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Management of Splenic Vascular Anomalies: A Review of 17 Pediatric Cases at a Single Institution
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-25 DOI: 10.1016/j.jpedsurg.2025.162254
Josephine L. Schmidt , Michael B. Gyimah , Bea B. Jeon , Amir H. Pezeshkmehr , Alex Chau , Ionela Iacobas , Kristy L. Rialon

Background

Splenic Vascular Anomalies (SVA) are rare splenic masses seen in the pediatric population. There is limited information regarding the management and appropriate follow-up for these patients. The aim of this study was to review our experience and create an algorithm to help guide clinical care.

Methods

A retrospective review of pediatric patients diagnosed with SVA from 2011 to 2024 was performed. Clinical history and outcomes data were summarized descriptively. A Mann–Whitney U test was performed to compare SVA diameters between surgical and nonsurgical groups.

Results

Seventeen patients met inclusion criteria for this study. SVA was identified incidentally in 15 patients (88 %). The median maximal SVA diameter was 5.2 cm (range: 5–7 cm) for the six surgical patients and 3.6 cm (range: 1–5.7 cm) for the 11 nonsurgical patients (p = 0.009). Five patients (83 %) underwent total splenectomy, and one patient (17 %) underwent partial splenectomy. Indications for splenectomy included persistent abdominal pain, large size on imaging, and concern for splenic rupture. Preoperative imaging correlated with postoperative pathology in 4/6 (66 %) surgical patients. Among all patients, the median number of days between the first imaging study to identify the SVA and the last study monitoring the SVA was 632 days (range: 0–2337 days). The median change in SVA maximal diameter during that time was 0.05 cm (range: −1.6 – 0.9 cm).

Conclusion

SVA can be monitored with serial imaging and do not undergo rapid growth. Most operative interventions are performed based on SVA size (>5 cm) and rarely on symptoms.

Level of evidence

4.
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引用次数: 0
A Growing Spine, a Lifelong Footprint: Rethinking Carbon and the Socioeconomic Exposome in Paediatric Spine Surgery.
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-24 DOI: 10.1016/j.jpedsurg.2025.162256
Christos Tsagkaris, Saverio Marmentini
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引用次数: 0
Neonatal Surgery for Congenital Lung Malformations: Indications, Outcomes and Association With Malignancy
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-21 DOI: 10.1016/j.jpedsurg.2025.162253
Ancuta Muntean , Laura Marsland , Oishi Sikdar , Christopher Harris , Niyi Ade-Ajayi , Shailesh B. Patel , James Cook , Maria Sellars , Anne Greenough , Kypros Nicolaides , Mark Davenport

Aim of the study

Congenital lung malformations (CLM) are a heterogeneous group of anomalies diagnosed with increasing frequency antenatally. Postnatal management is dictated by the clinical status in the early neonatal period. We report our centre's experience in managing symptomatic neonates with CLM.

Method

Single centre retrospective review (January 1995–December 2023) of all infants with CLM that underwent thoracotomy for resectional surgery during the neonatal period. Data are quoted as median (IQR), unless stated otherwise. SPSS version 28 (IBM) and GraphPad Prism were used for analysis of data. A P value of ≤0.05 was regarded as significant.

Results

During the study period 228 patients underwent surgery for CLM. Forty-nine infants (21.5 %) (33 males) required surgery during the first 30 days of life. Surgery in those infants was performed at 5 (1–10) days (emergency surgery, n = 24; expedited, n = 25). The indications included respiratory distress (n = 29; mechanical ventilation n = 16) and size of lesion (n = 20). Antenatal diagnosis was established in 45 (91.8 %) infants and 18 had undergone antenatal intervention with 7 fetuses having more than one procedure. The CLM was left sided in 29 (59 %). Postoperative stay for the surviving infants was 14 (7–21) days with extubation being achieved after 2 (1–4) days. The overall survival was 91.8 % (45/49).
Infants that underwent antenatal intervention had a higher rate of postnatal pneumothorax (P = 0.01) and required higher FiO2 delivery (P = 0.01). Infants that underwent surgery in an emergency setting were found to have a higher need for antenatal intervention (P = 0.01), postnatally required intubation (P < 0.001) with higher FiO2 delivery (p < 0.001) and developed pneumothorax (P = 0.002).
Histopathology findings showed the entire spectrum of CLM, with 6 cases of CPAM Type 1 associated with mucinous adenocarcinoma. Cross sectional radiological imaging was available for 31 patients and in 4 patients with malignancy, a solid component of the lesion was appreciable on CT. Median follow-up period was 6 (2.1–11) years. Sixteen (35 %) children developed a degree of chest wall deformity through none have required surgical intervention.

