Thoracic lipoblastoma in a 6-year-old African male: a case report.

Lipoblastoma is a very infrequent tumor, characteristic of early childhood. The thoracic location is infrequent, with isolated reports to date. We present the case of a 6-year-old male patient with a right thoracic tumor of months of evolution that was surgically removed by right anterolateral thoracotomy and in which the diagnosis of classic well-differentiated lipoblastoma was histologically confirmed. The patient evolved favorably and was discharged. He is currently under follow-up and without recurrence 1 year after surgery. This is, to our knowledge, the first thoracic lipoblastoma reported in an African pediatric patient. The importance of knowing the clinical, semiological, and intraoperative characteristics of this tumor becomes even more important, as in our case, in the context of international cooperation, where in many cases, there is no possibility of performing pre-operative imaging studies or subsequent genetic studies.

El lipoblastoma es un tumor muy infrecuente, característico de la primera infancia. La localización torácica es infrecuente, con reportes aislados hasta la fecha. Presentamos el caso de un paciente varón de 6 años con una tumoración torácica derecha de meses de evolución que fue extirpada quirúrgicamente mediante toracotomía anterolateral derecha y en la que se confirmó histológicamente el diagnóstico de lipoblastoma clásico bien diferenciado. El paciente evolucionó favorablemente y fue dado de alta. Actualmente se encuentra en seguimiento y sin recidiva un año después de la cirugía. Este es, hasta donde sabemos, el primer lipoblastoma torácico reportado en un paciente pediátrico africano. La importancia de conocer las características clínicas, semiológicas e intraoperatorias de este tumor cobra aún más importancia, como en nuestro caso, en el contexto de la cooperación internacional, donde en muchos casos no existe la posibilidad de realizar estudios de imagen preoperatorios ni estudios genéticos posteriores.

Post-Operative Increase in Serum Interleukin-6 Is Associated With Longer Hospital Stay in Children Operated on for Acute Appendicitis: A Pilot Study.

Background: Pediatric acute appendicitis (PAA) involves a substantial consumption of health and economic resources. The identification of serum biomarkers that may help predict the post-surgical evolution of these patients is a field of great interest. Patients and Methods: This was a prospective, observational substudy within the Biomarkers for the Diagnosis of Appendicitis in Pediatrics (BIDIAP) cohort aimed at evaluating the association between post-surgical increase in serum IL-6 and different outcomes related to the clinical evolution of children operated on for PAA. Sixty-nine children with a confirmed diagnosis of acute appendicitis and both pre-operative and post-operative serum IL-6 were included in the study. Three multivariable-adjusted linear regression models were fitted to analyze the association between an increase of >10% in post-operative serum IL-6 level with the length of stay, the number of post-operative emetic episodes, and the onset of oral feeding. Two multivariable-adjusted logistic regression models were fitted to assess the association of the same exposure with the indication of antibiotherapy at discharge and with positivity in peritoneal fluid culture. Results: Thirteen children showed an increase of >10% in the post-operative serum IL-6 value (group 1) whereas 56 showed only a minor increase, or no change (group 2). After accounting for potential confounders, children in group 1 had a mean of three-day longer hospital stay (difference, 3.33; 95% confidence interval [CI], 0.57-6.09) and higher odds of a positive result in peritoneal fluid culture (odds ratio [OR], 37.43; 95% CI, 1.02-1361.28) than children in group 2. Conclusions: An increase of >10% in post-operative serum IL-6 value could predict longer hospital stay and higher odds of positive peritoneal fluid culture. Future prospective studies are needed to replicate these findings and to broaden the range of biomarkers that could predict the post-operative evolution of children operated on for PAA.

From Aviation to Pediatric Surgery.

Aviation is a tremendously complex process involving multiple factors that can be subsidiary to human error. The implementation of checklists, tools that reduce this risk, has often been extrapolated to other fields, especially medicine. Through this reflection, we comment on the critical and relevant aspects of pediatric surgical patient safety, briefly discussing the existing literature and analyzing potential areas for improvement.

