Lymphangiectasia Research Articles

Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome

Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The Linfangiectasia intestinal en un paciente afectado de sindrome de Sanfilippo B Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.

Clinical and pathological features of 16 cases of childhood bullous pilomatricoma

Objective To analyze the clinical and pathological features of childhood bullous pilomatricoma. Methods The clinical and pathological features were analyzed in 16 patients with bullous pilomatricoma, who visited Department of Dermatology of Beijing Children′s Hospital from 2013 to 2017. Results Among the 16 patients, 5 were males, and 11 were females. Their age of onset ranged from 4 months to 11 years, and the median age of onset was 8.5 years. Their course of disease ranged from 2 months to 4 years, and the average course of disease was 10 months. The tumors were found predominantly on the upper limbs (10 cases, including 7 on the upper arm, 2 on the shoulder and 1 on the forearm) , followed by the face (4 cases) and the neck (2 cases) . These tumors manifested as limited pushable red lumps with blister-like appearance, and telangiectasia was observed on the surface of some lesions. The diameters of the lumps ranged from 0.5 cm to 3 cm, and hard nodules could be detected in the blisters by palpation. Under dermoscopy, uniform red background was observed in 16 cases, unstructured white area in 13, unstructured blue-grey area in 4, and liner or irregular branched vessels. Two or more dermoscopic features were observed in 15 patients. All the skin lesions were resected by surgery, and no recurrence was observed during the follow-up of 1 - 5 years. Histopathological examination showed that the tumors were located in the middle to deep dermis, and mainly consisted of basaloid cells and shadow cells, as well as transitional cells between the above two kinds of cells. Varying degrees of infiltration of inflammatory cells and hyperplasia and calcification of the fibrous connective tissue were observed in the tumor interstitium, with multinucleated giant cells in some areas. There were varying degrees of infiltration of inflammatory cells, lymphangiectasis, reduction or absence of elastic fibers in the dermis between the epidermis and tumors. Conclusions Childhood bullous pilomatricoma mostly occurs on the upper limbs, face and neck. Histopathologically, the tumor consists of basaloid cells and shadow cells with lymphangiectasis and reduction of elastic fibers in the dermis. The main dermoscopic features are red background and unstructured white areas. Its prognosis is good after surgical excision. Key words: Pilomatrixoma; Child; Dermoscopy; Skin manifestations; Pathologic processes; Bullous pilomatricoma

Primary intestinal lymphangiectasia in children

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. Key words: Lymphangiectasis; Intestinal; Lymphatic radionuclide imaging; Child

Management of pregnancy associated with intestinal lymphangiectasia

Management of pregnancy associated with intestinal lymphangiectasia

Bilateral renal lymphangiectasia: A case of cystic rarity

Renal lymphangiectasia is a benign, rare disorder of lymphatic system of the kidneys, characterized by dilatation of the perirenal lymphatics. This condition closely mimics other cystic lesions of the kidney such as polycystic kidney disease. The diagnosis can be made with classic radiological finding and other laboratory analysis. We report a 3-year-old male boy, a case of bilateral renal lymphangiectasia, diagnosed on ultrasonography and computed tomography typical imaging findings and the laboratory analysis of aspirated fluid.

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

Clinical characteristics and etiology analysis of 223 children undergoing colonoscopy

Objective To investigate the clinical characteristics and etiology in infants undergoing colonoscopy in order to improve the understandings of lower gastrointestinal tract diseases and their endoscopic manifestations. Methods The clinical and endoscopic data of the infants aged ≤3 years old who underwent conoloscopy at the Department of Gastroenterology, Children′s Hospital of Chongqing Medical University, from July 2010 to December 2014, were retrospectively analyzed.A total of 223 children were included, 148 male and 75 female.The age range was from 26 d to 3 years old(<6 months: 41 cases; 6-12 months: 68 cases; 1-3 years old: 114 cases). Results A total of 235 colonoscopies were performed under deep sedation by single or double manipulators.Cecal intubation was successful in 192 colonoscopies and terminal ileal intubation was completed in 29 colonoscopies, with a high success rate of 94.0%(221/235 colonoscopies). The main symptoms included hematochezia(124 cases, 55.6%), persistent/chronic diarrhea(55 cases, 24.7%), hematochezia with chronic diarrhea(22 cases, 9.5%), and others(22 cases, 9.5%). Of the 223 patients, clear diagnosis were established for 215 children(96.4%), with food protein-induced proctocolitis (FPIPC) (78/223 cases, 35.0%), colonic polyps (50/223 cases, 22.4%), colitis(29/223 cases, 13.0%), antibiotic associa-ted diarrhea (AAD) (19/223 cases, 8.5%), FPIPC with AAD (10/223 cases, 4.5%). Conclusions Colonoscopy serves as a very important tool for the accurate diagnosis of lower gastrointestinal diseases with hematochezia and/or chronic diarrhea.FPIPC and colonic polyps are the most common causes for hematochezia and/or chronic diarrhea.AAD may be another important cause of chronic diarrhea and bloody stool in infants.Moreover, ghe application of colonoscopy in combination with histopathology can also play an important role in the diagnosis of some rare diseases, such as intestinal tuberculosis, primary intestinal lymphangiectasia, Behcet′s disease and primary immunodeficiency disease. Key words: Infant; Colonoscopy; Hematochezia; Chronic diahrrhea

