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1.
Paediatr Anaesth ; 34(4): 289-292, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38130114

RESUMO

BACKGROUND: Surgical correction of tracheobronchomalacia (TBM) has evolved greatly over the past decade, with select pediatric institutions establishing dedicated surgery and anesthesia teams to navigate the complexities and challenges of surgical airway repairs. Although anesthetic techniques have evolved internally over many years to improve patient safety and outcomes, many of these methods remain undescribed in literature. TECHNIQUE: In this article, we describe the intraoperative negative pressure suction test. This simulates the negative pressure seen in awake and spontaneously breathing patients, including the higher pressures seen during coughing which induce airway collapse in patients with TBM. Also known as the Munoz maneuver in surgical literature, this test has been performed on over 300 patients since 2015. DISCUSSION: The negative pressure suction test allows for controlled intraoperative assessment of surgical airway repairs, replaces the need for risky intraoperative wake-up tests, increases the chances of a successful surgical repair, and improves anesthetic management for emergence and extubation. We provide a guide on how to perform the test and videos demonstrating its efficacy in intraoperative airway evaluation. CONCLUSIONS: As surgeries to repair TBM become more prevalent in other pediatric institutions, we believe that pediatric patients and anesthesia providers will benefit from the insights and methods described here.


Assuntos
Anestésicos , Traqueobroncomalácia , Humanos , Criança , Sucção , Traqueobroncomalácia/cirurgia , Respiração , Extubação
2.
J Pediatr ; 219: 160-166.e2, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31704054

RESUMO

OBJECTIVE: To determine the natural history of pulmonary function for survivors of congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a retrospective cohort study of survivors of CDH born during 1991-2016 and followed at our institution. A generalized linear model was fitted to assess the longitudinal trends of ventilation (V), perfusion (Q), and V/Q mismatch. The association between V/Q ratio and body mass index percentile as well as functional status was also assessed with a generalized linear model. RESULTS: During the study period, 212 patients had at least one V/Q study. The average ipsilateral V/Q of the cohort increased over time (P < .01), an effect driven by progressive reduction in relative perfusion (P = .012). A higher V/Q ratio was correlated with lower body mass index percentile (P < .001) and higher probability of poor functional status (New York Heart Association class III or IV) (P = .045). CONCLUSIONS: In this cohort of survivors of CDH with more severe disease characteristics, V/Q mismatch worsens over time, primarily because of progressive perfusion deficit of the ipsilateral side. V/Q scans may be useful in identifying patients with CDH who are at risk for poor growth and functional status.


Assuntos
Hérnias Diafragmáticas Congênitas/fisiopatologia , Pulmão/fisiopatologia , Relação Ventilação-Perfusão , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Adulto Jovem
3.
J Pediatr ; 216: 158-164.e2, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31704056

RESUMO

OBJECTIVE: To analyze longitudinal trends of pulmonary function testing in patients with congenital diaphragmatic hernia (CDH) followed in our multidisciplinary clinic. STUDY DESIGN: This was a retrospective cohort study of CDH patients born between 1991 and 2013. A linear mixed effects model was fitted to estimate the trends of percent predicted forced expiratory volume in 1 second (FEV1pp), percent predicted forced vital capacity (FVCpp), and FEV1/FVC over time. RESULTS: Of 268 patients with CDH who survived to discharge, 119 had at least 1 pulmonary function test study. The FEV1pp (P < .001), FVCpp (P = .017), and FEV1/FVC (P = .001) decreased with age. Compared with defect size A/B, those with defect size C/D had lower FEV1pp by an average of 11.5% (95% CI, 2.9%-20.1%; P = .010). A history of oxygen use at initial hospital discharge also correlated with decreased FEV1pp by an average of 8.0% (95% CI, 1.2%-15.0%; P = .023). CONCLUSIONS: In a select cohort of CDH survivors, average pulmonary function declines with age relative to expected population normative values. Those with severe CDH represent a population at risk for worsening pulmonary function test measurements who may benefit from recognition and monitoring for complications.


