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1.
Metabolomics ; 20(5): 109, 2024 Oct 05.
Artículo en Inglés | MEDLINE | ID: mdl-39369162

RESUMEN

INTRODUCTION: Biliary atresia (BA) is a rare progressive neonatal cholangiopathy with unknown pathophysiology and time of onset. Newborn Screening (NBS) in Germany is routinely performed in the first days of life to identify rare congenital diseases utilizing dried blood spot (DBS) card analyses. Infants with biliary atresia (BA) are known to have altered amino acid profiles (AAP) at the time point of diagnosis, but it is unclear whether these alterations are present at the time point of NBS. OBJECTIVES: We aimed to analyze amino acid profiles in NBS-DBS of infants with Biliary Atresia. METHODS: Original NBS-DBS cards of 41 infants who were later on diagnosed with BA were retrospectively obtained. NBS-DBS cards from healthy newborns (n = 40) served as controls. In some BA infants (n = 14) a second DBS card was obtained at time of Kasai surgery. AAP in DBS cards were analyzed by targeted metabolomics. RESULTS: DBS metabolomics in the NBS of at that time point seemingly healthy infants later diagnosed with BA revealed significantly higher levels of Methionine (14.6 ± 8.6 µmol/l), Histidine (23.5 ± 50.3 µmol/l), Threonine (123.9 ± 72.8 µmol/l) and Arginine (14.1 ± 11.8 µmol/l) compared to healthy controls (Met: 8.1 ± 2.6 µmol/l, His: 18.6 ± 10.1 µmol/l, Thr: 98.1 ± 34.3 µmol/l, Arg: 9.3 ± 6.6 µmol/l). Methionine, Arginine and Histidine showed a further increase at time point of Kasai procedure. No correlation between amino acid levels and clinical course was observed. CONCLUSION: Our data demonstrate that BA patients exhibit an altered AAP within 72 h after birth, long before the infants become symptomatic. This supports the theory of a prenatal onset of the disease and, thus, the possibility of developing a sensitive and specific NBS. Methionine might be particularly relevant due to its involvement in glutathione metabolism. Further investigation of AAP in BA may help in understanding the underlying pathophysiology.


Asunto(s)
Aminoácidos , Atresia Biliar , Pruebas con Sangre Seca , Tamizaje Neonatal , Humanos , Atresia Biliar/diagnóstico , Atresia Biliar/sangre , Atresia Biliar/metabolismo , Recién Nacido , Tamizaje Neonatal/métodos , Aminoácidos/sangre , Aminoácidos/metabolismo , Masculino , Femenino , Pruebas con Sangre Seca/métodos , Estudios Retrospectivos , Metabolómica/métodos , Lactante
2.
Pediatr Res ; 2024 Sep 28.
Artículo en Inglés | MEDLINE | ID: mdl-39341941

RESUMEN

BACKGROUND: Biliary atresia (BA) is a rare condition of unknown origin in newborns with jaundice. In BA bile ducts are non-functional, causing neonatal cholestasis and following liver fibrosis and failure. METHODS: This retrospective study included liver biopsies of 14 infants with BA aged [mean ± SD] 63 ± 23 days. Patients were grouped according to the clinical course (jaundice-free vs recurrent jaundice vs required liver transplantation or liver fibrosis (Ishak fibrosis score)) and followed for 1.61-5.64 years (mean 4.03). Transcriptome profiles were assessed using a panel of 768 fibrosis-specific genes, reanalyzed via qRT-PCR, and confirmed via immunostaining. Plasma from an additional 30 BA infants and 10 age-matched controls were used for amyloid precursor protein (APP) quantification by ELISA. RESULTS: Different clinical outcome groups showed a homogeneous mRNA expression. Altered amyloid-metabolism-related gene expression was found between cases with Ishak fibrosis score greater than 4. Immunostaining confirmed a distinct presence of APP in the livers of all BA subjects. APP plasma levels were higher in BA than in age-matched controls and correlated with the histological fibrosis grade. CONCLUSIONS: These results suggest that amyloidosis may contribute to BA and liver fibrosis, indicating that APP could serve as a potential liquid biomarker for these conditions. IMPACT: Biliary atresia patients with higher fibrosis scores according to Ishak have higher hepatic expression of amyloid-related genes while amyloid precursor protein accumulates in the liver and increases in the circulation. After a recent study revealed beta-amyloid deposition as a mechanism potentially involved in biliary atresia, we were able to correlate amyloid-metabolism-related transcript levels as well as amyloid precursor protein tissue and plasma levels with the degree of hepatic fibrosis. These findings suggest that amyloid precursor protein is a fibrosis marker in infants with biliary atresia, reinforcing the role of amyloid metabolism in the pathogenesis of this serious disease.

