Focal Pulmonary Lymphangiectasia Containing an Infantile Hemangioma: An Unprecedented Cause of Refractory Recurrent Unilateral Pneumothoraces

Pneumothoraces are a significant cause of neonatal respiratory distress. We report for the first time an infant with refractory recurrent unilateral left-sided pneumothoraces resulting from a pulmonary lymphangiectasia containing an infantile hemangioma. Known causes of recurrent pneumothoraces include both congenital and acquired lymphangiectasia. Infantile hemangiomas occur in viscera, especially liver, but presence in the lung is very rare. We postulate that lymphangiectasia in this infant was secondary to local lymphatic obstruction by the hemangioma. We recommend consideration of lymphangiectasia from an isolated mass such as congenital infantile hemangiomas in the differential as a newly identified cause of recurrent unilateral pneumothoraces. Established facts and novel insights: Established facts: Pneumothoraces are often caused by underlying neonatal lung pathology. Refractory recurrent unilateral pneumothoraxes are rare in the neonate. Novel insights: Infantile hemangioma can cause neonatal lung pathology. Congenital lymphangiectasia containing an infantile hemangioma for first time is reported to cause of refractory recurrent unilateral pneumothorax in the neonate. Alan Patrick Kenny1*, Jareen Meinzen-Derr2,3, Manish Patel N4, Kevin Bove1,5 and Henry Akinbi T1,2 1Department of Pediatrics, University of Cincinnati, USA 2Department of Neonatology, University of Cincinnati, USA 3Department of Otolaryngology, University of Cincinnati, USA 4Department of Radiology, University of Cincinnati, USA 5Department of Pathology and Laboratory Medicine, University of Cincinnati, USA Annals of Clinical Case Reports Pediatrics Remedy Publications LLC., | http://anncaserep.com/ 2019 | Volume 4 | Article 1765 2 Alan Patrick Kenny, et al., thoracentesis was performed with evacuation of 60 mL air. At the level 3 NICU, chest radiograph revealed left pneumothorax (Figure 1A); a chest tube was placed. He was extubated 8 h later and remained stable in a head hood until the next day when he was reintubated for increased work of breathing and had a second left needle thoracentesis and a second left chest tube placed for recurrent left pneumothorax with resolution on follow-up radiography. Chest radiography on the fifth day confirmed recurrent left tension pneumothorax. A left thoracic pigtail catheter was placed and the patient was replaced on the mechanical ventilator. On day 6, the pneumothorax was again resolved. Both chest tubes were removed the next day and the patient was extubated. Chest radiography revealed small pneumothorax reaccumulation and patient received a nitrogen washout. On day 8, the pneumothorax was slightly larger than before (Figure 1B). The patient was transferred to regional level IV NICU for surgical consultation on recurrent pneumothorax and persistent left upper lung lobe lucency, both of which were confirmed in the admission radiography. Chest Computed Tomography (CT) on day 9 revealed a moderate pneumothorax and non-specific gas-filled lesion in the peripheral anterior left upper lobe measuring 1.5 cm2 × 1.7 cm2 with a posterior rimmed nodule, likely calcified, containing soft tissue density (Figure 1C) positioned non-dependently along the anteroinferior aspect. It was deemed atypical for congenital pulmonary airway malformation, pneumatocele, bronchogenic cyst, congenital lobar emphysema, and pulmonary interstitial emphysema. On day 10 the patient underwent left thoracotomy and left upper lobe wedge resection of a cystic bleb, putatively lymphatic in nature in the left upper lobe (Figure 2A). The pneumothorax resolved after placement of thoracostomy tube during surgery, and the patient was extubated postoperatively. The chest tube was removed on day 12, and feedings were advanced with discharge home on day 16. Microscopy revealed a large subpleural markedly dilated lymphatic vessel focally lined by attenuated lymphatic endothelial cells (CD31 positive and weakly D2-40 positive; Figures 2F and 2F') with macrophage reaction. Adjacent to this dilated lymphatic channel was a circumscribed vascular lesion with lobular proliferation of capillary vessels. Capillary vessel lumens were slightly variable in size and most of them were patent and contained red blood cells (Figure 2B). These proliferating capillary vessels were lined by Glut-1 and CD31 positive, flat and plump endothelial cells (Figure 2D and 2E). In between capillary vessels were perithelial cells (SMA positive). This capillary lesion was compressing normal lung tissue except for collapsed alveoli with focal fresh hemorrhage; residual bronchioles were patent. Alveolar spaces were lined by normal pneumocytes (surfactant positive and cytokeratin positive; Figure 2C). CD68 stain highlighted the macrophage reaction in the large lymphatic channel and showed few alveolar macrophages in collapsed lung. Pathological interpretation was local lymphangiectasia produced by an expansile infantile hemangioma resulting in recurrent pneumothoraces. On day 25, the patient was reported to have no skin hemangiomas and was noted to have grunting only after feeds. His left lung lucency had resolved on follow-up chest radiograph (Figure 1D). On day 54, normal head and abdominal ultrasounds ruled out diffuse hemangiomas. On day 104, the patient was discharged from home nursing care with normal feeding and breathing. Discussion Pneumothorax occurs in 1% to 2% of live births but is only symptomatic in 0.05% to 0.07% [4-6]. Among 26,007 VLBW (Very Low Birth Weight) infants in 1999, 6.3% had pneumothorax [7]. In the Vermont-Oxford Network, pneumothoraces approached 15% in infants with birth weight 501 g to 750 g [7]. It occurs in 19% of infants with renal anomalies [6] and in 20% to 50% of infants with meconium aspiration syndrome [8]. The incidence has been decreasing Figure 1: Chest radiography (CXR) from patient over time. A) On DOL 3, left pneumothorax prompted transfer to a level III NICU for thoracostomy tube and surfactant treatment. B) On DOL 8, the pneumothorax recurred for the third time. C) DOL 9: CT revealed left upper lobe gas-filled lesion with a likely calcified rim with soft tissue in anteroinferior portion. D) DOL 25: Cystic lesion resolved post wedge resection. Congenital Pulmonary Airway Malformation (CPAM) Pulmonary sequestration