Assuntos
Volume Expiratório Forçado , Hérnias Diafragmáticas Congênitas/fisiopatologia , Capacidade Vital , Estudos de Coortes , Feminino , Humanos , Recém-Nascido , Estudos Longitudinais , Masculino , Testes de Função Respiratória , Estudos Retrospectivos
4.
J Surg Res ; 255: 549-555, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32640406

RESUMO

INTRODUCTION: The optimal method of esophageal replacement remains controversial. The aim of this study was to evaluate 30-d outcomes of children in the National Surgical Quality Improvement Project Pediatric (NSQIP-P) database who underwent esophageal replacement from 2012 to 2018. METHODS: Demographics, comorbidities, and procedural technique was identified in NSQIP-P and reviewed. Thirty-day outcomes were assessed and stratified by gastric pull-up or tube interposition versus small bowel or colonic interposition. Categorical and continuous variables were assessed by Pearson's chi-square, Fisher's exact, and Wilcoxon rank-sum tests, respectively. Multivariate logistic regression was performed to estimate the effects of procedure technique and clinical risk factors on patient outcomes. RESULTS: Of the 99 cases of esophageal replacement included, 52 (52.5%) utilized a gastric conduit, whereas 47 (47.5%) involved small bowel/colonic esophageal interposition. Overall risk of complications was 52.5%, the most common of which were perioperative transfusion (30.3%), surgical site infection (11.1%), and sepsis (9.1%). Risk of unplanned reoperation was 17.2%, and risk of mortality was 3.0%. Risk for complications, reoperation, and readmission did not differ significantly between those who underwent gastric esophageal replacement and those who underwent small bowel or colonic interposition. Median operative time was shorter in the gastric esophageal replacement group (5.2 versus 8.1 h, P = 0.009). CONCLUSIONS: Among children in NSQIP-P who underwent esophageal replacement from 2012 to 2018, the risk of 30-d complications, unplanned reoperation, and mortality was relatively frequent and was similar across operative techniques. Opportunities exist to improve preoperative optimization, utilization of blood transfusion services, and infectious complications in the perioperative period irrespective of operative technique. LEVEL OF EVIDENCE: Level III, retrospective comparative study.


Assuntos
Atresia Esofágica/cirurgia , Estenose Esofágica/cirurgia , Esofagoplastia/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Melhoria de Qualidade , Pré-Escolar , Colo/transplante , Bases de Dados Factuais , Atresia Esofágica/mortalidade , Estenose Esofágica/etiologia , Estenose Esofágica/mortalidade , Estenose Esofágica/patologia , Esofagoplastia/métodos , Esofagoplastia/estatística & dados numéricos , Esôfago/anormalidades , Esôfago/patologia , Esôfago/cirurgia , Feminino , Mortalidade Hospitalar , Humanos , Lactente , Intestino Delgado/transplante , Masculino , Duração da Cirurgia , Readmissão do Paciente/estatística & dados numéricos , Complicações Pós-Operatórias/etiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , Estômago/transplante , Resultado do Tratamento
5.
J Pediatr Gastroenterol Nutr ; 70(4): 462-467, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31764412

RESUMO

OBJECTIVES: The role of intralesional steroid injection (ISI) in the treatment of anastomotic stricture in patients with esophageal atresia remains unclear. The aim of this study was to evaluate the efficacy and safety of ISI. METHODS: A total of 158 patients with esophageal atresia with at least 1 ISI for the treatment of esophageal anastomotic stricture between 2010 and 2017 were identified. The change in stricture diameter (ΔD) was compared between procedures with dilation alone (ISI-) and dilation with steroid injection (ISI+). RESULTS: A total of 1055 balloon dilations were performed (452 ISI+). The median ΔD was significantly greater in the ISI+ group: 1 mm (interquartile range [IQR] 0, 3) versus 0 mm (IQR -1, 1.5) (P < 0.0001). The ISI+ group had greater percentage of improved diameter (P < 0.0001) and lesser percentages of unchanged and decreased diameters at subsequent endoscopy (P = 0.0009, P = 0.003). Multivariable logistic regression confirmed the significance of ISI on increasing the likelihood of improved stricture diameter with an adjusted odds ratio of 3.24 (95% confidence interval: 2.15-4.88) (P < 0.001). The ΔD for the first 3 ISI+ procedures was greater than the ΔD for subsequent ISI+ procedures: 1 mm (IQR 0, 3) versus 0.5 mm (IQR-1.25, 2) (P = 0.001). There was no difference in perforation incidence between ISI+ and ISI- groups (P = 0.82). CONCLUSIONS: ISI with dilation was well tolerated and improved anastomotic stricture diameter more than dilation alone. The benefit of ISI over dilation alone was limited to the first 3 ISI procedures.