Conclusions

This is one of the largest series documenting the postnatal management of symptomatic neonates with CLM. One fifth of the cohort undergoing surgery for CLM required neonatal intervention. Histology at surgery was heterogeneous with 12 % of cases being associated with mucinous adenocarcinoma.
研究目的先天性肺畸形(CLM)是一类异质性畸形,在产前诊断的频率越来越高。产后管理取决于新生儿早期的临床状况。我们报告了本中心在处理有症状的 CLM 新生儿方面的经验。方法单中心回顾性研究(1995 年 1 月至 2023 年 12 月)所有在新生儿期接受开胸切除手术的 CLM 婴儿。除非另有说明,否则数据均以中位数(IQR)表示。数据分析采用 SPSS 28 版(IBM)和 GraphPad Prism。研究期间,228 名患者接受了 CLM 手术。49名婴儿(21.5%)(33名男性)在出生后30天内需要接受手术。这些婴儿的手术时间为 5 (1-10) 天(急诊手术,24 例;快速手术,25 例)。手术指征包括呼吸窘迫(29 例;机械通气 16 例)和病变大小(20 例)。45名(91.8%)婴儿的产前诊断已经确定,18名接受过产前干预,其中7名胎儿接受过一次以上的产前干预。29例(59%)的CLM为左侧。存活婴儿的术后住院时间为 14 天(7-21 天),2 天(1-4 天)后即可拔管。接受产前干预的婴儿产后气胸发生率更高(P = 0.01),所需的 FiO2 分娩率更高(P = 0.01)。在急诊环境下接受手术的婴儿需要更多的产前干预(P = 0.01),产后需要插管(P < 0.001),FiO2分娩率更高(P < 0.001),并出现气胸(P = 0.002)。组织病理学结果显示了CLM的整个谱系,其中6例CPAM 1型与粘液腺癌有关。有31例患者获得了横断面放射影像学检查结果,其中4例患者为恶性肿瘤,CT检查结果显示病灶中可见实性成分。中位随访时间为 6(2.1-11)年。16名患儿(35%)出现了一定程度的胸壁畸形,但无一例需要手术干预。五分之一因 CLM 而接受手术的患儿需要新生儿干预。手术组织学表现不一,12%的病例伴有粘液腺癌。
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引用次数: 0
VACTERL Screening in Newborns With Anorectal Malformations - An Opportunity to Optimize Screening Practices, add Gynecologic and Spinal Conditions, and Utilize a New Acronym: VACTE(G)RLS.
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-20 DOI: 10.1016/j.jpedsurg.2025.162252
Thomas O Xu, Rachel E Hanke, Kirsten Das, Melanie Bowser, Butool Hisam, Inbal Samuk, Hussein Wissanji, Erin Teeple, Allison Mayhew, John S Myseros, Andrea Badillo, Marc A Levitt, Briony K Varda, Christina Feng

Introduction: Spinal cord and gynecologic anomalies may be overlooked as part of the traditional VACTERL screening for patients with anorectal malformations (ARM). We investigated the rates of associated anomalies in patients with ARM using the Pediatric Health Information Systems (PHIS) database.

Methods: A retrospective multi-institutional cohort study was performed between 1/2016-12/2022. ARM patients were identified using ICD10 diagnosis and procedure codes. We assessed the rate of associated anomaly diagnosis at a patient's initial newborn admission and across all hospital encounters using ICD10 diagnosis codes.

Results: 1467 patients (34 % female) were observed across 46 hospitals. The rates of VACTERL diagnoses on initial admission were 35.0 % vertebral/spinal, 72.9 % cardiac, 9.5 % TEF, 34.7 % renal, and 13.3 % limb. Diagnosis rates were all higher across all hospital encounters: 45.4 % vertebral/spinal, 77.4 % cardiac, 10.2 % TEF, 39.9 % renal, and 15.7 % limb. Among female patients, 16.1 % had a congenital gynecologic malformation diagnosed at birth and 25.8 % across all hospital encounters. Among sub-category diagnoses, the rates of lower Mullerian tract malformations (+11 %), spinal cord malformations (+12 %), and vesicoureteral reflux (+14 %) had the greatest discrepancy between newborn diagnosis and diagnosis across all hospital encounters on a one-tailed comparison of proportions (all p < 0.001) CONCLUSION: The most commonly associated anomalies in ARM are cardiac, vertebral/spinal, renal, and gynecologic (in females). Many are diagnosed as newborns however some are diagnosed later, suggesting an opportunity to improve screening, especially with spinal cord and gynecologic malformations. We suggest changing the VACTERL acronym to VACTE(G)RLS to emphasize the importance and frequency of these malformations in patients with ARM.

