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1.
J Pediatr Surg ; : 161880, 2024 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-39244418

RESUMO

INTRODUCTION: Tracheomalacia severity is difficult to quantify, however, ultrashort echo-time MRI objectively quantifies tracheomalacia in infants without sedation, radiation, or intubation. Patients with tracheoesophageal fistula and esophageal atresia (TEF/EA) commonly have tracheomalacia, however, the relationship between tracheomalacia severity and esophageal atresia has not been well defined. The primary objective of this study was to establish the relationship between EA and tracheomalacia severity and identify possible predictors of tracheomalacia severity. METHODS: A retrospective review of neonates with TEF/EA who had previously undergone UTE MRI was performed. The trachea was divided into thirds. Maximal eccentricity in each third was calculated by measuring the anterior posterior dimension (MinD) and dividing it by the maximum width of the trachea (MaxD). Frequency of respiratory related admissions, number of upper respiratory infections, and number of steroids courses were quantified in addition to eccentricity in short and long gap esophageal atresia patients. RESULTS: A total of 16 TEF/EA patients were included. Patients with long gap esophageal atresia had more severe tracheomalacia than short gap as measured by eccentricity in the upper (0.60 vs 0.72, p = 0.03), middle (0.48 vs 0.61, p = 0.02), and lower (0.5 vs 0.65, p = 0.01) trachea. Long gap esophageal atresia patients had more frequent respiratory readmissions (1.87 admissions/year vs 0.54 admissions/year) (p = 0.03). Following TEF/EA repair the trachea was less eccentric in the upper third (0.64 pre, 0.79 post, p < 0.01) and more eccentric in the lower third (0.69 pre, 0.56 post, p < 0.01). CONCLUSION: Differences in esophageal gap and repair status correlate with airway eccentricity and tracheomalacia symptoms.

2.
Pediatr Surg Int ; 40(1): 251, 2024 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-39251414

RESUMO

BACKGROUND: The technical complexity and limited casuistry of neonatal surgical pathology limit the possibilities of developing the necessary technical competencies by specialists in training. Esophageal atresia constitutes the paradigm of this problem. The use of synthetic 3D models for training is a promising line of research, although the literature is limited. METHODS: We conceptualized, designed, and produced an anatomically realistic model for the open correction of type III oesophageal atresia. We validated it with two groups of participants (experts and non-experts) through face, construct, and content-validity questionnaires. RESULTS: The model was validated by nine experts and nine non-experts. The mean procedure time for the experts and non-experts groups was 34.0 and 38.4 min, respectively. Two non-experts did not complete the procedure at the designed time (45 min). Regarding the face validity questionnaire, the mean rating of the model was 3.2 out of 4. Regarding the construct validity, we found statistically significant differences between groups for the equidistance between sutures, 100% correct in the expert group vs. 42.9% correct in the non-expert group (p = 0.02), and for the item "Confirms that tracheoesophageal fistula closure is watertight before continuing the procedure", correctly assessed by 66.7% of the experts vs. by 11.1% of non-experts (p = 0.05). Concerning content validity, the mean score was 3.3 out of 4 for the experts and 3.4 out of 4 for the non-experts. CONCLUSIONS: The present model is a cost-effective, simple-to-produce, and validated option for training open correction of type III esophageal atresia. However, future studies with larger sample sizes and blinded validators are needed before drawing definitive conclusions.