Assuntos
Atresia Esofágica , Estenose Esofágica , Constrição Patológica/etiologia , Constrição Patológica/terapia , Atresia Esofágica/cirurgia , Estenose Esofágica/etiologia , Estenose Esofágica/terapia , Humanos , Complicações Pós-Operatórias/tratamento farmacológico , Estudos Retrospectivos , Esteroides , Resultado do Tratamento
6.
J Pediatr Gastroenterol Nutr ; 69(2): 163-170, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30921254

RESUMO

OBJECTIVE: Esophagitis is highly prevalent in patients with esophageal atresia (EA). Peptic esophagitis has long been assumed to be the primary cause of esophagitis in this population, and prolonged acid suppressive medication usage is common; such treatment is of unknown benefit and carries potential risk. METHODS: To better understand the role of commonly used antireflux treatments in EA, we analyzed all patients with repaired EA who underwent endoscopy with biopsies at our institution between January 2016 and August 2018. Macroscopic erosive and histologic esophagitis on biopsy was graded per predefined criteria. Clinical characteristics including acid suppressive medication usage, type of EA and repair, presence of hiatal hernia, and history of fundoplication were reviewed. RESULTS: There were 310 unique patients (33.5% long gap EA) who underwent 576 endoscopies with biopsies during the study period. Median age at endoscopy was 3.7 years (interquartile range 21-78 months). Erosive esophagitis was found in 8.7% of patients (6.1% of endoscopies); any degree of histologic eosinophilia (≥1 eosinophil/high power field [HPF]) was seen in 56.8% of patients (48.8% of endoscopies), with >15 eosinophils/HPF seen in 15.2% of patients (12.3% of endoscopies). Acid suppression was common; 86.9% of endoscopies were preceded by acid suppressive medication use. Fundoplication had been performed in 78 patients (25.2%). Proton pump inhibitor (PPI) and/or H2 receptor antagonist (H2RA) use were the only significant predictors of reduced odds for abnormal esophageal biopsy (P = 0.011 for PPI, P = 0.048 for H2RA, and P = 0.001 for PPI combined with H2RA therapy). However, change in intensity of acid suppressive therapy by either dosage or frequency was not significantly associated with change in macroscopic erosive or histologic esophagitis (P > 0.437 and P > 0.13, respectively). Presence or integrity of a fundoplication was not significantly associated with esophagitis (P = 0.236). CONCLUSIONS: In EA patients, acid suppressive medication therapy is associated with reduced odds of abnormal esophageal biopsy, though histologic esophagitis is highly prevalent even with high rates of acid suppressive medication use. Esophagitis is likely multifactorial in EA patients, with peptic esophagitis as only one of multiple possible etiologies for esophageal inflammation. The clinical significance of histologic eosinophilia in this population warrants further investigation.


Assuntos
Atresia Esofágica , Esofagite Péptica/tratamento farmacológico , Antagonistas dos Receptores H2 da Histamina/uso terapêutico , Inibidores da Bomba de Prótons/uso terapêutico , Criança , Pré-Escolar , Esofagite Péptica/patologia , Esofagite Péptica/cirurgia , Feminino , Fundoplicatura , Antagonistas dos Receptores H2 da Histamina/administração & dosagem , Humanos , Lactente , Masculino , Inibidores da Bomba de Prótons/administração & dosagem
7.
J Surg Res ; 212: 146-152, 2017 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-28550900