Level of evidence: Observational study, III.

Study type: Multi-institutional retrospective cohort IRB APPROVAL: STUDY00000902.

{"title":"VACTERL Screening in Newborns With Anorectal Malformations - An Opportunity to Optimize Screening Practices, add Gynecologic and Spinal Conditions, and Utilize a New Acronym: VACTE(G)RLS.","authors":"Thomas O Xu, Rachel E Hanke, Kirsten Das, Melanie Bowser, Butool Hisam, Inbal Samuk, Hussein Wissanji, Erin Teeple, Allison Mayhew, John S Myseros, Andrea Badillo, Marc A Levitt, Briony K Varda, Christina Feng","doi":"10.1016/j.jpedsurg.2025.162252","DOIUrl":"https://doi.org/10.1016/j.jpedsurg.2025.162252","url":null,"abstract":"<p><strong>Introduction: </strong>Spinal cord and gynecologic anomalies may be overlooked as part of the traditional VACTERL screening for patients with anorectal malformations (ARM). We investigated the rates of associated anomalies in patients with ARM using the Pediatric Health Information Systems (PHIS) database.</p><p><strong>Methods: </strong>A retrospective multi-institutional cohort study was performed between 1/2016-12/2022. ARM patients were identified using ICD10 diagnosis and procedure codes. We assessed the rate of associated anomaly diagnosis at a patient's initial newborn admission and across all hospital encounters using ICD10 diagnosis codes.</p><p><strong>Results: </strong>1467 patients (34 % female) were observed across 46 hospitals. The rates of VACTERL diagnoses on initial admission were 35.0 % vertebral/spinal, 72.9 % cardiac, 9.5 % TEF, 34.7 % renal, and 13.3 % limb. Diagnosis rates were all higher across all hospital encounters: 45.4 % vertebral/spinal, 77.4 % cardiac, 10.2 % TEF, 39.9 % renal, and 15.7 % limb. Among female patients, 16.1 % had a congenital gynecologic malformation diagnosed at birth and 25.8 % across all hospital encounters. Among sub-category diagnoses, the rates of lower Mullerian tract malformations (+11 %), spinal cord malformations (+12 %), and vesicoureteral reflux (+14 %) had the greatest discrepancy between newborn diagnosis and diagnosis across all hospital encounters on a one-tailed comparison of proportions (all p < 0.001) CONCLUSION: The most commonly associated anomalies in ARM are cardiac, vertebral/spinal, renal, and gynecologic (in females). Many are diagnosed as newborns however some are diagnosed later, suggesting an opportunity to improve screening, especially with spinal cord and gynecologic malformations. We suggest changing the VACTERL acronym to VACTE(G)RLS to emphasize the importance and frequency of these malformations in patients with ARM.</p><p><strong>Level of evidence: </strong>Observational study, III.</p><p><strong>Study type: </strong>Multi-institutional retrospective cohort IRB APPROVAL: STUDY00000902.</p>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":" ","pages":"162252"},"PeriodicalIF":2.4,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542333","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
eHealth in Pediatric Surgery: Impact on Postsurgical Care After Reconstructive Surgery for Anorectal Malformations and Hirschsprung's Disease
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-19 DOI: 10.1016/j.jpedsurg.2025.162251
Matilda Wester Fleur , Björn A. Johnsson , Charlotte Castor , Pernilla Stenström

Background

To support care after discharge following surgery for anorectal malformations (ARM) and Hirschsprung's disease (HD), an eHealth device was invented, offering families bilateral communication through chat, photo, questionaries and video.

Aim

To explore the impact of eHealth on postoperative patterns of expert consultation, complications and treatment after discharge following surgery for ARM and HD, respectively, compared to conventional care.

Method

Interventional prospective case control study observing postoperative care 30 days after discharge after ARM and HD-reconstructions July 2018–July 2023, comparing outcomes between families using the eHealth device versus conventional care.

