To the Editor: In Hagmann and Berger's description of congenitalpulmonary 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 thenewborn period." Having followed several such patients for 12to 60 months, we disagree with this statement. The authors evidentlydid not see the report on radiographic findings in childrenwith congenital pulmonary lymphangiectasia who survive infancy.2Much of the literature on congenital pulmonary lymphangiectasiais heavily influenced by early reports that preceded moderncare provided in the neonatal intensive care unit. We believethat patients with suspected congenital pulmonary lymphangiectasiashould be treated aggressively, since at least a subgroup ofthem 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{at}pitt.edu
References
Hagmann C, Berger TM. Congenital pulmonary lymphangiectasia. N Engl J Med 2003;349:e21 (Web only)-e21 (Web only).
Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasia: after infancy. AJR Am J Roentgenol 1999;173:1583-1588.
The authors and a colleague reply: We thank Drs. Finder andSteinfeld for their thoughtful comments and take the opportunityto clarify our statement about the prognosis for symptomaticneonates 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 pulmonaryvenous obstruction, and congenital pulmonary lymphangiectasiaassociated with a generalized defect in lymphatic development.Pulmonary involvement is most severe in patients with isolatedcongenital pulmonary lymphangiectasia, and the prognosis forthe majority of these patients remains poor even with moderncare provided in a neonatal intensive care unit.2,3 However,there are some reports of long-term survivors.3,4,5 Althoughnot clearly specified in the report by Chung et al.,4 the onsetof symptoms was delayed beyond the neonatal period in the threesurvivors with isolated congenital pulmonary lymphangiectasiawho were described by Bouchard and colleagues.3 Therefore, asDrs. Finder and Steinfeld correctly point out, isolated congenitalpulmonary lymphangiectasia may indeed not be a uniformly fataldisease; its prognosis appears to depend on the severity ofsymptoms 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{at}ksl.ch
Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasias. Am J Dis Child 1970;120:314-319.
Case Records of the Massachusetts General Hospital (Case 13-1992). N Engl J Med 1992;326:875-884.
Bouchard S, Di Lorenzo M, Youssef S, Simard P, Lapierre JG. Pulmonary lymphangiectasia revisited. J Pediatr Surg 2000;35:796-800.
Chung CJ, Fordham LA, Barker P, Cooper LL. Children with congenital pulmonary lymphangiectasia. AJR Am J Roentgenol 1999;173:1583-1588.
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.
Hoehn, T., William, M., McPhaden, A. R., Stannigel, H., Mayatepek, E., Wadsworth, R. M.
(2006). Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis.. Eur Respir J
27: 1311-1315
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