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

Intestinal lymphangiectasia associated with recurrence of histiocytosis X.

Sept 2011

Hui CK.

Source

Centre for Alimentary Studies, 5/F Century Square, 1 D'Aguilar Street, Central, Hong Kong SAR, China. bckhui@gmail.com

Abstract

K e y w o r d s : h i s t i o c y t o s i s X , i n t e s t i n a l

lymphangiectasia, secondary cause

Intestinal lymphangiectasia may occur as a primary congenital disorder or a secondary disorder. Secondarylymphangiectasia could be associated with diseases such as abdominal carcinoma, retroperitoneal fibrosis or chronic pancreatitis. This is the first reported case of intestinal lymphangiectasia associated with recurrent histiocytosis X. This case report illustrates the need for more prospective, well-designed studies to determine the natural history and outcome of intestinal lymphangiectasia in the duodenum. Hopefully, these studies will also help clinicians identify which group of patients with intestinal lymphangiectasia in the duodenum is more likely to have a secondary cause.

SMJ

Cytomegalovirus-associated protein-losing enteropathy resulting from lymphangiectasia in an immunocompetent child.

Cytomegalovirus-associated protein-losing enteropathy resulting from

lymphangiectasia in an immunocompetent child.

Jpn J Infect Dis. 2009 May

Hoshina T, Kusuhara K, Saito M, Hara T, Mtsuura S, Yano T, Aoki T, Hara T.

Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. hoshina@pediatr.med.kyushu-u.ac.jp

An immunocompetent 8-year-old boy with cytomegalovirus (CMV)-associated transient protein-losing enteropathy (PLE) is described. Colonoscopic examination revealed lymphoid hyperplasia of the terminal ileum. Histological examination of the biopsied specimens showed marked dilation of the lymphatic vessels. Primary CMV infection was demonstrated by serological test and polymerase chain reaction. The child had complete resolution of the disease without antiviral treatment. The present case suggests the etiologic role of CMV infection in PLE resulting from intestinal lymphangiectasia in childhood.

NIH

Reversal of protein-losing enteropathy after heart transplantation in young patients.

Reversal of protein-losing enteropathy after heart transplantation in young patients

Rev Esp Cardiol. 2009 August

Soriano JR, Grima EZ, Vives MA, Sáez AO, Dolz LM, Bonet LA, Pérez MA, Sanz AS.

Servicio de Cardiología, Hospital Universitario La Fe, Valencia, Spain. ximo@ipeuropa.com

Protein-losing enteropathy is a rare but life-threatening complication that occurs in some patients who develop intestinal lymphangiectasis secondary to increased systemic venous pressure. Although different forms of treatment have been tried, with varying results, the majority were reported to be unsuccessful. The aim of this study was to demonstrate that heart transplantation may be an appropriate therapeutic option for patients who do not respond to medical treatment. At our center, we performed heart transplantations in three patients with this condition. The mean follow-up period was 11+/-2 months. No patient died and the enteropathy regressed in all three.

Cardiologia

Lymphangiogenesis in Crohn’s disease: an immunohistochemical study using monoclonal antibody D2-40

Lymphangiogenesis in Crohn’s disease: an immunohistochemical study using monoclonal antibody D2-40
F. Pedica1 , C. Ligorio1, P. Tonelli2, S. Bartolini3 and P. Baccarini1
(1)
Section of Pathology, Bellaria Hospital, University of Bologna, Via Altura 3, 40139 Bologna, Italy
(2)
General Surgery 1, Department of Medicine and General Surgery, Azienda Ospedaliero-Universitaria Careggi, Firenze, Italy
(3)

Unity of Oncology, Bellaria Hospital, Bologna, Italy
Received: 17 September 2007 Revised: 24 October 2007 Accepted: 26 October 2007 Published online: 27 November 2007

