Radiological Category Enter category here Principal Modality 1
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Radiological Category: Enter category here Principal Modality (1): Ultrasound Principal Modality (2): None Case Report # [] Submitted by: Joshua Franklin, M. D. Faculty reviewer: Abhay Srinivasan M. D Date accepted: 03 January 2002
Case History Routine head ultrasounds were performed on 2 different 1 month old formerly premature infants. Both infants are asymptomatic and have already had 2 normal head ultrasounds at 1 and 2 weeks of age.
Radiological Presentations Patient 1 Figure 1: Coronal head ultrasound. Prior image on right through same area for comparison
Radiological Presentations Patient 1 Figure 2 : Left parasagittal head ultrasound. Prior image on right through same area for comparison
Radiological Presentations Patient 1 Figure 3: Right parasagittal head ultrasound. Prior image on right through same area for comparison
Radiological Presentations Patient 2 Figure 4: Coronal head ultrasound. Prior image on right through same area for comparison
Radiological Presentations Patient 2 Figure 1: Left parasagittal head ultrasound. Prior image on right through same area for comparison
Radiological Presentations Patient 2 Figure 1: Right parasagittal head ultrasound. Prior image on right through same area for comparison
Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Grade 1 germinal matrix hemorrhage • Non-hemorrhagic germinal matrix hyperechogenicity • Periventricular leukomalacia • Intraparenchymal hemorrhage • Normal choroid plexus • Intraventricular hemorrhage
Findings and Differentials Findings: Between the ultrasounds performed at 2 weeks and 1 month of age, both cases show interval development of bilaterally symmetric, tear drop-shaped, echogenic foci in the germinal matrix. No other abnormalities were identified and there was no hydrocephalus or intraventricular hemorrhage. Differentials: • Non-hemorrhagic germinal matrix hyperechogenicity • Grade I germinal matrix hemorrhage
Discussion The germinal matrix is a highly vascular region in the ventricular wall of the developing brain containing neuronal and glial precurser cells. The vessels in this area are fragile and prone to hemorrhage. During the 3 rd trimester the germinal matrix begins to regress and it typically involutes by term; however, in premature infants it persists mostly in the caudo-thalamic groove. Most germinal matrix hemorrhages (GMH) occur in premature infants in the first week of life. In fact, in babies born at less than 32 weeks gestation and with a birth weight of less than 1500 g, 91% of germinal matrix hemorrhages occur by 6 days of life. There are 4 grades of germinal matrix hemorrhage: Grade I: Hemorrhage confined to the germinal matrix Grade II: Hemorrhage extends into ventricles without ventricular dilatation Grade III: Intraventricular extension of hemorrhage with ventricular dilatation Grade IV: Parenchymal hemorrhage Grades I and II have a generally positive prognosis while grades III and IV have higher incidence of neurologic deficit, hydrocephalus, and death. It should be noted that Grade IV GMH is now thought to represent hemorrhagic venous infarction from occlusion of ependymal veins as opposed to a true GMH.
Discussion On ultrasound, germinal matrix hemorrhage will appear as a round area of increased echogenicity in the caudo-thalamic groove. To avoid confusion, it is important to remember that the normally echogenic choroid plexus never extends as far anteriorly as the caudo-thalamic groove. Most commonly the hemorrhage will be asymmetric or unilateral. Germinal matrix hemorrhages may regress or rebleed and increase in severity, which necessitates follow up. When the hemorrhages regress, hemosiderin lined subenpendymal pseudocysts may develop. Non-hemorrhagic germinal matrix hyperechogenicity is an incompletely understood entity which can mimic grade I germinal matrix hemorrhage. This entity is found more commonly in term or near term infants and is hypothesized to represent either ischemia or infarction. Non-hemorragic germinal matrix hyperechogenicity has many associations, but it can also be an isolated finding. Associations include cerebral and non-cerebral infections, asphyxia, IUGR, prematurity, prenatal cocaine exposure, Zellweger syndrome, and Soto syndrome 1. On ultrasound, non-hemorrhagic germinal matrix hyperechogenicty has a characteristic appearance of bilaterally symmetric, tear-drop shaped, echogenic areas in the caudothalamic grooves. These areas may persist or develop into subependymal pseudocysts without hemosiderin.
Discussion Although characteristic findings have been described, distinguishing between germinal matrix hemorrhage and non-hemorrhagic germinal matrix hyperechogenicity with ultrasound alone can be difficult. MRI, especially with GRE/susceptibility sequences is more sensitive and specific for hemorrhage than ultrasound and could be used to differentiate these two entities. Some authors speculate that there could be prognostic value to distinguishing between hemorrhagic and non-hemorrhagic germinal matrix lesions and suggest using MRI 2. This however would largely represent an academic pursuit, and would not significantly alter patient management.
Diagnosis Non-hemorrhagic germinal matrix hyperechogenicity. The tear-drop shape and bilateral symmetry of the findings as well as the atypical timing for hemorrhage at 1 month of age are more suggestive of non-hemorrhagic germinal matrix hyperechogenicity than grade I GMH.
References 1. Guillerman RP. Infant Craniospinal Ultrasonography: Beyond Hemorrhage and Hydrocephalus. Semin Ultrasound CT MRI 31: 71 -85. 2. Baalen AV, Rohr A. From Fossil to Fetus: Nonhemorrhagic Germinal Matrix Echodensity Caused by Mineralizing Vasculitis Hypothesis of Fossilizing Germinolysis and Gliosis. J Child Neurol 2009; 24 - 36. 3. Statdx online. Germinal Matrix Hemorrhage. www. statdx. com 4. Cleveland clinic online pediatric radiology modules. Newborn Cranial Ultrasound. http: //pediatricradiology. clevelandclinic. org 5. Donnelly LF. Pediatric Imaging The Fundamentals. Philadelphia, PA: Elsevier, 2009; 217 -220.
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