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1.
Pediatr Res ; 95(4): 901-911, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37978315

RESUMO

In the last few years, current evidence has supported the use of point-of-care ultrasound (POCUS) for a number of diagnostic and procedural applications. Considering the valuable information that POCUS can give, we propose a standardized protocol for the management of neonates with a congenital diaphragmatic hernia (CDH-POCUS protocol) in the neonatal intensive care unit. Indeed, POCUS could be a valid tool for the neonatologist through the evaluation of 1) cardiac function and pulmonary hypertension; 2) lung volumes, postoperative pleural effusion or pneumothorax; 3) splanchnic and renal perfusion, malrotations, and/or signs of necrotizing enterocolitis; 4) cerebral perfusion and eventual brain lesions that could contribute to neurodevelopmental impairment. In this article, we discuss the state-of-the-art in neonatal POCUS for which concerns congenital diaphragmatic hernia (CDH), and we provide suggestions to improve its use. IMPACT: This review shows how point-of-care ultrasound (POCUS) could be a valid tool for managing neonates with congenital diaphragmatic hernia (CDH) after birth. Our manuscript underscores the importance of standardized protocols in neonates with CDH. Beyond the well-known role of echocardiography, ultrasound of lungs, splanchnic organs, and brain can be useful. The use of POCUS should be encouraged to improve ventilation strategies, systemic perfusion, and enteral feeding, and to intercept any early signs related to future neurodevelopmental impairment.


Assuntos
Hérnias Diafragmáticas Congênitas , Recém-Nascido , Humanos , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/terapia , Sistemas Automatizados de Assistência Junto ao Leito , Pulmão/patologia , Ultrassonografia , Medidas de Volume Pulmonar
2.
Pediatr Surg Int ; 40(1): 176, 2024 Jul 05.
Artigo em Inglês | MEDLINE | ID: mdl-38967682

RESUMO

PURPOSE: Daily postoperative anal dilations after endorectal pull-through for Hirschsprung disease (HD) are still considered a common practice. We analyzed the potential risks of this procedure and its effectiveness compared to a new internal protocol. METHODS: All infants (< 6 months of age) who underwent transanal endorectal pull-through between January 2021 and January 2023 were prospectively enrolled in a new postoperative protocol group without daily anal dilations (Group A) and compared (1:2 fashion) to those previously treated by postoperative anal dilations (Group B). Patients were matched for age and affected colonic tract. Patients with associated syndromes, extended total intestinal aganglionosis, and presence of enterostomy were excluded. Outcomes considered were: anastomotic complications (stenosis, disruption/leakage), incidence of enterocolitis, and constipation. RESULTS: Eleven patients were included in group A and compared to 22 matched patients (group B). There were no significant differences in the occurrence of anastomotic complications between the two groups. We found a lower incidence of enterocolitis and constipation among group A (p = 0.03 and p = 0.02, respectively). CONCLUSION: A non-dilation strategy after endorectal pull-through could be a feasible alternative and does not significantly increase the risk of postoperative anastomotic complications. Moreover, some preliminary advantages such as lower enterocolitis rate and constipation should be further investigated.


Assuntos
Doença de Hirschsprung , Complicações Pós-Operatórias , Humanos , Doença de Hirschsprung/cirurgia , Lactente , Masculino , Feminino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Prospectivos , Recém-Nascido , Enterocolite/etiologia , Enterocolite/prevenção & controle , Enterocolite/epidemiologia , Cuidados Pós-Operatórios/métodos , Canal Anal/cirurgia , Procedimentos Cirúrgicos do Sistema Digestório/métodos
3.
Pediatr Surg Int ; 40(1): 124, 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38713441

