Long-term neurodevelopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxygenation

Background/Purpose

Although there has been a marked improvement in the survival of children with congenital diaphragmatic hernia (CDH) in the past 2 decades, there are few reports of long-term neurodevelopmental outcome in this population. The present study examined neurodevelopmental outcomes in 10- to 16-year-old CDH survivors not treated with extracorporeal membrane oxygenation (ECMO).

Methods

Parents of 27 CDH survivors completed questionnaires assessing medical problems, daily living skills, educational outcomes, behavioral problems, and executive functioning. Fifteen CDH survivors and matched full-term controls completed standardized intelligence, academic achievement, phonological processing, and working memory tests.

Results

Non-ECMO-treated CDH survivors demonstrated high rates of clinically significant difficulties on standardized academic achievement measures, and 14 of the 27 survivors had a formal diagnosis of specific learning disability, attention deficit hyperactivity disorder, or developmental disability. Specific problems with executive function, cognitive and attentional weaknesses, and social difficulties were more common in CDH patients than controls. Perioperative hypocapnia was linked to executive dysfunction, behavioral problems, lowered intelligence, and poor achievement in mathematics.

Conclusions

Non-ECMO-treated CDH survivors are at substantial risk for neurodevelopmental problems in late childhood and adolescence.     

Tumor volume to fetal weight ratio as an early prognostic classification for fetal sacrococcygeal teratoma

Purpose

This study was designed to develop a prognostic factor for fetuses with sacrococcygeal teratoma (SCT) that may be useful to predict outcome and guide counseling early in pregnancy. We hypothesize that, in fetuses with SCT, the ratio of tumor size to estimated fetal weight in the second trimester predicts outcome.

Methods

We retrospectively reviewed charts of all patients evaluated at our Fetal Center for SCT between 2004 and 2009. Estimated fetal weight and tumor volume were calculated based on prenatal ultrasound or fetal magnetic resonance imaging. Patients were stratified based on tumor volume to fetal weight ratio (TFR), and their outcomes were analyzed by Fisher's Exact test.

Results

Tumor volume to fetal weight ratio before 24 weeks' gestation was predictive of outcome. Those with a TFR less than or equal to 0.12 (n = 5) had a significantly better outcome than patients with a TFR greater than 0.12 (n = 5, P < .05). All patients with poor outcomes had a TFR greater than 0.12 by 24 weeks' gestation. A TFR greater than 0.12 predicted poor outcome with 100% sensitivity and 83% specificity. All 4 patients who developed hydrops had a TFR greater than 0.12.

Conclusion

In our series of fetuses with SCT, TFR before 24 weeks' gestation correlates with outcome. This novel, prenatal diagnostic tool may be useful in prenatal counseling and for early identification of high-risk fetuses.     

The effect of obesity on pathological complete response and survival in breast cancer patients receiving uncapped doses of neoadjuvant anthracycline-taxane-based chemotherapy

PurposeThe effect of obesity in breast cancer patients undergoing neoadjuvant chemotherapy (NAC) remains controversial. The aim of this study was to determine the obesity-related effect on pathological complete response (pCR) and survival in women receiving full uncapped doses of NAC.MethodsWe retrospectively analyzed the data of all consecutive women who underwent anthracycline-taxane-based NAC for primary breast cancer between 2005 and 2015 at the Department of Obstetrics and Gynecology, Medical University of Vienna. Following the WHO criteria, women with a body mass index (BMI) ≥30 kg/m² at baseline were considered obese, whereas those with a BMI <30 kg/m² were considered non-obese. Those with dose reductions or dose capping were not eligible for study inclusion. Cox regression and logistic regression were performed. The Kaplan-Meier method was used to analyze disease-free, progression-free, and overall survival. The pCR served as the main outcome measure.ResultsAmong 120 women who received neoadjuvant epirubicin plus cyclophosphamide and docetaxel, 28 (23.3%) were obese and 92 (76.7%) were non-obese. In the multivariate logistic regression model that adjusted for potentially confounding variables, obesity had an independent positive predictive effect on pCR (OR 4.29, 95% CI, 1.42–13.91; p = 0.011), which was significant in the postmenopausal subgroup (OR 4.72, 95% CI, 1.47–15.84; p = 0.01). When comparing non-obese with obese women, we found that obese women experienced longer progression-free survival (HR 0.10, 95% CI, 8.448 × 10⁻⁴–0.81; p = 0.025).ConclusionsObese women receiving full uncapped doses of anthracycline-taxane-based NAC have increased pCR and favorable progression-free survival. This could result from increased dose intensity with increased efficacy and toxicity.     

