SciELO - Scientific Electronic Library Online

 
vol.89 issue4Sweet Syndrome in Pediatrics. A case reportScarlet fever associated with hepatitis in pediatrics. A case report author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

  • On index processCited by Google
  • Have no similar articlesSimilars in SciELO
  • On index processSimilars in Google

Share


Revista chilena de pediatría

Print version ISSN 0370-4106

Rev. chil. pediatr. vol.89 no.4 Santiago Aug. 2018

http://dx.doi.org/10.4067/S0370-41062018005000605 

CLINICAL CASE

Unilateral congenital pulmonary lymphangiectasia in a preterm infant

Jesús Javier Martínez García1  2 

Marisol Morín Hernández3 

Angélica Martínez Félix4 

Erí Peña Martínez5 

Nidia Maribel León Sicairos2  6 

Adrián Canizalez Román2  7 

1Pediatric Intensive Care Service, Pediatric Hospital of Sinaloa "Dr. Rigoberto Aguilar Pico ". Culiacán Sinaloa, Mexico.

2Faculty of Medicine (CIASAP), Autonomous University of Sinaloa. Culiacán Sinaloa, Mexico.

3Department of Neonatology, Pediatric Hospital of Sinaloa "Dr. Rigoberto Aguilar Pico. Culiacán Sinaloa, Mexico.

4Department of Neonatology, Women's Hospital, SSA. Culiacán Sinaloa, Mexico.

5 Department of Pathology, Pediatric Hospital of Sinaloa "Dr. Rigoberto Aguilar Pico. Culiacán Sinaloa, Mexico.

6Research Department, Pediatric Hospital of Sinaloa "Dr. Rigoberto Aguilar Pico. Culiacán Sinaloa, Mexico.

7Research Department, Women's Hospital, SSA. Culiacán Sinaloa, Mexico.

Abstract

Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. Objective: to present a case of CPL in a premature newborn. Clinical case: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopatho- logical study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. Conclusions: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.

Keywords: Lymphangiectasis; pulmonary; congenital; premature; prognosis

Introduction

Congenital pulmonary lymphangiectasia (CPL) is a rare vascular defect of unknown etiology1,2. The main characteristic for the diagnosis is the dilatation of the lymphatic vessels in multiple areas of the lung, which include the subpleural, interlobar, perivascular, and peribronchial regions3,4. It was originally described by Rudolf Virchow in 1856. The incidence of CPL is difficult to estimate since there are only publications of case reports and small series of cases. Autopsy studies suggest that approximately 0.5-1% of newborns who died in the neonatal period had CPL6,7,8. Generally, in the CPL both lungs are affected and have a poor prog nosis with a mortality rate of 50-98%8,9. Unilateral pre sentation is extremely rare, only few cases have been reported, it has a better prognosis and sometimes it has a spontaneous resolution10,11,12. In both presentations, newborns present severe respiratory failure in the first hours of life and require support with conventional mechanical ventilation and in some cases with high- frequency oscillatory ventilation (HFOV)13.

In this report we describe the case of a preterm newborn with unilateral CPL, histologically confir med, treated conventionally with exogenous surfac tant, mechanical ventilation and with satisfactory cli nical evolution.

Clinical Case

Male newborn of 33 weeks of gestational age, de livered by cesarean section due to maternal sepsis. 19-year-old mother who died in the immediate puerperium due to severe sepsis, with no history of con sanguinity. The pregnancy was monitored, and three ultrasound reports during pregnancy showed no signs of hydrops (pleural effusion, pericardial effusion or as cites). The birth weight was 2090g, Apgar score was six at the first minute and six at fifth minute. The clinical examination showed no phenotype that could suggest genetic syndrome. Two hours after birth, the newborn presented tachypnea and cyanosis with acute respira tory distress. He was treated with rescue exogenous surfactant due to the presence of signs and symptoms of respiratory distress syndrome; catecholamines, an tibiotics, and mechanical ventilation for 22 days; con ventional ventilation with 16 cm H2O maximum mean airway pressure, followed by HFOV due to respiratory acidosis and refractory hypoxemia (pCO2 75 mmHg and pO2 45 mmHg).

