CT outperforms radiographs at a comparable radiation dose in the assessment for spondylolysis.

BACKGROUND: Lumbar spondylolysis, a unilateral or bilateral fracture at pars interarticularis, is a common cause of low back pain in children. The initial imaging study in the diagnosis of lumbar spondylolysis has historically been lumbar spine radiographs; however, radiographs can be equivocal or false-negative. Definitive diagnosis can be achieved with computed tomography (CT), but its use has been limited due to the dose of ionizing radiation to the patient. OBJECTIVE: By limiting the z-axis coverage to the relevant anatomy and optimizing the CT protocol, we are able to provide a definitive diagnosis of fractures of the pars interarticularis at comparable or lower radiation dose than commonly performed lumbar spine radiographs. As there is no gold standard for the diagnosis of spondylolysis besides surgery, we compared interobserver agreement and degree of confidence to determine which modality is preferable. MATERIALS AND METHODS: Sixty-two patients with low back pain ages 5-18 years were assessed for the presence of spondylolyis. Forty-seven patients were evaluated by radiography and 15 patients were evaluated by limited field-of-view CT. Both radiographic and CT examinations were assessed anonymously in random order for the presence or absence of spondylolyisis by six raters. Agreement was assessed among raters using a Fleiss Kappa statistic for multiple raters. RESULTS: CT provided a significantly higher level of agreement among raters than radiographs (P < 0.001). The overall Kappa for rater agreement with radiographs was 0.24, 0.34 and 0.40 for 2, 3 or 4 views, respectively, and 0.88 with CT. CONCLUSION: Interobserver agreement is significantly greater using limited z-axis coverage CT when compared with radiographs. Radiologist confidence improved significantly with CT compared to radiographs regardless of the number of views.

Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES: In autosomal dominant polycystic kidney disease (ADPKD), progressive kidney cyst formation commonly leads to ESRD. Because important manifestations of ADPKD may be evident in childhood, early intervention may have the largest effect on long-term outcome. Statins are known to slow progressive nephropathy in animal models of ADPKD. This randomized double-blind placebo-controlled phase III clinical trial was conducted from 2007 to 2012 to assess the effect of pravastatin on height-corrected total kidney volume (HtTKV) and left ventricular mass index (LVMI) by magnetic resonance imaging (MRI) and urine microalbumin excretion (UAE) in children and young adults with ADPKD. DESIGNS, SETTING, PARTICIPANTS, & MEASUREMENTS: There were 110 pediatric participants with ADPKD and normal kidney function receiving lisinopril who were randomized to treatment with pravastatin or placebo for a 3-year period with evaluation at 0, 18, and 36 months. The primary outcome variable was a ≥ 20% change in HtTKV, LVMI, or UAE over the study period. RESULTS: Ninety-one participants completed the 3-year study (83%). Fewer participants receiving pravastatin achieved the primary endpoint compared with participants receiving placebo (69% versus 88%; P=0.03). This was due primarily to a lower proportion reaching the increase in HtTKV (46% versus 68%; P=0.03), with similar findings observed between study groups for LVMI (25% versus 38%; P=0.18) and UAE (47% versus 39%; P=0.50). The percent change in HtTKV adjusted for age, sex, and hypertension status over the 3-year period was significantly decreased with pravastatin (23% ± 3% versus 31% ± 3%; P=0.02). CONCLUSIONS: Pravastatin is an effective agent to slow progression of structural kidney disease in children and young adults with ADPKD. These findings support a role for early intervention with pravastatin in this condition.

Clinical presentation of cervical ribs in the pediatric population.

Cervical ribs may cause thoracic outlet syndrome in adults, but symptoms are poorly described in children. In our series, 88.8% of the 322 children were asymptomatic. The most common symptoms were neck mass and pain. Useful diagnostic tools were cervical spine and chest radiographs. Differential diagnosis of a supraclavicular mass includes cervical ribs.

Effect of statin therapy on disease progression in pediatric ADPKD: design and baseline characteristics of participants.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney condition and is associated with important renal and cardiovascular manifestations in childhood. Renal cystic disease can be documented in some cases as early as in utero. Early intervention is critical if the long-term complications of this condition, including end-stage renal disease, are to be ameliorated. Here we describe our ongoing randomized double-blind placebo-controlled phase III clinical trial to assess the effect of pravastatin treatment on renal and cardiovascular disease progression in 107 children and young adults age 8-22 years with ADPKD who are receiving the angiotensin converting enzyme inhibitor lisinopril. Baseline demographic and laboratory data are provided. Results of this study could markedly impact the standard of care for evaluation and treatment of ADPKD in this population.