Current Application of Dietary or Enteral Medium-Chain Triglycerides in a Chinese General Hospital: A Preliminary Investigation and Evidence-Based Evaluation

Background: Medium chain triglycerides (MCTs)are of nutritional interest for unique properties on ingestion, absorption and metabolism. However, the current situation of dietary and enteral MCTs application in Chinese hospitalized patients has been seldom investigated or evaluated. Methods: A retrospective analysis was conducted on 46 hospitalized patients who were administered MCTs therapy between January, 2012 and December, 2013 in Peking Union Medical College Hospital. The clinical parameters of subjects were evaluated 2 weeks after intervention. All indications and outcomes of MCTs therapy underwent evidence-based evaluation. A survey was conducted on a random sample of 77 doctors to clarify the awareness and knowledge of MCTs therapy among clinicians. Results: Among the total 46 cases undergoing MCTs therapy, there were 21 cases of gastrointestinal dysfunction (with improvement in 15 cases), 15 cases of lymphatic anomalies (with improvement in 7 cases), 5 cases of dyslipidemia (with improvement in 3 cases), 4 cases of exocrine pancreatic insufficiency (with improvement in 2 cases) and 1 case of epilepsy (without improvement). All indications were in agreement with evidence from previous literature. Although MCTs were utilized in an increasing number of patients, the survey revealed poor knowledge on physiochemical properties, medical indications and therapeutic mechanisms of MCTs among clinicians. Conclusions: MCTs therapy has beneficial effects on management of gastrointestinal malabsorption, pancreatic exocrine insufficiency, intestinal lymphangiectasia and dyslipidemia. Randomized control trials with larger samples and longer follow-ups are required to further testits efficacy, and more educational programs are needed to expand knowledge of MCTs therapy for clinicians.

Vulvar Lymphangiectasia in Crohn�s Disease

Vulvar Lymphangiectasia in Crohn�s Disease

The importance of histologic parameters of lacteal involvement in cases of canine lymphoplasmacytic enteritis.

The most frequent form of inflammatory bowel disease (IBD) in dogs is represented histologically, by lymphoplasmacytic enteritis (LPE), a histological category, often associated with other morphologic alterations including lymphangiectasia (LE). However, literature data on this latter topic are quite scarce and have mostly been obtained in single reports or in small series. We evaluated some morphologic parameters of intestinal villi and lacteals in a large cohort of dogs, and correlated them with serum albumin and cholesterol values. We investigated 136 dogs (94 with LPE, and 42 with gastrointestinal problems different from IBD) and analyzed their clinical, laboratory (albumin and cholesterol values), endoscopic, and histologic variables. The LPE group showed significantly impaired clinical, laboratory, endoscopic, and histologic variables compared to controls. Affected dogs showed significant correlations between canine inflammatory bowel disease activity index (CIBDAI) scores and endoscopic and histologic variables. Moreover, the grade of hematologic changes were strongly related to the intestinal histologic variables, in particular those concerning villous and lacteals morphology. Dogs with LPE had intestinal histologic abnormalities (height, width, height/width ratio, calculated for both villi and lacteals), whose degree correlated with the severity of hypoalbuminemia and hypocholesterolemia. Evaluation of endoscopic and histologic variables in association to the clinical findings may reveal useful insights for the pathogenesis of LPE and, hopefully, might lead to more targeted therapeutic approaches.

