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1.
Scand J Immunol ; 99(2): e13338, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38981850

RESUMEN

Due to the high rate of post-operative sepsis and other infectious complications, a routine immunological screening protocol has been initiated since 2015 in our paediatric surgery clinic for all patients admitted with oesophageal atresia (EA) and warrant a delayed definitive treatment. In our study, we aimed to evaluate the immunodeficiencies in EA patients, by comparing them to healthy age-matched controls. As a prospective cohort study, EA patients admitted between 2015 and 2022, who had their definitive operation after the newborn period (>28 days of age) were included. On admission, serum concentrations of IgG, IgA, IgM, lymphocyte subset levels, C3 and C4 levels, specific IgG antibody responses against hepatitis B, hepatitis A, measles, varicella zoster were evaluated. The patients were age-matched with healthy controls to compare the results and followed up until three years of age. If a humoral immunodeficiency was detected, intravenous immunoglobulin treatment was administered before major oesophageal surgery and during follow-up. 31 EA patients (18 M/13F) with a mean age of 13.3 ± 9.0 months were compared with 40 age-matched healthy controls. Mean serum IgG levels were found to be statistically lower than controls in all age groups (P < .05). Transient hypogammaglobulinemia of infancy (THI) and unclassified syndromic immunodeficiencies (USI) were found to be strikingly high, accounting for 29.0% and 22.5%, respectively, adding up to 51.5% of EA patients. This is the first study evaluating immunodeficiencies in EA patients found in the reviewed literature. More than half of EA patients that required delayed surgery had humoral immunodeficiency, so preoperative screening and immunology referral may improve patient outcomes.


Asunto(s)
Agammaglobulinemia , Atresia Esofágica , Síndromes de Inmunodeficiencia , Humanos , Atresia Esofágica/inmunología , Atresia Esofágica/cirugía , Agammaglobulinemia/inmunología , Agammaglobulinemia/diagnóstico , Masculino , Femenino , Lactante , Estudios Prospectivos , Síndromes de Inmunodeficiencia/inmunología , Inmunoglobulina G/sangre , Preescolar , Inmunoglobulinas Intravenosas/uso terapéutico , Recién Nacido
2.
Orphanet J Rare Dis ; 19(1): 253, 2024 Jul 04.
Artículo en Inglés | MEDLINE | ID: mdl-38965635

RESUMEN

INTRODUCTION: Eating, drinking and swallowing difficulties are commonly reported morbidities for individuals born with OA/TOF. This study aimed to determine the nature and prevalence of eating, drinking and oro-pharyngeal swallowing difficulties reported in this population. METHOD: A systematic review and meta-proportional analysis were conducted (PROSPERO: CRD42020207263). MEDLINE, EMBASE, CINAHL, Pubmed, Scopus, Web of Science databases and grey literature were searched. Quantitative and qualitative data were extracted relating to swallow impairment, use of mealtime adaptations and eating and drinking-related quality of life. Quantitative data were summarised using narrative and meta-proportional analysis methods. Qualitative data were synthesised using a meta-aggregation approach. Where quantitative and qualitative data described the same phenomenon, a convergent segregated approach was used to synthesise data. RESULTS: Sixty-five studies were included. Six oro-pharyngeal swallow characteristics were identified, and pooled prevalence calculated: aspiration (24%), laryngeal penetration (6%), oral stage dysfunction (11%), pharyngeal residue (13%), nasal regurgitation (7%), delayed swallow initiation (31%). Four patient-reported eating/drinking difficulties were identified, and pooled prevalence calculated: difficulty swallowing solids (45%), difficulty swallowing liquids (6%), odynophagia (30%), coughing when eating (38%). Three patient-reported mealtime adaptations were identified, and pooled prevalence calculated: need for water when eating (49%), eating slowly (37%), modifying textures (28%). Mixed methods synthesis of psychosocial impacts identified 34% of parents experienced mealtime anxiety and 25% report challenging mealtime behaviours reflected in five qualitative themes: fear and trauma associated with eating and drinking, isolation and a lack of support, being aware and grateful, support to cope and loss. CONCLUSIONS: Eating and drinking difficulties are common in adults and children with repaired OA/TOF. Oro-pharyngeal swallowing difficulties may be more prevalent than previously reported. Eating, drinking and swallowing difficulties can impact on psychological well-being and quality of life, for the individual and parents/family members. Long-term, multi-disciplinary follow-up is warranted.