Desarrollo y aplicación clínica de nuevo sistema de tracción cenital esternal en la toracoplastia percutánea videoasistida / Development and clinical application of a new sternal zenithal traction system in video-assisted percutaneous thoracoplasty

Introducción: La toracoplastia percutánea videoasistida implica un acceso quirúrgico complejo y con riesgo de dañar estructuras vitales durante el procedimiento. Históricamente se han aplicado distintos sistemas de tracción y elevación esternal durante la intervención con el fin de minimizar el riesgo asociado al paso del instrumental entre el esternón y el pericardio. Métodos: Se presenta mediante una descripción ilustrada un nuevo sistema de tracción esternal. Se extrajeron los datos clínicos y sociodemográficos de los 36 pacientes intervenidos en nuestro centro de Pectus Excavatum entre julio 2017 y agosto 2021. Todos los pacientes fueron intervenidos por el mismo cirujano. Se clasificó a los pacientes en función de si se había empleado el sistema de tracción esternal (TE) o no (ST). La asignación a uno u otro grupo de intervención se hizo siguiendo un criterio cronológico, sin aplicar el índice de Haller u otro índice. El análisis estadístico de los datos recogidos se realizó con STATA, versión 15.0 (StataCorp). Resultados: La distribución de las variables sociodemográficas muestra que no había diferencias significativas entre los grupos. En 25 de los pacientes se empleó el sistema TE y en 11 no se utilizó ningún ST. El Índice de Haller fue de 4,19±0,7 para el grupo TE y de 3,79±0,3 para el ST. El tiempo quirúrgico medio en minutos fue 88,13±18,1 para el grupo TE y 97,73±46,2 para el grupo ST (p=0,87). La media de días de ingreso fue 7,67±0,8 y 7,73±1,3 para el grupo TE y ST respectivamente. La media de días de PCA intravenosa fue 6,08±0,7 para el grupo TE y 5,89±1,5 para el ST. La media de días de PCA epidural fue 3,79±0,5 y de 3,36±0,5 para el grupo TE y ST respectivamente (p=0,01). Todos los pacientes presentaron una evolución postoperatoria favorable. Ninguno de los pacientes presentó molestias a nivel de las heridas esternales durante el ingreso en hospitalización. La evolución cosmética fue favorable en todos los pacientes. (AU)

Introduction: Video-assisted percutaneous thoracoplasty involves a complex surgical access with risk of damaging vital structures during the procedure. Historically, different traction and sternal elevation systems have been applied during the intervention to minimize the risk associated with the passage of the instruments between the sternum and the pericardium. Methods: A new sternal traction system is presented by means of an illustrated description. Clinical and sociodemographic data were extracted from the 36 patients operated in our center for Pectus Excavatum between July 2017 and August 2021. The Haller index was not applied as a criterion to determine the use or not of the sternal traction system. Patients were classified according to whether the sternal traction system (TE) or not (ST) had been employed. Statistical analysis of the data collected was performed with STATA, version 15.0 (StataCorp). Results: Both groups were comparable. In 25 of the patients the described traction system was used, and in 11 no traction system was used. Haller's index was 4.19±0.7 for the TE group and 3.79±0.3 for the ST group. All patients were operated on by the same surgeon. The mean operative time in minutes was 97.73±46.2 for the ST group and 88.13±18.1 for the TE group (P=.87). The mean total days of admission was 7.67±0.82 (TE) and 7.73±1.35 (ST). Mean days of intravenous PCA was 6.08±0.72 (TE) and 5.89±1.45 (ST). The mean number of epidural PCA days was 3.79±0.5 (TE) and 3.36±0.5 (ST) (P=.01). All patients presented a favorable postoperative evolution. None of the patients presented discomfort at the level of the sternal wounds during hospitalization. Cosmetic evolution was favorable in all patients. (AU)

The BIDIAP index: a clinical, analytical and ultrasonographic score for the diagnosis of acute appendicitis in children.