RESUMO

PURPOSE: Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a well described clinical condition, but reports are focused on microcolon and intestinal hypoperistalsis, while data on bladder management are scant. Aim of the study is to present urological concerns in MMIHS. METHODS: Retrospective evaluation of clinical data on urological management of MMIHS patients treated in the last 10 years. RESULTS: Six patients were enrolled (3 male, 3 female). Three girls had prenatal diagnosis of megacystis (1 vesicoamniotic shunt was placed). All patients had genetic diagnosis: 5 had ACTG2 gene mutations and 1 MYH11 mutation. All patients were addressed to our attention for urinary symptoms, such as urinary retention, urinary tract infections, acute renal injury. Two patients presented frequent stoma prolapses. All children underwent a complete urological evaluation, and then started a bladder management protocol (clean intermittent catheterization, via urethra or cystostomy-tube placement), with improvement of urinary infections, upper urinary tract dilation and stoma prolapses, if present. All patients had good renal function at last follow-up. CONCLUSION: We believe that MMIHS patients must be addressed soon and before onset of symptoms for a multidisciplinary evaluation, including an early assessment by a pediatric urologist expert in functional disorder, to preserve renal function at its best.


Assuntos
Anormalidades Múltiplas , Colo , Colo/anormalidades , Pseudo-Obstrução Intestinal , Bexiga Urinária , Bexiga Urinária/anormalidades , Humanos , Feminino , Estudos Retrospectivos , Masculino , Anormalidades Múltiplas/cirurgia , Colo/cirurgia , Bexiga Urinária/cirurgia , Lactente , Pseudo-Obstrução Intestinal/cirurgia , Pseudo-Obstrução Intestinal/diagnóstico , Recém-Nascido , Pré-Escolar , Mutação
4.
Pediatr Surg Int ; 40(1): 53, 2024 Feb 10.
Artigo em Inglês | MEDLINE | ID: mdl-38340215

RESUMO

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.


Assuntos
Pneumopatias , Anormalidades do Sistema Respiratório , Humanos , Criança , Pneumopatias/congênito , Anormalidades do Sistema Respiratório/cirurgia , Pneumonectomia/métodos , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Pulmão/anormalidades , Itália , Estudos Retrospectivos
5.
Pediatr Surg Int ; 39(1): 226, 2023 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-37410181

RESUMO

Anorectal malformation (ARM) is often associated to other congenital malformations, requiring a tailored management. Hypospadias' treatment in ARM is poorly described. Aim of study is to describe our experience in ARM-hypospadias patients especially in relation to occult spinal dysraphism (OSD). ARM patients treated from 1999 to 2022 were retrospectively reviewed, selecting male with hypospadias. Clinical data, hypospadias's severity, ARM sub-type (Group A: perineal fistula; Group B: urethral fistula, bladder fistula, no fistula), OSD, other associated malformations, NLUTD were evaluated. Exclusion criteria: uncomplete data. Among 395 ARMs, 222 were males, 22 (10%) had hypospadias. Two patients were excluded. Group A: 8 patients, Group-B: 12. Hypospadias: proximal 9 patients, distal 11. Neuro-urological evaluation was performed before hypospadias repair. Eleven patients (55%) had OSD. Four OSD patients presented NLUTD and underwent detethering and CIC (two via cystostomy button, two via appendicostomy); two of them had hypospadias repaired. All proximal hypospadias underwent two stages of surgery. Distal hypospadias was corrected in 4/11 cases. Hypospadias is quite common in ARM patients and its surgical management must be scheduled considering the possible OSD and NLUTD, with the possible need for intermittent catheterization. Complexity of ARM and hypospadias appears to be related to each other.


Assuntos
Malformações Anorretais , Hipospadia , Defeitos do Tubo Neural , Fístula Urinária , Humanos , Criança , Masculino , Feminino , Hipospadia/complicações , Hipospadia/cirurgia , Malformações Anorretais/complicações , Malformações Anorretais/cirurgia , Estudos Retrospectivos , Uretra/cirurgia , Defeitos do Tubo Neural/cirurgia
6.
Pediatr Cardiol ; 43(6): 1383-1391, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35260923

RESUMO

Herein we report the case of a neonate with a prenatally diagnosed large pulmonary arteriovenous malformation, managed with minimally invasive hemodynamic monitoring in our Neonatal Intensive Care Unit. The combination of Near-Infrared Spectroscopy and Pressure Recording Analytical Method could guide neonatal management of critical cases of vascular anomalies: immediate data are offered to clinicians, from which therapeutic decisions such as timing of surgical resection are made to achieve a positive outcome. We also systemically collected and summarized information on patients' characteristics of previous cases reported in literature to data, and we compared them to our case.


