Clinical Follow-Up without Radiographs Is Sufficient after Most Nonoperatively Treated Distal Radius Fractures in Children.

Distal forearm fractures are common in children and are usually treated nonoperatively. No consensus has been reached on how to perform clinical and radiographic follow-up of these fractures. Our aim was to study whether radiographic and clinical follow-up is justified. We included 100 consecutive patients with non-operatively treated distal forearm fractures who were treated at Oulu University Hospital in 2010-2011. The natural history of the fractures during the nonoperative treatment was analyzed by measuring the potential worsening of the alignment during the follow-up period. The limits of acceptable fracture position were set according to the current literature using "strict" or "wide" criteria for alignment. We determined the rate of worsening fracture position (i.e., patients who reached the threshold of unacceptable alignment). In relation to splinting, we evaluated how many patients benefited from clinical follow-up. Most of the fractures (98%) preserved acceptable alignment during the entire follow-up period when wide criteria were used. The application of stricter criteria for alignment in radiographs showed loss of reduction in 19% of the fractures. Worsening of the alignment was recognized at a mean of 13 days (range 5-29) after the injury. One in three (32%) patients needed some intervention due to splint loosening or failure. Radiographic follow-up of nonoperatively treated distal forearm fractures remains questionable. Instead, clinical follow-up is important, as 32% of patients needed their splints fixed.

Retrograde intramedullary nailing of the radius in children: A pilot magnetic resonance imaging study of soft-tissue findings.

Purpose: Unstable forearm shaft fractures in children are preferably treated surgically using elastic stable intramedullary nails. The radius is nailed retrograde from the distal metaphysis. There is a risk of surgery-related soft-tissue complications during the operation. Close evaluation of occult surgery-related soft-tissue lesions has not been possible previously, due to the titanium alloy hardware used in the process. The aim of the present study was to evaluate the potential findings in the surrounding soft tissues after intramedullary nailing of the radius, by using magnetic resonance imaging. Methods: The study population comprised 15 pediatric patients with forearm shaft fractures treated by polylactide-co-glycolide biodegradable intramedullary nails and postoperatively evaluated via magnetic resonance imaging. The main outcome was signal abnormality in any tendon at the entry point postoperatively. Secondarily, other changes in the soft tissues related to nailing were determined. Furthermore, the precise location of the entry point and the anatomic characteristics of the soft-tissue tunnel were described. Results: In total, 5 of 15 patients (33.3%) had transient signal pathology in a tendon postoperatively. Edema around the superficial radial nerve was detected in 13 of 15 patients (86.7%). The most common surgical approach was between the extensor pollicis brevis and the extensor carpi radialis longus tendons, which was applied in 10 of 15 patients (66.7%). Conclusions: One in three patients exhibited transient and occult surgery-related intraparenchymal signal pathology in a tendon, after forearm intramedullary nailing. Caution with surgical prepare of the soft-tissue cleavage is recommended. Level of evidence: IV.

Brain volumes and white matter microstructure in 8- to 10-year-old children born with fetal growth restriction.

BACKGROUND: Fetal growth restriction caused by placental insufficiency is associated with increased risk of poor neurodevelopment, even in the absence of specific perinatal brain injury. Placental insufficiency leads to chronic hypoxaemia that may alter cerebral tissue organisation and maturation. OBJECTIVE: The aim of this study was to assess the effects fetal growth restriction and fetal haemodynamic abnormalities have on brain volumes and white matter microstructure at early school age. MATERIALS AND METHODS: This study examined 32 children born with fetal growth restriction at 24 to 40 gestational weeks, and 27 gestational age-matched children, who were appropriate for gestational age. All children underwent magnetic resonance imaging (MRI) at the age of 8-10 years. Cerebral volumes were analysed, and tract-based spatial statistics and atlas-based analysis of white matter were performed on 17 children born with fetal growth restriction and 14 children with birth weight appropriate for gestational age. RESULTS: Children born with fetal growth restriction demonstrated smaller total intracranial volumes compared to children with normal fetal growth, whereas no significant differences in grey or white matter volumes were detected. On atlas-based analysis of white matter, children born with fetal growth restriction demonstrated higher mean and radial diffusivity values in large white matter tracts when compared to children with normal fetal growth. CONCLUSION: Children ages 8-10 years old born with fetal growth restriction demonstrated significant changes in white matter microstructure compared to children who were appropriate for gestational age, even though no differences in grey and white matter volumes were detected. Poor fetal growth may impact white matter maturation and lead to neurodevelopmental impairment later in life.