Results

Consecutively included were 95/141 families (eHealth: ARM n = 48, HD n = 25; respective conventional care: ARM n = 16, HD n = 6). Days to first contact after discharge were fewer for eHealth users, both for ARM 2 vs. 5.5 (p = 0.000) and HD 1 vs 7 (p = 0.000). Days with consultations were more numerous using eHealth: ARM 9 vs 4 (p = 0.000) and HD 12 vs 3.5 (p = 0.001) specifically nurse counseling; ARM 6 versus 1 (p = 0.000) and HD 11 vs 2.5 (p = 0.001). In ARM patients the complication frequency was 23 % (eHealth) versus 50 % (conventional), and the complication severity was lower: Clavien-Madadi 0 (0–4) versus 1 (0–3) (p = 0.040). Treatment adjustments were more frequent for HD patients using eHealth (3 vs. 2 (p = 0.041)).

Conclusion

Use of eHealth after ARM respective HD reconstructions implies earlier and more frequent postoperative counseling, especially with nurses. When using eHealth complications were less severe in ARM-patients and treatment adjustments more frequent in the HD patients, compared to those under conventional care.
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引用次数: 0
Simplifying Universal Pediatric Pre-operative Anesthesia Screening: Analysis of NSQIP Data to Evaluate the NARCO Score as a Tool for Pediatric Anesthesia Risk Stratification
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-18 DOI: 10.1016/j.jpedsurg.2025.162248
Gillian Michaelson , Lucille Hu , Shelley Ohliger , Anuja Sarode , Eiichi Miyasaka , Anne K. Mackow

Background

Universal pre-operative anesthesia screening may be resource-prohibitive and burdensome to hospitals and patients. However, patients at greatest anesthetic risk cannot be identified without some basis for risk-stratification.

Aims

This study investigated if a modified version of the Neurological, Airway, Respiratory, Cardiovascular, and Other (NARCO) pre-surgical pediatric risk score could stratify patients into high- and low-anesthetic-risk groups that would be predictive of perioperative adverse events.

Methods

A retrospective cohort study was conducted using 132,881 records from the pediatric database of the American College of Surgeons National Surgical Quality Improvement Program (2019 ACS NSQIP-P®). Recorded comorbidities were assigned NARCO categories and presumptive NARCO scores based on non-granular data. Two methodologies were used--one in which non-granular comorbidities were considered high-risk (overestimating the high-risk group) and another in which these were considered low-risk (overestimating the low-risk group). These groups were evaluated by demographic and adverse outcome data using chi-square or Wilcoxon-Mann-Whitney analyses (p < 0.05).

Results

Both methodologies accurately reflected overestimation of high- or low-risk patients, when comparing NARCO to ASA scores. In both methodologies, the occurrences of 23 of 24 adverse events were significantly (p < 0.05) associated with the high-risk group.

Conclusion

A modified version of the NARCO pre-surgical risk assessment can stratify pediatric patients into high- and low-risk groups based on non-granular patient data. In both methodologies, this stratification identifies patients at highest risk for perioperative adverse events, which is a marker for those who require more intensive pre-surgical screening.

Type of study

Retrospective cohort study using de-identified information from a large.