Assuntos
Fístula Arteriovenosa , Malformações Arteriovenosas , Veias Pulmonares , Fístula Arteriovenosa/diagnóstico por imagem , Fístula Arteriovenosa/cirurgia , Malformações Arteriovenosas/diagnóstico por imagem , Malformações Arteriovenosas/cirurgia , Hemodinâmica , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia
7.
J Pediatr ; 228: 155-163.e1, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-32918920

RESUMO

OBJECTIVE: To analyze the findings of both multichannel intraluminal impedance with pH (MII-pH) and endoscopy/histopathology in children with esophageal atresia at age 1 year, according to current recommendations for the evaluation of gastroesophageal reflux disease (GERD) in esophageal atresia. STUDY DESIGN: We retrospectively reviewed both MII-pH and endoscopy/histopathology performed in 1-year-old children with esophageal atresia who were followed up in accordance with international recommendations. Demographic data and clinical characteristics were also reviewed to investigate factors associated with abnormal GERD investigations. RESULTS: In our study cohort of 48 children with esophageal atresia, microscopic esophagitis was found in 33 (69%) and pathological esophageal acid exposure on MII-pH was detected in 12 (25%). Among baseline variables, only the presence of long-gap esophageal atresia was associated with abnormal MII-pH. Distal baseline impedance was significantly lower in patients with microscopic esophagitis, and it showed a very good diagnostic performance in predicting histological changes. CONCLUSIONS: Histological esophagitis is highly prevalent at 1 year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes. MII-pH may be a helpful tool in selecting patients who need closer endoscopic surveillance and/or benefit from acid suppression.


Assuntos
Atresia Esofágica/cirurgia , Esofagoplastia/efeitos adversos , Esôfago/fisiopatologia , Refluxo Gastroesofágico/diagnóstico , Complicações Pós-Operatórias , Adolescente , Criança , Impedância Elétrica , Endoscopia Gastrointestinal , Monitoramento do pH Esofágico/métodos , Esôfago/metabolismo , Feminino , Seguimentos , Refluxo Gastroesofágico/etiologia , Refluxo Gastroesofágico/fisiopatologia , Humanos , Masculino , Manometria , Estudos Retrospectivos , Fatores de Tempo
8.
Dis Esophagus ; 33(1)2020 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-31907526

RESUMO

Although eating problems have been described as long-term morbidities of esophageal atresia (EA), there have been few studies exploring eating outcomes in children born with EA as primary aim. Parents of children operated on for EA in our Institution from January 2012 to January 2016, answered a telephone structured interview developed specifically to conduct the present study, assessing eating skills at 3 years of age. Clinical data were collected from children's medical records. Parents (45 mothers and 6 fathers) of 51 children (male = 34; female = 17) with a median age of 3.5 years form the object of the study. Considering eating problems, parents reported that 23 children (45%) still have episodes of choking during meals at 3 years of age, 9 (45%) of these have more than one episode a week, and 19 parents (39%) reported higher levels of anxiety during mealtimes. Forty-four children (86%) were described by their parents as able to eat alone, 32 (65%) accepted all food textures and 45 (90%) was described as curious about food (3 years). Forty-three (86%) parents let their children eat with other people. Correlations showed that weaning age was significantly associated with number of dilatations (rs = 0.35, P = 0.012), days of mechanical ventilation (rs = 0.40, P < 0.001), and presence of gastrostomy tube at discharge (rs = 0.45, P < 0.001). Chewing age resulted associated with number of dilatations (rs = 0.34, P < 0.01) and days of mechanical ventilation (rs = 0.38, P < 0.01). Presence of choking episodes was associated with curiosity about food (rs = 0.29, P < 0.05), while frequent choking episodes were associated with higher parental anxiety during mealtimes (rs = 0.45, P < 0.05). In order to prevent delay in the achievement of eating developmental milestones in children operated on of EA, we advocate a dedicated preventive intervention from birth to follow-up.


