The present case is the fourth report describing a response to operative resection. [ncbi.nlm.nih.gov]

• Ascites

  Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy. [ncbi.nlm.nih.gov]

  Department of Internal Medicine, Daegu Fatima Hospital, Daegu, Korea Abstract Primary intestinal lymphangiectasia is a rare congenital cause of protein losing enteropathy that is characterized by chronic diarrhea, generalized edema, ascites, hypoproteinemia [e-ce.org]

• Weakness

  A patient initially presented at age 83 with symptoms of watery diarrhea, abdominal distention, 5-Kg-weight-gain, and weakness for one month, and had typical clinical findings of PIL including chylous ascites, pleural effusions, bilateral pitting leg [ncbi.nlm.nih.gov]

  During the last admission, the patient presented with weakness being unable to sit or stand, wasted, frequent crying associated with abdominal colic and distension, loose motions and vomiting with carpopedal spasms (tetany). [saudijgastro.com]

  Case Report A 12-year-old Japanese boy presented to a municipal hospital with a 3-month history of progressive diarrhea, abdominal pain, lower limb edema, tetany, and weakness. He had no relevant family history or medical history. [pediatrics.aappublications.org]

  […] dilatation in more than 75% of IL patients. 8 Similar to our patients, Hashemi et al 8 reported the use of various radiological studies in diagnosing IL, including sonography, CT scan, barium studies, and laparoscopy in a 42-year-old male who presented with weakness [smj.org.sa]

• Leg Edema

  Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. [ncbi.nlm.nih.gov]

  We encountered an 18-year-old woman who suffered from longstanding diarrhea and progressive leg edema. The laboratory findings showed the typical features of this disorder. [e-ce.org]

• Sepsis

  Despite intensive management, the patient expired due to circulatory collapse most probably secondary to severe hypoalbuminaemia and clinical sepsis. The case presented is not unusual in its clinical features. [saudijgastro.com]

  […] literature of diagnoses after the age of 3, such as the study published by Suresh et al. in 2008. 14 In all the cases, the clinical manifestations were characterized by diarrhea, edema of the extremities, and ascites; 2 of the patients presented with severe sepsis [revistagastroenterologiamexico.org]

  […] literature of diagnoses after the age of 3, such as the study published by Suresh et al. in 2008.14 In all the cases, the clinical manifestations were characterized by diarrhea, edema of the extremities, and ascites; 2 of the patients presented with severe sepsis [zdoc.site]

• Lymphadenopathy

  Computerised abdominal tomography showed a tumour-like thickened proximal ileum with no evidence of mesenteric lymphadenopathy. An x ray and CT scan of the chest were normal, as were hepatic and medullar biopsies. [gut.bmj.com]

  There were no hilar or mediastinal lymphadenopathy, and no pleural or pericardial effusion. These changes are consistent with pulmonary lymphangiectasia ( Figure 4 ). The patient was managed with albumin infusion and diuretics. [smj.org.sa]

• Aspiration

  The fine needle aspiration performed during an echoendoscopy, permitted the complete aspiration of the fluid and the cytological analysis that showed numerous lymphocytes. [oapublishinglondon.com]

  The work up to rule out a secondary cause for the protein-losing enteropathy, in addition to what has been mentioned above, included the duodenal aspirate which was negative for Giardia lamblia. [saudijgastro.com]

• Steatorrhea

  Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common [ncbi.nlm.nih.gov]

  Symptoms of Intestinal Lymphangiectasia swelling of the lower limbs, also known as edema mild diarrhea or steatorrhea weight loss, fatigue, and abdominal pain Diagnosis of Intestinal Lymphangiectasia Endoscopic examination Blood tests indicate a significant [ddc.musc.edu]

  Clinical presentation: The patient may be asymptomatic or present as vague abdominal pain, chronic diarrhea, steatorrhea, edema, chylous pleural effusion, ascites, hypoproteinemia, lymphocytopenia or protein-losing enteropathy. [histopathology-india.net]

  In some patients, vague abdominal discomfort or steatorrhea can be noted and, in cases of severe enteric protein loss, intestinal obstruction, chylous ascites, hypocalcemic tetany or growth retardation may develop 10 ). [kjim.org]

  In some, steatorrhea was also present. Biopsies of the small bowel are needed to confirm the diagnosis[ 2 ]. [wjgnet.com]

• Acute Abdomen

  […] and chronic gastrointestinal diseases, including acute abdomen, appendicitis, diverticulitis, inflammatory bowel diseases, neoplasms and masses, infections, malabsorption syndromes, and rare conditions. [books.google.com]

  During the course of the illness, the patient presented once with symptoms of acute abdomen with visible peristalsis. Intussusception was suspected clinically and radiologically, laparotomy was done but no intussusception was found. [saudijgastro.com]

