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Vol. 24, No. 3, 2008
Issue release date: October 2008
Fetal Diagn Ther 2008;24:225–229

Survival in Primary Congenital Pulmonary Lymphangiectasia with Hydrops Fetalis

Laje P. · Wilson R.D. · Guttenberg M. · Liechty K.W.
Department of General Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pa., USA

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The recent advances in neonatal and pediatric intensive care have changed the outcome of patients with congenital pulmonary lymphangiectasia of different types, including those cases with early neonatal symptoms. However, the patients who present the most severe form of the disease, manifested by in utero hydrops (including those treated by in utero thoracoamniotic shunting to relieve the mediastinal compression), have had an unvaryingly fatal ending in all published reports, with most cases dying before birth, and the few born alive dying during the first days of life. We present a patient with primary congenital pulmonary lymphangiectasia complicated by hydrops fetalis, who was treated in utero, survived the neonatal period after intensive medical and surgical support, and was discharged home at the age of 2 months. She is currently 6 months old, and has minimal signs or symptoms of chronic lung disease. The different aspects of the management of congenital pulmonary lymphangiectasia are discussed in this report, together with a review of the literature.

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    External Resources

  8. Barker PM, Esther CR Jr, Fordham LA, et al: Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J 2004;24:413–419.
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  10. Dempsey EM, Sant’Anna GM, Williams RL, et al: Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: not uniformly fatal. Pediatr Pulmonol 2005;40:270–274.

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