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1.
Adv Neonatal Care ; 21(1): 68-76, 2021 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-32384331

RESUMEN

BACKGROUND: Timely identification of esophageal atresia is challenging. Diagnosis may be suspected antenatally with a combination of polyhydramnios, associated with a small or absent stomach bubble or other anomalies. Esophageal atresia can be suspected postnatally in the presence of tachypnea, increased oral secretions, and an inability to advance an orogastric tube. Failure to recognize an esophageal atresia can have life-threatening implications. CLINICAL FINDINGS: A 5-day-old infant with a history of failure to thrive and respiratory distress presented in a community emergency department following a prolonged apnea associated with a breastfeed. PRIMARY DIAGNOSIS: Delayed postnatal diagnosis of esophageal atresia and tracheoesophageal fistula. INTERVENTIONS: During stabilization in the emergency department, a nasogastric tube was placed to decompress the stomach. A subsequent chest and abdominal radiograph identified the nasogastric tube curled in the upper esophagus, confirming an esophageal atresia. The abdominal radiograph demonstrated gaseous distension, suggesting the presence of a distal tracheoesophageal fistula. OUTCOMES: The neonate had a primary esophageal anastomosis and fistula ligation in a surgical neonatal unit. He was discharged home at 29 days of life. PRACTICE RECOMMENDATIONS: Understanding the challenges of an antenatal diagnosis and awareness of postnatal presentation with a view to improving postnatal recognition and better-quality outcomes for infants with an esophageal atresia and tracheoesophageal fistula.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Diagnóstico Tardío , Atresia Esofágica/diagnóstico , Atresia Esofágica/cirugía , Femenino , Humanos , Recién Nacido , Ligadura , Masculino , Embarazo , Diagnóstico Prenatal , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/cirugía
2.
Pediatr Surg Int ; 37(4): 503-509, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33388963

RESUMEN

PURPOSE: Preoperative echocardiography is used routinely in neonates with esophageal atresia to identify patients in whom congenital cardiac disease will impact upon anesthetic and surgical decision-making. We aimed to determine the suitability of selective preoperative echocardiography. METHODS: We performed a single-center retrospective review of neonates with esophageal atresia over 6 years (2010-2015) at our tertiary pediatric institution. Data included preoperative clinical examination, chest x-ray, and echocardiography. Endpoints were cardiovascular, respiratory, radiological, and echocardiography findings. Selective strategies were assessed using sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: We identified 115 neonates with esophageal atresia. All underwent preoperative echocardiography. Cardiac defects were identified in 49/115 (43%) (major 9/115, moderate 4/115). Sensitivity, specificity, positive predictive value, and negative predictive value of abnormal clinical and radiologic assessment for major and moderate cardiac defects were 92%, 25%, 13%, 96%; for clinical examination alone were 92%, 25%, 14%, 96%; for absence of murmur, cyanosis, and abnormal respiratory examination were 92%, 28%, 13%, 97%. Selective strategies reduce echocardiograms performed by 22%. CONCLUSION: Selective strategies allow for identification of neonates with esophageal atresia who may have deferral of echocardiogram unill after surgery. Selection may improve timeliness of care and resource utilization, without compromising patient safety.


Asunto(s)
Ecocardiografía , Atresia Esofágica/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Cuidados Preoperatorios/métodos , Femenino , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos , Fístula Traqueoesofágica/cirugía
3.
J Pediatr ; 219: 70-75, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-31952847

RESUMEN

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.


Asunto(s)
Atresia Esofágica/mortalidad , Bases de Datos Factuales , Atresia Esofágica/clasificación , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Alta del Paciente/estadística & datos numéricos , Estudios Retrospectivos , Medición de Riesgo
4.
J Pediatr ; 198: 60-66, 2018 07.
Artículo en Inglés | MEDLINE | ID: mdl-29628411

RESUMEN

OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P < .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.