  Some patients may even present with pleural effusion, chylous ascites, jejunal ileus or acute abdomen [ 2, 4 ]. Thus, taking the possible differential diagnosis of PIL into consideration is a major challenge. [academic.oup.com]

  Primary intestinal lymphangiectasia: A rare disease in the differential diagnosis of acute abdomen. J Pediatr Gastroenterol Nutr, 33 (2001), pp. 508-510 [16] T. Waldmann. Protein-losing enteropathy. Gastroenterology, 50 (1966), pp. 422-443 [17] M. [revistagastroenterologiamexico.org]

  Primary Intestinal Lymphangiectasia: a rare disease in the differential diagnosis of acute abdomen. J Pediatr Gastroenterol Nutr. 2001;33(4):508–510. doi: 10.1097/00005176-200110000-00019. [PubMed] [CrossRef] [Google Scholar] 6. [ncbi.nlm.nih.gov]

• Intestinal Disease

  TUBE DIGESTIF EXPLORATION RADIOLOGIQUE HOMME PATHOLOGIE DIGESTIVE GASTROENTEROLOGIE Keyword (en) LYMPHANGIECTASIS SMALL INTESTINE PRIMITIVE LOSING PROTEINS GUT LYMPHOGRAPHY FIBROSCOPY DUODENUM COLOR PHOTOGRAPHY BIOPSY ILEUM DIAGNOSTIC INTESTINAL DISEASE [pascal-francis.inist.fr]

  Depending on the cause of the disease, it can be classified into primary intestinal lymphangiectasia (PIL) or secondary intestinal lymphangiectasia, so the diagnosis of PIL must first exclude the possibility of secondary IL, e.g. other intestinal diseases [oapublishinglondon.com]

  Whipple’s disease from Tropheryma whipplei ) or inflammatory disorders (e.g. Crohn’s disease) may also present with profound changes in intestinal permeability and protein-losing enteropathy that also require exclusion. [wjgnet.com]

• Recurrent Abdominal Pain

  A 3-year-old Saudi girl (triplet III) was apparently well till the age of 18 months when she presented with a long history of edema of eyelids associated with chronic diarrhea, recurrent abdominal pain and vomiting. [saudijgastro.com]

• Erythema

  - Necrolytic migratory erythema can be seen [ 18 ]. - Recurrent hemolytic uremic syndrom e has been described[ 19 ]. - Osteomalacia resulting from vitamin D deficiency was reported in a 63-year-old woman [ 20 ]. - It remains open to debate whether recurrent [lymphedemapeople.com]

• Alopecia

  Intestinal lymphangiectasia: presentation in pregnancy and association with herpes zoster and alopecia. Indian J Gastroenterol 1997;16:153–4. [7]. Voigt HJ, Sailer D, Kolb S, et al. [journals.lww.com]

• Carpopedal Spasm

  During the last admission, the patient presented with weakness being unable to sit or stand, wasted, frequent crying associated with abdominal colic and distension, loose motions and vomiting with carpopedal spasms (tetany). [saudijgastro.com]

• Muscle Spasm

  […] tetany (tĕt`ənē), condition of mineral imbalance in the body that results in severe muscle spasms. Tetany occurs when the concentration of calcium ions (Ca ++ ) in extracellular fluids such as plasma falls below normal. [encyclopedia2.thefreedictionary.com]

• Seizure

  The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. [ncbi.nlm.nih.gov]

  Seizure 0001250 Thickened skin Thick skin 0001072 Vaginal lymphocele 0100676 Verrucae Warts 0200043 Percent of people who have these symptoms is not available through HPO Abnormal hair morphology Abnormality of the hair Hair abnormality [ more ] 0001595 [rarediseases.info.nih.gov]

  In general, these syndromes are easily distinguishable by the presence of facial abnormalities (Turner, Noonan, Hennekam), mental retardation (Hennekam, Noonan), seizures (Hennekam), severe limb and/or face lymphedema (Hennekam), neurofibromas and other [lymphedemapeople.com]

• Giardia Lamblia

  The work up to rule out a secondary cause for the protein-losing enteropathy, in addition to what has been mentioned above, included the duodenal aspirate which was negative for Giardia lamblia. [saudijgastro.com]

• Polyps

  Review the latest developments in the field and get up-to-date clinical information on hot topics like polyps, capsule endoscopy, and pancreatic treatments. [books.google.com]

  Upper gastrointestinal endoscopy showed multiple whitish pseudo polyps, suggesting lymphangiectasia, extending from the proximal duodenum to Treitz's ligament. Ileocolonoscopy proved normal. [gut.bmj.com]

  Protein-losing gastroenteropathy and gastric polyps: successful treatment by Helicobacter pylori eradication. Digestion 2007; 75 :99. 18. Settle SH, Washington K, Lind C et al. [nature.com]

