Neonatal hydrocolpos presenting as a rapidly progressive abdominal mass with inferior caval vein syndrome.

A 7-week-old infant was presented at the emergency department with an abdominal mass, unilateral swelling of the groin and suspicion of an inferior caval vein syndrome with bluish discolouration and oedema of the lower extremities. Abdominal imaging showed two large cysts and profound bilateral hydronephrosis. Following laparotomy, an extreme hydrocolpos and an overdistended urinary bladder were found. These findings turned out to be secondary to a transverse vaginal septum. She was treated surgically and was hospitalised for 2 weeks. Long-term follow-up showed normalisation of previously present hypercalciuria and hydronephrosis.A hydro(metro)colpos should be considered in the differential diagnosis of a female infant presenting with an abdominal mass, to apply the appropriate investigations and therapy.

Reducing pain and distress related to needle procedures in children with cancer: A clinical practice guideline.

BACKGROUND: Children with cancer often undergo long treatment trajectories involving repeated needle procedures that potentially cause pain and distress. As part of a comprehensive effort to develop clinical practice guidelines (CPGs) to address pain prevention and management in children with cancer, we aimed to provide recommendations on the pharmacological and psychological management of procedure-related pain and distress. METHODS: Of the international inter-disciplinary CPG development panel (44 individuals), two working groups including 13 healthcare professionals focused on procedural pain and distress. Grading of Recommendations Assessment, Development and Evaluation methodology was used, including the use of systematic literature reviews to inform recommendations and the use of evidence to decision frameworks. At an in-person meeting in February 2018, the guideline panel discussed these frameworks and formulated recommendations which were then discussed with a patient-parent panel consisting of 4 survivors and 5 parents. RESULTS: The systematic reviews led to the inclusion of 48 randomised controlled trials (total number of participants = 2271). Quality of evidence supporting the recommendations ranged from very low to moderate. Strong recommendations were made for the use of topical anesthetics in all needle procedures, for offering deep sedation (DS)/general anesthesia (GA) to all children undergoing lumbar puncture, for the use of DS/ GA in major procedures in children of all ages, for the use of hypnosis in all needle procedures and for the use of active distraction in all needle procedures. CONCLUSION: In this CPG, an evidence-based approach to manage procedure-related pain and distress in children with cancer is presented. As children with cancer often undergo repeated needle procedures during treatment, prevention and alleviation of procedure-related pain and distress is of the utmost importance to increase quality of life in these children and their families.

The forthcoming DSM-5, critical care medicine, and pediatric neuropsychiatry: which new concepts do we need?

Examination and comparison of the current DSM-IV-TR and the proposed revisions for the forthcoming DSM-5, with regard to neuropsychiatric aspects of critical illness, identified five important issues. These remain to be addressed in order to improve the care of critically ill patients. These are 1) sickness behavior, as part of the organic reaction types of the brain; 2) delirium in children and the "Differential Diagnosis of Mental Disorders Due to a General Medical Condition" in children; 3) catatonia; 4) regressive disorders in childhood in relation to somatic disorders (e.g., anti-NMDAR encephalitis); 5) age-related diagnostic criteria in relation to neuro-psychiatric disorders.

Safe and effective procedural sedation for gastrointestinal endoscopy in children.

OBJECTIVE: The aim of the study was to assess, by a review of published evidence, the safest and most effective way to provide procedural sedation (PS) in children undergoing gastrointestinal endoscopy (GIE). METHODS: The databases MEDLINE, Cochrane Library, and Embase were used. Search terms "endoscopy, gastrointestinal" or "endoscopy, digestive system" were combined with "sedation," "conscious sedation," "moderate sedation," "deep sedation," and "hypnotics and sedatives." The final review was restricted to studies reporting specifically on safety (incidences of adverse events) and/or effectiveness (time characteristics, need for supplemental sedation, need for restraint, procedural success, provider satisfaction, and patient comfort) of PS for GIE in children younger than 18 years. RESULTS: The search yielded 182 references and the final selection included 11 randomized controlled trials (RCTs) and 15 non-RCTs. Six sedation categories were identified: propofol, opioid/benzodiazepine, premedication, ketamine-, sevoflurane-, and midazolam-based. Only a few RCTs have compared different categories. Opioid/benzodiazepine- and propofol-based PS have a similar safety profile and a low incidence of major adverse events. Propofol-based sedation turned out to be the most effective regimen, with effectiveness comparable to general anesthesia. The addition of midazolam, fentanyl, remifentanil, and/or ketamine to propofol may increase the effectiveness without creating more adverse events. Data on midazolam-, ketamine- and sevoflurane-based sedation were generally too limited to draw conclusions. CONCLUSIONS: Despite a lack of RCTs containing all aspects of effectiveness and safety, the present evidence indicates propofol-based PS to be the best practice for PS in children undergoing GIE. Propofol can be safely administered by specifically trained nonanesthesiologists.

