MR Imaging Scoring System for White Matter Injury after Deep Medullary Vein Thrombosis and Infarction in Neonates.

BACKGROUND AND PURPOSE: Advanced imaging techniques have allowed earlier and more accurate detection of cerebral deep medullary vein thrombosis and infarction. Our objective was to develop an MR imaging scoring system to evaluate the severity of white matter injury in neonates with deep medullary vein thrombosis and infarction. MATERIALS AND METHODS: This was a retrospective study of infants born ≥32 weeks' gestation (2000-2016) diagnosed with deep medullary vein thrombosis and infarction on neuroimaging in the first 30 days of life. A 102-point deep medullary vein white matter injury global severity score was developed. MR images were scored by 2 pediatric radiologists. Subject clinical data and regional and global severity scores were recorded. RESULTS: Fifty-one patients (mean gestational age, 37.3 ± 2.2 weeks; mean birth weight, 3182 ± 720 g) were included with a mean age at diagnosis via MR imaging of postnatal day 10.1 ± 6.1. Global severity scores ranged from 1 to 53, with a median score of 11 (interquartile range, 5-25). Lesions were more common in the frontal and parietal regions and less common in the occipital and temporal regions. Fifty-five percent of the group had neonatal seizures. No difference in perinatal risk factors (gestational age, birthweight, 5-minute Apgar score, chorioamnionitis, delivery room resuscitation, ventilator, or inotrope requirement) was observed among severity score quartiles. CONCLUSIONS: An MR imaging scoring system provides a comprehensive and objective classification of WM injury after deep medullary vein thrombosis and infarction in late preterm and term neonates. The global severity score is independent of gestational age and other antenatal risk factors, consistent with presentation in previously healthy-appearing neonates.

Maternal reproductive history and the risk of isolated congenital malformations.

We examined the relationship between maternal reproductive history and the newborn's risk of isolated congenital malformations in a large case-control cohort from the Polish Registry of Congenital Malformations. Congenital malformations were classified into four categories: isolated congenital heart defects (n=1673), isolated cleft palate (n=255), cleft lip with or without cleft palate (n=448) and renal agenesis (n=103). The case groups were compared with a shared group of 2068 controls recruited in the same time period and geographic area. Multivariable logistic regression was used to assess the risk associated with maternal gravidity and of previous miscarriages after accounting for maternal age and other potential risk factors. In unadjusted analyses, maternal gravidity was significantly associated with increased risk of all four classes of congenital malformations. After adjustment, a significant association persisted for congenital heart defects [odds ratio (OR)=1.22, [95% confidence interval (CI) 1.09, 1.36], P=0.0007] and cleft lip with or without cleft palate (OR=1.21, [95% CI 1.09, 1.36], P=0.0005). A similar trend existed for isolated cleft palate (OR=1.18, [95% CI 1.02, 1.37], P=0.03). There was no appreciable increase in the risk of congenital malformations associated with a maternal history of miscarriages, but a trend for a protective effect on the occurrence of cleft lip with or without cleft palate was observed (OR=0.72, [95% CI 0.52, 0.99], P=0.045). Based on our data, maternal gravidity represents a significant risk factor for congenital heart defects and cleft lip with or without cleft palate in the newborn infant. Our data do not support an increase in risk because of past history of miscarriages.

Venous thromboembolism in trauma patients.

