Pediatric Protein-Losing Enteropathy Treatment & Management

Updated: Jul 25, 2017
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: Carmen Cuffari, MD  more...
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Treatment

Medical Care

Therapeutic approaches for protein-losing enteropathy (PLE) depend on the underlying etiology. In PLE associated with primary intestinal lymphangiectasia or lymphatic obstruction, relieving the pressure in the lymphatic system decreases lymphatic flow and intestinal protein loss. Obstruction of lymphatics has been reported with structural heart disease, constrictive pericarditis, cardiomyopathy, and surgical repair of congenital heart disease. When losses from the intra-abdominal lymphatic system are the cause of PLE, removal of long-chain triglycerides from the diet decreases the pressure in the lacteals and the lymphatic circulation. Replacing fat in the diet with medium-chain triglycerides (MCTs) increases net fat absorption and the nutritional status of the patient. The use of MCT oil in these cases does not relieve any inflammation, but because MCT oil is not absorbed via the lymphatic system, it reduces the pressure of the lacteals. Fat soluble (ADEK) vitamin supplementation is required as their absorption is attenuated by the compromise in the lymphatic flow. 

PLE that results after heart surgery (with increased pressure in the right side) is a known postoperative complication of the Fontan procedure that has been a challenge to the surgical procedure's long-term success. Multiple treatments have been used, including corticosteroids, heparin, and additional surgical intervention (baffle fenestration or heart transplantation). [48]  

As many as 13.4% of patients undergoing a Fontan procedure develop PLE within 10 years of surgery, and the mortality rate associated with this complication has been reported to be as high as 56% in 5 years.

The use of steroids has produced temporary clinical and pathological resolution of PLE.

A single-center retrospective review examined the use of budesonide, an oral steroid with extensive first pass metabolism, for 6 months or longer in Fontan-related PLE patients. This treatment was associated with significant symptomatic improvement and sustained increases in serum albumin but did not markedly change the ultimate outcome and was associated with significant side effects. [49]

Heparin has also been reported to improve PLE in children after the Fontan procedure. Heparin is thought to possibly have a stabilizing effect on the capillary endothelium, reducing protein leakage into the extravascular space and gut lumen, although the precise mechanism of action is unknown. Although heparin has been successfully used to treat some patients with PLE that develops after the Fontan procedure, it is by no means the treatment of choice for all the etiologies of PLE.

A retrospective review of 42 patients with PLE following the Fontan procedure found that treatments used more frequently in survivors included spironolactone (68%), octreotide (21%), sildenafil (19%), and surgical intervention (71%). [17]

Corticosteroids, including budesonide, have been used in patients with PLE associated with collagen vascular diseases, inflammatory bowel disease, heart surgery, and others. Sporadic case reports have documented the successful use of other agents such as cyclosporine for PLE. Immunosuppressive drugs should never be used in cases of PLE secondary to infections.

Case reports have described success in patients with PLE secondary to primary intestinal lymphangiectasia (Waldmann disease) using everolimus [50]  and rapamycin in a child with tuberous sclerosis complex. [51]

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Surgical Care

Conner et al reported a case in which localized resection of the involved bowel successfully treated the condition. [52]

In patients who have undergone a Fontan procedure, fenestration of the baffle that separates the systemic venous pathway from the pulmonary venous atrium has been performed to treat PLE, and in some cases the symptoms have resolved, presumably because of the decrease in systemic venous pressure.

Cardiac transplantation has also been performed for the management of intractable PLE related to previous Fontan surgery, with complete resolution of symptoms in most cases and with survival comparable to patients with other indications for undergoing transplantation. [53]

In another case series of 3 patients who presented with PLE after undergoing the Fontan procedure and 2 patients after undergoing thoracic duct ligation, abnormal lymphatics visualized on lymphatic imaging were percutaneously accessed and embolized with lipiodol or n-BCA glue. This resulted in improvement in albumin level and symptoms. [47]

Pericardiectomy resulted in significant improvement of severe hypoalbuminemia in patients with constrictive pericarditis-associated PLE. [54]

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Diet

In patients whose PLE is related to lymphatic pathology, decreasing the lymphatic circulation provides some benefit. This requires dietary limitation of long-chain triglycerides because their absorption from the gut stimulates lymphatic flow. In order to provide adequate energy, medium-chain triglycerides must be added as an alternative source of lipid calories.

As described below, fat soluble vitamins must also be supplemented because their absorption is compromised in these patients.

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Long-Term Monitoring

Serial measurements of albumin, immunogloblins, lymphocytes, cholesterol, and fat soluble vitamins should be incorporated into the management of any patients with previous history or risk factors for PLE. 

 

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