Treatment of Unicameral Bone Cysts Utilizing the Sclerograft™ Technique.

PURPOSE: To evaluate the Sclerograft™ procedure, which is an image-guided, minimally invasive approach of chemical sclerotherapy followed by bone grafting of unicameral bone cysts (UBC). MATERIALS AND METHODS: A retrospective evaluation from August 2018 through August 2023 was performed at a single institution on patients that underwent the Sclerograft™ procedure for UBCs. Radiographic healing was evaluated utilizing the Modified Neer Classification. Two different regenerative grafts, CaSO4-CaPO4 and HA-CaSO4 were utilized. A total of 50 patients were evaluated with 41 patients grafted with CaSO4-CaPO4 and 9 patients grafted with HA-CaSO4. RESULTS: The average age of the patient was 12.1 years with an average radiographic follow-up of 14.5 months. Average cyst size was 5.5 cm in the largest dimension and average cyst volume was 20.2 cc. 42 out of 50 (84%) showed healed cysts (Modified Neer Class 1) on the most recent radiograph or MRI. Recurrences occurred on average at 7.2 months. Activity restrictions were lifted at 3-4.5 months post-procedure. Cyst stratification by size did not show a difference in recurrence rates (p = 0.707). There was no significant difference in recurrence rate between lesions abutting the physis compared to those that were not abutting the physis (p = 0.643). There were no major complications. CONCLUSIONS: The Sclerograft™ procedure is an image-guided approach to treating unicameral bone cysts, utilizing chemical sclerosis and regenerative bone grafting. The radiographic healing of cysts compares favorably to open curettage and grafting as determined utilizing previously published trials.

Melanoma in infants, caused by a gene fusion involving the anaplastic lymphoma kinase (ALK).

We describe the first cases of pediatric melanoma with ALK fusion gene arising within giant congenital melanocytic nevi. Two newborn boys presented with large pigmented nodular plaques and numerous smaller satellite nevi. Additional expansile nodules developed within both nevi and invasive melanomas were diagnosed before 10 months of age in both boys. Oncogenic driver mutations in NRAS and BRAF were absent in both cases. Instead, oncogenic ZEB2::ALK fusion genes were identified in both the nevus and melanoma developing within the nevus. In both cases, tumors were noted by ultrasound in utero, demonstrated significant nodularity at birth, and progressed to melanoma in the first year of life suggesting that congenital nevi with ALK fusion genes may behave more aggressively than those with other mutations. As ALK kinase inhibitors are effective against a range of tumors with similar ALK fusion kinases, identifying ALK fusion genes in congenital melanocytic nevi may provide an opportunity for targeted therapy.

Audit of antimicrobial stewardship in medical inpatients in Waikato, New Zealand 2021.

AIMS: Given the threat of rising antimicrobial resistance (AMR), 10 audit standards were selected to audit antimicrobial stewardship (AMS) in secondary care to assess guideline adherence and establish quality improvement initiatives in antimicrobial prescribing. METHODS: Patients were included if they received intravenous (IV) antibiotics across seven medical wards in Waikato or Thames hospitals, New Zealand, in November 2021. Audit standards were defined from the regional antimicrobial prescribing policy and adult antimicrobial guidelines. RESULTS: In total, 205 patients were audited. Microbiological sampling standards were met in 87 of 126 occasions (69.0%). Antimicrobial choices adhered to guidelines in 89 of 163 patients (54.6%), where guidelines were available. Documentation of antimicrobial indications in the medical notes and antimicrobial review at 48 to 72 hours met the standards at over 90%. Only 2 of 13 patients (15.4%) receiving piperacillin/tazobactam or a carbapenem were discussed with Infectious Diseases (ID). Documentation of indications and durations on paper-based medication charts was infrequent, around 12%. Evaluating for health equity, similar results were observed for Maori and non-Maori. CONCLUSIONS: Our audit identified specific areas for AMS quality improvement initiatives. Regular audit should become an essential element of the New Zealand AMS strategy. We believe increased AMS resources are required.