Assuntos
Atresia Esofágica , Modelos Anatômicos , Atresia Esofágica/cirurgia , Humanos , Competência Clínica , Treinamento por Simulação/métodos , Pediatria/educação , Inquéritos e Questionários , Cirurgiões/educação
3.
J Pediatr Surg ; : 161662, 2024 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-39218728

RESUMO

BACKGROUND: The aim was to evaluate short- and long-term outcomes for thoracoscopic repair of EA/TEF and compare with open repair. METHODS: Patients who underwent EA/TEF repair during 2000-2020 were evaluated retrospectively. Patients with delayed repair were excluded. Demographic, operative, outcome data was collected. Outcomes were compared using Wilcoxon-rank sum tests for continuous, Chi-squared/Fisher's exact tests for categorical data. RESULTS: There were 104 patients with primary repair, 49 (47.1%) underwent thoracoscopic repair per surgeon's choice. Type C accounted for 101 (97.1%) of the cases. Gestational age and birth weight were higher in the thoracoscopy group (p = 0.001). The rate of ≥3 VACTERL anomalies was higher in the OR group (p = 0.016). Operative time, rate of anastomotic leak, time to first oral feeding were similar (p > 0.05). Thoracoscopy group had decreased length of ventilation (p = 0.026) and length of stay (p = 0.029). The incidence of anastomotic stricture was higher in the thoracoscopy group (p = 0.012). Recurrent TEF was one case in each group. Rates of tube feeding at discharge and in first year were similar (p > 0.05), rate in third year was decreased (p = 0.032) in the thoracoscopy group. Rates of anti-reflux medication in first and third years, and fundoplication rate were similar (p > 0.05). CONCLUSIONS: Many of the short- and long-term outcomes are comparable between thoracoscopic and open repair of EA/TEF. Length of ventilation, length of stay are decreased in the thoracoscopy group. Anastomotic stricture is higher, the need for long-term tube feeding is lower after thoracoscopic repair. Although these results could be affected by selection bias, they are still promising for advancing thoracoscopic repair of EA/TEF safely and efficiently. LEVEL OF EVIDENCE: Level III.

4.
J Pediatr Surg ; 2024 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-39089893

RESUMO

BACKGROUND: Oesophageal atresia (OA) is often accompanied by tracheomalacia (TM). The aim of this study was to evaluate its presence in OA patients during routine rigid tracheobronchoscopy (TBS) before primary correction and compare this to postoperative TBS and clinical signs of TM. METHODS: This retrospective cohort study included patients born with OA between June 2013 and December 2022 who had received a TBS before OA correction and had been followed for at least twelve months. Definite TM was postoperatively diagnosed through TBS, and probable TM was defined as having symptoms of TM. RESULTS: We analysed data from 79 patients, of whom 87% with OA type C. Preoperatively, TM was observed in 33 patients (42% of all patients), seven of whom had severe TM. Definite TM was observed in 21 patients (27%), of whom 15 had severe TM. Forty-one patients (52% of all patients) had developed symptoms of TM within twelve months, including harsh barking cough (n = 15), stridor and/or wheezing (n = 20), recurrent respiratory insufficiency (n = 11), or needing airway surgery (n = 7). The sensitivity of preoperative TBS for the presence of postoperative (definite and probable combined) TM is 50.0%, 95% CI [35.2-64.8], and the specificity 67.6%, 95% CI [51.7-81.1]. Clinical characteristics did not differ between the patients with or without postoperative TM. CONCLUSIONS: More than half of the studied patients with OA experienced symptoms of TM. While preoperative TBS is routinely performed prior to surgical OA correction, its predictive value for the presence of postoperative TM remains limited. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Study of Diagnostics Test.