Level of evidence

3.
{"title":"Simplifying Universal Pediatric Pre-operative Anesthesia Screening: Analysis of NSQIP Data to Evaluate the NARCO Score as a Tool for Pediatric Anesthesia Risk Stratification","authors":"Gillian Michaelson ,&nbsp;Lucille Hu ,&nbsp;Shelley Ohliger ,&nbsp;Anuja Sarode ,&nbsp;Eiichi Miyasaka ,&nbsp;Anne K. Mackow","doi":"10.1016/j.jpedsurg.2025.162248","DOIUrl":"10.1016/j.jpedsurg.2025.162248","url":null,"abstract":"<div><h3>Background</h3><div>Universal pre-operative anesthesia screening may be resource-prohibitive and burdensome to hospitals and patients. However, patients at greatest anesthetic risk cannot be identified without some basis for risk-stratification.</div></div><div><h3>Aims</h3><div>This study investigated if a modified version of the Neurological, Airway, Respiratory, Cardiovascular, and Other (NARCO) pre-surgical pediatric risk score could stratify patients into high- and low-anesthetic-risk groups that would be predictive of perioperative adverse events.</div></div><div><h3>Methods</h3><div>A retrospective cohort study was conducted using 132,881 records from the pediatric database of the American College of Surgeons National Surgical Quality Improvement Program (2019 ACS NSQIP-P®). Recorded comorbidities were assigned NARCO categories and presumptive NARCO scores based on non-granular data. Two methodologies were used--one in which non-granular comorbidities were considered high-risk (overestimating the high-risk group) and another in which these were considered low-risk (overestimating the low-risk group). These groups were evaluated by demographic and adverse outcome data using chi-square or Wilcoxon-Mann-Whitney analyses (<em>p</em> &lt; <em>0.05</em>).</div></div><div><h3>Results</h3><div>Both methodologies accurately reflected overestimation of high- or low-risk patients, when comparing NARCO to ASA scores. In both methodologies, the occurrences of 23 of 24 adverse events were significantly (p &lt; 0.05) associated with the high-risk group.</div></div><div><h3>Conclusion</h3><div>A modified version of the NARCO pre-surgical risk assessment can stratify pediatric patients into high- and low-risk groups based on non-granular patient data. In both methodologies, this stratification identifies patients at highest risk for perioperative adverse events, which is a marker for those who require more intensive pre-surgical screening.</div></div><div><h3>Type of study</h3><div>Retrospective cohort study using de-identified information from a large.</div></div><div><h3>Level of evidence</h3><div>3.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 5","pages":"Article 162248"},"PeriodicalIF":2.4,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143549702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Guidelines for Wound Care Considerations in Pilonidal Disease: Lessons Learned From a Pilonidal Treatment Clinic.
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-18 DOI: 10.1016/j.jpedsurg.2025.162247
Norah E Liang, Claire Abrajano, Somin Jo, Bill Chiu
{"title":"Guidelines for Wound Care Considerations in Pilonidal Disease: Lessons Learned From a Pilonidal Treatment Clinic.","authors":"Norah E Liang, Claire Abrajano, Somin Jo, Bill Chiu","doi":"10.1016/j.jpedsurg.2025.162247","DOIUrl":"https://doi.org/10.1016/j.jpedsurg.2025.162247","url":null,"abstract":"","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":" ","pages":"162247"},"PeriodicalIF":2.4,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143515765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Real-world Multi-institutional Data From the Midwest Pediatric Surgery Consortium (MWPSC) to Assess the Effect of Delayed Kasai Procedure on Biliary Drainage in Patients With Biliary Atresia
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-18 DOI: 10.1016/j.jpedsurg.2025.162250
Zishaan A. Farooqui , Stephen J. Hartman , Alyssa E. Stetson , Emily J. Schepers , Karen E. Speck , Samir K. Gadepalli , Kyle J. Van Arendonk , Christina Georgeades , Dave R. Lal , Katherine J. Deans , Peter C. Minneci , Jordan C. Apfeld , Jacqueline M. Saito , Grace Z. Mak , Mark B. Slidell , Caroline Lemoine , Riccardo Superina , Tiffany N. Wright , Cynthia D. Downard , Lekha P. Devara , Gregory M. Tiao

Purpose

Early Kasai portoenterostomy (KPE) for infants with biliary atresia (BA) increases the chance of transplant-free survival (TFS). However, early timing of KPE is not consistently achieved in the United States. Clearance of jaundice at three months is predictive of TFS. Among a cohort of patients with BA, we investigated institutional variability in the initiation of hyperbilirubinemia evaluation and operative timing to identify factors associated with successful jaundice clearance.

Methods

A multi-institutional, retrospective study was performed at eleven U.S. tertiary children’s hospitals. Infants diagnosed with BA between 10/1/2015–10/1/2020 were identified. Age at initiation of diagnostic workup and age at KPE were collected. Adjusted multivariable logistic regression was used to determine factors associated with direct bilirubin normalization at three months following KPE.

Results

In 161 infants, the median age at initiation of jaundice evaluation was 35 days (IQR 8–60). Among 148 patients who underwent KPE, median age at surgery was 53 days (IQR 37.3–67.5). Each 10-day increase in age at KPE was associated with a 18.8 % decrease in odds of normalizing bilirubin at three months (OR 0.81, 95 % CI 0.66–0.99), with infants who underwent KPE ≤50 days significantly more likely to normalize bilirubin (OR 2.6, CI 1.1–6.1) compared to KPE >50 days. There was significant variation among institutions in the time from initiation of workup to KPE (range 0–24.5 days, p = 0.02) and the odds of patients normalizing direct bilirubin at three months (range 0.04–0.89, p = 0.044).

