Figure 1
Teaching Case
SectionPaediatric radiology
Case TypeClinical Cases
Authors
G.D.E. Papini1,2, S. Viganò1,2, G. Isgrò4, V. Sansone2,5, B. Cotticelli3, F. Sardanelli2,3
1Scuola di Specializzazione in Radiodiagnostica, Facoltà di Medicina e Chirurgia, Università degli Studi di Milano, Milano/IT
2Dipartimento di Scienze Medico-Chirurgiche, Università degli Studi di Milano, San Donato Milanese, Milano/IT
Unità di 3Radiologia, 4Terapia Intensiva e 5Neurologia, IRCCS Policlinico San Donato; San Donato Milanese/IT
Connected authors
2 months, male
A two month-old male baby with transposition of the great arteries treated with Rashkind procedure at 15 days of life.
He presented no facial dysmorphism, lethargy, hypotonia, impaired gaze and poor papillary light reflex.
Ten days after surgical correction with arterial switch, he developed clonic seizures involving the right hemisoma.
He presented no facial dysmorphism, lethargy, hypotonia, impaired gaze and poor papillary light reflex.
Ten days after surgical correction with arterial switch, he developed clonic seizures involving the right hemisoma.
Multicystic encephalomalacia.
Cerebral infarction
Hydranencephaly
Multicystic encephalomalacia
Porencephaly
Perinatal asphyxia
Transcranial brain ultrasonography showed enlarged third and lateral ventricles and bilateral cortical fronto-parietal hyperechogenity (Fig. 1-3).
MRI was performed four days later with a 1.5-T scanner (Siemens, Sonata). T1-weighted, T2-weighted, FLAIR, gradient-echo as well as diffusion-weighted images (DWI) were acquired (Fig. 4-8). The MRI showed diffuse, bilateral lesions with cystic appearance involving the cortex and subcortical white matter.
MRI was performed four days later with a 1.5-T scanner (Siemens, Sonata). T1-weighted, T2-weighted, FLAIR, gradient-echo as well as diffusion-weighted images (DWI) were acquired (Fig. 4-8). The MRI showed diffuse, bilateral lesions with cystic appearance involving the cortex and subcortical white matter.
Hydranencephaly is unlikely to be the correct diagnosis as it represents a condition in which most of the brain mantle (cortical plate and hemispheric white matter) has been destroyed and resorbed. The cerebral hemispheres appear nearly completely replaced by cerebrospinal fluid (CSF), the brain stem is usually atrophic, and the cerebellum almost always has a normal appearance [1].
Congenital cyanotic heart disease is the most common cause of embolic cerebral infarction in newborns, and stroke in the neonatal period tipically presents with neonatal seizures, hypotonia, or lethargy [2]. However this is unlikely to be the correct diagnosis as in newborns the infarcted cortex becomes edematous and therefore becomes isointense with the underlying unmyelinated white matter, and a focal infarction appears as a "missing" segment of the cortex, especially on T2-weighted images, with typical restricted diffusion on DWI images.
Porencephaly is not the correct answer as the term is used to describe focal cavities with smooth wall and minimal surrounding glial reaction. Those cavities result from focal brain destruction prior to approximately the 26th gestational week, are lined by displastic gray matter, and usually accompanied by polimicrogyria [1].
In little patients with perinatal asphyxia, MRI shows bilateral, symmetric signal anomalies with four different patterns of damage (deep structures, primarily cortical, periventricular white matter, and mixed pattern) [3]. In the chronic phase, the injured areas of the brain are shrunken, ex vacuo ventricular dilation may be present, and the white matter in the boundary zones (“watershed areas”) is abnormally hyperintense.
Multicystic encephalomalacia usually is characterized by a bilateral, multiple-cystic pattern of the lesions. The affected area appears as an ill-defined area showing prolonged T1 and T2 relaxation times, containing loculations of fluid with some heterogeneity, as a result of combination of variably sized glial septae and CSF within the lesions [1]. The lesions are located in the cortex and peripheral white matter, primarily in the intervascular boundary zones (watershed areas), suggesting a diffuse insult to the brain late in gestation, or during birth [1]. If caused by thrombotic or embolic infarction, the affected area are along the distribution of a major cerebral artery branch. Although recent evidence suggests that herpesviruses may represent an etiologic agent, severe hypoxia is the more common etiology of multicystic encephalomalacia [4]. The CT appearance is that of hypodense tissue containing cysts of various sizes also presenting septations and sometimes calcifications [3].
Congenital cyanotic heart disease is the most common cause of embolic cerebral infarction in newborns, and stroke in the neonatal period tipically presents with neonatal seizures, hypotonia, or lethargy [2]. However this is unlikely to be the correct diagnosis as in newborns the infarcted cortex becomes edematous and therefore becomes isointense with the underlying unmyelinated white matter, and a focal infarction appears as a "missing" segment of the cortex, especially on T2-weighted images, with typical restricted diffusion on DWI images.
Porencephaly is not the correct answer as the term is used to describe focal cavities with smooth wall and minimal surrounding glial reaction. Those cavities result from focal brain destruction prior to approximately the 26th gestational week, are lined by displastic gray matter, and usually accompanied by polimicrogyria [1].
In little patients with perinatal asphyxia, MRI shows bilateral, symmetric signal anomalies with four different patterns of damage (deep structures, primarily cortical, periventricular white matter, and mixed pattern) [3]. In the chronic phase, the injured areas of the brain are shrunken, ex vacuo ventricular dilation may be present, and the white matter in the boundary zones (“watershed areas”) is abnormally hyperintense.
Multicystic encephalomalacia usually is characterized by a bilateral, multiple-cystic pattern of the lesions. The affected area appears as an ill-defined area showing prolonged T1 and T2 relaxation times, containing loculations of fluid with some heterogeneity, as a result of combination of variably sized glial septae and CSF within the lesions [1]. The lesions are located in the cortex and peripheral white matter, primarily in the intervascular boundary zones (watershed areas), suggesting a diffuse insult to the brain late in gestation, or during birth [1]. If caused by thrombotic or embolic infarction, the affected area are along the distribution of a major cerebral artery branch. Although recent evidence suggests that herpesviruses may represent an etiologic agent, severe hypoxia is the more common etiology of multicystic encephalomalacia [4]. The CT appearance is that of hypodense tissue containing cysts of various sizes also presenting septations and sometimes calcifications [3].
References
[1] Raybaud C (1983) Destructive lesions of the brain. Neuroradiology 25:265-91 (PMID: 6605492)
[2] Lanska MJ, Lanska DJ, Horwitz SJ, Aram DM (1991) Presentation, clinical course, and outcome of childhood stroke. Pediatr Neurol 7:333-41 (PMID: 1764134)
[3] Barkovich AJ, Westmark K, Partridge C, Sola A, Ferriero DM (1995) Perinatal asphyxia: MR findings in the first 10 days. AJNR Am J Neuroradiol 16:427-38 (PMID: 7793360)
[4] Weidenheim KM, Bodhireddy SR, Nuovo GJ, Nelson SJ, Dickson DW (1995) Multicystic encephalopathy: review of eight cases with etiologic considerations. J Neuropathol Exp Neurol 54:268-75 (PMID: 7876894)
Case information
| URL: | https://www.eurorad.org/case/8930 |
| DOI: | 10.1594/EURORAD/CASE.8930 |
| ISSN: | 1563-4086 |
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7
Figure 8
Figure 1
Figure 2
Figure 3
Figure 4
Figure 5
Figure 6
Figure 7
Figure 8