Outcome following osteosynthesis or primary arthrodesis of calcaneal fractures: A cross-sectional cohort study

BackgroundCalcaneal fractures are uncommon and have a substantial impact on hindfoot function and quality of life. Several surgical treatment options are available; both in surgical approach and type of operation. The aim of this study was to compare functional outcome and quality of life following ORIF and primary arthrodesis. Furthermore, predictors of worse functional outcome were explored.MethodsA retrospective cross-sectional cohort study was performed in patients with surgical fixation of a calcaneal fracture with a minimum follow-up of 18 months. Patients received ORIF through the 1) Extended Lateral Approach (ELA), 2) Sinus Tarsi Approach (STA) or 3) primary arthrodesis via STA. Participants were presented a questionnaire containing demographics, the AOFAS hindfoot scale, Foot Function Index, SF-36, EQ-5D and patient satisfaction.ResultsIn total 95 patients participated in this study. The three groups were comparable regarding patient characteristics. A median score of 74.5 points on the AOFAS hindfoot scale and 11.9 on the FFI was found for the entire group. There were no statistically significant differences between patients with ORIF of primary arthrodesis. Patients scored a median of 49.0 on the Physical Component Scale of the SF-36 and 55.4 on the Mental Component Scale. On the EQ-5D patients scored a median of 0.8 points. Again no statistically significant differences were observed between the three subgroups. Socio-economic status was the only statistically significant predictor of worse functional outcome (β: 4.06, 95% CI: 0.50–7.62) after multivariable analysis.InterpretationGood midterm outcomes following in terms of functional outcome and in quality of life are observed. We observed no statistical significant difference in functional outcome between patients with ORIF and patients with primary arthrodesis. The only predictor of worse functional outcome is a lower socio-economic status.     

Serum neuron-specific enolase as a predictor of short-term outcome and its correlation with Glasgow Coma Scale in traumatic brain injury

Elevated serum neuron-specific enolase levels are correlated with brain cell damage. Low scores according to Glasgow Coma Scale are also considered as serious poor prognostic factor. The aims of the study were to investigate whether there is a correlation between the two measurements in patients with traumatic brain injury and whether serum neuron-specific enolase levels have potential as a screening test to predict outcome. A total of 169 consecutive patients with traumatic brain injury admitted to our clinic between 2002 and 2005 are included in this study. Those patients, who had any major health problem before trauma, were excluded from the study. However, patients with isolated head injury were included in the study. Serial serum neuron-specific enolase concentrations taken at the first 2, 24, and 48 h after traumatic brain injury were analyzed. A computed tomography was performed on each patient on admission. Their Glasgow Coma Scale scores were recorded serially. The relationship between Glasgow Coma Scale scores and the serum neuron-specific enolase levels were assessed by statistical methods. There was a significant negative correlation between the serum neuron-specific enolase levels and Glasgow Coma Scale scores. The levels of neuron-specific enolase were significantly higher in the patients who died in 30 days after trauma and whose scores were lower than or equal to 8 points in Glasgow Coma Scale. Although there are several serious limitations of the use of neuron-specific enolase as a biomarker in traumatic brain injury (i.e., hypoperfusion, extracranial trauma, bleeding, liver, or kidney damage also increase the level of neuron-specific enolase), its concentrations may be useful as a practical and helpful screening test to identify neurotrauma patients who are at increased risk and may provide supplementary estimation with radiological and clinical findings.     

Evaluation of morbidity,mortality and outcome following cervical spine injuries in elderly patients

We analysed the morbidity, mortality and outcome of cervical spine injuries in patients over the age of 65 years. This study was a retrospective review of 107 elderly patients admitted to our tertiary referral spinal injuries unit with cervical spine injuries between 1994 and 2002. The data was acquired by analysis of the national spinal unit database, hospital inpatient enquiry system, chart and radiographic review. Mean age was 74 years (range 66–93 years). The male to female ratio was 2.1:1 (M = 72, F = 35). The mean follow-up was 4.4 years (1–9 years) and mean in-hospital stay was 10 days (2–90 days). The mechanism of injury was a fall in 75 and road traffic accident in the remaining 32 patients. The level involved was atlanto-axial in 44 cases, sub-axial in 52 cases and the remaining 11 had no bony injury. Multilevel involvement occurred in 48 patients. C2 dominated the single level injury and most of them were type II odontoid fractures. Four patients had complete neurology, 27 had incomplete neurology, and the remaining 76 had no neurological deficit. Treatment included cervical orthosis in 67 cases, halo immobilization in 25, posterior stabilization in 12 patients and anterior cervical fusion in three patients. The overall complication rate was 18.6% with an associated in-hospital mortality of 11.2%. The complications included loss of reduction due to halo and Minerva loosening, non-union and delayed union among conservatively treated patients, pin site and wound infection, gastrointestinal bleeding and complication due to associated injuries. Among the 28.9% patients with neurological involvement, 37.7% had significant neurological recovery. Outcome was assessed using a cervical spine outcome questionnaire from Johns Hopkins School of Medicine. Sixty-seven patients (70%) completed the form, 20 patients (19%) were deceased at review and 8 patients (7%) were uncontactable. Functional disability was more marked in the patients with neurologically deficit at time of injury. Outcome of the injury was related to increasing age, co-morbidity and the severity of neurological deficit. Injuries of the cervical spine are not infrequent occurrence in the elderly and occur with relatively minor trauma. Neck pain in the elderly patients should be thoroughly evaluated to exclude C2 injuries. Most patients can be managed in an orthosis but unstable injuries require rigid external immobilization or surgical stabilization.     

