulomatous inflammation of the lymphatic vessels of the intestinal wall and mesentery characterized by lipogranulomas. The purpose of this retrospective, multi-center, case series study is to report the ultrasonographic features of canine ILL. Ten dogs with a histologically confirmed ILL undergoing preoperative abdominal ultrasound were retrospectively included. Additional CT was available in two cases. Lesion distribution was focal in eight dogs and multifocal in two. All dogs presented with intestinal wall thickening and two had a concomitant mesenteric mass adjacent to the intestinal lesion. All lesions were in the small intestine. Ultrasonographic features were altered wall layering with predominantly muscular and to a lesser extent submucosal layer thickening. Other findings included hyperechoic nodular tissue within the muscular, serosa/subserosal, and mucosal layers, hyperechoic perilesional mesentery, enlarged submucosal blood/lymphatic vessels, mild peritoneal effusion, intestinal corrugation, and mild lymphadenomegaly. The two intestinal to mesenteric masses presented heterogeneous echostructure, predominantly hyperechoic with multiple hypo/anechoic cavitations filled with mixed fluid and fat attenuation content on CT. Histopathological findings included lymphangiectasia, granulomatous inflammation, and structured lipogranulomas affecting mainly submucosa, muscularis, and serosa. The intestinal to mesenteric cavitary masses revealed severe granulomatous peritonitis with steatonecrosis. In conclusion, ILL should be considered as a differential diagnosis for dogs with this combination of ultrasonographic features.

Computed tomographic features of focal lipogranulomatous lymphangitis for differentiating from malignant intestinal lesions in a dog.

An eight-year-old Maltese dog presented with diarrhea and anorexia. Ultrasonography revealed marked focal wall thickening with loss of layering in the distal ileum. Contrast-enhanced computed tomography (CT) revealed a preserved wall layer with hypoattenuating middle wall thickening. In some segments of the lesion, small nodules protruding toward the mesentery from the outer layer were observed. Histopathology revealed focal lipogranulomatous lymphangitis (FLL) with lymphangiectasia. This is the first report to describe the CT features of FLL in a dog. CT features of preserved wall layers with hypoattenuating middle wall thickening and small nodules can assist in diagnosing FLL in dogs.

Periocular intravascular papillary endothelial hyperplasia: A retrospective study.

INTRODUCTION: Intravascular papillary endothelial hyperplasia (IPEH) is a rare proliferation of endothelial cells with uncertain etiology related to thrombus formation. Diagnosis is usually confirmed histopathologically. This condition has been previously described in the periocular region but not in the conjunctiva. METHODS: It is a retrospective case series in which we evaluated seven patients with histopathologically confirmed IPEH cases. Data regarding the demographics, clinical presentation, radiological description, histopathological features including any IHC staining, suspected underlying vascular etiology, management options, and follow up outcome were collected. RESULTS: A total of seven cases of histologically confirmed IPEH were included. Five out of seven patients were male (71.4%). The age range was between 6 and 69 years with a median age of 36 years. Three cases involved the eyelid (42.8%) and another three were found in the conjunctiva (42.8%). Pre-existing underlying vascular lesions were observed in all patients, five malformations (mostly lymphatic-venous) and two conjunctival hemorrhagic lymphangiectasis. All cases were treated with excisional biopsy with no signs of recurrence within an average of 7 months follow up. CONCLUSIONS: Periocular IPEH is a rare tumor that is likely to coexist with underlying vascular lesions and thrombus formation. We are reporting its existence in the conjunctiva for the first time. Therefore, pathologists should be aware of the histopathological spectrum of this lesion.

The Update of Treatment for Primary Intestinal Lymphangiectasia.

Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.

Primary intestinal lymphangiectasia presenting as limb hemihyperplasia: a case report and literature review.

BACKGROUND: Primary intestinal lymphangiectasia is an exceedingly rare disorder. Epidemiology is unknown. It usually presents with lower extremity swelling, diarrhea, ascites, and protein-losing enteropathy. Since the pathogenesis of edema is usually due to hypoalbuminemia; both extremities are typically involved. The edema can rarely be due to abnormal lymphatic circulation, causing lymphedema, which usually involves both extremities as well. Diagnosis is made by the constellation of clinical, biochemical, endoscopic, and histological findings. Treatment involves dietary modification, to reduce lymphatic dilation in response to dietary fat. Other pharmacologic (e.g., octreotide) and replacement measures may be indicated as well. The most serious long-term complication is intestinal lymphoma. Herein is a case of Primary intestinal lymphangiectasia presenting with unilateral lower limb swelling. CASE PRESENTATION: A 4-year-old boy presents with left foot swelling since the age of 4 months, in addition to intermittent diarrhea, and abdominal swelling. The foot swelling had been evaluated by different health care professionals in the past, and was mislabeled as either cellulitis, or congenital hemihyperplasia. Physical examination revealed mild ascites, and a non-pitting foot edema with a positive Stemmer's sign (lymphedema). Blood work revealed hypoalbuminemia (albumin 2 g/dl), and hypogammaglobulinemia. Endoscopy showed dilated lacteals throughout the duodenum. Histopathologic examination revealed massively dilated lamina propria lymphatics in the duodenal biopsies. The patient was diagnosed with primary intestinal lymphangiectasia. He was treated with high-protein and low-fat diet, and supplemental formula high in medium chain triglycerides. On follow-up, the patient's diarrhea completely resolved, and his ascites and edema improved significantly. CONCLUSIONS: The presence of unilateral lower limb edema should not preclude the diagnosis of systemic disorders, and a high index of suspicion is required in atypical presentations. A good knowledge about Primary intestinal lymphangiectasia manifestations, and physical examination skills to differentiate edema or lymphedema from tissue overgrowth can significantly aid in the diagnosis.

