Congenital ependymoblastoma arising in the sacrococcygeal soft tissue: a case study.

Ependymoblastomas are distinct embryonal tumors of the central nervous system reported only rarely in the literature. Most examples arise in young children under the age of 2 years, in the supratentorial compartment, and may or may not be related to the ventricular system. We report the case of a one-day-old infant who presented with a 6.4 x 5.6 x 3.5 cm ruptured buttock mass. Ultrasound demonstrated a solid mass at the base of the spine that displaced the bladder anteriorly with resultant hydronephrosis. Magnetic resonance images confirmed the presence of a solid mass surrounding the lower sacrum with an internal component partially encircling and deviating the rectum. Histopathological evaluation confirmed the diagnosis of ependymoblastoma. Of note, immunohistochemical analysis revealed diffuse staining with vimentin and patchy expression of synaptophysin, glial fibrillary acidic protein, neurofilament proteins, neuron-specific enolase, CD99 and nestin. On the 42nd day of life, chemotherapy was initiated with a modified Children's Oncology Group (COG) AGCT-01P1 (cyclophosphamide, cisplatin, 70% etoposide, no bleomycin) regimen. The authors describe their experience and review the literature, emphasizing that ependymoblastomas should be considered in the differential diagnosis of sacral masses in the newborn.

In vivo assessment of placental and brain volumes in growth-restricted fetuses with and without fetal Doppler changes using quantitative 3D MRI.

OBJECTIVE: The relationship between placental and fetal brain growth is poorly understood and difficult to assess. The objective of this study was to interrogate placental and fetal brain growth in healthy pregnancies and those complicated by fetal growth restriction (FGR). STUDY DESIGN: In a prospective, observational study, pregnant women with normal pregnancies or pregnancies complicated by FGR underwent fetal magnetic resonance imaging (MRI). Placental, global and regional brain volumes were calculated. RESULTS: A total of 114 women (79 controls and 35 FGR) underwent MRI (median gestational age (GA) 30 weeks, range 18 to 39). All measured volumes increased exponentially with advancing GA. Placental, total brain, cerebral and cerebellar volumes were smaller in FGR compared with controls (P<0.05). Increasing placental volume was associated with increasing cerebral and cerebellar volumes (P<0.05). CONCLUSION: Quantitative fetal MRI can accurately detect decreased placental and brain volumes in pregnancies with FGR and may provide insight into the timing and mechanisms of brain injury in FGR.

Patterns of Brain Injury in Newborns Treated with Extracorporeal Membrane Oxygenation.

BACKGROUND AND PURPOSE: Neonates treated with extracorporeal membrane oxygenation are at risk for brain injury and subsequent neurodevelopmental compromise. Advances in MR imaging and improved accessibility have led to the increased use of routine MR imaging after extracorporeal membrane oxygenation. Our objective was to describe the frequency and patterns of extracorporeal membrane oxygenation-related brain injury based on MR imaging findings in a large contemporary cohort of neonates treated with extracorporeal membrane oxygenation. MATERIALS AND METHODS: This was a retrospective study of neonatal patients treated with extracorporeal membrane oxygenation from 2005-2015 who underwent MR imaging before discharge. MR imaging and ultrasound studies were reviewed for location and type of parenchymal injury, ventricular abnormalities, and increased subarachnoid spaces. Parenchymal injury frequencies between patients treated with venoarterial and venovenous extracorporeal membrane oxygenation were compared by χ2 tests. RESULTS: Of 81 neonates studied, 46% demonstrated parenchymal injury; 6% showed infarction, mostly in vascular territories (5% anterior cerebral artery, 5% MCA, 1% posterior cerebral artery); and 20% had hemorrhagic lesions. The highest frequency of injury occurred in the frontal (right, 24%; left, 25%) and temporoparietal (right, 14%; left, 19%) white matter. Sonography had low sensitivity for these lesions. Other MR imaging findings included volume loss (35%), increased subarachnoid spaces (44%), and ventriculomegaly (17% mild, 5% moderate, 1% severe). There were more parenchymal injuries in neonates treated with venoarterial (49%) versus venovenous extracorporeal membrane oxygenation (29%, P = .13), but the pattern of injury was consistent between both modes. CONCLUSIONS: MR imaging identifies brain injury in nearly half of neonates after treatment with extracorporeal membrane oxygenation. The frontal and temporoparietal white matter are most commonly affected, without statistically significant laterality. This pattern of injury is similar between venovenous and venoarterial extracorporeal membrane oxygenation, though the frequency of injury may be higher after venoarterial extracorporeal membrane oxygenation.

