Refractory perirenal effusion in a pediatric patient with antithrombin III deficiency and KILT syndrome: a case report

Fatal Outcome of Recombinant Factor VIIa in Heart Transplantation With Extracorporeal Membrane Oxygenation

Reports of Death with Use of Propofol (Diprivan) for Nonprocedural (Long-term) Sedation and Literature Review

Peter J. Davis, MD Appearing in this issue of the journal are four clinical papers on the use of dexmedetomidine in children (1–4). Previous studies of the use of this drug in children have dealt with sedation in the intensive care unit (5), prevention of postoperative agitation associated with sevoflurane anesthesia (6,7), and sedation for noninvasive procedures (8–12). These articles also illustrate a number of subtle concerns that are relevant to drug development in children and the ethical principles that guide research in human subjects as proposed by the National Commission for the Protection of Human Subjects of Biomedical and Behavioral Research, and summarized in the Belmont Report. In a randomized study of 30 pediatric cardiac surgical patients anesthetized with isoflurane and undergoing cardiopulmonary bypass (CPB), Mukhtar et al. (1) evaluated the effects of a continuous dexmedetomidine infusion on patients’ underlying hemodynamics and neuroendocrine system compared with patients receiving a saline control infusion. The authors infused dexmedetomidine after anesthetic induction until the termination of CPB. Dexmedetomidine attenuated the increase in the neuroendocrine markers of stress caused by sternotomy and CPB. In addition, dexmedetomidine provided better intraoperative hemodynamic stability in children with congenital heart disease. The report focused on hemodynamic outcome and did not address how this drug affected tracheal extubation time, recovery, length of hospital stay, or long-term outcome. Thus, the reader is left to ponder whether the reduction in the cortisol and norepinephrine, markers of neuroendocrine response, affect outcome. Moreover, we do not know how to titrate dexmedetomidine or if the optimum dose was administered. Are there dexmedetomidine doserelated adverse effects in this population? Should the dose be altered at the start of CPB? What is the effect of the fluctuation in the patient’s temperature (37° to 25° and return) on plasma levels achieved using a standard infusion rate? Why was dexmedetomidine not continued until the end of surgery? Why did the dexmedetomidine infusion not allow for less anesthetic administration, as had been shown in an adult study (13)? Did dexmedetomidine improve hemodynamic stability after bypass? Although this study measured biochemical endpoints to assess stress response, can we infer that the effect of this drug will be as salutary as the effect of opioids in the mortality of children having surgery for congenital heart disease? These questions were not answered because the study was not funded, limiting the number of subjects and the resources available to the investigators. Had this study been funded by the pharmaceutical manufacturer, the authors could have enrolled more patients, perhaps included other study centers, assayed blood for dexmedetomidine concentrations, developed pharmacokinetic models of dexmedetomidine in this population, and performed a pharmacodynamic concentration-versus-response analysis. In a study of 60 children undergoing magnetic resonance imaging, From the Department of Anesthesiology, Wake Forest University School of Medicine, Winston-Salem, North Carolina; Department of Anesthesia, Stanford University, Stanford, California; Department of Anesthesiology, University of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania. Accepted for publication March 22, 2006. Address correspondence and reprint requests to Peter J. Davis, MD, Professor of Anesthesia and Pediatrics Anesthesiologistin-Chief, University of Pittsburgh School of Medicine, Children’s Hospital of Pittsburgh, Pittsburgh, PA 15213. Address e-mail to davispj@anes.upmc.edu. Copyright © 2006 International Anesthesia Research Society DOI: 10.1213/01.ANE.0000220033.34889.CE