3.
JPGN Rep ; 5(3): 309-316, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39149194

RESUMEN

Objectives: In 2022, the Biliary Atresia and Related Diseases (BARD) community reached a consensus for the definition of suspected and confirmed cholangitis for biliary atresia (BA) patients after hepatoportoenterostomy (HPE). This study assessed the new standardized BARD definition in a retrospective, multicenter cohort study. Methods: We included BA cases managed between 2010 and 2020 at the Hannover Medical School and Geneva University Hospitals' Swiss Pediatric Liver Center. The standardized BARD cholangitis definition assesses four clinical items and four imaging/laboratory items to define cholangitis. The definition was retrospectively applied to all BA cases having presented, according to their physician, cholangitis within the first year after the HPE. The diagnosis defined by the standardized BARD definition was compared with the final clinical diagnosis made by physicians. The Spearman's correlation coefficient was used to test for correlation between diagnoses made by standardized and clinical appreciation. Results: Of 185 consecutive BA patients, 59 (32%) had at least one episode of cholangitis within the first year after HPE. The correlation between the clinician's impression and the standardized BARD definition was very strong (r = 0.8). Confirmed cholangitis definition coincided with the clinician's impression (2.5 [±0.7]/4 clinical items, 2.6 [±0.5]/4 imaging/laboratory items). For suspected cholangitis, the threshold for diagnosis was lower within the standardized BARD definition (1.1 [±0.3]/4 clinical items, 2.2 [±0.8]/4 laboratory/imaging items). Conclusions: This first retrospective application of the standardized BARD cholangitis definition reveals a very strong correlation with the physician's assessment before standardization. A prospective study is needed to further refine the standardized definition for cholangitis in BA patients.

4.
Innov Surg Sci ; 9(2): 93-98, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-39100717

RESUMEN

Objectives: Choledochal malformation (CM) is a rare disease that can lead to malignancy and potential long-term sequelae despite surgical resection. There is no long-term follow-up data on patients after CM resection in Germany. We aimed to determine the long-term outcome of our patients with a duration of follow-up >10 years and focused on long-term sequelae and health-related quality of life (HRQOL). Methods: All patients who had undergone CM-resection in our department from 01/1978 to 06/2009 were contacted. Patients were interviewed about postoperative complications and their present medical attendance. HRQOL was determined using Pediatric Quality of Life Inventory 4.0 (PedsQL), version for adults. The PedsQL scales the HRQOL from 0 to 100, with higher scores indicating a better HRQOL. Scores were compared to those published for a healthy population. Results: Out of 56 patients who were contacted, 23 (41 %) participated. The median age at time of surgery was 3.1 years (6 days-16.1 years) and at time of the survey 24.3 years (11.1-53.8 years). Eighteen patients (78 %) had ceased their gastroenterologic follow-up at a median time of 4.3 years after surgery. Five (22 %) were still in gastroenterologic follow-up, two of these had an uneventful clinical course, and three (13 %) had ongoing complications attributed to the CM. One of these had undergone hemihepatectomy 34 years postoperatively due to bile duct stenosis, one had undergone removal of bile duct stones 14 years postoperatively, and one suffered from portal vein thrombosis with esophageal and jejunal varices. There was no mortality in our series. Median total HRQOL score was 89. There was no significant difference in the median total health, physical health, and psychosocial health scores of our patients in comparison to the healthy population. Conclusions: We confirmed that the majority of patients after CM resection are lost to follow-up. Those who answered our questionnaire showed a good HRQOL. Given the high rate of severe long-term complications and the life-long risk of malignancy, we recommend a transition program for all patients.