On the utility of diagnostic instruments for pediatric delirium in critical illness: an evaluation of the Pediatric Anesthesia Emergence Delirium Scale, the Delirium Rating Scale 88, and the Delirium Rating Scale-Revised R-98.

PURPOSE: Delirium is a poor-prognosis neuropsychiatric disorder. Pediatric delirium (PD) remains understudied, particularly at pediatric intensive care units (PICU). Although the Pediatric Anesthesia Emergence Delirium (PAED) scale, the Delirium Rating Scale (DRS-88), and the Delirium Rating Scale-Revised (DRS-R-98) are available, none have been validated for use in PICU settings. The aim of the present study was to investigate the use of the DRS/PAED instruments as diagnostic tools for PD in the PICU. METHODS: A prospective panel study was conducted, under circumstances of routine clinical care, investigating the diagnostic properties of the PAED, DRS-88, and DRS-R-98 in PICU patients at a tertiary university medical center. A total of 182 non-electively admitted, critically ill pediatric patients, aged 1-17 years, were included between November 2006 and February 2010. Sensitivity, specificity, and receiver operating characteristic (ROC) curves were calculated. Three psychometric properties were analyzed: (1) internal consistency (2) proportion of items not rateable, and (3) discriminative ability. RESULTS: The PAED could be completed in 144 (93.5%) patients, much more frequently than either the DRS-88 (66.9%) or the DRS-R-98 (46.8%). Compared with the clinical gold standard diagnosis of delirium, the PAED had a sensitivity of 91% and a specificity of 98% (AUC 0.99). The optimal PAED cutoff score as a screening instrument in this PICU setting was 8. Cronbach's alpha was 0.89; discriminative ability was high. CONCLUSIONS: The PAED is a valid instrument for PD in critically ill children, given its reliance on routinely rateable observational signs and symptoms.

Professional skills and competence for safe and effective procedural sedation in children: recommendations based on a systematic review of the literature.

Objectives. To investigate which skills and competence are imperative to assure optimal effectiveness and safety of procedural sedation (PS) in children and to analyze the underlying levels of evidence. Study Design and methods. Systematic review of literature published between 1993 and March 2009. Selected papers were classified according to their methodological quality and summarized in evidence-based conclusions. Next, conclusions were used to formulate recommendations. Results. Although the safety profiles vary among PS drugs, the possibility of potentially serious adverse events and the predictability of depth and duration of sedation define the imperative skills and competence necessary for a timely recognition and appropriate management. The level of effectiveness is mainly determined by the ability to apply titratable PS, including deep sedation using short-acting anesthetics for invasive procedures and nitrous oxide for minor painful procedures, and the implementation of non-pharmacological techniques. Conclusions. PS related safety and effectiveness are determined by the circumstances and professional skills rather than by specific pharmacologic characteristics. Evidence based recommendations regarding necessary skills and competence should be used to set up training programs and to define which professionals can and cannot be credentialed for PS in children.

Fatal cerebral edema associated with serine deficiency in CSF.

Two young girls without a notable medical history except for asthma presented with an acute toxic encephalopathy with very low serine concentrations both in plasma and cerebrospinal fluid (CSF) comparable to patients with 3-phosphoglycerate dehydrogenase (3-PGDH) deficiency. Clinical symptoms and enzyme measurement (in one patient) excluded 3-PGDH deficiency. Deficiencies in other serine biosynthesis enzymes were highly unlikely on clinical grounds. On basis of the fasting state, ketone bodies and lactate in plasma, urine and CSF, we speculate that reduced serine levels were due to its use as gluconeogenic substrate, conversion to pyruvate by brain serine racemase or decreased L-serine production because of a lack of glucose. These are the first strikingly similar cases of patients with a clear secondary serine deficiency associated with a toxic encephalopathy.

Prolonged stay at the paediatric intensive care unit associated with paediatric delirium.

The objective of this study was to investigate, under circumstances of routine care, the impact of paediatric delirium (PD) on length of stay in the paediatric intensive care unit (PICU) as well as on direct financial costs. A five-year prospective observational study (2002-2007) was carried out in a tertiary eight-bed PICU in the Netherlands. Critically ill children aged 1 to 18 years who were acutely, non-electively and consecutively admitted to the PICU and detected as having PD in routine care were compared to critically ill children aged 1 to 18 years without signs of PD. PD, population characteristics and severity of illness at admission were used as predictors for length of PICU stay. Differences in length of stay yielded short-term, direct medical costs associated with PD. Forty-nine children with and 98 children without PD were included. PD prolonged length of PICU stay with 2.39 days, independent of severity of illness, age, gender, mechanical ventilation and medical indication for admission (B = 0.38, P < 0.001). PD increased direct medical costs with 1.5%. The results suggest a negative prognostic influence of PD on duration of PICU stay in routine care, resulting in an increase of direct medical costs.

