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

Intestinal lymphangiectasia in adults.

Feb 2011

Freeman HJ, Nimmo M.

Source

Hugh James Freeman, Department of Medicine (Gastroenterology), University of British Columbia, Vancouver, BC, V6T 1W5, Canada.

Abstract

Intestinal lymphangiectasia in the adult may be characterized as a disorder with dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine and resultant hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopenia. Most often, intestinal lymphangiectasia has been recorded in children, often in neonates, usually with other congenital abnormalities but initial definition in adults including the elderly has become increasingly more common. 

Shared clinical features with the pediatric population such as bilateral lower limb edema, sometimes with lymphedema, pleural effusion and chylous ascites may occur but these reflect the severe end of the clinical spectrum. In some, diarrhea occurs with steatorrhea along with increased fecal loss of protein, reflected in increased fecal alpha-1-antitrypsin levels, while others may present with iron deficiency anemia, sometimes associated with occult small intestinal bleeding. Most lymphangiectasia in adults detected in recent years, however, appears to have few or no clinical features of malabsorption. Diagnosis remains dependent on endoscopic changes confirmed by small bowel biopsy showing histological evidence of intestinal lymphangiectasia. In some, video capsule endoscopy and enteroscopy have revealed more extensive changes along the length of the small intestine. 

A critical diagnostic element in adults with lymphangiectasia is the exclusion of entities (e.g. malignancies including lymphoma) that might lead to obstruction of the lymphatic system and "secondary" changes in the small bowel biopsy. In addition, occult infectious (e.g. 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. Conversely, rare B-cell type lymphomas have also been described even decades following initial diagnosis of intestinal lymphangiectasia. 

Treatment has been historically defined to include a low fat diet with medium-chain triglyceride supplementation that leads to portal venous rather than lacteal uptake. A number of other pharmacological measures have been reported or proposed but these are largely anecdotal. Finally, rare reports of localized surgical resection of involved areas of small intestine have been described but follow-up in these cases is often limited.

NIH

Duodenal endoscopic findings and histopathologic confirmation of intestinal lymphangiectasia in dogs.

Duodenal endoscopic findings and histopathologic confirmation of intestinal lymphangiectasia in dogs.

Sept 2012

Larson RN, Ginn JA, Bell CM, Davis MJ, Foy DS.

Source

Department of Medical Sciences, University of Wisconsin-Madison, School of Veterinary Medicine, Madison, WI.

Abstract

BACKGROUND:

The diagnosis of intestinal lymphangiectasia (IL) has been associated with characteristic duodenal mucosal changes. However, the sensitivity and specificity of the endoscopic duodenal mucosal appearance for the diagnosis of IL are not reported.

HYPOTHESIS/OBJECTIVES:

To evaluate the utility of endoscopic images of the duodenum for diagnosis of IL. Endoscopic appearance of the duodenal mucosal might predict histopathologic diagnosis of IL with a high degree of sensitivity and specificity.

ANIMALS:

51 dogs that underwent upper gastrointestinal (GI) endoscopy and endoscopic biopsies.

METHODS:

Retrospective review of images acquired during endoscopy. Dogs were included if adequate biopsies were obtained during upper GI endoscopy and digital images were saved during the procedure. Images were assessed for the presence and severity of IL. Using histopathology as the gold standard, the sensitivity and specificity of endoscopy for diagnosing IL were calculated.

RESULTS:

Intestinal lymphangiectasia (IL) was diagnosed in 25/51 dogs. Gross endoscopic appearance of the duodenal mucosa had a sensitivity and specificity (95% confidence interval) of 68% (46%, 84%) and 42% (24%, 63%), respectively for diagnosis of IL. Endoscopic images in cases with lymphopenia, hypocholesterolemia, and hypoalbuminemia had a sensitivity of 80%.

CONCLUSIONS AND CLINICAL IMPORTANCE:

Endoscopic odenal mucosa appearance alone lacks specificity and has only a moderate sensitivity for diagnosis of IL. Evaluation of biomarkers associated with PLE improved the sensitivity; however, poor specificity for diagnosis of IL supports the need for histopathologic confirmation.

Wiley OnLine

Cryptoccocal menigitis as a primary manifestation in a patient with intestinal lymphangictasia.

July 2012

Jabeen SA, Murthy A, Kandadai RM, Meena AK, Borgohain R, Uppin MS.

Source

Department of Neurology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.