Primary chylous reflux syndrome I : a case report

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. Key words: Chylous reflux syndrome; Vaginal fistula; Chyle; Lymphangiectasis

Diagnositic value and image classification of 99Tcm-DX lymphoscintigraphy in intestinal lymphangiectasia

Objective To investigate the image classification and diagnostic value of 99Tcm-DX lymphoscintigraphy in intestinal lymphangiectasia (IL).Methods A total of 68 patients (the study group) with clinical suspicion of IL from June 2007 to September 2012 were retrospectively analyzed.Using a stratified sampling method,60 patients with lower limb lymphedema or chylous ascites (the control group) were selected by age-and gender-matched with the study group.All patients underwent 99Tcm-DX lymphoscintigraphy.The lymphoscintigraphic findings were classified according to the imaging characteristics in abdomen.The intestinal tract could be shown on the images of 99Tcm-DX lymphoscintigraphy was the diagnostic standard of IL.Results According to the histopathology results,60 cases of the study group were finally diagnosed as IL and 8 cases were excluded.Five patterns of 99Tcm-DX images in abdomen were classified as following:pattern Ⅰ,consecutive visualization of intestine; pattern Ⅱ,delayed visualization of intestine; pattern Ⅲ,accumulation in ascites ; pattern Ⅳ,hybrid; pattern Ⅴ,negative results.The cases of Ⅰ,Ⅱ,Ⅲ,ⅣandⅤin the study group and control group were 19 vs 0,17 vs 4,11 vs 13,6 vs 0 and 7 vs 43,respectively.The sensitivity,specificity,accuracy,positive predictive value and negative predictive value of 99Tc m-DX lymphoscintigraphy in diagnosing IL were 70.0％ (42/60),94.1％ (64/68),82.8 ％ (106/128),91.3％(42/46) and 78.0％(64/82),respectively.Conclusions 99Tcm-DX lymphoscintigraphy may be an important diagnostic method for IL,and the visualization of intestine is an important evidence for the diagnosis of IL. Key words: Lymphangiectasis, intestinal; Radionuclide imaging; Dextrans

Clinical observation of lymphangiectasis in conjunctivochalasis cases

To investigate the relationship between the occurrence and development of conjunctivochalasis and bulbar conjunctival lymphangiectasia. Case control study. One hundred cases with conjunctivochalasis treated from January to March 2012 were selected to study, and 100 cases with no conjunctivochalasis as the control group at the same time. To observe bulbar conjunctiva lymphatic duct dilatation using slit lamp microscope, analysis bulbar conjunctiva and fascia images by OCT scanning, and ablate lymphatic of conjunctival tissue for pathologic examine. Twenty-nine eyes of the bulbar conjunctiva lymphangiectasia associated with 100 cases (183 eyes) conjunctivochalasis patients, accounting for 15.84%; 8 eyes of the ball conjunctival lymphatic dilation in control group of 100 cases ( 200 eyes), accounting for 4.00%. The difference between the two groups was statistically significant (χ(2) = 15.36, P < 0.001). OCT scanning showed that lymphangiectasia of the conjunctiva is at the subcutaneous mainly, some in the conjunctival lamina propria. They are border-clear, full-filled fluid, single-lumen or multi lumens, not involving the fascia. The histopathological examination showed that the lamina propria of the bulbar conjunctiva mildly chronic inflammatory changes accompanied by a large number of lymphangiectasia. Bulbar conjunctival lymphangiectasia may be one of the reasons for the conjunctivochalasis.

Primary intestinal lymphangiectasia in 8 infants

Objective To improve the diagnosis and treatment level of primary intestinal lymphangiectasia(PIL) in infants. Methods Clinical, laboratory, gastroscopy imaging, lymph radionuclide imagining, and therapeutic intervention data in eight patients admitted with PIL in Beijing Children's Hospital from Jan.2007 to Feb.2012 were reviewed. Results Their ages ranged from 4 to 8 months old.The common complaints were edema(8/8 cases), diarrhea(8/8 cases), infection(8/8 cases), and ascites(7/8 cases). Other symptoms included vomiting(5/8 cases), low body weight(2/8 cases), and convulsions(2/8 cases). Infections involving respiratory tract, blood, and gastrointestinal system were prominent.The causal pathogens for the infections were bacteria, virus, fungi, and ectosarc.The laboratory abnormalities included lymphocytopenia(8/8 cases), hypoalbuminemia(8/8 cases), and hypogammaglobulinemia(8/8 cases). On immunologic evaluation, the CD4 cell counts and serum IgG levels were significantly decreased(7/7 cases) while B cell and NK cell counts were normal.The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots(8/8 cases). Further pathological examinations indicated dilated lymphatic channel in mucosal and submucosal(5/8 cases). Lymph radionuclide imaging discovered abnormalities consistent with PIL in 6 out of 8 patients, including 3 cases that were negative for pathological examination.A multidisciplinary approach was taken to treat each patient.The low-fat and medium chain triglycerides-rich diet was introduced with supplements of albumin and globulin.Infection control and edema alleviation were well managed through medication.However, diarrhea remained a problem.Only 2 out of 8 patients had recovered from hypoalbuminemia and lymphocytopenia.One case had a complete remission after surgical therapy withno relapse. Conclusions PIL in infant has common clinical presentations with an exception of more severe infection.Lymph radionuclide imaging is a sensitive diagnostic method.Medication treatment for infant PIL is not satisfactory.Surgical management is recommended if the locus of pathology is confined. Key words: Primary intestinal lymphangiectasia; Lymph radionuclide imaging; Infant

