National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Congenital pulmonary lymphangiectasia

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What should be the diet for an affected infant? What is the long term outcome for affected children?

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Are there dietary recommendations for infants with congenital pulmonary lymphangiectasia?

We are not aware of published dietary guidelines for infants with congenital pulmonary lymphangiectasia (CPL). However, as nutrition can play a role in limiting lymphatic production, considerations that have been suggested in the literature include total parenteral nutrition (TPN) and/or supplementation with medium-chain triglycerides (MCTs).[1][2] Enteral nutrition with MCTs and TPN have been successfully used.[3] If chylothorax occurs, a number of components are lost, including fats (mainly phospholipids, cholesterol, and triglycerides); proteins (mainly albumin, immunoglobulins, and fibrinogen); electrolytes; and fat-soluble vitamins in concentrations similar to those found in plasma.[3]
Last updated: 1/3/2017

What is the long-term outlook for children with congenital pulmonary lymphangiectasia?

Conflicting data have been reported regarding the outcome for children with congenital pulmonary lymphangiectasia (CPL).[3] The prognosis has previously been reported to be very poor, with mortality at about 100% before the 1990s.[4][5] However, more recent reports suggest that CPL is not a uniformly fatal condition, and that in survivors, the condition improves. This might be due to advances in modern intensive care therapy and also to the fact that severity can vary among affected children.[4] It has been suggested that children with generalized lymphangiectasia who have pulmonary involvement may have less severe CPL and a better prognosis; having a primary developmental defect of the pulmonary lymphatic vessels tends to be associated with a higher mortality.[6] Those with CPL who do survive infancy often continue to have medical problems that are characteristic of chronic lung disease. Gastroesophageal reflux and poor growth are also not uncommon during the first year of life, especially between six and twelve months of age, and are closely related to chronic lung disease.[3]
Last updated: 1/3/2017

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GARD Information Specialist

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  1. Bonioli E, Bellini C. Congenital Pulmonary Lymphangiectasia. National Organization for Rare Disorders (NORD). 2012; https://rarediseases.org/rare-diseases/congenital-pulmonary-lymphangiectasia/.
  2. Reiterer F, Grossauer K, Morris N, Uhrig S, Resch B. Congenital pulmonary lymphangiectasis. Paediatr Respir Rev. September, 2014; 15(3):275-280.
  3. Carlo Bellini, Francesco Boccardo, Corradino Campisi, and Eugenio Bonioli. Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis. 2006; 1:43:
  4. Reiterer F, Grossauer K, Pfleger A, Häusler M, Resch B, Eber E, Popper H, Urlesberger B. Severe primary pulmonary lymphangiectasis in a premature infant: management and follow up to early childhood. Pediatr Int. 2015; 57(1):166-169.
  5. Gray M, Kovatis KZ, Stuart T, Enlow E, Itkin M, Keller MS, French HM. Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography. J Perinatol. September, 2014; 34(9):720-722.
  6. Hwang JH, Kim JH, Hwang JJ, Kim KS, Kim SY. Pneumonectomy case in a newborn with congenital pulmonary lymphangiectasia. J Korean Med Sci. April, 2014; 29(4):609-613.