Long-Term Results of Pediatric Congenital Pulmonary Malformation: A Population-Based Matched Case-Control Study with a Mean 7-Year Follow-Up.

Symptomatic congenital pulmonary malformations (CPMs) are a group of anomalies involving the lungs. The long-term outcomes of these patients are not well known. The present research aimed to study the pulmonary function, respiratory morbidity, and health-related quality of life (QoL) of patients treated for CPMs. All children (<16 years of age) treated for CPMs in 2002−2012 (in Oulu University Hospital) were invited to the follow-up visit. Altogether, there were 22 patients, out of which 17 (77%) participated. The mean follow-up time was 6.6 (ranged from 3 to 16) years. Pulmonary function tests, diffusing capacity, respiratory morbidity, and QoL were determined as the primary outcomes. Potential residual malformations and lung anatomy were investigated using computer tomography (CT) imaging. The outcomes were compared to the age- and sex-matched healthy controls. The forced expiratory volume at 1 s (FEV1, Z-score) remained lower in operated patients compared to the healthy controls (−1.57 ± SD 1.35 vs. −0.39 ± SD −0.86, p-value 0.005). There were no differences in respiratory morbidity or QoL between the patients and the controls. The surgical approach (lobectomy vs. partial resection) did not affect lung function. A younger age (<1 year of age) at the time of the surgery seemed to result in a higher lung capacity, but the finding was not statistically significant. Patients with CPMs treated with surgery were satisfied with their wellbeing in the long-term. A lower lung function did not have an impact on their wellbeing. However, there was a slight decrease in lung function compared to the healthy controls, and a clinical follow-up of the patients was recommended.

Randomized Controlled Trial of the Clinical Recovery and Biodegradation of Polylactide-co-glycolide Implants Used in the Intramedullary Nailing of Children's Forearm Shaft Fractures with at Least Four Years of Follow-Up.

The preferred surgical fixation of forearm shaft fractures in children is Elastic Stable Intramedullary Nailing (ESIN). Due to known disadvantageous effects of metal implants, a new surgical method using biodegradable polylactide-co-glycolide (PLGA) intramedullary nails has been developed but its long-term outcomes are unclear. The aim of this study was to compare the long-term outcomes of Biodegradable Intramedullary Nailing (BIN) to ESIN and assess the biodegradation of the study implants via magnetic resonance imaging (MRI). The study population of the prospective, randomized trial consisted of paediatric patients whose forearm shaft fractures were treated with BIN (n = 19) or ESIN (n = 16). Forearm rotation at minimally four years' follow-up was the main outcome. There was no clinically significant difference in the recovery of the patients treated with the BIN as compared to those treated with the ESIN. More than half of the implants (57.7%, n = 15/26) were completely degraded, and the rest were degraded almost completely. The PLGA intramedullary nails used in the treatment of forearm shaft fractures in this study resulted in good function and anatomy. No unexpected disadvantages were found in the degradation of the implants. However, two implant failures had occurred in three months postoperatively.

Childhood Urinary Tract Infections and Pregnancy-Related Complications in Adult Women.