BACKGROUND: Pediatric acute appendicitis (PAA) continues to be a diagnostic challenge today. The diagnostic performance of classical indices is only moderate, especially in pediatric population. This study aimed to define a clinical, radiological and analytical index for the diagnosis of PAA. MATERIALS AND METHODS: This prospective study included 151 patients divided into two groups: (1) 53 patients with non-surgical abdominal pain (NSAP) and (2) 98 patients with a confirmed PAA. Sociodemographic and clinical characteristics were compared between groups using the Mann-Whitney U test and the Fisher exact test. To identify the predictors of PAA, we performed a multivariable logistic regression using a forward stepwise analysis and we assigned multiples of integer values to the selected variables. The diagnostic performance of the index was assessed by calculating the area under the receiver operating characteristic curve. Intra-cohort calibration was assessed with the Hosmer-Lemeshow test. RESULTS: We developed the BIDIAP index (BIomarkers for the DIagnosis of Appendicitis in Pediatrics), which included three variables that independently predicted higher odds of PAA: appendiceal caliber (≥ 6.9 mm), systemic immune-inflammation index (≥ 890) and peritoneal irritation, which scored 4, 3 and 2 points, respectively. Mean (SD) score of the participants was 2.38 (2.06) in group 1 and 7.89 (1.50) in group 2. The area under the ROC was 0.97 (95% CI 0.95-0.99). The cut-off point was established at 4 points, resulting in a sensitivity of 98.98% and a specificity of 77.78%. CONCLUSIONS: The BIDIAP index has an exceptional diagnostic performance in PAA. The importance of these results lies in its novelty and in the simplicity of the index. Although external validation will be necessary, initial results look promising.

Coagulation Profile: Alterations and Diagnostic Yield in Pediatric Acute Appendicitis: A Prospective Validation Study.

Background: The literature regarding alterations in the coagulation profile in pediatric acute appendicitis (PAA) is scarce and mainly limited to retrospective studies. Evidence on the diagnostic yield of coagulation parameters is limited to fibrinogen. Patients and Methods: This is a prospective study with 151 patients divided into two groups: patients with nonsurgical abdominal pain (NSAP) in whom the diagnosis of PAA was excluded (n = 53) and patients with a confirmed diagnosis of PAA (n = 98). In 93 patients (62%), a coagulation study was obtained at the time of diagnosis and international normalized ratio (INR), activated partial thromboplastin time (aPTT), d-dimer, platelets, mean platelet volume, and platelet-to-lymphocyte ratio were analyzed. The PAA group was further classified into complicated (n = 19) and non-complicated PAA (n = 40). Quantitative variables were compared between groups using the Mann-Whitney U test. Diagnostic performance of the coagulation profile was evaluated with the area under the receiver operating characteristic (ROC) curves. Results: Patients with NSAP had lower median levels of INR, fibrinogen and d-dimer than those with PAA. Moreover, patients with complicated PAA had higher median values of INR and fibrinogen. None of the patients needed specific treatment for the correction of coagulopathy. Fibrinogen was the parameter with the highest diagnostic yield for distinguishing between NSAP and PAA (area under the curve [AUC], 0.74; 95% confidence interval [CI], 0.65-0.85), as well as between complicated versus non-complicated PAA (AUC, 0.71; 95% CI, 0.57-0.86). Conclusions: This study found a moderate extrinsic pathway coagulopathy in patients with PAA, especially in complicated PAA. Fibrinogen is a parameter with moderate diagnostic yield for the diagnosis of PAA.

Cooperation and Scientific Contribution: The Search for a Common Nexus.

International cooperation in pediatrics and pediatric surgery entails important barriers such as sociocultural differences, language difficulties, lack of infrastructure and resources, and short duration of campaigns. In this work, we share our personal experience in relation to the scientific publication of works carried out in pediatric international cooperation, and we make a critical reflection on the aspects to be considered for this field to develop in the future.

Giant Perineal Plexiform Neurofibroma in an 8-Year-Old African Male.

The perineal presentation of plexiform neurofibroma is exceptional, with only two cases reported to date.We present an 8-year-old African male with a large perineal tumor of years of evolution. He had no associated symptoms. Café au lait stains were observed on examination, without other findings of relevance. The patient had no preoperative radiological studies. Partial excision of the lesion was performed. Histopathological study of the specimen revealed a plexiform neurofibroma.The lack of diagnostic suspicion due to the atypical nature of the location, the anatomical complexity of surgical resection and the potential urological and rectal involvement make this lesion a diagnostic-therapeutic challenge. Among the differential diagnoses, schwannoma, congenital lipoma, hamartoma and lipoblastoma should be considered.

