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

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

Primary intestinal lymphangiectasia (Waldmann's disease).

Primary intestinal lymphangiectasia (Waldmann's disease).

Feb 2008 Orphanet J Rare Dis.

Vignes S, Bellanger J.

ABSTRACT:

Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. Prevalence is unknown. The main symptom is predominantly bilateral lower limb edema. Edema may be moderate to severe with anasarca and includes pleural effusion, pericarditis or chylous ascites. Fatigue, abdominal pain, weight loss, inability to gain weight, moderate diarrhea or fat-soluble vitamin deficiencies due to malabsorption may also be present. In some patients, limb lymphedema is associated with PIL and is difficult to distinguish lymphedema from edema. Exsudative enteropathy is confirmed by the elevated 24-h stool alpha-1 antitrypsin clearance. Etiology remains unknown. Very rare familial cases of PIL have been reported. Diagnosis is confirmed by endoscopic observation of intestinal lymphangiectasia with the corresponding histology of intestinal biopsy specimens. Videocapsule endoscopy may be useful when endoscopic findings are not contributive. Differential diagnosis includes constrictive pericarditis, intestinal lymphoma, Whipple's disease, Crohn's disease, intestinal tuberculosis, sarcoidosis or systemic sclerosis. Several B-cell lymphomas confined to the gastrointestinal tract (stomach, jejunum, midgut, ileum) or with extra-intestinal localizations were reported in PIL patients. A low-fat diet associated with medium-chain triglyceride supplementation is the cornerstone of PIL medical management. The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. Medium-chain triglycerides are absorbed directly into the portal venous circulation and avoid lacteal overloading. Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids. Surgical small-bowel resection is useful in the rare cases with segmental and localized intestinal lymphangiectasia. The need for dietary control appears to be permanent, because clinical and biochemical findings reappear after low-fat diet withdrawal. PIL outcome may be severe even life-threatening when malignant complications or serous effusion(s) occur.

Orphanet

Clinical application and diagnostic yield of wireless capsule endoscopy in children

Clinical application and diagnostic yield of wireless capsule endoscopy in children

J Laparoendosc Adv Surg Tech A. 2007 Jun

Antao B, Bishop J, Shawis R, Thomson M.
Pediatric Surgical Unit and Sheffield Children's Hospital, Sheffield, United Kingdom.

Objective:

The small bowel is anatomically difficult to examine and was investigated by invasive, indirect modalities, such as push enteroscopy and small bowel follow-through. The aim of this study was to assess the efficacy and clinical impact of wireless capsule endoscopy (WCE) in children.

Materials and Methods:

Over the last 3 years (2002-2005), 37 patients with suspected small-bowel disease were investigated with WCE at a median age of 11 years (range, 16 months-16 years). The indications for WCE was suspected Crohn's disease (CD) (18), obscure or occult gastrointestinal bleeding (7), polyposis syndromes (5), protein losing enteropathy (4), recurrent abdominal pain (2), and malabsorption syndrome (1). All patients had preceding upper gastrointestinal endoscopy (OGD), ileocolonoscopy, and 26 cases had a small bowel follow-through (SBFT). These results were compared with the findings on WCE.

Results:

Thirty-three (33) cases successfully completed the WCE through the small bowel. Four (4) patients were unable to swallow the capsule, 3 of which had to be placed in the duodenum endoscopically. In 3 patients, the capsule remained in the stomach and no small bowel images were obtained. The overall diagnostic yield was 85% (28/33 patients). The diagnostic findings included CD (13), source of gastrointestinal bleeding (7), polyposis syndromes (3), erosive enteropathy and patchy lymphangiectasia (4), and intussusception (1). WCE was found to be more sensitive for small bowel pathology than SBFT (20 vs. 6 [30% sensitivity, compared to WCE]) and endoscopic investigations (28 vs. 12 [43% sensitivity compared to WCE]). As a result of WCE findings, there was a positive alteration in the management in 28 of 33 (85%) cases.

Conclusions:

WCE is a novel, noninvasive, and useful tool for the investigation of the small intestine in children. It is superior and more sensitive than other conventional endoscopic and radiologic investigations in the assessment of the small bowel. It can help in guiding surgical decisions and should be routinely integrated as a part of the diagnostic work-up of small bowel pathology.

Article

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