Assuntos
Obstrução das Vias Respiratórias/epidemiologia , Atresia Esofágica/fisiopatologia , Transtornos da Alimentação e da Ingestão de Alimentos/epidemiologia , Obstrução das Vias Respiratórias/etiologia , Pré-Escolar , Atresia Esofágica/complicações , Atresia Esofágica/terapia , Comportamento Alimentar/fisiologia , Transtornos da Alimentação e da Ingestão de Alimentos/etiologia , Feminino , Humanos , Masculino , Mastigação/fisiologia , Pais , Inquéritos e Questionários
9.
Am J Med Genet A ; 170(3): 707-11, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26639892

RESUMO

Gershoni-Baruch syndrome is an extremely rare malformation complex characterized by omphalocele, diaphragmatic hernia, radial ray defects, and cardiovascular abnormalities. Autosomal recessive inheritance was suggested. To date, academic literature described only seven patients fulfilling the diagnostic criteria for the condition. None survived the neonatal period. This study reports the first individual with Gershoni-Baruch syndrome surviving past early infancy.


Assuntos
Oftalmopatias Hereditárias/diagnóstico , Hérnia Umbilical/diagnóstico , Hérnias Diafragmáticas Congênitas/diagnóstico , Anormalidades Múltiplas , Humanos , Lactente , Masculino , Fenótipo , Radiografia Abdominal , Ultrassonografia Pré-Natal
10.
Fetal Pediatr Pathol ; 35(1): 37-42, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26720091

RESUMO

Bladder agenesis is a rare condition, mostly affecting females, where diagnosis is usually made in infancy when investigating urinary incontinence. Neonatal cases are uncommon, and none have been reported antenatally. The few male patients with this condition rarely survive: among associated anomalies, different degrees of penoscrotal transposition are the most evident feature. The association of genital transposition in a female infant with prenatal description of bladder agenesis has not been previously reported. Early diagnosis is important, enabling planning of surgical reconstruction early in life and appropriate parental counseling.


Assuntos
Doenças dos Genitais Femininos/patologia , Doenças da Bexiga Urinária/patologia , Adulto , Feminino , Doenças dos Genitais Femininos/diagnóstico , Doenças dos Genitais Femininos/cirurgia , Humanos , Lactente , Gravidez , Diagnóstico Pré-Natal , Resultado do Tratamento , Doenças da Bexiga Urinária/diagnóstico , Doenças da Bexiga Urinária/cirurgia
11.
J Pediatr Gastroenterol Nutr ; 58(4): 438-42, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24231643

RESUMO

OBJECTIVE: The patients with ultra-short bowel syndrome (U-SBS) have been considered potential candidates for a preemptive/rehabilitative intestinal transplantation owing to the high risk of death from the underlying disease. We hypothesized that children with U-SBS, in the absence of intestinal failure-associated liver disease (IFALD), could also have a good rate of survival on home parenteral nutrition (HPN). METHODS: A prospective database from the "Bambino Gesù" Artificial Nutrition and Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with ≤ 10 cm of small bowel enrolled since 2000. RESULTS: Eleven patients were identified with a median bowel length of 7.5 (3-9) cm. Eight patients developed IFALD, which reversed in 7 of them; the IFALD progressively worsened in 1 patient until death. One patient underwent isolated intestinal transplantation and 1 patient is no longer receiving parenteral nutrition (PN) and both are fully enterally fed. The other patients remained at least partially dependent on HPN. The number of days of inpatient care decreased in all of the patients except for the 1 who had repeated episodes of central line infections. CONCLUSIONS: The survival of patients with U-SBS receiving HPN was good. Although IFALD was frequent, it had been manageable in most of the patients, but in a single complex case, it led to death. The multidisciplinary management warranted to these patients to approach the school age, to grow, and to maintain the oral intake. Patients with U-SBS are rare, and to better understand their long-term survival, further studies, including more large patient populations, are required.