Nonparasitic Splenic Cysts in Children: Outcomes after Sclerotherapy or Surgical Resection as Initial Management.

Management of splenic cysts in children remains undefined. Sclerotherapy is an innovative, less invasive treatment. This study examined the safety and preliminary effectiveness of sclerotherapy for splenic cysts in children compared with those of surgical treatment. A retrospective review of pediatric patients treated for nonparasitic splenic cysts from 2007 to 2021 was performed at a single institution. Posttreatment outcomes for patients who underwent either expectant management, sclerotherapy, or surgery were reviewed. Thirty patients aged between 0 and 18 years met the inclusion criteria. Cysts in 3 of 8 patients who underwent sclerotherapy were either unresolved or recurred. Patients who underwent sclerotherapy and required surgery for residual symptomatic cyst had an initial cyst diameter of >8 cm. Symptoms resolved in 5 of 8 patients who underwent sclerotherapy, with a significantly reduced cyst size compared with that in patients with continued symptoms who underwent sclerotherapy (61.4% vs 7.0%, P = .01). Sclerotherapy is an effective treatment for splenic cysts, particularly those measuring <8 cm. However, surgical excision may be preferable for large cysts.

Accuracy of Chest Computed Tomography in Distinguishing Cystic Pleuropulmonary Blastoma From Benign Congenital Lung Malformations in Children.

Importance: The ability of computed tomography (CT) to distinguish between benign congenital lung malformations and malignant cystic pleuropulmonary blastomas (PPBs) is unclear. Objective: To assess whether chest CT can detect malignant tumors among postnatally detected lung lesions in children. Design, Setting, and Participants: This retrospective multicenter case-control study used a consortium database of 521 pathologically confirmed primary lung lesions from January 1, 2009, through December 31, 2015, to assess diagnostic accuracy. Preoperative CT scans of children with cystic PPB (cases) were selected and age-matched with CT scans from patients with postnatally detected congenital lung malformations (controls). Statistical analysis was performed from January 18 to September 6, 2020. Preoperative CT scans were interpreted independently by 9 experienced pediatric radiologists in a blinded fashion and analyzed from January 24, 2019, to September 6, 2020. Main Outcomes and Measures: Accuracy, sensitivity, and specificity of CT in correctly identifying children with malignant tumors. Results: Among 477 CT scans identified (282 boys [59%]; median age at CT, 3.6 months [IQR, 1.2-7.2 months]; median age at resection, 6.9 months [IQR, 4.2-12.8 months]), 40 cases were extensively reviewed; 9 cases (23%) had pathologically confirmed cystic PPB. The median age at CT was 7.3 months (IQR, 2.9-22.4 months), and median age at resection was 8.7 months (IQR, 5.0-24.4 months). The sensitivity of CT for detecting PPB was 58%, and the specificity was 83%. High suspicion for malignancy correlated with PPB pathology (odds ratio, 13.5; 95% CI, 2.7-67.3; P = .002). There was poor interrater reliability (κ = 0.36 [range, 0.06-0.64]; P < .001) and no significant difference in specific imaging characteristics between PPB and benign cystic lesions. The overall accuracy rate for distinguishing benign vs malignant lesions was 81%. Conclusions and Relevance: This study suggests that chest CT, the current criterion standard imaging modality to assess the lung parenchyma, may not accurately and reliably distinguish PPB from benign congenital lung malformations in children. In any cystic lung lesion without a prenatal diagnosis, operative management to confirm pathologic diagnosis is warranted.

Pathologic Fracture as Presentation of Generalized Lymphangiomatosis in an Eight-Year-Old: A Case Report and Literature Review.

Pathologic fractures commonly occur secondary to abnormal skeletal physiology in the context of benign or malignant lesions. Rarely, pathologic fractures may occur in the context of a lymphatic abnormality, such as generalized lymphangiomatosis. This rare disorder is characterized by variable presentations in a broad age range of patients. By understanding the effect of widespread lymphatic anomalies on various organ systems, clinicians will be able to make this diagnosis earlier and with more certainty.