Serial venous duplex scans (VDS) were done in 507 trauma patients with at least one risk factor (RF) for venous thromboembolism (VTE) during a 2-year study period. Deep vein thrombosis (DVT) was detected in 31 (6.1%) patients. This incidence was 3.1 per cent in low (1-2 RFs), 3.4 per cent in moderate (3-5 RFs), and 7.7 per cent in high (> or =6 RFs) VTE scores (P = 0.172). Incidence was statistically different (3% vs. 7.2%, P = 0.048) on reanalyzing patients in two risk categories, low-risk (1-4 RFs) and high-risk (> or =5 RFs). Only 4 of 16 RFs had statistically higher incidence of DVT in patients with or without RFs: previous VTE (27.3% vs. 5.6%, odds ratio (OR) 6.628, P = 0.024), spinal cord injury (22.6% vs. 5%, OR 5.493, P = 0.001), pelvic fractures (11.4% vs. 5.1%, OR 2.373, P = 0.042), and head injury with a greater than two Abbreviated Injury Score (10.5% vs. 4.2%, OR 2.639, P = 0.014). On reanalyzing patients with > or =5 RFs vs. <5RFs, obesity (14.3 vs. 6.1%, P = 0.007), malignancy (5.6% vs. 0.6%, P = 0.006), coagulopathy (10.8% vs. 1.8%, P = 0.000), and previous VTE (3.2% vs. 0%, P = 0.019) were significant on univariate analysis. Patients with DVT had 3.70 +/- 1.75 RFs and a 9.61 +/- 4.93 VTE score, whereas, patients without DVT had 2.66 +/- 1.50 RFs and a 6.83 +/- 3.91 VTE score (P = 0.000). DVTs had a direct positive relationship with higher VTE scores, length of stay, and number of VDS (>1 r, P < or = 0.001). Increasing age was a weak risk factor (0.03 r, P = 0.5). First two VDS diagnosed 77 per cent of DVTs. Patients with injury severity score of > or =15 and 25 had higher DVTs compared with the ones with lower injury severity score levels (P < or = 0.05). Pulmonary embolism was silent in 63 per cent and DVTs were asymptomatic in 68 per cent.

Impact of apomorphine on BOLD signal during movement in normals.

Our group investigated modulatory effects of apomorphine on cerebral activation patterns during finger tapping movements in six healthy right-handed volunteers using an established fMRI protocol. Apomorphine application disclosed a reduction of cerebral activation to the contralateral precentral and postcentral gyrus and ipsilateral cerebellum, with a prominent net reduction of BOLD signal in cerebellar areas. These findings contradict those of similar studies performed on dopaminergic function and Parkinson's disease (PD), which predominantly found augmentation of cerebral activation patterns in normal volunteers and PD patients after dopaminergic stimulation. One conceivable explanation for our singular results would be preferred binding of apomorphine to presynaptic dopaminergic receptors, leading to inhibition of endogenous dopamine release and resultant diminished dopaminergic stimulation, reflected in diminished cerebral activation patterns. These findings warrant future consideration and further investigation of possible central inhibitory effects of dopaminergic therapy in functional imaging studies of the dopaminergic system in general and PD in particular.

Mitochondrial respiratory-chain defects presenting as nonspecific features in children.

Patients with mitochondrial respiratory-chain defects frequently exhibit lactic acidosis, ragged red fibers in skeletal muscle samples, and abnormal enzyme assays for the respiratory-chain complex. However, ragged red fibers and lactic acidosis are not always seen in all patients with mitochondrial respiratory-chain defects. We have encountered six children with biochemically proven respiratory chain defects, but typical ragged red fibers were not found in all six patients, and only five patients had increased serum lactate levels. Initially, they present with nonspecific features. However, persistent or progressive clinical features or multiple organ involvement eventually led to the diagnosis of respiratory-chain defects in these patients. Mitochondrial respiratory-chain defects should be considered in the differential diagnosis when persistent, progressive features and especially multiple organ involvement occur.

Importance of hypoxic/ischemic conditions in the development of cerebral lenticulostriate vasculopathy.

BACKGROUND: We have observed many infants with lenticulostriate vasculopathy (LSV) on neurosonograms who do not have classic histories of prenatal infection, trisomy, or prenatal drug exposure. OBJECTIVE: To investigate the underlying clinical conditions in patients with LSV. MATERIALS AND METHODS: Sixty-three cases of LSV were identified among approximately 2,400 neurosonograms performed over 42 months. All neurosonograms were reviewed. Medical records were reviewed for information regarding prenatal infection, chromosomal abnormality, respiratory and cardiac disease, and other pertinent diagnoses. RESULT: Hypoxic/ischemic conditions accounted for 33 cases: cardiac disease in 13, respiratory distress syndrome in 15, and perinatal asphyxia in 5. Twelve of these 33 had initially normal or minimal findings, with LSV developing or progressing on subsequent neurosonograms. The remaining 30 cases had varied clinical associations: congenital infection in 5, trisomies in 9, fetal substance exposure in 4, and unclear etiology in 12. Coexistent anomalies included posterior urethral valves, MCDK, myelomeningocele, placental abruption, and others. Thirty patients were premature. Fifteen patients died; histologic changes of LSV were found at autopsy in one patient. CONCLUSION: LSV has varied clinical associations. The common association with hypoxic/ischemic conditions and the progressive changes seen in 12 patients with cardiac and pulmonary disease suggest that postnatal hypoxia/ischemia is an important etiologic factor.

