Surgical Dilemma: Exomphalos Major with Accessory Liver Lobe and Congenital Liver Cysts.

The association of exomphalos major, congenital liver cysts, and an accessory lobe of the liver is very rare. There are only two previously reported cases in the literature, both describing surgical excision of the accessory lobe with liver cysts during primary closure of the exomphalos defect. We report a case of this rare association, managed by delayed primary closure, where the cysts underwent spontaneous regression. This case, along with those previously reported, supports the etiopathogenesis theory of a malformative sequence of exomphalos and hepatic trauma within the sac of this rare association. Spontaneous regression of these cysts would favor a delayed primary closure in such cases.

Caput membranaceum: A novel clinical presentation of ZIC1 related skull malformation and craniosynostosis.

We report clinical and radiological features of a patient born with an isolated skull malformation of caput membranaceum and partial bicoronal craniosynostosis with a novel, de novo heterozygous missense variant in ZIC1 [NM_003412.3:c.1183C>G, p.(Pro395Ala)]. Caput membranaceum, or boneless skull, is a rare manifestation of skull ossification defect. It can result from an isolated, enlarged parietal foramina or it can present as part of skeletal dysplasia syndromes associated with poor mineralization such as hypophosphatasia, osteogenesis imperfecta type II, and Saethre-Chotzen syndrome. Their causative genes are well described. ZIC1, Zinc Finger protein of the cerebellum 1 (OMIM #600470) belongs to ZIC family genes, each encoding a Cys2 His2-type zinc finger domain-containing transcription factors. Recent studies have shown that pathogenic variants in ZIC1 have deleterious effect in developing human central nerves system and skull bone. ZIC1 related clinical conditions are reported and include cerebellum malformation, Dandy-Walker malformation, spinal dysraphism, microcephaly, and craniosynostosis with associated intellectual disability. To-date, there is no report of pathogenic variant in ZIC1 causing isolated caput membranaceum. Our observation adds to the clinical spectrum of ZIC1 related skull malformation.

A review of filamin A mutations and associated interstitial lung disease.

The filamin A gene (FLNA) on Xq28 encodes the filamin A protein. Mutation in FLNA causes a wide spectrum of disease including skeletal dysplasia, neuronal migration abnormality, cardiovascular malformation, intellectual disability and intestinal obstruction. Recently, childhood-onset interstitial lung disease associated with a range of FLNA mutations has been recognised and reported. We document our personal experience of this emerging disorder and compile a comprehensive overview of clinical features and molecular changes in all identifiable published cases. Reviewing the emerging dataset, we underline this unanticipated phenotypic consequence of pathogenic FLNA mutation-associated pulmonary disease.Conclusion: From the emerging data, we suggest that while reviewing complex cases with a sustained oxygen requirement against a clincial background of cardiac concerns or intestinal obstruction to have a high index of suspicion for FLNA related pathology and to instigate early MRI brain scan and FLNA mutation analysis. What is Known: • FLNA gene on Xq28 encodes the filamin A protein and mutation therein is associated with variable phenotypes depending on its nature of mutation. • Loss-of-function mutation of filamin A is associated with X-linked inherited form of periventricular nodular heterotopia with or without epilepsy with most individuals affected being female. There is a recently recognised associated respiratory phenotype. What is New: • The respiratory phenotype in the form of childhood interstitial lung disease is a recently recognised clinical consequence of loss-of-function FLNA mutation. • Rare male patients with loss-of-function FLNA mutation-associated lung disease with residual protein function can survive into infancy with a severe form of the phenotype.

Predictors of emotional distress a year or more after diagnosis of cancer: A systematic review of the literature.

