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1.
Semin Pediatr Surg ; 33(4): 151443, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38972214

RESUMO

With improvements in initial care for patients with congenital diaphragmatic hernia (CDH), the number of CDH patients with severe disease who are surviving to discharge has increased. This growing population of patients faces a unique set of long-term challenges, multisystem adverse outcomes, and post-intervention complications requiring specialized multidisciplinary follow-up. Early identification and intervention are essential to mitigate the potential morbidity associated with these challenges. This manuscript outlines a general framework for long-term follow-up for the CDH patient, including cardiopulmonary, gastrointestinal, neurodevelopmental, surgical, and quality of life outcomes.


Assuntos
Hérnias Diafragmáticas Congênitas , Humanos , Hérnias Diafragmáticas Congênitas/terapia , Hérnias Diafragmáticas Congênitas/cirurgia , Hérnias Diafragmáticas Congênitas/diagnóstico , Qualidade de Vida , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Complicações Pós-Operatórias/diagnóstico , Seguimentos , Recém-Nascido
2.
J Pediatr Surg ; 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38806317

RESUMO

OBJECTIVES: Our study examines if the disease severity profile of our Congenital Diaphragmatic Hernia (CDH) patient cohort adherent to long-term follow-up differs from patients lost to follow-up after discharge and examines factors associated with health care utilization. METHODS: Retrospective review identified CDH survivors born 2005-2019 with index repair at our institution. Primary outcome was long-term follow-up status: "active" or "inactive" according to clinic guidelines. Markers of CDH disease severity including CDH defect classification, oxygen use, tube feeds at discharge, and sociodemographic factors were examined as exposures. RESULTS: Of the 222 included patients, median age [IQR] was 10.2 years [6.7-14.3], 61% male, and 57 (26%) were insured by Medicaid. Sixty-three percent (139/222) of patients were adherent to follow-up. Seventy-six percent of patients discharged on tube feeds had active follow-up compared to 55% of patients who were not, with similar findings for oxygen at discharge (76% vs. 55%). Kaplan-Meier analysis showed patients with smaller defect size had earlier attrition compared to patients with larger defect size. Other race (Hispanic, Asian, Middle Eastern) patients had 2.87 higher odds of attrition compared to white patients (95% CI 1.18-7.0). Medicaid patients had 2.64 higher odds of attrition compared to private insurance (95% CI 1.23-5.66). CONCLUSION: Loss to follow-up was associated with race and insurance type. Disease severity was similar between the active and inactive clinic cohorts. Long-term CDH clinic publications should examine attrition to ensure reported outcomes reflect the discharged population. This study identified important factors to inform targeted interventions for follow-up adherence. LEVEL OF EVIDENCE: Level III.

3.
World J Pediatr Surg ; 7(2): e000758, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38618013

RESUMO

Neonates with congenital diaphragmatic hernia encounter a number of surgical and medical morbidities that persist into adulthood. As mortality improves for this population, these survivors warrant specialized follow-up for their unique disease-specific morbidities. Multidisciplinary congenital diaphragmatic hernia clinics are best positioned to address these complex long-term morbidities, provide long-term research outcomes, and help inform standardization of best practices in this cohort of patients. This review outlines long-term morbidities experienced by congenital diaphragmatic hernia survivors that can be addressed in a comprehensive follow-up clinic.