Assuntos
Hepatopatias/etiologia , Nutrição Parenteral no Domicílio , Síndrome do Intestino Curto/complicações , Síndrome do Intestino Curto/terapia , Criança , Desenvolvimento Infantil , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Intestino Delgado/fisiopatologia , Masculino , Estudos Retrospectivos , Síndrome do Intestino Curto/fisiopatologia , Taxa de Sobrevida , Fatores de Tempo
12.
Eur J Pediatr Surg ; 34(1): 63-68, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37459887

RESUMO

INTRODUCTION: The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers. MATERIALS AND METHODS: Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers). Clinical, radiological, and surgical data, as well as FU data were revised. A multidisciplinary team followed the patients after discharge at scheduled time points. RESULTS: Seventy-seven patients were included. After surgery, patients were followed for a median of 8 years (range: 1-19 years) until they reached a median age of 8 years (range: 2-19 years). Thirty patients (39%) developed wheezing and 21 (27%) had lower respiratory tract infections (LRTIs) within 4 years of age. However, more than 50% of patients with respiratory symptoms underwent complete remission in the following 4 years. Thirty-one patients (40%) developed at least one minimal musculoskeletal deformity. Eighteen (23%) had scoliosis, 17 (22%) thoracic asymmetry, 10 (12%) pectus excavatum, and 5 (6%) winged scapula. CONCLUSIONS: Patients operated for CCAML had good overall outcomes despite pulmonary symptoms and musculoskeletal sequelae. Even though these issues are frequently paucisymptomatic, trying to use less-invasive procedures (such as minimally axillary open "muscle-sparing" thoracotomy or thoracoscopy) may reduce this burden. A structured multidisciplinary FU is required.


Assuntos
Malformação Adenomatoide Cística Congênita do Pulmão , Tórax em Funil , Lactente , Humanos , Recém-Nascido , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Malformação Adenomatoide Cística Congênita do Pulmão/cirurgia , Estudos Retrospectivos , Pulmão/anormalidades , Pneumonectomia , Tórax em Funil/cirurgia
13.
Horm Res Paediatr ; 2024 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-38574486

RESUMO

INTRODUCTION: GATA6 is a gene that encodes a transcription factor with a key role in the development of several organ systems, including the development of the pancreas. It is associated with neonatal diabetes but also with other extra-pancreatic anomalies. CASE PRESENTATION: This report describes the association of tracheoesophageal fistula (TEF), pulmonary vein stenosis (PVS), and neonatal diabetes caused by a novel mutation of the GATA6 gene in a small-for-gestational-age male neonate born at 32 weeks of gestation. Next-Generation Sequencing revealed the novel heterozygous variant c.1502C>G in the GATA6 gene, which determines the introduction of the premature stop codon p.Ser501Ter at the protein level. This de novo nonsense variant was not detected in the analyzed parental DNA samples and has not been previously described in the literature. At about two months of life, a PVS was suspected. The PVS progressively increased with the development of an intramural component, resulting in severe postcapillary pulmonary hypertension. The child died at about 4 months of life. CONCLUSION: TEF can be associated with GATA6 variants. In the case of neonatal diabetes and TEF, neonatologists should be aware of this association and should also investigate the child for complex congenital heart disorders, such as in our case, with a cardiac computed tomography.

14.
Children (Basel) ; 11(4)2024 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-38671640

RESUMO

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

15.
Pediatr Blood Cancer ; 60(1): 152-5, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-22997183

RESUMO

Long-term tunneled central venous catheters (CVC) are employed in critically ill patients. Manufacturers do not provide patient-customized devices; therefore, trimming is required for pediatric use. Scanning Electron Microscopy (SEM) coupled with energy-dispersive X-ray spectroscopy and attenuated total reflection-Fourier transform infrared spectroscopy (ATR-FTIR) was used to assess changes induced by different trimming methods on single and double lumen Hickman-Broviac catheters. Increased roughness, exposure of inorganic macroaggreagates and increase in surface inorganic charges were generated by the trimming procedure, with the scalpel producing a smoother surface compared to scissors. Trimming produces changes on the CVC surface that may influence the rate of long-term complications.


