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J Thorac Cardiovasc Surg 2009;138:237-239
© 2009 The American Association for Thoracic Surgery

Brief Clinical Report

Persistent interstitial pulmonary emphysema requiring pneumonectomy

Elizabeth Belcher, MRCP, FRCS, PhDa, M. Ali Abbasi, MRCSa, David M. Hansell, FRCP, FRCRb, Lorrette Ffolkes, MRCP, FRCPathc, Andrew G. Nicholson, FRCPathc, Peter Goldstraw, FRCSa,*

a Department of Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Radiology, Royal Brompton Hospital, London, United Kingdom
c Department of Histopathology, Royal Brompton Hospital, London, United Kingdom

Received for publication March 27, 2008; revisions received May 2, 2008; accepted for publication June 9, 2008.

* Address for reprints: Peter Goldstraw, FRCS, Department of Thoracic Surgery, Royal Brompton Hospital, Sydney St, London, SW3 6NP, UK. Telephone 44 (0) 2073518559. (Email: p.goldstraw@rbht.nhs.uk).

The first 20% of the full text of this article appears below.


   Introduction
 
Persistent interstitial pulmonary emphysema (PIPE) is a rare but serious disorder of neonates characterized by abnormal accumulation of air in the pulmonary interstitium. Although localized PIPE has the potential for curative resection, generalized PIPE affecting both lungs carries a 100% mortality.1Go Classically associated with antecedent mechanical ventilation, it can occur in the absence of prior ventilatory support.2Go


   Clinical Summary
 
A growth-restricted (0.4 growth percentile) male twin was delivered by means of caesarian section at 36 weeks and 2 days' gestation. He required feeding and temperature support but no ventilatory assistance. On day 14 after delivery, he showed signs of respiratory distress. Chest radiographic analysis showed hyperinflation of the left lung with cystic air spaces and contralateral shift of the mediastinum. Computed tomographic (CT) analysis of the chest confirmed hyperinflation and multiple cystic air spaces throughout the left lung. The right lung (Figure 1, A ) showed marked compression by the displaced mediastinum. The differential diagnosis was believed to be either congenital cystic adenomatoid malformation (CCAM) or congenital lobar emphysema. Oxygen (fraction of inspired oxygen, 0.3), physiotherapy, and intravenous antibiotics were instituted.


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  		Figure 1. A, Enhanced computed tomographic scan showing . . . [Full Text of this Article]

 



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[Abstract] [Full Text] [PDF]


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