Pediatric Surgery International最新文献

{"title":"Does serum albumin at the onset of necrotisıng enterocolitis predict severe disease in preterm infants?","authors":"Gökçe Çıplak, Fatma Nur Sarı, Elif Emel Erten, Müjdem Nur Azılı, Süleyman Arif Bostancı, Cüneyt Tayman, Evrim Alyamaç Dizdar, Emrah Şenel","doi":"10.1007/s00383-024-05850-6","DOIUrl":"https://doi.org/10.1007/s00383-024-05850-6","url":null,"abstract":"<p><strong>Objective: </strong>To investigate whether laboratory markers obtained at the onset of necrotising enterocolitis (NEC) predict the severity of the disease in preterm infants.</p><p><strong>Methods: </strong>Prospective cohort study conducted in a tertiary referance hospital. A total of 88 preterm infants were included in the study. Of those, 60 infants had the diagnosis of severe NEC, while the remaining 28 infants constituted the non-severe NEC group. Severe NEC was defined as surgical NEC or NEC-related mortality. Infants with and without severe NEC were compared in terms of demographic, clinical and laboratory characteristics.</p><p><strong>Results: </strong>At the onset of disease, infants with severe NEC noted to have lower platelet count and serum ALB levels (p = 0.011, p = 0.004; respectively), whereas higher CRP, and serum lactate levels (p = 0.009, p = 0.008; respectively). Multiple binary logistic regression analyses showed that CRP (1.03(1.01-1.05), p = 0.024) and serum albumin level (0.16(0.04-0.64), p = 0.010) were statistically significant independent risk factors for severe NEC. The optimal cut-off value for the serum ALB level was found to be 23 g/L with 52% sensitivity (95%CI: 37-68%) and 84% specificity (95%CI: 60-97%) (AUC 0.727; p = 0.002).</p><p><strong>Conclusion: </strong>Serum ALB level at NEC onset might be a reliable biomarker for severe disease in preterm infants.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"267"},"PeriodicalIF":1.5,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical management protocol for disk battery ingestion.","authors":"Arianna Roggero, Vittorio Guerriero, Federica Lena, Serena Arrigo, Francesco Santoro, Roberto D'Agostino, Maria Beatrice Damasio, Francesca Rizzo, Paolo Gandullia, Andrea Moscatelli, Girolamo Mattioli, Michele Torre","doi":"10.1007/s00383-024-05849-z","DOIUrl":"https://doi.org/10.1007/s00383-024-05849-z","url":null,"abstract":"<p><strong>Purpose: </strong>Disk battery (DB) ingestion in children can lead to severe complications and mortality. This study details our experience in managing DB ingestion and its complications.</p><p><strong>Methods: </strong>We analyzed data from all patients treated for DB ingestion at our hospital from June 2010 to January 2024. A protocol established in 2010 requires angio-CT scans for esophageal DB cases and a multidisciplinary approach involving gastroenterologists, otolaryngologists, pediatric and airway surgeons, and cardiac surgeons.</p><p><strong>Results: </strong>We treated 22 patients. In June 2010, following the tragic death of a patient from an undiagnosed DB ingestion that led to an aortoesophageal fistula, our protocol was established. All DBs were removed endoscopically. Four patients needed additional surgery: two had tracheal resection/anastomosis and esophageal repair for large tracheoesophageal fistulas; one required aortic wall reinforcement with a patch; one underwent endoscopic removal with a sternal split to explore the aortic arch. All 22 patients survived and recovered clinically. One developed bilateral vocal cord palsy as a complication.</p><p><strong>Conclusion: </strong>Effective management of DB ingestion complications necessitates a collaborative, multidisciplinary approach. Our protocol has improved management strategies and patient outcomes.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"268"},"PeriodicalIF":1.5,"publicationDate":"2024-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical outcomes of children with COVID-19 and appendicitis: a propensity score matched analysis.","authors":"Marjorie N Odegard, Olivia A Keane, Shadassa A Ourshalimian, Christopher J Russell, William G Lee, Makayla L O'Guinn, Laura M C Houshmand, Lorraine I Kelley-Quon","doi":"10.1007/s00383-024-05817-7","DOIUrl":"10.1007/s00383-024-05817-7","url":null,"abstract":"<p><strong>Objective: </strong>Early in the COVID-19 pandemic, many children with appendicitis and COVID-19 were initially treated non-operatively and later underwent interval appendectomy. Currently, children with both appendicitis and COVID-19 frequently undergo upfront appendectomy. The impact of this return to upfront surgical management on patient outcomes is unknown. This study compared outcomes of pediatric patients with and without COVID-19 infection undergoing appendectomy.