Pediatric Imaging Pearl of the Week

View the illustrated DDX of phakomatoses

Toddler with seizures

MRI of tuberous sclerosis — Axial T2 MRI without contrast of the brain (left) shows three rounded low intensity subependymal lesions along the lateral aspect of the left lateral ventricle. Axial FLAIR MRI image without contrast of the brain (right) shows multiple bilateral poorly defined hyperintense areas located just beneath the cortex of the brain.

The diagnosis was multiple subependymal nodules and subcortical tubers in tuberous sclerosis.

School ager with seizures

MRI of tuberous sclerosis — Coronal FLAIR MRI images without contrast of the frontal (above left), middle (above right) and posterior (below) aspects of the brain shows multiple bilateral poorly defined hyperintense areas located just beneath the cortex of the brain.

The diagnosis was multiple subcortical tubers in tuberous sclerosis.

School ager with seizures

CT of Sturge-Weber syndrome — Axial CT with contrast of the brain shows diffuse leptomeningeal enhancement involving the right temporal and parietal and occipital lobes, an enlarged enhancing right choroid plexus, an enlarged medullary vein draining into the deep venous system, and some mild atrophy of the right cerebral hemisphere.

The diagnosis was Sturge-Weber syndrome.

Preschooler with seizures

MRI of optic pathway gliomas in neurofibromatosis type I — Axial (left) and coronal T1 MRI with contrast of the orbits shows marked thickening of the optic nerves bilaterally.

The diagnosis was bilateral optic pathway gliomas in a patient with neurofibromatosis type I.

Preschooler with cafe au lait spots

MRA of Moya Moya syndrome in neurofibromatosis type I — AP view of a 3D maximum intensity projection of an MRA of the neck with contrast (above) and AP (below left) and inferior (below right) views of a 3D maximum intensity projection of an MRA of the brain without contrast shows diffuse hypoplasia of the left internal carotid artery. There is stenosis and occlusion of the cavernous and supraclinoid segments of the left internal carotid artery and then there is a thin left M1 segment without evidence of collaterals in the basal ganglia. Additionally, aneurysms are noted in the left A1 and A2 segments.

The diagnosis was Moya Moya syndrome in a patient with neurofibromatosis type I.

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View the illustrated DDX of phakomatoses

Infant with biphasic stridor that is most pronounced over the upper trachea

Surgical image of double aortic arch — Surgical image through a left posterolateral thoracotomy shows the red vessel loops around the double aortic arch and the blue vessel loops around the ligamentum arteriosum. The posterior aortic arch was divided, causing an impressive release of the ring with the 2 ends of the posterior arch separating by 2 to 3 centimeters. The ligamentum arteriosum was then divided.

The diagnosis was double aortic arch.

Pediatric Imaging Pearl of the Week

View the illustrated DDX of extracranial birth trauma

Newborn who was dropped on the floor in the delivery room

CT of skull molding — Axial (left) and coronal (right) CT without contrast of the brain show the occiptal bone of the skull to be pushed underneath the parietal bones of the scalp. No skull fractures are seen.

The diagnosis was skull molding.

Infant who had a forceps delivery 3 months ago

Radiograph of calcified cephalohematoma — AP radiograph of the skull shows bilateral calcified cresenteric areas in the subcutaneous tissues on the top of the skull that do not cross suture lines.

The diagnosis was bilateral calcified cephalohematomas.

Newborn with failure to progress during delivery due to dystocia who is now apneic

CT of caput succedaneum — Axial CT without contrast of the brain shows a cresenteric high-density fluid collection in the subcutaneous tissues of the right scalp that crosses suture lines and a cresenteric low-density fluid collection in the subcutaneous tissues of the left scalp that crosses suture lines. Intracranially, there is diffuse loss of gray matter-white matter differentiation secondary to diffuse cerebral edema.

The diagnosis was a left caput succedaneum and a right subgaleal hematoma in a patient with hypoxic ischemic encephalopathy.

Newborn who experienced prolonged labor

CT of caput succedaneum — Axial (above), coronal (below left) and sagittal (below right) CT without contrast of the brain show a large low density fluid collection in the subcutaneous tissues of the scalp that crosses sutures and is seen to surround the skull on the coronal view. Intracranially, there is diffuse loss of gray matter-white matter differentiation secondary to diffuse cerebral edema.

The diagnosis was caput succedaneum in a patient with hypoxic ischemic encephalopathy.

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View the illustrated DDX of extracranial birth trauma

Newborn with an anterior chest wall defect including a split anterior half of the sternum, an omphalocele and a thoracoabdominal ectopic cordis

Surgical image of Pentalogy of Cantrell — Surgical image after the omphalocele sac was excised shows the apex of the heart (superiorly) and the liver (inferiorly). The pericardium was intact. The diaphragm was intact. The omphalocele was closed by approximating the abdominal fascia. The heart was covered with skin.

The diagnosis was Pentalogy of Cantrell.

Teenager with a chest wall deformity

Clinical image of pectus excavatum — Clinical image shows a marked concavity of the chest wall.

The diagnosis was pectus excavatum.

Newborn with congestive heart failure

Gross pathological image of infradiaphragmatic total anomalous pulmonary venous return — Gross pathological image shows the pulmonary veins joining into a common vein which descends through the diaphragm and which drains into the main portal vein within the liver.

The diagnosis was infradiaphragmatic total anomalous pulmonary venous return.

Newborn with abnormal kidneys and oligohydramnios on prenatal ultrasound

Clinical image of Potter syndrome due to pulmonary hypoplasia — Clinical image shows Potter’s syndrome – a flattened nose, recessed chin, prominent epicanthial folds and low set of abnormal ears.

The diagnosis was Potter syndrome due to pulmonary hypoplasia.

Newborn with respiratory distress

Gross pathological image of right pulmonary agenesis — Gross pathological image shows a normal appearing left mainstem bronchus and left upper lobe and left lower lobe. There is complete absence of the right mainstem bronchus and right lung.

The diagnosis was right pulmonary agenesis.

Preschooler with recurrent respiratory infections

Surgical image of congenital pulmonary airway malformation — Surgical image shows multiple cystic lesions in the right upper lobe. The right middle and right lower lobes were normal.

The diagnosis was congenital pulmonary airway malformation.

Toddler with progressive respiratory distress

Gross pathological image of congenital lobar emphysema — Gross pathological image shows a distended and emphysematous right upper lobe with focal areas of hemorrhage and subpleural bullae.

The diagnosis was congenital lobar emphysema.

Toddler with progressive respiratory distress

Clinical image of congenital lobar emphysema — Clinical image shows a hyperexpanded right hemithorax when compared to the left hemithorax.

The diagnosis was right-sided congenital lobar emphysema.

Newborn with a mass in the left chest on prenatal ultrasound

Gross pathological image of congenital diaphragmatic hernia — Gross pathological image shows the heart and great vessels flipped superiorly, revealing an extremely hypoplastic left lung. The right lung is also somewhat hypoplastic in size.

The diagnosis was left-sided congenital diaphragmatic hernia.

Teenager with an anterior chest wall deformity

Clinical image of pectus excavatum — Clinical image shows marked depression of the sternum in the midline of the chest.

The diagnosis was pectus excavatum.

Pediatric Imaging Pearl of the Week

View the illustrated DDX of craniosynostosis