Primary intestinal lymphangiectasia: is it always bad? Two cases with different outcome.

Mar 2013

Xinias I, Mavroudi A, Sapountzi E, Thomaidou A, Fotoulaki M, Kalambakas A, Karypidou E, Kollios K, Pardalos G,Imvrios G.

Source

Third Pediatric Department, Papageorgiou Hospital, Thessaloniki, Greece.

Abstract

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema,lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.

KEYWORDS:

Children,,Outcome, Primary intestinal lymphangiectasia, Protein-losing enteropathy, Wireless capsule endoscopy

PubMed

Primary intestinal lymphangiectasia diagnosed by double-balloon enteroscopy and treated by medium-chain triglycerides: a case report.

Jan 2013

Lai Y, Yu T, Qiao XY, Zhao LN, Chen QK.

Abstract

INTRODUCTION: 

Primary intestinal lymphangiectasia is a disorder characterized by exudative enteropathy resulting from morphologic abnormalities of the intestinal lymphatics. Intestinal lymphangiectasia can be primary or secondary, so the diagnosis of primary intestinal lymphangiectasia must first exclude the possibility of secondary intestinal lymphangiectasia. A double-balloon enteroscopy and biopsy, as well as the pathology can be used to confirm the diagnosis of intestinal lymphangiectasia. A polymeric diet containing medium-chain triglycerides and total parenteral nutrition may be a useful therapy.

CASE PRESENTATION:

A 17-year-old girl of Mongoloid ethnicity was admitted to our hospital with a history of diarrhea and edema. She was diagnosed with protein-losing enteropathy caused by intestinal lymphangiectasia. This was confirmed by a double-balloon enteroscopy and multi-dot biopsy. After treatment with total parenteral nutrition in hospital, which was followed by a low-fat and medium-chain triglyceride diet at home, she was totally relieved of her symptoms.

CONCLUSION:

Intestinal lymphangiectasia can be diagnosed with a double-balloon enteroscopy and multi-dot biopsy, as well as the pathology of small intestinal tissue showing edema of the submucosa and lymphangiectasia. Because intestinallymphangiectasia can be primary or secondary, the diagnosis of primary intestinal lymphangiectasia must first exclude the possibility of secondary intestinal lymphangiectasia. A positive clinical response to the special diet therapy, namely a low-fat and medium-chain triglyceride diet, can further confirm the diagnosis of primary intestinal lymphangiectasia.

Journal of Medical Case Reports

A case of enteric muco-submucosal elongated polyp.

2012

Nishimura J, Nishikawa J, Tanabe R, Hashimoto S, Matsunaga T, Nakamura M, Goto A, Hamabe K, Okamoto T, Sakaida I.

Source

Department of Gastroenterology and Hepatology, Yamaguchi University Graduate School of Medicine.

Abstract

A woman in her seventies was admitted because of black stool. Esophagogastroduodenoscopy and colonoscopy revealed no bleeding lesions. Balloon endoscopy showed a long and slender polyp. The polyp was covered with normal mucosa and accompanied with ulcerations. The long polypoid lesion was found to be located in the duodenojejunal flexure by a small bowel series. We performed endoscopic polypectomy. The polyp was composed of edematous mucosa and submucosa with dilated blood vessels and lymphangiectasia. The histologic findings of resected specimen were compatible with colonic muco-submucosal elongated polyp. The polyp was considered to be classified as enteric muco-submucosal elongated polyp.

jStage

See also:

Lymphangiectasia Online Yahoo Support Group

Intestinal Lympheangiectasia

Pulmonary Lymphangiectasia

Hennekam Syndrome

Hennekam Lymphangiectasia Syndrome

Hennekam Syndrome  

 

Hennekam lymphangiectasia syndrome

Oct-Dec 2011 - Just released PubMed

G. Lakshminarayana, A. Mathew, R. Rajesh, G. Kurien, and V. N. Unni

Abstract

Key Words:  Facial and dental anomalies, renal lymphangiectasia, Hennekam lymphangiectasia syndrome

Hennekam lymphangiectasia syndrome is a rare disorder comprising of intestinal and renal lymphangiectasia, dysmorphic facial appearance and mental retardation. The facial features include hypertelorism with a wide, flat nasal bridge, epicanthic folds, small mouth and small ears. We describe a case of a multigravida with bad obstetric history and characteristic facial and dental anomalies and bilateral renal lymphangiectasia. To our knowledge this is the first case of Hennekam lymphangiectasia syndrome with anodontia to be reported from India.

Introduction

Hennekam Lymphangiectasia syndrome is a rare disorder characterized by presence of intestinal and renal lymphangiectasia, dysmorphic facial appearance and mental retardation. The facial features include hypertelorism with a wide, flat nasal bridge, epicanthic folds, small mouth and small ears. This syndrome thought to be due to developmental disorder of the lymphatics. The synonym for this disease is multiple congenital anomaly/mental retardation (MCA/MR) syndrome.

Complete Full Text

NIH - Indian Journal of Nephrology

Liquid Nitrogen Cryotherapy for Conjunctival Lymphangiectasia: A Case Series

Dec 2009 - Just released

Frederick W. Fraunfelder, MD^*

^Abstract

Purpose:

To report a case series of conjunctival lymphangiectasia treated with liquid nitrogen cryotherapy.