Magnetic resonance imaging of kidney and cyst volume in children with ADPKD.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and has important clinical manifestations in childhood. Numerous studies have documented the superiority of magnetic resonance imaging (MRI) for serial monitoring of kidney and cyst volume in this condition in adults. However, no studies have examined the utility of MRI for serial assessment of kidney and cyst volume in children with ADPKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Subjects 4 to 21 years of age with ADPKD underwent abdominal MRI on an annual basis for 5 years. Subjects were grouped according to BP as hypertensive (HBP; BP≥95th percentile for age, height, and gender) or as normotensive (NBP; BP<95th percentile). Total kidney volume (TKV), cyst volume, and cyst number were assessed by stereology. RESULTS: MRI studies (n=302) were obtained in 77 children with ADPKD. TKV and cyst volume were significantly increased in HBP versus NBP subjects. HBP subjects demonstrated a greater increase in fractional cyst volume over time versus NBP subjects. Cyst number increased more rapidly in HBP ADPKD children. CONCLUSIONS: This is the first large-scale clinical study examining the utility of MRI for serial assessment of TKV, cyst volume, and cyst number in children with ADPKD. These results demonstrate that MRI is an acceptable means to follow these parameters in children with ADPKD. Because of the embryonic occurrence of cysts, interventional trials are needed in ADPKD children and MRI may be the preferred renal imaging approach.

Prospective change in renal volume and function in children with ADPKD.

BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease (ADPKD) is a progressive hereditary disorder affecting children and young adults. Early intervention may be necessary to significantly affect the long-term consequences of this disease. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: The authors conducted a 5-yr randomized clinical trial to assess the effect of BP control with angiotensin-converting enzyme inhibition (ACEI) on disease progression in 85 children and young adults with ADPKD. Study groups were determined by subject BP, including hypertension (BP >or= 95th percentile), borderline hypertension (BP 75 to 95th percentile), and severe ADPKD (BP 10 renal cysts). The primary outcome variable was renal volume by ultrasound, with secondary outcome variables including left ventricular mass index (LVMI) and microalbuminuria. In secondary analysis, the authors compared results between hypertensive and normotensive groups. RESULTS: The authors were not able to demonstrate a significant effect of ACEI on renal growth in young subjects with ADPKD. Hypertensive children were at particular risk for increases in renal volume and LVMI and decreased renal function as compared with the other study groups, and borderline hypertensive children were at high risk to develop hypertension over time. However, ACEI treatment was associated with stable renal function and LVMI in this group of children. CONCLUSIONS: Close monitoring of cardiovascular and renal status is indicated in ADPKD children with hypertension or borderline hypertension. In contrast to effects in hypertensive ADPKD children, ACEI treatment in normotensive or borderline hypertensive ADPKD children may prevent the development of increased LVMI and deterioration in renal function.

Facial fractures in children: unique patterns of injury observed by computed tomography.

OBJECTIVE: To determine the patterns of facial fractures observed in pediatric patients after acute trauma. MATERIALS AND METHODS: The computed tomography studies of 338 patients (63% male, 37% female; 7 months to 18 years of age) performed after acute nonpenetrating facial trauma were retrospectively reviewed to evaluate for facial fractures and associated orbital hematomas or contiguous skull fractures. Fracture patterns were characterized as orbital roof, orbital floor, medial orbital wall, nasal bone, naso-orbital-ethmoid, zygomatic complex, isolated zygomatic arch, Le Fort type (I, II, or III), maxillary sagittal, alveolar ridge, or mandibular. The frequency of the various fracture types was determined. The correlation between fracture type and orbital hematomas or contiguous skull fractures was assessed (Kendall tau rank correlation). RESULTS: Computed tomography demonstrated facial fractures in 188 (54%) patients. The number and frequency of the fractures observed were as follows: orbital roof, 67 (36%); zygomatic complex, 38 (20%); naso-orbital-ethmoid, 30 (16%); orbital floor, 28 (15%); nasal bone, 25 (13%); mandibular, 24 (13%); medial orbital wall, 16 (9%); maxillary sagittal, 11 (6%); alveolar ridge, 8 (4%); isolated zygomatic arch, 3 (2%); Le Fort type I, 4 (2%); Le Fort type II, 4 (2%); and Le Fort type III, 0 (0%). Fifty children (27%) had multiple fractures. Orbital hematomas were seen in 28 patients (15%), and contiguous skull fractures were seen in 54 patients (29%). There was strong correlation between orbital hematomas and orbital roof fractures (0.62, P < 0.0001), orbital hematomas and naso-orbital-ethmoid fractures (0.18, P = 0.001), contiguous skull and orbital roof fractures (0.57, P < 0.0001), and contiguous skull and naso-orbital-ethmoid fractures (0.39, P < 0.0001). CONCLUSIONS: Fractures of the orbital roof are the most common facial fractures observed in pediatric patients after acute nonpenetrating trauma. Orbital roof and naso-orbital-ethmoid fractures are frequently associated with orbital hematomas and contiguous skull fractures.