5.
J Pediatr Surg ; 2024 Jul 16.
Artigo em Inglês | MEDLINE | ID: mdl-39097496

RESUMO

BACKGROUND: The traction-induced esophageal growth (Foker) process for the treatment of long gap esophageal atresia (LGEA) relies on applying progressive tension to the esophagus to induce growth. Due to its anti-fibrotic and muscle-relaxing properties, we hypothesize that Botulinum Toxin A (BTX) can enhance traction-induced esophageal growth. METHODS: A retrospective two-center cohort study was conducted on children who underwent a BTX-enhanced Foker process for LGEA repair from 2021 to 2023. BTX (10 units/ml, 2 units/kg, per esophageal pouch) was applied at the time of traction initiation. Time on traction, complications, and anastomotic outcomes were compared against historical controls (Foker process without BTX) from 2014 to 2021. RESULTS: Twenty infants (LGEA type A:12, B:4, C:4; 35% reoperative; median [IQR] age 3 [2-5] months), underwent BTX-enhanced Foker process (thoracotomy with external traction: 9; minimally invasive [MIS] multi-staged internal traction: 11). Mean gap lengths were similar between BTX-enhanced external and external traction control patients (mean [SD], 50.6 mm [12.6] vs. 44.5 mm [11.9], p = 0.21). When compared to controls, the BTX-enhanced external traction process was significantly faster (mean [SD], 12.1 [1.6] days vs. 16.6 [13.2] without BTX, p = 0.04) despite similar preoperative gap lengths. There was no difference in time on traction for those undergoing a minimally invasive process. There were no significant differences in complications or anastomotic outcomes in either cohort. CONCLUSION: Botulinum toxin may play a role in accelerating the traction-induced esophageal growth process for LGEA repair. Minimizing time on traction can decrease sedation and paralysis burden while on external traction. Further studies are needed to elucidate the effects of BTX on the esophagus. LEVEL OF EVIDENCE: Level III. TYPE OF STUDY: Retrospective, Two-center, Cohort study.

6.
J Pediatr Surg ; 2024 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-39147683

RESUMO

INTRODUCTION: Minimally invasive surgery (MIS) is gaining traction as a first-line approach to repair congenital anomalies. This study aims to evaluate outcomes for neonates undergoing open versus MIS repairs for esophageal atresia/tracheoesophageal fistula (EA/TEF). METHODS: In this retrospective study, neonates undergoing EA/TEF repair from 2013 to 2020 were identified using the National Surgical Quality Improvement Program-Pediatric database. Proportions of operative approach (open vs. MIS) over time were analyzed. A propensity score-matched analysis using preoperative characteristics was performed and outcomes were compared including composite morbidity and reintervention rates (overall, major [thoracoscopy, thoracotomy], and minor [chest/feeding tube placement, endoscopy]) between operative approaches. Pearson's chi-square or Fisher's exact tests were used as appropriate. RESULTS: We identified 1738 neonates who underwent EA/TEF repair. MIS utilization increased over time. Pre-match, neonates undergoing open repair were more likely to be premature, lower weight, ventilator dependent, and have cardiac risk factors with higher severity. Post-match, the groups were similar and included 340 neonates per group. MIS repair was associated with longer median operative time (209 vs. 174 min, p < 0.001) and increased overall post-operative intervention rates (7.6% vs. 2.9%, p = 0.01). There were no differences in composite morbidity (24.4% vs. 25.0%, p = 0.86) outside of reintervention. CONCLUSION: MIS approach for neonates with EA/TEF appears to be associated with a higher rate of reinterventions. Further studies evaluating MIS approaches for the repair of EA/TEF are needed to better define short- and long-term outcomes. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

7.
J Pediatr Surg ; : 161671, 2024 Aug 08.
Artigo em Inglês | MEDLINE | ID: mdl-39209685

RESUMO

BACKGROUND: The management of neonates with long-gap esophageal atresia (LGEA) combined with distal congenital esophageal strictures (CES) is challenging. We sought to review our approach for this rare set of anomalies. METHODS: We reviewed children with LGEA + CES surgically treated at two institutions (2018-2024). LGEA repair was performed using the Foker technique (traction-induced esophageal lengthening). A CES strategy was chosen based on preoperative evaluations and intraoperative findings. The configuration and length of the CES were assessed using retrograde flexible esophagoscopy via gastrostomy with contrast fluoroscopy. RESULTS: Eight patients (75% male) with LGEA + CES were treated: Four had type A and four had type B EA. Median gap length was 3.5 cm. Three underwent thoracoscopic esophageal lengthening. After a median follow-up of 18 months (IQR: 9-25), all retained their native esophagus. However, those who had CES resection concurrent with the lengthening process or at the time of EA anastomosis had more challenging perioperative courses: one required additional time on traction and another required esophageal anastomotic stricture resection. CONCLUSIONS: Our experience with LGEA and distal CES emphasizes tailoring surgical approaches to each patient's unique condition, avoiding a one-size-fits-all strategy. However, if the esophageal tissue above the distal CES is in good condition, our preference has shifted towards retaining the CES during traction, performing gentle dilation at anastomosis time, and conducting definitive endoscopic management subsequently. We would caution against making the assumption that salvage of the native esophagus is not possible or that resection of the CES is always needed. LEVEL OF EVIDENCE: Level III.

