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1.
J Pediatr ; 269: 113963, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38369237

RESUMO

OBJECTIVE: To evaluate for disparities in surgical care among US children with hepatoblastoma (HB) and hepatocellular carcinoma (HCC). STUDY DESIGN: In this retrospective National Cancer Database study (2004-2015), children aged <18 years with HB or HCC were included. Multivariable mixed-effects logistic regression was used to evaluate the association of sociodemographic factors (age, sex, race and ethnicity, insurance status, income, proximity to treating hospital) with the odds of undergoing surgical treatment after adjusting for disease-related factors (tumor size, metastasis, comorbidities) and hospital-level effects. Subgroup analyses by tumor histology were performed. RESULTS: A total of 811 children were included (HB: 80.9%; HCC: 19.1%), of which 610 (75.2%) underwent surgical treatment. Following adjustment, decreased odds of undergoing surgical treatment were associated with Black race (OR: 0.46 vs White, 95% CI [95% CI]: 0.26-0.80, P = .01), and having Medicaid (OR: 0.58 vs private, 95% CI: 0.38-0.88, P = .01) or no insurance (OR: 0.33 vs private, 95% CI: 0.13-0.80, P = .02). In children with HB, Black race was associated with decreased odds of undergoing surgical treatment (OR: 0.47 vs White, 95% CI: 0.25-0.89, P = .02). In children with HCC, Medicaid (OR: 0.10 vs private, 95% CI: 0.03-0.35, P < .001), or no insurance status (OR: 0.10 vs private, 95% CI: 0.01-0.83, P = .03) were associated with decreased odds of undergoing surgical treatment. Other than metastatic disease, no additional factors were associated with likelihood of surgical treatment in any group. CONCLUSIONS: Black race and having Medicaid or no insurance are independently associated with decreased odds of surgical treatment in children with HB and HCC, respectively. These children may be less likely to undergo curative surgery for their liver cancer.


Assuntos
Carcinoma Hepatocelular , Disparidades em Assistência à Saúde , Hepatoblastoma , Neoplasias Hepáticas , Humanos , Hepatoblastoma/cirurgia , Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/patologia , Masculino , Feminino , Criança , Estudos Retrospectivos , Pré-Escolar , Lactente , Estados Unidos , Disparidades em Assistência à Saúde/estatística & dados numéricos , Disparidades em Assistência à Saúde/etnologia , Adolescente , Fatores Sociodemográficos , Medicaid/estatística & dados numéricos , Fatores Socioeconômicos , Bases de Dados Factuais
2.
Pediatr Surg Int ; 40(1): 76, 2024 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-38466447

RESUMO

BACKGROUND: Pneumatosis intestinalis (PI, presence of air in bowel wall) develops in a variety of settings and due to a variety of insults which is then characterized by varying severity and clinical course. Anecdotally, many of these cases are benign with few clinical sequelae; however, we lack evidence-based guidelines to help guide management of such lower-risk cases. We aimed to describe the clinical entity of low-risk PI, characterize the population of children who develop this form of PI, determine if management approach or clinical outcomes differed depending on the managing physician's field of practice, and finally determine if a shortened course of NPO and antibiotics was safe in the population of children with low-risk PI. METHODS: We performed a retrospective review of all children over age 1 year treated at Children's Hospital Colorado (CHCO), between 2009 and 2019 with a diagnosis of PI who did not also have a diagnosis of cancer or history of bone marrow transplant (BMT). Data including demographic variables, clinical course, and outcomes were obtained from the electronic medical record. Low-risk criteria included no need for ICU admission, vasopressor use, or urgent surgical intervention. RESULTS: Ninety-one children were treated for their first episode of PI during the study period, 72 of whom met our low-risk criteria. Among the low-risk group, rates of complications including hemodynamic decompensation during treatment, PI recurrence, Clostridium difficile colitis, and death did not differ between those who received 3 days or less of antibiotics and those who received more than 3 days of antibiotics. Outcomes also did not differ between children cared for by surgeons or pediatricians. CONCLUSIONS: Here, we define low-risk PI as that which occurs in children over age 1 who do not have a prior diagnosis of cancer or prior BMT and who do not require ICU admission, vasopressor administration, or urgent surgical intervention. It is likely safe to treat these children with only 3 days of antibiotic therapy and NPO. LEVEL OF EVIDENCE: Level III.