Conclusion

Our results confirmed that increasing age at KPE decreases the odds of clearing bilirubin at three months post-KPE. We identified significant institutional variability in the time from workup to KPE that may have impacted the likelihood of successful biliary drainage.

Level of Evidence

IV (Well-designed case–control or cohort study).
{"title":"Real-world Multi-institutional Data From the Midwest Pediatric Surgery Consortium (MWPSC) to Assess the Effect of Delayed Kasai Procedure on Biliary Drainage in Patients With Biliary Atresia","authors":"Zishaan A. Farooqui ,&nbsp;Stephen J. Hartman ,&nbsp;Alyssa E. Stetson ,&nbsp;Emily J. Schepers ,&nbsp;Karen E. Speck ,&nbsp;Samir K. Gadepalli ,&nbsp;Kyle J. Van Arendonk ,&nbsp;Christina Georgeades ,&nbsp;Dave R. Lal ,&nbsp;Katherine J. Deans ,&nbsp;Peter C. Minneci ,&nbsp;Jordan C. Apfeld ,&nbsp;Jacqueline M. Saito ,&nbsp;Grace Z. Mak ,&nbsp;Mark B. Slidell ,&nbsp;Caroline Lemoine ,&nbsp;Riccardo Superina ,&nbsp;Tiffany N. Wright ,&nbsp;Cynthia D. Downard ,&nbsp;Lekha P. Devara ,&nbsp;Gregory M. Tiao","doi":"10.1016/j.jpedsurg.2025.162250","DOIUrl":"10.1016/j.jpedsurg.2025.162250","url":null,"abstract":"<div><h3>Purpose</h3><div>Early Kasai portoenterostomy (KPE) for infants with biliary atresia (BA) increases the chance of transplant-free survival (TFS). However, early timing of KPE is not consistently achieved in the United States. Clearance of jaundice at three months is predictive of TFS. Among a cohort of patients with BA, we investigated institutional variability in the initiation of hyperbilirubinemia evaluation and operative timing to identify factors associated with successful jaundice clearance.</div></div><div><h3>Methods</h3><div>A multi-institutional, retrospective study was performed at eleven U.S. tertiary children’s hospitals. Infants diagnosed with BA between 10/1/2015–10/1/2020 were identified. Age at initiation of diagnostic workup and age at KPE were collected. Adjusted multivariable logistic regression was used to determine factors associated with direct bilirubin normalization at three months following KPE.</div></div><div><h3>Results</h3><div>In 161 infants, the median age at initiation of jaundice evaluation was 35 days (IQR 8–60). Among 148 patients who underwent KPE, median age at surgery was 53 days (IQR 37.3–67.5). Each 10-day increase in age at KPE was associated with a 18.8 % decrease in odds of normalizing bilirubin at three months (OR 0.81, 95 % CI 0.66–0.99), with infants who underwent KPE ≤50 days significantly more likely to normalize bilirubin (OR 2.6, CI 1.1–6.1) compared to KPE &gt;50 days. There was significant variation among institutions in the time from initiation of workup to KPE (range 0–24.5 days, <em>p</em> = 0.02) and the odds of patients normalizing direct bilirubin at three months (range 0.04–0.89, <em>p</em> = 0.044).</div></div><div><h3>Conclusion</h3><div>Our results confirmed that increasing age at KPE decreases the odds of clearing bilirubin at three months post-KPE. We identified significant institutional variability in the time from workup to KPE that may have impacted the likelihood of successful biliary drainage.</div></div><div><h3>Level of Evidence</h3><div>IV (Well-designed case–control or cohort study).</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 5","pages":"Article 162250"},"PeriodicalIF":2.4,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143521025","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Gastrostomy Tube Placement in Patients With Trisomy 13 and 18: Surgical Decision Making and Outcomes
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-18 DOI: 10.1016/j.jpedsurg.2025.162249
Sindhu V. Mannava , Rodica Muraru , Fikir M. Mesfin , Niloufar Hafezi , Zoe M. Saenz , James C. Soderstrom , Jasmin D. Sanchez , Deborah F. Billmire , Gabrielle C. Geddes , Brian W. Gray

Purpose

Trisomy 13 (T13) and trisomy 18 (T18) are chromosomal abnormalities which portend high rates of feeding dysfunction and infant mortality risk. Although gastrostomy tube (GT) placement is commonly performed in this population, there is limited data assessing outcomes associated with this procedure. Our aim was to determine survival outcomes among GT and non-GT patients with T13/18.