OBJECTIVE: Why some people recover emotionally after diagnosis and treatment of cancer and others do not is poorly understood. To identify factors around the time of diagnosis that predict longer-term distress is a necessary step in developing interventions to reduce patients' vulnerability. This review identified the demographic, clinical, social, and psychological factors available at or within 3 months of diagnosis that are reliable predictors of emotional distress at least 12 months later. METHODS: A systematic search of literature for prospective studies addressing our research question and predicting a range of distress outcomes was conducted. Thirty-nine papers (reporting 36 studies) were subjected to narrative synthesis of the evidence. RESULTS: There was no consistent evidence that demographic, clinical, or social factors reliably predicted longer-term distress. Of the psychological factors examined, only baseline distress (significant in 26 of 30 relevant papers; 24 of 28 studies) and neuroticism (significant in all 5 papers/studies that examined it) consistently predicted longer-term distress. The heterogeneity of included studies, particularly in populations studied and methodology, precluded meta-analytic techniques. CONCLUSIONS: This review supports current clinical guidance advising early assessment of distress as a marker of vulnerability to persistent problems. Additionally, neuroticism is also indicated as a useful marker of vulnerability. However, the review also highlights that more sophisticated research designs, capable of identifying the psychological processes that underlie the association between these marker variables and persistent distress, are needed before more effective early interventions can be developed.

Umbilical Venous Catheter Position: The Value of Acquiring a Lateral in Addition to a Frontal Chest Radiograph.

Introduction Umbilical venous catheters (UVCs) are standardly used for central venous access in acutely sick neonates. Complications associated with UVCs include thrombosis, infection, diffuse intravascular coagulopathy, arrhythmia, tamponade, and liver injury, many of which are related to misplacement of the catheters. Therefore, this study aimed to institute a policy of obtaining lateral and frontal radiographs to improve the determination of the UVC position. Methods We retrospectively reviewed UVC placement from 132 radiographs. We compared interpretations by different reviewers of frontal versus frontal and lateral chest radiographs for the most accurate determination of the UVC position. The reviewers completed questionnaires indicating their assessment of the catheter tip position, as well as the appropriate catheter manipulation required for optimal positioning. Their assessment was derived from frontal chest radiographs followed by frontal plus lateral view radiographs a week later. Results The reviewers (junior neonatology fellow, senior neonatology fellow, pediatric radiology fellow, and senior pediatric radiologist) revised their assessment with regard to the UVC positioning between frontal and frontal plus lateral radiographs in 24.6%, 22.7%, 19.6%, and 15.9% of cases, respectively, and indicated that the lateral view was helpful in 18%, 13.6%, 19.6%, and 31% of the cases, respectively. UVCs were placed appropriately at the first attempt in only 13.6% of the cases. Conclusion Correct initial placement of a UVC is uncommon. A lateral radiograph is beneficial in determining the UVC position. Hence, we suggest the inclusion of a lateral view along with the frontal chest radiograph for the evaluation of the UVC position if real-time ultrasound cannot be performed before UVC usage.

Impact on the incidence of suspected physical abuse in children under 24 months of age during a global pandemic: A multi-centre Irish regional retrospective cross-sectional analysis.

OBJECTIVES: The advent of the COVID-19 pandemic has resulted in periods of nationwide restrictions in Ireland including school and workplace closures. The authors hypothesised that this disruption to society may have led to a change in patterns of suspected physical abuse (SPA) presentations to the paediatric emergency department (ED), whilst ED attendance fell dramatically during the period. We reviewed data to determine whether there was an increase in presentations of SPA during periods of social restrictions. METHODS: The National Integrated Medical Imaging Service was searched for all skeletal survey examinations performed between the dates of the 1 March 2016 and 28 Feb 2021 for studies performed in cases of SPA. Electronic records of attendance were extracted from the emergency department administrative system at the three paediatric emergency departments which serve the 400,000 children regionally. The data were reviewed to determine if SPA presentations increased during restriction periods. RESULTS: 311 individual paediatric patients aged 24 months and under were referred for SPA skeletal survey during the study period. During the 2020/2021 period, 60 children were referred for SPA workup and there was no statistically significant difference between monthly referrals (mean 5, sd 2.92) in this period and matched periods over the preceding 4 years (mean 5.23, sd 2.69). CONCLUSIONS: The incidence of SPA did not increase during the period of national restrictions during the COVID-19 pandemic. ADVANCES IN KNOWLEDGE: Periods of social restrictions taken to protect the public health during a pandemic do not result in short term increases in suspected physical abuse in the regional paediatric population.

Relationship Between MRI Scoring Systems and Neurodevelopmental Outcome at Two Years in Infants With Neonatal Encephalopathy.