Assuntos
Neoplasias , Pneumatose Cistoide Intestinal , Criança , Humanos , Lactente , Estudos Retrospectivos , Fatores de Risco , Progressão da Doença , Neoplasias/complicações , Antibacterianos/uso terapêutico , Pneumatose Cistoide Intestinal/diagnóstico , Pneumatose Cistoide Intestinal/cirurgia
3.
Genet Med ; 25(3): 100348, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36571464

RESUMO

PURPOSE: RAS genes (HRAS, KRAS, and NRAS) are commonly found to be mutated in cancers, and activating RAS variants are also found in disorders of somatic mosaicism (DoSM). A survey of the mutational spectrum of RAS variants in DoSM has not been performed. METHODS: A total of 938 individuals with suspected DoSM underwent high-sensitivity clinical next-generation sequencing-based testing. We investigated the mutational spectrum and genotype-phenotype associations of mosaic RAS variants. RESULTS: In this article, we present a series of individuals with DoSM with RAS variants. Classic hotspots, including Gly12, Gly13, and Gln61 constituted the majority of RAS variants observed in DoSM. Furthermore, we present 12 individuals with HRAS and KRAS in-frame duplication/insertion (dup/ins) variants in the switch II domain. Among the 18.3% individuals with RAS in-frame dup/ins variants, clinical findings were mainly associated with vascular malformations. Hotspots were associated with a broad phenotypic spectrum, including vascular tumors, vascular malformations, nevoid proliferations, segmental overgrowth, digital anomalies, and combinations of these. The median age at testing was higher and the variant allelic fraction was lower in individuals with in-frame dup/ins variants than those in individuals with mosaic RAS hotspots. CONCLUSION: Our work provides insight into the allelic and clinical heterogeneity of mosaic RAS variants in nonmalignant conditions.


Assuntos
Mosaicismo , Malformações Vasculares , Humanos , Proteínas Proto-Oncogênicas p21(ras)/genética , Mutação , Alelos , Malformações Vasculares/genética
4.
Pediatr Surg Int ; 39(1): 203, 2023 May 23.
Artigo em Inglês | MEDLINE | ID: mdl-37219695

RESUMO

BACKGROUND: We aimed to identify prognostic indicators in pneumatosis intestinalis (PI) in a pediatric oncology population. We hypothesized that neutropenia would be an independent risk factor for adverse outcomes, including the need for abdominal operation to treat PI and for the development of recurrent PI. METHODS: We performed a retrospective review of all patients treated for PI between 2009 and 2019 with a diagnosis of cancer or history of bone marrow transplant (BMT). RESULTS: Sixty-eight children were treated for their first episode of PI; 15 (22%) were not neutropenic at presentation; eight underwent urgent abdominal operation (12%). Patients with neutropenia were more likely to receive TPN, had a longer course of NPO, and received a longer course of antibiotics. Neutropenia at presentation was associated with a decreased risk of PI recurrence (40% vs 13%, p = 0.03). Children who required an abdominal operation were more likely to require vasopressors at diagnosis (50% vs 10%, p = 0.013). CONCLUSIONS: Among pediatric cancer patients, need for vasopressors at the time of PI is a marker of severe PI, with increased likelihood of requiring operative intervention. The presence of neutropenia is associated with lower rates of PI recurrence. LEVEL OF EVIDENCE: Level III.


Assuntos
Neoplasias , Neutropenia , Criança , Humanos , Antibacterianos , Pacientes , Fatores de Risco
5.
J Pediatr ; 248: 81-88.e1, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35605646

RESUMO

OBJECTIVE: To evaluate the effect of the RAS-MAPK pathway inhibitor trametinib on medically refractory chylous effusions in 3 hospitalized patients with Noonan syndrome. STUDY DESIGN: Pharmacologic MEK1/2 inhibition has been used to treat conditions associated with Noonan syndrome, given that activation of RAS-MAPK pathway variants leads to downstream MEK activation. We describe our experience with 3 patients with Noonan syndrome (owing to variants in 3 distinct genes) and refractory chylous effusions treated successfully with MEK inhibition. A monitoring protocol was established to standardize medication dosing and monitoring of outcome measures. RESULTS: Subjects demonstrated improvement in lymphatic leak with additional findings of improved growth and normalization of cardiac and hematologic measurements. Trametinib was administered safely, with only moderate skin irritation in 1 subject. CONCLUSIONS: Improvements in a variety of quantifiable measurements highlight the potential utility of MEK1/2 inhibition in patients with Noonan syndrome and life-threatening lymphatic disease. Larger, prospective studies are needed to confirm efficacy and assess long-term safety.