Asunto(s)
Trastornos de Deglución , Atresia Esofágica , Humanos , Trastornos de Deglución/etiología , Atresia Esofágica/cirugía , Fístula Traqueoesofágica/cirugía , Calidad de Vida , Ingestión de Alimentos/fisiología , Ingestión de Líquidos/fisiología
3.
BMJ Open ; 14(6): e081153, 2024 Jun 11.
Artículo en Inglés | MEDLINE | ID: mdl-38862230

RESUMEN

INTRODUCTION: Oesophageal discontinuity remains a challenge for thoracic and foregut surgeons globally. Whether arising emergently after catastrophic oesophageal or gastric disruption or arising in the elective setting in the case of staged reconstruction for esophagectomy or long gap atresia in the paediatric population, comprehensive review of this patient population remains unexplored within the surgical literature.The goal of this scoping review is to map the landscape of literature exploring the creation and takedown of cervical oesophagostomy with the intent to answer four questions (1) What are the primary indications for oesophageal discontinuity procedures? (2) What are the disease-specific and healthcare utilisation outcomes for oesophageal discontinuity procedures? (3) What is the primary indication for reversal procedures? (4) What are the disease-specific and healthcare utilisation outcomes for reversal procedures? METHODS: This review will follow the Arksey and O'Malley (2005) framework for scoping reviews. Paediatric (<18 years old) and adult (>18 years old) patients, who have received a cervical oesophagostomy in the context of a gastrointestinal discontinuity procedure or those who have had reversal of a cervical oesophagostomy, will be included for analysis. We will search MEDLINE, EMBASE and Cochrane Central Register of Controlled Trials (CENTRAL) databases for papers from 1990 until 2023. Interventional trials, prospective and retrospective observational studies, reviews, case series and qualitative study designs will be included. Two authors will independently review all titles, abstracts and full texts to determine which studies meet the inclusion criteria. ETHICS AND DISSEMINATION: No ethics approval is required for this review. Results will be disseminated through scientific presentations and relevant conferences targeted for researchers examining upper gastrointestinal/foregut surgery. REGISTRATION DETAILS: This protocol is registered with Open Science Framework (osf.io/s3b4g).


Asunto(s)
Esofagostomía , Humanos , Esofagostomía/métodos , Esofagectomía/métodos , Proyectos de Investigación , Atresia Esofágica/cirugía , Esófago/cirugía , Literatura de Revisión como Asunto
4.
Pediatr Surg Int ; 40(1): 149, 2024 Jun 03.
Artículo en Inglés | MEDLINE | ID: mdl-38829446

RESUMEN

PURPOSE: The surgical indication of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula is under debate. The current study aimed to investigate the outcome of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula in patients weighing < 2000 g and those who underwent emergency surgery at the age of 0 day. METHODS: The surgical outcomes were compared between patients weighing < 2000 g and those weighing > 2000 g at surgery and between patients who underwent surgery at the age of 0 day and those who underwent surgery at age ≥ 1 day. RESULTS: In total, 43 patients underwent thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula. The surgical outcomes according to body weight were similar. Patients who underwent surgery at the age of 0 day were more likely to develop anastomotic leakage than those who underwent surgery at the age of ≥ 1 day (2 vs. 0 case, p = 0.02). Anastomotic leakage was treated with conservative therapy. CONCLUSION: Thoracoscopic primary repair is safe and useful for esophageal atresia with tracheoesophageal fistula even in newborns weighing < 2000 g. However, emergency surgery at the age of 0 day should be cautiously performed due to the risk of anastomotic leakage.