Abstract

Crohn’s disease (CD) is a chronic inflammatory bowel disorder of unknown etiology. An involvement of the intestinal lymphatic system has been suggested. Recently, monoclonal antibodies have become available to distinguish lymphatic vessels from blood vessels. The aim of the study was to examine the distribution of lymphatic vessels in ileal and colic walls of patients affected by CD and compare it with healthy controls and other inflammatory bowel diseases. Twenty-eight cases of CD, 13 cases of other inflammatory bowel diseases, and 10 normal ileal and colic walls were studied. Immunohistochemical staining was performed using the monoclonal antibody D2-40. Quantification of lymphatic vessels was performed by identifying four fields with high density of lymphatics and then counting the number of lymphatic vessels at high resolution. Lymphatic diameter was also evaluated by using an ocular micrometer. Lymphatic vessels showed the highest density in CD specimens. The median number of lymphatics was significantly higher both in ileal and colic samples of CD than the other inflammatory diseases as well as normal controls. Moreover, in patients with CD, diffuse lymphangiectasia was also observed. The present data suggest that lymphangiogenesis and lymphangiectasia probably play a role in the pathogenesis of CD.

Springer Link

Intestinal Lymphangiectasia With Protein-Losing Enteropathy in Waldenstrom Macroglobulinemia.

Intestinal Lymphangiectasia With Protein-Losing Enteropathy in Waldenstrom Macroglobulinemia.

Medicine (Baltimore). 2007 Jul

Pratz KW, Dingli D, Smyrk TC, Lust JA.
From Department of Medical Oncology (KWP), Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, Baltimore, Maryland; Division of Hematology (DD, JAL), Department of Internal Medicine; and Department of Laboratory Medicine and Pathology (TCS), Mayo Clinic College of Medicine, Rochester, Minnesota.

Gastrointestinal complications of Waldenstrom macroglobulinemia (WM) are unusual but often treatable. We report a case of WM associated with significant gastrointestinal involvement manifest as chronic diarrhea with protein-losing enteropathy and recurrent venous thromboses. Small bowel biopsy was negative for amyloidosis but revealed intestinal lymphangiectasia with deposition of monoclonal IgM. The patient was treated with cyclophosphamide, vincristine, and prednisone with rapid and complete resolution of the peripheral edema and diarrhea. We follow the case report with a retrospective analysis of patients with WM and gastrointestinal symptoms seen at our institution, and review the available literature on this unusual association.

An increased awareness of the gastrointestinal manifestations of WM may help to explain and to treat the chronic, debilitating, and potentially life-threatening symptoms in patients with this lymphoproliferative disorder.

Lippincott, Williams & Wilkins

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) with lymphangiectasia

Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome).
With lymphangiectasia as a complication

Diagn Pathol. 2007 Jul

Vankalakunti M, Gupta K, Kakkar N, Das A.

ABSTRACT
BACKGROUND: Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.

CASE PRESENTATION: We describe two unrelated cases of Renal-hepatic-pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-hepatic-pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.

CONCLUSION: These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.

Diagnostic Pathology

Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia.

Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia.
J Nephrol. 2007 Mar-Apr
Kalman S,
Bakkaloglu S,
Dalgic B,
Ozkaya O,
Soylemezoglu O,
Buyan N.

Department of Pediatric Nephrology, Gazi University, Besevler, Ankara - Turkey.

A 17-year-old boy was admitted to the hospital twice in a year for 2 episodes of hemolytic uremic syndrome (HUS). During these 2 HUS episodes he had diarrhea, decreased serum complement, decreased total protein and decreased serum albumin concentrations. We suggest that protein-losing enteropathy and hypocomplementemia due to intestinal lymphangiectasia is may be a rare cause of atypical HUS.