5.
Children (Basel) ; 9(10)2022 Oct 20.
Artículo en Inglés | MEDLINE | ID: mdl-36291526

RESUMEN

Introduction The Kasai procedure in children with biliary atresia (BA) is associated with several complications in the short-term. The Comprehensive Complication Index (CCI®) is a validated metric in adult surgery for the analysis of complications and morbidity in surgical patients. We aimed to analyze the CCI® for the first time in BA infants and to correlate its association with outcomes. Material and Methods We conducted a retrospective review of medical records of infants with type III BA undergoing the Kasai procedure between January 2011 and December 2021 at our institution. All unexpected events were ranked according to the Clavien−Dindo classification, and the CCI® per patient was subsequently calculated. Clavien−Dindo grades, individual events, CCI®, and total event numbers per patient were correlated with one- and two-year outcomes post-surgery. Results A total of 131 events were identified in 101 patients (ranging 0−11 per patient). Forty-four Grade I (33.6%), 67 Grade II (51.1%), 18 Grade III (13.7%), and two sentinel events [>Grade IV] (1.5%) were documented according to Clavien−Dindo, including one death in a cardiac-associated BA patient. None of the complications significantly correlated with a poor outcome. Sixty-three (62.4%) CCI® scores were calculated (range 0−100). The mean CCI® score during the in-patient treatment post-surgery was significantly higher in patients with a poorer outcome than patients with native liver survival at one- and two-year follow-up (22.7 ± 21.7 vs. 13.2 ± 18.1; p = 0.02). Conclusion Not the severity of complications, but the accumulation of numerous events related to Kasai procedure were associated with a poorer outcome. Therefore, the CCI® is an excellent instrument for the postoperative morbidity assessment of BA patients.

6.
Eur J Pediatr Surg ; 32(6): 521-528, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35764302

RESUMEN

BACKGROUND: Numerous studies from Asian countries, including large collectives, have reported excellent results after laparoscopic resection of choledochal malformation (CM). However, the role of laparoscopic CM resection is still controversial outside Asia. We aimed to analyze the outcome of laparoscopic CM resection in our institution and to compare our outcome with the data reported in the literature. METHODS: All patients who underwent laparoscopic CM resection in our pediatric surgical department from 2002 to 2019 were retrospectively analyzed for surgical details and postoperative complications, which were graded according to the Clavien-Dindo classification. A systematic literature search identified all reports on over 10 cases of laparoscopic pediatric CM resection and surgical details, follow-up, and complication rates were extracted. RESULTS: Fifty-seven patients (72% female) with a mean age of 3.6 + 4.1 years underwent laparoscopic CM resection in our department. Conversion rate was 30%. Total complication rate was 28%. The rate of major complications (Clavien-Dindo grade III or more) was 16% and included stricture of the biliodigestive or enteric anastomosis (n = 4), adhesive ileus (n = 3), portal vein thrombosis (n = 1), and recurrent cholangitis with consecutive liver transplantation (n = 1). With increasing experience, complication rates decreased. The majority of publications on laparoscopic CM resections originated from Asia (n = 36) and reported on low complication rates. In contrast, publications originating from non-Asian countries (n = 5) reported on higher complications following laparoscopic CM resection. CONCLUSION: Our data indicate that laparoscopic CM resection can be safely performed. The learning curve in combination with the low incidence calls for a centralization of patients who undergo laparoscopic CM resection. There seems to be a discrepancy on complications rates reported from Asian and non-Asian countries following laparoscopic CM resection.


Asunto(s)
Laparoscopía , Humanos , Femenino , Niño , Preescolar , Masculino , Estudios Retrospectivos , Laparoscopía/efectos adversos , Laparoscopía/métodos , Anastomosis Quirúrgica , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Constricción Patológica , Resultado del Tratamiento
7.
Eur J Pediatr Surg ; 32(1): 80-84, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34918313

RESUMEN

INTRODUCTION: Age at Kasai portoenterostomy (KPE) has been identified as a predictive factor for native-liver survival in patients with biliary atresia (BA). Outcomes of pediatric liver transplantation (LT) have improved over recent years. It has been proposed to consider primary LT as a treatment option for late-presenting BA infants instead of attempting KPE. We present our experience with patients older than 90 days undergoing KPE. MATERIALS AND METHODS: A retrospective chart review of patients with BA undergoing KPE at our institution between January 2010 and December 2020 was performed. Patients 90 days and older at the time of surgery were included. Patients' characteristics, perioperative data, and follow-up results were collected. Eleven patients matched the inclusion criteria. Mean age at KPE was 108 days (range: 90-133 days). RESULTS: Postoperative jaundice clearance (bilirubin < 2 mg/dL) at 2-year follow-up was achieved in three patients (27%). Eight patients (73%) received a liver transplant at a mean of 626 days (range: 57-2,109 days) after KPE. Four patients (36%) were transplanted within 12 months post-KPE. Two patients died 237 and 139 days after KPE due to disease-related complications. One patient is still alive with his native liver, currently 10 years old. CONCLUSION: Even when performed at an advanced age, KPE can help prolong native-liver survival in BA patients and offers an important bridge to transplant. In our opinion, it continues to represent a viable primary treatment option for late-presenting infants with BA.