A Four-Week-Old Infant With Respiratory Distress: An Emergency Department Case Presentation of Congenital Lobar Emphysema.

Congenital lobar emphysema (CLE) and congenital pulmonary lymphangiectasis (CPL) are rare conditions that are most often identified with prenatal ultrasonography. Occasionally, this disease process is first identified in the emergency department (ED), where the physician should avoid common pitfalls in order to prevent acute decompensation. To the best of our knowledge, there are no prior reports in the emergency medicine literature of CLE or CPL presenting to the ED as undifferentiated respiratory distress in an infant. Here, we describe one such case and then discuss the importance of differentiating these congenital anomalies from more commonly encountered emergency diagnoses, such as pneumothorax and pneumonia. Management differs radically, and the use of chest tubes and positive pressure ventilation in CLE may precipitate acute cardiovascular decompensation.

Therapeutic Lymphatic Embolization in Pediatric Primary Intestinal Lymphangiectasia.

Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. This procedure is typically reserved for adult patients, although as shown in this case, it can be properly performed in children. His serum albumin level was initially 1.5 g/dL, but elevated to 5.0 g/dL after two sessions of lymphatic embolization. Accordingly, we suggest that embolization could potentially be considered a first-line treatment for focal lesions of primary intestinal IL.

Late diagnosis of generalized lymphangiomatosis in a woman presenting with respiratory distress.

Generalized lymphangiomatosis (GLA) is a rare lymphatic abnormality, mostly affects children and young individuals and can be a diagnostic challenge because of wide spectrum of clinical manifestations. A 26-year-old woman presented to the emergency department of our institution with respiratory distress and hypoxia. The patient reported similar episodes for the past 10 years without a definite diagnosis. The imaging study demonstrated findings suggestive of GLA with pulmonary, retroperitoneal and osseous involvements which was confirmed on pathological studies from a lung biopsy. A concise review of the clinical, imaging and pathological findings of GLA is provided in this study. A comprehensive history and physical examination, laboratory and pathological work up and imaging is required to make the diagnosis of GLA. The characteristic imaging findings play an essential role to rule out other possible diagnoses and raise the possibility of GLA.

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.

Penile Lymphangioma: review of the literature with a case presentation.

BACKGROUND: Penile lymphangiomas are rare manifestations of lymphangiomas or lymphatic malformations which are more commonly found in the head or neck region of the body. Lymphangiomas are further categorized as lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, or acquired lymphangiomas (also known as lymphangiectasia), based on their depth and etiology. RESULTS: A literature review revealed only 30 cases of penile lymphangioma between 1947 and March 30, 2018. Several causes were attributed to the acquired penile lymphangiomas, including trauma, phimosis, and infection. While penile lymphangiomas can be initially mistaken for an infection, a thorough history and physical examination is sufficient to clinically diagnose a lymphangioma of the penis. Historically, surgical excision has been the gold standard of treatment for this condition. When asymptomatic, patients may opt for conservative management with avoidance of mechanical trauma alone. Other physicians have revealed novel treatment plans to rid patients of their penile lymphangioma such as a staged laser procedure. CONCLUSION: In this article, we elucidate the causes, symptoms, treatments, and outcomes associated with penile lymphangiomas found in the literature while also presenting the case of a 30-year-old African-American man diagnosed with acquired penile lymphangioma.