Increased left ventricular mass in children with autosomal dominant polycystic kidney disease and borderline hypertension.

Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary condition that may be diagnosed in utero. Our goal was to evaluate symptoms of ADPKD in children, including left ventricular mass index (LVMI), renal volume, renal function and microalbuminuria in relation to systolic and diastolic blood pressure. Eighty-five children were stratified by blood pressure into three cohorts: hypertensive (95th percentile and over), borderline hypertensive (75-95th percentile) and normotensive (75th percentile and below). There were no differences in gender, age, height, renal function, or microalbuminuria between the groups. Both the hypertensive and borderline hypertensive children had a significantly higher LVMI than normotensive children, with no significant difference between hypertensive and borderline hypertensive groups. There was a significant correlation between renal volume and both systolic and diastolic blood pressures in all subjects. Renal volume in hypertensive children was significantly larger than in the borderline hypertensive group, with no significant difference between normotensive and borderline hypertensive groups. These findings show that an increase in LVMI may be detected earlier than an increase in renal volume in children with ADPKD and borderline hypertension, suggesting that close monitoring of cardiac status is indicated in these children.

ACR Appropriateness Criteria on headache-child.

In a pediatric patient with an isolated headache or a classic migraine unaccompanied by neurologic signs, presence of a seizure, or supporting historical data, an imaging workup is usually not indicated. For a sudden severe headache or a headache with positive neurologic signs or symptoms or supporting historical data, MRI or CT should be considered. For the acute severe (thunderclap) headache, CTA, MRA, or catheter angiography may be appropriate.

Early inhaled nitric oxide therapy in premature newborns with respiratory failure.

BACKGROUND: The safety and efficacy of early, low-dose, prolonged therapy with inhaled nitric oxide in premature newborns with respiratory failure are uncertain. METHODS: We performed a multicenter, randomized trial involving 793 newborns who were 34 weeks of gestational age or less and had respiratory failure requiring mechanical ventilation. Newborns were randomly assigned to receive either inhaled nitric oxide (5 ppm) or placebo gas for 21 days or until extubation, with stratification according to birth weight (500 to 749 g, 750 to 999 g, or 1000 to 1250 g). The primary efficacy outcome was a composite of death or bronchopulmonary dysplasia at 36 weeks of postmenstrual age. Secondary safety outcomes included severe intracranial hemorrhage, periventricular leukomalacia, and ventriculomegaly. RESULTS: Overall, there was no significant difference in the incidence of death or bronchopulmonary dysplasia between patients receiving inhaled nitric oxide and those receiving placebo (71.6 percent vs. 75.3 percent, P=0.24). However, for infants with a birth weight between 1000 and 1250 g, as compared with placebo, inhaled nitric oxide therapy reduced the incidence of bronchopulmonary dysplasia (29.8 percent vs. 59.6 percent); for the cohort overall, such treatment reduced the combined end point of intracranial hemorrhage, periventricular leukomalacia, or ventriculomegaly (17.5 percent vs. 23.9 percent, P=0.03) and of periventricular leukomalacia alone (5.2 percent vs. 9.0 percent, P=0.048). Inhaled nitric oxide therapy did not increase the incidence of pulmonary hemorrhage or other adverse events. CONCLUSIONS: Among premature newborns with respiratory failure, low-dose inhaled nitric oxide did not reduce the overall incidence of bronchopulmonary dysplasia, except among infants with a birth weight of at least 1000 g, but it did reduce the overall risk of brain injury. (ClinicalTrials.gov number, NCT00006401 [ClinicalTrials.gov].).