RESUMO

BACKGROUND: Although commonly performed in adult swine, unilateral pneumonectomy in piglets requires significant modifications in the surgical approach and perioperative care because of their smaller size and limited physiological reserve. METHODS: Nineteen neonatal piglets underwent a left pneumonectomy. They were allowed 5-7 d of preoperative acclimation and nutritional optimization. Preoperative weight gain and laboratory values were obtained before the time of surgery. A "ventro-cranial" approach is adopted where components of the pulmonary hilum were sequentially identified and ligated, starting from the most ventral and cranial structure, the superior pulmonary vein. The principle of gentle ventilation was followed throughout the entire operation. RESULTS: The median age of the piglets at the time of surgery was 12 (10-12) d. The median preoperative weight gain and albumin level were 20% (16-26%) and 2.3 (2.1-2.4) g/dL, respectively. The median operative time was 59 (50-70) min. Five of the first nine piglets died from complications, two from poor preoperative nutritional optimization (both with <10% weight gain and 2 g/dL for albumin), one from an intubation complication, one from intra-operative bleeding, and one in the postoperative period from a ruptured bulla. No mortality occurred for the next 10 cases. CONCLUSIONS: Successful outcomes for unilateral pneumonectomy in piglets require special attention to preoperative nutritional optimization, gentle ventilation, and meticulous surgical dissection. Preoperative weight gain and albumin levels should be used to identify appropriate surgical candidates. The "ventro-cranial" approach allows for a technically straightforward completion of the procedure.


Assuntos
Modelos Animais , Assistência Perioperatória/métodos , Pneumonectomia/métodos , Suínos/cirurgia , Animais , Feminino
8.
Am J Hematol ; 90(3): 187-92, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25382665

RESUMO

The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005 to 2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤30 days after surgery), and long-term AEs (31-365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 g/dl, 52 week 12.8 ± 1.6 g/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process.


Assuntos
Síndrome Torácica Aguda/patologia , Anemia Hemolítica Congênita/cirurgia , Anemia Falciforme/cirurgia , Anquirinas/deficiência , Complicações Pós-Operatórias/patologia , Infecções Respiratórias/patologia , Esferocitose Hereditária/cirurgia , Esplenectomia/métodos , Síndrome Torácica Aguda/etiologia , Adolescente , Anemia Hemolítica Congênita/patologia , Anemia Falciforme/patologia , Bilirrubina/sangue , Criança , Pré-Escolar , Feminino , Hemoglobinas/metabolismo , Humanos , Masculino , Sistema de Registros , Infecções Respiratórias/etiologia , Reticulócitos/patologia , Esferocitose Hereditária/patologia , Resultado do Tratamento , Estados Unidos
9.
JPGN Rep ; 5(1): 74-78, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38545279

RESUMO

Endoscopic vacuum-assisted therapy offers an easier and safer alternative to thoracic surgery, self-expanding stents, or esophageal clips and has been shown to be a promising technique for management of pediatric esophageal perforations. In this report, we present a novel application of a percutaneous endoscopic gastrostomy-assisted pull technique, wherein a preexisting gastrostomy is reaccessed to allow safe placement of the vacuum sponge with a more comfortable and effective endoscopic vacuum-assisted closure therapy compared to transnasal or transoral options. A 7-year-old male with a history of type C esophageal atresia with distal tracheoesophageal fistula complicated by leak and refractory esophageal stricture, severe tracheomalacia, and prior esophageal stricture resection presented for posterior tracheoplasty and tracheopexy complicated by esophageal perforation. A preexisting gastrostomy site was re-accessed to allow for a novel approach for endoluminal sponge placement in endoscopic vacuum-assisted closure (EVAC) therapy by gastrostomy-assisted pull technique. The patient had appropriate healing without further leak 1 month after repair. This case highlights the use of EVAC as a minimally invasive option for repair of esophageal perforation using a pull-through method at the percutaneous endoscopic gastrostomy tube site as gastric access. This method may improve control of placement and reduce sponge migration, reduce intraluminal distance of sponge placement, and reduce morbidity by avoiding thoracotomy.