Assuntos
Antineoplásicos , Síndrome de Noonan , Criança , Humanos , Quinases de Proteína Quinase Ativadas por Mitógeno , Síndrome de Noonan/complicações , Síndrome de Noonan/tratamento farmacológico , Síndrome de Noonan/genética , Piridonas/uso terapêutico , Pirimidinonas/uso terapêutico
6.
Pediatr Blood Cancer ; 69 Suppl 3: e29246, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-36070215

RESUMO

Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency, and immunodeficiency. Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusions and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.


Assuntos
Quilotórax , Ascite Quilosa , Transtornos Respiratórios , Ascite/diagnóstico , Ascite/etiologia , Ascite/terapia , Criança , Quilotórax/diagnóstico , Quilotórax/terapia , Ascite Quilosa/diagnóstico , Ascite Quilosa/etiologia , Ascite Quilosa/terapia , Drenagem , Humanos
7.
Pediatr Surg Int ; 38(12): 1965-1970, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36242600

RESUMO

PURPOSE: Pneumatosis intestinalis (PI) remains difficult to treat as it can lead to a broad range of clinical sequalae and there are little published data available to guide management. Our aim was to evaluate how pediatric surgeons currently manage children with PI, how treatment varies based on etiology, and to identify opportunities to optimize current PI management strategies. METHODS: We administered a web-based survey of practicing pediatric surgeons in the United States and Canada. The survey was distributed to all members of the American Pediatric Surgical Association. RESULTS: Of 1508 distributed surveys, 333 responses were received (22% response rate); 174 were complete and included in analysis (12% analyzed). For all scenarios, respondents recommended treatment for PI include a median 7 days of bowel rest and 7 days antibiotics. Only 41% reported their approach to PI management was optimal. Ways to optimize care include treatment based on etiology (83%), decreased number of repeat images (64%), shorter NPO course (49%), and shorter antibiotic course (47%). CONCLUSION: Pediatric surgeons manage PI similarly regardless of etiology but most report this is suboptimal. Future work is needed to prospectively evaluate management protocols that consider etiology.


Assuntos
Pneumatose Cistoide Intestinal , Cirurgiões , Criança , Humanos , Estados Unidos , Pneumatose Cistoide Intestinal/cirurgia , Pneumatose Cistoide Intestinal/tratamento farmacológico , Inquéritos e Questionários , Intestinos , Antibacterianos/uso terapêutico
8.
Emerg Radiol ; 29(5): 903-914, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35678950

RESUMO

PURPOSE: While interventional radiologists occupy a critical role in adult trauma management, the role of interventionalist in pediatric trauma continues to evolve. The indications for transarterial embolization (TAE) are significantly different in pediatric patients in whom non-operative management (NOM) has a much more prominent role than in adults. Contrast extravasation on imaging may not require acute surgical or interventional management as it would in an adult. There are also areas in which pediatric interventional radiology is increasingly useful such as pelvic TAE in failed management, or splenic embolization to treat bleeding without the loss of splenic function inherent to surgical splenectomy. The rapid evolution of techniques and devices in pediatric patients is also changing what interventions are possible in pediatric trauma management which necessitates frequent reassessment of the guidelines and interventional radiology's role in caring for these patients. CONCLUSION: This review seeks to consolidate the recent literature to describe the evolving role of the interventional radiologist in pediatric trauma management.