5.
J Pediatr Surg ; 59(8): 1451-1457, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38388286

RESUMO

BACKGROUND: We aimed to investigate the clinical characteristics and outcomes of patients with isolated left-sided congenital diaphragmatic hernia (CDH) who developed preoperative pneumothorax and determine its risk factors. METHODS: We performed an international cohort study of patients with CDH enrolled in the Congenital Diaphragmatic Hernia Study Group registry between January 2015 and December 2020. The main outcomes assessed included survival to hospital discharge and preoperative pneumothorax development. The cumulative incidence of pneumothorax was estimated by the Gray test. The Fine and Gray competing risk regression model was used to identify the risk factors for pneumothorax. RESULTS: Data for 2858 neonates with isolated left-sided CDH were extracted; 224 (7.8%) developed preoperative pneumothorax. Among patients with a large diaphragmatic defect, those with pneumothorax had a significantly lower rate of survival to discharge than did those without. The competing risks model demonstrated that a patent ductus arteriosus with a right-to-left shunt flow after birth (hazard ratio [HR]: 1.78; 95% confidence interval [CI]: 1.21-2.63; p = 0.003) and large defects (HR: 1.65; 95% CI: 1.13-2.42; p = 0.01) were associated with an increased risk of preoperative pneumothorax. Significant differences were observed in the cumulative incidence of pneumothorax depending on defect size and shunt direction (p < 0.001). CONCLUSIONS: Pneumothorax is a significant preoperative complication associated with increased mortality in neonates with CDH, particularly in cases with large defects. Large diaphragmatic defects and persistent pulmonary hypertension were found to be risk factors for preoperative pneumothorax development. LEVEL OF EVIDENCE: LEVEL Ⅲ Retrospective Comparative Study.


Assuntos
Hérnias Diafragmáticas Congênitas , Pneumotórax , Humanos , Hérnias Diafragmáticas Congênitas/cirurgia , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/mortalidade , Pneumotórax/etiologia , Pneumotórax/epidemiologia , Recém-Nascido , Fatores de Risco , Masculino , Feminino , Estudos Retrospectivos , Incidência , Período Pré-Operatório , Estudos de Coortes , Sistema de Registros
6.
J Pediatr Surg ; 58(9): 1656-1662, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36709093

RESUMO

BACKGROUND: The optimal timing of surgical repair for infants with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO) support remains controversial. The risk of surgical bleeding is considered by many centers as a primary factor in determining the preferred timing of CDH repair for infants requiring ECMO support. This study compares surgical bleeding following CDH repair on ECMO in early versus delayed fashion. METHODS: A retrospective review of 146 infants who underwent CDH repair while on ECMO support from 1995 to 2021. Early repair occurred during the first 48 h after ECMO cannulation (ER) and delayed repair after 48 h (DR). Surgical bleeding was defined by the requirement of reoperative intervention for hemostasis or decompression. RESULTS: 102 infants had ER and 44 infants DR. Surgical bleeding was more frequent in the DR group (36% vs 5%, p < 0.001) with an odds ratio of 11.7 (95% CI: 3.48-39.3, p < 0.001). Blood urea nitrogen level on the day of repair was significantly elevated among those who bled (median 63 mg/dL, IQR 20-85) vs. those who did not (median 9 mg/dL, IQR 7-13) (p < 0.0001). Duration of ECMO support was shorter in the ER group (median 13 vs 18 days, p = 0.005). Survival was not statistically different between the two groups (ER 60% vs. DR 57%, p = 0.737). CONCLUSION: We demonstrate a significantly lower incidence of bleeding and shorter duration of ECMO with early CDH repair. Azotemia was a strong risk factor for surgical bleeding associated with delayed CDH repair on ECMO. LEVEL OF EVIDENCE: Level III cohort study.


Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas , Lactente , Humanos , Hérnias Diafragmáticas Congênitas/cirurgia , Estudos de Coortes , Perda Sanguínea Cirúrgica , Fatores de Risco , Estudos Retrospectivos
7.
Ann Surg ; 277(3): 520-527, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-34334632

RESUMO

OBJECTIVE: To determine if risk-adjusted survival of patients with CDH has improved over the last 25 years within centers that are long-term, consistent participants in the CDH Study Group (CDHSG). SUMMARY BACKGROUND DATA: The CDHSG is a multicenter collaboration focused on evaluation of infants with CDH. Despite advances in pediatric surgical and intensive care, CDH mortality has appeared to plateau. Herein, we studied CDH mortality rates amongst long-term contributors to the CDHSG. METHODS: We divided registry data into 5-year intervals, with Era 1 (E1) beginning in 1995, and analyzed multiple variables (operative strategy, defect size, and mortality) to assess evolution of disease characteristics and severity over time. For mortality analyses, patients were risk stratified using a validated prediction score based on 5-minute Apgar (Apgar5) and birth weight. A risk-adjusted, observed to expected (O:E) mortality model was created using E1 as a reference. RESULTS: 5203 patients from 23 centers with >22years of participation were included. Birth weight, Apgar5, diaphragmatic agenesis, and repair rate were unchanged over time (all P > 0.05). In E5 compared to E1, minimally invasive and patch repair were more prevalent, and timing of diaphragmatic repair was later (all P < 0.01). Overall mortality decreased over time: E1 (30.7%), E2 (30.3%), E3 (28.7%), E4 (26.0%), E5 (25.8%) ( P = 0.03). Risk-adjusted mortality showed a significant improvement in E5 compared to E1 (OR 0.78, 95% CI 0.62-0.98; P = 0.03). O:E mortality improved over time, with the greatest improvement in E5. CONCLUSIONS: Risk-adjusted and observed-to-expected CDH mortality have improved over time.


