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Background: The objective of this paper is to describe the clinical and epidemiological profile and the early outcomes of patients with omphalocele born in a fetal medicine, pediatric surgery, and genetics reference hospital in Rio de Janeiro - Brazil. To determine its prevalence, describe the presence of genetic syndromes, and congenital malformations, emphasizing the characteristics of congenital heart diseases and their most common types. Methods: Using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) database and records review, a retrospective cross-sectional study was performed, including all patients born with omphalocele between January 1st, 2016, and December 31st, 2019. Results: During the period of the study, our unity registered 4,260 births, 4,064 were live births and 196 stillbirths. There were 737 diagnoses of any congenital malformation, among them 38 cases of omphalocele, 27 were live born, but one was excluded for missing data. 62.2% were male, 62.2% of the women were multiparous and 51.3% of the babies were preterm. There was an associated malformation in 89.1% of the cases. Heart disease was the most common (45.9%) of which tetralogy of Fallot was the most frequent (23.5%). Mortality rate was 61.5%. Conclusions: Our data showed a good correspondence with the existing literature. Most patients with omphalocele had other malformations, especially congenital heart disease. No pregnancy was interrupted. The presence of concurrent defects showed a huge impact on prognosis, since, even if most survived birth, few remained alive and received hospital discharge. Based on these data, fetal medicine and neonatal teams must be able to adjust parents counseling about fetal and neonatal risks, especially when other congenital diseases are present. Summary of findings: Most patients with omphalocele had other malformations, especially congenital heart disease. The presence of concurrent defects showed a huge impact on prognosis. A clear perinatal plan should be developed for couples with a multidisciplinary team, aimed at minimizing maternal morbidity and providing better care for the newborn. [ABSTRACT FROM AUTHOR]

Purpose: Myelomeningocele (MMC) is an open neural tube defect that causes great morbidity. Prenatal open repair is the standard treatment; however, there are many complications related to the procedure. This study reports preliminary findings of open in utero repair of MMC in a public tertiary hospital in Brazil and describes factors that could be associated with increased surgical morbidity. Methods: Thirty-nine patients underwent open in utero repair of MMC from October 2015 to August 2019. The Clavien–Dindo classification of surgical complications and a classification system with the preterm definitions of the World Health Organization were used, respectively, for maternal and fetal complications. Results: A total of 28 mothers (71.8%) and 31 fetuses (79.5%) experienced at least one minor to major complication. Three mothers (7.7%) had a severe grade 4 complication. Fetal complications grades 3 to 5 occurred in 13 fetuses (33.3%). Gestational age at surgery and at birth were 24.88 ± 1.16 weeks and 33.23 ± 3.68 weeks, respectively. Preterm delivery occurred in 30 patients (76.9%), membrane rupture in 18 patients (46.2%) and chorioamnionitis in 13 patients (33.3%). Conclusion: Open fetal surgery for MMC was performed at a Brazilian public tertiary care center, resulting in three grade 4 maternal complications. Relevant fetal complications were also present. The use of a standard classification system for complications renders studies more comparable and data more useful for counseling patients. Adjustments of perioperative procedures and long-term follow-up are needed to determine the real benefit of open in utero repair of MMC at our hospital. [ABSTRACT FROM AUTHOR]

Objective: Describe outcomes of open fetal surgery for myelomeningocele (MMC) repair in two Brazilian hospitals and the impact of surgical experience on outcome.Design: Retrospective cohort study.Setting: Sao Paulo, Brazil.Population: 237 pregnant women carrying a fetus with an open spinal defect.Methods: Surgical details, and maternal and fetal outcomes collected from all patients.Main Outcome Measures: Analysis of surgical and perinatal outcome parameters.Results: Total surgical time was 119 ± 7.6 minutes. Preterm labour occurred in 24.2%, premature rupture of membranes in 26.7%, placental abruption in 0.8%, need for a blood transfusion at delivery in 2.1%, and dehiscence at the repair site in 2.5%. Reversal of hindbrain herniation at birth occurred in 71.4%. There were no maternal deaths or severe maternal morbidities. The failure rate with the patient anaesthetised was 0.42% and perinatal mortality was 2.1% (three intrauterine demises and two neonatal deaths). Comparing results from our study in the first 3 years with the last 3 years demonstrated improvement in the total surgical time (121.2 ± 6.4 versus 118.5 ± 8.2 minutes, P = 0.005) and an increase in reversal of hindbrain herniation at birth (64.0 versus 77.1%, P = 0.042).Conclusion: Our open fetal surgical approach for MMC was effective and results were comparable to past studies. Improvements in surgical performance and perinatal outcome increased as the surgical team became more familiar with the procedure.Funding: The study was funded solely by institutional funds.Tweetable Abstract: Brazilian experience of in utero open surgery for myelomeningocele repair. [ABSTRACT FROM AUTHOR]