Assuntos
Papel Profissional , Radiologistas , Ferimentos e Lesões , Criança , Embolização Terapêutica/métodos , Humanos , Baço/diagnóstico por imagem , Baço/lesões , Baço/cirurgia , Esplenectomia , Ferimentos e Lesões/diagnóstico por imagem , Ferimentos e Lesões/terapia
9.
J Surg Res ; 233: 256-261, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30502256

RESUMO

BACKGROUND: Lymphatic malformations (LMs) are congenital and arise from errors in vascular embryogenesis. LMs are categorized by cyst size as microcystic, macrocystic, or combined. Abdominal LMs are rare. Surgical resection of abdominal LMs has been the mainstay of therapy, but recurrence and morbidity are high. We sought to determine the effectiveness of sclerotherapy treatment for abdominal LM. METHODS: A single-center, retrospective review from 2014 to 2018 was conducted evaluating pediatric patients with abdominal LM. RESULTS: Ten patients were included, n = 9 had macrocystic LM and one patient had combined disease. The average age at first treatment was 6.8 y. The most common presenting symptoms were abdominal distention, pain, infection, and anemia. Preprocedural imaging was performed for all patients; median pretreatment volume was 1572.9 cm3 (range, 67.2-13,226.4). LMs were accessed using ultrasound guidance and injected with opacified doxycycline. Patients received a mean of 7.1 sclerotherapy injections. Complications included intraperitoneal doxycycline extravasation (n = 1), managed conservatively, and LM infection (n = 1), treated with intravenous antibiotics and drainage. One patient went on to surgical resection due to inability gain stable intracystic access; follow-up ultrasonography showed no recurrence. Postprocedural imaging was available in n = 8. Volume decreased by 96.7% after sclerotherapy. The median remaining volume was 0 cm3 (range, 0-599.7) (P = 0.016). Postsclerotherapy magnetic resonance imaging was obtained in n = 6, with complete resolution in 83.3%. All patients had resolution of presenting symptoms. Follow-up duration was 12.3 mo. CONCLUSIONS: Initial results demonstrate that sclerotherapy is an effective and durable treatment for symptom resolution and volume reduction of abdominal LM.


Assuntos
Doxiciclina/administração & dosagem , Anormalidades Linfáticas/terapia , Escleroterapia/métodos , Prevenção Secundária/métodos , Cavidade Abdominal/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Extravasamento de Materiais Terapêuticos e Diagnósticos/epidemiologia , Extravasamento de Materiais Terapêuticos e Diagnósticos/etnologia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Anormalidades Linfáticas/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Recidiva , Estudos Retrospectivos , Escleroterapia/efeitos adversos , Resultado do Tratamento , Ultrassonografia de Intervenção
11.
Pediatr Surg Int ; 34(8): 857-860, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29876644

RESUMO

BACKGROUND/PURPOSE: The utility of EDT in the adult trauma population, using well-defined guidelines, is well established, especially for penetrating injuries. Since the introduction of these guidelines, reports on the use of EDT for pediatric trauma have been published, and these series reveal a dismal, almost universally fatal, outcome for EDT following blunt trauma in the child. This report reviews the clinical outcomes of EDT in the pediatric population. MATERIALS/METHODS: We performed a review of EDT in the pediatric population using the published data from 1980 to 2017. Variables extracted included mechanism of injury and mortality. To minimize bias, single case reports were not included in the review. RESULTS: Upon review of four decades of published literature on the use of emergency department thoracotomy (EDT) in the pediatric population, mortality rates are comparable between adults and pediatric patients for penetrating thoracic trauma. In contrast, in pediatric patients sustaining blunt trauma, no patient under the age of 15 has survived. CONCLUSION: In patients between 0 and 14 years of age presenting with no signs of life following blunt trauma, withholding EDT should be considered. Patients between the ages of 15 and 18 should be treated in accordance with adult ATLS principles for the management of thoracic trauma. LEVEL OF EVIDENCE: Level IV.


Assuntos
Serviço Hospitalar de Emergência , Traumatismos Torácicos/mortalidade , Toracotomia , Ferimentos não Penetrantes/mortalidade , Ferimentos Penetrantes/mortalidade , Parada Cardíaca/etiologia , Humanos , Ressuscitação/métodos , Traumatismos Torácicos/terapia , Ferimentos não Penetrantes/terapia , Ferimentos Penetrantes/terapia
12.
J Surg Res ; 199(1): 149-52, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25972312

