ElHassan et al. Journal of Medical Case Reports 2010, 4:284 http://www.jmedicalcasereports.com/content/4/1/284 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access A neonate with left pulmonary artery thrombosis and left lung hypoplasia: a case report Nahed O ElHassan1*, Christi Sproles1, Ritu Sachdeva2, Sadaf T Bhutta3, Joanne S Szabo1 Abstract Introduction: Spontaneous intrauterine arterial thrombosis and congenital pulmonary hypoplasia are rare conditions and have not been reported to occur together. The literature rather includes two reports of babies with neonatal pulmonary artery occlusion and post-infarction cysts of the lungs. Case presentation: We report a case of a live Caucasian male newborn with left lung hypoplasia that occurred in association with left pulmonary artery thrombosis. Despite a critical neonatal course, including extracorporeal membrane oxygenation, this infant is alive and well at 18 months of age without any neurodevelopmental sequelae or reactive airway disease. Conclusion: This association suggests the possibility of an intrauterine vascular event between the fifth and eighth weeks of gestation during early pulmonary artery and lung development. Introduction The prevalence of symptomatic neonatal arterial throm- bosis is approximately 1 in 40,000 births, with 90% of cases linked to indwelling intra-arterial catheters [1-4]. Other risk factors are sepsis, polycythemia, maternal dia- betes, asphyxia, and inherited thrombophilias [1,3,4]. Very few cases of spontaneous neonatal arterial throm- bosis have ever been described [3,4]. Although congenital pulmonary hypoplasia can be idiopathic, it is most commonly associated with con- ditions that reduce the intrathoracic space [5]. A lim- ited number of reports exist of neonates with congenital pulmonary hypoplasia and no clear evi- dence of fetal chest compression [5-7]. Two previous reports exist in the literature of neonates with conge- nital left pulmonary occlusion and postinfarction cysts of the lung [8,9]. We here describe the case of a liveborn male infant with spontaneous intrauterine left pulmonary artery thrombosis and probably associated left lung hypoplasia. * Correspondence: ElhassanNahed@uams.edu 1Department of Pediatrics, Neonatology, University of Arkansas for Medical Sciences, College of Medicine, Arkansas Children’s Hospital, 1 Children’s Way, Slot 512-5, Little Rock, AR 72202-3591, USA Full list of author information is available at the end of the article Case presentation A male Caucasian baby was born by spontaneous vagi- nal delivery at 35 weeks of gestation to a 26-year-old gravida 3, para 1 mother. The mother had well-con- trolled type 2 diabetes mellitus and two previous mis- carriages of unclear etiology. No evidence was found of congenital malformations on prenatal ultrasounds. No family history was known of spontaneous thrombosis. The birth weight was 2353 g and appropriate for gesta- tional age. At 20 minutes of life, he became severely tachypneic. A sepsis evaluation was performed and intravenous antibiotics were begun. Chest radiography revealed a right tension pneumothorax and complete left lung field opacity. The infant was intubated and a chest tube was placed. An echocardiogram at 10 hours of life revealed right ventricular dilatation and hyper- trophy with flattening of the ventricular septum, con- sistent with persistent pulmonary hypertension of the neonate (PPHN). Left pulmonary artery (LPA) blood flow could not be visualized and a thrombus appeared to occlude the LPA (Figure 1). Inhaled nitric oxide was administered. Computed tomography angiography (CTA) at 16 hours of life was performed and showed an intact tracheobronchial tree and a markedly hypo- plastic left lung (Figure 2). The main and right pul- monary arteries were normal in caliber, with an occlusion of the LPA by a low-density mass suggestive © 2010 ElHassan et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.