Asunto(s)
Atresia Esofágica , Toracoscopía , Fístula Traqueoesofágica , Humanos , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Recién Nacido , Toracoscopía/métodos , Masculino , Femenino , Estudios Retrospectivos , Resultado del Tratamiento , Recién Nacido de Bajo Peso , Fuga Anastomótica/cirugía
5.
J Surg Res ; 299: 172-178, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38759333

RESUMEN

INTRODUCTION: The number of patients with congenital disease living to adulthood continues to grow. Often undergoing surgical correction in infancy, they continue to require lifelong care. Their numbers are largely unknown. We sought to evaluate hospital admissions of adult patients with esophageal atresia with tracheoesophageal fistula (EA/TEF), congenital diaphragmatic hernia (CDH), and Hirschsprung disease (HD). METHODS: The Florida Agency for Healthcare Administration inpatient database was merged with the Distressed Communities Index and Centers for Medicare and Medicaid Services Hospital and Physician Compare datasets. The dataset was queried for adult patients (≥18 y, born after 1970) with EA/TEF, CDH, and HD in their problem list from 2010 to 2020. Patient demographics, hospitalization characteristics, and discharge information were obtained. RESULTS: In total, 1140 admissions were identified (266 EA/TEF, 135 CDH, 739 HD). Patients were mostly female (53%), had a mean age of 31.6 y, and often admitted to an adult internist in a general hospital under emergency. Principal diagnoses and procedures (when performed) varied with diagnosis and age at admission. EA patients were admitted with dysphagia and foregut symptoms and often underwent upper endoscopy with dilation. CDH patients were often admitted for diaphragmatic hernias and underwent adult diaphragm repair. Hirschsprung patients were often admitted for intestinal obstructive issues and frequently underwent colonoscopy but trended toward operative intervention with increasing age. CONCLUSIONS: Adults with congenital disease continue to require hospital admission and invasive procedures. As age increases, diagnoses and performed procedures for each diagnoses evolve. These data could guide the formulation of multispecialty disease-specific follow-up programs for these patients.


Asunto(s)
Atresia Esofágica , Hernias Diafragmáticas Congénitas , Enfermedad de Hirschsprung , Humanos , Femenino , Masculino , Adulto , Enfermedad de Hirschsprung/cirugía , Enfermedad de Hirschsprung/epidemiología , Hernias Diafragmáticas Congénitas/cirugía , Hernias Diafragmáticas Congénitas/epidemiología , Florida/epidemiología , Atresia Esofágica/cirugía , Adulto Joven , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/epidemiología , Persona de Mediana Edad , Sobrevivientes/estadística & datos numéricos , Hospitalización/estadística & datos numéricos , Adolescente , Estudios Retrospectivos , Lactante , Bases de Datos Factuales/estadística & datos numéricos
6.
Pediatr Surg Int ; 40(1): 135, 2024 May 20.
Artículo en Inglés | MEDLINE | ID: mdl-38767779

RESUMEN

AIM: Van der Zee (VdZ) described a technique to elongate the oesophagus in long-gap oesophageal atresia (LGOA) by thoracoscopic placement of external traction sutures (TPETS). Here, we describe our experience of using this technique. METHOD: Retrospective review of all LGOA + / - distal tracheo-oesophageal fistula (dTOF) cases where TPETS was used in our institutions. Data are given as medians (IQR). RESULTS: From 01/05/2019 to 01/03/2023, ten LGOA patients were treated by the VdZ technique. Five had oesophageal atresia (Gross type A or B, Group 1) and five had OA with a dTOF (type C, Group 2) but with a long gap precluding primary anastomosis. Age of first traction procedure was Group 1 = 53 (29-55) days and Group 2 = 3 (1-49) days. Median number of traction procedures = 3; time between first procedure and final anastomosis was 6 days (4-7). Four cases were converted to thoracotomy at the third procedure. Three had anastomotic leaks managed conservatively. Follow-up was 12-52 months. All patients achieved oesophageal continuity and were orally fed; no patient required an oesophagostomy. CONCLUSION: In this series, TPETS in LGOA facilitated delayed primary anastomoses and replicated the good results previously described but, in addition, was successful in cases with dTOF. We believe traction suture placement and tensioning benefit from being performed thoracoscopically because of excellent visualisation and the fact that the tension does not change when the chest is closed. Surgical and anaesthetic planning and expertise are crucial. It is now our management of choice in OA patients with a long gap with or without a distal TOF.