10.
J Pediatr Surg ; 59(1): 10-17, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37903674

RESUMO

PURPOSE: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.


Assuntos
Atresia Esofágica , Traqueobroncomalácia , Fístula Traqueoesofágica , Lactente , Recém-Nascido , Humanos , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Resultado do Tratamento , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Fístula Traqueoesofágica/cirurgia , Fístula Traqueoesofágica/complicações , Traqueobroncomalácia/complicações , Morbidade , Estudos Retrospectivos
11.
Pediatr Pulmonol ; 59(7): 1922-1931, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38629381

RESUMO

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.


Assuntos
Broncoscopia , Traqueobroncomalácia , Humanos , Traqueobroncomalácia/cirurgia , Traqueobroncomalácia/complicações , Masculino , Feminino , Lactente , Pré-Escolar , Resultado do Tratamento , Broncoscopia/métodos , Criança , Fístula Traqueoesofágica/cirurgia , Fístula Traqueoesofágica/complicações , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Estudos Retrospectivos
12.
JPGN Rep ; 4(4): e350, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38034456

RESUMO

A 2-year-old male with VACTERL association and asthma presented to the emergency room due to asthma exacerbation. Chest radiography revealed lingular pneumonia and thickening of the left paraspinal line of the gastroesophageal junction. Chest computed tomography confirmed a heterogeneous fluid- and gas-filled structure at the left posterior lateral posterolateral aspect of the esophagus, which was suspected to be an esophageal diverticulum on an upper gastrointestinal series. The esophageal diverticulum was excised via left thoracoscopy, and pathological examination revealed pancreatic tissue. Heterotopic pancreas lacks anatomical, vascular, or ductal continuity with the native pancreas. It is usually asymptomatic, but when discovered, it usually occurs later in life. It has been described in the foregut, but is not as common in the esophagus, especially in the pediatric population. This case report highlights the rare occurrence, and importance of considering, esophageal heterotopic pancreas within an esophageal diverticulum in an asymptomatic patient with VACTERL association.

13.
J Pediatr Surg ; 58(12): 2375-2383, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37598047

RESUMO

BACKGROUND: Anastomotic strictures (AS) after esophageal atresia (EA) repair are common. While most respond to endoscopic therapy, some become refractory and require surgical intervention, for which the outcomes are not well established. METHODS: All EA children with AS who were treated surgically at two institutions (2011-2022) were retrospectively reviewed. Surgical repair was performed for those with AS that were either refractory to endoscopic therapy or clinically symptomatic and undergoing surgery for another indication. Anastomotic leak, need for repeat stricture resection, and esophageal replacement were considered poor outcomes. RESULTS: 139 patients (median age: 12 months, range 1.5 months-20 years; median weight: 8.1 kg) underwent 148 anastomotic stricture repairs (100 refractory, 48 non-refractory) in the form of stricturoplasty (n = 43), segmental stricture resection with primary anastomosis (n = 96), or stricture resection with a delayed anastomosis after traction-induced lengthening (n = 9). With a median follow-up of 38 months, most children (92%) preserved their esophagus, and the majority (83%) of stricture repairs were free of poor outcomes. Only one anastomotic leak occurred in a non-refractory stricture. Of the refractory stricture repairs (n = 100), 10% developed a leak, 9% required repeat stricture resection, and 13% required esophageal replacement. On multivariable analysis, significant risk factors for any type of poor outcome included anastomotic leak, stricture length, hiatal hernia, and patient's weight. CONCLUSIONS: Surgery for refractory AS is associated with inherent yet low morbidity and high rates of esophageal preservation. Surgical repair of non-refractory symptomatic AS at the time of another thoracic operation is associated with excellent outcomes. LEVEL OF EVIDENCE: Level III.