Multi inflammatory syndrome in a 16-year-old male following first dose of m-RNA COVID-19 vaccination.

Multisystem Inflammatory Syndrome (MIS) is an uncommon systemic illness that occurs 4-6 weeks after primary infection with SARS-CoV-2. There are emerging reports of MIS arising following vaccination against SARS-CoV-2. We report a 16-year-old male with a multi system inflammatory condition meeting the case definition for MIS following BTN162b2 mRNA SARS-CoV-2 (Pfizer BioNTech) vaccine with no other identifiable precipitant or evidence of primary infection with SARS-Cov-2.

Weight gain and resource utilization in infants after fundoplication versus gastrojejunostomy.

PURPOSE: There is wide practice variation in the use of laparoscopic fundoplication (LF) versus gastrojejunostomy (GJ) tube insertion for children who do not tolerate gastric feeds. Using weight gain as an objective proxy of adequate nutrition, we sought to evaluate the difference in weight gain between LF and GJ. METHODS: A retrospective, cohort study was conducted of patients ≤ 2 years who underwent LF or GJ between 2014 and 2019 at a single institution. Patient characteristics, change in weight 1-year post-procedure and frequency of unplanned healthcare utilization encounters were collected and examined. RESULTS: A total of 125 patients (50.4%LF, 49.6%GJ) were identified. Adjusted modeling demonstrated that on average, there was an additional 0.85-unit increase in weight-for-age Z scores in the LF compared to the GJ cohort (p = 0.01). The GJ cohort had significantly more unplanned healthcare utilization encounters (4.2, SD 3.4) compared to LF (3.0, SD 3.1) (p = 0.03). Furthermore, the GJ cohort underwent an average of 3.3 planned GJ exchanges within 1-year post-procedure. CONCLUSION: In the first year post-operatively, LF is associated with increased weight gain and fewer unplanned and overall healthcare encounters compared to GJ. Long-term outcomes including weight gain and quality-of-life measures should be studied to develop standardized guidelines for this common clinical scenario.

Intralesional Bleomycin Injection and Skin Hyperpigmentation: A Case Series of a Single-Center Experience With a Standardized Skin-Protective Protocol.

Bleomycin is a cytotoxic antibiotic that has a sclerosing effect on vascular endothelium. Small doses can be injected locally to reduce the size of various vascular malformations. Recognition that targeted intralesional injection carries the potential to cause hyperpigmentation, specifically in areas of skin trauma, and has led our department to implement a bleomycin skin-protective protocol. On review of cases performed following protocol implementation, no patients developed hyperpigmentation attributable to iatrogenic skin trauma. We present our bleomycin skin protection protocol to guide peer institutions in the initiation of similar quality improvement initiatives.

Minimally Invasive Treatment for Unicameral Bone Cysts with Chemical Sclerosis and Bone Graft Substitute: A Preliminary Report.

PURPOSE: Unicameral bone cysts (UBCs) are benign lesions that primarily occur in childhood and can present with pain, pathologic fracture and growth disturbances. Existing treatment approaches for the management of UBCs are highly variable and recurrence is not uncommon. This study seeks to evaluate near-term outcomes of an image-guided, minimally invasive combination of chemical sclerotherapy and synthetic grafting as a novel outpatient management treatment option for UBCs. MATERIALS AND METHODS: Retrospective evaluation from August 2018 to July 2020 of fourteen pediatric patients, ages 5-14 years, undergoing treatment for a UBC, at a single institution. All UBCs were treated in a single, minimally invasive, image-guided procedure using percutaneous needle access into the UBC followed by chemical sclerotherapy and injection of regenerative synthetic graft. Patients were followed clinically and with serial radiographs to evaluate for healing and complications, with an average follow-up of 13.7 months. Descriptive statistics were performed. RESULTS: Twelve of 14 (85.7%) patients showed Modified Neer classification class 1 or 2 healed cysts at their most recent follow-up. There were two recurrences. All patients were pain-free and returned to normal physical activity on average within 2.0 months, and all patients with healed cysts remained asymptomatic at the most recent follow-up. There were no complications related to the procedures. CONCLUSIONS: Image-guided chemical sclerosis and bone grafting is a minimally invasive treatment option for unicameral bone cysts and compares favorably to other existing treatment options. The preliminary findings of this technique are promising as an alternative management option for UBCs.