Assuntos
Hérnias Diafragmáticas Congênitas , Lactente , Criança , Humanos , Hérnias Diafragmáticas Congênitas/cirurgia , Peso ao Nascer , Sistema de Registros
8.
J Pediatr Surg ; 58(2): 209-212, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36396471

RESUMO

AIM OF THE STUDY: Small bowel obstruction (SBO) is a known complication after congenital diaphragmatic hernia (CDH) repair, which can require surgery and even extensive bowel resection causing short bowel syndrome (SBS). We investigate whether specific bowel rotation and fixation can be used as a predictor for SBO including volvulus. METHODS: A retrospective review of 256 CDH survivors following repair from 2003 to 2020 was performed. Operative notes and upper gastrointestinal series (UGI) were screened to determine the rotation and fixation of the bowel. Primary outcomes included SBO occurrence, SBO treated surgically, and volvulus. For statistical analysis Fisher's exact test was utilized. RESULTS: Twenty-two (9%) patients presented with SBO and majority, 19 (86%), required surgery. Adhesion were observed in 10 (45%), recurrence in 5 (23%), and extensive volvulus leading to SBS in 3 (14%). Both rotation and fixation were recorded in 117 (46%). Presence of left CDH with malrotation and nonfixation was a significant predictor for SBO requiring surgery (P<0.05 vs all other groups). All 3 patients with extensive volvulus had left CDH with nonfixed bowel (100%), however only 1 had malrotation (33%). CONCLUSIONS: Malrotation and nonfixation are associated with increased SBO in CDH. Normal rotation is not protective and patients are still at risk for volvulus resulting in SBS. SBO requiring surgical intervention is common in CDH. Bowel rotation and fixation are important determinants that, should be routinely documented and education about the risk of SBO should be included in family counseling. LEVEL OF EVIDENCE: Level IV - Case Series.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Hérnias Diafragmáticas Congênitas , Obstrução Intestinal , Volvo Intestinal , Humanos , Hérnias Diafragmáticas Congênitas/complicações , Hérnias Diafragmáticas Congênitas/cirurgia , Volvo Intestinal/cirurgia , Volvo Intestinal/complicações , Rotação , Obstrução Intestinal/epidemiologia , Obstrução Intestinal/etiologia , Obstrução Intestinal/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Estudos Retrospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia
9.
J Pediatr Gastroenterol Nutr ; 75(5): 578-583, 2022 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-35897140

RESUMO

OBJECTIVES: Pediatric functional constipation (FC) may require invasive evaluations [like colon manometry (CM)] and surgical interventions [including diverting ostomy (DO)]. We evaluated the utility of CM in guiding surgery after DO. METHODS: Children with medically refractory FC undergoing an ostomy were included. Institutional Review Board approval was obtained for this retrospective study. Demographics and CM variables [high amplitude propagating contractions (HAPCs)] were recorded. Outcome measures: response to ostomy closure defined as successful if no need for further surgery after ostomy closure, and improvement on baseline CM after ostomy. A CM-guided ostomy closure algorithm was developed based on previous studies. We evaluated the association between response to ostomy closure and demographics, ostomy indication and CM improvement, and evaluated the role of CM predicting response using algorithm. RESULTS: A total of 60 children underwent ostomy for FC (median age: 7.1 years, range 0.15-23.6 years, 50% female). Ostomy was closed in 30 patients and deemed successful in 23 of 30. CM was performed in 42 of 60 patients before ostomy and in 29 of 30 before ostomy closure. We found no association between ostomy outcome and age, gender, weight, imaging studies, follow-up time, time with ostomy, HAPCs, and CM improvement. We found an association between failed response and ostomy indication of antegrade colonic enemas (ACE) failure ( P = 0.026) and successful response when ostomy closure was guided by algorithm ( P = 0.03). CONCLUSIONS: DO is a useful intervention in selected children with medically refractory FC, improving colon motility in most. CM can successfully guide the timing and type of ostomy closure. Larger studies are needed to further validate our findings.