  Intestinal lymphangiectasia and colonic polyps: Surgical intervention. J Pediatr Surg, 14 (1979), pp. 530-532 [30] J. Seok, J. Seong, H. Sun, et al. Protein-losing enteropathy detected on Tc-99m HAS and Tc-99m MDP scintigraphy. [revistagastroenterologiamexico.org]

  Intestinal lymphangiectasia and colonic polyps: Surgical intervention. J Pediatr Surg. 1979;14:530---2. 30. Seok J, Seong J, Sun H, et al. Protein-losing enteropathy detected on Tc-99m HAS and Tc-99m MDP scintigraphy. [zdoc.site]

Duration of the octreotide treatment changed between 3 and 37 months. Stool frequency decreased in all of the patients after starting octreotide treatment. Serum albumin could be maintained at normal levels in 3 patients. [ncbi.nlm.nih.gov]

There is no consensus on the treatment of this condition. Treatment is generally symptomatic and may include nutritional therapy and replacement therapy. [pediatrics.aappublications.org]

Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined. [ncbi.nlm.nih.gov]

Treatment and prognosis Treatment has usually comprised of a low fat diet along with a medium-chain triglyceride oral supplements. History and etymology It was first described by TA Waldmann in 1961 1. [radiopaedia.org]

Prognosis - Waldmann disease Not supplied. Treatment - Waldmann disease The main treatment for PIL is a low-fat diet with medium-chain triglyceride (MCT) supplements. [checkorphan.org]

Prognosis The clinical course of intestinal lymphangiectasia is highly variable with about 23% of patients showing improvement and 64% remaining unchanged; the mortality rate is 13%. [emedicine.medscape.com]

Etiological work-up reveals pleural and pericardial tuberculosis. The clinical aggravation of an enteropathy, particularly in adulthood, requires a search for a secondary etiology. Tuberculosis should be sought systematically. [ncbi.nlm.nih.gov]

It can occur from a number etiologies which include. primary/congenital form : primary idiopathic intestinal lymphangiectasia / Waldmann disease 5 secondary form : resulting from a tumor, fibrosis, or another cause of lymphatic obstruction. [radiopaedia.org]

Epidemiology Because the condition is so multifactorial, the prevalence rate is not known. [patient.info]

[…] also be affected. [4] Etiology The following conditions can cause intestinal lymphangiectasia: Abdominal or retroperitoneal carcinoma Mesenteric tuberculosis Retroperitoneal fibrosis Intestinal endometriosis Sclerosing mesenteritis Lymphenteric fistula Epidemiology [emedicine.medscape.com]

Epidemiology The prevalence of clinically overt PIL is unknown. However, PIL can be asymptomatic; it primarily affects children (generally diagnosed before 3 years of age) and young adults but may be diagnosed later in adults[ 2, 3 ]. [lymphedemapeople.com]

Since such condition is extremely rare, no precise classification and pathophysiological explanation are so far available. We observed a case of duodenal lymphangiectasia associated to a lymphangioma of the 4th finger of the left hand. [ricerca.univaq.it]

Lower Digestive Tract, 2nd Edition, part 2 in the 3-book Digestive System volume, covers the small bowel and colon, and provides a concise and highly visual approach from normal anatomy and physiology through pathophysiology, diagnostics and treatment [books.google.com]

Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Vol. 2. 5th ed. Philadelphia: WB Saunders Co; 1993:1379-1393. [consultant360.com]

[…] include diarrhea, nausea, swelling of the legs, protein-losing enteropathy, immunodeficiency and loss of lymphatic fluid into the intestines. [2] [4] It is usually diagnosed before the patient is 3 years old, but it is sometimes diagnosed in adults. [3] Pathophysiology [en.wikipedia.org]

Morbidity/mortality Morbidity is related to the pathophysiology of this disease. [emedicine.medscape.com]

The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. [ncbi.nlm.nih.gov]

Can it be prevented? As doctors do not know what causes PIL, there is no way it can be prevented. When should you seek medical help? A child should be taken to the doctor if parents notice any swelling in the legs. [gosh.nhs.uk]

It is likely that the absence of fat in the diet prevents engorgement of the intestinal lymphatics with chyle, thereby preventing their rupture that results in protein and T-cell loss. [2] Thus, a low-fat diet with supplemental MCT forms the cornerstone [jdeonline.in]

Prevention - Waldmann disease Not supplied. Diagnosis - Waldmann disease Diagnosis of PIL is often made in childhood, usually before age 3. The presence of PIL is suggested by edema of the legs. [checkorphan.org]

This prevented expression of the CD55 protein, which contributes to immune regulation by inhibiting complement activation. [healio.com]