Vanishing spleen after Nissen fundoplication: a case report.

Nissen fundoplication is a generally accepted treatment for severe gastro-oesophageal reflux after conservative management has failed. The surgical techniques and the complications that may develop following the operation have been well described. However, necrosis of the spleen is a rare complication. We report here a patient with Down syndrome with a vanishing spleen after a Nissen fundoplication, who died of overwhelming pneumococcal septic shock 7 months after the operation. Vascular anomaly in Down syndrome, inadvertent ligation of the splenic artery or volvulus of the spleen may have caused a compromised splenic arterial circulation.Conclusion Nissen fundoplication may be associated with vanishing spleen and, consequently, with devastating consequences.

Pediatric illness severity measures predict delirium in a pediatric intensive care unit.

CONTEXT: Delirium in children is a serious but understudied neuropsychiatric disorder. So there is little to guide the clinician in terms of identifying those at risk. OBJECTIVE: To study, in a pediatric intensive care unit (PICU), the predictive power of widely used generic pediatric mortality scoring systems in relation to the occurrence of pediatric delirium (PD). DESIGN AND METHODS: Four-year prospective observational study, 2002-2005. Predictors used were the Pediatric Index of Mortality (PIM) and Pediatric Risk of Mortality (PRISM II). SETTING: A tertiary 8-bed PICU in The Netherlands. PATIENTS: 877 critically ill children who were acutely, nonelectively, and consecutively admitted. MAIN OUTCOME MEASURE: Pediatric delirium. MAIN RESULTS: Out of 877 children with mean age 4.4 yrs, 40 were diagnosed with PD (Cumulative incidence: 4.5%), 85% of whom (versus 40% with nondelirium) were mechanically ventilated. The area under the curve was 0.74 for PRISM II and 0.71 for the PIM, with optimal cut-off points at the 60th centile (PRISM: sensitivity: 76%; specificity: 62%; PIM: sensitivity: 82%; specificity: 62%). A PRISM II or PIM score above the 60th centile was strongly associated with later PD in terms of relative risk (PRISM II: risk ratio = 4.9; 95% confidence interval: 2.3-10.1; PIM: RR = 6.7; 95% confidence interval: 3.0-15.0). Given the low incidence of PD, values for positive predictive value were lower (PRISM II: 8.3%; PIM: 8.9%, rising to, respectively, 10.1% and 10.6% in mechanically ventilated patients) and values for negative predictive value were higher (PRISM II: 98.3%; PIM: 98.7%). LIMITATIONS: Given the relatively low incidence of delirium, a low detection rate biased toward the most severe cases cannot be excluded. CONCLUSIONS: Given the fact that PIM and PRISM II are widely used mortality scoring instruments, prospective associations with PD suggest additional value for ruling in, or out, patients at risk of PD.

Pediatric delirium in critical illness: phenomenology, clinical correlates and treatment response in 40 cases in the pediatric intensive care unit.

OBJECTIVE: To study the phenomenology, clinical correlates, and response to treatment of delirium in critically ill children in the pediatric intensive care unit (PICU). DESIGN, SETTING AND PATIENTS: Descriptive study of a cohort of child psychiatric consultations from a tertiary PICU between January 2002 and December 2005. Demographic data, clinical presentation, and response to treatment of children subsequently diagnosed with delirium were analyzed. RESULTS: Out of 877 admissions (age distribution 0-18 years) arose 61 requests for psychiatric assessment. Of the 61 children, 40 (15 girls and 25 boys) were diagnosed with delirium (cumulative incidence 5%; mean age 7.6 years). Age-specific incidence rates varied from 3% (0-3 years) to 19% (16-18 years). In addition to the classical hypoactive and hyperactive presentations, a third presentation was apparent, characterized mainly by anxiety, with a higher prevalence in boys. All but 2 of the 40 children received antipsychotic medication: 27 (68%) haloperidol, 10 (25%) risperidone, and 1 both in succession. Two children treated with haloperidol experienced an acute torticollis as side effect. All children made a complete recovery from the delirium; five, however, died of their underlying disease. CONCLUSION: The rate of delirium in critically ill children on a PICU is not negligible, yet prospective studies of the phenomenology, risk factors and treatment of childhood delirium are very rare. Once pediatric delirium has been recognized, it generally responds well to treatment.