BACKGROUND: Magnetic resonance imaging (MRI) scoring systems are used in the neonatal period to predict outcome in infants with neonatal encephalopathy. Our aim was to assess the relationship between three MRI scores and neurodevelopmental outcome assessed using Bayley Scales of Infant and Toddler Development, third edition (Bayley-III), at two years in infants with neonatal encephalopathy. METHODS: Term-born neonates with evidence of perinatal asphyxia born between 2011 and 2015 were retrospectively reviewed. MRI scanning was performed within the first two weeks of life and scored using Barkovich, National Institute of Child Health and Human Development (NICHD) Neonatal Research Network (NRN), and Weeke systems by a single assessor blinded to the infants clinical course. Neurodevelopmental outcome was assessed using composite scores on the Bayley-III at two years. Multiple linear regression analyses were used to assess the association between MRI scores and Bayley-III composite scores, with postmenstrual age at scan and sex included as covariates. RESULTS: Of the 135 recruited infants, 90 infants underwent MRI, and of these, 66 returned for follow-up. MRI abnormalities were detected with the highest frequency using the Weeke score (Barkovich 40%, NICHD NRN 50%, Weeke 77%). The inter-rater agreement was good for the Barkovich score and excellent for NICHD NRN and Weeke scores. There was a significant association between Barkovich, NICHD NRN, and Weeke scores and Bayley-III cognitive and motor scores. Only the Weeke score was associated with Bayley-III language scores. CONCLUSIONS: Our findings confirm the predictive value of existing MRI scoring systems for cognitive and motor outcome and suggest that more detailed scoring systems have predictive value for language outcome.

Diffusion-weighted imaging and pathological correlation in pediatric medulloblastomas-"They are not always restricted!".

INTRODUCTION: Some investigators have suggested that medulloblastomas can be distinguished from other cerebellar neoplasms by demonstrating "restricted diffusion" on the Apparent Diffusion Coefficient (ADC) map obtained from diffusion-weighted imaging (DWI) sequences on magnetic resonance imaging. Previous authors have postulated that this observed restricted diffusion is a reflection of very high cell density. There has been a tendency to assert that pediatric medulloblastoma uniformly demonstrates restricted diffusion on DWI. However, our clinical observation has been that there are pediatric medulloblastomas that exhibit normal or even increased diffusion on DWI. The current study was undertaken primarily to determine whether restricted diffusion is uniformly present in pediatric medulloblastoma and secondly to look for pathological features that might distinguish medulloblastomas with and without restricted diffusion. METHODS: The DWI characteristics of pathologically confirmed medulloblastomas diagnosed at our institution were retrospectively reviewed. The ADC was obtained in two non-overlapping, solid, non-hemorrhagic, non-necrotic regions of tumor and averaged. An ADC below 1 × 10(-3) mm(2)/s was considered to represent restricted diffusion. A detailed pathologic review of each tumor was conducted. RESULTS: Ten cases of medulloblastoma were reviewed, of which two demonstrated average ADCs above 1 × 10(-3) mm(2)/s (1.223 and 1.169 × 10(-3) mm(2)/s, respectively), indicating no restricted diffusion. Pathologic review revealed that both of these non-restricting cases displayed a lack of reticulin deposition by light microscopy. CONCLUSION: DWI does not appear to be an entirely reliable means of distinguishing medulloblastomas from other cerebellar neoplasms. Histologically, restricted diffusion in medulloblastomas may be related to reticulin deposition.

Natural history of untreated syringomyelia in pediatric patients.