Is a chest radiograph necessary in the evaluation of every febrile infant less than 8 weeks of age?

This study was designed to examine the relationship between respiratory signs and the likelihood of having an abnormal chest radiograph in a sample of febrile infants less than 8 weeks of age. The sample consisted of 242 infants who were admitted during a 3-year period with temperatures greater than or equal to 38 degrees C (100.4 degrees F) and had a chest radiograph. The house officer recorded the presence of respiratory signs and symptoms including rhinorrhea, tachypnea, cough, rales, wheezes, retractions, and rhonchi. Each chest radiograph was reviewed independently according to predetermined criteria by a senior radiology resident and an attending pediatric radiologist. Interobserver agreement was 91%. Both observers were blind to the infants' respiratory signs. The chest radiograph interpretations were compared with the presence of respiratory signs. Of the 242 cases, 228 had chest radiographs available for interpretation. Of these, 27 chest radiographs (12%) were identified as abnormal, including 6 where there was initial disagreement as to the presence of an abnormality. Twenty-five (31%) of 80 infants with any respiratory signs had an abnormal chest radiograph, whereas only 2 (1%) of 148 asymptomatic infants did. The sensitivity of respiratory signs was 93% (confidence interval = 76% to 99%). These findings suggest that in the absence of respiratory signs, febrile infants are unlikely to have an abnormal chest radiograph.

Routine chest radiographs in pediatric intensive care: a prospective study.

The clinical value of routine chest radiographs was prospectively evaluated in a pediatric intensive care unit. Physicians were asked to predict findings of clinical impact in 353 routine morning chest radiographs performed in 101 patients after examining the patients. In 81 instances (23%), the clinical impact of the chest radiographs was incorrectly predicted and significant alterations in management would have potentially been missed had the chest radiographs not been available. These 81 chest radiographs included 72 unpredicted radiographic changes of clinical significance, and nine chest radiographs in which a significant radiographic change was incorrectly predicted. Thirty five (43.2%) of these 81 chest radiographs had unpredicted pulmonary findings and 46 (56.8%) showed unpredicted appliance malpositions. Incorrect predictions were significantly associated with radiographs from patients who were younger, intubated, mechanically ventilated, and had indwelling central venous catheters. Level of training of the predicting physicians did not affect prediction accuracy. In analysis of 43 routine postintubation chest radiographs and 39 routine postcentral venous catheter placement chest radiographs, appliance malpositions were disclosed in 34.9% and 43.6%, respectively. Routine daily and post-appliance placement chest radiographs have significant clinical value in the pediatric intensive care unit.

Patient with craniosynostosis and marfanoid phenotype (Shprintzen-Goldberg syndrome) and cloverleaf skull.

Marfanoid phenotype with craniosynostosis (Shprintzen-Goldberg syndrome) is a rare disorder previously described in only 5 patients. We report on the sixth known patient with this condition. The findings which distinguish our patient from others reported previously are that she was ascertained prenatally as having a cloverleaf skull; this is the first female patient described with this condition. Postnatally, she presented with arachnodactyly, camptodactyly, and clover-leaf skull. Imaging studies of the brain documented microcephaly with malformed brain, hydrocephaly, and hypoplasia of the corpus callosum. She also had choanal atresia and stenosis, a clinical finding previously reported only once, in this disorder.

Intracranial abnormalities in infants treated with extracorporeal membrane oxygenation: update on sonographic and CT findings.