8.
Orphanet J Rare Dis ; 19(1): 304, 2024 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-39169371

RESUMO

The International Network on Esophageal Atresia (INoEA) stands as a beacon of collaboration in addressing the complexities of this congenital condition on a global scale. The eleven board members, from various countries (USA, Canada, France, Australia, Italy, Sweden, Germany, and The Netherlands) and backgrounds (pediatric gastroenterology, pediatric surgery, pediatric pulmonology, nursing, and parents) met in a face-to-face symposium in Lille in November 2023, to identify challenges and solutions for improving global collaboration of the network.


Assuntos
Atresia Esofágica , Atresia Esofágica/cirurgia , Humanos , Cooperação Internacional , Austrália
9.
Pediatr Rep ; 16(3): 717-723, 2024 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-39189294

RESUMO

In this case report, we described a rare complication-lung torsion after esophageal atresia repair in a newborn. Torsion of the lung is a critical condition in which either the entire lung or a lung lobe twists, leading to occlusion of blood vessels and airways. The patient's clinical condition was poor after the primary operation. An emergency thoracotomy showed 180° torsion of the right upper lobe (RUL) and right middle lobe (RML). After detorsion, perioperatively, the lung was gradually reperfused and had a normal appearance. After surgery, the patient was unstable, which culminated in a fatal end (bradycardia, reperfusion injury). Immediate intervention can preserve the affected lung or lung lobe. However, pulmonary torsion typically has a poor prognosis due to misdiagnosis and delayed treatment. Additionally, diagnosis in the neonatal period is even more challenging because the clinical symptoms are nonspecific. In any case, the question is whether detorsion is the right solution or whether a segmentectomy is necessary.

10.
JA Clin Rep ; 10(1): 45, 2024 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-39078532

RESUMO

BACKGROUND: In esophageal atresia type C, identifying the tracheoesophageal fistula (TEF) location is crucial for airway management. However, a thin bronchoscope may not always be available. CASE PRESENTATION: We report on a low-birth-weight neonate with esophageal atresia type C who required immediate gastrostomy after birth. With no suitable thin bronchoscope available, alternative methods were utilized to estimate the TEF location post-gastrostomy. Submerging the gastrostomy tube tip in water and applying positive pressure ventilation via a tracheal tube allowed for observation of air bubbles emerging from the gastrostomy tube. As the tracheal tube was advanced, the cessation of bubbles indicated that the TEF was sealed by the tracheal tube. The location of the tracheal tube tip, confirmed by chest radiographs, was consistent with the TEF location identified during corrective surgery for TEF. CONCLUSIONS: This innovative technique facilitated successful estimation of the TEF location without bronchoscopy, demonstrating its efficacy in resource-limited settings.