BACKGROUND AND OBJECTIVES: Long-term outcomes of urinary tract infection (UTI) in childhood are not well known. Pregnancy may reveal latent renal damage caused by a UTI because of stress on the kidneys. METHODS: Our cohort included adult women with an ultrasonography taken because of a childhood UTI in 1981-1991 (N = 1175). Nine women with a severe congenital kidney malformation or urinary tract obstruction were excluded. Altogether, 260 mothers with a childhood UTI and 500 population-based control mothers without a childhood UTI matched for age, and delivery dates were compared. Our primary end point was the proportion of women with essential or gestational hypertension, preeclampsia, proteinuria, or pyelonephritis during the first pregnancy. RESULTS: The pregnancy outcomes of the women with a UTI in childhood did not differ from those of the controls because 105 of 260 (40%) patients met the primary end point compared with 204 of 500 (41%) controls (relative risk [RR] 0.99; 95% confidence interval [CI] 0.82 to 1.2; P = .91). Similarly, there were no significant differences between the 2 groups in essential hypertension (RR 1.0; 95% CI 0.65 to 1.6; P = .92), gestational hypertension (RR 0.93; 95% CI 0.74 to 1.2; P = .54), preeclampsia (RR 1.5; 95% CI 0.91 to 2.5; P = .11), proteinuria (RR 1.2; 95% CI 0.81 to 1.8; P = .36) or pyelonephritis (2 controls and none of the patients; P = .55) during the first pregnancy. CONCLUSIONS: Childhood UTIs did not increase the risk of pregnancy-related complications in this controlled population-based study. Thus, UTIs in childhood without severe urinary tract abnormalities appear to have a minimal effect on kidney health in early adulthood.

Interventional magnetic resonance imaging as a diagnostic and therapeutic method in treating acute pediatric atlantoaxial rotatory subluxation.

Atlantoaxial rotatory subluxation or fixation (AARF) is a rare condition, usually occurring in pediatric patients. It mimics benign torticollis but may result in permanent disability or death. The condition requires prompt diagnosis by thorough examination to avoid any treatment delays. Spiral computed tomography (CT) with three-dimensional reconstruction CT is recommended for identifying incongruence between C1 and C2 vertebrae, and magnetic resonance imaging (MRI) may be performed to exclude ligamentous injuries. In addition to static imaging, dynamic CT involves the reduction between C1 and C2 being confirmed using CT with the head turned maximally to the left and right. The present report (level of evidence, III) provides a method for treating AARF that has similar advantages as dynamic CT but avoids ionizing radiation by replacing CT with interventional MRI. The new method comprised simultaneous axial traction and manual closed reduction, performed under general anesthesia, and the use of interventional MRI to ensure that reduction was achieved and held. The head is turned maximally to the right and left during the manual reduction. A rigid cervical collar was used following reduction. Dynamic CT was not required but prior diagnostic static CT was performed in preparation. No further CT was required. There appears to be no previous studies on interventional MRI in AARF care. Being superior in its diagnostic soft-tissue visualization performance and lacking ionizing radiation, interventional MRI is a potential option for investigating and treating acute AARF in non-syndromic patients with no trauma history.

Intramedullary nailing of forearm shaft fractures by biodegradable compared with titanium nails: Results of a prospective randomized trial in children with at least two years of follow-up.

There are disadvantages in Elastic Stable Intramedullary Nailing (ESIN) of forearm-shaft fractures, such as the need of implant removal. Biodegradable Intramedullary Nailing (BIN) is a new technique developed for these fractures. We hypothesized that there is no difference in rotational ROM between the patients treated by BIN vs. ESIN. A randomized, controlled clinical trial included patients, aged 5-15 years, requiring surgery for forearm-shaft fractures. Biodegradable polylactide-co-glycolide (PLGA) nails (Activa IM-Nail™, Bioretec Ltd., Finland) were used in 19 and titanium nails (TEN®, SynthesDePuy Ltd., USA) in 16 patients. Rotational ROM of forearm after two years was the primary outcome. Elbow and wrist ROM, pain and radiographic bone healing were secondary outcomes. Forearm rotation was mean 162° and 151° in BIN and ESIN groups, respectively (P = 0.201). No difference between the groups was found in any other ROMs. Three cases in the ESIN vs. none in the BIN group reported pain (P = 0.113). There was no clinically significant residual angulation in radiographs. Two adolescents in the BIN group vs. none in the ESIN (P = 0.245) were excluded because of implant failure; another two with complete bone union suffered from re-injury. Therefore, satisfactory implant stability among older children needs to be studied.