Background and objectives: Pelvic inflammatory disease (PID), primarily associated with sexually transmitted infections (STIs), represents a diagnostic challenge in virgin pediatric patients due to the often vague, non-specific symptomatology, which can mimic other conditions. Management prioritizes targeted antimicrobial therapy, with surgical intervention reserved for complications like tubo-ovarian abscess (TOA). The present systematic review aimed to critically evaluate the available evidence from case reports of PID in virgin pediatric and adolescent patients. Methods: The search strategy was in accordance with PRISMA guidelines. Case reports published up to March 2025 were searched through PubMed, Embase, Scopus, and Google Scholar databases. We included English-language case reports on non-sexually active pediatric and adolescent patients with available full text, excluding commentaries, reviews, and editorials. The Critical Appraisal Checklist for Case Reports was used for the quality assessment of case reports. Through descriptive analysis, PID symptoms, diagnostic, and management modalities were reviewed. The quality of the included case reports was assessed using the JBI Critical Appraisal Checklist. This review was not registered and did not receive external funding. Results: Among the 56 case reports searched, 20 reports were selected and analyzed based on eight criteria. The most frequently reported symptoms were lower abdominal pain (95.8%), fever (63.6%), and gastrointestinal symptoms (50%). Common comorbidities included urinary tract infections (22.7%), congenital anomalies (18.1%), and appendicitis history (18.1%). Escherichia coli and Streptococcus species were the predominant pathogens identified. All patients received antibiotic therapy, while 90.9% underwent surgical intervention. Favorable outcomes were achieved in 72.7% of cases, though 27.2% experienced complications or recurrences. Conclusions: Although commonly linked to sexual activity, PID should be considered in sexually inactive pediatric patients presenting with abdominal pain and adnexal masses. Early diagnosis, appropriate imaging, and timely treatment are crucial to improve outcomes and reduce complications. The evidence in this review is limited by its reliance on case reports, which may introduce bias and restrict generalizability.

Human Immunodeficiency Virus–Associated Cerebral Aneurysmal Vasculopathy: A Systematic Review

Clostridium difficile is the leading cause of nosocomial gastrointestinal illness in adult patients in hospitals. Even though C. difficile disease in adults has been well studied, research on pediatric C. difficile disease is still in its infancy. For many years it has been believed that C. difficile was a disease that affected only adults and was not a problem for children. This erroneous belief arose from the observation that neonates acquire C. difficile quickly (within 48 hours of birth) but show no intestinal symptoms (1–4). More recent evidence has been documented in case reports of pediatric C. difficile and outbreaks of C. difficile disease in pediatric populations (5– 7). Pediatric C. difficile disease has also been associated with the occurrence of severe complications and high mortality rates (8–10). The cascade of events in the pathogenesis of C. difficile disease is similar in children and adults. Normal intestinal microflora are disrupted by antibiotic exposure, medications, or surgery. If the child is then exposed to C. difficile (or its spores), colonization may occur, with production of toxins A and B. These toxins act on enterocytes, causing an inflammatory response and morphologic changes that lead to diarrhea or colitis. Host factors (age, diet, immune response) play an important role in determining whether C. difficile develops into asymptomatic carriage or active disease. Treatment for pediatric C. difficile disease usually relies on metronidazole or vancomycin, but clinical guidelines have not been defined for the pediatric population (11). As in adults, recurrent C. difficile disease that does not respond to conventional therapy develops in a proportion of children treated with antibiotic therapy.

Haemorrhage is a leading cause of death in paediatric trauma patients. Predefined massive transfusion protocols (MTP) have the potential to significantly reduce mortality by treating haemorrhagic shock and coagulopathy, in adhering to the principles of haemostatic resuscitation with rapid administration of balanced ratios of packed red blood cells (RBC), fresh frozen plasma (FFP) and platelets (PLT).Because of their substantial physiological reserve, initial vital signs may not be good predictors of early haemorrhage in paediatric patients. Determining the triggers for MTP activation in paediatric trauma patients is challenging, and the optimal blood product ratio that will increase survival in massively bleeding paediatric trauma patients has yet to be determined. To date, only a few small descriptive studies and case reports have investigated the use of predefined MTP in paediatric trauma patients.MTP with increased FFP or PLT to RBC ratios combined with viscoelastic haemostatic assay (VHA) guided haemostatic resuscitation have not yet been tested in paediatric populations but based on results from adult trauma patients, this therapeutic approach seems promising.Considering the high prevalence of early coagulopathy in paediatric trauma patients, immediate identification and implementation of VHA-directed treatment of traumatic coagulopathy could ensure faster haemostasis and thereby, potentially, reduce bleeding as well as the total transfusion requirements and further improve outcome in paediatric trauma patients. Prospective randomized trials investigating this therapeutic approach in paediatric trauma patients are highly warranted.