Asunto(s)
Atresia Esofágica , Técnicas de Sutura , Toracoscopía , Humanos , Atresia Esofágica/cirugía , Estudios Retrospectivos , Toracoscopía/métodos , Masculino , Femenino , Recién Nacido , Lactante , Fístula Traqueoesofágica/cirugía , Tracción/métodos , Resultado del Tratamiento , Anastomosis Quirúrgica/métodos , Esófago/cirugía , Esófago/anomalías
7.
Sci Rep ; 14(1): 11489, 2024 05 20.
Artículo en Inglés | MEDLINE | ID: mdl-38769358

RESUMEN

We developed a 3D-printed thoracoscopic surgery simulator for esophageal atresia with tracheoesophageal fistula (EA-TEF) and assessed its effectiveness in educating young pediatric surgeons. Prototype production and modifications were repeated five times before producing the 3-D printed final product based on a patient's preoperative chest computed tomography. A 24-item survey was used to rate the simulator, adapted from a previous report, with 16 young surgeons with an average of 6.2 years of experience in pediatric surgery for validation. Reusable parts of the thoracic cage were printed to combine with replaceable parts. Each structure was fabricated using diverse printing materials, and subsequently affixed to a frame. In evaluating the simulator, the scores for each factor were 4.33, 4.33, 4.27, 4.31, 4.63, and 4.75 out of 5, respectively, with the highest ratings in value and relevance. The global rating was 3.38 out of 4, with ten stating that it could be used with slight improvements. The most common comment from participants was that the esophageal anastomosis was close to the actual EA-TEF surgery. The 3D-printed thoracoscopic EA-TEF surgery simulator was developed and reflected the actual surgical environment. It could become an effective method of training young pediatric surgeons.


Asunto(s)
Atresia Esofágica , Impresión Tridimensional , Cirujanos , Toracoscopía , Fístula Traqueoesofágica , Atresia Esofágica/cirugía , Atresia Esofágica/diagnóstico por imagen , Fístula Traqueoesofágica/cirugía , Humanos , Toracoscopía/métodos , Cirujanos/educación , Entrenamiento Simulado/métodos , Modelos Anatómicos
8.
J Pediatr ; 271: 114060, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38641166

RESUMEN

OBJECTIVE: To evaluate genetic testing utilization and diagnostic yield in infants with esophageal atresia (EA)/tracheoesophageal fistula (TEF) over the past 12 years to inform future practices and individualize prognostication and management. STUDY DESIGN: A retrospective cohort study was performed for all infants with EA or EA/TEF hospitalized between January 2011 and January 2023 at a quaternary children's hospital. For each infant, demographic information, prenatal and postnatal history, and genetic testing were reviewed. RESULTS: There were 212 infants who were classified as follows: 1) complex/syndromic with EA/TEF plus an additional major anatomic anomaly (n = 114, of which 74 met VACTERL criteria); 2) isolated/nonsyndromic EA/TEF (n = 88) and 3) isolated/nonsyndromic EA (n = 10). A range of genetic tests were sent with varying diagnostic rates including karyotype analysis in 12 (all with complex/syndromic phenotypes and all positive), chromosomal microarray analysis in 189 (114 of whom were complex/syndromic with an overall diagnostic rate of 3/189), single gene testing for CHD7 in 18 (4 positive), and exome analysis in 37 complex/syndromic patients (8 positive). CONCLUSIONS: EA/TEF with and without additional anomalies is genetically heterogeneous with a broad range of associated phenotypes. While the genetic etiology of EA/TEF with or without VACTERL remains largely unknown, genome wide testing (exome or genome) including copy number analysis is recommended over chromosomal microarray testing. We anticipate that expanded genetic/genomic testing modalities such as RNA sequencing and tissue specific molecular testing are needed in this cohort to improve our understanding of the genomic contributors to EA/TEF.


