We report the case of a neonatal male with alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV). ACD/MPV is a rare, fatal disorder of the development of the alveolar vasculature. In this disorder, alveolar capillaries are reduced in number and are improperly located within the walls of the alveoli impeding oxygen exchange. In addition to alveolar capillary abnormalities, there are other associated abnormalities with the cardiovascular, gastrointestinal tract, and genitourinary systems. In this report, we describe the case of a term male neonate with a prenatal diagnosis of hypoplastic left heart who developed respiratory distress shortly after birth. The patient required prolonged ECMO and underwent evaluation with CT angiography of the chest. CT findings included diffuse bilateral interstitial thickening with enhancing airspace disease scattered in the left lung. The respiratory distress continued to be unresponsive to treatment, with difficulty weaning ECMO and respiratory support. Life support was withdrawn on day 30. Pathologic examination at autopsy demonstrated ACD/MPV. Our case adds to the limited literature available in describing imaging findings of ACD/MPV and support for early diagnosis with lung biopsy as it can prevent aggressive futile interventions. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Singh Jaspreet, Tuburan Smyrna

Keywords: Interstitial Lung Disease, diffuse lung developmental disease

A 4 day old female, born at term without complications and with reported proper prenatal care was referred to a tertiary care center after her parents were notified of an abnormal 17-OHP on newborn screening. Work-up for suspected congenital adrenal hyperplasia was initiated. An abdominal ultrasound showed a 2.8 x 3.6 x 3.7 cm heterogeneously hypoechoic right suprarenal mass with peripheral calcification and large feeding vessels. An MRI abdomen was then obtained, which showed a round circumscribed 3.6 x 3.5 x 3.4 cm T1 hypointense and T2 iso-hyperintense heterogeneously enhancing mass. The radiologist raised the suspicion for an adrenocortical tumor, with neuroblastoma or teratoma as less likely etiologies. An MIBG scan was then obtained, revealing absence of activity in the region of the known mass, further supporting the diagnosis of an adrenocortical neoplasm. This information, in addition to normal levels of VMA and HVA, reduced the likelihood that the mass was of neural crest origin. IR then performed an ultrasound guided biopsy of the right adrenal mass. Pathology reported features consistent with an adrenal cortical neoplasm and without features of neuroblastoma. Due to concern for potential aggressive behavior, pathology recommended complete surgical excision. There is a paucity of literature secondary to the extremely low incidence of congenital adrenal cortical neoplasms. Therefore, a multidisciplinary committee, which included pediatric surgery and radiology, was held. The consensus was that the mass would be best treated by, and is amenable to, surgical excision. At 28 days old, the patient underwent an open right adrenalectomy. Pathology confirmed an adrenal cortical neoplasm of uncertain malignant potential. The post-operative course was without complications. Per pediatric hematology-oncology recommendations, a nuclear medicine whole body bone scan was obtained and demonstrated no osteoblastic metastasis. The patient was discharged and will continue to follow up with hematology-oncology and endocrinology as an outpatient. As illustrated above, the early and correct identification of a potentially aggressive neoplasm by radiology accelerated the treatment course, allowing for potentially curative interventions. This case demonstrates the necessity for multidisciplinary management to include both diagnostic and interventional radiologists and to communicate an appropriate range of differential diagnoses, regardless of incidence. Read More

Meeting name: IPR 2016 Conjoint Meeting & Exhibition , 2016

Authors: Bowman Sean, Trinh Austin, Tuburan Smyrna

Keywords: Adrenal, Tumor, adrenal cortical neoplasm

Demonstrate how a pediatric radiology division at a single institution structures short-interval feedback to residents. Describe how the division collects interval resident feedback. Demonstrate resident responses to this pilot program of instituting standardized weekly feedback. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Gilyard Shenise, Dickson Paula, Loewen Jonathan, Richer Edward, Riedesel Erica, Tuburan Smyrna

Keywords: education, feedback, radiology resident

Demonstrate how the Pediatric Radiology Department at a single institution structures short-interval feedback to residents. Describe how the department collects short-interval feedback from residents regarding their experiences. Demonstrate resident responses to this pilot program of instituting standardized weekly feedback Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Gilyard Shenise, Dickson Paula, Loewen Jonathan, Richer Edward, Riedesel Erica, Tuburan Smyrna

Keywords: education, feedback, radiology resident