OBJECT: The natural history of syringomyelia in pediatric patients remains uncertain. Although symptomatic and operative cases of syringomyelia are well studied, there are fewer articles in the literature on the nonoperative syrinx and its clinical and radiological course. The purpose of this research was to analyze the natural history of untreated syringomyelia in pediatric patients presenting with minimal neurological symptoms. METHODS: A review of the neurosurgery database at British Columbia's Children's Hospital identified all pediatric patients (< 18 years of age) with syringes identified on MR imaging. Patients were included in this study if they had at least 2 MR images of the spine, at least 1 year apart, while receiving nonoperative treatment. Magnetic resonance imaging was used to determine changes in the size of the syrinx over time. Clinic notes were analyzed to establish demographic and clinical features and to determine any clinical changes over time. RESULTS: A total of 17 patients were included in the study. Symptoms at presentation were often mild and included limb numbness (3 cases), headaches (2 cases), mild sensory deficits (2 cases), mild motor deficits (3 cases), and intermittent incontinence (7 cases). The consultant neurosurgeon believed that the syrinx was not contributing to the symptoms in these 17 patients. The syrinx either remained unchanged (7 cases) or diminished in size (8 cases) in a total of 15 patients (88%). In the remaining 2 patients the authors noted an increase in syrinx size, in 1 of whom the clinical course also worsened. Both of these patients had a Chiari malformation and subsequently underwent craniocervical decompression. Overall, the mean change was -0.7 mm of maximal axial diameter (range -2.6 to +2.7 mm). Sixteen patients (94%) exhibited no worsening of symptoms over time. CONCLUSIONS: Syringomyelia often remains stable in patients receiving nonoperative treatment. However, given that 2 (12%) of 17 syringes in this series enlarged, it is likely appropriate to include periodic imaging in the follow-up of these cases.

Langerhans cell histiocytosis in children under 12 months of age: The spectrum of imaging and clinical findings: Experience in an Irish tertiary referral centre.

OBJECTIVES: Langerhans cell histiocytosis is an uncommon condition and it is unusual for it to present in children under one year of age. Our aim is to review the clinical presentation, and present the radiologic findings and clinical outcome in this particular cohort of patients and determine how this differs from the imaging features of older children presenting with LCH. MATERIALS AND METHODS: A database of 17 patients with LCH who presented between 0 and 12 months of age was retrospectively reviewed. Radiologic findings, initial clinical presentation and ultimate clinical outcome were documented in table format. RESULTS: Eight patients (47 %) initially presented with cutaneous stigmata, seven patients (41 %) had skeletal involvement, five patients (29 %) had splenic involvement, two patients (24 %) had central nervous system involvement either at presentation or at follow-up, three patients (18 %) had lymphadenopathy, two patients (12 %) had liver involvement, and two patients (12 %) had gastrointestinal (GI) involvement. Four patients (24 %) had multisystem involvement either at presentation or at follow-up. One patient died during follow-up. CONCLUSION: LCH in children under one year of age is uncommon and may have an unusual clinical presentation. The radiologic findings are varied and may differ from the classical imaging appearance more commonly seen in the older age group, with multisystem involvement seen more readily in younger patients.

The questionable efficacy of manualized psychological treatments for distressed breast cancer patients: An individual patient data meta-analysis.

Previous meta-analyses conclude that psychological treatments are efficacious for emotional distress in breast cancer (BCa). However, the practical relevance of these meta-analyses is questionable; none focused specifically on clinically distressed patients or whether treatment effects were clinically significant. In a two-stage individual patient data (IPD) meta-analysis of 17 randomized controlled trials of manualized psychological treatments in BCa, we evaluated treatment efficacy in distressed BCa patients (n = 1591) using clinical significance and effect size analyses. Outcomes were anxiety, depression, and general distress, evaluated at post-treatment and follow-up. Moderators examined were treatment type, treatment format, therapists' profession, control condition, age, outcome measure, and trial quality. Treated patients were more likely than controls to recover from anxiety and general distress at post-treatment (14-15% more treated patients recovered), but not at mean 8-months follow-up. Overall recovery rates were low: across outcomes, at post-treatment, only 30-32% of treated patients and 15-25% of controls recovered; at follow-up, only 21-30% of treated patients and 18-35% of controls recovered. Small between-group effect sizes in favour of treatment were found across outcomes at post-treatment (g = 0.32-0.34) but not at follow-up. Across the different analysis methods, few moderator effects were found. More efficacious psychological treatments are needed for distressed BCa patients.

Diffusion tensor imaging in neonatal encephalopathy: a systematic review.