Methods

We performed a retrospective cohort study of patients with T13 and T18 treated at our institution from 2005 through 2020. We compared baseline characteristics and survival data between GT and non-GT patients and performed multivariable survival analysis.

Results

We analyzed a total of 86 patients (23 GT, 63 non-GT). Over one-third of GT patients underwent the procedure during initial admission and most GTs were used for longer than one year (60.9 %). Significantly more GT patients survived initial discharge (87 % vs. 57.1 %, p < 0.001) and were alive at follow-up (43.5 % vs. 6.3 %, p < 0.001) compared to non-GT patients. Thirty-day post-discharge survival was determined solely by GT status and not impacted by predictors. GT patients had reduced overall mortality risk compared to non-GT patients in the first year of life (HR = 0.10 [95 % CI 0.04–0.29], p < 0.001) and during the fifteen-year study period (HR = 0.15 [95 % CI 0.06–0.35], p < 0.001).

Conclusions

GT status predicted 30-day post-discharge survival in our cohort. GT patients had reduced overall mortality risk compared to non-GT patients. In patients with expected survival to initial discharge and feeding difficulty, GT placement at or after initial admission may be associated with improved survival outcomes.

Level of evidence

III.
{"title":"Gastrostomy Tube Placement in Patients With Trisomy 13 and 18: Surgical Decision Making and Outcomes","authors":"Sindhu V. Mannava ,&nbsp;Rodica Muraru ,&nbsp;Fikir M. Mesfin ,&nbsp;Niloufar Hafezi ,&nbsp;Zoe M. Saenz ,&nbsp;James C. Soderstrom ,&nbsp;Jasmin D. Sanchez ,&nbsp;Deborah F. Billmire ,&nbsp;Gabrielle C. Geddes ,&nbsp;Brian W. Gray","doi":"10.1016/j.jpedsurg.2025.162249","DOIUrl":"10.1016/j.jpedsurg.2025.162249","url":null,"abstract":"<div><h3>Purpose</h3><div>Trisomy 13 (T13) and trisomy 18 (T18) are chromosomal abnormalities which portend high rates of feeding dysfunction and infant mortality risk. Although gastrostomy tube (GT) placement is commonly performed in this population, there is limited data assessing outcomes associated with this procedure. Our aim was to determine survival outcomes among GT and non-GT patients with T13/18.</div></div><div><h3>Methods</h3><div>We performed a retrospective cohort study of patients with T13 and T18 treated at our institution from 2005 through 2020. We compared baseline characteristics and survival data between GT and non-GT patients and performed multivariable survival analysis.</div></div><div><h3>Results</h3><div>We analyzed a total of 86 patients (23 GT, 63 non-GT). Over one-third of GT patients underwent the procedure during initial admission and most GTs were used for longer than one year (60.9 %). Significantly more GT patients survived initial discharge (87 % vs. 57.1 %, p &lt; 0.001) and were alive at follow-up (43.5 % vs. 6.3 %, p &lt; 0.001) compared to non-GT patients. Thirty-day post-discharge survival was determined solely by GT status and not impacted by predictors. GT patients had reduced overall mortality risk compared to non-GT patients in the first year of life (HR = 0.10 [95 % CI 0.04–0.29], p &lt; 0.001) and during the fifteen-year study period (HR = 0.15 [95 % CI 0.06–0.35], p &lt; 0.001).</div></div><div><h3>Conclusions</h3><div>GT status predicted 30-day post-discharge survival in our cohort. GT patients had reduced overall mortality risk compared to non-GT patients. In patients with expected survival to initial discharge and feeding difficulty, GT placement at or after initial admission may be associated with improved survival outcomes.</div></div><div><h3>Level of evidence</h3><div>III.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 5","pages":"Article 162249"},"PeriodicalIF":2.4,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143488119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Functional Outcomes for Patients With Congenital Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence Based Practice Committee
IF 2.4 2区 医学 Q1 PEDIATRICS Pub Date : 2025-02-18 DOI: 10.1016/j.jpedsurg.2025.162243
Kristy L. Rialon , Caitlin Smith , Rebecca M. Rentea , Shannon N. Acker , Robert Baird , Alana L. Beres , Henry L. Chang , Emily R. Christison-Lagay , Diana L. Diesen , Brian R. Englum , Katherine W. Gonzalez , Brian C. Gulack , P. Benson Ham III , Carlos T. Huerta , Afif N. Kulaylat , Tamar L. Levene , Donald J. Lucas , Sara A. Mansfield , Christopher Pennell , Robert L. Ricca , Akemi Kawaguchi

Objective

Consensus on functional outcomes for anorectal malformations (ARM) is hindered by the heterogeneity of the available literature. Optimal patient counseling includes discussion of short- and long-term outcomes for bowel and urinary continence, sexual and psychosocial function, transitional care, and quality of life. This systematic review examines and summarizes the current literature available related to functional outcomes for children with ARM.