Structural Pulmonary Abnormalities Still Evident in Schoolchildren with New Bronchopulmonary Dysplasia.

BACKGROUND: A new pattern of bronchopulmonary dysplasia (BPD) has emerged with the improved survival of preterm children. OBJECTIVES: Our aim was to characterize structural abnormalities associated with new BPD and to evaluate whether the severity of high-resolution computed tomography (HRCT) changes is associated with lung function. METHODS: HRCT scans were performed on 21 schoolchildren with a history of new BPD (mild, n = 9; moderate, n = 4; and severe, n = 8) with a mean age of 12.7 years (range: 8.7-16.7). Scans were interpreted by 2 radiologists using a structured scoring system. Spirometry (forced expiratory volume in 1 s [FEV1] and maximum mid-expiratory flow [MMEF]) and the diffusion capacity of the lung for carbon monoxide (DLCO) were measured. RESULTS: At least 1 HRCT abnormality was evident in 17 children (81%), including linear-to-triangular subpleural opacities (71%), air trapping (29%), mosaic perfusion (24%), peribronchial thickening (14%), and emphysema (14%). The HRCT score was higher in the severe BPD group (11.50; 95% CI 2.86-20.14) than in the mild or moderate BPD group (1.39; 95% CI 0.24-2.54, and 2.75; 95% CI 0.28-5.22, respectively). HRCT scores were inversely related to FEV1 (ß -4.23; 95% CI -6.97 to -1.49, p = 0.004) and MMEF (ß -3.45; 95% CI -6.10 to -0.80, p = 0.013) but not to DLCO. The duration of the initial mechanical ventilation was associated with HRCT scores (p = 0.014). CONCLUSIONS: Structural lung abnormalities are common among schoolchildren with a history of new BPD, resembling abnormalities described in the presurfactant era. HRCT abnormalities are associated with the duration of early mechanical ventilation and the severity of BPD and they are correlated with spirometry.

Riboflavin-Responsive Multiple Acyl-CoA Dehydrogenase Deficiency Associated with Hepatoencephalomyopathy and White Matter Signal Abnormalities on Brain MRI.

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare inborn error of metabolism affecting both fatty acid and amino acid oxidation. It can manifest at any age, but riboflavin-responsiveness has mainly been described in less severely affected patients. We describe an infant with severe MADD presenting with profound hypotonia and hepatomegaly. Treatment with riboflavin improved his muscle strength, liver size, and biochemical markers. A homozygous mutation of electron transfer flavoprotein dehydrogenase (ETFDH) was found. His motor skills continued to progress until a fatal infection-triggered deterioration at the age of 34 months. We show changes in brain magnetic resonance imaging over the course of the disease, with profound white matter abnormalities during the deterioration phase. Aggregates of mitochondria with abnormal cristae in muscle electron microscopy were noticed already in infancy. An unusual lactate dehydrogenase (LDH) isoenzyme pattern with LDH-1 predominance was additionally observed. This case demonstrates riboflavin-responsiveness in a severely affected infant with both muscular and extramuscular involvement and further underlines the variable nature of this disease.

Effect of display type and room illuminance in chest radiographs.

OBJECTIVES: To compare diagnostic accuracy in the detection of subtle chest lesions on digital chest radiographs using medical-grade displays, consumer-grade displays, and tablet devices under bright and dim ambient light. METHODS: Five experienced radiologists independently assessed 50 chest radiographs (32 with subtle pulmonary findings and 18 without apparent findings) under bright (510 lx) and dim (16 lx) ambient lighting. Computed tomography was used as the reference standard for interstitial and nodular lesions and follow-up chest radiograph for pneumothorax. Diagnostic accuracy and sensitivity were calculated for assessments carried out in all displays and compared using the McNemar test. The level of significance was set to p < 0.05. RESULTS: Significant differences in sensitivity between the assessments under bright and dim lighting were found among consumer-grade displays in interstitial opacities with, and in pneumothorax without, Digital Imaging and Communication in Medicine-Grayscale Standard Display Function (DICOM-GSDF) calibration. Compared to 6 megapixel (MP) display under bright lighting, sensitivity in pneumothorax was lower in the tablet device and the consumer-grade display. Sensitivity in interstitial opacities was lower in the DICOM-GSDF calibrated consumer-grade display. CONCLUSIONS: A consumer-grade display with or without DICOM-GSDF calibration or a tablet device is not suitable for reading digital chest radiographs in bright lighting. No significant differences were observed between five displays in dim light. KEY POINTS: • Ambient lighting affects performance of consumer-grade displays (with or without DICOM-GSDF calibration). • Bright light decreases detection of pneumothorax on non-medical displays. • Bright light decreases detection of interstitial opacities on DICOM-GSDF-calibrated, consumer-grade displays. • Dim light is sufficient to detect subtle chest lesions from all displays.