A Case of Primary Intestinal Lymphangiectasia Diagnosed by Double Balloon Enteroscopy

54세 남자 환자는 수년 전부터 악화와 호전을 반복하는 하지 부종이 있었으며, 최근 증상이 악화되어 타 병원에서 시행한 혈액 검사 결과 저알부민혈증을 보이고 소장조영술 검사에서 소장 점 막 주름의 비후가 있어 소장내시경을 위해 본원으로 전원되었 다. 과거력 및 가족력에서 특이소견은 없었다. 신체검사에서 신 장 164 cm, 체중 62 kg, 혈압 110/70 mmHg, 맥박수 80회/분, 호 흡수 18회/분, 체온 36.5°C였다. 전신 상태는 만성 병색을 보였 으며, 흉곽은 함몰없이 대칭적이었고 간, 비장 등은 촉지되지 않 았다. 복부는 팽만, 이동탁음, 경한 함요 부종 소견을 보였고, 하 지의 함요 부종도 관찰되었다. 신경학적 소견은 정상이었다. 양 쪽 폐음은 깨끗하였고 심음은 규칙적이었으며 심잡음은 들리지 않 았다. 혈액검사에서 백혈구수 6,000/mm, 림프구수 1,100/μL (18.5%), 혈색소 11.9 g/dL, 헤마토크릿 35.2%, 혈소판 374,000/ mm, 생화학검사에서 총 단백 3.4 g/dL, 알부민 2.3 g/dL, 총 빌리 루빈 0.1 mg/dL, AST/ALT 26/30 IU/L, alkaline phosphatase/γGT 38/6 IU/L, BUN/creatinine 25.8/1.0 mg/dL, 칼슘 8.2 mg/ dL, 인 3.1 mg/dL, 마그네슘 2.3 mg/dL, 나트륨/칼륨 142/4.5 mEq/L, PT/PTT 13.0 (international normalized ratio 1.02)/35.1 초, triglyceride 64 mg/dL, cholesterol 100 mg/dL이었다. 요단백 및 신기능검사, 대변내 기생충 검사, 혈청 면역글로불린치는 모두 정상 범위였다. 상부위장관내시경검사, 대장내시경검사에서 특이소견 없 A Case of Primary Intestinal Lymphangiectasia Diagnosed by Double Balloon Enteroscopy

Jejunal lymphangioma: rare case of GI bleeding

A 20-year-old Thai man presented with melena of 1 week's duration, unaccompanied by abdominal pain or other alarming symptoms. On admission, hemoglobin and hematocrit were 6.2 g/dL and 20.6%, respectively. EGD and colonoscopy had normal results. Double-balloon enteroscopy was subsequently performed by the oral route to a distance of about 360 cm from the incisors. A 15-cm segment of distal jejunum was seen to have a nearly circumferential abnormality, including edematous mucosa, thickened folds, submucosal hemorrhage, and segmental lymph angiectasia with oozing of blood (A).

Acquired lymphangiectasia of the vulva

Acquired lymphangiectasia of the vulva

Lymphangiectasia of the vulva, treatment with CO2laser

Lymphangiectasia of the vulva, treatment with CO₂laser

Primary intestinal lymphangiectasia

Primary intestinal lymphangiectasia is a rare disease, which is characterized by the obstruction of lymphatic return, dilated lymphatics and distorted structure of villi. Lymph fluid leaks into the small bowel lumen as a result of the obstruction of lymphatics and elevated pressure of lymphatics, and leads to malabsorption and loss of proteins. The causes and pathogenesis of PIL are not clear, hut the abnormal immunologic mechanism has got people's attention. Recently, the endoscopic technique has been improved, and the diagnosis rate of PIL has increased obviously. Antisecosis is the basic and effective treatment, especially for children. Key words: Primary intestinal lymphangiectasia; Child; Diarrhea; Edema

A Case of Lymphangiectasia of Both Forearms

A Case of Lymphangiectasia of Both Forearms