Methods

The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted consensus-based questions regarding anorectal malformations. Pertinent articles from 1985 to 2021 were reviewed.

Results

More than 10,843 publications were reviewed with 109 being included in the final recommendations. Recommendations are primarily based on C-D levels of evidence. Continence and constipation rates were higher in patients with perineal fistula and rectovestibular fistula, although symptoms tended to improve as patients got older. Urological anomalies are common and longer term urologic surveillance protocols for patients with ARM need to be further outlined. Sexual and psychosocial issues are common, but ARM patients can have a good quality of life when gastrointestinal symptoms are minimized. Many of the problems associated with ARM can persist into adulthood, supporting structured care plans as patients transition to adult care.

Conclusions

Evidence to support best practices and achieve optimal outcomes for patients with ARM is lacking for many aspects of care. Multi-institutional registries have begun to address management and prognosis for these patients. Prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.

Level of evidence

4.
{"title":"Functional Outcomes for Patients With Congenital Anorectal Malformations: A Systematic Review and Evidence-based Guideline From the APSA Outcomes and Evidence Based Practice Committee","authors":"Kristy L. Rialon ,&nbsp;Caitlin Smith ,&nbsp;Rebecca M. Rentea ,&nbsp;Shannon N. Acker ,&nbsp;Robert Baird ,&nbsp;Alana L. Beres ,&nbsp;Henry L. Chang ,&nbsp;Emily R. Christison-Lagay ,&nbsp;Diana L. Diesen ,&nbsp;Brian R. Englum ,&nbsp;Katherine W. Gonzalez ,&nbsp;Brian C. Gulack ,&nbsp;P. Benson Ham III ,&nbsp;Carlos T. Huerta ,&nbsp;Afif N. Kulaylat ,&nbsp;Tamar L. Levene ,&nbsp;Donald J. Lucas ,&nbsp;Sara A. Mansfield ,&nbsp;Christopher Pennell ,&nbsp;Robert L. Ricca ,&nbsp;Akemi Kawaguchi","doi":"10.1016/j.jpedsurg.2025.162243","DOIUrl":"10.1016/j.jpedsurg.2025.162243","url":null,"abstract":"<div><h3>Objective</h3><div>Consensus on functional outcomes for anorectal malformations (ARM) is hindered by the heterogeneity of the available literature. Optimal patient counseling includes discussion of short- and long-term outcomes for bowel and urinary continence, sexual and psychosocial function, transitional care, and quality of life. This systematic review examines and summarizes the current literature available related to functional outcomes for children with ARM.</div></div><div><h3>Methods</h3><div>The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee drafted consensus-based questions regarding anorectal malformations. Pertinent articles from 1985 to 2021 were reviewed.</div></div><div><h3>Results</h3><div>More than 10,843 publications were reviewed with 109 being included in the final recommendations. Recommendations are primarily based on C-D levels of evidence. Continence and constipation rates were higher in patients with perineal fistula and rectovestibular fistula, although symptoms tended to improve as patients got older. Urological anomalies are common and longer term urologic surveillance protocols for patients with ARM need to be further outlined. Sexual and psychosocial issues are common, but ARM patients can have a good quality of life when gastrointestinal symptoms are minimized. Many of the problems associated with ARM can persist into adulthood, supporting structured care plans as patients transition to adult care.</div></div><div><h3>Conclusions</h3><div>Evidence to support best practices and achieve optimal outcomes for patients with ARM is lacking for many aspects of care. Multi-institutional registries have begun to address management and prognosis for these patients. Prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.</div></div><div><h3>Level of evidence</h3><div>4.</div></div>","PeriodicalId":16733,"journal":{"name":"Journal of pediatric surgery","volume":"60 5","pages":"Article 162243"},"PeriodicalIF":2.4,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143511964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Journal of pediatric surgery
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