Pediatric Vascular Malformations: Imaging Guidelines and Recommendations.

Vascular malformations are commonly encountered in the pediatric population. This article reviews the imaging appearances of simple and syndromic vascular malformations in infants and children that radiologists should know and provides imaging guidelines based on an evidence-based approach. Malformations are discussed within the framework of the International Society for the Study of Vascular Anomalies classification system.

In patients with acute HF and iron deficiency, IV ferric carboxymaltose improved quality of life at 4 to 24 wk.

SOURCE CITATION: Jankowska EA, Kirwan BA, Kosiborod M, et al. The effect of intravenous ferric carboxymaltose on health-related quality of life in iron-deficient patients with acute heart failure: the results of the AFFIRM-AHF study. Eur Heart J. 2021;42:3011-20. 34080008.

Role of Interventional Radiology in Children with Biliary and Gallbladder Diseases.

Biliary and gallbladder diseases in infants and children often present unique diagnostic and therapeutic challenges that require a fundamental understanding of notable biliary diseases and anatomical variations. Surgical and endoscopic approaches that are often the gold standard in adult biliary treatment may be technically challenging and are associated with a high morbidity that may warrant a multidisciplinary treatment approach. This article will provide a comprehensive overview of the biliary conditions where interventional radiology can play a vital role in the diagnosis, management, and treatment. Differences in approach or technique between children and adults will be highlighted.

Population pharmacokinetics of free flucloxacillin in patients treated with oral flucloxacillin plus probenecid.

Oral flucloxacillin may be coadministered with probenecid to reduce flucloxacillin clearance and increase attainment of pharmacokinetic-pharmacodynamic (PK/PD) targets. The aims of this study were to develop a population PK model of free flucloxacillin when administered orally with probenecid, and to identify optimal dosing regimens for this combination. METHODS: We performed a prospective observational study of adults (45 participants) treated with oral flucloxacillin 1000 mg and probenecid 500 mg 8-hourly for proven or probable staphylococcal infections. Steady-state mid-dose-interval flucloxacillin measurements (45 concentrations) were combined with existing data from a crossover study of healthy participants receiving flucloxacillin with and without probenecid (11 participants, 363 concentrations). We developed a population pharmacokinetic model of free flucloxacillin concentrations within Monolix, and used Monte Carlo simulation to explore optimal dosing regimens to attain PK/PD targets proposed in the literature (free drug time above minimum inhibitory concentration). RESULTS: Flucloxacillin disposition was best described by a 1-compartment model with a lag time and first-order absorption. Free flucloxacillin clearance depended on probenecid, allometrically-scaled fat free mass (FFM) and estimated glomerular filtration rate (eGFR). Predicted PK/PD target attainment was suboptimal with standard dosing regimens with flucloxacillin alone, but substantially improved in the presence of probenecid. CONCLUSION: The simulation results reported can be used to identify dose regimens that optimise flucloxacillin exposure according to eGFR and FFM. Patients with higher FFM and eGFR may require the addition of probenecid and 6-hourly dosing to achieve PK/PD targets. The regimen was well-tolerated, suggesting a potential for further evaluation in controlled clinical trials to establish efficacy.

Midline venous catheters as an alternative to central line catheter placement: a product evaluation.

Midline catheters have emerged as a successful alternative to peripheral venous catheters (PVCs) and central venous catheters (CVCs) in select patients. Midline catheters allow for greater duration of access when compared with PVCs and avoid the critical complications associated with CVCs. This article describes the implementation of the Powerwand® midline at a large paediatric tertiary care facility in acute and intensive care settings, and presents illustrative cases of the catheter in use. Product evaluation was performed by a paediatric vascular access team of registered nurses and included information on patient outcomes, inserter satisfaction, patient satisfaction, and cost effectiveness.

