School ager with Trisomy 21

Radiograph of atlanto occipital instability — Lateral radiograph of the cervical spine in flexion (left) shows a normal atlanto dens interval and normal relationship of the occipital condyles to the lateral masses of C1 while on extension (right) there is posterior translation of the occipital condyles in relation to the lateral masses of C1.

The diagnosis was atlanto occipital instability in a patient with Trisomy 21.

Preschooler with Trisomy 21

Radiograph of atlanto axial instability — Lateral radiographs of the cervical spine show a mildly enlarged atlanto dens interval in neutral position (left), that increases significantly on flexion (center) and reduces on extension (right).

The diagnosis was atlanto axial instability in a patient with Trisomy 21.

Our DDX For This Week Is

View the illustrated DDX of cervical spine instability.

Newborn with bilious vomiting

Surgical image of malrotation with midgut volvulus — Surgical image shows the midgut is dusky but not necrotic.

The diagnosis was malrotation with midgut volvulus.

Pediatric Imaging Pearl of the Week

View the illustrated DDX of cervical vertebral junction anomalies.

Teenager with progressive spasticity

CT of os odontoideum — Sagittal CT without contrast of the cervical spine (left) shows a large well-corticated ossicle posterior and superior to the anterior arch of C1 and superior to the dens, hypertrophy of the anterior arch of C1, and a large atlanto-dens interval due to atlanto-axial dislocation. Sagittal T2 MRI without contrast of the cervical spine (right) shows a narrow spinal canal secondary to the atlanto axial dislocation with spinal cord compression and abnormal bright signal in the spinal cord at C1-C2.

The diagnosis was dystopic os odontoideum.

School ager with headaches

CT of basilar invagination — Sagittal CT without contrast of the cervical spine shows the tip of the dens is 6 mm above Chamberlain’s line.

The diagnosis was basilar invagination.

Our DDX For This Week Is

View the illustrated DDX of cervical vertebral junction anomalies.

Newborn with abdominal distension and failure to pass meconium

Surgical image of Hirschsprung disease — Surgical image shows a visible transition zone in the mid sigmoid colon with the forceps at the actual site of transition. The sigmoid colon (to the right) is dilated and had ganglion cells on biopsy. The rectum (to the left), descending into the pelvis, is not dilated and had no ganglion cells on biopsy.

The diagnosis was Hirschsprung disease.

Pediatric Imaging Pearl of the Week

View the illustrated DDX of Chiari malformations.

Newborn with polyhydramnios on prenatal ultrasound and bilious vomiting after birth

Surgical image of jejunal stenosis due to jejunal web — Surgical image after the bowel has been incised shows a jejunal web within the bowel lumen.

The diagnosis was jejunal stenosis due to a jejunal web.

Newborn with vomiting

Surgical image of jejunal atresia — Surgical image shows a dilated stomach and duodenum (above) due to an atresia in the proximal jejunum. The remainder of the jejunum is spiraled around the ileocolic artery (apple peel atresia).

The diagnosis was jejunal atresia.

Newborn with vomiting

Surgical image of jejunal atresia — Surgical image obtained after resection and anastomosis of an atresia in the proximal jejunum (upper left) shows the jejunum spiraled around the ileocolic artery (apple peel atresia).

The diagnosis was jejunal atresia.

School ager with crampy abdominal pain and a fixed small bowel-small bowel intussusception

Surgical image of Non-Hodgkin lymphoma in the mesentery — Surgical image obtained after intussusception reduction and resection of the intussusception’s pathological lead point shows multiple large mesenteric lymph nodes.

The diagnosis was diffuse B-cell lymphoma (Non-Hodgkin lymphoma).

School ager with crampy abdominal pain and a non-transient small bowel-small bowel intussusception

Surgical image of small bowel - small bowel intussusception due to Non-Hodgkin lymphoma — Laparoscopic surgical image (above) shows the small bowel-small bowel intussusception. Surgical image of the small bowel after reduction of the intussusception (below) shows the serosa is pink-tan and intact with a 1.5 x 0.6 cm puckered, red, firm area that when opened in pathology revealed a 2.6 x 2.0 cm red-pink mass.

