Prenatal diagnosis of esophageal atresia (EA) remains challenging, with indirect signs such as polyhydramnios, a small or absent stomach bubble, and a dilated upper esophageal pouch often being nonspecific. Only 10-40% of EA cases are diagnosed prenatally. Fetal MRI can overcome ultrasound limitations; however, constraints like motion can hinder evaluation of the esophagus. One approach to improve image quality is super-resolution imaging with slice-to-volume reconstruction (SVR). This technique enhances diagnostic accuracy by generating high-resolution 3D images from standard fetal MRI slices. We present two instances where super-resolution imaging with SVR was employed to accurately diagnose EA and assess the presence or absence tracheoesophageal fistulas (TEF). Case 1: A 30 3/7-week gestation male fetus was referred for fetal MRI following ultrasonographic findings of a dilated right atrium, a single umbilical artery, a small fetal stomach, and a urinary tract dilation observed at 27 2/7 weeks. Fetal MRI revealed a dilated upper esophageal pouch with a small amount of fluid in the distal esophagus, and a diminutive stomach. 3D-rendered super-resolution images demonstrated a dilated atretic upper esophageal pouch and a TEF originating from the left mainstem bronchus. The application of super-resolution imaging with SVR was crucial to determine the precise origin of the TEF from the left mainstem bronchus, findings that were later confirmed by bronchoscopy and surgery. Case 2: A 33 2/7-week gestation male fetus was referred for fetal MRI due to polyhydramnios and inability to visualize the stomach on an ultrasound performed at 31 weeks. Fetal MRI confirmed polyhydramnios with a distended, fluid-filled proximal esophagus, consistent with EA. The stomach was decompressed, and the distal esophagus was not visualized. 3D-rendered super-resolution imaging with SVR confirmed the dilated upper esophagus and revealed that it ended 0.5 cm below the level of the carina. The imaging also demonstrated a gap between the distal end of the esophageal pouch and the gastroesophageal junction at the level of the diaphragm, supporting the absence of the distal esophagus. The use of super-resolution imaging with SVR provided detailed, pre-surgical 3D anatomical depictions in both cases of EA, with and without TEF. This technique demonstrates significant potential for accurately delineating the relevant surgical anatomy, which can improve surgical planning and outcomes. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Loken Delaney, Goncalves Luis, Patel Mittun

Keywords: 3D Imaging, Esophageal Atresia, Tracheoesophageal Fistula

Congenital diaphragmatic hernia (CDH) is a life-threatening fetal anomaly characterized by herniation of abdominal contents into the thoracic cavity due to a defect in the diaphragm, most commonly left-sided. Accurate prenatal diagnosis and risk stratification are essential for guiding perinatal management, parental counseling, and prognostication. While ultrasound remains the first-line modality for initial detection, fetal MRI provides superior soft tissue contrast and volumetric assessment, offering critical additional information, particularly in complex or equivocal cases. This educational exhibit serves as a practical guide for radiologists interpreting fetal MRI for CDH. We review the typical imaging features of CDH on MRI, including identification of herniated organs, mediastinal shift, and assessment of the ipsilateral and contralateral lungs. Emphasis is placed on the standardized approach to measuring total and observed-to-expected lung volumes (o/e TLV), as well as lung area to head cicumference ratio (LHR), which serve as key prognostic markers. The exhibit also addresses the significance of liver position, stomach location, and diaphragmatic defect size, all of which correlate with postnatal outcomes and surgical complexity. We illustrate how MRI findings influence the clinical decision-making process, including eligibility for fetal intervention such as fetoscopic endoluminal tracheal occlusion (FETO). Radiologists play a vital role in the multidisciplinary care of these patients by providing accurate and reproducible measurements that impact prognosis and management. This exhibit aims to equip radiologists with the essential tools and knowledge required to confidently evaluate CDH on fetal MRI and contribute meaningfully to perinatal planning. Read More