Extensive Keloid and Hypertrophic Mixed Scarring Pattern in Ear Lobes of a 14-Year-Old African Female: A Case Report.

Background Massive earlobe scarring/keloid formation can occur after ear piercing in individuals of African descent. Case report: A 14-year-old African girl with pierced ears in childhood presented with two progressively growing and disfiguring tumors on both earlobes. The maximum diameter of each lesion was 5.5 centimeters, and the weight of each lesion was approximately 20 grams. Histologically, there was a mixed pattern of keloid and hypertrophic scarring. Discussion: Massive keloids can occur after ear piercing in childhood. It is unclear why some individuals develop these massive keloids.

Diagnostic performance of calprotectin and APPY-1 test in pediatric acute appendicitis: a systematic review and a meta-analysis.

BACKGROUND: Pediatric acute appendicitis (AA) is a challenging pathology to diagnose. In the last decades, multiple biomarkers have been evaluated in different human biological samples to improve diagnostic performance. This study aimed to examine the diagnostic performance of serum, fecal and urinary calprotectin as well as the role of the APPY-1 biomarker panel in pediatric acute appendicitis. METHODS: We conducted a systematic review of the literature that involved an extensive search in the main databases of medical bibliography (Medline, PubMed, Web of Science and SciELO). Two independent reviewers selected the relevant articles based on the previously defined inclusion and exclusion criteria. Methodological quality of the selected article was rated using the QUADAS2 index. Data extraction was performed by two independent reviewers. A synthesis of the results, a standardization of the metrics and two random-effect meta-analyses, one for serum calprotectin and one for APPY-1, were performed. RESULTS: The research resulted in 173 articles. Thirty-eight duplicates were removed. Among the remaining 135 articles, we excluded 125 following the inclusion and exclusion criteria, resulting in the 10 studies included in this review. This systematic review included data from of 3901 participants (1276 patients with confirmed diagnosis of AA and 2625 controls). The age of the participants ranged from 0 to 21 years. Four of the studies compared serum calprotectin values and reported significant differences between groups, but inconsistent results regarding cutoff points, sensitivity and specificity. Two publications compared urinary values of calprotectin and presented inconsistent results regarding sensitivity and specificity as well. One publication evaluated the diagnostic performance of fecal calprotectin, but it did not provide data on measured values. Four studies evaluated the diagnostic performance of APPY-1 test in pediatric acute appendicitis. The calculated pooled sensitivity and specificity of those studies were 97.37 (95% CI 95.60-98.44) and 36.74 (95% CI 32.28-41.44), respectively, and the calculated pooled NLR, 0.0714 (95% CI 0.041-0.115). CONCLUSION: Serum calprotectin has limited diagnostic yield in pediatric acute appendicitis. Its performance seems to increase with the hours of clinical evolution and in advanced AA, although the evidence is limited. There is not enough evidence on the usefulness of urinary or fecal calprotectin in the diagnosis of pediatric acute appendicitis. On the other hand, the APPY-1 is a reliable test to exclude the diagnosis of AA in patients at low or moderate risk according to PAS and Alvarado Score.

Unilateral Anophthalmia and Congenital Frontal Cranioschisis Associated with Extradural Neuroglial Heterotopia: new Insights into a Possible New Malformative Spectrum.

Background: Neuroglial heterotopia, characterized by mature -neuroglial tissue outside the central nervous system, has not been previously associated with cranioschisis. Case Report: A 4-year-old female patient, with left congenital anophthalmia, had a nasofrontal neuroglial heterotopia protruding through an ossification defect. Discussion: Nasofrontal cranioschisis may be associated with neuroglial heterotopias. The combination of anophthalmia and neuroglial heterotopia, previously described only once in the literature, may be part of a broader malformation spectrum that has not been properly characterized to date.

Development and clinical application of a new sternal zenithal traction system in video-assisted percutaneous thoracoplasty.

