1. MedPix Medical Image Database COW - Case of the Week Case Contributor: Natalia P Gaulke Affiliation: Childrens Hospital of Dayton, OH

2. MedPix No: 14435 - History Pt Demographics: Age = 1 y.o. Gender = boy 9 month old male presented to ED in respiratory distress. History was provided by Mother. Patient developed upper respiratory symptoms for the past week, previously had been healthy meeting age appropriate milestones. Mother denies trauma. During ED course patient developed tachypnea and seizure activity. He was intubated, CT of the head was obtained and neurological consult was placed. PMH: Former 34 week gestation infant, intubated at birth for one day. Uncomplicated C-section delivery. Immunizations not up to date. Social: Mother has prior history of child neglect. Downloaded by (-1)

3. MedPix No: 14435 - EXAM & LABS VitalsTemperature: 36.3 C (97.3 F)Heart Rate: 146Respirations: 23Blood Pressure: 92/55 mmHgO2 Sat (%): 100 %Weight: 7 kgPhysical Exam Constitutional: sedated. HENT: Ophtalmologic exam is negative for retinal hemorrhages. Head: Anterior fontanelle is flat. Nose: Nasal discharge (scant clear crusting around nares b/l) present. Mouth/Throat: Mucous membranes are moist. Cardiovascular: Normal rate, regular rhythm, S1 normal and S2 normal. Pulses are palpable. No murmur.Pulmonary/Chest: Breath sounds normal. Abdominal: Soft. Bowel sounds are normal, no distension, no hepatosplenomegaly, no tenderness. Genitourinary: Penis normal. Circumcised. No discharge found. Musculoskeletal: no edema, no deformity and no signs of injury. Lymphadenopathy: No occipital or cervical adenopathy is present. Skin: Skin is warm and moist. Capillary refill takes less than 3 seconds. Turgor is turgor normal. No petechiae or purpura or injuries anywhere on skin LABS:sodium of 131, potassium 3.3, chloride 99, total carbon dioxide 18, glucose 121, BUN 7, creatinine less than 0.3, calcium 8.3, and albumin of 3.6. Bilirubin 1.1, AST is 1225, ALT is 777 and total protein is 7.7. Ammonia is 57. white count is 7.9 thousand. Neutrophils are 51, bands are 26. RIDP is positive for rhinovirus. Hepatitis panel is negative. Serum HSV negative. Negative blood cultures.Hemoglobin is 10.6, hematocrit is 30.4, and platelet count is 236,000. PT, PTT and INR are prolonged at 24.7, 32.3. INR of 2.3. Negative toxicology. Genetic evaluation was initiated: etyhylene glycol, plasma AA, alpha1 antitrypsin, methanol, isopropranolol, acylcarnitines all normal. Hereditary fructose intolerance returned negative.

4. Diffuse cerebral edema The ventricles are very small. Basilar cisterns are nearly - inapparent. The cerebral hemispheres show abnormal low attenuation with limited gray- white matter differentiation. Sulcal effacement. No acute intracranial hemorrhage is seen. No midline shift is evident. Gray-white - differentiation is preserved in the cerebellum. Downloaded by (-1)

5. Diffuse cerebral edema The ventricles are very small. Basilar cisterns are nearly - inapparent. The cerebral hemispheres show abnormal low attenuation with limited gray- white matter differentiation. Sulcal effacement. No acute intracranial hemorrhage is seen. No midline shift is evident. Downloaded by (-1)

6. Diffuse cerebral edema The ventricles are very small. Basilar cisterns are nearly - inapparent. The cerebral hemispheres show abnormal low attenuation with limited gray- white matter differentiation. Sulcal effacement. No acute intracranial hemorrhage is seen. No midline shift is evident. Gray-white - differentiation is preserved in the cerebellum. Downloaded by (-1)

7. Hypoxic-ischemic injury Patchy T2 prolongation present throughout the - cerebral hemispheres bilaterally and symmetrically involving cortex and white matter are similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. Downloaded by (-1)

8. Hypoxic-ischemic injury Patchy T2 prolongation present throughout the - cerebral hemispheres bilaterally and symmetrically involving cortex and white matter are similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. Downloaded by (-1)

9. Hypoxic-ischemic injury Patchy T2 prolongation present throughout the - cerebral hemispheres bilaterally and symmetrically involving cortex and white matter are similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. Downloaded by (-1)

10. Hypoxic-ischemic injury Patchy T2 prolongation present throughout the - cerebral hemispheres bilaterally and symmetrically involving cortex and white matter are similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. Downloaded by (-1)

