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Table of Contents
Vol. 24, No. 3, 2008
Issue release date: October 2008
Fetal Diagn Ther 2008;24:225–229
(DOI:10.1159/000151343)

Survival in Primary Congenital Pulmonary Lymphangiectasia with Hydrops Fetalis

Laje P. · Wilson R.D. · Guttenberg M. · Liechty K.W.
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Abstract

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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References

  1. Noonan JA, Walters LR, Reeves JT: Congenital pulmonary lymphangiectasias. Am J Dis Child 1970;120:314–319.
  2. Esther CR Jr, Barker PM: Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol 2004;38:308–313.
  3. Bellini C, Boccardo F, Campisi C, et al: Congenital pulmonary lymphangiectasia. Orphanet J Rare Dis 2006;1:43.
  4. Wilson RD, Pawel B, Bebbington M, et al: Congenital pulmonary lymphangiectasias sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Prenat Diagn 2006;26:1058–1061.
  5. Fukunaga M: Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumors. Histopathology 2005;46:396–402.
  6. Kaiserling E: Immunohistochemical identification of lymph vessels with D2–40 in diagnostic pathology. Pathologe 2004;25:362–374.
  7. Siu SL, Lam DS: Spontaneous neonatal chylothorax treated with octreotide. J Pediatr Child Health 2006;42:65–67.

    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.
  9. Scott C, Wallis C, Dinwiddie R, et al: Primary pulmonary lymphangiectasias in a premature infant: resolution following intensive care. Pediatr Pulmonol 2003;35:405–406.
  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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