INTRODUCTION: Video-assisted percutaneous thoracoplasty involves a complex surgical access with risk of damaging vital structures during the procedure. Historically, different traction and sternal elevation systems have been applied during the intervention to minimize the risk associated with the passage of the instruments between the sternum and the pericardium. MATERIAL AND METHODS: A new sternal traction system is presented by means of an illustrated description. Clinical and sociodemographic data were extracted from the 36 patients operated in our center for Pectus Excavatum between July 2017 and August 2021. The Haller index was not applied as a criterion to determine the use or not of the sternal traction system. Patients were classified according to whether the sternal traction system (TE) or not (ST) had been employed. Statistical analysis of the data collected was performed with STATA, version 15.0 (StataCorp). RESULTS: Both groups were comparable. Haller's Index was 4.19 ± 0.7 for the sternal traction (TE) group and 3.79 ± 0.3 for the no sternal traction (ST) group. In 25 of the patients the described traction system was used, and in 11 no traction system was used. All patients were operated on by the same surgeon. The mean operative time in minutes was 97.73 ± 46.2 for the ST group and 88.13 ± 18.1 for the TE group (p = 0.87). The mean total days of admission was 7.67 ± 0.82 (TE) and 7.73 ± 1.35 (ST). Mean days of intravenous PCA was 6.08 ± 0.72 (TE) and 5.89 ± 1.45 (ST). The mean number of epidural PCA days was 3.79 ± 0.5 (TE) and 3.36 ± 0.5 (ST) (p = 0.01). All patients presented a favorable postoperative evolution. None of the patients presented discomfort at the level of the sternal wounds during hospitalization. Cosmetic evolution was favorable in all patients. CONCLUSIONS: The sternal traction system presented provides safety when performing retrosternal dissection and placement of the thoracoplasty bar, leading to a decrease in surgical time in cases with a Haller Index higher than 3.5. There have been no complications associated with its use, nor has there been an increase in the number of days of hospitalization or analgesic requirements. There are no aesthetic sequelae for the patients.

Congenital Prepubic Sinus as a Variant of Incomplete Urethral Dorsal Duplication: A Case Report with New Insights into its Immunohistochemical Characterization and a Comprehensive Literature Review.

Congenital prepubic sinus (PS) is an extremely infrequent malformation consisting of a prepubic fistulous tract that classically does not communicate with the genitourinary system. Previous studies centered on its immunohistochemical characterization have shown inconsistent results, and the etiology has not been clarified. We present the case of a 2-year-old male who presented since birth with a fistulous orifice on the dorsum of the penis. He had no associated symptoms. Under general anesthesia, the fistulous tract was explored, and methylene blue was instilled through it. After cystoscopically verifying the absence of communication with the urethra, a complete resection of the lesion was performed. The immunohistochemical study showed positivity for low and high molecular weight keratins and a transitional pattern for keratin 7 and GATA3, with positivity at cul de sac level and negativity at proximal level. These findings suggest that this lesion is an incomplete dorsal duplication variant.

Glans Penis Ischemia after Circumcision in a 15-Year-Old Patient Managed with Intravenous Pentoxifylline: A Case Report and a Diagnostic-Therapeutic Guideline Proposal.

Glans ischemia is an extremely infrequent complication characterized by a total or partial compromise in the penile arterial perfusion. A 15-year-old male patient suffered an episode of ischemia in the glans penis post-circumcision 24 h after surgery. Intravenous treatment with continuous perfusion of pentoxifylline was started for 4 days, with favorable evolution. Complete resolution was observed with no sequelae. There is no consensus on the best therapeutic management. The favorable evolution reported in most of the cases despite different therapeutic approaches leads us to think that the role of the treatments proposed so far is probably less than we believe. Additionally, we present a proposal for a diagnostic and therapeutic guide for this entity. Although the evidence in the literature is scarce and this guideline should be interpreted with caution, we believe that it can constitute a support resource for cases similar to ours.

Diagnostic performance of serum pentraxin-3 in pediatric acute appendicitis: a prospective diagnostic validation study.