Indications, Protocols, and Outcomes of Drug Desensitizations for Chemotherapy and Monoclonal Antibodies in Adults and Children

Balloon by balloon, inch by inch

Arnold Chiari malformation (ACM) is the most common cause of central sleep apnea (CSA) in otherwise healthy children. Although there are several case reports and series reported on this topic, there are limited descriptions of the long-term course of these children after the surgical interventions. Posterior fossa decompression surgery to relieve pressure of the herniating cerebellum on the brainstem is generally thought to significantly improve CSA in most cases, however, there are very limited data on the natural course of CSA in children following decompression surgery. There may be a subset of children in whom it may take much longer for CSA to resolve, and in some it may not resolve completely. Hence, these children need to be followed closely with sleep studies to document resolution of CSA. In this case report, we describe a 10-year-old male with severe CSA who was subsequently diagnosed with type 1 ACM and underwent posterior fossa decompressive surgery. However after surgery, although there was improvement in his CSA, he still had a significant degree of residual CSA which required bilevel positive pressure therapy and took more than 7.5 years to resolve. This case report illustrates the need for close follow-up in these children and for providers to understand the natural course so they can accurately counsel families about expectations after surgical treatments.

Background:The pediatric lung transplant is a very important treatment for patients with end-stage lung diseae, and pulmonary rehabilitation (PR) is also an important factor in determining the prognosis. However, there is no much literature available on pulmonary rehabilitation in pediatric patients’ post lung transplant. Through this case report, we would like to present our intensive PR program for pediatric patients’ post-lung transplant.Patient concerns:The 10-year-old boy's breathing before receiving a lung transplant continued to deteriorate and he eventually became dependent on a wheelchair.Diagnosis:He was diagnosed with infantile acute lymphoblastic leukemia at 6 months of age. At the age of one year, he underwent allogeneic bone marrow transplantation, but was diagnosed with post-transplantation bronchiolitis obliterans (PTBO) two months later. He had a lung transplant at the age of 10.Interventions:He was hospitalized and received an initial assessment. This assessment included functional, cognitive, and psychological evaluations. He additionally completed PR exercises twice daily for two weeks. After discharge, he continued to participate in an outpatient-based PR program for three months. During the outpatient phase, PR exercises were performed once weekly, in addition to home-based cognitive training.Outcomes:Our intensive post-lung PR program improved our patient's exercise capacity, lung function, and quality of life. As a comprehensive rehabilitation service, our program also included a cognitive training component.Conclusion:We describe an intensive PR program tailored to pediatric patients’ post-lung transplant. The program was feasible and resulted in improvements in functional exercise capacity, lung function, and quality of life. Future research into our method is necessary for continued improvement of this novel program.

Inflammatory bowel disease (IBD) is a chronic autoimmune condition that can have a wide range of symptoms among pediatric patients. Although clinical symptoms like hematochezia, diarrhea, and abdominal pain are commonly addressed, health-related quality of life (HRQOL) is often overlooked in patients with IBD and pediatric patients with chronic disease in general. Examining HRQOL can help improve patient outcomes, but it has been studied sparingly. In this review, we aim to compare HRQOL between pediatric patients suffering from IBD and healthy children, as well as those suffering from other illnesses. We searched through peer-reviewed primary literature related to IBD and HRQOL and selected 10 articles from the PubMed database to be reviewed. Our inclusion criteria included articles published after the year 2000 in English, primary studies, and those that corresponded to the aim of this review. Case reports and secondary and tertiary articles were excluded from our review. We found that patients with IBD reported worse HRQOL in terms of overall health and in various subdomains, including physical health and fatigue, compared to their healthy counterparts. However, children with IBD demonstrated a comparable HRQOL with children suffering from functional abdominal pain (FAP) and obesity. Additionally, children with IBD displayed a greater HRQOL than pediatric patients with gastroesophageal reflux disease (GERD) and chronic constipation. In addressing the aim of this review, we found that children with IBD had a lower HRQOL when compared to healthy children, but a comparable or greater HRQOL than other sick children. Some factors associated with a reduced HRQOL include disease activity, age, fatigue, gender, psychological variables, and associated symptoms. Going forward, HRQOL should be considered by practitioners when caring for pediatric IBD patients in a clinical setting as it can help improve patient care. More studies need to be conducted to further explore HRQOL in pediatric patients. This can help implement early psychosocial interventions in children to reduce the disease burden.