</p><p><strong>Study design: </strong>A retrospective cohort study of children < 21y who underwent appendectomy from 3/19/2020 to 7/31/2022 at 50 Pediatric Health Information System children's hospitals was conducted. Children with documented COVID-19 were identified. Exclusions included preoperative ventilator or supplemental oxygen dependence, and missing data. To evaluate COVID-19 positive versus COVID-19 negative patients, we used a propensity score matched on sociodemographics, comorbidities, laparoscopy, perforation, and hospital. Chi-square and Mann-Whitney U tests identified differences between groups in length of stay, postoperative drain placement, 30-day re-admission, and mechanical ventilation requirements.</p><p><strong>Results: </strong>Overall, 51,861 children of median age 11y (IQR: 8-14) underwent appendectomy, of whom 1,440 (2.3%) had COVID-19. Most were male (60.3%), White (72.1%) and non-Hispanic (61.4%). Public insurance was the most common (47.5%). We created a matched cohort of 1,360 COVID-19 positive and 1,360 COVID-19 negative children. Children with COVID-19 had shorter hospitalizations (1d, IQR: 1-4 vs. 2d, IQR: 1-5, p = 0.03), less postoperative peritoneal drain placement (2.4% vs. 4.1%, p = 0.01), and fewer 30-day readmissions (9.0% vs. 11.4%, p = 0.04). However, no difference in incidence or duration of mechanical ventilation (p > 0.05) was detected.</p><p><strong>Conclusions: </strong>Our findings suggest that upfront appendectomy for children with appendicitis and COVID-19 has similar outcomes compared to children without COVID-19.</p><p><strong>Level of evidence: </strong>Level III.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"266"},"PeriodicalIF":1.5,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11461633/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142392366","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgical treatment of invasive pulmonary fungal infections in immunocompromised pediatric patients: Aspergillus spp. and other emerging fungi.","authors":"Sergio López-Fernández, José Andrés Molino, Pere Soler-Palacín, Natalia Mendoza-Palomar, Maria Luz Uria Oficialdegui, Marta Martos Rodríguez, Manuel López, Gabriela Guillén","doi":"10.1007/s00383-024-05851-5","DOIUrl":"https://doi.org/10.1007/s00383-024-05851-5","url":null,"abstract":"<p><strong>Purpose: </strong>Invasive Pulmonary Fungal Infections (IPFIs) represent a diagnostic and therapeutic challenge. The exact role of surgery is not well defined. This study analyzes our experience with surgical treatment of IPFI in immunocompromised pediatric patients and, secondarily, compares IPFI caused by Aspergillus spp. with other fungal infections.</p><p><strong>Methods: </strong>This is a retrospective review (2000-2019) of patients with IPFI surgically treated at our pediatric institution. Statistical analysis was used to compare data between Aspergillus spp. and non-Aspergillus IPFI.</p><p><strong>Results: </strong>Twenty-five patients (64% female) underwent 29 lung resections. Median age at surgery was 7.19 years (1.63-19.14). The most frequent underlying condition (64%) was acute leukemia. Surgical indications included persistence or worsening of symptoms and pathological image findings (52%) or asymptomatic suspicious lesions in patients scheduled for intensive cytotoxic treatments or hematopoietic stem cell transplantation (48%). All patients underwent atypical lung resections, except one lobectomy. Aspergillus spp. was the most frequently isolated pathogen (68%). Follow-up was 4.07 years (0.07-18.07). Surgery-related mortality was 0%, but 4 patients died in the 100 days following surgery (2 due to disseminated fungal infection); the remaining 21 did not show signs of IPFI recurrence. Non-specific consolidations on CT scan were more frequent in non-Aspergillus IPFI (p < 0.05).