PURPOSE: To determine the frequency of intracranial lesions in infants treated with extracorporeal membrane oxygenation (ECMO), to evaluate trends in frequency during an 8-year period, and to determine which infants are at highest risk for intracranial injury. METHODS: Daily sonograms were obtained in 386 infants during treatment with ECMO. Cranial CT scans were acquired after decannulation in 286 of 322 survivors. Abnormalities were classified as major or minor and hemorrhagic or nonhemorrhagic. Results were correlated with infant demographic data. RESULTS: Intracranial abnormalities were detected in 203 (52%) of the 386 infants; 73 (19%) hemorrhagic, 86 (22%) nonhemorrhagic, and 44 (11%) combined lesions. Eighty-two lesions (21%) were classified as major. Forty-six (94%) of 49 major hemorrhages were identified at sonography. CT contributed additional information in 73% of neonates with intracranial abnormalities, of which 17 were major lesions not identified at sonography. The frequency of intracranial hemorrhage was increased in infants who were septic or premature or weighed less than 2.5 kg. An increase in time spent on ECMO bypass increased the risk for nonhemorrhagic injury. During an 8-year period, the frequency of hemorrhagic and major nonhemorrhagic lesions remained constant, whereas minor nonhemorrhagic abnormalities increased significantly. CONCLUSION: Infants treated with ECMO continue to be at high risk for cerebrovascular injury. Although daily sonograms are useful in identifying major hemorrhages, follow-up CT scans are crucial for accurate evaluation of intracranial abnormalities.

Preterm anoxic injury. Radiologic evaluation.

Diagnostic imaging plays an important role in evaluating the preterm infant with hypoxic-ischemic injury. The pathologic and radiographic findings of IVH, focal white matter necrosis, and severe anoxic damage are well documented. New observations, including diffuse white matter and cerebellar insults in some survivors, are being made. Understanding the complex relationships between these findings, clinical events (both prenatal and postnatal), and neurocognitive outcome of the preterm infants, however, requires further study.

Perinatal and postnatal chest sonography.

Sonography is the primary method used to image the fetal chest. Many significant congenital anomalies such as pleural effusion, congenital diaphragmatic hernia, cystic adenomatoid malformation, pulmonary sequestration, and congenital heart disease can be detected during early prenatal sonography. Fetal sonography also permits accurate assessment of the severity of these processes, allowing for parental counseling and optimal planning of postnatal care. After birth, sonography is the primary method for evaluating cardiac anatomy and diagnosing congenital heart disease. Sonography also serves as a useful adjunct to plain film radiology and other modalities in evaluation of the mediastinum, diaphragm, pleura, and chest wall.

Acute abdomen. Laboratory evaluation and imaging.

The child with an acute abdomen requires a thorough history and physical examination followed by a focused laboratory and imaging evaluation. The laboratory evaluation is more beneficial in determining management than in establishing diagnosis. Ultrasonography has become increasingly useful in the evaluation of the child with acute abdominal pain.

Prenatal evaluation of fetal anomalies.

Advances in sonographic imaging have allowed for detailed examination of the fetus. A wide range of abnormalities of the fetal central nervous system, chest, gastrointestinal tract, genitourinary system, and skeleton are detectable by prenatal sonography. This article reviews the sonographic findings and prognostic implications of these abnormalities.

Pattern of neuropsychological deficit at age five years following neonatal unilateral brain injury.

The pattern of language deficit following left-hemisphere brain injury and visual/spatial deficit following right-hemisphere injury in an adult or older child is well recognized, but has been inconsistently reported following presumed neonatal brain injury. Our prospective study of 24 children at age 5 with documented neonatal unilateral brain injury lends support to the theory of hemisphere specialization at the time of birth. Twelve children who had unilateral left-hemisphere lesion were compared to 12 children with unilateral right-hemisphere lesion of similar timing and severity. Relative visual/spatial deficit following right-hemisphere lesion and receptive language deficit following left-hemisphere lesion were identified. Lateralized measures of grip strength, fine motor speed, and fine motor dexterity were not significantly different between the groups for either hand in this nonhemiparetic study sample. Only one child with a left-hemisphere lesion was left-handed, and only one child (right-lesion) had a hemiparesis.

A strategy for resection of Wilms' tumor with vena cava or atrial extension.

