Correspondence

Congenital Pulmonary Lymphangiectasia

N Engl J Med 2004; 350:948February 26, 2004

Article

To the Editor:

In Hagmann and Berger's description of congenital pulmonary lymphangiectasia in Images in Clinical Medicine (Nov. 27 issue),1 the authors state, “Congenital pulmonary lymphangiectasia . . . is a uniformly fatal disease when it manifests in the newborn period.” Having followed several such patients for 12 to 60 months, we disagree with this statement. The authors evidently did not see the report on radiographic findings in children with congenital pulmonary lymphangiectasia who survive infancy.2 Much of the literature on congenital pulmonary lymphangiectasia is heavily influenced by early reports that preceded modern care provided in the neonatal intensive care unit. We believe that patients with suspected congenital pulmonary lymphangiectasia should be treated aggressively, since at least a subgroup of them have a good prognosis for long-term survival.

Jonathan Finder, M.D.
Jonathan Steinfeld, M.D.
Children's Hospital of Pittsburgh, Pittsburgh, PA 15213
finder@pitt.edu

2 References

1. 1

   Hagmann C, Berger TM. Congenital pulmonary lymphangiectasia. N Engl J Med 2003;349:e21-e21
   Full Text | Web of Science | Medline

2. 2

   Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasia: after infancy. AJR Am J Roentgenol 1999;173:1583-1588
   Web of Science | Medline

Author/Editor Response

We thank Drs. Finder and Steinfeld for their thoughtful comments and take the opportunity to clarify our statement about the prognosis for symptomatic neonates with isolated congenital pulmonary lymphangiectasia. According to Noonan et al.,1 there are three forms of the disorder: isolated congenital pulmonary lymphangiectasia (as in our patient), congenital pulmonary lymphangiectasia associated with pulmonary venous obstruction, and congenital pulmonary lymphangiectasia associated with a generalized defect in lymphatic development. Pulmonary involvement is most severe in patients with isolated congenital pulmonary lymphangiectasia, and the prognosis for the majority of these patients remains poor even with modern care provided in a neonatal intensive care unit.2,3 However, there are some reports of long-term survivors.3-5 Although not clearly specified in the report by Chung et al.,4 the onset of symptoms was delayed beyond the neonatal period in the three survivors with isolated congenital pulmonary lymphangiectasia who were described by Bouchard and colleagues.3 Therefore, as Drs. Finder and Steinfeld correctly point out, isolated congenital pulmonary lymphangiectasia may indeed not be a uniformly fatal disease; its prognosis appears to depend on the severity of symptoms observed in the immediate postnatal period.

Thomas M. Berger, M.D.
Cornelia Hagmann, M.D.
Children's Hospital of Lucerne, CH-6000 Lucerne, Switzerland
thomas.berger@ksl.ch

Jan-Olaf Gebbers, M.D.
Kantonsspital Luzern, CH-6000 Lucerne, Switzerland

5 References

1. 1

   Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasias. Am J Dis Child 1970;120:314-319
   Web of Science | Medline

2. 2

   Case Records of the Massachusetts General Hospital (Case 13-1992). N Engl J Med 1992;326:875-884
   Full Text | Web of Science | Medline

3. 3

   Bouchard S, Di Lorenzo M, Youssef S, Simard P, Lapierre JG. Pulmonary lymphangiectasia revisited. J Pediatr Surg 2000;35:796-800
   CrossRef | Web of Science | Medline

4. 4

   Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasia. AJR Am J Roentgenol 1999;173:1583-1588
   Web of Science | Medline

5. 5

   Scott C, Wallis C, Dinwiddie R, Owens C, Coren M. Primary pulmonary lymphangiectasia in a premature infant: resolution following intensive care. Pediatr Pulmonol 2003;35:405-406
   CrossRef | Web of Science | Medline

Citing Articles (3)

Citing Articles

1. 1

   Zuo-Yuan Xiao, Yu Tao, Xin-Yi Tang, Guo-Juan Chen, Lei Guo. (2009) Congenital pulmonary lymphangiectasis. World Journal of Pediatrics 5:1, 68-70
   CrossRef

2. 2

   Mustafa Akcakus, Esad Koklu, Mehmet Bilgin, Selim Kurtoglu, Levent Altunay, Mehmet Canpolat, Nurten Budak. (2007) Congenital Pulmonary Lymphangiectasia in a Newborn: A Response to Autologous Blood Therapy. Neonatology 91:4, 256-259
   CrossRef

3. 3

   E.M. Dempsey, G.M. Sant'Anna, R.L. Williams, R.T. Brouillette. (2005) Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: Not uniformly fatal. Pediatric Pulmonology 40:3, 270-274
   CrossRef