The chest X-ray showed reticular infiltration in both lungs and lung distension without consolidations (Figure 1). The chest CT scan showed reticular changes in all areas, interstitial thickening, multiple bullae and air entrapment in the left lung, as well as right-sided pneumonia (Figure 2). The presence of lymphangiec tasia in other organs was dismissed through abdominal CT scan. Lung biopsy was performed and, for indica tion of pediatric surgery, left lower lobectomy. In the histopathological study, a cystic dilation of the bron- chiovascular lymphatic channels was observed (Figu re 3). Through echocardiography, ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed and surgically corrected at 30 days of age. The evolution was satisfactory, the pediatric follow-up at 19 months of age reported appropriate weight and height for his age, normal psychomotor development without infectious symptoms of the lower respiratory tract and did not require supplementary oxygen; the chest CT scan indicated interstitial thickening, small diffuse emphysematous bullae and improvement com pared to the initial CT scan (Figure 4).

Figure 1 Chest radiograph shows lung distension with inters titial infiltrate. 

Figure 2 Computed tomography shows thickening of the in- terstitium, multiple emphysematous bullae, air entrapment and hyperinflation of the left lung and right pneumonia. 

Figure 3 Hematoxylin and eosin stain, image at 40 magnifi cations shows pulmonary tissues with hemorrhage and cystic dilatation of the bronchial vascular lymphatic channels. 

Figure 4 Computed tomography taken at 19 months of age shows generalized interstitial thickening and small emphysema tous bullae in the left lung. 

Discussion

CPL is characterized by a dilatation of the pulmo nary lymphatic vessels, its etiology is unknown but is probably a pathology with maternal-fetal environ mental factors14. The pulmonary lymphatics vessels are well developed at the end of 14 weeks of gestation and the lymphangiectasia is a secondary dilatation of previously normal lymphatics vessels15. The main hy pothesis is the failure in the normal regression of the pulmonary lymphatics vessels in the fetus after the week 18-20 of gestation, and this can lead to CPL16. It has also been associated with congenital heart diseases with increased lymphatic circulation that contributes to the lymphatic dilatation, such as total anomalous pulmonary venous drainage, pulmonary stenosis and mitral stenosis, hypoplastic left heart syndrome, cor triatriatum, pulmonary vein atresia, and atrioventricu lar canal defects17,18. Although it is mostly a sporadic presentation, an autosomal recessive genetic etiology with intrafamilial variability has also been described in the LPC. Some cases have been described in association with genetic disorders, such as the Noonan, Down, Turner, Fryns, and Ullrich-Turner syndromes19.

The CPL has gone through several classifications by different authors in recent decades, the first classification of vascular malformations was proposed by Virchow in 1863 and was described as simple hemangioma, cavernous hemangioma, racemosum he mangioma, and lymphangioma5. In 1970, Noonan et al. divided CPL into three types: generalized (lym phedema with intestinal lymphangiectasia), secondary (pulmonary hypertension or venous obstruction), and primary (primary pulmonary development deficiencies)20. In 1978, Wagenaar et al. classified two types of CPL: primary and secondary with three subtypes of the primary type: limited to the lung with lung and me diastinum involvement, and the generalized type21. In 2004, Esther and Barker proposed a classification sys tem for the CPL and divided it into two categories: with primary manifestations (including generalized mani festations to organs or lungs and added to a syndro me or a genetic disease), and secondary manifestations that define those caused by the pulmonary or lymphatic venous obstruction (cardiovascular obstruction), and acquired through other means (infection, surgery, radiation or tumors)17. The last classification was pro posed in 1996 and updated in 2014 by the International Society for the Study of Vascular Anomalies (ISSVA), stratifying vascular anomalies as vascular tumors or vascular malformations, the last one includes venous and lymphatic malformations such as CPL22.