RESUMO

BACKGROUND: Recent progress has been made in the care of infants with hypertrophic pyloric stenosis (HPS), including earlier operative intervention and shorter hospital length of stay (LOS), which is attributable to expedited postoperative feeding protocols developed and implemented by surgeons. We hypothesized that patients with HPS admitted to a unit that is co-managed by nonsurgeon providers postoperatively have a longer LOS than those on the surgical ward. MATERIALS AND METHODS: We reviewed the medical records of infants who underwent pyloromyotomy for HPS at a single institution from April, 2009-July, 2013. RESULTS: A total of 259 patients underwent pyloromyotomy (35 female; 13.5%), 205 (79%) were admitted to the surgical ward; 46 had a planned neonatal intensive care unit (NICU) admission (18%) and were co-managed with the neonatology team. Eight (3%) had an unplanned NICU admission and were excluded from the analysis. The groups did not differ in terms of sex, age, serum electrolytes at presentation, or time between surgeon evaluation and operative intervention. Surgical ward patients had longer preoperative symptom duration. Operative time was longer in the NICU patients. Comparing the two groups, there was no difference in postoperative apnea, hypoxic, or bradycardic episodes. NICU patients achieved ad libitum feeds later than floor patients (2.0 versus 1.4 d; P = 0001) and had a longer postoperative LOS (2.2 versus 1.6 d; P = 0.0012). CONCLUSIONS: Patients with HPS admitted to the NICU postoperatively had a longer time to full feeds and hospital LOS. The reduction in LOS between hospital wards may be improved with implementation of a hospital-wide postoperative protocol for patients with HPS.


Assuntos
Unidades de Terapia Intensiva Neonatal , Tempo de Internação/estatística & dados numéricos , Cuidados Pós-Operatórios/métodos , Estenose Pilórica Hipertrófica/cirurgia , Centro Cirúrgico Hospitalar , Protocolos Clínicos/normas , Nutrição Enteral/métodos , Nutrição Enteral/estatística & dados numéricos , Feminino , Humanos , Lactente , Recém-Nascido , Unidades de Terapia Intensiva Neonatal/normas , Masculino , Cuidados Pós-Operatórios/normas , Cuidados Pós-Operatórios/estatística & dados numéricos , Centro Cirúrgico Hospitalar/normas , Resultado do Tratamento
13.
Pediatr Dermatol ; 32(6): 808-12, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26223454

RESUMO

OBJECTIVES: Hepatic hemangiomas are often found in association with multiple cutaneous infantile hemangiomas. Screening abdominal ultrasonography has been recommended for patients with five or more cutaneous lesions. We sought to determine whether hemangiomas found through screening had improved clinical outcomes. METHODS: Patients entered into our hepatic hemangioma registry between 1995 and 2012 were reviewed. RESULTS: Seventy-two patients with multiple cutaneous and hepatic hemangiomas were identified; 43 (60%) were detected through screening. The median age at diagnosis was 41 days for screened patients and 53 days for those not screened. Screening detected 40 (93%) multifocal and 3 (7%) diffuse hemangiomas, compared to 18 (62%) and 11 (38%), respectively, in the nonscreened group. Patients identified by screening had lower incidences of congestive heart failure and hypothyroidism and were less likely to receive treatment for their hemangiomas. The mortality rate in the children not screened was 28% (n = 8). None of the patients found by screening died (p < 0.001). Multivariate analysis of treated patients demonstrated that screening was a significant predictor of reduced mortality (p = 0.04). CONCLUSION: Hepatic hemangiomas found through screening ultrasonography are less likely to develop serious clinical sequelae. Although the reasons for this may include detection of hemangiomas that are less likely to progress to symptomatic disease, it appears that it also allows for earlier intervention for more concerning (e.g. diffuse) subtypes. Screening may allow for closer surveillance and earlier treatment before life-threatening progression in a subset of infants with liver hemangiomas, preventing complications and reducing mortality.