The diagnosis was diffuse B-cell lymphoma (Non-Hodgkin lymphoma) causing small bowel-small bowel intussusception.

School ager with abdominal pain and a non-transient small bowel-small bowel intussusception

Gross pathological image of small bowel - small bowel intussusception due to Burkitt lymphoma — Gross pathological images of the resected segment of intussuscepted small bowel shows on its serosal surface (above) a 2.0 x 1.2 cm irregular, tan-white centrally ulcerated and umbilicated firm area. The opened image of small bowel (below) reveals a 3.4 x 2.9 x 1.2 cm irregular, tan-white, smooth, firm mass which was sectioned to reveal smooth, tan-white, glistening, homogenous cut surfaces.

The diagnosis was Burkitt lymphoma causing small bowel-small bowel intussusception.

Newborn with emesis and bloody stools with an ileocolic intussusception which spontaneously perforated during reduction

Surgical image of ileocolic intussusception — Surgical image shows forceps in a 1.5 cm perforation in the mid point of the transverse colon. The ascending colon (on the right) is distended and is full of necrotic intussuscepted ileum up to the level of the hepatic flexure.

The diagnosis was ileocolic intussusception.

Infant with a large abdominal mass

Gross pathological image of inflammatory myofibroblastic tumor — Sectioned gross pathological image shows a tan to pink, firm, irregular solid mass measuring 14 x 13.5 x 8 cm. The mass was well encapsulated. No hemorrhagic necrosis or cystic degeneration was seen.

The diagnosis was inflammatory myofibroblastic tumor.

Infant with constipation

Surgical image of Hirschsprung disease — Surgical image shows a visible transition zone between the dilated sigmoid colon (in the upper middle being pinched between the two fingers) which contained ganglion cells and the narrowed rectum (in the lower middle) which was found to be aganglionic.

The diagnosis was Hirschsprung disease.

Toddler with constipation

Surgical image of Hirschsprung disease — Surgical image shows decompressed small bowel (on the left) and an extremely dilated sigmoid colon which contained ganglion cells (in the upper middle) with a transition zone near the end of the sigmoid colon (on the right) and a narrowed rectosigmoid colon and rectum (in the lower middle) which turned out to be aganglionic.

The diagnosis was Hirschsprung disease.

Newborn with a lumbar myelomeningocele

Radiograph of lacunar skull in Chiari II malformation — Lateral radiograph of the skull shows a disordered arrangement of the inner table of the skull.

The diagnosis was lacunar skull in a patient with a Chiari II malformation.

Newborn with an abnormal bump in the posterior midline at the skull base

CT of Chiari III malformation — Axial CT without contrast of the cervical spine shows a defect in the posterior skull at the craniocervical junction with cerebellar tissue herniating posteriorly through it.

The diagnosis was Chiari III malformation with an occipital encephalocele.

Newborn with a myelomeningocele

MRI of Chiari II malformation and myelomeningocele — Sagittal T1 MRI without contrast of the brain (left) shows a small posterior fossa with downward cerebellar tonsil herniation and a small fourth ventricle. There is kinking of the spinal cord at the cervico-medullary junction. Sagittal (above right) and axial (below right) T2 MRI without contrast of the spine shows a low-lying conus medullaris with the spinal cord nerve roots terminating in a posteriorly located cerebrospinal fluid filled sac which is not covered by skin at the level of the L5-S1 vertebral bodies.

The diagnosis was Chiari II malformation with a myelomeningocele.

Infant status post myelomeningocele repair

MRI of Chiari II malformation — Sagittal T1 MRI without contrast of the brain shows a small posterior fossa with downward cerebellar tonsil herniation and a small fourth ventricle. There is kinking of the spinal cord at the cervico-medullary junction. There is also agenesis of the body and rostrum of the corpus callosum.

The diagnosis was Chiari II malformation and agenesis of the corpus callosum.

School ager with vomiting at night

CT of Chiari I malformation — Sagittal midline 2D reconstruction of CT without contrast of the brain shows the cerebellar tonsils to be herniating > 5 mm beneath the foramen magnum.

The diagnosis was Chiari I malformation.