Haemophilus influenzae purulent pericarditis in an immunocompetent individual.

Purulent pericarditis is a rare bacterial illness in the post-antibiotic era that is defined as pericardial infection with gross or microscopic purulence in the pericardium. Common causes include nosocomial bloodstream infections, direct spread through thoracic surgery, or immunosuppression. We present a case of a 66-year-old male with a history of mantle cell lymphoma status post chemotherapy, completed about 4 years before presentation, in general, good health presented with acute typical chest pain associated with dyspnea on exertion. 12-lead EKG demonstrated ST elevations in anterolateral and lateral leads. Patient was initially being managed as Acute Coronary Syndrome, though, preliminary bedside echocardiogram demonstrated a large pericardial effusion with pretamponade physiology, which was confirmed with a STAT transthoracic echocardiogram. He underwent an emergent pericardial window which drained 350-400 ml of yellow murky pericardial fluid. Blood cultures and pericardial fluid cultures grew Haemophilus influenzae (H. influenzae). Upon further history taking, patient revealed experiencing upper respiratory symptoms and being diagnosed with pansinusitis 2 months before his admission. He was treated with IV ceftriaxone for 4 weeks from the day of negative blood cultures. H. influenzae upper respiratory infection is usually seen in the unvaccinated pediatric population, or in immunocompromised individuals; however, few cases in vaccinated adults have been reported, as in the above case. Sequalae from H. influenzae infection is usually limited to upper respiratory symptoms and mastoiditis, but rarely, pericarditis could occur. It is important to include pericarditis in the differential for chest pain in a patient with a recent history of upper respiratory symptoms. Pericarditis is a rare but potentially serious complication of recent upper respiratory tract infection, and needs to be promptly identified and treated to avoid further morbidity.

Tricuspid valve endocarditis in pregnancy: a case report and review of the literature.

Infective endocarditis (IE) is a rare complication in pregnancy that is associated with significant morbidity and mortality to both mother and fetus. We present a case of a 27-year-old female at 22-weeks gestation with a history of intravenous drug abuse (IVDA) who developed methicillin sensitive Staphylococcus aureus tricuspid valve endocarditis with persistent bacteremia and septic emboli necessitating tricuspid valve extirpation. Four days later, worsening decompensated heart failure required cesarean section at 23w5d. Although the patient's volume status and dyspnea improved significantly, fetal demise occurred 9 days after operative delivery.

Hypochloraemia and 30 day readmission rate in patients with acute decompensated heart failure.

AIMS: Despite recent advances in guideline-directed therapy, rehospitalization rates for acute decompensated heart failure (ADHF) remain high. Recently published studies demonstrated the emerging role of hypochloraemia as a predictor of poor outcomes in patients with ADHF. This study sought to determine the correlation between low serum chloride and 30 day hospital readmission in patients with ADHF. METHODS AND RESULTS: We retrospectively reviewed electronic medical records of 1504 patients who were admitted to one 700 bed US tertiary care centre with the diagnosis of ADHF between June 2013 and December 2014. Of the 1504 reviewed records, 1241 were selected for further analysis. Hypochloraemia (either on admission or at discharge) was identified in 289 patients (23.3%) and was associated with significantly higher 30 day hospital readmission rate or death (42.2% vs. 33.7%, P = 0.008). This association persisted in multivariate analysis when controlling for serum sodium, weight loss, diuretic dose, adjunct thiazide use, serum blood urea nitrogen, and BNP levels (OR: 1.35, 95% CI: 1.02-1.77, P = 0.033); however, the predictive value of the overall model was low (Naglkerke R2 = 0.040). Hypochloraemia was also found to be associated with increased 12 month mortality in our cohort (31.4% vs. 20.2%, P = 0.015) that correlates with the results of previously published studies. CONCLUSIONS: Low serum chloride measured in patients admitted for ADHF is independently but weakly associated with increased 30 day readmission rate and demonstrated low predictive value as a potential biomarker in this cohort.