J Korean Med Sci.  2014 Apr;29(4):609-613. 10.3346/jkms.2014.29.4.609.

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

• Hwang JH
• Kim JH
• Hwang JJ
• Kim KS
• Kim SY

• KMID: 1774471
• DOI: http://doi.org/10.3346/jkms.2014.29.4.609

Abstract

Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.

Keyword

Lymphangiectasia, Pulmonary, Congenital; Infant, Newborn; Pneumonectomy

MeSH Terms

Female
Gestational Age
Humans
Infant, Newborn
Lung/pathology
Lung Diseases/*congenital/diagnosis/pathology/radiography
Lymphangiectasis/*congenital/diagnosis/pathology/radiography
Lymphatic Vessels/pathology
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Chest radiograph acquired on day 5 showing asymmetric overinflation and a diffusely thickened interstitium in the right lung.

• 

  Fig. 2 High-resolution computed tomography reveals thickening of the interstitium, as well as air-trapping and hyperinflation of the right lung resulting in mediastinal shift to the left.

• 

  Fig. 3 Cystic dilatation of lymphatics is evident on gross inspection of the cut and subpleural surfaces of the lung.

• 

  Fig. 4 Histologic section of the right lung. (A) Cystic dilatation of bronchovascular lymphatic channels (H&E, ×40). (B) D2-40-positive flattened cells, specific for lymphatic endothelium, lining the dilated cystic space. (C) The lesion is CD34-positive. (D) The same lesion is CD31-negative.

Reference

1. Esther CR Jr, Barker PM. Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol. 2004; 38:308–313.

2. Virchow R. Gesammelte abdhandlungen zur Wissenschaftliche Medicin. Frankfurt: Meidinger, Sohn & Co.;1856. p. 982.

3. Laurence KM. Congenital pulmonary lymphangiectasis. J Clin Pathol. 1959; 12:62–69.

4. Mettauer N, Agrawal S, Pierce C, Ashworth M, Petros A. Outcome of children with pulmonary lymphangiectasis. Pediatr Pulmonol. 2009; 44:351–357.

5. Bouchard S, Di Lorenzo M, Youssef S, Simard P, Lapierre JG. Pulmonary lymphangiectasia revisited. J Pediatr Surg. 2000; 35:796–800.

6. Scott C, Wallis C, Dinwiddie R, Owens C, Coren M. Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care. Pediatr Pulmonol. 2003; 35:405–406.

7. Akcakus M, Koklu E, Bilgin M, Kurtoglu S, Altunay L, Canpolat M, Budak N. Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy. Neonatology. 2007; 91:256–259.

8. Brown M, Pysher T, Coffin CM, Brown M, Pysher T, Coffin CM. Lymphangioma and congenital pulmonary lymphangiectasis: a histologic, immunohistochemical, and clinicopathologic comparison. Mod Pathol. 1999; 12:569–575.

9. Rettwitz-Volk W, Schlösser R, Ahrens P, Hörlin A. Congenital unilobar pulmonary lymphangiectasis. Pediatr Pulmonol. 1999; 27:290–292.

10. Moore KL. Persaud TVN: the developing human: clinically oriented embryology. 7th ed. Philadelphia: Saunders;2003.

11. Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasis. Am J Dis Child. 1970; 120:314–319.

12. Laurence KM. Congenital pulmonary cystic lymphangiectasis. J Pathol Bacteriol. 1955; 70:325–333.

13. Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med. 2000; 161:1037–1046.

14. Chapdelaine J, Beaunoyer M, St-Vil D, Oligny LL, Garel L, Bütter A, Di Lorenzo M. Unilobar congenital pulmonary lymphangiectasis mimicking congenital lobar emphysema: an underestimated presentation? J Pediatr Surg. 2004; 39:677–680.

15. Fukunaga M. Expression of D2-40 in lymphatic endothelium of normal tissues and in vascular tumours. Histopathology. 2005; 46:396–402.

16. Jabra AA, Fishman EK, Shehata BM, Perlman EJ. Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation. AJR Am J Roentgenol. 1997; 169:1381–1384.

17. Yamada S, Hisaoka M, Hamada T, Araki S, Shiraishi M. Congenital pulmonary lymphangiectasis: report of an autopsy case masquerading as pulmonary interstitial emphysema. Pathol Res Pract. 2010; 206:522–526.

18. Fujishiro J, Komuro H, Ono K, Urita Y, Shinkai T, Minami Y, Kawabata Y, Kishimoto H, Masumoto K. Massive pneumatic expansion of lymphatic vessel resulting in cystic lesions in the pulmonary parenchyma: a rare case of persistent interstitial pulmonary emphysema in a non-ventilated infant. J Pediatr Surg. 2012; 47:e21–e25.

19. Hirano H, Nishigami T, Okimura A, Nakasho K, Uematsu K. Autopsy case of congenital pulmonary lymphangiectasis. Pathol Int. 2004; 54:532–536.

20. Yalcin S, Ciftci A, Karnak I, Ekinci S, Tanyel FC, Senocak M. Childhood pneumonectomies: two decades' experience of a referral center. Eur J Pediatr Surg. 2013; 23:115–120.