Assuntos
Constipação Intestinal , Ileostomia , Humanos , Criança , Feminino , Lactente , Pré-Escolar , Adolescente , Adulto Jovem , Adulto , Masculino , Estudos Retrospectivos , Constipação Intestinal/cirurgia , Colo/cirurgia , Manometria/métodos , Motilidade Gastrointestinal/fisiologia
10.
J Matern Fetal Neonatal Med ; 35(6): 1070-1074, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32188329

RESUMO

BACKGROUND: Infants prenatally suspected of having a choledochal cyst (CDC) typically undergo ultrasound imaging shortly after birth. This study sought to evaluate features on the initial postnatal ultrasound (IPU) that could identify newborns at risk for early complications. METHODS: Following IRB approval, patients from four US fetal centers with prenatal suspicion for CDC and postnatal imaging from 2000 to 2017 were reviewed. Imaging and clinical courses were assessed. RESULTS: Forty-two patients had prenatal ultrasounds suspicious for CDC. Nineteen (45.2%) were excluded due to diagnostic revision (n = 9), cyst resolution (n = 5), lack of IPU measurements (n = 3), or lack of follow-up (n = 2). The 23 remaining patients were included in the study. Of these, five (21.7%) developed symptoms at a median age of 16.5 days (IQR 16-19 days), and 18 (78.3%) remained asymptomatic throughout the first year after birth. Five patients (21.7%) had cysts ≥ 4.5 cm on IPU (Symptomatic: n = 3; Asymptomatic: n = 2). Eighteen patients (78.3%) had cysts < 4.5 cm on IPU (Symptomatic: n = 2; Asymptomatic: n = 16). An IPU cyst size ≥ 4.5 cm was associated with neonatal symptom manifestation (p = 0.048), with 88.9% specificity (95% CI 65.3-98.6%) and 60% sensitivity (95% CI 14.7-94.7%). CONCLUSIONS: In newborns with prenatally diagnosed CDC, a cyst size ≥ 4.5 cm on IPU is associated with symptom development during the first month after birth and therefore early cyst excision is recommended.


Assuntos
Cisto do Colédoco , Cisto do Colédoco/diagnóstico por imagem , Cisto do Colédoco/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Parto , Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos
11.
J Pediatr Surg ; 57(2): 195-198, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34865833

RESUMO

AIM OF STUDY: Congenital Diaphragmatic Hernia (CDH) is associated with lung hypoplasia and pulmonary hypertension. Many patients receive care in specialty centers requiring air travel upon discharge and for subsequent follow-up care. Premature infants can experience significant hypoxia in flight, but this has not been studied in the CDH population. This report describes our center's experience with simulated altitude testing among CDH patients. METHODS: In a single center retrospective cohort study, CDH patients who underwent a High Altitude Simulation Test (HAST) from 2006 to 2019 were analyzed. HAST simulates increased altitude by reducing oxygen tension to an FIO2 of 0.15. Patients were tested only when flight was anticipated. Patients requiring oxygen were challenged on their baseline requirement. To pass, patients had to maintain oxygen saturation > 90%, and 94% if diagnosed with pulmonary hypertension. Supplemental oxygen was titrated as needed to achieve this goal. RESULTS: Of twenty patients tested, only six (30%) passed on their first attempt. Ten (50%) eventually passed, after an average of 3.2 additional attempts over 1.8 years. No patient passed initially who utilized ECMO support, diaphragmatic agenesis, or had elevated right ventricular pressure on echocardiogram. All patients achieved the targeted SpO2 with supplemental oxygen. CONCLUSION: CDH patients experience hypoxia when exposed to the simulated hypobaric nature of air travel and therefore may become hypoxic in flight, requiring oxygen supplementation. Disease severity seems to correlate with risk of in-flight hypoxia. This data suggests that CDH patients should be screened to assess their need for supplemental oxygen to ensure safe air travel. LEVEL OF EVIDENCE: Level 4 case series.