Postconcussive symptoms in children with mild closed head injuries.

OBJECTIVE: To examine the incidence and neuropsychological, behavioral, and neuroimaging correlates of postconcussive symptoms (PCS) in children with mild closed head injuries (CHI). DESIGN: 26 Children with mild CHI and 8 of their uninjured siblings, from 8 to 15 years old, were recruited prospectively and assessed at baseline (ie, within 7 days of injury) and at 3 months postinjury. Parents rated PCS, motivation and affective lability, and behavioral adjustment. Baseline ratings assessed premorbid functioning retrospectively, and follow-up ratings assessed postinjury status. On both occasions, children completed neuropsychological testing, and those with mild CHI also underwent magnetic resonance imaging (MRI). RESULTS: Children with mild CHI did not differ from siblings in baseline ratings of premorbid PCS but displayed higher ratings on several PCS at 3 months postinjury. Thirty-five percent of children with mild CHI showed increases in PCS, compared with baseline premorbid ratings, but none of the siblings did so. Children with mild CHI whose PCS increased from premorbid levels showed poorer neuropsychological functioning at baseline than did children whose PCS did not increase, although the differences had partially resolved by 3 months. They also displayed decreased motivation over time. Their behavioral adjustment was poorer and they had smaller white matter volumes on MRI, but the latter differences were present at baseline and did not change over time, suggesting that they existed prior to the injury. CONCLUSION: Postinjury increases in PCS occur in a sizable minority of children with mild CHI and more often than among uninjured siblings. Increases in PCS following mild CHI are associated with premorbid neurological and psychosocial vulnerability, but also with postinjury decrements in neuropsychological and neurobehavioral functioning.

Emergency brain computed tomography in children with seizures: who is most likely to benefit?

OBJECTIVE: To determine whether the recently published guidelines on neuroimaging in patients with new-onset seizures are applicable to children. METHODS: We carried out a retrospective analysis of 107 neurologically normal children (excluding children with simple febrile seizures) who had undergone neuroimaging when they presented to the emergency department with a possible "first seizure." RESULTS: Eight of the 107 children had nonepileptic events (gastroesophageal reflux, syncopal event, rigor). Of the remaining 99 children, 49 had provoked seizures (complicated febrile seizure, meningo-encephalitis, toxic or metabolic abnormalities), and 50 had unprovoked seizures. A total of 19 children had brain abnormalities identified on computed tomography (CT) scan; 7 received further investigation or intervention as a result of CT scan findings (2 with tumors, 3 with vascular anomalies, 1 with cysticercosis, and 1 with obstructive hydrocephalus). CT scan abnormalities requiring treatment or monitoring were more frequently seen in children with their first unprovoked seizure (P < .01) and in those children whose seizure onset had been focal or who had focal abnormalities identified on postictal neurologic examination (P < .04). CONCLUSION: In a child, a seizure in the setting of a fever rarely indicates the presence of an unexpected CT scan lesion requiring intervention.

Lytic skull metastasis from a follicular thyroid carcinoma in a child.

We present a case of solitary lytic skull metastasis from a follicular thyroid carcinoma in a child. Salient clinical features, radiological findings, and histological distinctions of the metastatic skull tumor are described. Excision of the skull tumor followed by total thyroidectomy and (131)I-radioablation therapy has resulted in good short-term outcome. This is the first published report of a metastatic skull lesion from a follicular thyroid carcinoma in a child. Such a malignant lesion can radiologically mimic a benign skull tumor and should be considered in the differential diagnosis of a solitary lytic skull lesion with a sclerotic margin.

Congenital muscular dystrophy with complete laminin-alpha2-deficiency, cortical dysplasia, and cerebral white-matter changes in children.

Congenital muscular dystrophy consists of Fukuyama congenital muscular dystrophy, Walker-Warburg syndrome, muscle-eye-brain disease, and occidental congenital muscular dystrophy, which is further divided into laminin-alpha2-positive and laminin-alpha2-negative subgroups. These forms of congenital muscular dystrophy are frequently associated with abnormal white-matter changes, whereas the Fukuyama form, Walker-Warburg syndrome, and muscle-eye-brain disease are also frequently found to have polymicrogyria. We now report two infants with complete laminin-alpha2-deficiency who have not only abnormal cerebral white-matter lesions, but also bioccipital polymicrogyria. There are significant similarities in the clinical and cerebral manifestations among the various types of congenital muscular dystrophy. The diagnosis of the Fukuyama form, laminin-alpha2-deficiency, Walker-Warburg syndrome, and muscle-eye-brain disease cannot always be established on radiological studies alone.