Assuntos
Atresia Esofágica , Estenose Esofágica , Criança , Humanos , Lactente , Atresia Esofágica/cirurgia , Fístula Anastomótica/etiologia , Constrição Patológica/etiologia , Estudos Retrospectivos , Complicações Pós-Operatórias/etiologia , Estenose Esofágica/cirurgia , Anastomose Cirúrgica/efeitos adversos , Resultado do Tratamento
14.
J Pediatr Surg ; 58(6): 1213-1218, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36931942

RESUMO

INTRODUCTION: Endoscopic surveillance guidelines for patients with repaired esophageal atresia (EA) rely primarily on expert opinion. Prior to embarking on a prospective EA surveillance registry, we sought to understand EA surveillance practices within the Eastern Pediatric Surgery Network (EPSN). METHODS: An anonymous, 23-question Qualtrics survey was emailed to 181 physicians (surgeons and gastroenterologists) at 19 member institutions. Likert scale questions gauged agreement with international EA surveillance guideline-derived statements. Multiple-choice questions assessed individual and institutional practices. RESULTS: The response rate was 77%. Most respondents (80%) strongly agree or agree that EA surveillance endoscopy should follow a set schedule, while only 36% claimed to perform routine upper GI endoscopy regardless of symptoms. Many institutions (77%) have an aerodigestive clinic, even if some lack a multi-disciplinary EA team. Most physicians (72%) expressed strong interest in helping develop evidence-based guidelines. CONCLUSIONS: Our survey reveals physician agreement with current guidelines but weak adherence. Surveillance methods vary greatly, underscoring the lack of evidence-based data to guide EA care. Aerodigestive clinics may help implement surveillance schedules. Respondents support evidence-based protocols, which bodes well for care standardization. Results will inform the first multi-institutional EA databases in the United States (US), which will be essential for evidence-based care. LEVEL OF EVIDENCE: This is a prognosis study with level 4 evidence.


Assuntos
Atresia Esofágica , Fístula Traqueoesofágica , Criança , Humanos , Atresia Esofágica/cirurgia , Atresia Esofágica/epidemiologia , Fístula Traqueoesofágica/cirurgia , Estudos Prospectivos , Inquéritos e Questionários
15.
Radiol Case Rep ; 17(6): 2034-2037, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35432680

RESUMO

Mucoepidermoid carcinoma (MEC) is an uncommon type of salivary gland tumor that can present as an endobronchial neoplasm, most commonly in the adult population. Neuroendocrine carcinoid tumors comprise the majority of bronchial neoplasms in the pediatric population and are nearly indistinguishable from MEC on imaging. We present a rare case of MEC in a 3-year-old presenting with recurrent symptoms of lower airway obstruction and discuss its typical associated symptoms and imaging features.

16.
Pediatr Transplant ; 15(7): E142-4, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20412506

RESUMO

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.


Assuntos
Transplante de Rim/métodos , Transplante de Fígado/métodos , Trombose/patologia , Veia Cava Inferior/fisiopatologia , Aorta/patologia , Ductos Biliares/cirurgia , Biópsia/métodos , Feminino , Hepatoblastoma/patologia , Hepatoblastoma/cirurgia , Humanos , Lactente , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Modelos Anatômicos , Veia Porta/cirurgia , Tomografia Computadorizada por Raios X/métodos
17.
JTCVS Tech ; 8: 160-169, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34401841

