Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature
Abstract Background Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM managemen...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2023-05-01
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| Series: | BMC Pediatrics |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12887-023-04049-3 |
| _version_ | 1797827334788612096 |
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| author | Megan Ottomeyer Charles Huddleston Rachel M. Berkovich David S. Brink Joyce M. Koenig Kurtis T. Sobush |
| author_facet | Megan Ottomeyer Charles Huddleston Rachel M. Berkovich David S. Brink Joyce M. Koenig Kurtis T. Sobush |
| author_sort | Megan Ottomeyer |
| collection | DOAJ |
| description | Abstract Background Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited. Case presentation We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. Conclusions Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies. |
| first_indexed | 2024-04-09T12:46:25Z |
| format | Article |
| id | doaj.art-a28b38eabb4b4451825854fde70894bd |
| institution | Directory Open Access Journal |
| issn | 1471-2431 |
| language | English |
| last_indexed | 2024-04-09T12:46:25Z |
| publishDate | 2023-05-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pediatrics |
| spelling | doaj.art-a28b38eabb4b4451825854fde70894bd2023-05-14T11:28:01ZengBMCBMC Pediatrics1471-24312023-05-012311910.1186/s12887-023-04049-3Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literatureMegan Ottomeyer0Charles Huddleston1Rachel M. Berkovich2David S. Brink3Joyce M. Koenig4Kurtis T. Sobush5Department of Pediatrics, Division of Neonatology, Saint Louis UniversityDepartment of Surgery, Division of Pediatric Cardiothoracic Surgery, Saint Louis UniversityDepartment of Radiology, SSM Health Cardinal Glennon Children’s HospitalDepartment of Pathology, Division of Pediatric Pathology, Saint Louis UniversityDepartment of Pediatrics, Division of Neonatology, Saint Louis UniversityDepartment of Pediatrics, Division of Pediatric Pulmonary and Sleep Medicine, Saint Louis UniversityAbstract Background Congenital pulmonary airway malformations (CPAMs) are a heterogenous collection of congenital lung malformations, often diagnosed prenatally. The Stocker Type III CPAM is a rare CPAM sub-type, and, when large, may be associated with hydrops. Furthermore, reports of CPAM management which may include surgical resection in extreme preterm infants are limited. Case presentation We report a case of a female neonate born at 28 weeks of gestation with severe respiratory distress and diffuse pulmonary opacification on the right concerning for a large congenital lung lesion. This lesion was not detected on routine antenatal imaging, and she did not have clinical findings of associated hydrops. Her respiratory status improved dramatically after surgical resection of a mass at 12 day of age. The mass was consistent pathologically with a Stocker Type III CPAM. Lung expansion showed subsequent improvement at 16 months of age. Conclusions Our case describes a preterm neonate with severe respiratory distress that was found postnatally to have a large, unilateral congenital lung lesion despite a normal prenatal ultrasound. Additionally, this lesion required excision early in life due to severity of respiratory compromise. This case highlights that rare congenital lung lesions, like this rare sub-type of CPAM, should remain a diagnostic consideration in neonates with severe respiratory distress. Early lung resection for CPAM in preterm infants is not well described and the favorable outcomes of this case help expand perspectives on potential management strategies.https://doi.org/10.1186/s12887-023-04049-3CPAMCongenital lung malformationPrematurityRespiratory distress |
| spellingShingle | Megan Ottomeyer Charles Huddleston Rachel M. Berkovich David S. Brink Joyce M. Koenig Kurtis T. Sobush Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature BMC Pediatrics CPAM Congenital lung malformation Prematurity Respiratory distress |
| title | Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature |
| title_full | Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature |
| title_fullStr | Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature |
| title_full_unstemmed | Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature |
| title_short | Early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate: a case report and review of the literature |
| title_sort | early resection of a rare congenital pulmonary airway malformation causing severe progressive respiratory distress in a preterm neonate a case report and review of the literature |
| topic | CPAM Congenital lung malformation Prematurity Respiratory distress |
| url | https://doi.org/10.1186/s12887-023-04049-3 |
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