Abstract

Opportunistic infections usually occur in patients with an immunocompromised state, and can be severe. Cryptoccocal meningitis is a fatal condition if left untreated, and is usually found in such patients. We report the case of an adult patient with cryptoccocal meningitis secondary to intestinal lymphangiectasia. A 30 year old female was admitted to our hospital for meningitis. Biochemical and radiological investigations were performed. A cerebrospinal fluid latex agglutination test showed positive cryptoccocal antigen. In addition, there were features of humoral and cell mediated immunity deficiency (lymphopenia, hypoalbuminemia, hypogammaglobulinemia), with a negative human immunodeficiency virus (HIV) test by enzyme linked immunosorbent assay and polymerase chain reaction. An upper gastroduodenoscopy was performed, which showed multiple lymphangiectasias, and a biopsy confirmed the diagnosis of primary intestinal lymphangiectasia (PIL). The patient was treated with intravenous amphotericin B and oral flucytosine, and the meningitis resolved. PIL should be suspected in patients with cryptoccocal meningitis, combined with humoral and cell mediated immunity with a negative HIV test. The management issues, in addition to antifungal therapy, include nutritional supplements for the protein losing enteropathy.

Full text:

Annals of Indian Academy of Neurology/Pub Med

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy.

Nov 2011

Oh TG, Chung JW, Kim HM, Han SJ, Lee JS, Park JY, Song SY.

Source

Tak Geun Oh, Joo Won Chung, Hee Man Kim, Jung Yeob Park, Si Young Song, Division of Gastroenterology, Department of Internal Medicine, Yonsei Institute of Gastroenterology, Yonsei University College of Medicine, Seoul 120-752, South Korea.

Abstract

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.

PubMedCentral

Edema and protein-losing enteropathy (lymphangiectasia) complicating chronic constrictive pericarditis associated with an ascending aortic aneurysm

Edema and protein-losing enteropathy complicating chronic constrictive pericarditis associated with an ascending aortic aneurysm

**Here is an interesting abstract showing how swelling (ab edema) from pericarditis will trigger protein losing enteropathy (dilation of intestinal lymphatics/protein lose)**

Presse Med. 2007 Nov 5
Article in French]

Lesaffre F, Sandras R, Saade YA, Duval S, Metz D.Département de cardiologie et pathologies vasculaires, CHU de Reims, Hôpital Robert Debré, Avenue du Général Koenig, F-51100 Reims, France.

INTRODUCTION: Chronic constrictive pericarditis is suspected on clinical and echocardiographic grounds. Its treatment is surgical.

CASE: We report here the case of a 45-year-old man, admitted for edema characteristic of lymphatic obstruction. Examination revealed ascending aorta ectasia, associated with chronic constrictive pericarditis. Measurement of alpha-1 antitrypsin clearance confirmed protein-losing enteropathy. Total recovery followed pericardectomy and aneurysm resection.

DISCUSSION: The clinical edema in this case was due to several phenomena: protein-losing enteropathy from a functional lymphatic overload, induced by chronic constrictive pericarditis and by compression of the right atrium and vena cava by an aortic aneurysm. The hypoalbuminemia induced by protein loss may also magnify edema. An association between chronic constrictive pericarditis and ascending aortic aneurysm is uncommon. No cause for this association was found.

PMID: 17988828 [PubMed - as supplied by publisher]

Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis.

Primary gastrointestinal lymphangiectasia presenting as cryptococcal meningitis.
Ann Allergy Asthma Immunol. 2007 May

Cole SL, Ledford DK, Lockey RF, Daas A, Kooper J.
Department of Medicine, Division of Allergy and Immunology, University of South Florida College of Medicine, James A. Haley Veterans' Hospital, Tampa, Florida 33612, USA. scole@health.usf.edu

BACKGROUND: Opportunistic infections commonly occur in immunocompromised patients; however, it is unusual for an adult to present with a combined cellular and humoral immunodeficiency. Cryptococcal meningitis is a fatal condition if untreated and is usually found in patients with cellular immunodeficiency.

OBJECTIVE: To report the case of an adult patient with cryptococcal meningitis secondary to intestinal lymphangiectasia.

METHODS: A 59-year-old man was admitted to the hospital for disseminated cryptococcal meningitis and osteomyelitis. Laboratory evaluation, computed tomography, esophagogastroduodenoscopy, and biopsy were performed.