RESUMO

OBJECTIVE: To report our experience with novel external tracheal and bronchial placed bioresorbable splints in children with severe symptomatic airway collapse. METHODS: Retrospective review of patients undergoing bioresorbable splint placement. RESULTS: Between July 2018 and February 2020, 14 patients received 16 external splints (trachea, n = 8; left bronchus, n = 7; and right bronchus, n = 1). Preoperatively, 7 patients had a tracheostomy; 6 of them were receiving mechanical ventilation with ventilator settings so high that they required an inpatient setting, often in an intensive care unit. Median age at implant was 14.5 months (range, 2 months-14 years). Splints were formed from moldable bioresorbable plates (RapidSorb; Synthes, Oberdorf, Switzerland) and were customized intraoperatively around a Hegar dilator. A series of Prolene sutures were placed through into the airway cartilage under simultaneous bronchoscopic and direct visualization and then tied securing the airway within the splint. Concomitant procedures were also performed in the region of the airway splints, consisting of airway reconstruction, cardiovascular procedures, and/or esophageal rotation (related to posterior tracheopexy). Median follow-up was 20 months (interquartile range, 12-21 months). Four patients required no further intervention. Although not necessarily in the splinted region, 7 patients required additional procedures, including posterior tracheobronchopexy (n = 2), temporary tracheal stent placement (n = 1), tracheal resection with end-to-end anastomosis (n = 1), closure tracheostomy (n = 1), and tracheostomy placement (n = 2). One patient required splint replacement and in 1 patient, the splint was removed later. All patients (except 2 deaths from unrelated causes) were discharged home. Three patients required mechanical ventilation at lower settings that allowed home ventilation (1 of those only at night), and 4 patients required tracheostomy collar. Indications for tracheostomy included subglottic stenosis, vocal cord paralysis, pulmonary insufficiency, small airway malacia, and laryngomalacia. CONCLUSIONS: An external bioresorbable splint can provide temporary external support while allowing the age-proportional growth of the airway. We applied readily available bioresorbable plates that were custom-molded based on the location, shape, and length of the collapsing airway in selected patients presenting with severe tracheobronchomalacia from loss of structural support and/or cartilage deformation. Further study that includes long-term outcomes are necessary to define the best role for these external splints as part of comprehensive airway management.

18.
J Pediatr Surg ; 55(7): 1302-1308, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31422856

RESUMO

BACKGROUND: Tracheobronchial compression (TBC) from great vessel anomalies (GVA) can contribute to tracheobronchomalacia (TBM) symptoms. The frequency, impact on symptoms and optimal management of GVA in these patients, with or without a history of esophageal atresia (EA), are still unclear. STUDY DESIGN: Patients who underwent surgery for TBM/ TBC between 2001 and 2017 were reviewed. Demographics, type of GVA, and operative interventions were collected. The frequency and treatment modalities of GVA between EA and non-EA patients were compared. RESULTS: Overall, 209 patients met criteria; 120 (57.4%) patients had at least one GVA, including double aortic arches (n = 4, 1.9%), right aortic arches (n = 14, 6.7%), aberrant right subclavian arteries (n = 15, 7.2%), and innominate artery compression (n = 71, 34.0%). Non-EA patients were more likely to have surgery later in life (29.5 months versus 16 months, p = 0.0002), double aortic arch (p = 0.0174), right aortic arch (p < 0.0001), and undergo vascular reconstruction concurrently with their airway procedure (25% vs 8.4%, p = 0.002). Vessel reconstruction was performed in 25 patients; six required cardiac bypass. CONCLUSION: The frequency of GVA in patients with symptomatic airway collapse is substantial. Multidisciplinary evaluation is imperative for operative planning as many require complex reconstruction and collaboration with cardiac surgery, particularly patients without a history of EA. LEVEL OF EVIDENCE: Level III.


Assuntos
Atresia Esofágica/complicações , Cardiopatias Congênitas/complicações , Traqueobroncomalácia , Malformações Vasculares/complicações , Pré-Escolar , Humanos , Lactente , Traqueobroncomalácia/complicações , Traqueobroncomalácia/epidemiologia , Traqueobroncomalácia/cirurgia
19.
Laryngoscope ; 130(2): E65-E74, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-30908672