[Acute hematogenous osteomyelitis in children]. / Lasten akuutti hematogeeninen osteomyeliitti.

In children, osteomyelitis is usually acute and hematogenous, the metaphysis of long bones being the most common location. Common symptoms include limb pain and fever. Essential investigations include laboratory tests measuring inflammation, and blood culture. Although osteomyelitis is initially undetectable by plain X-ray, the investigation is nevertheless necessary, e.g. in order to exclude fractures. Magnetic resonance imaging is useful especially in cases of suspected complications. A bone scan is utilized in unclear cases. Treatment of the disease is started with an intravenous antimicrobial medication directed against the most common causative agent, Staphylococcus aureus.

Long-term follow-up of patients after childhood urinary tract infection.

OBJECTIVE: To evaluate the long-term outcome of children with urinary tract infection (UTI). DESIGN: Follow-up examination 6 to 17 years after childhood UTI. SETTING: Secondary to tertiary referral center. PATIENTS: From an original population-based cohort of 1185 children with a history of UTI on whom both ultrasonography (US) and voiding cystourethrography had been performed between January 1, 1993, and December 31, 2003, we excluded 24 cases with major renal dysplasia or obstruction of the urinary tract to form a study cohort of 1161 patients. We took a stratified random sample of 228 patients for follow-up, and a total of 193 (85%) participated. Of the 193 participating patients, 103 (53%) had received antibiotic prophylaxis and 42 (22%) had undergone surgery. MAIN EXPOSURE: Urinary tract infection. MAIN OUTCOME MEASURES: Renal growth and parenchymal damage in US examination, kidney function, and blood pressure. RESULTS: Unilateral renal parenchymal defect was found in 22 of the 150 patients (15%) studied with US at follow-up, and unilateral kidney growth retardation was found in 5 patients (3%). All but 1 of the renal parenchymal defects seen on US were in patients with grade III to V vesicoureteral reflux. Despite the parenchymal defects seen on US, the serum cystatin C concentration, estimated glomerular filtration rate, and blood pressure were within the normal ranges in all patients. CONCLUSIONS: The risk of long-term consequences from childhood UTI seems to be very low. Owing to the observational nature of our study, we cannot exclude the effects of the given treatment on the outcome of our patients.

Transient defect in nitric oxide generation after rupture of fetal membranes and responsiveness to inhaled nitric oxide in very preterm infants with hypoxic respiratory failure.

OBJECTIVE: To study antenatal risk factors and inflammatory responses during hypoxic respiratory failure (HRF) in infants of very low gestational age (VLGA, ≤32.0 weeks). STUDY DESIGN: Of a cohort of 765 VLGA infants, 144 required mechanical ventilation. Airway specimens from these patients were prospectively studied. Infants who developed HRF (oxygenation index >25) with echocardiographic diagnosis of pulmonary hypertension were treated with inhaled nitric oxide (iNO). Three gestation comparison groups were formed on the basis of specific antenatal complications: prolonged preterm rupture of membranes (PPROM), spontaneous preterm birth, and preeclampsia. Chest radiographs were studied and airway specimens were analyzed for concentrations of tumor necrosis factor-α, interleukin (IL)-6, IL-8, IL-10, IL-12p70, IL-1ß, and nitrite + nitrate over 4 days. RESULTS: Seventeen (2.2% of all VLGA infants) developed HRF. In all 17 cases, PPROM complicated the antenatal course; these infants responded to iNO, regardless of infection or PPROM. The chest radiographs of HRF and non-HRF PPROM infants were similar. Airway proinflammatory cytokines and nitrite + nitrate levels were low in infants with HRF, but they increased during iNO treatment and remained elevated after discontinuation of iNO. Each of the 3 comparison groups had different and characteristic patterns of airway cytokines and nitrite + nitrate levels. CONCLUSIONS: Seven percent of VLGA infants with preterm rupture of membranes and 15% of those with PPROM developed HRF, characterized by pulmonary hypertension that acutely responds to iNO. These infants may have a transient deficiency in the inflammatory response, including a defect in nitric oxide generation in airspaces.