Asunto(s)
Atresia Esofágica , Pruebas Genéticas , Fístula Traqueoesofágica , Humanos , Fístula Traqueoesofágica/genética , Fístula Traqueoesofágica/diagnóstico , Atresia Esofágica/genética , Atresia Esofágica/diagnóstico , Estudios Retrospectivos , Masculino , Femenino , Recién Nacido , Lactante , Genómica
9.
Neonatal Netw ; 43(2): 65-75, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38599775

RESUMEN

Tracheoesophageal fistula (TEF) with or without esophageal atresia (EA) results from maldevelopment of the trachea and esophagus during maturation of the primitive foregut. EA/TEF commonly presents shortly after birth because of increased oral secretions and the inability to advance a nasogastric or orogastric tube to the proper depth. Given that prenatal diagnosis is uncommon and early intervention is important to reduce morbidity and mortality risk, early recognition and diagnosis are imperative. We present a case series of two neonates diagnosed with EA/TEF, type "C" and type "E," born at low-acuity centers, who required transport to a tertiary center for surgical support. The pathophysiology as well as types of TEFs, symptomology, stabilization goals, corrective treatment, and long-term implications will be examined. Finally, the educational needs of parents and caregivers will be discussed.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Humanos , Recién Nacido , Atresia Esofágica/complicaciones , Atresia Esofágica/diagnóstico , Atresia Esofágica/terapia , Tráquea , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/terapia
10.
Pediatr Surg Int ; 40(1): 112, 2024 Apr 24.
Artículo en Inglés | MEDLINE | ID: mdl-38656340

RESUMEN

PURPOSE: This study aims to evaluate different surgical approaches to long-gap esophageal atresia (LGEA) with or without tracheoesophageal fistula (TEF) is unclear. METHODS: A systematic literature review was done comparing gastric transposition versus esophageal lengthening with delayed primary anastomosis in infants with LGEA+/-TEF. The primary outcome was time to full oral feeds. Secondary outcomes were time to full enteric feeds, need for further surgery, growth, mortality, and postoperative adverse events. RESULTS: No comparative studies were found. However, the literature was re-interrogated for non-comparative studies. Four hundred thirty-eight articles were identified and screened, and 18 met the inclusion criteria. All were case series. Forty-three infants underwent gastric transposition, and 106 had esophageal lengthening with delayed primary anastomosis. One study on gastric transposition reported time to full oral feeds, and one study in each group reported growth. Time to full enteric feeds was reported in one study in each group. 30% of infants had further surgery following gastric transposition, including hiatus hernia repair (5/43, 12%) and esophageal dilation (7/43, 16%). Following esophageal lengthening, 62/106 (58%) had anti-reflux surgery, 58/106 (55%) esophageal dilatation and 11/106 (10%) esophageal stricture resection. Anastomotic complications occurred in 13/43 (30%), gastrointestinal in 16/43 (37%), respiratory in 17/43 (40%), and nerve injury in 2/43 (5%) of the gastric transposition group. In the esophageal lengthening group, anastomotic complications occurred in 68/106 (64%), gastrointestinal in 62/106 (58%), respiratory in 6/106 (6%), and none sustained nerve injury. Each group had one death due to a cause not directly related to the surgical procedure. CONCLUSIONS: This systematic review highlights the morbidity associated with both surgical procedures and the variety in reporting outcomes.