BACKGROUND AND OBJECTIVE: Diffusion tensor imaging (DTI) during the first few days of life can be used to assess brain injury in neonates with neonatal encephalopathy (NE) for outcome prediction. The goal of this review was to identify specific white matter tracts of interest that can be quantified by DTI as being altered in neonates with this condition, and to investigate its potential prognostic ability. METHODS: Searches of Medline and the Cochrane Database of Systematic Reviews were conducted to identify studies with diffusion data collected in term-born neonates with NE. RESULTS: 19 studies were included which described restricted diffusion in encephalopathic neonates as compared with healthy controls, with the posterior limb of the internal capsule and the genu and splenium of the corpus callosum identified as particular regions of interest. Restricted diffusion was related to adverse outcomes in the studies that conducted a follow-up of these infants. CONCLUSIONS: Obtaining diffusion measures in these key white matter tracts early in life before pseudonormalisation can occur can not only identify the extent of the damage but also can be used to examine the effectiveness of treatment and to predict neurodevelopmental outcome.

Dysregulated Monocyte and Neutrophil Functional Phenotype in Infants With Neonatal Encephalopathy Requiring Therapeutic Hypothermia.

Neonatal encephalopathy (NE) is a significant cause of morbidity and mortality. Persistent inflammation and activation of leukocytes mediate brain injury in NE. The standard of care for NE, therapeutic hypothermia (TH), does not improve outcomes in nearly half of moderate to severe cases, resulting in the need for new adjuvant therapies, and immunomodulation holds promise. Our objective was to explore systemic leukocyte phenotype in infants with NE and healthy controls in response to lipopolysaccharide (LPS). Twenty-four infants with NE (NE II-20; NE III = 4) requiring TH and 17 term neonatal controls were enrolled, and blood samples were analyzed between days 1 and 4 of life at a mean (SD) timepoint of 2.1 (± 0.81) days of postnatal life at the time of the routine phlebotomy. Leukocyte cell surface expression levels of Toll-like receptor 4, NADPH oxidase (NOX2), CD11b, mitochondrial mass, and mitochondrial superoxide production were measured by flow cytometry. Gene expression of TRIF (TIR domain-containing adapter-inducing interferon-ß), MyD88 and IRAK4 was measured by reverse transcription-polymerase chain reaction. Infants with NE had significantly lower expression of neutrophil CD11b and NOX2 with LPS stimulation compared to healthy term controls. Mitochondrial mass in neutrophils and monocytes was significantly increased in NE infants with LPS compared to controls, potentially indicating a dysregulated metabolism. Infants with NE had significantly lower IRAK4 at baseline than controls. NE infants display a dysregulated inflammatory response compared to healthy infants, with LPS hyporesponsiveness to CD11b and NOX2 and decreased IRAK4 gene expression. This dysregulated immune profile may indicate an adaptable response to limit hyperinflammation.

Brief Metacognitive Therapy for Emotional Distress in Adult Cancer Survivors.

Background: Adult cancer survivors often experience substantial psychological morbidity following the completion of acute cancer treatment. Unfortunately, current psychological interventions are of limited efficacy. This study explored if metacognitive therapy (MCT); a brief transdiagnostic psychological intervention was potentially efficacious and could be delivered effectively to adult cancer survivors with psychological morbidity. Methods: An open trial with 3- and 6-month follow-up evaluated the treatment effects of MCT in 27 consecutively referred individuals to a clinical psychology health service specializing in psycho-oncology. Each participant received a maximum of six 1-hour sessions of MCT. Levels of anxiety, depression, fear of cancer recurrence, post-traumatic stress symptoms, health related quality of life, and metacognitive beliefs and processes were assessed using self-report questionnaires. Results: MCT was associated with statistically significant reductions across all outcome measures which were maintained through to 6-month follow-up. In the ITT sample on the primary treatment outcome measure, the Hospital Anxiety and Depression Scale-Total, 59% of participants met recovery criteria at post-treatment and 52% at 6-month follow-up, respectively. No participants significantly deteriorated. In the completer sample (N = 20), 80% recovered at post-treatment and 70% at 6-month follow-up. MCT was acceptable to patients with approximately 75% of patients completing all treatment sessions. Conclusion: MCT, a brief transdiagnostic psychological intervention can be delivered effectively to a heterogenous group of cancer survivors with promising treatment effects. Examining the efficacy of brief MCT against the current gold standard psychological intervention would be a valuable advance toward improving the quality of life of cancer survivors.