RESUMO

OBJECTIVES/HYPOTHESIS: Combined anterior and posterior tracheobronchopexy is a novel surgical approach for the management of severe tracheobronchomalacia (TBM). We present our institutional experience with this procedure. Our objective was to determine the utility and safety of anterior and posterior tracheopexy in the treatment of severe TBM. STUDY DESIGN: Retrospective chart review. METHODS: All patients who underwent anterior and posterior tracheopexy from January 2013 to July 2017 were retrospectively reviewed. Charts were reviewed for indications, preoperative work-up, tracheobronchomalacia classification and severity, procedure, associated syndromes, synchronous upper aerodigestive tract lesions, and aberrant thoracic vessels. Main outcomes measured included improvement in respiratory symptoms, successful extubation and/or decannulation, vocal fold immobility, and new tracheotomy placement. RESULTS: Twenty-five patients underwent anterior and posterior tracheopexy at a mean age of 15.8 months (range, 2-209 months; mean, 31 months if 2 outliers of 206 and 209 months included). Mean length of follow-up was 26.8 months (range, 13-52 months). Indications for surgery included apneic events, ventilator dependence, need for positive pressure ventilation, tracheotomy dependence secondary to TBM, recurrent pneumonia, and exercise intolerance. Many patients had other underlying syndromes and synchronous upper aerodigestive tract lesions (8 VACTERL, 2 CHARGE, 1 trisomy 21, 1 Feingold syndrome, 17 esophageal atresia/tracheoesophageal fistula, 20 cardiac/great vessel anomalies, 1 subglottic stenosis, 1 laryngomalacia, 7 laryngeal cleft). At preoperative bronchoscopy, 21 of 25 patients had >90% collapse of at least one segment of their trachea, and the remaining four had 70% to 90% collapse. Following anterior and posterior tracheopexy, one patient developed new bilateral vocal-fold immobility; one patient with a preoperative left cord paralysis had a new right vocal-fold immobility. Postoperatively, most patients had significant improvement in their respiratory symptoms (21 of 25, 84%) at most recent follow-up. Three patients with preexisting tracheotomy were decannulated; two patients still had a tracheotomy at last follow-up. Two patients required new tracheotomy for bilateral vocal-fold immobility. CONCLUSIONS: Combined anterior and posterior tracheopexy is a promising new technique for the surgical management of severe TBM. Further experience and longer follow-up are needed to validate this contemporary approach and to minimize the risk of recurrent laryngeal nerve injury. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:E65-E74, 2020.


Assuntos
Brônquios/cirurgia , Traqueia/cirurgia , Traqueobroncomalácia/cirurgia , Adolescente , Criança , Pré-Escolar , Humanos , Lactente , Procedimentos Cirúrgicos Otorrinolaringológicos/efeitos adversos , Procedimentos Cirúrgicos Otorrinolaringológicos/métodos , Estudos Retrospectivos , Índice de Gravidade de Doença , Traqueobroncomalácia/complicações , Resultado do Tratamento
20.
J Pediatr Surg ; 55(12): 2682-2689, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32444171

RESUMO

PURPOSES: The descending thoracic aorta typically crosses posterior to the left mainstem bronchus (LMSB). We sought to evaluate patient factors that may lead one to consider a posterolateral descending thoracic aortopexy (PLDA) in addition to a posterior tracheopexy (PT) in the surgical treatment of symptomatic tracheobronchomalacia (TBM) that involves the LMSB. METHODS: Retrospective review of patients who underwent PT with or without PLDA between 2012 and 2017. Severity and extent of TBM were assessed using dynamic tracheobronchoscopy. Aortic positioning compared to the anterior border of the spine (ABS) at the level of the left mainstem bronchus was identified on computed tomography (CT). Factors associated with performing a PLDA were evaluated with logistic regression. RESULTS: Of 188 patients who underwent a PT, 70 (37%) also had a PLDA performed. On multivariate analysis, >50% LMSB compression on bronchoscopy (OR 8.06, p < 0.001), >50% of the aortic diameter anterior to the ABS (OR 2.06, p = 0.05), and more recent year of surgery (OR 1.61, p = 0.003) were associated with performing a PLDA. CONCLUSION: When performing a PT, a PLDA should be considered for patients who have >50% LMSB compression on dynamic bronchoscopy, and in those with a descending thoracic aorta located >50% anterior to the ABS. LEVEL OF EVIDENCE: III TYPE OF STUDY: Retrospective comparative study.


Assuntos
Aorta Torácica/cirurgia , Traqueia/cirurgia , Traqueobroncomalácia , Aorta Torácica/diagnóstico por imagem , Brônquios/diagnóstico por imagem , Brônquios/cirurgia , Broncopatias , Broncoscopia , Humanos , Estudos Retrospectivos , Traqueobroncomalácia/cirurgia
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