Asunto(s)
Atresia Esofágica/complicaciones , Fundoplicación , Reflujo Gastroesofágico/cirugía , Preescolar , Femenino , Estudios de Seguimiento , Reflujo Gastroesofágico/etiología , Humanos , Lactante , Laparoscopía , Masculino , Reoperación , Estudios Retrospectivos , Resultado del Tratamiento
5.
Pediatrics ; 147(5)2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33911029

RESUMEN

BACKGROUND AND OBJECTIVES: Presence of a syndrome (or association) is predictive of poor survival in esophageal atresia (EA). However, most reports rely on historical patient outcomes, limiting their usefulness when estimating risk for neonates born today. We hypothesized improved syndromic EA survival due to advances in neonatal care. METHODS: A retrospective single-center review of survival in 626 consecutive patients with EA from 1980 to 2017 was performed. Data were collected for recognized risk factors: preterm delivery; birth weight <1500 g; major cardiac disease; vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL); and non-VACTERL syndromes. Cox proportional hazards regression models were used to evaluate temporal trends in survival with respect to year of birth and syndromic EA. RESULTS: Overall, 87% of 626 patients with EA survived, ranging from 82% in the 1980s to 91% in the 2010s. After adjusting for confounders, syndromic EA survival did not improve during the study, with no association found between year of birth and survival (hazard ratio [HR] 0.98, 95% confidence interval [CI]: 0.95-1.01). Aside from lethal non-VACTERL syndromes, patients with nonlethal non-VACTERL syndromes (HR 6.85, 95% CI: 3.50-13.41) and VACTERL syndrome (HR 3.02, 95% CI: 1.66-5.49) had a higher risk of death than those with nonsyndromic EA. CONCLUSIONS: Survival of patients with syndromic EA has not improved, and patients with non-VACTERL syndromes have the highest risk of death. Importantly, this is independent of syndrome lethality, birth weight, and cardiac disease. This contemporary survival assessment will enable more accurate perinatal counseling of parents of patients with syndromic EA.


Asunto(s)
Atresia Esofágica/mortalidad , Femenino , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Síndrome
6.
J Pediatr Surg ; 56(4): 686-691, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32807528

RESUMEN

BACKGROUND: The long-term outcomes of H-type tracheoesophageal fistula (TOF), an uncommon variant of esophageal atresia/tracheoesophageal fistula (OA/TOF), are rarely described in the literature. We reviewed our institutional experience of 70 years. METHODS: The Nate Myers Oesophageal Atresia Database was queried for patients with an H-type TOF (1948-2017). Data included presentation, diagnostic workup, surgical management, and outcomes. RESULTS: Of 1088 patients with OA/TOF, 56 (5.1%) had an H-type TOF. The most common presenting symptoms were cyanotic episodes (68%), choking with feeds (52%), and aspiration pneumonitis (46%). The majority (82%) were symptomatic in the first week of life. Coexisting congenital anomalies were present in 46%: cardiac (13/56, 23%), genitourinary (10/56, 18%), and vertebral/skeletal (9/56, 16%). Patients were consistently diagnosed with prone contrast tube esophagogram (77% sensitivity on the first study and 96% after a second study). The fistula was most commonly approached through a right cervical collar incision. Right vocal cord palsy occurred in 22%, with one case of bilateral palsies. Other complications included leak (5.6%), recurrence (9.3%), stricture (1.9%), and diverticulum (1.9%). Although there was a trend towards a lower recurrence rate when interposition material was used, this was not statistically significant (3.3% vs 16.7%, p = 0.16). Survival in operative cases was 98.2%, and when all diagnosed cases were considered was 89.3%. CONCLUSIONS: We have reported the largest single-center series of H-type TOF. Diagnosis is challenging, and surgical morbidity remains high. Despite this, long-term outcomes are favorable. LEVEL OF EVIDENCE: IV.