Objective: To present the feasibility of fetal myelomeningocele (MMC) repair through a mini-hysterotomy and to describe the perinatal results from our initial experience.Methods: A descriptive study of cases of fetal MMC correction via mini-hysterotomy performed between 2014 and 2016.Results: Forty-five women underwent fetal surgery and 87% (39/45) delivered. A complete multilayer correction of the MMC was possible in all cases. There were no maternal, fetal or neonatal deaths. No maternal or fetal complications occurred from fetal MMC correction until maternal hospital discharge. The average gestational age (GA) at surgery was 24.5 weeks (standard deviation, SD: 1.7; range: 20.7-26.9). The median hysterotomy length was 3.05 cm (SD: 0.39; range: 2.50-3.50). One patient (1/39; 2.6%) experienced chorioamniotic separation. Nine patients (9/39; 23.1%) had premature preterm rupture of membranes at a median GA of 34.1 weeks (range: 31.1-36.0). The average GA at delivery was 35.3 weeks (SD: 2.2; range: 27.9-39.1). Ninety-five percent (37/39) of our patients had an intact hysterotomy site at delivery. Ventriculoperitoneal shunt placement was necessary for 7.7% (3/39) of the neonates.Conclusion: Fetal MMC repair is feasible through a mini-hysterotomy. This approach appears to be associated with reduced risks of very preterm delivery and maternal, fetal and neonatal complications. [ABSTRACT FROM AUTHOR]

Purpose: Present the outcomes of six cases submitted to intrauterine myelomeningocele (MMC) repair. Methods: Descriptive observational study of six children submitted to antenatal surgical repair of MMC between 26 and 27 weeks gestation. All deliveries were through cesarean section. The following neonatal variables were assessed: gestational age at delivery, birth weight, Apgar scores, need for intubation, duration of hospital stay and need for postnatal shunt procedures. After 3.5 years, the children were evaluated using the Columbia Mental Maturity Scale or Denver II tests and the Hoffer Ambulation Scale. Results: All deliveries were preterm at a mean gestational age of 32 + 4 weeks and mean birth weight was 1,942 g. Two infants had Apgar scores <7 at 1 min and 1 at 5 min. Ventricular-peritoneal shunts were placed in two cases. All six children are alive: five have normal cognitive development and one has a neuropsychomotor developmental delay. Two children had normal leg movements, a sacral functional level and are community ambulators. Three children had upper lumbar anatomical level lesions and one had a lower thoracic level lesion at the time of fetal surgery. One child, with an L1-L2 anatomical level lesion, in noambulatory and fully dependent on a wheelchair for mobility. Conclusion: Antenatal surgical repair of MMC reduced the need for postnatal shunt placements. Despite preterm delivery, the cognitive development of most children at 3.5 years was normal. Antenatal surgery seemed to improve lower limb motor function in these cases. [ABSTRACT FROM AUTHOR]

Background: This study aims to assess orthopaedic surgeon knowledge in Brazil about ionizing radiation and its health implications on surgical teams and patients. Methods: A 15-question survey on theoretical and practical concepts of ionizing radiation was administered during the 23rd Brazilian Orthopaedic Trauma Association annual meeting. The survey addressed issues within orthopedic surgery, such as radiation safety concepts, protection, exposure, as well as the participant gender. Participants were either orthopedic surgeons or orthopedic surgery residents working at institutions in Brazil. Results: One thousand surveys were distributed at the moment of the meeting registration, and 258 were answered completely (25.8% response rate). Only 5.8% of participants used basic radiation protection equipment; 47.3% used a dosimeter; 2.7% reached the annual maximum permissible radiation dose; 10.5% knew the period of increased risk to fetal gestation; 5.8% knew the maximum permissible radiation dose during pregnancy; 58.5% knew that the hands, eyes, and thyroid are the most exposed areas and at greater risk of radiation-related lesions; 25.2% knew the safe distance from a radiation-emitting tube is 3 m or more; 44.2% knew the safest positioning of the radiation-emitting tube; 25.2% knew that smaller tubes emit greater radiation at the entrance dose to magnify the image; and 55.4% knew that the surgery team receives more scattered radiation in surgical procedures performed on obese patients. Conclusion: This study revealed inadequate theoretical and practical knowledge about radiation exposure among orthopaedic surgeons in Brazil. Only a minority of orthopaedic surgeons used basic radiation protection equipment. No significant differences in knowledge were found when comparing all orthopedic surgery specialties. Our findings indicate an urgent need for education to increase knowledge among orthopaedic surgeons about the hazards of ionizing radiation. Personal protection and implementation of the ALARA (as low as reasonably achievable) protocol in daily practice are important behaviors to prevent the harmful effects of ionizing radiation. [ABSTRACT FROM AUTHOR]

No abstract available Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Objective: To describe the surgical technical aspects associated with the development of urological fistulas after fetal antegrade cystoscopic laser fulguration of the posterior urethral valves (PUV)., Methods: The perioperative data for all fetal cystoscopies performed between January 2004 and August 2013 at three institutions in the USA, France and Brazil were reviewed, with particular emphasis on surgical technical aspects of the procedure and the complications encountered., Results: A total of 40 fetal cystoscopies were performed at the three institutions. Laser fulguration of the PUV was performed in 23 of these cases, with a survival rate of 60.9% (14/23) and normal renal function in 85.7% (12/14) of these infants. Urological fistulas were diagnosed postnatally in four (10%) newborns. The presence of fistulas was associated with a higher gestational age at diagnosis of PUV (P < 0.01) and with the use of semi-curved rather than curved sheaths (P < 0.01), the use of a diode laser (P < 0.01) and the use of higher laser power and energy (P < 0.01 and P < 0.01, respectively), as well as with less operator experience (P < 0.01) and with absence of fetal anesthesia/immobilization (P = 0.02)., Conclusion: Urological fistulas are a severe complication of fetal cystoscopic laser fulguration of PUV and are associated with type, energy and power settings of the laser and instrumentation. The use of appropriate technique and proper training of the operator are necessary to perform this fetal intervention safely., (Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.)