Stein Jill,  Browne Lorna

Final Pr. ID: Poster #: CR-005

Congenital focal lung malformations (CLM) are a heterogeneous group of lesions that have been commonly classified by their features and the presence of systemic vascular supply into bronchial atresia, congenital pulmonary airway malformation, bronchogenic cyst and bronchopulmonary sequestration, however, pathologically there is considerable overlap not recognized by this classification method. Literature has hypothesized that a common pathogenetic origin of developmental bronchial obstruction, variable in timing and extent, is responsible for the spectrum observed.

The purpose of this case report is to describe the findings of an unusual CLM with concurrent intra and extrapulmonary lesions demonstrated by pre and postnatal imaging, gross and microscopic pathology.

A 19 year-old G1P0 female presented at 34 weeks gestation for evaluation of a fetal chest mass, first identified by US at 32 weeks. Fetal US showed a large right thoracic lung lesion containing two distinct macro cysts; fetal MR demonstrated T2 hyperintense signal throughout the lesion and increased volume of the affected lung with resultant mild mediastinal shift. No systemic feeding vessel was evident.

The baby was born at 39 weeks and required intubation due to respiratory distress. Postnatal CTA showed a solid lesion right thoracic apex outlined by pleural air with systemic arterial supply from the subclavian artery, no visible airways, and a central cyst with peripheral atelectasis. There was an additional unilocular cyst with air-fluid level in the perihilar right middle lung.

Thoracotomy on day 4 described an extrapulmonary sequestration (EPS) right thoracic apex and cystic lesions in the right upper and middle lobes, all of which were resected without complication. Pathology revealed similar histology throughout all resected tissue characterized by markedly dilated proximal bronchi and parenchymal maldevelopment with immature alveolar spaces as is seen in bronchial atresia, in addition to diffuse pulmonary interstitial glycogenosis.

This case of a CLM with concurrent solid and cystic lesions supports a common pathogenetic mechanism based on developmental bronchial obstruction both with and without retention of embryonic systemic vascular communication. Prenatal classification into distinct lesions is hampered by overlapping pathological features. Characterization of vascular and airway connections and awareness of potential complications are most important to guide counseling and therapy. Read More

Authors:  Stein Jill , Browne Lorna

Keywords:  CPAM, Sequestration, Bronchial Atresia

Ledbetter Karyn,  Adler Elena,  Subramanyam Rajeev,  Mahmoud Mohamed,  Kline-fath Beth,  Fleck Robert

Final Pr. ID: Poster #: SCI-046

Congenital lung lesions continue to be diagnosed prenatally with increasing frequency. While the etiologies frequently encountered have been well described, there are contradictions in the literature about the prevalence of pathologies. Additionally, no gender or lobar predilection has been confidently reported. The goal of this study was to delineate the epidemiology of congenital lung lesions and their mimickers at our institution to provide comparison to published data. Read More

Authors:  Ledbetter Karyn , Adler Elena , Subramanyam Rajeev , Mahmoud Mohamed , Kline-fath Beth , Fleck Robert

Keywords:  bronchial atresia, Congenital, CPAM

Valdez Quintana Melissa,  Ugas Carlos,  Katekaru Tokeshi Doris,  Matos Rojas Irma,  Delgado Edgar

Final Pr. ID: Poster #: CR-073

We report a case of a term neonate who shortly after birth developed respiratory distress and left-sided pneumothorax. The pneumothorax was drained by a chest tube and the patient was referred to our institution. On day 17 of life a chest Xray and chest CT were performed demonstrating a multiloculated cystic lesion compromising the left upper lobe, most likely representing congenital pulmonary airway malformation (CPAM) type II (Figure 1). No abnormal systemic vessels were identified. At that time the patient was diagnosed with B influenza infection and the surgical intervention was deferred. Over the next several days the patient demonstrated improvement of the respiratory distress and progressive decrease of supplementary oxygen need. On day 25 of life the patient presented significant clinical improvement and, at physical examination, breath sounds were found on the left hemithorax. A follow-up chest xray demonstrated spontaneous resolution of the prior visualized cystic lesion that was confirmed with a chest CT (Figure 2).

Postnatal spontaneous resolution of CPAM have been reported in only few cases, with resolution of the malformation in few months or years. Although we do not have pathologic confirmation, we postulate that our case represent CPAM type II that resolved spontaneously before the first month of life. The management of CPAM diagnosed during the neonatal period remains controversial, some authors recommend postpone surgical intervention in asymptomatic neonates and in patients with prompt resolution of respiratory distress without other significant anomalies.
Our case illustrates the possibility of spontaneous resolution of CPAM and adds to the few reported cases that recommend conservative treatment. Read More

Authors:  Valdez Quintana Melissa , Ugas Carlos , Katekaru Tokeshi Doris , Matos Rojas Irma , Delgado Edgar

Keywords:  CPAM, spontaneous resolution, neonate, CT

Maddocks Alexis,  Ayyala Rama,  Jacobs Shimon,  Miller Russell,  Duron Vincent

Final Pr. ID: Poster #: SCI-013

Congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS), and hybrid lesions are the most common congenital lung lesions. They are primarily diagnosed prenatally via ultrasound and further characterized by MRI. While most affected neonates are asymptomatic at birth, some may experience varying severities of respiratory distress requiring intervention. We seek to develop a prognostic model for prediction of post-natal outcomes in patients with congenital lung lesions using fetal MRI calculated observed to expected normal lung volume (O/E NLV). Read More

Authors:  Maddocks Alexis , Ayyala Rama , Jacobs Shimon , Miller Russell , Duron Vincent

Keywords:  CPAM, prenatal, MRI