</p><p><strong>Conclusion: </strong>Surgical treatment of IPFI should be considered as a part of the treatment in selected pediatric immunocompromised patients, and it may have both diagnostic and therapeutic advantages over non-surgical management. When there is clinical suspicion of IPFI but CT scan shows unspecific alterations, the possibility of a non-Aspergillus IPFI should be considered.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"263"},"PeriodicalIF":1.5,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"National centralization of Hirschsprung's disease in Sweden: a comparison of postoperative outcome.","authors":"Linnea Söderström, Christina Graneli, Daniel Rossi, Kristine Hagelsteen, Anna Gunnarsdottir, Jenny Oddsberg, Pär-Johan Svensson, Helena Borg, Matilda Bräutigam, Elisabet Gustafson, Anna Löf Granström, Pernilla Stenström, Tomas Wester","doi":"10.1007/s00383-024-05842-6","DOIUrl":"10.1007/s00383-024-05842-6","url":null,"abstract":"<p><strong>Background: </strong>In Sweden, surgical treatment of Hirschsprung's disease (HSCR) was centralized from four to two pediatric surgery centers 1st of July 2018. In adults, centralization of surgical care for complex or rare diseases seems to improve quality of care. There is little evidence supporting centralization of pediatric surgical care. The aim of this study was to assess surgical management and postoperative outcome in HSCR patients following centralization of care.</p><p><strong>Methods: </strong>This study retrospectively analyzed data of patients with HSCR that had undergone pull-through at a pediatric surgery center in Sweden from 1st of July 2013 to 30th of June 2023. Patients managed from 1st of July 2013 to 30th of June 2018 (before centralization) were compared with patients managed from 1st of July 2018 to 30th of June 2023 (after centralization) regarding surgical treatment, unplanned procedures under general anesthesia or readmissions up to 90 days after pull-through as well as complications classified according to Clavien-Madadi up to 30 days after pull-through.</p><p><strong>Results: </strong>In the 5-year period prior to centralization, 114 individuals from 4 treating centers were included and compared to 83 patients from 2 treating centers in the second period. There was no difference regarding age at pull-through or proportion of patients with a stoma prior to pull-through. An increase of laparoscopically assisted endorectal pull-through (8.8% to 39.8%) was observed (p < 0.001). No significant differences were seen in postoperative hospital stay, unplanned procedures under general anesthesia, or readmissions up to 90 days after pull-through. There was no difference in severe complications (Clavien-Madadi ≥ 3); however, HAEC treated with antibiotics increased following centralization (10.5-24.1%; p = 0.018).</p><p><strong>Conclusion: </strong>Centralization of care for HSCR does not seem to delay time to pull-through nor reduce severe complications, unplanned procedures under general anesthesia or readmissions up to 90 days after pull-through. The increased HAEC rate may be due to increased awareness of mild HAEC.</p><p><strong>Level of evidence: </strong> Level III.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"265"},"PeriodicalIF":1.5,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11455800/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Utility of the oxygenation index in management of congenital diaphragmatic hernia: a report from a Thai University Surgical Centre.","authors":"Issariyaporn Kulngamnetr, Pharuhad Pongmee, Paul D Losty, Suraida Aeesoa, Ampaipan Boonthai","doi":"10.1007/s00383-024-05848-0","DOIUrl":"10.1007/s00383-024-05848-0","url":null,"abstract":"<p><strong>Background: </strong>Oxygenation index (OI) is associated with severity of newborn pulmonary hypertension (PH) in congenital diaphragmatic hernia (CDH). Higher OI may indicate worst degree(s) of PH.</p><p><strong>Objectives: </strong>This study reports OI dynamic(s) over the first 72 h of life and its correlation with (1) perioperative morbidity and (2) CDH mortality.</p><p><strong>Methods: </strong>Medical records of inborn CDH babies during 2002-2022 were examined. OI on Days (s) 1-3 and perioperative OI trends were recorded. Operation (primary vs patch repair) and survival rates (%) were studied.