Central diabetes insipidus (DI) is frequently identified preoperatively and/or postoperatively in patients with sellar or parasellar lesions. Early diagnosis and effective perioperative management of central DI is critical to minimize disruptions in fluid homeostasis. In particular, although venous thromboembolism (VTE) is generally less common in pediatric patients than their adult counterparts, isolated reports suggest that VTE occurs at a higher frequency in pediatric patients with central DI. Using the PubMed, Scopus, and Springer Link databases, the authors performed a systematic review of the literature with regard to the incidence of VTE in pediatric patients with central DI. Inclusion criteria were availability of the full text in English, diagnosis of central DI and VTE in the same patient, and pediatric age defined as ≤ 21 years. Data were reported as median and interquartile range for continuous variables and as frequencies and percentages for categorical variables. Risk of bias assessments of the individual studies were performed using the Joanna Briggs Institute Critical Appraisal Checklists for case series and case reports. Of 2094 search results, 12 articles met the inclusion criteria and described a total of 17 cases of VTE in pediatric patients with central DI. Two additional patients from the authors' institution were added to this cohort. The underlying pathologies included craniopharyngioma (n = 6), suprasellar germinoma (n = 4), epileptic encephalopathy (n = 2), pilocytic astrocytoma (n = 2), prolactinoma (n = 2), Cushing disease (n = 1), failure to thrive (n = 1), and congenital hypothalamic syndrome (n = 1). Thrombotic complications included deep vein thrombosis (n = 10 [53%]), cerebral venous sinus thrombosis (n = 6 [32%]), pulmonary embolism (n = 4 [21%]), inferior vena cava thrombosis (n = 2 [11%]), and disseminated intravascular coagulation (n = 1 [5%]). There was a 26% mortality rate. VTE is a rare but potentially devastating postoperative complication that appears to have a higher incidence among patients with central DI. Although this review was limited by heterogeneous information across limited reports, pediatric neurosurgical patients with DI may benefit from more aggressive VTE surveillance and prophylaxis.

Although it is generally agreed upon that surgery for high-grade spondylolisthesis (HGS) is associated with more complications than low-grade spondylolisthesis, its description is primarily based on case reports and relatively small case series. To assess short-term complication rates associated with the surgical treatment of HGS in pediatric and adult patients and to identify factors associated with increased complication rates. All cases of HGS from the Scoliosis Research Society Morbidity and Mortality database for the year 2007 were reviewed. Patients were classified as pediatric (≤18 years) or adult (>18 years). Complications were tabulated, and the rates were compared between the patient groups and based on clinical and surgical factors. 165 cases of HGS were reported (88 pediatric, 77 adult). There were 49 complications (29.7%) in 41 patients (24.8%), with no difference in the proportions of pediatric vs adult patients with a complication (P = .86). Occurrence of new neurological deficit after surgery was the most common complication, seen in 19 (11.5%) patients. Performance of an osteotomy was associated with a higher incidence of new neurological deficits in both adult and pediatric groups (P = .02 and P = .012, respectively). Although most of the new neurological deficits improved over follow-up, 10% had no improvement. This study provides short-term complication rates associated with surgical treatment for HGS in adult and pediatric patients and may prove valuable for patient counseling, surgical planning, and in efforts to improve the safety of patient care.

BackgroundIt remains unclear whether transcatheter arterial embolisation (TAE) is as safe and effective for paediatric patients with blunt torso trauma as it is for adults in Japan, owing to few trauma cases and sporadic case reports. The study aimed to compare the efficacy and safety of TAE performed in paediatric (age ≤ 15 years) and adult patients with blunt torso trauma.MethodsThis was a single-centre, retrospective chart review study that included blunt torso trauma patients who underwent TAE in the trauma centre from 2012 to 2017. The comparative study was carried out between a ‘paediatric patient group’ and an ‘adult patient group’. The outcome measures for TAE were the success of haemorrhage control and complications and standardised mortality ratio (SMR).ResultsA total of 504 patients with blunt torso trauma were transported to the trauma centre, out of which 23% (N = 114) with blunt torso trauma underwent TAE, including 15 paediatric and 99 adult patients. There was no significant difference between the use of TAE in paediatric and adult patients with blunt torso trauma (29% vs 22%, P = .221). The paediatric patients’ median age was 11 years (interquartile ranges 7–14). The predicted mortality rate and SMR for paediatric patients were lower than those for adult patients (18.3% vs 25.9%, P = .026, and 0.37 vs 0.54). The rate of effective haemorrhage control without repeated TAE or additional surgical intervention was 93% in paediatric patients, which was similar to that in adult patients (88%). There were no complications in paediatric patients at our centre. There were no significant differences in the proportion of paediatric patients who underwent surgery before TAE or urgent blood transfusion (33% vs 26%, P = .566, or 67% vs 85%, P = .084).ConclusionsIt is possible to provide an equal level of care related to TAE for paediatric and adult patients as it relates to TAE for blunt torso trauma with haemorrhage in the trauma centre. Alternative haemorrhage control procedures should be established as soon as possible whenever the patients reach a haemodynamically unstable state.