Asunto(s)
Anastomosis Quirúrgica , Atresia Esofágica , Esófago , Atresia Esofágica/cirugía , Humanos , Anastomosis Quirúrgica/métodos , Esófago/cirugía , Recién Nacido , Fístula Traqueoesofágica/cirugía , Estómago/cirugía , Resultado del Tratamiento
11.
S Afr J Surg ; 62(1): 43-47, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38568125

RESUMEN

BACKGROUND: Oesophageal atresia (OA) is one of the most common congenital gastrointestinal (GI) abnormalities. Due to advances in multidisciplinary care, early prognosis has improved with emphasis shifting to the long-term impact of this disease. Literature suggests a higher incidence of Barrett's and eosinophilic oesophagitis in these children, with an increased risk of oesophageal carcinoma. Guidelines for adults born with OA include routine endoscopy and lifelong screening of the upper gastrointestinal tract (GIT). Despite this, uncertainty remains regarding the necessity and frequency of endoscopic surveillance for children born with OA. We describe our endoscopic findings in children born with OA. METHODS: A prospective analytic cohort study was undertaken, which included all children born with OA, that were followed-up in our unit between 2020 and 2022. History regarding feeding and GI symptoms were documented after which an endoscopy was performed. RESULTS: During the study period, 37 endoscopies were performed in patients born with OA at a median age of 25 months. The most common clinical appearance on endoscopy was anastomotic strictures followed by oesophagitis. Twelve patients had biopsies taken, with abnormal histology in all but one patient. The most common histological finding was oesophagitis with lymphocytes and chronic gastritis. Two patients had Helicobacter Pylori infection, and one had findings suggestive of eosinophilic oesophagitis. CONCLUSION: All patients with a clinical indication for an endoscopy had abnormal clinical or histological findings, thus concurring with the literature in highlighting the need for regular endoscopy. We recommend regular clinical follow-up and endoscopic surveillance if clinically indicated for children born with OA.


Asunto(s)
Esofagitis Eosinofílica , Atresia Esofágica , Infecciones por Helicobacter , Helicobacter pylori , Adulto , Niño , Humanos , Preescolar , Atresia Esofágica/epidemiología , Atresia Esofágica/cirugía , Sudáfrica/epidemiología , Estudios de Cohortes , Estudios Prospectivos
12.
Pediatr Surg Int ; 40(1): 99, 2024 Apr 06.
Artículo en Inglés | MEDLINE | ID: mdl-38581456

RESUMEN

PURPOSE: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications. METHODS: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated. RESULTS: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%). CONCLUSION: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair.


Asunto(s)
Atresia Esofágica , Estenosis Esofágica , Fístula Traqueoesofágica , Recién Nacido , Niño , Humanos , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Estudios Retrospectivos , Resultado del Tratamiento , Estenosis Esofágica/etiología , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Anastomosis Quirúrgica/efectos adversos
13.
J Pediatr Gastroenterol Nutr ; 78(6): 1225-1233, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38623953

RESUMEN

OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.


Asunto(s)
Impedancia Eléctrica , Atresia Esofágica , Unión Esofagogástrica , Manometría , Humanos , Atresia Esofágica/cirugía , Atresia Esofágica/fisiopatología , Manometría/métodos , Femenino , Lactante , Masculino , Unión Esofagogástrica/fisiopatología , Preescolar , Niño , Adolescente , Deglución/fisiología , Estudios de Casos y Controles , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/fisiopatología
14.
Dis Esophagus ; 37(7)2024 Jul 03.
Artículo en Inglés | MEDLINE | ID: mdl-38476012