</p><p><strong>Results: </strong>Fifty-five CDH newborns (54.5% male: 45.5% female)-mean birth GA 37.5 ± 2.7 wks. had a mean birth weight 2813 ± 684 g with prenatal diagnosis in 32.7% cases. 52/55 (94.5%) were intubated at birth and HFOV deployed in 29 (55.8%). Those requiring HFOV had higher OI on DOL1 (24.8 ± 17 vs 10.3 ± 11.5; p < 0.05), DOL 2 (26.3 ± 22.9 vs 6.7 ± 12.1; p < 0.05) and DOL 3 (21.9 ± 33.8 vs 5.5 ± 9.3; p = 0.04). Operation was undertaken in 36/55 (65.5%). Preoperative mortality group had significant higher OI on DOL 2 (42.1 ± 21.0 vs 14.9 ± 9.3; p = 0.04). CDH defects were-Type A N = 27 (75%), Type B N = 7 (19.4%) and Type C N = 2 (5.6%). Overall mortality was 40% (22/55). Statistically significant OI trends were recorded in non-survival vs. survival groups on DOL 1 (31.6 ± 16.8. vs 10.5 ± 9.0; p < 0.05, DOL 2 (38.1 ± 21.9 vs 6.3 ± 7.1; p < 0.05), and DOL 3 (38.8 ± 39.4; p = 0.012).</p><p><strong>Conclusions: </strong>OI dynamics are highly predictive for accurate monitoring of CDH cardiorespiratory physiology and crucially may guide ventilatory management as well as timing of surgery.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"264"},"PeriodicalIF":1.5,"publicationDate":"2024-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11455676/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378090","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Appendicitis in children: correlation between the surgical and histological diagnosis.","authors":"Balazs Fadgyas, Georgina Monostori, Dorottya Ori, Peter Vajda","doi":"10.1007/s00383-024-05846-2","DOIUrl":"10.1007/s00383-024-05846-2","url":null,"abstract":"<p><strong>Purpose: </strong>Study was designed in order to evaluate the discrepancies between surgical and histological diagnosis in pediatric acute appendicitis (AA) and to compare the outcomes of laparoscopic (LA) and open appendectomies (OA).</p><p><strong>Methods: </strong>In a retrospective observational cohort, AA patients were included under 18 years of age, operated between 2011 and 2020. Surgical diagnosis was defined by the operating surgeon. The histological findings were classified as uncomplicated and complicated AA. The LOS and complications were also statistically analyzed.</p><p><strong>Results: </strong>Altogether, 1444 patients were included. Significant strong correlation and a moderate to substantial agreement were found between the surgeon's and the histopathological findings in all appendectomy cases (weighted kappa value in OA: 0.633, LA: 0.639, total sample: 0.637). If the surgeon's diagnosis was less severe than the pathologist's, the LOS was 4 (3;7) days, whereas if the surgical diagnosis was correct, the LOS was 3 (3;5) days (p < 0.0001).</p><p><strong>Conclusions: </strong>In contrary to the literature, our study revealed a strong correlation and moderate agreement between the intraoperative and histopathological findings regarding the severity of AA. Complicated cases are distinctly recognizable during the surgery. In case the surgeon underestimates the severity of AA, the chance of complications is higher.</p><p><strong>Level of evidence: </strong>II.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"262"},"PeriodicalIF":1.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11452465/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142375736","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia.","authors":"Yudai Tsuruno, Koshiro Sugita, Toshihiro Muraji, Ryuta Masuya, Toshio Harumatsu, Keisuke Yano, Shun Onishi, Takafumi Kawano, Chihiro Ichikawa, Haruo Ohtani, Yuko Bitoh, Satoshi Ieiri","doi":"10.1007/s00383-024-05845-3","DOIUrl":"10.1007/s00383-024-05845-3","url":null,"abstract":"<p><strong>Purpose: </strong>We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA).</p><p><strong>Methods: </strong>LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups.</p><p><strong>Results: </strong>HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018).</p><p><strong>Conclusions: </strong>Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"261"},"PeriodicalIF":1.