Serial Measurement of Arterial Lactate Concentrations as a Prognostic Indicator in Relation to the Incidence of Disseminated Intravascular Coagulation in Patients with Systemic Inflammatory Response Syndrome

To demonstrate the prognostic value of measuring blood lactate concentrations and to investigate the mechanisms of lactate production in patients with systemic inflammatory response syndrome (SIRS), we conducted a prospective cohort study. Among 22 patients with SIRS, there were 9 survivors and 13 nonsurvivors. Serial arterial lactate concentrations were measured on the day of admission to the intensive care unit (day 0), then on days 1–4. The subjects of this study consisted of 14 patients with SIRS, 6 with severe sepsis, and 2 with septic shock. On admission, the lactate concentrations did not differ between the two groups, but remained high in the nonsurvivors throughout the study period, while they progressively decreased in the survivors. The incidence of disseminated intravas-cular coagulation (DIC) was significantly higher in the nonsurvivors than in the survivors. The nonsurvivors had persistently higher DIC scores and lower platelet counts than the survivors. The changes in lactate concentration over time were statistically different between the patients with DIC and those without DIC. The findings of this study clearly demonstrated that serial arterial lactate measurements can predict a poor outcome in patients with SIRS, severe sepsis, or septic shock. DIC might play an important role in the pathogenesis of lactate production in these newly defined critically ill patients. Received: March 1, 2000 / Accepted March 6, 2001     

卵巢低反应患者大剂量超排卵的助孕结局

在辅助生殖助孕中通常采用控制性卵巢刺激(controlled ovarian hyperstimulation, COS),以获得适当数量的卵子用于体外受精,既希望获得理想的辅助生殖助孕成功率,又能降低卵巢过度刺激并发症.COS中约有9%~24%[1,2]的卵巢低反应发生率.卵巢低反应者获得有效卵子数少、可移植胚胎数少或无胚胎移植、取消治疗周期率高,累计成功率更低等,导致较差的治疗结局,是辅助生殖助孕中面临的棘手问题.针对卵巢低反应者,大多数辅助生殖中心仍会采用增加促性腺激素(Gn)的起始和(或)总用量以期获得提高助孕结局的目的.而大剂量Gn是否为改善低反应患者助孕结局的有效策略却仍存在争议.     

Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome

Background/Purpose

The incidence of congenital diaphragmatic hernia (CDH) approximates 1 in 3000 births, with mortality rates up to 50%. The ability to accurately and easily predict the outcomes of these infants could be a valuable management tool. The purpose of this study was to develop and validate a simplified clinical method for predicting survival outcomes in infants born with CDH.

Methods

The Wilford Hall/Santa Rosa clinical prediction formula (WHSR_PF = highest Pao₂ − highest Pco₂) was generated from arterial blood gas values obtained during the initial 24 hours of life, but before surgical repair or extracorporeal membrane oxygenation, in a local group of infants with CDH identified by prospective and retrospective review. The WHSR_PF was validated using a comparative group from the Congenital Diaphragmatic Hernia Study Group (CHDSG). Bivariate, multivariable, and area under the receiver operating curve (AUC) analysis was performed using SigmaStat and SPSS statistical programs (SPSS, Chicago, Ill).

Results

As initially developed from the local data, the WHSR_PF had a positive predictive value (PPV) of 82%, a negative predictive value of 88% and AUC of 0.87. When validated against the CDHSG data, the positive predictive value was 83%, negative predictive value was 66%, and AUC 0.79. Area under the receiver operating curve analysis by the previously published CDHSG predictive equation was 0.76.

Conclusion

This novel formula is an easy to apply clinical tool with similar or better predictive abilities compared to previous methods of predicting survival in infants born with CDH. Currently, no method appears to have sufficient clinical accuracy for predicting the outcome of an individual infant with CDH. Further studies are indicated.     

Continuous monitoring tools for pediatric surgical outcomes: an example using biliary atresia

Purpose

Continuous monitoring tools can be used to monitor surgical outcomes over time. We illustrate the use of CUmulative SUM (CUSUM) charts in monitoring outcomes of Kasai portoenterostomy for treatment of biliary atresia at a supraregional unit.

Methods

Data on 57 consecutive infants who underwent a Kasai portoenterostomy performed by a single surgeon between June 1994 and June 2006 were collected. A procedure was defined as successful if clearance of jaundice (plasma bilirubin level <20 μmol/l) was achieved within 6 months of surgery. We applied cumulative observed-minus-expected, sequential probability ratio test (SPRT), and zero-resetting SPRT CUSUM charts and compared the results with those of standard aggregate data analyses. An expected failure rate of 43.0%, based on the national average failure rate, was used.

Results

The failure rate observed after 57 operations was 29.8%. The zero-resetting SPRT chart indicated a lower-than-expected failure rate earlier than did the aggregate data analyses and any of the other continuous monitoring techniques.

Conclusions

The CUSUM chart method provides ongoing feedback that can be used for continuous monitoring of the outcome of a procedure to ensure that standards of care are maintained. Its use as a routine monitoring tool in pediatric surgery deserves wider recognition.