Qualitative Evaluation of Cancer Survivors' Experiences of Metacognitive Therapy: A New Perspective on Psychotherapy in Cancer Care.

BACKGROUND: Preliminary evidence suggests that metacognitive therapy (MCT), a brief, process-focused psychological intervention, alleviates distress in cancer survivors. In a longitudinal qualitative study nested in an open trial of MCT for cancer survivors, we explored how patients understood, experienced and applied MCT. METHODS: Patients received six MCT sessions. Consenting patients provided semi-structured interviews post-intervention (n = 19), and at 3- and 6-months follow-up (n = 14 and 10 respectively). Interviews were audio-recorded and transcribed. Analysis followed a constant comparison approach. RESULTS: Participants felt "overwhelmed" by worry before starting MCT and doubted that such brief therapy could help. Their accounts focused on feeling "challenged" to think differently by the psychologist. Those completing therapy were enthusiastic about it. They described having learned that thoughts are "only thoughts," that feelings of worry or sadness are a normal part of life, and that they were in control of whether and how they engaged with thoughts. Consequently, most described a sense of freedom to live free from worry. A minority described being unable to apply MCT to certain thoughts. Two patients who withdrew before completing MCT did not describe having learned what MCT was intended to achieve. CONCLUSION: MCT is an acceptable brief intervention for distressed cancer survivors. Feeling challenged to understand the processes maintaining their distress was central to their enthusiasm for it, irrespective of their presenting difficulties. IMPLICATIONS FOR CANCER SURVIVORS: The complexity of emotional distress in cancer survivors can potentially be addressed using a transdiagnostic model which focuses on the psychological processes which maintain distress.

Metacognitive Therapy for Emotional Distress in Adult Cancer Survivors: A Case Series.

Many adult cancer survivors experience persistent emotional distress after completing cancer treatment. The aim of this study was to test the potential of a brief transdiagnostic psychological intervention-metacognitive therapy (MCT)-in reducing emotional distress in adult cancer survivors. A non-concurrent multiple baseline design with 3- and 6-months follow-up was used to evaluate the effects of MCT in four patients consecutively referred to a psycho-oncology service. Each patient received six 1-h sessions of MCT. Anxiety, depression, worry/rumination, fear of cancer recurrence and metacognitive beliefs were assessed using self-report questionnaires. MCT was associated with clinically significant reductions in anxiety, depression, fear of cancer recurrence, worry/rumination and metacognitive beliefs at the end of treatment, and gains were maintained in all patients to 3-months follow-up and in three out of four patients to 6-months follow-up. MCT is a promising brief transdiagnostic approach to psychological morbidity in adult survivors of cancer. Larger scale controlled trials are now required.

Patients' experience of cancer: evidence of the role of 'fighting' in collusive clinical communication.

Clinical communication with patients with cancer should be based on an accurate understanding of psychological responses to cancer, but existing assumptions about these responses may not be accurate. Male and female ambulant patients (N = 30) provided a sample that varied in type, stage and prognosis of cancer. Interviews about their experience of cancer were audio-recorded and analysed qualitatively. The predominant response was a desire to conceal emotional distress, often to protect others including family and clinicians. Patients perceived clinicians as promoting 'fighting' and a 'positive attitude'. However, for patients, these responses meant resisting the expression of emotional distress rather than the disease. By encouragement to 'fight' and 'be positive', clinicians may therefore collude with patients' emotional suppression. For some patients, cancer led to re-evaluation of life; future research should examine whether and how clinical communication influences this form of adjustment.

Reading, language and memory skills: a comparative longitudinal study of children with Down syndrome and their mainstream peers.