RESUMEN

As neonatal mortality rates have decreased in esophageal atresia (EA), there is a growing focus on quality of life (QoL) in these children. No study from Africa has reported on this topic. This pilot study aimed to describe disease-specific QoL in EA children and its applicability as part of long-term follow-up in an academic facility in South Africa. Disease-specific QoL in children born with EA was assessed utilizing the EA-QoL questionnaire for children aged 2-17 years during a patient-encounter. The parent-report for children aged 2-7 years compromised 17 items categorized into three domains: eating, physical health and treatment, and social isolation/stress. The 24-item EA-QL questionnaire for children aged 8-18 (child- and parent-report) explored four domains: eating, body perception, social relationships, and health and well-being. A total of 13 questionnaires for children aged 2-7 years were completed by five parents. A negative perceived impact on their child's eating was reported by 46-92% of parents, and less impact in the other two domains. A total of 27 questionnaires were completed by eight children aged 8-17 years and 10 parents. Similar percentages children and parents reported a negative impact in the eating, social relationships, and body perception domains. More than half reported a negative impact on the child's health and well-being. This study supports the concept that assessment of disease-specific QoL should play a vital role in the comprehensive follow-up approach for children born with EA. We identified that parents of younger children were more likely to report eating disorders, whereas parents of older children were more likely to report health difficulties with different perceptions when it came to the child's scar.


Asunto(s)
Atresia Esofágica , Calidad de Vida , Humanos , Proyectos Piloto , Niño , Sudáfrica , Atresia Esofágica/psicología , Masculino , Femenino , Preescolar , Estudios de Seguimiento , Adolescente , Encuestas y Cuestionarios , Padres/psicología
17.
Pediatr Surg Int ; 40(1): 89, 2024 Mar 22.
Artículo en Inglés | MEDLINE | ID: mdl-38517524

RESUMEN

PURPOSE: Congenital esophageal stenosis (CES) associated with esophageal atresia (EA) is rare, and no standard treatment has been established. We reviewed cases of EA-associated CES to assess the clinical characteristics and treatment outcomes, especially the feasibility of endoscopic dilatation. METHODS: We retrospectively examined patients with EA-associated CES. We also compared treatment outcomes of EA-associated CES with those of EA patients without CES who developed postoperative anastomotic stricture. RESULTS: Among 44 patients with EA, ten had CES (23%). Postoperative complications were not significantly different between EA patients with CES and those without CES but with anastomotic stricture. All CES patients underwent balloon dilatation as initial treatment. Eight of nine patients (89%) were successfully treated by dilatation only, and one patient underwent surgical resection. The median number of balloon dilatations for CES was five (2-17), which was higher than that for anastomotic stricture in patients without CES (p = 0.012). Esophageal perforation occurred in five patients with CES (5/9, 56%) after dilatation, but all perforations were successfully managed conservatively with an uneventful post-dilatation course. CONCLUSIONS: Twenty-three percent of patients with EA had CES. Although balloon dilatation for EA-associated CES required multiple treatments and carried a risk of perforation, balloon dilatation showed an 89% success rate and all perforations could be managed conservatively.


Asunto(s)
Atresia Esofágica , Estenosis Esofágica , Humanos , Atresia Esofágica/complicaciones , Atresia Esofágica/cirugía , Estenosis Esofágica/terapia , Estenosis Esofágica/cirugía , Dilatación/efectos adversos , Estudios Retrospectivos , Constricción Patológica/complicaciones , Resultado del Tratamiento , Complicaciones Posoperatorias/etiología , Anastomosis Quirúrgica/efectos adversos
18.
Afr J Paediatr Surg ; 21(2): 129-133, 2024 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-38520230