Resection of a Wilms' tumor that extends into the vena cava or right atrium results in excellent survival when combined with adjuvant therapy. Preoperative identification of the presence of intravascular tumor thrombus and the level of vascular involvement is essential. It facilitates safe surgical resection, with cardiopulmonary bypass immediately available for retrohepatic and atrial tumors. Six patients with intracaval or intracardiac tumor thrombus were treated over a 5-year period with no perioperative deaths. Preoperative chemotherapy was useful in two patients with extensive tumors and pulmonary metastases. Our results using an integrated management plan suggest that an aggressive surgical approach is justified for this extensive variant of Wilms' tumor.

Surfactant (beractant) therapy for infants with congenital diaphragmatic hernia on ECMO: evidence of persistent surfactant deficiency.

Infants with congenital diaphragmatic hernia (CDH) on extracorporeal membrane oxygenation (ECMO) can have initial lung atelectasis which, in survivors, gradually improves over time. To test the hypothesis that these patients could benefit from surfactant therapy, infants with CDH (born at > 34 weeks' gestation) on ECMO received either four doses of modified bovine lung surfactant extract (beractant) (surfactant group, n = 9) or an equal volume of air (control group, n = 8). Tracheal aspirate surfactant protein-A (SP-A) concentrations were initially low, and then increased over time in both CDH groups (P = .0021); however, levels remained low when compared with those of infants on ECMO who had other diagnoses (P = .04). Lung compliance (CL), time to extubation, time on oxygen, and total no. of hospital days were not different between the two groups. Infants with CDH had persistently elevated right ventricular pressure (RVP) at cessation of bypass when compared with non-CDH infants on ECMO (RVP = 53.25 mm Hg +/- 19.52 in the CDH group, 32.90 +/- 10.63 in the non-CDH group; P = .0121). The findings suggest that the postnatal surfactant deficiency may be more persistent in CDH infants than in non-CDH infants on ECMO. However, CDH remains a multifactorial condition, with delayed improvement, because of persistence of pulmonary hypertension, difficulties with vascular remodeling, degree of lung hypoplasia, or compromised respiratory mechanics.

Splenic injuries in children after blunt abdominal trauma.

Management of splenic injuries in children has evolved over the past two decades. Splenectomies or splenorrhaphies are now performed infrequently, with the majority of hemodynamically stable children with splenic injuries managed nonoperatively. This article reviews the imaging features of acute splenic injuries in children as well as the appearance of healing splenic injuries. Follow-up evaluation and outcomes in children with splenic injuries also are addressed.

Ectrodactyly, diaphragmatic hernia, congenital heart defect, and agenesis of the corpus callosum.

A unique case of a female born with four major malformations, ectrodactyly, diaphragmatic hernia, ventricular septal defect, and agenesis of the corpus callosum is reported. The patient had a normal birth weight, normal head circumference and a normal karyotype. There was no significant facial dysmorphism. The family history was unremarkable for birth defects, recurrent pregnancy loss, limb anomalies or consanguinity. We propose that this represents a new constellation of multiple malformations.

Prevalence and spectrum of in utero structural brain abnormalities in fetuses with complex congenital heart disease.

BACKGROUND AND PURPOSE: Brain injury is a major complication in neonates with complex congenital heart disease. Preliminary evidence suggests that fetuses with congenital heart disease are at greater risk for brain abnormalities. However, the nature and frequency of these brain abnormalities detected by conventional fetal MR imaging has not been examined prospectively. Our primary objective was to determine the prevalence and spectrum of brain abnormalities detected on conventional clinical MR imaging in fetuses with complex congenital heart disease and, second, to compare the congenital heart disease cohort with a control group of fetuses from healthy pregnancies. MATERIALS AND METHODS: We prospectively recruited pregnant women with a confirmed fetal congenital heart disease diagnosis and healthy volunteers with normal fetal echocardiogram findings who underwent a fetal MR imaging between 18 and 39 weeks gestational age. RESULTS: A total of 338 fetuses (194 controls; 144 with congenital heart disease) were studied at a mean gestational age of 30.61 ± 4.67 weeks. Brain abnormalities were present in 23% of the congenital heart disease group compared with 1.5% in the control group (P < .001). The most common abnormalities in the congenital heart disease group were mild unilateral ventriculomegaly in 12/33 (36.4%) and increased extra-axial spaces in 10/33 (30.3%). Subgroup analyses comparing the type and frequency of brain abnormalities based on cardiac physiology did not reveal significant associations, suggesting that the brain abnormalities were not limited to those with the most severe congenital heart disease. CONCLUSIONS: This is the first large prospective study reporting conventional MR imaging findings in fetuses with congenital heart disease. Our results suggest that brain abnormalities are prevalent but relatively mild antenatally in fetuses with congenital heart disease. The long-term predictive value of these findings awaits further study.