11. Hypoxic-ischemic injury Patchy T2 prolongation present throughout the - cerebral hemispheres bilaterally and symmetrically involving cortex and white matter are similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. Downloaded by (-1)

12. FINDINGS CT head WO contrast:Small ventricles. Basilar cisterns are nearlyinapparent. The cerebral hemispheres show abnormal low attenuation with limited gray-white matter differentiation and loss of normal sulcal pattern. No acute intracranialhemorrhage is seen. No midline shift is evident. At bone windows, no fracture is identified. MRI brain WO contrast:Findings: Patchy T2 prolongation present throughout thecerebral hemispheres bilaterally and symmetrically involving cortex and white matter, similar involvement of the globus pallidus bilaterally extending into cerebral peduncles. Restricted diffusion is present in these areas as well. On the T2 and gradient echo images, there is lowsignal intensity present involving the cortices bilaterally andsymmetrically which is most evident in the frontal and occipital regions and may represent mineralization or hemorrhage. There is no asymmetric involvement of the right frontotemporal region compared with the left side. Findings most likely represent hypoxic ischemic injury due to metabolic disorder. Infection less likely given the bilaterally symmetric distribution.Skeletal survey:1. No acute or healing fractures are identified.2. The sutures are widened consistent with cerebral edema.

13. DIFFERENTIAL DIAGNOSIS What is your Differential Diagnosis? Hypoxic - Ischemic Injury due to: - Toxic/Infectious Encephalopathy - Metabolic encephalopathy - Traumatic cerebral edema - -

14. Diagnosis: Hypoxic-ischemic injury, Rhinovirus Dx Confirmed by: Diagnosis by exclusion-negative for: HSV, hepatitis, blood cultures, genetic, toxicology.

15. DISCUSSION Hypoxic-ischemic injury from malnutrition and exacerbated by Rhinovirus infection. Diagnosis by exclusion: negative testing for possible infections agents such as HSV, hepatitis, negative blood cultures, negative genetic testing for inborn metabolic errors, negative toxicology. Ophthalmologic exam negative, no signs of retinal hemorrhages. Skeletal survey negative. No signs of acute trauma. Positive social indicators for possible child neglect. The patient initially presented to ED department in respiratory distress. Further evaluation revealed diffuse cerebral edema, acute liver failure, and metabolic acidosis. Potential causes for diffuse cerebral edema would include trauma, infection and inborn errors of metabolism which were not confirmed by laboratory or radiology tests. - Hypoxic-ischemic injury frequently results in death or long-term neurologic deficit and disability of variable degree, depending on size of the insult. Treatment is primarily supportive and does not prevent the ongoing injury following the causative insult. Early detection of cerebral injury in important in order to prevent further insult and potentially reduce degree of the injury. - - Early imaging findings can be subtle and difficult to detect. - Regardless of specific cause of injury the process that leads to hypoxic injury is due to diminished cerebral blood flow and reduced oxygenation of brain parenchyma. The following areas of brain with higher energy demands will demonstrate early signs of injury in young children less than 1 year old: basal ganglia, lateral thalami, dorsal midbrain and cerebral cortex (particularly the anterior frontal and parieto-occipital cortex). Early CT findings include: loss of gray-white matter differentiation, cisternal and sulcal effacement, as it was seen in this patient. *White cerebellum sign* in which diffuse edema and hypo attenuation of the cerebral hemispheres contrasts with spared cerebellar parenchyma, this sign was also demonstrated on the patients CT of the head. MRI imaging may demonstrate increased T2 signal throughout white matter, cortex and basal ganglia with corresponding restricted diffusion in these areas, as it was seen in this patient. - In presented case, MRI findings are most consistent with diffuse hypoxic ischemic injury with possible underlying metabolic dissorder, less likely due to infectious cause taking into concideration symmetric involvement of the brain parenchyma. - - - References: - 1. Huang, B. Y., and M. Castillo. *Hypoxic-Ischemic Brain Injury: Imaging Findings from Birth to Adulthood.* Radiographics 28.2 (2008): 417-39. Web. 24 July 2014.. - - 2. Donnelly, L. F. (2005). Section 7 Neuro in Diagnostic imaging: Pediatrics. Salt Lake City, Utah: Amirsys. - - 3. Grant, E. (n.d.). Diagnostic Imaging for Radiology | STATdx. Hypoxic Ischemic Injury. Retrieved July 24, 2014, from https://my.statdx.com/STATdxMain.jsp?rc=false#dxContent;hie__term -