RESUMEN

ABSTRACT: Management of oesophageal atresia (OA) with tracheoesophageal fistula (TOF) in Nigeria and the West African subregion has no doubt been a very demanding task for paediatric surgeons, not necessarily due to lack of skills, but due to the significant demand on neonatal intensive care, which in our region, is often fitted with the poor infrastructure needed to make this a success. Furthermore, the use of open thoracotomy has increased this demand resulting in a significant number having severe morbidities and significant mortality rates. Hence, in our subregion, there is still a slow progression to meet up with the evolving trend of the management of this complex condition in the developed world. Following the first documented successful thoracoscopic repair of OA with TOF since 2000, there has been a progressive evolution and refinement of this technique, such that thoracoscopic repair is fast becoming the gold standard for the repair of all types of OAs, including long-gap anomalies. This article reports our experience with the first two cases of thoracoscopic repair of OA with TOF in the West African subregion.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Recién Nacido , Niño , Humanos , Atresia Esofágica/cirugía , Toracoscopía/métodos , Fístula Traqueoesofágica/cirugía , Hospitales , Complicaciones Posoperatorias
19.
J Pediatr Surg ; 59(6): 1066-1071, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38429129

RESUMEN

BACKGROUND: Airway anomalies, symptoms and interventions are commonly reported in children with oesophageal atresia with tracheoesophageal fistula (OA/TOF). The purpose of this study was to assess the incidence of these airway pathologies and those requiring interventions in the long-term. METHODS: A retrospective case note review of all patients admitted to the Neonatal Unit at the Royal Hospital for Children, Glasgow between January 2000 and December 2015 diagnosed with OA/TOF. Included patients had a minimum of 5 years follow-up. RESULTS: 121 patients were identified. 118 proceeded to OA/TOF repair. 115 patients had long-term follow-up data. Ninety-five (83%) children had one or more airway symptom recorded. Thirty-six (31%) neonates underwent airway endoscopy at the time of their initial OA/TOF repair. Forty-six (40%) children underwent airway endoscopy at a later date due to airway symptoms. Airway pathologies identified included airway malacia, thirty-two (28%), subglottic stenosis, eleven (10%), tracheal pouch, twenty-five (22%), laryngeal cleft, seven (6%) and recurrent fistula, five (4%). Airway interventions included endoscopic division of tracheal pouch, ten (9%), tracheostomy, seven (6%), aortopexy, six (5%), repair of recurrent fistula, five (4%), endoscopic repair of laryngeal cleft, three (3%) and four (3%) required open airway reconstruction for subglottic stenosis. One child (1%) remains tracheostomy dependent. CONCLUSIONS: Long-term airway pathologies are common in children with OA/TOF. Many of these are remediable with surgical intervention. Clinicians should be cognisant of this and refer to Airway Services appropriately.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Humanos , Fístula Traqueoesofágica/cirugía , Fístula Traqueoesofágica/complicaciones , Atresia Esofágica/cirugía , Atresia Esofágica/complicaciones , Estudios Retrospectivos , Recién Nacido , Masculino , Femenino , Estudios de Seguimiento , Lactante , Resultado del Tratamiento , Preescolar , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Laringoestenosis/cirugía , Recurrencia , Laringe/anomalías , Laringe/cirugía , Anomalías Congénitas
20.
Pediatr Surg Int ; 40(1): 61, 2024 Feb 29.
Artículo en Inglés | MEDLINE | ID: mdl-38421506

RESUMEN

PURPOSE: This study aimed to explore parents' experience of sham feeding their baby born with esophageal atresia at home, waiting for reconstructive surgery. METHOD: Semi-structured interviews were conducted with parents of six children born with esophageal atresia waiting for delayed reconstruction. The interviews were analyzed using qualitative content analysis. RESULTS: Parents experienced that sham feed reinforced the healthy abilities in their baby. They had faith in their own ability as parents to care for their child as well as to see to their baby's strength to cope with difficulties. Parents expressed that the health care system can hinder as well as be a major support on their way to a more normal life at home while waiting for reconstructive surgery. CONCLUSION: The experience of sham feeding at home while waiting for reconstructive surgery is characterized by positive aspects both for children born with esophageal atresia and their parents.


Asunto(s)
Atresia Esofágica , Cirugía Plástica , Lactante , Niño , Humanos , Atresia Esofágica/cirugía , Estado de Salud , Padres , Investigación Cualitativa
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