Assuntos
Hemangioma Capilar/epidemiologia , Hemangioma/epidemiologia , Neoplasias Hepáticas/epidemiologia , Triagem Neonatal/métodos , Sistema de Registros , Neoplasias Cutâneas/epidemiologia , Estudos de Coortes , Comorbidade , Feminino , Seguimentos , Hemangioma/diagnóstico , Hemangioma Capilar/congênito , Hemangioma Capilar/diagnóstico , Hospitais Pediátricos , Humanos , Incidência , Lactente , Recém-Nascido , Neoplasias Hepáticas/diagnóstico , Masculino , Monitorização Fisiológica , Análise Multivariada , Valor Preditivo dos Testes , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Neoplasias Cutâneas/diagnóstico , Taxa de Sobrevida
14.
J Craniofac Surg ; 23(5): 1495-8, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22976644

RESUMO

Variable resorption occurs whenever calvarial bone graft is used for onlay cranioplasty. The recipient ectocortex may be burred to expose vessels and osteocytes to maximize healing. The purpose of this study was to determine whether abrading the recipient site improves the volume of onlay graft. The parietal bones of 17 rabbits were sectioned into split-thickness and full-thickness grafts. The right frontal cortex was abraded with a bur to punctate bleeding. Pairs of split-thickness (n = 48) or full-thickness (n = 20) grafts were onlayed to the burred right frontal bone and to the nonburred left frontal bone. Micro-computed tomography was used to determine graft volume immediately postoperatively and 16 weeks later. Histology, including tartrate-resistant acid phosphatase staining, was performed to quantify vascular channels and osteoclasts per high-power field 10 days postoperatively. Split-thickness graft volume decreased 58.0% when placed on the burred calvarial site, compared with grafts on the nonburred cortex (28.4%) (P = 0.01). Full-thickness grafts showed a similar trend: greater resorption (39.1%) when onlayed onto abraded calvaria compared with nonburred ectocortex (26.0%) (P = 0.11). Split-thickness graft orientation (cortical vs cancellous side in contact with the recipient site) did not affect resorption (P = 0.67). Onlay grafts placed on the burred recipient site had more vascular channels (11.8) and osteoclasts (5.7), compared with grafts over nonabraded cortex (3.4 and 4.2, respectively) (P < 0.05). Burring the recipient site cortex before onlay cranial bone grafting promotes resorption, possibly by increasing vascularization and osteoclastic activity. This technique cannot be recommended.


Assuntos
Reabsorção Óssea , Transplante Ósseo/métodos , Craniotomia/métodos , Osso Frontal/cirurgia , Osso Parietal/transplante , Animais , Osso Frontal/diagnóstico por imagem , Osso Parietal/diagnóstico por imagem , Coelhos , Microtomografia por Raio-X
15.
J Pediatr Surg ; 57(6): 1067-1071, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35264304

RESUMO

BACKGROUND: There is a paucity of data on the frequency of transfusion during pediatric surgery index cases and guidelines for pretransfusion testing, defined as type and screen and crossmatch testing, prior to operation are not standardized. This study aimed to determine the incidence of perioperative blood transfusions during index neonatal operations and identify risk factors associated with perioperative blood transfusion to determine which patients benefit from pretransfusion testing. METHODS: A retrospective review of infants who underwent index neonatal cases between 2013 and 2019 was performed. Data were collected for patients who underwent operations for Hirschsprung's disease, esophageal atresia/tracheoesophageal fistula (EA/TEF), biliary atresia, anorectal malformation, omphalocele, gastroschisis, duodenal atresia, congenital diaphragmatic hernia (non-ECMO) or pulmonary lobectomy. Infants under 6 months were included except in the case of lobectomy where infants up to 12 months were included. RESULTS: Analysis was performed on 420 patients. Twenty-five (6.0%) patients received perioperative blood transfusion. Patients who received perioperative transfusion most commonly underwent EA/TEF repair. Patients who received perioperative transfusion had higher rates of structural heart disease (52.0% vs 17.7%, p<0.001), preoperative transfusion (48.0% vs 8.9%, p<0.001), and prematurity (52.0% vs 25.6%, p = 0.005). Presence of all three risk factors resulted in a 48% probability of requiring perioperative transfusion. CONCLUSIONS: Blood transfusion during the perioperative period of neonatal index operations is rare. Factors associated with increased risk of perioperative transfusion include prematurity, structural heart disease, and history of previous blood transfusion. LEVEL OF EVIDENCE: III.