Asunto(s)
Atresia Biliar , Trasplante de Hígado , Atresia Biliar/complicaciones , Atresia Biliar/cirugía , Niño , Humanos , Lactante , Hígado/cirugía , Portoenterostomía Hepática , Estudios Retrospectivos , Resultado del Tratamiento
8.
Int J Neonatal Screen ; 7(4)2021 Nov 04.
Artículo en Inglés | MEDLINE | ID: mdl-34842600

RESUMEN

BACKGROUND: Stool color card (SCC) screenings for biliary atresia (BA) have shown to improve Kasai timing and outcome significantly. Both obligatory and non-obligatory screenings with passive distribution strategies have proven to be effective. Therefore, we have initiated a voluntary SCC program and aim to describe our experience. METHODS: Since 2017 we supply all maternity wards in Lower-Saxony with SCC. Attending pediatricians and parents of BA infants were contacted via questionnaires and asked for their evaluation of the SCC screening. RESULTS: 85.2% of attending pediatricians support the SCC screening, but only 78.1% considered the initiative useful. In their clinical routine, only 67% of visiting parents report to have received an SCC at the maternity hospital. In the group of parents of BA infants, only 54% (7/13) had received an SCC. Out of those seven parents, only one had referred their child to a children's hospital based on pathological SCC results. The lack of SCC education in the maternity hospitals was made responsible by parents. Within three years, only one infant with BA was identified through the SCC. CONCLUSIONS: Our voluntary SCC screening shows serious limitations with inacceptable distribution of SCCs and low acceptance of attending pediatricians. SCC programs in decentralized health care systems without educational campaigns, standardized diagnostic and treatment algorithms and the definition of reference centers are additional burdens for local health care providers without the promised benefit.

9.
Innov Surg Sci ; 6(4): 151-160, 2021 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-35937850

RESUMEN

Survival rates of patients with visceral congenital malformations have increased considerably. However, long-term morbidity in these patients is high. In the last decades, these circumstances have led to a shift in goals of caretakers and researchers with a new focus on patients' perspectives and long-term morbidity. Health-related quality of life (HrQoL) is the most commonly used patient-reported outcome measure to assess the impact of chronic symptoms on patients' everyday lives. Most pediatric surgical conditions can cause a significantly decreased HrQoL in affected patients compared to the healthy population. In order to guarantee life-long care and to minimize the impact on HrQoL a regular interdisciplinary follow-up is obligatory. The period of transition from child-centered to adult-oriented medicine represents a critical phase in the long-term care of these complex patients. This scoping review aims to summarize relevant pediatric surgical conditions focusing on long-term-morbidity and HrQoL assessment in order to demonstrate the necessity for a well-structured and standardized transition for pediatric surgical patients.

10.
Eur J Pediatr Surg ; 31(1): 80-85, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32820494

RESUMEN

INTRODUCTION: Evidence supports long-term oral antibiotic prophylaxis to prevent cholangitis after Kasai procedure. Data regarding perioperative intravenous prophylaxis are lacking. Ascending pathogens from the intestine are made responsible for recurrent cholangitis. Therefore, we analyzed the flora in the upper jejunum during the Kasai procedure and their potential impact on postoperative cholangitis. MATERIALS AND METHODS: In 26 patients, swabs were taken at the bowel prepared for the Roux-en-Y-loop. Our postoperative protocol includes intravenous third-generation cephalosporins for 2 weeks and rectal steroids starting at day 4. Cholangitis was defined as the postoperative reappearance of acholic stools or increase of serum bilirubin in combination with fevers or increase of inflammatory parameters. In this scenario, Tazocin was administered for another 2 weeks. RESULTS: Swabs remained sterile in nine patients (34.6%). In 17 patients (65.4%), gram-positive and gram-negative pathogens were identified; all belonging to physiological intestinal flora. A total of 96.2% pathogens were covered by the antibiotic prophylaxis. The cholangitis incidence was 55.6% in the sterile cohort, and 23.5% in the gram-positive and gram-negative cohort (p = 0.06). In the cholangitis cohort, no significant differences were detected for the age at Kasai and the pre- and postoperative total bilirubin. CONCLUSION: We found that our antibiotic regiment covered bacteria in the upper gastrointestinal (GI) tract in the majority of our patients at the time of Kasai. Nonetheless, a significant proportion of patients developed signs of cholangitis. There was no higher rate of cholangitis in patients with resistant bacteria. Thus, our data do not support the hypothesis of extended postoperative intravenous antibiotics to prevent ascending cholangitis.


