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Objective:To summarize the clinical and imaging features of pediatric neuroglial heterotopia (NGH) in different locations.Methods:The clinical and preoperative imaging data of 9 patients (6 boys and 3 girls, median age 3 months, range from 1 to13 months) with NGH confirmed by pathology were retrospectively reviewed in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science and Technology from October 2009 to December 2020. All patients underwent preoperative CT or/and MR examination. Follow-up was performed in 12 to 60 months after operation, with a median follow-up time of 24 months. The location, range, size, density/signal intensity of the lesions were reviewed.Results:Of all 9 cases, three cases were located in nose (2 extranasal type and 1 mixed type), with the maximum diameter of 13, 13 and 15 mm; there were 3 lesions in tongue, all of which were located on the dorsum of tongue, with the maximum diameter of 13, 18 and 23 mm; there were also 2 cases located in nasopharynx, maximum diameter of 15 and 22 mm, respectively. One case was in sacrococcygeal area, with the maximum diameter as 18 mm. All lesions presented as solid masses with well-defined margins, displaying slightly low density compared to grey matter. The CT value ranged from 25 to 47 HU. Compared to grey matter or spinal cord, MRI demonstrated isointense or slight hypointense on T 1WI and slight hyperintense on T 2WI. All masses presented homogenous density or signal intensity, with mild homogenous enhancement. During postoperative follow-up, no recurrence was found in 8 cases. One case of nasal NGH with gradeⅡcleft lip recurred at 1 month follow-up after surgery, and no recurrence was found after the second surgery. Conclusions:The NGH in children has typical imaging features, and is mostly located at the extracranial midline structure.It presents as solid mass, with quasi-circular morphology, well-defined margins and homogeneous density or signal intensity similar to gray matter or spinal cord. The postoperative recurrence rate is low.
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Objective:To investigate the clinical features and prognosis of acute necrotizing encephalopathy (ANE) in children.Methods:The clinical data and follow-up information of 41 pediatric patients with ANE treated in Wuhan Children′s Hospital, Tongji Medical College, Huazhong University of Science & Technology from January 2014 to September 2019 were retrospectively reviewed.Results:The 41 patients included 23 males and 18 females with the onset age of (4.4±3.2) years.The main prodromal symptoms were gastrointestinal (20/41 cases, 48.8%) and respiratory infections (19/41 cases, 46.3%). Acute encephalopathy progressed rapidly following the prodromal infection [29 cases (70.7%) ≤2 days], and patients had clinical manifestations of coma (32/41 cases, 78.0%), convulsion (32/41 cases, 78.0%), multiple organ dysfunction (27/41 cases, 65.9%), shock and disseminated intravascular coagulation were rarely occured, and 28 cases (68.3%) were admitted to intensive care unit for treatment.Brain magnetic resonance imaging (MRI) showed lesion involving thalamus (41/41 cases, 100.0%), periventricular white matter (34/41 cases, 82.9%), brainstem (31/41 cases, 75.6%), basal ganglia (26/41 cases, 63.4%), cerebral cortex and subcortex (20/41 cases, 48.8%) and cerebellum (18/41 cases, 43.9%). The common presentations on the apparent diffusion coefficient mapping of brain MRI were " tricolor pattern" or " bicolor pattern" of the thalamus.During follow-up (≥ 6 months), MRI showed that hemorrhage, cystic degeneration and atrophy changed dynamically with the progression of ANE.All cases were treated with glucocorticoids, 38 cases(92.7%) with intravenous immune globulin.Seven cases (17.1%) were died and the 34 survivors had different degrees of neurological dysfunction.Conclusions:ANE in children is a distinctive type of clinicoradiologic syndrome with rapid progression and various presentations.Brain MRI has typical imaging characteristics and dynamically indicates the progression of this disease.The treatment options are still limited, the prognosis is poor and the survivors are often with neurological dysfunction.
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Objective:To analyze the imaging features of acute necrotizing encephalopathy of childhood (ANEC), and try to investigate its potential clinical value.Methods:The clinical and imaging findings of 22 children from Wuhan Children′s Hospital diagnosed with ANEC were retrospective analyzed, from January 2013 to October 2018. All children were presented with hyperpyrexia and rapidly developed into rapid neurological deterioration after prodromic infection. In the initial imaging examination, all patients underwent head MRI, and 6 cases underwent additional head CT. During MRI follow-up, 4 cases were lost, 6 cases were followed up only once (<14 days), and 12 cases were followed up 1 to 2 times at short-term and 1 to 4 times at long-term (>14 days).The presence of hemorrhage and encephalomalacia in thalamus, brainstem, white matter and basal ganglia was carefully investigated throughout the follow-up.Results:For the imaging manifestations of ANEC, bilateral thalamus were involved in all children. Other symmetrical lesions included white matter (14 cases), basal ganglia (15 cases), brainstem (16 cases), cerebellum (9 cases), corpus callosum (2 cases) and hippocampus (1 case). There were 3 children with asymmetric lesions, which were found in white matter (2 cases) and cerebellum (1 case).In the acute phase, the most typical head MRI showed "tricolor pattern"(high signal intensity in the center with surrounding low-signal, and hyperintense signals in the periphery of thalamus) or "bicolor pattern"(low signal in the central thalamus with surrounding hyperintense signals) of the thalamus on the apparent diffusion coefficient (ADC) imaging. Hemorrhage and encephalomalacia on MRI may suggest poor clinical outcome.Conclusions:ANEC is a rapid progressive encephalopathy with typical imaging features. Hemorrhage and encephalomalacia on MRI may be associated with poor prognosis.