Assuntos
Hérnias Diafragmáticas Congênitas , Hipertensão Pulmonar , Altitude , Hérnias Diafragmáticas Congênitas/complicações , Humanos , Hipertensão Pulmonar/etiologia , Lactente , Testes de Função Respiratória , Estudos Retrospectivos
12.
Fetal Diagn Ther ; 48(5): 400-406, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33951639

RESUMO

INTRODUCTION: Several fetal therapies involve repeated amniotic fluid intervention. We hypothesize that a minimally invasive approach can be used to safely implant an intrauterine catheter infusion system in a fetal ovine model for chronic use during pregnancy. METHOD: Five pregnant sheep underwent operation between gestational days 110 and 115 (term 145 days). A Codman® implantable infusion pump was adapted for intrauterine use. The chamber was placed in the maternal flank and the tunneled catheter laparoscopically inserted into the amniotic cavity, secured with a pursestring. Three had an additional uterine anchoring suture. Ewes were sacrificed after natural delivery, and the uterus underwent gross and microscopic analyses. RESULTS: There were no maternal mortalities, abortions, or preterm labor. Pumps were accessed and remained functional throughout gestation. Four ewes delivered healthy term lambs; the other delivered twins with failure to progress and demise. On necropsy, catheters secured with an anchoring suture remained in place, while the other 2 dislodged during labor. There was no chorioamnionitis by culture or histology. CONCLUSION: Laparoscopically placed intra-amniotic infusion catheters were implanted safely and remained functional until delivery in an ovine model. This novel approach has promise in providing safe, durable amniotic fluid access for the potential treatment of fetal disease.


Assuntos
Corioamnionite , Trabalho de Parto Prematuro , Líquido Amniótico , Animais , Catéteres , Feminino , Gravidez , Ovinos , Útero
13.
Fetal Diagn Ther ; 48(3): 235-242, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33730724

RESUMO

INTRODUCTION: Prenatal suprarenal lesions represent diverse pathologies. This study investigated prenatal imaging features and regression patterns associated with specific lesion diagnoses. METHODS: This is a multicenter retrospective review of fetuses with prenatally diagnosed suprarenal lesions between 2001 and 2019. Prenatal ultrasound and MRI characteristics, postnatal imaging, and clinical course were reviewed. Prenatal imaging findings were compared by the most common diagnoses and regression patterns. RESULTS: Forty-four fetuses were prenatally diagnosed with suprarenal lesions. Diagnoses included pulmonary sequestration (n = 12; 27.3%), adrenal hemorrhage (n = 12; 27.3%), upper quadrant cyst (including 2 duplication cysts, 1 splenic cyst, and 3 indeterminate cysts), neuroblastoma (n = 4), adrenal hyperplasia (n = 3), bilateral adrenal calcifications (n = 1), and indeterminate lesions (n = 6). Sequestrations were uniformly left-sided (100 vs. 50%; p = 0.014) and diagnosed earlier in gestation than adrenal hemorrhages (p = 0.025). Sequestrations were also significantly more likely to have a prenatal feeding vessel (p = 0.005), low T1 MRI signal (p = 0.015), and no MRI blood products (p = 0.018) compared to adrenal hemorrhages. When comparing all 44 patients, a prenatal feeding vessel and low T1 signal on prenatal MRI were significantly associated with lesion persistence (p = 0.003; p = 0.044). DISCUSSION/CONCLUSION: Imaging findings on prenatal ultrasound and MRI aid in the diagnosis of suprarenal lesions, including differentiating pulmonary sequestrations and adrenal hemorrhages.