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11452420/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142372480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of cryoablation in Nuss bar placement on opioid utilization and length of stay.","authors":"Christopher Clinker, Jack Scaife, Davian Martinez, Anastasia M Kahan, R Scott Eldredge, Katie W Russell","doi":"10.1007/s00383-024-05838-2","DOIUrl":"https://doi.org/10.1007/s00383-024-05838-2","url":null,"abstract":"<p><strong>Background: </strong>Our institution recently transitioned from paravertebral nerve blocks (PVBs) to intercostal nerve cryoablation (INC) for pain control following minimally invasive repair of pectus excavatum (MIRPE). This study aimed to determine how INC affected the operative time, length of stay, complication rates, inpatient opioid use, and outpatient prescription of opioids at a single center.</p><p><strong>Methods: </strong>A retrospective review was performed at a single pediatric referral center of all patients who underwent MIRPE between 2018 and 2023. Patient demographics, operative details, and perioperative course were collected. The use of INC versus PVB was recorded. Univariate analyses were performed using Wilcoxon rank sum tests for continuous variables and chi-squared tests for categorical variables.</p><p><strong>Results: </strong>255 patients were included with a median age of 15 years, median BMI of 18.50 kg/m², and median Haller index of 4.40. INC was utilized in 41% (105/255), and 59% (150/255) received PVB. The two groups did not differ significantly in BMI, Haller index, or complications, though the INC patients were older by 1 year (15.0 vs. 16.0, p = 0.034). INC was associated with an increased operative time (INC: 92 min vs. PVB: 67 min, p < 0.001), decreased length of stay (3 vs. 4 days, p =  < 0.001), more than twofold decrease in inpatient opioids per day (INC: 16 MME vs. PVB: 41 MME, p < 0.001), and a fourfold decrease in the amount of opioids prescribed at discharge (INC: 90 MME vs. PVB: 390 MME, p < 0.001).</p><p><strong>Conclusion: </strong>INC after MIRPE significantly decreased both the inpatient opioid utilization and our outpatient prescribing practices while also decreasing our overall length of stay without increasing complications.</p><p><strong>Level of evidence: </strong>Level III.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"260"},"PeriodicalIF":1.5,"publicationDate":"2024-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142372479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital diaphragmatic hernia and cleft lip and palate: looking for a common genetic etiology.","authors":"Petra Nord, Ashley H Ebanks, Petra Peterson, Erik Iwarsson, Matthew T Harting, Carmen Mesas Burgos","doi":"10.1007/s00383-024-05843-5","DOIUrl":"10.1007/s00383-024-05843-5","url":null,"abstract":"<p><strong>Purpose: </strong>Congenital diaphragmatic hernia (CDH) and cleft lip and/or palate (CL/P) are inborn closure defects. Genetic factors in and outcomes for patients with both anomalies (CDH+CL/P) remain unclear. We aimed to investigate associated genetic aberrations, prevalence of, and outcomes for, CDH+CL/P.</p><p><strong>Methods: </strong>Data from Congenital Diaphragmatic Hernia Study Group (CDHSG) registry were collected. CL/P prevalence in CDH patients was determined. Genetic abnormalities and additional malformations in CDH+CL/P were explored. Patient characteristics and outcomes were compared between CDH+CL/P and isolated CDH (CDH-) using Fisher's Exact Test for categorical, and t-test or Mann-Whitney U-test for continuous, data. p < 0.05 was considered statistically significant.</p><p><strong>Results: </strong>Genetic anomalies in CDH+CL/P included trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome (4p16.3 deletion). CL/P prevalence in CDH was 0.7%. CDH+CL/P had lower survival rates than CDH-, a nearly fourfold risk of death within 7 days, were less supported with extracorporeal life support (ECLS), had higher non-repair rates, and survivors had longer length of hospital stay.</p><p><strong>Conclusion: </strong>Genetic anomalies, e.g. trisomy 13, 8p23.1 deletion, and Wolf-Hirschhorn syndrome, are seen in patients with the combination of CDH and orofacial clefts. CL/P in CDH patients is rare and associated with poorer outcomes compared to CDH-, influenced by goals of care decision-making.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"259"},"PeriodicalIF":1.5,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11445286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}