BACKGROUND: Many of today's young adults with Down syndrome never had the opportunity to learn to read. However, an increasing number of children with Down syndrome are currently attending mainstream schools and being taught to read. As a consequence, it is now possible to systematically study reading development in children with Down syndrome. AIMS: The aim of this study was to chart the development of reading, language, and memory skills in children with Down syndrome and to investigate the relationships between these abilities. SAMPLE: Twenty-four children with Down syndrome aged between 4 and 12 were followed over two years and compared to 31 children matched for reading age, and 42 children of average reading ability, selected from the same mainstream classes as the children with Down syndrome. METHOD: Standardised assessments were administered annually to obtain measures of reading, spelling, language, memory, and general intelligence. RESULTS: The children with Down syndrome had relatively advanced single word reading ability compared to their other cognitive skills. The reading progress of the children with Down syndrome did not differ significantly from that of the reading matched group even after two years. Different cognitive abilities were highly correlated with one another in all groups. However, after controlling for age, many of the partial correlations between reading and the other measures were reduced to non-significant levels. CONCLUSIONS: Most children with Down syndrome are capable of learning to read single words. However, there was no evidence over this two-year period to support the hypothesis that learning to read will help to enhance language and memory development in children with Down syndrome.

The effect of surgical timing on operative duration and quality of reduction in Type III supracondylar humeral fractures in children.

PURPOSE: To determine the influence of the time from injury to surgery of Type III supracondylar fractures on operative duration and quality of reduction. METHODS: A retrospective review of Type III supracondylar fractures treated by closed reduction and percutaneous pinning over a 3-year period was performed. RESULTS: The subjects were divided into two groups based on the time from injury to surgery (IST): Group 1 (<8 h) 48 subjects and Group 2 (>8 h) 39 subjects. There was no difference in the mean age or gender ratio between the two groups. There were no cases of compartment syndrome or conversion to open reduction in either group. The mean IST was 669 min. The mean IST for Group 1 was 340 min and it was 1,074 min for Group 2. The operative duration for Group 1 was 32.56 min and for Group 2 it was 31.72 min (P = 0.77). There were no significant differences in the quality of reduction. CONCLUSIONS: There was no difference in the operative duration demonstrated between IST <8 h and IST >8 h. This failure to demonstrate a difference should not be interpreted as demonstrating equivalence. This study does not conclude that all displaced supracondylar fractures should be delayed, though it does inform the surgeon that, if compelled to delay surgery, this series did not demonstrate an increased risk of complications, nor a worsened quality of reduction.

Microfibrillar collagen hemostat-induced necrotizing granulomatous inflammation developing after craniotomy: a pediatric case series.

OBJECT: Microfibrillar collagen hemostat (MCH; trade name Avitene) is a partially water-insoluble acid salt of purified bovine corium collagen. This agent has been widely used to control hemorrhage at surgery, and especially during pediatric neurosurgeries at the authors' institution. Despite its effectiveness, rare case reports detailing adverse inflammatory reactions to MCH have been documented. Based primarily on MR imaging, postoperative reactions have most commonly elicited clinical differential diagnoses of tumor recurrence or abscess. According to the literature, MCH induces a very characteristic mixed inflammatory response that is rich in eosinophils; in light of these observations, many authors have suggested an allergy-based pathogenesis. METHODS: The authors retrospectively reviewed 3 pediatric neurosurgical cases treated at their institution, wherein a common histomorphological inflammatory reaction to MCH was elicited at the site of prior craniotomy. RESULTS: Case 1 is that of a 10-year-old girl whose diagnosis was a right temporal lobe ganglioglioma, classified as WHO Grade I. Case 2 is that of a 9-year-old boy whose diagnosis was a left parietal lobe anaplastic ependymoma, classified as WHO Grade III. Finally, Case 3 is that of a 15-year-old girl whose diagnosis was focal cortical dysplasia Type IIA affecting the left occipital lobe. Each patient presented with new or recurrent seizures 5­6 weeks after the initial resection. The postsurgical reactions incited by MCH mimicked the radiological appearance of either an abscess (Cases 2 and 3) or recurrent tumor (Case 1). Histologically, the mixed inflammatory infiltrate was typified by the presence of MCH-centric necrotizing granulomas that were surrounded by a palisade of macrophages and often several eosinophils. CONCLUSIONS: The findings are in keeping with previous case reports describing the clinicopathological features of adverse reactions occurring due to MCH. Based on the authors' observations, the possibility of an idiopathic inflammatory reaction to MCH should be considered when either seizures, a typical radiological appearance (that is, consistent with tumor recurrence or abscess formation), or both arise shortly after initial surgery. A conservative treatment approach to this type of inflammatory lesion appears to be the most appropriate management strategy.