11.
Surg Endosc ; 38(9): 5076-5085, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39020118

RESUMO

BACKGROUND: This study aimed to analyze the results, feasibility and safety of the thoracoscopic approach for patients with esophageal atresia with tracheoesophageal fistula (EA/TEF) depending on the patient's birth weight. METHODS: The study involved only type C and D EA/TEF. Among the analyzed parameters were the patients' characteristics, surgical treatment and post-operative complications: early mortality, anastomosis leakage, anastomosis strictures, chylothorax, TEF recurrence, and the need for fundoplication or gastrostomy. RESULTS: 145 consecutive newborns underwent thoracoscopic EA with TEF repair. They were divided into three groups-A (N = 12 with a birth weight < 1500 g), B (N = 23 with a birth weight ≥ 1500 g but < 2000 g), and C-control group (N = 110 with a birth weight ≥ 2000 g). Primary one-stage anastomosis was performed in 11/12 (91.7%) patients-group A, 19/23 (82.6%)-group B and 110 (100%)-group C. Early mortality was 3/12 (25%)-group A, 2/23 (8.7%)-group B, and 2/110 (1.8%)-group C and was not directly related to the surgical repair. There were no significant differences in operative time and the following complications: anastomotic leakage, recurrent TEF, esophageal strictures, and chylothorax. There were no conversions to an open surgery. Fundoplication was required in 0%-group A, 4/21 (19.0%)-group B, and 2/108 (1.9%)-group C survivors. Gastrostomy was performed in 1/9 (11.1%)-group A, 3/21 (14.3%)-group B and 0%-group C. CONCLUSION: In an experienced surgeon's hands, even in the smallest newborns, the thoracoscopic approach may be safe, feasible, and worthy of consideration. Birth weight seems to be not a direct contraindication to the thoracoscopic approach.


Assuntos
Peso ao Nascer , Atresia Esofágica , Toracoscopia , Fístula Traqueoesofágica , Humanos , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Fístula Traqueoesofágica/cirurgia , Estudos Retrospectivos , Recém-Nascido , Masculino , Feminino , Toracoscopia/métodos , Estudos de Viabilidade , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Anastomose Cirúrgica/métodos , Fístula Anastomótica/etiologia , Fístula Anastomótica/epidemiologia , Resultado do Tratamento
12.
J Pediatr Surg ; 2024 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-39054116

RESUMO

BACKGROUND: Pediatric minimally invasive surgery requires advanced technical skills. Off-the-job training (OJT), especially when using disease-specific models, is an effective method of acquiring surgical skills. To achieve effective OJT, it is necessary to provide objective and appropriate skill assessment feedback to trainees. We aimed to construct a system that automatically evaluates surgical skills based on forceps movement using deep learning (DL). METHODS: Using our original esophageal atresia OJT model, participants were tasked with performing esophageal anastomosis. All tasks were recorded for image analysis. Based on manual objective skill assessments, each participant's surgical skills were categorized into two groups: good and poor. The motion of the forceps in both groups was used as training data. Employing this training data, we constructed an automated system that recognized the movement of forceps and determined the quality of the surgical technique. RESULTS: Thirteen participants were assigned to the good skill group and 32 to the poor skill group. These cases were validated using an automated skill assessment system. This system showed a precision of 75%, a specificity of 94%, and an area under the receiver operating characteristic curve of 0.81. CONCLUSIONS: We constructed a system that automatically evaluated the quality of surgical techniques based on the movement of forceps using DL. Artificial intelligence diagnostics further revealed the procedures important for suture manipulation. LEVELS OF EVIDENCE: Level IV.