Assuntos
Transfusão de Sangue , Anormalidades Congênitas , Criança , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/terapia , Atresia Esofágica/complicações , Atresia Esofágica/cirurgia , Cardiopatias/congênito , Cardiopatias/cirurgia , Humanos , Incidência , Lactente , Recém-Nascido , Período Perioperatório , Estudos Retrospectivos , Fatores de Risco , Fístula Traqueoesofágica/complicações , Fístula Traqueoesofágica/epidemiologia , Fístula Traqueoesofágica/cirurgia
16.
J Craniofac Surg ; 22(1): 319-23, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21239926

RESUMO

The purpose of this study was to determine whether particulate bone graft maintains its volume when used for onlay cranioplasty. Twenty-five adult, male, New Zealand white rabbits were divided into 5 groups (n = 5/group). Groups 1 to 3 were controls: group 1, untreated; group 2, sham procedure; and group 3, burring the cortical surface. Group s 4 and 5 had augmentation of the parietal bones with particulate graft harvested from the frontal bone with a brace and bit. The particulate graft was placed on native parietal bone (group 4) or on parietal bone that had been abraded to punctuate bleeding with an electric burr (group 5). Volume maintenance and osseointegration of the grafts were determined by micro-computed tomography and histology. At 16 weeks postoperatively, the mean (SD) volumes of the parietal bones in control groups 1, 2, and 3 were 555.8 (29.2), 550.8 (36.8), and 539.0 (39.0) mm, respectively. Immediately after cranioplasty, the mean (SD) volumes of augmented parietal bone were 846.0 (10.8) mm for group 4 and 831.8 (11.8) mm for group 5. Sixteen weeks postoperatively, 100% of the group 4 grafts had resorbed (551.8 [SD, 24.0] mm), and parietal volume was no different from controls (P = 0.89). Group 5 maintained 54.2% of volume (695.6 [SD, 22.0] mm), which was greater than those of the controls (P < 0.0001). Particulate graft may be used for onlay cranioplasty if the recipient site is burred. Approximately one half of the onlay graft is resorbed, and its original shape is not maintained.


Assuntos
Transplante Ósseo/métodos , Craniotomia/métodos , Osso Frontal/transplante , Osso Parietal/cirurgia , Animais , Adesivo Tecidual de Fibrina , Osso Frontal/diagnóstico por imagem , Masculino , Osseointegração , Osso Parietal/diagnóstico por imagem , Coelhos , Estatísticas não Paramétricas , Microtomografia por Raio-X
17.
J Craniofac Surg ; 22(6): 2405-8, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22134293

RESUMO

Facial infiltrating lipomatosis causes diffuse overgrowth of subcutaneous fat, muscle, and bone. Because adipose tissue mass is angiogenesis dependent, the purpose of this study was to determine whether neovascularization is upregulated in this disease.Infiltrating lipomatosis tissue was collected prospectively from the preauricular cheek of 5 patients; neovascularization was compared to normal postauricular adipose. Specimens were analyzed using immunofluorescence for CD31 (microvascular density), α-smooth muscle actin (pericyte marker), CD31/Ki67 (proliferating endothelial cells), and CD34/CD133 (endothelial progenitor cells). Quantitative reverse transcription-polymerase chain reaction was used to determine messenger RNA expression of progenitor cells (CD133) and factors that recruit them: vascular endothelial growth factor (VEGF-A), hypoxia-inducible factor 1α, matrix metalloproteinase 9 (MMP-9), and stromal cell-derived factor 1α. Angiopoietin 1 and 2, MMP-2, VEGF receptors, and neuropilin receptors were quantified using quantitative reverse transcription polymerase chain reaction. There was no difference in microvascular density, pericytic density, or endothelial proliferation between infiltrating lipomatosis and normal adipose tissue (P = 0.2). Expressions of VEGF-A, hypoxia-inducible factor 1α, stromal cell-derived factor 1α, angiopoietin 1 and 2, MMP-2 and -9, VEGF receptors 1 and 2, neuropilin receptors 1 and 2, and CD133 messenger RNA were not elevated compared to control fat (P = 0.1). Endothelial progenitor cells were not present in specimens of infiltrating lipomatosis. Infiltrating lipomatosis does not exhibit elevated angiogenic or vasculogenic factors compared to normal fat; the vasculature is stable. Neovascularization does not seem to play a role in the pathogenesis of this condition.