PMID: 17514630 [PubMed - in process]

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

Nat Clin Pract Gastroenterol Hepatol. 2007 May
Herfarth H,
Hofstadter F,
Feuerbach S,
Jurgen Schlitt H,
Scholmerich J,
Rogler G.
Department of Medicine, Division of Gastroenterology and Hepatology, University of North Carolina, Chapel Hill, NC 27599, USA. hherf@med.unc.edu

BACKGROUND: A 40-year-old male with pentalogy of Fallot (a congenital heart defect with five anatomical components) presented with recurrent gastrointestinal bleeding. He had recently recovered from a heart operation, which was performed to reconstruct the right ventricular outflow tract.

INVESTIGATIONS: Laboratory tests and absorption tests, esophagogastroduodenoscopy, capsule endoscopy, human serum albumin scintigraphy, lymphoscintigraphy, CT and abdominal lymph-node histology.

DIAGNOSIS: Intestinal lymphangiectasia with concurrent protein-losing gastroenteropathy and recurrent gastrointestinal bleeding.

MANAGEMENT: Despite a low-fat diet and surgical suturing of multiple small-bowel ulcerations the gastrointestinal bleeding continued. Serum albumin levels remained very low and severe lymphedema occurred. Unfortunately, the patient developed severe sepsis and died of multiple organ failure.

PMID: 17476211 [PubMed - in process]

Intestinal lymphangiectasia secondary to radiotherapy and chemotherapy.

This is an older article going back to 1987. I found it interesting however as it is a report of secondary intestinal lymphangiectasia from an unexpected souce, cancer treatment.

The article:

Intestinal lymphangiectasia secondary to radiotherapy and chemotherapy.

Dig Dis Sci. 1987 Aug

Rao SS,
Dundas S,
Holdsworth CD.

We report a case of intestinal lymphangiectasia secondary to radiotherapy and chemotherapy. The patient also had small bowel bacterial overgrowth and pancreatic insufficiency. Lymphatic ectasia as a histological feature has been described previously in association with postradiotherapy malabsorption, but radiation-induced lymphangiectasia producing clinical manifestations has hitherto not been reported. Replacement of dietary long-chain fats with medium-chain triglycerides, pancreatic enzyme supplements, and a short course of oxytetracycline, resulted in dramatic clinical improvement.

The possibility of intestinal lymphangiectasia should be borne in mind in patients with postradiotherapy malabsorption. A low serum albumin and lymphocyte count should draw attention to this possibility.

PMID: 3608736 [PubMed - indexed for MEDLINE]

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

Capsule Endoscopy in the Pediatric Patient

Capsule Endoscopy in the Pediatric Patient
Curr Treat Options Gastroenterol. 2006 Sep

Seidman EG,
Dirks MH.
Division of Gastroenterology, Montreal Children's Hospital, 2300 Tupper Street, Room D562, Montreal, Quebec H3H 1P3, Canada. digestivelab@hotmail.com.

Wireless capsule endoscopy represents an extraordinary technical innovation in diagnostic gastrointestinal endoscopy. As in adult patients, it opens new horizons that permit an accurate and noninvasive approach to identifying occult lesions in the small bowel in children and adolescents. A limitation in the pediatric age group is the size of the capsule, precluding its use in infants and small toddlers.

In children unable to swallow the capsule, "front loading" the gastroscope to introduce it into the duodenum is a suitable alternative approach. Capsule endoscopy is highly useful to evaluate for inflammatory changes in patients suspected to have small bowel Crohn's disease in whom conventional imaging failed to confirm the diagnosis. It is now the method of first choice to assess for small bowel polyps or tumors, to find a source of blood loss in obscure intestinal bleeding, and for undiagnosed malabsorptive conditions such as intestinal lymphangiectasia.

Capsule retention is the one major potential adverse effect of capsule endoscopy. In patients suspected to have a small bowel stenosis, consideration should be given to using the patency capsule prior to using the real videocapsule so as to decrease the risk of capsule retention.

Gastroenterology

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.

