Ebai Jerky,  Nicholas Jennifer,  Foust Alexandra

Final Pr. ID: Poster #: CR-023

An afebrile 23-month-old boy with history of VSD, PFO and GERD who presented with one day of acute left sided abdominal pain, nausea and vomiting. Following a benign clinical work-up only significant for mild leukocytosis and transient small bowel to small bowel intussusception on abdominal US, he was discharged home. He re-presented a few days later with worsening abdominal pain and low grade fever and was found to have increased leukocytosis and elevated CRP. KUB demonstrated a new left lower lobe opacity. Non-contrast CT abdomen showed a well circumscribed ovoid hyperdense soft tissue mass in the inferomedial left lower lobe, a small left pleural effusion, and adjacent passive atelectasis. On chest MRI , the left lower lobe soft tissue mass demonstrated T1 isointense to hyperintense and STIR hypointense signal with mild restricted diffusion and no enhancement on post-contrast images. MRA of the chest was negative for any feeding vessel extending to the lesion. Video-assisted thoracotomy surgery revealed a torsed, bluish left lower lobe mass adherent to the mediastinum with thrombosed vessels at its pedicle. Pathology results confirmed the suspected diagnosis of an infarcted and hemorrhagic extralobar pulmonary sequestration.

Pulmonary sequestration is a rare congenital malformation of the foregut, where a nonfunctional supranumary segment of the lung lacking a normal connection to the tracheobronchial tree develops below the normal lung bud. The sequestration can be intralobar or extralobar, depending on the pleural investment, has arterial supply from systemic circulation, and venous drainage via pulmonary and/or systemic venous drainage. Torsed pulmonary sequestrations are extremely rare and occur when the sequestration twists around the axis of its vascular pedicle resulting in ischemia or infarction of the non-functional lung tissue. In the case of a full torsion, the vascular supply may not be visualized at imaging. Thus it is important to consider pulmonary sequestration as a differential diagnosis in a pediatric patient presenting with lower chest or abdominal pain with a circumscribed lower lobe pulmonary mass, even without characteristic feeding vessels on MRI/MRA. Read More

Authors:  Ebai Jerky , Nicholas Jennifer , Foust Alexandra

Keywords:  Pulmonary, Sequestration, Torsed

Quintero Pulgarín Daniel,  Rozo Juan,  García Torres Alberto,  Forero Melo Julian,  Acosta Izquierdo Laura

Final Pr. ID: Poster #: CR-047

Pulmonary sequestration is a rare - but well-characterized - congenital malformation of the foregut, the hallmark of the diagnosis consists in the detection of an anomalous systemic arterial supply to a segment of the lung that is isolated from the tracheobronchial tree and therefore nonfunctional, with a pulmonary and/or systemic venous drainage. Depending on the relationship of the abnormal parenchyma with the pleura, it can be classified as intralobar or extralobar.
Nevertheless, the spectrum of congenital lung malformations includes lesions that are predominantly parenchymal, predominantly vascular or hybrid, with variable foregut or airway components intertwined. The exact physiopathological mechanisms for each one of these malformations are yet to be fully understood.
We present a 15-month-old girl with a history of dilated cardiomyopathy. Mild-to-moderate left ventricular (LV) enlargement and a small persistent ductus arteriosus (PDA) were seen on the echocardiogram. The size of the PDA did not explain the LV dilation. An angiography was done to assess the coronary arteries, which were normal. However, it showed an aberrant vessel from the descending aorta to the left lower lobe and severe pulmonary hypertension in the supplied lung, reactive to oxygen. A CT was performed to assess the airway, ruling out the possibility of a pulmonary sequestration and confirming the diagnosis of an isolated major aortopulmonary collateral artery (MAPCA). No evidence of structural heart disease was seen. Unifocalization and ductal ligation were planned.
MAPCA in the absence of an associated congenital heart disease is a rare entity, most seen in preterm babies. The isolated MAPCA tends to regress spontaneously in most cases. However, in a smaller group of patients, it can persist and lead to recurrent respiratory tract infection,
pulmonary hypertension, and lastly heart failure due to left-to-right shunt. Contrast-enhanced computed tomography (CT) is an excellent method for correct identification and treatment guidance.
The purpose of this presentation is to describe an unusual case of our practice, in which the imaging finding of an aberrant arterial supply to the lung is not always related to a pulmonary sequestration, and therefore to draw attention to the fact that in the spectrum of congenital lung
anomalies that are predominantly vascular, isolated MAPCA can be found as the only alteration. Read More

Authors:  Quintero Pulgarín Daniel , Rozo Juan , García Torres Alberto , Forero Melo Julian , Acosta Izquierdo Laura

Keywords:  MAPCA, Congenital malformation, Pulmonary Sequestration

Ferreira Botelho Marcos,  Greenfield Susan,  Hughes Katrina,  John Susan

Final Pr. ID: Poster #: EDU-027

Congenital bronchopulmonary malformations are frequently encountered in the routine clinical practice. The specific diagnosis of pulmonary sequestration, associated or not with congenital pulmonary airway malformations (hybrid lesion) has important treatment and prognostic values. The purpose of this exhibit is to explain the current understanding of pulmonary sequestrations and improve diagnostic accuracy among general and pediatric radiologists when describing such lesions. Read More

Authors:  Ferreira Botelho Marcos , Greenfield Susan , Hughes Katrina , John Susan

Keywords:  Pulmonary sequestration, Congenital bronchopulmoary malformation, Hybrid lesion