Assuntos
Sequestro Broncopulmonar , Ultrassonografia Pré-Natal , Sequestro Broncopulmonar/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Gravidez , Diagnóstico Pré-Natal , Estudos Retrospectivos
14.
Ann Surg ; 274(1): 186-194, 2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-31425289

RESUMO

OBJECTIVE: To determine the optimal timing of congenital diaphragmatic hernia (CDH) repair after extracorporeal membrane oxygenation (ECMO) cannulation. SUMMARY BACKGROUND DATA: The timing of CDH repair after ECMO cannulation remains a controversial topic due to studies with low power or strong selection bias. METHODS: This is a 2-aim retrospective cohort study based on the CDH Study Group registry for the period of 2007-2017. Aim 1-Compare On versus After ECMO repair. Aim 2-Compare Early versus Late repair on ECMO. In order to minimize selection bias and account for non-repairs, subjects in each aim were stratified into study groups based on their treatment center's characteristics. In each aim, the study groups were matched based on propensity score (PS). The main outcomes included mortality rate and incidence of non-repair. RESULTS: In aim 1, 136 patients remained in each group after PS matching. Compared to the After ECMO group, patients in the On ECMO group demonstrated a lower mortality rate, hazard ratio (HR) 0.54 (0.38, 0.77) (P < 0.001), and lower incidence of non-repair, 5.9% versus 33.8% (P < 0.001). In aim 2, 77 patients remained in each group after PS matching. Compared to the Late group, Early repair of CDH on ECMO was associated with a lower mortality rate, HR 0.51 (0.33, 0.77) (P = 0.002), and lower incidence of non-repair, 9.1% versus 44.2% (P < 0.001). CONCLUSIONS: The approach of early repair after ECMO cannulation is associated with improved survival compared to delayed surgical correction.


Assuntos
Oxigenação por Membrana Extracorpórea , Hérnias Diafragmáticas Congênitas/cirurgia , Herniorrafia , Tempo para o Tratamento , Feminino , Humanos , Recém-Nascido , Masculino , Pontuação de Propensão , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Análise de Sobrevida
15.
Am J Med Genet A ; 182(8): 1890-1895, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32573094

RESUMO

Esophageal atresia (EA) is a congenital anomaly occurring in 2.3 per 10,000 live births. Due to advances in prenatal imaging, EA is more readily diagnosed, but data on the associated genetic diagnoses, other anomalies, and postnatal outcome for fetuses diagnosed prenatally with EA are scarce. We collected data from two academic medical centers (n = 61). Our data included fetuses with suspected EA on prenatal imaging that was confirmed postnatally and had at least one genetic test. In our cohort of 61 cases, 29 (49%) were born prematurely and 19% of those born alive died in the first 9 years of life. The most commonly associated birth defects were cardiac anomalies (67%) and spine anomalies (50%). A diagnosis was made in 61% of the cases; the most common diagnoses were vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula with esophageal atresia, radial or renal dysplasia, and limb anomalies association (43%, although 12% met only 2 of the criteria), trisomy 21 (5%), and CHARGE syndrome (5%). Our findings suggest that most fetuses with prenatally diagnosed EA have one or more additional major anomaly that warrants a more comprehensive clinical genetics evaluation. Fetuses diagnosed prenatally appear to represent a cohort with a worse outcome.


Assuntos
Anormalidades Múltiplas/diagnóstico , Anormalidades Congênitas/diagnóstico , Atresia Esofágica/diagnóstico , Cardiopatias Congênitas/diagnóstico , Diagnóstico Pré-Natal , Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/fisiopatologia , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/fisiopatologia , Síndrome de Down/diagnóstico , Síndrome de Down/diagnóstico por imagem , Síndrome de Down/fisiopatologia , Atresia Esofágica/complicações , Atresia Esofágica/diagnóstico por imagem , Atresia Esofágica/fisiopatologia , Esôfago/diagnóstico por imagem , Esôfago/fisiopatologia , Feminino , Feto/anormalidades , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/fisiopatologia , Humanos , Recém-Nascido , Rim/diagnóstico por imagem , Rim/fisiopatologia , Masculino , Gravidez , Traqueia/diagnóstico por imagem , Traqueia/fisiopatologia
16.
J Pediatr Surg ; 55(11): 2322-2328, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32200977