Enteroclysis in older children and teenagers

Enteroclysis in older children and teenagers
Pediatr Radiol. 2007 Mar 22

Kurugoglu S,
Korman U,
Adaletli I,
Selcuk D.
Department of Radiology, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, 34303, Istanbul, Turkey, sebuh.k@superonline.com.

BACKGROUND: Enteroclysis (EC) has been widely and successfully used for evaluation of the small bowel in adults for about 30 years. However, despite recently improved intubation and examination techniques, in many paediatric radiology centres it is still not the preferred conventional barium study for the evaluation of small bowel pathology in children.

OBJECTIVE: To share our 10 years of experience and review the feasibility of EC in 83 older children and teenagers, in terms of both technique and pathological findings.

MATERIALS AND METHODS: Between 1996 and 2006, EC was performed by the standard technique described by Herlinger to 83 children between 7 and 18 years of age. The indication for the study was jointly decided by the paediatric radiologist and the clinician. None of the examinations was converted to follow-through studies because of patient refusal or technical failure. Morphological changes, mucosal abnormalities, luminal abnormalities, perienteric structures, the location of the disease, indirect findings regarding the bowel wall and functional information were evaluated.

RESULTS: All the children tolerated the procedure without difficulty. Out of 83 patients, 63 had abnormal findings. The spectrum of diagnoses were Crohn disease (n = 23), nonspecific enteritis (n = 10), malabsorption (n = 8), intestinal tuberculosis (n = 6), intestinal lymphoma (n = 5), Peutz-Jegher syndrome (n = 3), adhesions (n = 2), Behcet disease (n = 2), back-wash ileitis due to ulcerative colitis (n = 2), common-variable immune deficiency (n = 1) and lymphangiectasis (n = 1).

CONCLUSION: EC can easily be performed in children over 7 years of age and when performed using a correct technique it shows high diagnostic performance without any complications in the evaluation of small bowel diseases in older children and teenagers.

SpringLink

Results of transcatheter Fontan fenestration to treat protein losing enteropathy

Results of transcatheter Fontan fenestration to treat protein losing enteropathy/ intestinal lymphangiectasia
Catheter Cardiovasc Interv. 2007 Feb

Vyas H,
Driscoll DJ,
Cabalka AK,
Cetta F,
Hagler DJ.
Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic College of Medicine, Rochester, Minnesota.

Transcatheter fenestration to create an interatrial communication has been used to treat patients with protein losing enteropathy (PLE) after Fontan operation. No systematic data have been reported assessing the results of this procedure. Our institutional database was queried to identify patients after Fontan operation who had transcatheter fenestration to treat PLE. Clinical notes, laboratory data, echocardiograms, and cardiac catheterization data were reviewed.

From 1995 to 2005, 16 transcatheter fenestration procedures were performed in seven patients. Median age at fenestration was 18 years (range 13-41 years). Median duration of follow-up was 3.6 years (range 0.2-10.4 years). Techniques for fenestration included blade/balloon septostomy, stent placement, Amplatzer-fenestrated ASD device, and balloon dilation of previous stent. Size of the fenestration created was 5.2 +/- 1.1 mm. Systemic venous pressure remained unchanged after fenestration. Cardiac index increased significantly. Reduction of ascites and edema was noted after 9 of the 16 procedures. Ten of 16 (63%) of fenestrations spontaneously occluded.

Three patients are free of ascites although recurrence of PLE occurred in all. One patient with a patent fenestration continues to have ascites. Two patients had Fontan takedown. One patient had conversion to a fenestrated extracardiac conduit Fontan and died postoperatively. The results of transcatheter Fontan fenestration are often disappointing. Maintaining fenestration patency is difficult. Even after "successful" fenestration, resolution of PLE may be incomplete and recurrences have occurred in all. Early consideration should be given to Fontan takedown or cardiac transplant in severely symptomatic patients with PLE who do not respond to fenestration. Transcatheter fenestration may be a bridge to a definitive procedure.

(c) 2007 Wiley-Liss, Inc.

Pub Med