Leigh syndrome, cytochrome C oxidase deficiency and hypsarrhythmia with infantile spasms.

A rare patient with infantile spasms, hypsarrhythmia, cytochrome c oxidase deficiency and Leigh syndrome is reported. Although rare, infantile spasms and Leigh syndrome may occur simultaneously. Leigh syndrome should be included in the differential diagnosis of infantile spasms.

Cerebellar infarction from a traumatic vertebral artery dissection in a child.

Infarction due to vertebral dissection is a rarely reported event in children. We describe the clinical presentation, radiological findings and surgical treatment of a child with cerebellar infarction resulting from a traumatic vertebral artery dissection. Review of the literature on stroke due to a vertebral artery dissection in the pediatric population shows that trauma is a common preceding event. Although the most common site of traumatic vertebral artery dissection is at C1-2 level, our case illustrates that the vertebral artery dissection may also involve the lower cervical segment. We emphasize that vertebral artery dissection should be considered in a child with acute symptoms and signs of posterior circulation ischemia and that MRI and MR angiography may be helpful in the diagnosis of infarction and vertebral artery dissection.

Radiological and clinical criteria for the management of epidural hematomas in children.

OBJECTIVE: Modern neuroimaging and intensive care permit precise delineation and specific treatment of head injury. Children sustaining cranial trauma associated with epidural hematoma (EDH) represent a heterogeneous group with a variety of clinical outcomes. Treatment consists of simple observation or surgical evacuation. We attempted to define radiological characteristics of the EDH patients that underwent surgical evacuation. METHODS: We reviewed the records and computed tomography scans of 33 children sustaining cranial trauma associated with EDH treated at the Children's National Medical Center between October 1990 and August 1994. The radiological and clinical characteristics of children treated surgically (n = 13) and nonsurgically (n = 20) were compared. RESULTS: Mass effect, a temporal clot location, thickness, length and volume of the clot, and midline shift (p < 0.05) differed significantly between groups. The most important radiological parameters in determining the therapeutic intervention were thickness, midline shift, mass effect, and EDH location. A thickness of the EDH > 18 mm, a midline shift >4 mm, and moderate or severe mass effect correctly predicted therapy in 29 out of 33 patients. By adding the location as a fourth parameter, therapy was accuratly predicted in 31 of 33 patients. Mechanism of injury, interval from injury to initial computed tomography scan, age, sex, Glasgow coma score on admission, or lengths of hospital and intensive care unit stays were not significantly different between groups. CONCLUSION: Although radiological criteria predict surgical intervention for larger EDH, patients harboring intermediate-size EDH will continue to require careful individualized clinical judgement.

Tumoral multiple sclerosis of the cerebellum in a child.

We present a case of cerebellar tumoral multiple sclerosis in an 11-year-old girl and emphasize these two features: (1) Tumoral multiple sclerosis can occur within the posterior fossa and should be strongly considered in the differential diagnosis of mass lesions of the posterior fossa when typical white matter lesions are seen on T2-weighted images. (2) Tumoral multiple sclerosis can demonstrate ring enhancement on MR.

Percutaneous computed tomography-guided stabilization of posterior pelvic fractures.

Nineteen patients with 21 unstable fractures of the posterior pelvic complex were treated with percutaneous computed tomography (CT)-guided posterior stabilization. CT allowed satisfactory visualization of the fracture and direction and length of the screw. It also allowed the authors to perform the procedure percutaneously, while causing only minimal blood loss. CT cuts that showed the best and safest sites for screw placement were selected. The length and direction of the screw was identified, and the skin entry site was determined. The guide pin was inserted across the fracture or disrupted sacroiliac joint; its correct position was confirmed and a cannulated screw was inserted over the pin. In the majority of patients, a pelvic external fixator was applied in the CT suite. Although assessing the deformity was possible, reduction was difficult but ultimately acceptable. All patients maintained reduction of their sacroiliac complex without loss of fixation, and all fractures healed without clinical or radiographic signs of pelvic instability. This technique was not associated with neurologic injury or deep infection. All but 2 patients returned to preinjury levels of activity and function. Two patients had associated injuries that affected their final functional outcome.