RESULTS: Laboratory evaluation revealed a lymphopenia, hypoalbuminemia, hypogammaglobulinemia, and negative human immunodeficiency virus test results by enzyme-linked immunosorbent assay and polymerase chain reaction. The complete blood cell count, urinalysis, serum and urine protein electrophoresis, and functional antibody responses to protein and polysaccharide antigens were normal. Results of computed tomography of the chest, abdomen, and pelvis were unremarkable. Multiple lymphangiectasias were visualized with esophagogastroduodenoscopy and confirmed by biopsy. The patient was treated with intravenous amphotericin B and flucytosine, and the meningitis resolved.

CONCLUSIONS: Based on a computerized search of the medical literature, this is the first description of cryptococcal meningitis secondary to intestinal lymphangiectasias. The combination of lymphopenia, hypogammaglobulinemia, and hypoalbuminemia should alert the clinician to the possibility of intestinal lymphangiectasias and the potential for immune dysfunction.

A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.

A case of protein-losing enteropathy caused by intestinal lymphangiectasia in a preterm infant.
Pediatrics. 2001 Feb

Salvia G,
Cascioli CF,
Ciccimarra F,
Terrin G,
Cucchiara S.
Via Sergio Pansini 5, I-80131, Naples, Italy.

Intestinal lymphangiectasia is characterized by obstruction of lymph drainage from the small intestine and lacteal dilation that distorts the villus architecture. Lymphatic vessel obstruction and elevated intestinal lymphatic pressure in turn cause lymphatic leakage into the intestinal lumen, thus resulting in malabsorption and protein-losing enteropathy. Intestinal lymphangiectasia can be congenital or secondary to a disease that blocks intestinal lymph drainage. We describe the first case of intestinal lymphangiectasia in a premature infant. The infant presented with peripheral edema and low serum albumin; high fecal concentration of alpha(1)-antitrypsin documented intestinal protein loss. Endoscopy showed white opaque spots on the duodenal mucosa, which indicates dilated lacteal vessels. Histology confirmed dilated lacteals and also showed villus blunting. A formula containing a high concentration of medium chain triglycerides resulted in a rapid clinical improvement and normalization of biochemical variables.

These features should alert neonatologists to the possibility of intestinal lymphangiectasia in newborns with hypoalbuminemia and peripheral edema. The intestinal tract should be examined for enteric protein losses if other causes (ie, malnutrition and protein loss from other sites) are excluded. The diagnosis rests on jejunal biopsy demonstrating dilated lymphatic lacteal vessels.

Pediatrics

Protein-losing enteropathy in Crohn's disease.

Protein-losing enteropathy in Crohn's disease.

Ferrante M, Penninckx F, De Hertogh G, Geboes K, D'Hoore A, Noman M, Vermeire S,
Rutgeerts P, Van Assche G.

Department of Gastroenterology, University Hospital Gasthuisberg, Leuven, Belgium.

Protein-losing enteropathy (PLE) is a rare but severe complication of Crohn's disease (CD) and hypoalbuminemia can be one of the presenting symptoms of this illness. The diagnosis of PLE can only be made after exclusion of malnutrition and liver or kidney failure. Significant intestinal leakage can be caused by mucosal injury, increased lymphatic pressure or dilated lymphatics and has been reported in a large number of diseases.

The protein-losing can be diagnosed by assessing the excretion of different radiolabeled macromolecules in the faeces or by the clearance of alpha-1-antitrypsine in stools. The primary approach should be the optimization of the nutritional status. Medical treatment of the underlying disease is primordial. In other cases surgical resection of the most affected areas is inevitable. We report a case of a 21-year-old male with a 4 year history of CD, who developed significant hypoproteinemia with pitting oedema, initially in the absence of any other sign of severe disease activity.

A "Cr-chloride albumin excretion confirmed our hypothesis of protein-losing enteropathy. Because of sub-obstruction signs some months later, a laparotomy was performed which revealed a severely affected loop with dilatation of the proximal jejunum. Interestingly, multiple large lymph nodes and dilated lymphatics were seen. A partial jejunal resection was performed for stricturing Crohn's disease. Histology showed severe mesenteric granulomatosis, dilated lymph vessels and granulomatous vasculitis. After the resection our patient improved without further albumin infusions and the oedema resolved.

PMID: 17343081 [PubMed - in process]

Like intestinal lymphangiectasia Crohn's disease involves distortions in the intestinal lymphatics. I thought it would be interesting to present this.