Asunto(s)
Antibacterianos/administración & dosificación , Profilaxis Antibiótica/métodos , Atresia Biliar/cirugía , Cefalosporinas/administración & dosificación , Colangitis/microbiología , Microbioma Gastrointestinal , Administración Intravenosa , Anastomosis en-Y de Roux , Colangitis/diagnóstico , Colangitis/prevención & control , Femenino , Humanos , Lactante , Yeyuno/cirugía , Masculino , Estudios Retrospectivos
11.
Eur J Pediatr Surg ; 31(1): 20-24, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32820496

RESUMEN

INTRODUCTION: In patients with choledochal cysts (CDC), a hyperplasia-dysplasia-carcinoma sequence can lead to biliary tract malignancy. The limited data available suggest that the risk decreases considerably after excision in childhood. We analyzed samples of resected CDC from pediatric patients histologically and performed mutational analysis of the proto-oncogenes KRAS and BRAF as early markers of malignant alteration in cholangiocytes. MATERIALS AND METHODS: After institutional review board approval, patients undergoing resection for CDC in our center from 2011 to 2019 were retrospectively identified. Histopathological reports were searched for inflammation and endothelial alteration. Cases with sufficient tissue specimen were tested for KRAS codon 12/13 and BRAF codon 600 mutations by pyrosequencing. RESULTS: In total, 42 patients underwent resection for choledochal cyst in the study period. Median age at surgery was 2.4 years (range = 18 days-18 years). Histopathological analysis showed no malignancy, but various degrees of inflammation or fibrosis in approximately 50% of the patients and in all age groups. Sufficient tissue for mutation analysis was available for 22 cases, all of which tested negative for KRAS or BRAF mutation. CONCLUSION: In our series, chronic inflammatory changes were frequently present in CDC of infants and children. However, the lack of KRAS and BRAF mutations suggests that no malignant changes have been initiated in this group of European patients undergoing early resection.


Asunto(s)
Quiste del Colédoco/cirugía , Adolescente , Biomarcadores de Tumor/análisis , Niño , Preescolar , Quiste del Colédoco/genética , Quiste del Colédoco/patología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Proteínas Proto-Oncogénicas B-raf , Proteínas Proto-Oncogénicas p21(ras) , Estudios Retrospectivos
12.
Eur J Pediatr Surg ; 31(1): 34-39, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32820497

RESUMEN

INTRODUCTION: According to the Declaration of Helsinki, medical research and new therapeutic interventions involving human subjects require prior informed consent and ethical approval. In 2010, 46% of pediatric surgical publications lacked documentation of ethical approval and 84% lacked documentation of informed parental consent with lowest rates of ethical adherence found in articles concerning novel methods. The aim of this study was to investigate whether adherence to ethical standards has improved in pediatric surgical publications. MATERIALS AND METHODS: All 3,093 consecutive articles published in Journal of Pediatric Surgery, European Journal of Pediatric Surgery, and Pediatric Surgery International over the last 5 years were systematically reviewed for publications describing novel surgical methods. Novel methods were defined as surgical methods not published before or not considered common practice. The publications were reviewed as to whether ethical approval and informed consent to participate was documented. RESULTS: In total, 105 articles describing novel surgical methods were identified (61 Journal of Pediatric Surgery, 16 European Journal of Pediatric Surgery, and 28 Pediatric Surgery International). Authors reported on new operative techniques (62%), modified techniques (31%), or use of new materials (7%). Ethical approval was documented in 52% of the articles with almost half reporting approval for retrospective data analysis only but not the application of the novel method. Informed consent was documented in 21% of publications. Complications were reported in 48% of the studies, including recurrences and reinterventions for the unsuccessful novel methods. Two authors reported mortalities due to underlying disease, one of which failed to report prior ethical approval or informed consent. CONCLUSION: Adherence to ethical publication principles in pediatric surgery has improved over the last years but is still lacking in many publications. When implementing new methods, prior ethical approval and informed consent and their documentation are mandatory, specifically in the light of potential hazard to patients.