Management of high grade renal trauma: 20-year experience at a pediatric level I trauma center.

PURPOSE: In the last 20 years the management of high grade, blunt renal trauma at our institution has evolved from primarily an operative approach to an expectant nonoperative approach. To evaluate our experience with the expectant nonoperative management of high grade, blunt renal trauma in children, we reviewed our 20-year experience regarding evaluation, management and outcomes in patients treated at our institution. MATERIALS AND METHODS: We retrospectively studied all patients sustaining renal trauma between 1983 and 2003. Medical records were reviewed for mechanism of injury, assigned grade of renal injury, patient treatment, indications for and timing of surgery, and outcome. Injuries were categorized as either low grade (I to III) or high grade (IV to V). RESULTS: We reviewed the medical records of 164 consecutive children who sustained blunt renal trauma between 1983 and 2003. A total of 38 patients were excluded for inadequate information. Of the remaining 126 children 60% had low grade and 40% had high grade renal injuries. A total of 11 patients (8.7%) required surgical or endoscopic intervention for renal causes, including 2 for congenital renal abnormalities and 1 for clot retention. Eight patients (6.3%) required surgical intervention for isolated renal trauma, of whom 2 (1.6%) required immediate surgical intervention for hemodynamic instability and 6 (4.8%) were treated with a delayed retroperitoneal approach. Only 4 patients (3.2%) required nephrectomy. All patients receiving operative intervention had high grade renal injury. CONCLUSIONS: Initial nonsurgical management of high grade blunt renal trauma in children is effective and is recommended for the hemodynamically stable child. When a child has persistent symptomatic urinary extravasation delayed retroperitoneal drainage may become necessary to reduce morbidity. Minimally invasive techniques should be considered before open operative intervention. Early operative management is rarely indicated for an isolated renal injury, except in the child who is hemodynamically unstable.

Traumatic atlanto-occipital dislocation in children.

During a 5-year period, 11 children were identified as having traumatic atlanto-occipital dislocation injury on the basis of clinical and radiographic findings. Radiographic criteria including the distance between the tip of the dens and the basion (DB distance), the BC/OA ratio (ratio of the distance between the basion and the posterior arch of C-1 divided by the distance between the opisthion and the anterior arch of C-1; normal ratio, < 1), and the width of the atlanto-occipital joint (normal width, < or = 5 mm) were correlated with clinical presentation and outcome. Normal pediatric values for DB distance were reviewed in a series of 110 lateral cervical spine radiographs. The normal DB distance was 8.3 mm +/- 4.2 (mean +/- 2 standard deviations). All 11 children had DB distances greater than 1.4 cm (mean, 1.7 cm). The BC/OA ratio was greater than 1 in only six patients. Of the 11 children, six died with severe neurologic deficits and five survived with minimal or no neurologic sequela. Without the clinical presentation variable, widened DB distance may be the first abnormality identified to suggest atlanto-occipital injury.

Prenatal diagnosis and management of congenital volvulus.

We present a case of a twin gestation in which one twin developed a complex abdominal mass and signs of small bowel obstruction. Following delivery, an exploratory laparotomy revealed a meconium pseudocyst and midgut volvulus. While the infant survived, a large portion of small bowel was atretic, resulting in short bowel syndrome. The prenatal diagnosis of volvulus is rare. When volvulus is present, the risk of bowel infarction is high, with variable perinatal outcome.