Assuntos
Biomarcadores/metabolismo , Bochecha , Lipomatose/metabolismo , Neovascularização Patológica/metabolismo , Adolescente , Criança , Pré-Escolar , Feminino , Imunofluorescência , Humanos , Lipomatose/patologia , Masculino , Neovascularização Patológica/patologia , Estudos Prospectivos , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Estatísticas não Paramétricas , Regulação para Cima , Adulto Jovem
18.
Semin Pediatr Surg ; 29(5): 150971, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33069296

RESUMO

Lymphatic malformations are low-flow vascular malformations that arise due to errors in vascular development. Lymphatic malformations are benign and usually noted at birth or in the first few years of life. Lymphatic mass lesions are composed of varying size of cysts; this article focuses on discussion of cystic lymphatic malformations. Lymphatic malformations can occur throughout the body especially in lymphatic rich areas such as the cervical and axillary locations as well as the groin, trunk, retroperitoneum, extremities, abdominal or thoracic cavities. Treatment options vary based upon size of cysts and location. A multimodal and interdisciplinary approach is essential to care for patients with lymphatic malformations. Management options include observation, pharmacotherapy, sclerotherapy, and surgical procedures.


Assuntos
Anormalidades Linfáticas/terapia , Malformações Vasculares/terapia , Humanos , Lactente , Anormalidades Linfáticas/complicações , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/patologia , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico , Malformações Vasculares/patologia
19.
Semin Pediatr Surg ; 28(3): 183-188, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31171155

RESUMO

Trauma is the leading cause of morbidity and mortality in the pediatric population. Due to a variety of factors, many pediatric trauma patients are initially evaluated and stabilized at adult hospitals that lack pediatric specific emergency medicine and surgical expertise. While similar to adult patients, the initial evaluation and resuscitation of pediatric patients does differ. Many of these key differences contribute to missed injury and susceptibility to error in the treatment of children. Here, we highlight a variety of differences between pediatric and adult trauma patients and clarify reasoning for these differences. Error traps that are discussed include missed cases of non-accidental trauma, missed blunt cerebrovascular injury, over use of CT (computed tomography) scans with unnecessary radiation exposure, missed small bowel or mesenteric injury, and unrecognized hemodynamic instability.


Assuntos
Lesões das Artérias Carótidas/diagnóstico , Maus-Tratos Infantis , Traumatismos Craniocerebrais/diagnóstico , Hipovolemia/diagnóstico , Intestinos/lesões , Erros Médicos , Segurança do Paciente , Medicina de Emergência Pediátrica/normas , Artéria Vertebral/lesões , Ferimentos não Penetrantes/diagnóstico , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Medicina de Emergência Pediátrica/métodos
20.
J Laparoendosc Adv Surg Tech A ; 28(12): 1517-1519, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30016184

RESUMO

Introduction: Laparoscopic duodenoduodenostomy can be performed to repair congenital duodenal obstructions from atresia or duodenal web. There are only a few published case series in the literature. We are reporting on a single surgeon's experience with the operation and discuss the technical aspects of the operation. Material and Methods: A retrospective chart review was performed using the electronic medical record identifying all patients who underwent laparoscopic duodenoduodenostomy or duodenojejunostomy at two institutions by a singular surgeon. Results: Fifteen patients were identified as having undergone laparoscopic duodenoduodenostomy from 2010 until 2017. The weight at the time of the operation ranged from 1.5 to 8.7 kg (median 2.5 kg). The age ranged from 0 days to 15 months (median 3 days). Operative time (including other procedures) ranged from 2 hours 10 minutes to 3 hours 45 minutes with a median of 2 hours 55 minutes. One case was converted to open due to poor visualization. One patient developed a stricture that required open anastomotic revision 4 weeks after the initial surgery. In 1 patient, an enterotomy in the first portion of the duodenum was created from a retraction stitch-this was immediately recognized and repaired by primary laparoscopic closure. One patient had a small anastomotic leak that was treated with antibiotics. There were no mortalities and no intraoperative blood loss requiring transfusion. Conclusion: Laparoscopic duodenoduodenostomy is an operation that can be performed with excellent outcomes following simple steps that are easily taught in a teaching setting.


Assuntos
Obstrução Duodenal/cirurgia , Duodenostomia/métodos , Duodeno/cirurgia , Laparoscopia/métodos , Anastomose Cirúrgica/métodos , Feminino , Humanos , Recém-Nascido , Masculino , Duração da Cirurgia , Estudos Retrospectivos , Resultado do Tratamento
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