Asunto(s)
Ética en Investigación , Publicaciones Periódicas como Asunto/estadística & datos numéricos , Humanos , Consentimiento Informado/ética , Pediatría/normas , Estudios Retrospectivos , Especialidades Quirúrgicas/normas
13.
Eur J Pediatr Surg ; 30(2): 172-180, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-32146718

RESUMEN

The use of minimally invasive surgery (MIS) in pediatric patients has increased over the past decades. The process of mastering a new procedure is termed the learning curve, during which the ability to operate increases but poorer outcomes are produced. We aim to analyze the current evidence on learning curves in pediatric MIS and evaluate its impact on patient's clinical outcomes. A systematic literature search was performed for studies listed on PubMed that reported on the learning curve for MIS surgical procedures. Studies were included if they stated the number of procedures required to reach a consistency in outcomes or if they compared outcomes between early and late period of MIS experience regarding the endpoints operative time, conversions, and intra-/postoperative complications. A total of 22 articles reporting on 11 surgical procedures were included in the study. Most authors reported a significant decrease in operative time as well as peri- and postoperative complications with increasing experience of the surgeon. Complications ranged from minor to major, the latter being especially severe for patients receiving pyloromyotomy (5-7% higher risk of mucosal perforation), esophageal atresia repair (15% higher leakage rate and 19-77% higher stenosis rate), or Kasai portoenterostomy (26-35% more liver transplants in the first year after surgery) during the learning curve period. Pediatric MIS comes with a considerable learning curve that may have a significant impact on the patient's clinical outcomes. Efforts should be made to minimize the effect of the learning curve on the patients.


Asunto(s)
Curva de Aprendizaje , Procedimientos Quirúrgicos Mínimamente Invasivos/educación , Niño , Humanos , Procedimientos Quirúrgicos Mínimamente Invasivos/normas , Tempo Operativo , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Cirujanos/educación
14.
Eur J Pediatr Surg ; 30(1): 45-50, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31344711

RESUMEN

INTRODUCTION: Management strategies for large omphaloceles remain controversial. In this study, we discuss the use of GRAVITAS (gravitational autoreposition sutures), the method used at our institution when successful primary closure is deemed questionable. Patient's primary clinical course and long-term outcomes were analyzed. MATERIALS AND METHODS: This is a single-center retrospective analysis of all consecutive patients with omphaloceles treated between 1997 and 2018. Decision for GRAVITAS was made when the defect was estimated too large for primary closure. Traction sutures were placed in the fascia surrounding the defect and then suspended from the top of the incubator to allow gravitational autoreposition of the herniated organs. Ventilation and muscle relaxation were maintained until secondary closure, which was performed after the obtruding viscera had been reduced by repeated adjustment of the suture's tension. Data are presented as mean ± standard deviation. RESULTS: Out of 49 patients with omphaloceles, 12 were treated with GRAVITAS, 33 underwent primary closure, and 4 were treated using Schuster's technique. Mean time to secondary closure after GRAVITAS was 7 ± 10 days. In nine of the patients who had isolated omphalocele, secondary closure was achieved after 4 ± 2 days. Ventilation time was 5 ± 2 days, and time to full feeds was 18 ± 16 days. In three patients (one with Fallot's tetralogy, one with Cantrell's pentalogy, and one with lung hypoplasia), abdominal closure was achieved after 17 ± 15 days. Due to cardiorespiratory comorbidity, ventilation time was >30 days. Five patients received initial closure of the skin and secondary fascial closure after 18 ± 15 months. One patient with prior fascial closure underwent later repair of an abdominal wall hernia. During follow-up (30 ± 35 months), one patient with gastrointestinal obstruction due to adhesions required laparotomy, and one patient with gastroesophageal reflux disease underwent fundoplication. CONCLUSION: GRAVITAS is a feasible method for staged closure of large omphaloceles when successful primary closure is deemed questionable.


Asunto(s)
Técnicas de Cierre de Herida Abdominal , Hernia Umbilical/cirugía , Técnicas de Sutura , Fasciotomía , Femenino , Estudios de Seguimiento , Gravitación , Humanos , Recién Nacido , Masculino , Complicaciones Posoperatorias , Reoperación , Estudios Retrospectivos
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