13.
Eur J Pediatr ; 183(9): 3777-3783, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38864876

RESUMO

Surgically repaired esophageal atresia (EA) is associated with chronic esophageal and respiratory morbidity that require ongoing management. The care of chronically ill children can exert considerable stress on parents, thereby potentially affecting their mental well-being. In response to this, disease-specific support groups have emerged with the aim to bring together individuals facing similar challenges, facilitating the exchange of experiences within a nurturing environment, and providing essential emotional support. In the context of this international collaborative study, we investigated the role played by EA-specific support groups in promoting the emotional well-being of EA families. An anonymous online survey was distributed through a network comprising 12 international EA support groups affiliated with the Federation of Esophageal Atresia and Tracheoesophageal Fistula (EAT) during May and August of 2022. In the study, 96 parents from 23 different countries completed the survey. Fifty-six percent indicated a lack of interaction with other EA families during the initial diagnosis, with 91% expressing the belief that such contact would have been beneficial. Participants exhibited a unanimous recognition of the critical role played by disease-specific support groups in navigating the emotional journey associated with EA.Conclusion: The findings of this global survey align with existing literature, reaffirming the beneficial impact of disease-specific support groups tailored for EA patients and their families on their emotional well-being. These groups provide a valuable platform for the exchange of personal experiences and narratives, delivering advantages to both those who share their stories and those who receive this valuable information.


Assuntos
Atresia Esofágica , Pais , Grupos de Autoajuda , Humanos , Atresia Esofágica/psicologia , Pais/psicologia , Feminino , Masculino , Inquéritos e Questionários , Recém-Nascido , Lactente , Adulto , Apoio Social
14.
Pediatr Surg Int ; 40(1): 149, 2024 Jun 03.
Artigo em Inglês | MEDLINE | ID: mdl-38829446

RESUMO

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.


Assuntos
Atresia Esofágica , Toracoscopia , Fístula Traqueoesofágica , Humanos , Fístula Traqueoesofágica/cirurgia , Fístula Traqueoesofágica/complicações , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Recém-Nascido , Toracoscopia/métodos , Masculino , Feminino , Estudos Retrospectivos , Resultado do Tratamento , Recém-Nascido de Baixo Peso , Fístula Anastomótica/cirurgia
15.
J Surg Res ; 301: 18-23, 2024 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-38905769

RESUMO

INTRODUCTION: Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a congenital malformation that occurs in about 1 in 2500-4000 live births. After surgical repair, despite the lack of evidence supporting the routine use of postoperative esophagram, most surgeons report obtaining an esophagram prior to enteral feeding. We hypothesized that abnormal indicators in vital signs, drain characteristics, and chest radiograph (CXR) could be used to screen for anastomotic leak, thus reducing the need for a routine esophagram. METHODS: A single institution, retrospective chart review of all patients born with EA with or without TEF between 2009 and 2022 was performed. Vital signs, postoperative CXR, chest drain characteristics, and esophagram results were analyzed for patients who underwent repair. RESULTS: Forty-five patients who underwent EA/TEF repair were included in the study, and 40 patients had routine esophagram. Out of the twenty-two patients who had at least one abnormal indicator, 14 (64%) had an anastomotic leak. Seventeen patients (43%) had the absence of abnormalities of all three indicators, and none of these patients had an anastomotic leak (100% negative predictive value). Moreover, changes in drain characteristics and vital signs together presented high sensitivity (87.5%), specificity (90%), and negative predictive value (94%). CONCLUSIONS: In the absence of abnormalities in vital signs, CXR, and drain characteristics in patients undergoing EA/TEF repair, routine esophagram can be safely avoided prior to enteral feeding. Abnormalities in drain characteristics and vital signs together were highly sensitive and specific for anastomotic leak, thus potentially eliminating the need for routine CXR and thereby minimizing radiation exposure and cost.

16.
J Ultrasound ; 2024 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-38904735

RESUMO

PURPOSE: This is an extremely rare case of complicated fetal esophageal atresia (EA) with tracheoesophageal fistula (TEF) and interrupted inferior vena cava (IVC) diagnosed by prenatal ultrsonography and successfully treated with surgical repair. METHODS: A 35-year-old pregnant woman was referred to our center for prenatal ultrasound, and the fetus was found to have a series of abnormalities, such as an interrupted IVC associated with a dilated azygos vein, an upper neck pouch sign of the thorax, and polyhydramnios. With suspicion of EA with TEF and interrupted IVC, the infant was born at 39 weeks of gestation, and successfully underwent the surgical operation. RESULTS: The baby was doing well after 21 months of follow-up. CONCLUSION: It is beneficial for the prenatal ultrasonic diagnosis of EA with TEF in optimizing labor care, postpartum treatment, and prompting neonatal management.