RESUMO

BACKGROUND/PURPOSE: The Spitz classification for esophageal atresia with/without tracheoesophageal fistula (EA/TEF) predicts mortality. This study evaluates the contemporary relevance of the Spitz classification and investigates predictors of morbidity. METHODS: EA/TEF patients born between 1995 and 2018 at two centers were retrospectively reviewed. Clinical variables including sex, prenatal diagnosis, birth weight, prematurity, major congenital heart disease (MCHD), and pre-operative mechanical ventilation (POMV) were collected. Index admission composite morbidity was considered positive if: length-of-stay >90th percentile (139 days), ventilation days >90th percentile (24 days), and/or gastrostomy was used for long-term feeding. Multivariable regression determined predictors of index admission mortality and composite morbidity. A composite morbidity predictive algorithm was created. ROC curves evaluated model discrimination. RESULTS: Of 253 patients, 13 (5.1%) experienced index admission mortality. Of the patients not suffering mortality, 74 (31.6%) experienced composite morbidity. Only MCHD predicted mortality (p = 0.001); birth weight did not (p = 0.173). There was no difference between the Spitz classification and MCHD alone in predicting mortality risk (p = 0.198); both demonstrated very good discrimination. Prenatal diagnosis, POMV, prematurity, and male sex predicted composite morbidity risk (p < 0.001; p = 0.008; p = 0.009; p = 0.05). An algorithm incorporating these predictors demonstrated good discrimination (AUC = 0.784; 95% CI: 0.724, 0.844). CONCLUSIONS: The Spitz classification maintains contemporary relevance for mortality risk, though birth weight can be de-emphasized. A new morbidity risk algorithm is proposed for early postnatal counseling. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level IV.


Assuntos
Atresia Esofágica/diagnóstico , Atresia Esofágica/mortalidade , Fístula Traqueoesofágica , Feminino , Humanos , Recém-Nascido , Masculino , Morbidade , Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Fístula Traqueoesofágica/epidemiologia , Resultado do Tratamento
17.
Pediatr Surg Int ; 36(3): 325-331, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31707604

RESUMO

PURPOSE: This retrospective cohort study compares the natural history of patients with extralobar sequestrations (ELS) who do not undergo intervention with those who undergo resection to assess the safety of non-operative management. METHODS: 126 patients with pulmonary sequestrations or congenital pulmonary airway malformations born between 1999 and 2016 were identified. 49 patients had ELS on postnatal imaging, but two were excluded for associated congenital diaphragmatic hernia. Demographic and clinical data were retrospectively reviewed, with phone follow-up for non-operative patients with no records for > 1 year. Statistical analysis was by Fisher's exact test or Wilcoxon signed-rank test (two-tailed p < 0.05). RESULTS: 40% (19/47) were managed non-operatively and 60% (28/47) underwent resection. Non-operative patients were less likely to have an intrathoracic ELS: 47% (9/19) vs. 75% (21/28), p = 0.07. No symptoms were attributable directly to the ELS. Non-operative patients had median follow-up 3.2 years, during which time 88% (15/17) of ELS decreased in size on serial imaging. For patients who underwent resection, there was 100% concordance between imaging and intraoperative findings. There was no evidence of inflammation, infection or malignancy on final pathology, though 57% (16/28) of resected lesions had foci of non-aerated cysts. CONCLUSIONS: Although further longitudinal study is required, this study supports the safety of non-operative ELS management.


Assuntos
Sequestro Broncopulmonar/terapia , Tratamento Conservador/métodos , Sequestro Broncopulmonar/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Estudos Longitudinais , Masculino , Pneumonectomia , Estudos Retrospectivos , Resultado do Tratamento
18.
J Pediatr Surg ; 54(6): 1257-1260, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30827488

RESUMO

Jejunoileal atresia (JIA) is a congenital defect that can result in significant loss of bowel length. The traditional classification of JIA was first proposed by Grosfeld and includes 4 subtypes. Among these, type IIIB, or apple-peel atresia, is characterized by a proximal atretic jejunum and a distal segment of spiraled bowel that terminates at the cecum. Owing to this anatomy, patients with type IIIB JIA are at increased risk for short bowel syndrome and intestinal failure. In this report, we described the case of a neonate with a prenatal diagnosis of JIA. At exploration, she was initially found to have a type IIIB atresia. However, instead of terminating at the cecum, the distal spiraled segment was followed by 75 cm of normal small bowel and mesentery. Surgical correction proceeded with minimal resection and primary anastomosis. She recovered well from this procedure, tolerated full enteral nutrition by mouth, and displayed good weight gain at outpatient follow-up. Owing to the unique anatomy of the gastrointestinal tract in this case report, we propose the addition of a new class of JIA, type IIIC, to better reflect its prognostication and surgical management.