Meeting name: IPR 2026 Congress , 2026

Authors: Mousa Abeer, Perez Rachel, Goncalves Luis

Keywords: Fetal, Congenital Diaphragmatic Hernia, Lung

Congenital high airway obstruction syndrome (CHAOS) is a rare but life-threatening fetal condition resulting from obstruction of the fetal upper airway, most commonly due to laryngeal or tracheal atresia. Accurate prenatal diagnosis is critical for perinatal management and potential surgical planning, including consideration of an ex utero intrapartum treatment (EXIT) procedure. While ultrasound is typically the initial modality for detection, fetal MRI provides superior anatomic detail and plays an increasingly important role in confirming the diagnosis, assessing the level and extent of obstruction, and evaluating associated anomalies. In this case series, we present fetal MRI findings from four cases of CHAOS, each diagnosed in the second trimester. MRI demonstrated hallmark features including bilaterally enlarged, hyperintense lungs on T2-weighted images, flattened or inverted diaphragms, central compression of the heart, and dilated airways distal to the point of obstruction. Associated anomalies, including ascites and hydrops fetalis, were variably present. In all cases, MRI provided critical information for determining the severity and level of obstruction, distinguishing CHAOS from other causes of airway obstruction or other thoracic masses such as congenital pulmonary airway malformation (CPAM), and aiding multidisciplinary decision-making regarding delivery planning and neonatal resuscitation. This case series underscores the diagnostic value of fetal MRI in the evaluation of CHAOS and highlights key imaging features that can facilitate accurate prenatal diagnosis. Early and precise recognition of this rare condition allows for appropriate counseling of expectant parents and coordination of specialized perinatal interventions that may improve neonatal outcomes. Read More

Meeting name: IPR 2026 Congress , 2026

Authors: Mousa Abeer, Perez Rachel, Goncalves Luis

Keywords: Fetal, Airway, Atresia

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

Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023

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

Keywords: lung sequestration, neuroblastoma, adrenal rest

Kagami-Ogata syndrome (KOS) is a rare and potentially underdiagnosed imprinting disorder caused by paternal uniparental disomy for chromosome 14 or microdeletions and epimutations involving differentially methylated regions (DMRs) at 14q32.2. Depending on phenotypic expression, KOS may mimic trisomy 18 and asphyxiating thoracic dysplasia. Therefore, awareness is essential for prenatal diagnosis. Coat hanger ribs as seen by x-rays have been reported as s pathognomonic sign but have not been reported prenatally. Herein we present images of the coat hanger ribs as seen by low-dose computerized tomography (CT) as well as detailed imaging of the multiple phenotypic features by ultrasound and MRI of KOS in two cases seen prenatally. Case 1: G1P0 singleton pregnancy had fetal echocardiogram at 25 weeks due to concern for congenital heart disease, thickened nuchal fold and thickened skin of the thoracic wall. The exam showed left superior vena cava draining to coronary sinus, dilated right atrium and right ventricle, total anomalous pulmonary venous return, and hydrops. Skin edema was redemonstrated by follow up US at 29 weeks. Fetal MRI and follow-up US at 32 weeks showed polyhydramnios, diffuse skin edema, prominent cheeks and frontal bossing by 3D US, mild shortening of the ulna and right clubfoot, and a bell-shaped thorax by MRI. Chromosomal microarray was positive for terminal deletion of 14q32.2, consistent with KOS. Coat hanger ribs were present on postnatal x-rays. The patient is G-tube and tracheostomy tube dependent, has global developmental delay, neuromuscular scoliosis, left hip subluxation, wrist contractures and planovalgus feet at the age of 2 ½ years. Case 2: G3P1, singleton pregnancy with negative non-invasive prenatal testing (NIPT). US performed at 29 weeks showed polyhydramnios, clenched hands, and bell-shaped small thorax. Multimodality fetal imagin (US, MRI and CT) at 32 weeks showed a narrowed elongated thorax by US and MRI but ribs were poorly visualized. Additional anomalies included polyhydramnios, rhizomelic bone shortening, clenched hands, and prominent cheeks by 3D US. Low-dose CT showed coat hanger ribs. Postnatal x-rays confirmed coat hanger ribs and other abnormalities. Postnatal genetic testing showed paternal uniparental disomy for chromosome 14, consistent with KOS. The patient has restrictive lung disease due to hypoplastic thoracic cage, hypotonia, scoliosis, developmental delay, and umbilical hernia at 13 months. Read More

Meeting name: SPR 2023 Annual Meeting & Postgraduate Course , 2023

Authors: Goncalves Luis, Patel Mittun, Wermers Josh, Simmons Curtis, Vaughn Jennifer, Pfeifer Cory, Cornejo Patricia

Keywords: kagami-ogata, fetal MRI, fetal CT