Giant cystic abdominal masses in children.

In this pictorial essay the common and uncommon causes of large cystic and cyst-like abdominal masses in children are reviewed. We discuss and illustrate the following: mesenchymal hamartoma, choledochal cyst, hydrops of the gallbladder, congenital splenic cyst, pancreatic pseudocyst, pancreatic cystadenoma, hydronephrosis, multicystic dysplastic kidney, multilocular cystic nephroma, adrenal hemorrhage, mesenteric and omental cysts, gastrointestinal duplication cyst, meconium pseudocyst, ovarian cysts and cystic neoplasms, hematocolpos, urachal cysts, appendiceal abscess, abdominal and sacrococcygeal teratoma, and CSF pseudocyst. We also describe imaging features and clues to the diagnosis.

Design and baseline characteristics of participants in the study of antihypertensive therapy in children and adolescents with autosomal dominant polycystic kidney disease (ADPKD).

In this manuscript, we describe our ongoing randomized clinical trial to assess the efficacy of blood pressure control with angiotensin converting enzyme (ACE) inhibition on renal cyst growth over a 5-year study period in children and young adults aged 4-21 years with autosomal dominant polycystic kidney disease (ADPKD). Baseline demographic and laboratory data for the study groups are reported. Results of this study could significantly impact the standard of care for management of ADPKD in this population.

The positive predictive value of rib fractures as an indicator of nonaccidental trauma in children.

BACKGROUND: Rib fractures have a strong association with nonaccidental trauma (NAT) and severe trauma. The purposes of this study were to evaluate rib fractures in children to determine (1) the positive predictive value of a rib fracture in defining NAT and (2) the frequency of rib fractures as the only skeletal manifestation of NAT. METHODS: We reviewed the medical records and imaging of all children with rib fractures over a 6-year period. NAT was determined by the Child Advocacy and Protection team. RESULTS: In children younger than 3 years of age, the positive predictive value (PPV) of a rib fracture as an indicator of NAT was 95%. The positive predictive value increased to 100% once historical and clinical circumstance excluded all other causes for rib fractures. CONCLUSION: In this study, rib fracture(s) were the only skeletal manifestation of NAT in 29% of the children.

Physiologic response to moderate altitude exposure among infants and young children.

Substantial numbers of children are exposed to moderate altitude while traveling to mountain resorts with their families. Although there has been extensive study of the adult physiologic response to altitude exposure, few studies of infants and young children exist. This investigation examines the acute physiologic responses to moderate altitude exposure among young children and the relationship of these responses to the development of acute mountain sickness (AMS). Children 3 to 36 months old participated in the prospective observational study, which included baseline measurements at 1610 m and measurements after a 24-h exposure to 3109 m. Measurements included pulse and respiratory rate, end-tidal CO(2), arterial oxygen saturation (pulse oximetry), cerebral tissue oxygenation (St(O2)) by near-infrared spectroscopy, middle cerebral artery resistive index by transcranial Doppler, lateral ventricle volumes (ultrasound), and clinical evaluation for the presence of acute mountain sickness (Children's Lake Louise Score). Twenty-four children (13 girls and 11 boys, age 14.5 +/- 10.2 months) participated. After acute exposure to 3109 m, these children showed an increase in respiratory rate from 45 +/- 13 to 51.9 +/- 15 breaths/min (p < 0.008), accompanied by a decrease of end-tidal CO(2) from 31 +/- 3 to 28 +/- 2 mm Hg (p < 0.001) and a reduction of arterial oxygen saturation from 95 +/- 2 to 91 +/- 2% (p < 0.001). St(O2) also decreased from 78 +/- 8 to 67 +/- 13% (p < 0.001), and this reduction appeared to be related to age (r = 0.58, p < 0.05), with lower saturations found in younger children. No evidence of increased intracranial pressure, as assessed by middle cerebral artery resistive index, was seen during ascent. Seven subjects developed symptoms of AMS; however, no relationship was found between the physiologic changes observed and the presence of symptoms. Ascent from 1610 to 3109 m resulted in tachypnea, relative hypoxia, hypocapnia, and a reduction in cerebral tissue oxygenation (St(O2)). The reduction in St(O2) appeared to be related to age, with infants appearing to be the most susceptible to cerebral tissue oxygen desaturation at high altitude. No relationship appears to exist between the presence of AMS and the physiologic measurements.