INTRODUCTION: Pediatric acute appendicitis (PAA) is a pathology with a high rate of diagnostic error. The search for new diagnostic tools is justified by the high morbidity and healthcare costs associated with diagnostic error. METHODS: We designed a prospective study to validate serum pentraxin-3 (PTX3) as a diagnostic tool in PAA. Participants were divided into three groups: (1) patients with no underlying pathology (2) patients with non-surgical abdominal pain and (3) patients with a confirmed diagnosis of PAA. For further analyses, patients in group 3 were divided into complicated or uncomplicated PAA. Quantitative variables were expressed as medians and interquartile ranges and categorical variables as percentages. Quantitative variables were compared using the Kruskal-Wallis test and the Mann-Whitney U test. Diagnostic performance was evaluated with ROC curves. RESULTS: This study included 215 patients divided into group 1 (n = 63), group 2 (n = 53) and group 3 (n = 99). Median serum PTX3 values were 2.54 (1.70-2.95) ng/mL, 3.29 (2.19-7.64) ng/mL and 8.94 (6.16-14.05) in groups 1, 2 and 3, respectively (p = 0.001). Patients with complicated PAA showed significantly higher values than patients with uncomplicated PAA (p = 0.04). The AUC (group 2 vs. 3) was 0.77 (95% CI 0.69-0.85) and the best cut-off point was at 7.28 ng/mL, with a sensitivity of 61.3% and a specificity of 73.1%. The AUC (complicated vs. uncomplicated PAA) was 0.65 (95% CI 0.54-0.77) and the best cut-off point was 12.33 ng/mL, with a sensitivity of 51.72% and a specificity of 72.73%. CONCLUSIONS: The diagnostic ability of serum PTX3 in PAA is only moderate and therefore it cannot be considered a definitive diagnostic test. The discriminatory ability of PTX3 between complicated and uncomplicated PAA is poor. These findings, which contrast with those reported to date, should be validated with future properly designed prospective studies.

Alterations and diagnostic performance of capillary ketonemia in pediatric acute appendicitis: a pilot study.

INTRODUCTION: The diagnostic performance of capillary ketonemia (CK) has been previously evaluated in context of pediatric acute gastroenteritis. To our knowledge, there is no literature on its performance in the setting of pediatric acute appendicitis (PAA). MATERIALS AND METHODS: In this study, 151 patients were prospectively included and divided into two groups: (1) patients with non-surgical abdominal pain in whom the diagnosis of PAA was excluded (n = 53) and (2) patients with a confirmed diagnosis of PAA (n = 98). In 80 patients (Group 1, n = 23 and group 2, n = 57) a CK was measured at the time of diagnosis. The PAA group was further classified into complicated (n = 18) and uncomplicated PAA (n = 39). Quantitative variables were compared between groups using the Mann-Whitney U test. Diagnostic performance of CK was evaluated with ROC curves. RESULTS: CK values were 0.3 [0.1-0.9] mmol/L in group 1 and 0.7 [0.4-1.4] mmol/L in group 2 (p = 0.01). Regarding the type of PAA, CK values were 0.6 [0.4-0.9] mmol/L in uncomplicated PAA and 1.2 [0.8-1.4] mmol/L in complicated PAA (p = 0.02). The AUC for the discrimination between groups 1 and 2 was 0.68 (95% IC 0.53-0.82) (p = 0.24) and the AUC for the discrimination between uncomplicated PAA and complicated PAA was 0.69 (95% IC 0.54-0.85) (p = 0.04). The best cut-off point (group 1 vs group 2) resulted in 0.4 mmol/L, with a sensitivity of 80.7% and a specificity of 52.2%. The best cut-off point (non-complicated vs complicated PAA) resulted in 1.1 mmol/L, with a sensitivity of 61.1% and a specificity of 76.9%. CONCLUSIONS: This study found significantly higher levels of CK in patients with PAA than in those with NSAP. Similarly, significantly higher levels were observed in patients with complicated than in those with uncomplicated PAA. Nevertheless, the diagnostic performance of CK was only moderate in the two settings analyzed. The potential usefulness of CK determination as a tool to guide the preoperative rehydration regimen of patients with PAA to prevent postoperative hyporexia and vomiting is a promising line of research and should be evaluated in future studies.