17.
Fetal Diagn Ther ; : 1-7, 2024 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-38934150

RESUMO

INTRODUCTION: Congenital microgastria (CM) is a rare condition due to early interruption of stomach development between the 4th and 8th week of gestation, leading to a small midline tubular stomach. Prenatal diagnosis of CM is a challenge with important implications. This study explores the value of biochemical amniotic fluid (AF) analysis and fetal magnetic resonance imaging (MRI) for the prenatal diagnosis of CM in case of nonvisible stomach on fetal ultrasound. CASE PRESENTATION: Four cases of CM were retrospectively investigated in terms of fetal ultrasound, MRI findings, and biochemical AF analyses. The patients were referred to the Prenatal Diagnosis Unit of the Hôpital Femme Mère Enfant (Lyon, France) at a mean age of 21 weeks of gestation for absent or small fetal stomach on ultrasound with a suspected diagnosis of esophageal atresia (EA). Ultrasound examination confirmed that the stomach was absent in two of the four fetuses and small in the other two. This feature was associated with a congenital heart defect in two cases and a terminal transverse limb defect in one case. Standard genetic workup (array-CGH) results were normal. Biochemical AF analysis, including the EA index, was not suggestive of EA. Fetal MRI showed a small midline tubular stomach, associated with a dilated esophagus, highly suggestive of CM. CONCLUSION: If the fetal stomach is absent on ultrasound, CM should be considered if the AF volume is normal, especially during the third trimester, and if the EA index is not suggestive of gastrointestinal obstruction. In these cases, the diagnosis can be confirmed by fetal MRI, through observation of a small midline tubular stomach associated with a dilated esophagus.

18.
Am J Med Genet A ; : e63792, 2024 Jun 17.
Artigo em Inglês | MEDLINE | ID: mdl-38884184

RESUMO

This study investigates the long-term outcomes of palliative and definitive surgeries for esophageal atresia (EA) in patients with trisomy 18 syndrome. A retrospective study included 25 cases undergoing EA surgery at our center between 2008 and 2022. The Palliative group (n = 16) comprised 13 cases with esophageal banding and 3 with tracheoesophageal fistula (TEF) division. The Definitive group (n = 9) included 5 cases with primary repair and 4 with staged repair following TEF division. The patient characteristics exhibited no significant differences between the groups. In the Definitive group, 56% (5/9) were successfully weaned off mechanical ventilation, compared with none in the Palliative group (p = 0.002). Survival-to-discharge rates were 31% (5/16) in the Palliative group and 67% (6/9) in the Definitive group. Home ventilator management was required for all 5 cases that required ventilation in the Palliative group, whereas only 17% (1/6) in the Definitive group needed it. The Palliative group also required continuous oral suction for persistent saliva removal, with two cases undergoing laryngotracheal separation. Overall, definitive surgery for EA in patients with trisomy 18 syndrome may provide enhanced respiratory stability, thereby improving the survival-to-discharge rate and overall quality of life for patients and their families.

19.
J Indian Assoc Pediatr Surg ; 29(3): 292-294, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38912014

RESUMO

Esophageal atresia (OA) with or without tracheoesophageal fistula affects approximately 1 in 4000 births and commonly presents with polyhydramnios. This appears to be the first report regarding the utility of cervical cerclage with serial amnioreduction to prolong the gestational age of a neonate with OA, thereby improving outcomes for reconstructive surgery.

20.
World J Gastrointest Surg ; 16(5): 1474-1481, 2024 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-38817291

RESUMO

Magnetic compression anastomosis is a promising treatment option for patients with complex esophageal atresia; but, at the present time, should not be the first therapeutic option in those cases where the surgeon can perform a primary anastomosis of the two ends of the esophagus with acceptable tension.

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