CONTEXTE: Les lymphangiomes du pénis sont des manifestations rares des lymphangiomes ou des malformations lymphatiques qui sont plus fréquemment observés dans les régions de la tête ou du cou au niveau du corps. Les lymphangiomes sont aussi classés en lymphangiome circumscriptum, lymphangiome caverneux, hygroma kystique, ou en lymphangiomes acquis (aussi connus sous l'appellation lymphangiectasie), selon leur profondeur et leur étiologie. RÉSULTATS: Une revue de la littérature ne révèle que 30 cas de lymphangiomes du pénis rapportés entre 1947 et Mars 2018. Plusieurs causes ont été attribuées aux lymphangiomes acquis du pénis, parmi lesquelles les traumatismes, le phimosis et les infections. Bien que les lymphangiomes péniens puissent être initialement pris pour une infection, une recherche des antécédents et un examen clinique minutieux sont suffisants pour diagnostiquer cliniquement un lymphangiome du pénis. L'excision chirurgicale a toujours constitué le gold standard du traitement de cette maladie. Lorsqu'elle est asymptomatique, les patients peuvent choisir un traitement conservateur en évitant les seuls traumatismes mécaniques. D'autres médecins ont proposé de nouveaux traitements ayant pour objectif de débarrasser les patients de leurs lymphangiomes péniens, comme la procédure au laser par étapes. CONCLUSION: A partir des données de la littérature, nous avons clarifié dans cet article les causes, symptômes, traitements et résultats associés aux lymphangiomes du pénis ; nous avons aussi présenté le cas d'un homme afro-américain âgé de 30 ans diagnostiqué comme porteur d'un lymphangiome pénien acquis. MOTS-CLÉS: Caverneux, Circumscriptum, Kystique, Hygroma, Lymphangiome, Lymphangiectasie, Pénien, Pénis.

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.

Pulmonary lymphangiectasis presenting as solitary pulmonary coin lesion.

Pulmonary lymphangiectasis is a rare anomaly of infancy characterized by dilatation of pulmonary lymphatic vessels. It is considered to occur exclusively in young children and neonates, and pulmonary lymphangiectasis in asymptomatic adults is rare. Moreover, because chest radiography usually demonstrated the generalized congestive pattern of increased pulmonary vascularity or a reticulonodular pattern, it is extremely unusual for pulmonary lymphangiectasis to be demonstrated as a solitary nodular lesion. There were only two case reports of lymphangiectasis that developed as a nodular shadow in asymptomatic adults in English and Japanese literature. Here, we describe two cases of pulmonary lymphangiectasis that were demonstrated as solitary coin lesions in asymptomatic adults without any extrapulmonary manifestations. An 84-year-old male and a 48-year-old male were found to have a gradually increasing round pulmonary nodule. Chest computed tomography (CT) demonstrated a smooth round nodule on the lung surface, and video-assisted thoracoscopic wedge resection was performed. The nodule was a thin-walled cyst containing yellowish liquid. Histological examination of the resected lung demonstrated lymphangiectasis in the subpleural and interlobular connective tissue and no abnormal cell proliferation. Therefore, we considered that the pulmonary nodule had been formed as a result of local fluid collection in pulmonary lymphangiectasis.

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].

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.

Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states.

Protein losing enteropathy (PLE) has been associated with more than 60 different conditions, including nearly all gastrointestinal diseases (Crohn's disease, celiac, Whipple's, intestinal infections, and so on) and a large number of non-gut conditions (cardiac and liver disease, lupus, sarcoidosis, and so on). This review presents the first attempt to quantitatively understand the magnitude of the PLE in relation to the associated pathology for three different disease categories: 1) increased lymphatic pressure (e.g., lymphangiectasis); 2) diseases with mucosal erosions (e.g., Crohn's disease); and 3) diseases without mucosal erosions (e.g., celiac disease). The PLE with lymphangiectasis results from rupture of the mucosal lymphatics, with retrograde drainage of systemic lymph into the intestinal lumen with the resultant loss of CD4 T cells, which is diagnostic. Mucosal erosion PLE results from macroscopic breakdown of the mucosal barrier, with the epithelial capillaries becoming the rate-limiting factor in albumin loss. The equation derived to describe the relationship between the reduction in serum albumin (CP) and PLE indicates that gastrointestinal albumin clearance must increase by at least 17 times normal to reduce the CP by half. The strengths and limitations of the two quantitative measures of PLE (51Cr-albumin or α1-antitrypsin [αAT] clearance) are reviewed. αAT provides a simple quantitative diagnostic test that is probably underused clinically. The strong, unexplained correlation between minor decreases in CP and subsequent mortality in seemingly healthy individuals raises the question of whether subclinical PLE could account for the decreased CP and, if so, could the mechanism responsible for PLE play a role in the increased mortality? A large-scale study correlating αAT clearance with serum albumin concentrations will be required in order to determine the role of PLE in the regulation of the serum albumin concentration of seemingly healthy subjects.

Gastrointestinal haemorrhage due to lymphangiectasia caused by protein-losing enteropathy in the Fontan circulation.

We report the case of a 14-year-old boy with severe protein-losing enteropathy after Fontan surgery that led to lymphangiectasia, which caused gastrointestinal haemorrhage and required invasive treatment to stop the bleeding. Through this case and a review of the literature on protein-losing enteropathy after Fontan surgery, we highlight a rare and serious presentation of the disease and the difficulties of diagnosis and management.