Imaging the urinary tract in children with urinary tract infection.

AIM: To evaluate whether ultrasonography (US) alone is sufficient in imaging the urinary tract in 1185 children with urinary tract infection (UTI). METHODS: The reports on US and voiding cystourethrography (VCUG) were reviewed. RESULTS: Initial US was normal in 861/1185 patients (73%). VCUG revealed abnormal findings in 285/861 (33%), of which grade III-V vesicoureteral reflux (VUR) comprised 97 cases (11%). During follow-up, VUR had resolved in 88/97 (91%) patients: in 50/57 (88%) patients without active treatment for VUR, in 27/29 (93%) with endoscopic and in 11/11 (100%) with open surgery for VUR. During follow-up, 11/97 patients (11%) had developed new renal scarring detectable in US, but no renal impairment occurred. Except for VUR, VCUG showed nonobstructive urethral valves in two infant boys with normal initial US. Thus, in 861 children with normal initial US, 40 patients with grade III-V VUR and two patients with significant nonreflux pathology may have benefited from surgical treatment, giving the total number of possibly missed pathological finding in 42/861 (4.9%) cases if VCUG had not been performed. CONCLUSION: We suggest that children with UTI could be examined using US alone and to use VCUG only after additional indications.

Chemokine CCL18 predicts intraventricular hemorrhage in very preterm infants.

BACKGROUND: Intraventricular hemorrhage (IVH) in very preterm infants is a common disease associated with long-term consequences. Risk factors of IVH remain to be further defined. AIMS: To determine whether specific immunoproteins at birth predict the risk of IVH and whether their receptors are localized at the bleeding site. METHODS: A prospective cohort consisted of 163 infants born before 32 weeks of gestation. Altogether 107 cord blood immunoproteins and 12 cytokines from peripheral blood obtained 1 and 7 days after birth were analyzed. Serial brain ultrasounds were assessed. Immunohistochemistry of a chemokine receptor from 14 autopsies was studied. RESULTS: Low levels of cord chemokine CCL18 (chemokine (C-C motif) ligand 18) robustly predicted the risk of IVH grade II-IV when ante- and neonatal risk factors were considered. Cord CCL18 increased from 32 weeks to term. During the first week after very preterm birth CCL18 increased as the risk of new IVH cases decreased. CCL18 receptor, CCR3, was detectable in choroid plexus, periventricular capillary endothelium, ependymal cells, and in germinal matrix. CONCLUSION: Low cord blood CCL18 is an independent risk factor of IVH. CCL18 may inhibit signal transduction of its receptor in periventricular cells. Defining the function and regulation of CCL18 may help to decrease the risk of IVH.

Apparent diffusion coefficient on magnetic resonance imaging in pons and in corona radiata and relation with the neurophysiologic measurement and the outcome in very preterm infants.