Methods:

A 1.5-mm Brymill cryoprobe was applied in a double freeze-thaw method after an incisional biopsy of a portion of the conjunctiva in patients with conjunctival lymphangiectasia. Freeze times were 1 to 2 seconds with thawing of 5 to 10 seconds between treatments. Patients were reexamined at 1 day, 2 weeks, 3 months, 6 months, and yearly following cryotherapy.

Results:

Five eyes of 4 patients (3 male and 1 female) with biopsy-proven conjunctival lymphangiectasia underwent liquid nitrogen cryotherapy. The average patient age was 53 years. Ocular examination revealed large lymphatic vessels that were translucent and without conjunctival injection. Subjective symptoms included epiphora, ocular irritation, eye redness, and occasional blurred vision. After treatment with liquid nitrogen cryotherapy, the patients’ symptoms and signs resolved within 2 weeks. Lymphangiectasia recurred twice in one patient, at 1 and 3 years postoperatively. In another patient, lymphangiectasia recurred at 6 months. The average time to recurrence in these 3 eyes was 18 months. Average length of follow-up was 24.5 months for all subjects.

Conclusion:

Liquid nitrogen cryotherapy may be an effective surgical alternative in the treatment of conjunctival lymphangiectasia. Cryotherapy may need to be repeated in some instances.

Complete Text with Images

NCBI-NLM-NIH

Surgical Therapy of Segmental Jejunal, Primary Intestinal Lymphangiectasia.

Dec 2012

[Article in German]

Kneist W, Drescher DG, Hansen T, Kreitner KF, Lang H.

Source

Klinik und Poliklinik für Allgemein- und Abdominalchirurgie, Universitätsmedizin der Johannes-Gutenberg-Universität, Mainz.

Abstract

 

Key Words: primary intestinal lymphangiectasia - chylaskos - protein-losing gastroenteropathy - small bowel resection

Primary intestinal lymphangiectasia (PIL) is a protein-losing, exsudative gastroenteropathy causing lymphatic obstruction. Diagnosis depends on clinical examination and histological findings. Conservative treatment modalities include a low-fat diet and enteral nutritional therapy in order to reduce enteric protein loss and to improve fat metabolism. Other treatment options consist of administration of antiplasmin or octreotide to lower lymph flow and secretion. We report on a 58-year-old patient who underwent exploratory laparotomy due to a worsening physical status, recurrent chylaskos and leg oedema under conservative dietary therapy. Intraoperative findings showed a typical PIL of the jejunum about 20 cm distal to the Treitz's ligament. Histological examinations confirmed this diagnosis. One year after segmental small bowel resection (105 cm) with end-to-end anastomosis the patient is healthy, free of symptoms, has gained weight and his serum protein level has increased. Intraabdominal ascites and leg oedema (lymphedema) have not reoccurred since.

Thieme Journal

Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

Nov 2012

Al Sinani S, Rawahi YA, Abdoon H.

Source

Siham Al Sinani, Yusria Al Rawahi, Hamed Abdoon, Child Health Department, Sultan Qaboos University Hospital, Muscat 123, Sultanate of Oman.

Abstract

A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.

World Journal of Gastroenterology

Abdominal Lymphatics

Abdominal Lymphatics

We always mention the abdominal lymphatics when discussing lymphangiectasia.

Click on the below for a good image spectrum of those vessels.

RadioGraphics

This page gives a good discussion as well:

The Lymph Glands of the Abdomen and Pelvis

and

Lymphatic Drainage of the Abdomen

and

Lymphatics of the Abdomen - Listed Alphabetically

Lymphatic pump function in the inflamed gut.

**Editor's Note:  I am including this brief article on inflammation and the intestinal regions as IL causes a tremendous amount of inflammation, as does lymphedema.  This describes what transpires during that inflammation. Pat**

Oct 2010

Von Der Weid PY, Rehal S.

Source

Department of Physiology and Pharmacology, Inflammation Research Network and Smooth Muscle Research Group, Snyder Institute of Infection, Immunity, and Inflammation, University of Calgary, Alberta, Canada. vonderwe@ucalgary.ca

Abstract

The role of the lymphatic circulation to actively remove fluid, cells, proteins, and other particles from the interstitium to prevent mounting edema is well appreciated, but whether and how this function is compromised during inflammation has been scarcely investigated. We discuss here the mechanisms of lymphatic pumping and their modulation in inflammatory conditions or by inflammatory mediators in the context of inflammatory bowel disease (IBD), an ensemble of disorders typically described with abnormal or dysfunctional intestinal or mesenteric lymphatic vessels. We report our findings showing impaired mesenteric lymphatic contractile activity in an animal model of intestinal inflammation that recapitulates some features of IBD and suggests a role for prostanoids in this dysfunction. With the knowledge that prostaglandin E(2) and prostacyclin are implicated in IBD pathogenesis and induce a potent inhibition of lymphatic pumping, we established the pharmacological profile for these prostaglandin receptors in mesenteric lymphatic vessels and their respective role in pumping inhibition. Inhibition of mesenteric lymphatic pumping during inflammation may be a cause of edema, compromised immune response, and granuloma associated with IBD.

Wiley Online