Rapid Communication| Volume 47, ISSUE 12, e21-e25, December 2012

Download started.


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


      We report the case of 2-week-old female infant with cystic lung disease who presented with mild tachypnea and had no history of mechanical ventilation. Chest CT demonstrated multiple air-filled cystic lesions in right upper lobe, and the patient subsequently underwent a right upper lobectomy. Histology revealed cystic lesions located in the pulmonary parenchyma and showed that the lesions were lined by lymphatic endothelium and were communicating with dilated lymphatic vessels in the interstitium. Additionally, multinucleated foreign body giant cells were attached to the lumen of the cyst. On the basis of these findings, we considered this a case of persistent interstitial pulmonary emphysema (PIPE) with massive pneumatic expansion of the lymphatic vessels, resulting in cystic lesions with lymphatic endothelium in the pulmonary parenchyma. While PIPE is extremely rare in term non-ventilated infants, our case demonstrated that this disease should be added to the differential diagnosis of cystic lung diseases with lymphatic endothelium even in infants without mechanical ventilation. When cystic lesions and symptoms persist despite conservative treatment, open or thoracoscopic resection is an appropriate option for diagnosis and treatment.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Faul J.L.
        • Berry G.J.
        • Colby T.V.
        • et al.
        Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome.
        Am J Respir Crit Care Med. 2000; 161: 1037-1046
        • Stocker J.T.
        Cystic lung disease in infants and children.
        Fetal Pediatr Pathol. 2009; 28: 155-184
        • Stocker J.T.
        • Madewell J.E.
        Persistent interstitial pulmonary emphysema: another complication of the respiratory distress syndrome.
        Pediatrics. 1977; 59: 847-857
        • Donnelly L.F.
        • Lucaya J.
        • Ozelame V.
        • et al.
        CT findings and temporal course of persistent pulmonary interstitial emphysema in neonates: a multiinstitutional study.
        AJR Am J Roentgenol. 2003; 180: 1129-1133
        • Jabra A.A.
        • Fishman E.K.
        • Shehata B.M.
        • et al.
        Localized persistent pulmonary interstitial emphysema: CT findings with radiographic-pathologic correlation.
        AJR Am J Roentgenol. 1997; 169: 1381-1384
        • Leonidas J.C.
        • Bhan I.
        • McCauley R.G.
        Persistent localized pulmonary interstitial emphysema and lymphangiectasia: a causal relationship?.
        Pediatrics. 1979; 64: 165-171
        • Berk D.R.
        • Varich L.J.
        Localized persistent pulmonary interstitial emphysema in a preterm infant in the absence of mechanical ventilation.
        Pediatr Radiol. 2005; 35: 1243-1245
        • Pursnani S.K.
        • Amodio J.B.
        • Guo H.
        • et al.
        Localized persistent interstitial pulmonary emphysema presenting as a spontaneous tension pneumothorax in a full term infant.
        Pediatr Surg Int. 2006; 22: 613-616
        • Yamada S.
        • Hisaoka M.
        • Hamada T.
        • et al.
        Congenital pulmonary lymphangiectasis: report of an autopsy case masquerading as pulmonary interstitial emphysema.
        Pathol Res Pract. 2010; 206: 522-526
        • Lee C.H.
        • Kim Y.D.
        • Kim K.I.
        • et al.
        Intrapulmonary cystic lymphangioma in a 2-month-old infant.
        J Korean Med Sci. 2004; 19: 458-461
        • Minato H.
        • Kaji S.
        • Kinoshita E.
        • et al.
        Solitary intrapulmonary cystic lymphangioma in an infant: a case report with literature review.
        Pathol Res Pract. 2010; 206: 851-856
        • Takahashi M.
        • Okumura N.
        • Kameyama K.
        • et al.
        Emergency surgery for macrocystic lymphatic malformation in the lung of a neonate.
        J Thorac Cardiovasc Surg. 2011; 142: e133-e134
        • Jetley N.K.
        • Al Assiry A.H.
        • Dawood A.A.
        • et al.
        Diagnostic dilemmas, course, management and prognosis of traumatic lung cysts in children.
        Indian J Pediatr. 2010; 77: 200-202
        • Schimpl G.
        • Schneider U.
        Traumatic pneumatoceles in an infant: case report and review of the literature.
        Eur J Pediatr Surg. 1996; 6: 104-106
        • Brooks J.G.
        • Bustamante S.A.
        • Koops B.L.
        • et al.
        Selective bronchial intubation for the treatment of severe localized pulmonary interstitial emphysema in newborn infants.
        J Pediatr. 1977; 91: 648-652
        • Martinez-Frontanilla L.A.
        • Hernandez J.
        • Haase G.M.
        • et al.
        Surgery of acquired lobar emphysema in the neonate.
        J Pediatr Surg. 1984; 19: 375-379
        • Schwartz A.N.
        • Graham C.B.
        Neonatal tension pulmonary interstitial emphysema in bronchopulmonary dysplasia: treatment with lateral decubitus positioning.
        Radiology. 1986; 161: 351-354