Asunto(s)
Atresia Esofágica , Fístula Traqueoesofágica , Niño , Atresia Esofágica/complicaciones , Atresia Esofágica/cirugía , Hospitales , Humanos , Estudios Retrospectivos , Columna Vertebral , Fístula Traqueoesofágica/epidemiología , Fístula Traqueoesofágica/cirugía
7.
J Pediatr Surg ; 55(11): 2329-2334, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-32143903

RESUMEN

AIM: To describe the burden of esophageal dilatations in patients following esophageal atresia (EA) repair. METHOD: A retrospective review was performed at The Royal Children's Hospital, Melbourne, of all neonates undergoing operative repair for EA over a 17-year period (1999-2015). Stricture was defined by radiological and/or intra-operative findings of narrowing at the esophageal anastomosis. Data recorded included EA type, perinatal details, operative approach, esophageal anastomosis outcome, dilatation requirement, and survival. Key endpoints were anastomotic leakage and tension, esophageal dilatation technique, dilatation frequency, fundoplication, and complications. RESULTS: During the study period, 287 newborn EA patients were admitted, of which 258 underwent operative repair and survived to primary discharge. Excluding 11 patients with isolated tracheoesophageal fistula, 247 patients were included in the final analysis. Intra-operative anastomotic tension was documented in 41/247 (16.6%), anastomotic leak occurred in 48/247 (19.4%), and fundoplication was performed in 37/247 (15.0%). Dilatations were performed in 149/247 (60.3%). Techniques included bougie-alone (92/149, 61.7%), combination of bougie and balloon (51/149, 34.2%), and balloon-alone (6/149, 4.0%). These patients underwent 1128 dilatations; median number of dilatations per patient was 4 (interquartile range 2-8). Long-gap EA and anastomotic tension were risk factors (p < 0.01) for multiple dilatations. Complications occurred in 13/1128 (1.2%) dilatation episodes: 11/13 esophageal perforation, 2/13 clinically significant aspiration. Perforations were rare events in both balloon (6/287, 2.1%) and bougie dilatations (4/841, 0.5%); one patient had a perforation from guidewire insertion. CONCLUSIONS: Esophageal dilatation occurred in a majority of EA patients. Long-gap EA was associated with an increased burden of esophageal dilatation. Perforations were rare events in balloon and bougie dilatations. TYPE OF STUDY: Original article - retrospective review. LEVEL OF EVIDENCE: II.


Asunto(s)
Dilatación , Atresia Esofágica , Estenosis Esofágica , Esofagoplastia , Anastomosis Quirúrgica , Atresia Esofágica/cirugía , Estenosis Esofágica/epidemiología , Estenosis Esofágica/etiología , Humanos , Recién Nacido , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Resultado del Tratamiento
8.
J Pediatr Surg ; 49(12): 1762-6, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25487479

RESUMEN

BACKGROUND: Long-gap oesophageal atresia (LGOA) causes significant early and long-term morbidity. We conducted a retrospective 25-year review comparing outcomes of delayed primary anastomosis versus oesophageal replacement with greater curvature gastric tube. METHODS: Records of 44 consecutive patients undergoing LGOA repair (1986-2010) were obtained from OA database with ethics approval and were analysed for complication and long-term outcomes. Analysis was conducted using Student's t-test for quantitative and Fisher exact test for qualitative data. RESULTS: Thirty (68%) patients underwent delayed primary anastomosis and 14 (32%) had oesophageal replacement. Oesophageal replacement patients had longer gaps (mean 5.5 vertebrae, range 4-9) compared to delayed primary anastomosis (mean 3.9, range 2-6) (p=0.004), but no difference in perioperative complications (p=0.2) (Table 1). Oesophageal replacement had more long-term complications (86%) compared to delayed primary anastomosis (30%) (p=0.005). Almost all patients (>90%) experienced gastro-oesophageal reflux and 21 delayed primary anastomosis patients (70%) underwent fundoplication. 60% of delayed primary anastomosis and 64.3% of oesophageal replacement patients had continued gastrointestinal symptoms years after repair. CONCLUSIONS: Our experience indicates that LGOA can be repaired safely using both methods, with no deaths and similar perioperative risk, but high long-term morbidity mandates long-term follow-up of these patients. Delayed primary anastomosis has a better long-term outcome compared to oesophageal replacement with gastric tube.


Asunto(s)
Nutrición Enteral/instrumentación , Atresia Esofágica/terapia , Esofagoplastia/métodos , Esófago/cirugía , Predicción , Gastroplastia/métodos , Estómago/cirugía , Adolescente , Adulto , Anastomosis Quirúrgica/métodos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Fundoplicación , Reflujo Gastroesofágico/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Factores de Tiempo , Adulto Joven
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