Assuntos
Cateteres Venosos Centrais , Cateteres Venosos Centrais/efeitos adversos , Criança , Humanos , Microscopia Eletrônica de Varredura , Espectroscopia de Infravermelho com Transformada de Fourier
16.
Front Pediatr ; 11: 1194928, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37260794

RESUMO

Background: Limited evidence exists about outcomes after gastric tube formation as "rescue" technique to avoid esophageal replacement in long gap esophageal atresia (LGEA). The last ERNICA Consensus Conference on the Management of LGEA has placed the techniques of gastric tubulization among the priorities for future research. Aims: Evaluate personal experience with Isoperistaltic Gastric Tube (IGT) and compare its outcomes with other more popular techniques for LGEA. Methods: A case-control study has been conducted. A retrospective monocentric analysis of LGEA patients (period: 2010-19) has been conducted in all consecutive IGT patients and each of these has been type matched with two cases of LGEA treated with other techniques. The follow-up (FU) considered was 24-months. Results: IGT and controls showed no statistically significant differences regarding preoperative variables like sex, gestational age, birth weight, syndromes, and EA type. However, IGT patients had a significantly longer esophageal GAP under boost pressure (4.5 vertebral bodies vs. 3.6, p = 0.019) at time of surgery. The analysis showed no statistical difference among the two groups about perioperative outcomes, ICU, or overall postoperative stay. No differences have been shown between IGT and controls during the follow-up regarding GERD, esophagitis, fundoplication, dysphagia, vocal cord paralysis, stenosis, and dilatations, auxologic data, need for anastomosis revision, oral aversion, and death. Conclusions: Isoperistaltic Gastric Tube is safe and effective even in LGEA patients with longer gaps, with good perioperative, post-operative and middle-term outcomes. This procedure may be considered as an alternative to avoid esophageal substitution when a primary anastomosis seems impossible for a residual gap after traction and growth techniques.

17.
Eur J Pediatr Surg ; 2023 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-37557903

RESUMO

INTRODUCTION: Neonatal ovarian simple cyst management from the pediatric surgical aspect is unclear on cyst size, follow-up, and preferred surgical approach. Therefore, this topic was selected for the 2022 Consensus Session meeting of the European Paediatric Surgeons' Association (EUPSA). METHODS: The literature was reviewed on a predefined set of questions relating to the management of the neonatal ovarian simple cysts by a panel of 7 EUPSA members, on current evidence-based opinion and practice outlined. Each question (1) outcomes of fetal interventions in neonates after birth and consensus on size/timing of intervention, (2) consensus on the type of interventions, and (3) complications in neonatal ovarian cysts and follow-up recommendations in nonoperated/operated cysts, was presented with available evidence to congress session participants. The management approach was agreed by participants and comments were accounted to formulate the consensus statement. RESULTS: There is still limited data on potential benefits and complications of prenatal ultrasound-guided aspiration; however, neonates after such procedures should be followed for 6 months. Neonates with simple ovarian cysts larger than 4 cm should be offered surgical interventions within the 2 weeks of life with complete laparoscopic cyst aspiration and fenestration with bipolar instruments being the preferred approach. Ultrasound follow-up after surgical intervention after 3 months and with the conservative approach after every 3 to 4 months until 1 year. CONCLUSION: A peer-reviewed consensus statement for the management of neonatal ovarian simple cyst was formulated based on current evidence and peer practice. The EUPSA recognizes that the statement can be useful for pediatric surgeons in decision making for this pathology.

18.
Pediatr Neonatol ; 2023 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-38163746

RESUMO

OBJECTIVE: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. STUDY DESIGN: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. RESULTS: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). CONCLUSIONS: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. LEVEL OF EVIDENCE: III retrospective comparative study.