BACKGROUND: New imaging techniques allow a detailed visualization of the brain and the findings possibly correlate with neurophysiologic measurements and neurosensory and motor outcomes. Postnatal clinical factors known to associate with neurologic disabilities may contribute to brain abnormalities not visible to the naked eye. OBJECTIVES: We evaluated whether quantitative measurement of organized water diffusion on MR imaging, apparent diffusion coefficient (ADC), relates to neurophysiologic function and to clinical risk factors and motor outcome in preterm infants. METHODS: Diffusion-weighted imaging was successfully performed at term age on 30 infants born <32 weeks of gestation, birth weight <1,000 g. Infants with major destructive brain lesions were excluded from the final analysis (n = 2). ADC was calculated within regions of interest placed in pons and in white matter regions known to contain motor fibers. Brain stem auditory evoked potentials (BAEP) were registered at term age. Gross motor outcome was assessed using Griffiths Scales at 2 years of corrected age. RESULTS: A positive correlation was found between ADC in the pons and the latency of wave III in BAEP (r = 0.619, p = 0.024). Need for inotrope support during the postnatal period and bronchopulmonary dysplasia associated with high ADC values in the pons and in the corona radiata. Infants with poor gross motor outcome had higher ADC in the corona radiata than infants with better outcome (mean = 1.343 vs. 1.197, p = 0.004). CONCLUSIONS: This is the first study to report a relationship between ADC and BAEP measurements in the pons. The study may add to the current knowledge about the relation between postnatal clinical factors and diffusion-weighted imaging when evaluating the high-risk infants.

Weismann-Netter syndrome and mental retardation: a new patient and review of the literature.

In 1954, Weismann-Netter and Stuhl described three sporadic adults and a mother and her three children with short stature and congenital anterior bowing of lower legs [Weismann-Netter and Stuhl (1954); Presse Méd 62:1618-1622]. They named the condition "toxopachyostéose diaphysaire tibio-péronière," which presently is known as Weismann-Netter syndrome (WNS) (OMIM 112350). Since then more than 100 patients have been published. Nearly all have been case reports in French medical literature, and the first report in the Anglo-American literature appeared in 1988. Only a minority of the publications have appeared during the past two decades. The diagnostic findings of WNS are anterior bowing of the diaphyses of tibia and fibula, broadening or "tibialization" of the fibula and posterior cortical thickening of the two bones. Also the diaphyses of other long bones may be similarly affected but usually to a milder degree. The cause of the condition is unknown, but frequent familial cases suggest a genetic defect with autosomal dominant inheritance. Several of the WNS patients have also had mental retardation (MR), but the existence of a WNS-MR syndrome is still pending. We describe a sporadic patient with typical WNS skeletal findings and MR. He also had postnatal growth deceleration with partially corrective pubertal growth, normal head size and normal brain structures on MRI. We review the WNS literature.

Population cohort associating chorioamnionitis, cord inflammatory cytokines and neurologic outcome in very preterm, extremely low birth weight infants.

Intrauterine inflammation may relate to neurologic disability among preterm children. We investigated the relationship between chorioamnionitis, cord serum cytokines, and neurologic outcome. Sixty-one consecutively born very preterm extremely low birth weight (ELBW) infants were prospectively enrolled. Histologic inflammation in placenta and umbilical cord and vascular pathology were evaluated. Cord sera were analyzed for five proinflammatory cytokines. Serial brain ultrasound and magnetic resonance imaging were performed for evaluation of intraventricular hemorrhage (IVH grade I-III) and white matter damage (WMD: cystic periventricular leukomalacia or IVH grade IV). Neurologic and neurocognitive outcomes were assessed at the corrected age of 2 y. The incidences of HCA, WMD, and abnormal neurologic outcome were 48%, 13% and 19%, respectively. HCA or high IL-6 in cord serum predicted spontaneous preterm labor with high accuracy. HCA increased the risk of IVH grade II-III. In HCA, without either clinical chorioamnionitis or histologic placental perfusion defect, the children had a low risk of WMD (0%) and a low risk of abnormal neurologic outcome (6%). In HCA, the concentration of IL-6 in cord serum was lower in children with abnormal neurologic outcome than in children with normal neurologic outcome. In HCA and placental perfusion defect (compound defect) the risk of abnormal neurologic outcome was high. Compound placental defect and WMD additively predicted abnormal neurologic outcome. We propose that HCA together with other insults (placental perfusion defect or maternal systemic infection) increases the risk of poor neurologic outcome in very preterm ELBW infants.