Goncalves Luis,  Jamshidi Ramin,  Biyyam Deepa,  Patel Mittun,  Cornejo Patricia

Final Pr. ID: Poster #: SCI-006

The purpose of this case report is to alert the reader to a rare differential diagnosis for infra- as well as intradiaphragmatic lung sequestration. A 38-year-old G2P0010 pregnant patient was referred for fetal magnetic resonance imaging (MRI) due to a 14.2 x 20.2 x 18 mm left paraspinal hyperechogenic mass with no internal vascularity and no convincing systemic arterial feeding vessel concerning for neuroblastoma as seen by ultrasound (US) performed at 34 weeks. Fetal MRI performed the same week showed a homogeneous T2-hyperintense left paraspinal mass in close apposition to and associated with a small area of loss of continuity in the diaphragm. This has been previously reported by postnatal computerized tomography as the "split diaphragm" sign in a case of intradiaphragmatic lung sequestration (Meier AH, Eggli KD, Cillei RE. Intradiaphragmatic extralobar sequestration: a rare pulmonary anomaly. Pediatr Surg 200;44:e27-29). Thus, the differential diagnosis provided at the time was congenital intradiaphragmatic sequestration and neuroblastoma. The fetus delivered via uncomplicated spontaneous vaginal delivery at term. Postnatal US performed at the age of 15 days showed a left hyperechogenic mass extending from the left lower chest to the ipsilateral retroperitoneum through a small defect in the diaphragm, favored to represent an extrapulmonary lung sequestration. The left adrenal gland was normal. A follow-up CT performed at 7 months of age showed a 2.6 cm left paraspinal mass with no systemic arterial blood supply to support the diagnosis of sequestration. The differential diagnosis at the time included neurogenic tumor or a myofibroma arising from the diaphragm. Follow-up CT at 13 months of age showed similar findings. The patient underwent uneventful laparoscopic removal of the mass with a final pathological diagnosis of congenital adrenal rest. Congenital adrenal rest presenting as a diaphragmatic mass is rare and as been reported once in an adult patient with an adenoma in heterotopic adrenal tissue located in the left diaphragm, diganosed because of mass effect in the gastric fundus during an uper gastrointestinal series (Keirns MM. Two unusual tumors of the diaphragm. Radiology 1952; 52:542-547). We hope this report raises awareness of this entity as a potential differential diagnosis for prenatal masses seen in close relationship with the adrenal gland and/or diaphragm. Read More

Authors:  Goncalves Luis , Jamshidi Ramin , Biyyam Deepa , Patel Mittun , Cornejo Patricia

Keywords:  lung sequestration, neuroblastoma, adrenal rest

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

Chilukuri Sanjeev,  Karmazyn Boaz,  Wanner Matthew,  Brown Brandon,  Gray Brian,  Marine Megan

Final Pr. ID: Poster #: EDU-081

Bronchopulmonary sequestration (BPS) is a rare congenital abnormality of the lower airway resulting in a mass of nonfunctional lung tissue. Our purpose is to present the prenatal and postnatal imaging findings of a spectrum of BPS cases and to correlate with pathology findings.

We present a series of BPS evaluated with prenatal imaging (US and MRI) and postnatal imaging (radiographs, US, CT, and MRI). Pathology will be reviewed.

Our series includes isolated BPS, hybrid BPS associated with cystic pulmonary airway malformation (CPAM), BPS associated with diaphragmatic hernia, and challenging cases of intradiaphragmatic, subdiaphragmatic, and mediastinal sequestrations that can mimic other masses. We also include a case of sequestration with an associated communication to the GI tract (bronchopulmonary foregut malformation) and a case of isolated BPS that spontaneously involutes over time. Read More

Authors:  Chilukuri Sanjeev , Karmazyn Boaz , Wanner Matthew , Brown Brandon , Gray Brian , Marine Megan

Keywords:  Sequestration, Fetal, Bronchopulmonary

Patel Chinky,  Maller Vijetha

Final Pr. ID: Poster #: EDU-097

An isolated anomalous systemic arterial supply to a segment of the lung without associated sequestration is a rare congenital pulmonary abnormality. This anomaly was initially believed to be a type of pulmonary sequestration (Pryce type 1). However, it was later found to have different characteristics than a true sequestration. The affected pulmonary segment is supplied by an aberrant systemic artery, however, there is no abnormality of lung parenchyma such as a mass or cystic change to suggest sequestration. In addition, there is a normal pulmonary venous drainage into the left atrium and a normal bronchial tree. However, there is a fistulous communication between the abdominal aortic branch supplying the lung segment and the respective pulmonary vein which can cause an elevated systemic-to-pulmonary flow ratio due to left-to-left shunting. Sade and associates introduced a sequestration spectrum for the classification of various complexes of bronchovascular anomalies. Clements and Warner presented this anomaly as a “pulmonary malinosculation spectrum.”

Patients with this condition are usually asymptomatic. They may present with hemoptysis and exertional dyspnea with a continuous heart murmur. A large shunt can lead to left-sided overload and congestive heart failure.

On chest radiographs, the appearance of a tubular opacity from the upper abdomen to the right lower lobe (abdominal aortic supply to the lung) may mimic the appearance seen in Scimitar syndrome (a type of partial anomalous pulmonary venous return with hypoplasia of the right lung, which is drained by an anomalous pulmonary vein into the systemic venous system). CT angiography chest is required to make an accurate diagnosis to distinguish this anomaly from Scimitar syndrome as well as exclude the possibility of sequestration, as management is different in these conditions.

In this educational poster, we will review the clinical presentation, describe the imaging findings of anomalous systemic arterial supply to normal lung using examples from our own institution, and discuss the treatment options as well as the natural progression of the disease. Recognition of the imaging features in anomalous systemic arterial supply to normal segments of the lung is important to identify patients at risk of developing left-to-left shunt. Endovascular coil embolization or surgical ligation of the aberrant systemic artery to the lung segment is usually the treatment of choice in symptomatic patients. Read More

Authors:  Patel Chinky , Maller Vijetha

Keywords:  malinosculation, sequestration, bronchovascular

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

Misiura Anne,  Urbine Jaqueline,  Mallon Mea,  Kazmi Faaiza,  Malik Archana,  Poletto Erica

Final Pr. ID: Poster #: EDU-009

Pulmonary sequestration comprises a spectrum of congenital lung anomalies in which one or more of the following are present: bronchial sequestration, aberrant systemic arterial supply, and/or anomalous pulmonary venous drainage. Treatment is surgical; however, evaluation of the lesion with chest computed tomography (CT) prior to surgery is vital for planning as there are different surgical interventions required based on the components of the sequestration. Sequestrations can be associated with additional congenital abnormalities, some of which can also be evaluated by chest CT prior to surgery. Read More

Authors:  Misiura Anne , Urbine Jaqueline , Mallon Mea , Kazmi Faaiza , Malik Archana , Poletto Erica

Keywords:  Pulmonary, Congential, 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