19.
J Pediatr Surg ; 58(3): 405-411, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36150933

RESUMO

BACKGROUND: Aim of study is to report our preliminary experience with magnetic anastomosis (magnamosis) treating long-gap esophageal atresia (LGEA), the most challenging condition of esophageal atresia continuum. Magnamosis has been reported in 20 patients worldwide as an innovative and marginally invasive option. METHODS: Prospective evaluation of all LGEA patients treated with magnamosis was performed (study registration number: 2535/2021). Main outcomes considered were demographic and surgical features, postoperative complications and feeding within 6-month of follow-up. RESULTS: Between June 2020 and January 2021, 5 LGEA patients (Type A, Gross classification) were treated. Median preoperative gap was 5 vertebral bodies. Magnetic bullets were placed at an average age of 81 days of life, leading to successful magnamosis in all cases: 4 infants had primary magnetic repair (one after thoracoscopic mobilization of the pouches), 1 patient had a delayed magnamosis after Foker's procedure. Esophageal anastomosis was achieved after an average of 8 days. No anastomotic leak was found. All patients developed anastomotic stenosis at 6-month follow-up, requiring a mean of 6 dilations each. Full oral feeding was achieved in 3 patients, while 2 were still on oral-gastrostomy feeding. One patient experienced small esophageal perforation after dilation (3 months after magnamosis), distal to the anastomotic stricture and subsequently developed oral aversion. CONCLUSIONS: Our preliminary results suggest magnamosis a safe and effective minimally invasive option in patients with LGEA. Absence of postoperative esophageal leaks may represent a major advantage of magnamosis over conventional surgery, although possible high rate of esophageal stenosis should be further evaluated. LEVELS OF EVIDENCE: IV (Case series with no comparison group).


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório , Atresia Esofágica , Estenose Esofágica , Lactente , Humanos , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Estenose Esofágica/etiologia , Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Magnetismo , Anastomose Cirúrgica/métodos , Resultado do Tratamento
20.
Diagnostics (Basel) ; 13(5)2023 Feb 27.
Artigo em Inglês | MEDLINE | ID: mdl-36900042

RESUMO

Introduction: The use of a lung ultrasound (LUS) score has been described in the early phases of neonatal respiratory distress syndrome; however, there is still no data regarding the application of the LUS score to neonates with a congenital diaphragmatic hernia (CDH). The objective of this observational cross-sectional study was to explore, for the first time, the postnatal changes in LUS score patterns in neonates with CDH, with the creation of a new specific CDH-LUS score. Methods: We included all consecutive neonates with a prenatal diagnosis of CDH admitted to our Neonatal Intensive Care Unit (NICU) from June 2022 to December 2022 who underwent lung ultrasonography. Lung ultrasonography (LUS) was determined at scheduled time points: (T0) during the first 24 h of life; (T1) at 24-48 h of life; (T2) within 12 h of surgical repair; (T3) a week after the surgical repair. We used a modified LUS score (CDH-LUS), starting from the original 0-3 score. We assigned 4 as a score in the presence of herniated viscera in the hemithorax (liver, small bowel, stomach, or heart in the case of a mediastinal shift) in the preoperative scans or pleural effusions in the postoperative scans. Results: We included in this observational cross-sectional study 13 infants: twelve/13 had a left-sided hernia (2 severe, 3 moderate, and 7 mild cases), while one patient had a right-sided severe hernia. The median CDH-LUS score was 22 (IQR 16-28) during the first 24 h of life (T0), 21 (IQR 15-22) at 24-48 h of life (T1), 14 (IQR 12-18) within 12 h of surgical repair (T2) and 4 (IQR 2-15) a week after the surgical repair (T3). The CDH-LUS significantly dropped over time from the first 24 h of life (T0) to a week after the surgical repair (T3), according to ANOVA for repeated measures. Conclusion: We showed a significant improvement in CDH-LUS scores from the immediate postoperative period, with normal ultrasonographic evaluations a week after surgery in most patients.

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