According to the classification proposed by Esther and Barker, our patient presented secondary pulmo nary lymphangiectasia due to lymphatic obstruction. We considered that the congenital heart disease (VSD and PDA) was not a causative factor of CPL, especially since it could be part of the natural history in a preterm newborn. The CPL of the left lung was probably secon dary to an obstructive process of the lymphatic system. The normal lungs have two interconnected lymphatic systems: the superficial one that drains into the sub-pleural space and the external surface of the lung and a deep system of lymphatic channels that extend into the interlobular septa and along the bronchovascular bundles. Both systems of lymphatic vessels drain into the hilum and then form the bronchomediastinal trunk that extends along the trachea and drains into the large lymphatics systems (thoracic duct) or directly into the brachiocephalic veins17,23.

The clinical presentation of our case is similar to other published reports: no significant history, pre term newborn with severe respiratory insufficiency and difficulty after a few hours of life and who recei ved supportive treatment. The supportive treatment for CPL consists of drainage of pleural and peritoneal effusions, intubation within the first hours of life, con ventional mechanical ventilation and HFOV in cases of severe hypoxemia; for neonates with persistent pulmo nary hypertension, nitric oxide and oxygenation with extracorporeal membrane are indicated24,25. Surgical resection may be indicated in some cases of CPL26,27. Treatment with pneumonectomy has only been descri bed in a newborn of 39 weeks of gestation with right sided pulmonary lymphangiectasia and refractory res piratory insufficiency to the conventional treatment. The authors concluded that pneumonectomy can be considered for cases with unilateral CPL, with respira tory insufficiency that is difficult to control and with a high probability of survival28.

After one year of age, the clinical evolution of the presented case was satisfactory, which is very impor tant, especially since most of the cases described with CPL died during the neonatal period.

Conclusions

The CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress, the prognosis will depend on the timely treatment of the newborn respiratory distress syndrome, the type of CPL and the severity of lung involvement.

Ethical responsibilities

Human Beings and animals protection: Disclosure the authors state that the procedures were followed according to the Declaration of Helsinki and the World Medical Association regarding human experimenta tion developed for the medical community.

Data confidentiality: The authors state that they have followed the protocols of their Center and Local regulations on the publication of patient data.

Rights to privacy and informed consent: The authors have obtained the informed consent of the patients and/or subjects referred to in the article. This docu ment is in the possession of the correspondence author.

Financial Disclosure: Authors state that no economic support has been asso ciated with the present study.

Conflicts of Interest: Authors declare no conflict of interest regarding the present study.

Referencias

1. Reiterer F, Grossauer K, Morris N, et al. Congenital pulmonary lymphangiectasis. Paediatr Respir Rev. 2014; 15:275-80. [ Links ]

2. Barker PM, Esther CR, Fordham LA, et al. Primary pulmonary lymphangiectasia in infancy and childhood. Eur. Respir. J. 2004; 24: 413-19. [ Links ]

3. Bouchard S, Di Lorenzo M, Youssef S, et al. Pulmonary Lymphangiectasia Revisited. J Pediatr Surg. 2000; 35:796-800. [ Links ]

4. Bellini C, Boccardo F, Campisi C, et al. Pulmonary lymphangiectasia. Lymphology. 2005; 38:111-21. [ Links ]

5. Virchow R. Gesammelte abdhandlungen zur Wissenschaftliche Medicin. Frankfurt: Meidinger, Sohn & Co., 1856, 982. [ Links ]

6. Barker PM, Esther CR Jr, Fordham LA, Maygarden SJ, Funkhouser WK. Primary pulmonary lymphangiectasia in infancy and childhood. Eur Respir J. 2004; 24:413-9. [ Links ]

7. Laurence KM. Congenital pulmonary lymphangiectasis. J Clin Pathol 1959; 12:62-9. [ Links ]

8. Gupta K, Das A, Menon P, Kakkar N, Rao KL, Joshi K. Revisiting the histopathologic spectrum of congenital pulmonary developmental disorders. Fetal Pediatr Pathol; 31:74-86. [ Links ]