Assuntos
Atresia Intestinal , Doenças do Jejuno , Procedimentos Cirúrgicos do Sistema Digestório , Feminino , Humanos , Íleo/cirurgia , Recém-Nascido , Jejuno/cirurgia
19.
Pediatr Surg Int ; 35(7): 813-821, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30770976

RESUMO

BACKGROUND: Although the quality of online health information (OHI) for adult surgical conditions is well described, the availability of quality OHI for pediatric surgical conditions, and the comparison to that of adult surgical OHI, remains undefined. METHODS: Medical and lay terms for 15 pediatric and 15 adult surgical conditions were searched using Google in English. The Health on the Net Foundation, a non-governmental OHI accreditation body, designates approval for quality websites. We compared the role of patient population while controlling for disease incidence (pediatric vs. adult), term complexity (medical vs. lay), and order (earlier vs. later listing of websites) on availability of quality OHI among the first 100 websites for each term. RESULTS: Among the first 100 websites, the adjusted mean number of quality websites was 11.80 for pediatric vs. 17.92 for adult medical search terms, and 13.27 for pediatric vs. 18.20 for adult lay search terms (P < 0.05 for all). Term complexity did not affect quality, and earlier appearing results were more likely to be of high quality. CONCLUSION: Availability of quality pediatric surgical OHI lags behind that of adult surgical OHI, even when controlling for disease incidence. These findings highlight the potential need for increased quality OHI in pediatric surgery.


Assuntos
Cirurgia Geral/estatística & dados numéricos , Internet , Informática Médica/métodos , Especialidades Cirúrgicas/estatística & dados numéricos , Telemedicina/métodos , Adulto , Criança , Humanos
20.
J Pediatr Surg ; 54(3): 486-490, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30409477

RESUMO

PURPOSE: Children failing medical management for severe constipation and/or fecal incontinence may undergo surgical intervention for antegrade enema administration. We present a modification of the laparoscopic-assisted percutaneous endoscopic cecostomy (LAPEC) procedure that allows primary placement of a skin-level device. METHODS: A single-institution retrospective review was performed from 2009 to 2015. In the modified technique the colonoscope is advanced to the cecum, cecal suspension sutures are placed under laparoscopic visualization, and percutaneous needle puncture of the cecum is performed under direct laparoscopic and endoscopic visualization. A skin-level cecostomy tube is then placed over a guide wire. Patient characteristics and 30-day results were analyzed by Fisher's exact test. RESULTS: Fifty-two patients underwent attempted LAPEC. Successful LAPEC using both laparoscopic and endoscopic guidance was achieved in 46 (88.5%). A MIC-KEY device was placed in 38. Corflo PEG tube placement was necessary in 14 due to high BMI (mean 28.4). Colonoscopy failed to reach the cecum in 6 and laparoscopy alone was utilized to achieve successful tube placement. Cecostomy site infections occurred in 3 (5.8%), only in those undergoing PEG placement using a pull technique (p < 0.05). CONCLUSION: Primary placement of a skin-level device was successful in the majority of patients undergoing cecostomy tube placement for bowel management utilizing antegrade colonic enemas. This technique avoids a second anesthesia for tube conversion. Visualization via colonoscopy with the use of cecal suspension sutures is recommended. High BMI necessitates initial placement of a PEG tube and complications exclusively occurred in this group. TYPE OF STUDY: Clinical. LEVEL OF EVIDENCE: IV Case series study.


Assuntos
Cecostomia/métodos , Colonoscopia/métodos , Constipação Intestinal/cirurgia , Enema/métodos , Laparoscopia/métodos , Cecostomia/efeitos adversos , Criança , Pré-Escolar , Colo/cirurgia , Colonoscopia/efeitos adversos , Enema/efeitos adversos , Incontinência Fecal/etiologia , Feminino , Humanos , Laparoscopia/efeitos adversos , Masculino , Estudos Retrospectivos
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