9. Verlaat CW, Peters HM, Semmekrot BA, et al. Congenital pulmonary lymphangiectasis presenting as a unilateral hyperlucent lung. Eur J Pediatr. 1994;153:202-5. [ Links ]

10. Rettwitz Volk W, Schlösser R, Ahrens P, Hörlin A. Congenital unilobar pulmonary lymphangiectasia. Pediatr Pulmonol. 1999;27:290-2. [ Links ]

11. Hwang JH, Kim JH, Hwang JJ, et al. Pneumonectomy case in a newborn with congenital pulmonary lymphangiectasia. J Korean Med Sci. 2014; 29:609-13. [ Links ]

12. Tan Vinh L, Van Duc T, Huault G, et al. Unilateral congenital pulmonary lymphangiectasis. Nouv Presse Med. 1975; 4:879-82. [ Links ]

13. Yuan SM. Congenital pulmonary lymphangiectasia. J Perinat Med. 2017; 20; 45:1023-30. [ Links ]

14. Stevenson DA, Pysher TJ, Ward RM, Carey JC. Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia. Am J Med Genet A. 2006; 140:368-72. [ Links ]

15. Jakus Z, Gleghorn JP, Enis DR, Sen A, Chia S, Liu X, et al. Lymphatic function is required prenatally for lung inflation at birth. J Exp Med. 2014; 211:815-26. [ Links ]

16. Moerman P, Vandenberghe K, Devlieger H, Van Hole C, Fryns JP, Lauweryns JM. Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality. Am J Med Genet. 1993; 47:54-8. [ Links ]

17. Esther CR Jr, Barker PM. Pulmonary lymphangiectasia: diagnosis and clinical course. Pediatr Pulmonol. 2004; 38:308-13. [ Links ]

18. Bellini C, Boccardo F, Campisi C, et al. Congenital pulmonary Lymphagiectasia. Orphanet J Rare Dis. 2006; 1: 1-13. [ Links ]

19. Moros Peña M, Molina Chica I, Ruiz Moreno JA, et al. Linfangiectasia pulmonar congénita y otras malformaciones asociadas: A propósito de dos observaciones. An Esp Pediatr. 1999; 51:540-2. [ Links ]

20. Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasis. Am J Dis Child. 1970; 120: 314-19. [ Links ]

21. Wagenaar SS, Swierenga J, Wagenvoort CA. Late presentation of primary pulmonary lymphangiectasis. Thorax 1978; 33: 791-95. [ Links ]

22. Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al.; ISSVA Board and Scientific Committee. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 2015; 136:e203-14. [ Links ]

23. Javett SN, Webster I, Braudo JL. Congenital dilatation of the pulmonary lymphatics. Pediatrics 1963; 31:416-25. [ Links ]

24. Reiterer F, Grossauer K, Pfeger A, et al. Severe primary pulmonary lymphagiectasis in a premature infant: management and follow up to early childhood. Pediatr Int. 2015; 57: 166-69. [ Links ]

25. Mettauer N, Agrawal S, Pierce C, et al. Outcome of children with pulmonary lymphangiectasis. Pediatr Pulmonol. 2009; 44: 351-7. [ Links ]

26. Yalcin S, Ciftci A, Karnak I, et al. Childhood pneumonectomies: two decades’ experience of a referral center. Eur J Pediatr Surg. 2013; 23: 115-20. [ Links ]

27. Scott C, Wallis C, Dinwiddie R, et al. Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care. Pediatr Pulmonol. 2003; 35: 405-6. [ Links ]

28. Hwang JH, Kim JH, Hwang JJ, Kim KS, Kim SY. Pneumonectomy case in a newborn with congenital pulmonary lymphangiectasia. J Korean Med Sci. 2014; 29:609-13. [ Links ]

Received: February 02, 2018; Accepted: May 17, 2018

Correspondence: Jesús Javier Martínez García. E-mail: jjmtz64@hotmail.com.

Creative Commons License Este es un artículo publicado en acceso abierto bajo una licencia Creative Commons