Search Results (15)

Fetal surgery has emerged as a viable option for the management of selected congenital anomalies that result in severe or lethal outcomes if left untreated until birth. Conditions such as spina bifida, urinary tract obstruction, congenital cystic adenomatoid malformation, diaphragmatic hernia, sacrococcygeal teratoma, and twin–twin transfusion syndrome have shown improved prognosis after in utero intervention, open, or fetoscopically. Despite significant advances in surgical methods and anesthesia, preterm labor remains a primary concern. Stem cell transplantation and in utero gene therapy are developing, and they have the potential to expand the treatment window, as they minimize maternal complications. Hematopoietic stem cell transplantation, which is based on the immaturity of the fetal immune system, is a promising treatment for inherited disorders. Although many procedures of fetal interventions are now established, their safety and efficacy must be ensured and this requires optimal patient selection and choice of appropriate timing for intervention, adherence to ethical principles, and continuous research. Therefore, a multidisciplinary team, including specialists in maternal–fetal medicine, pediatric surgery, anesthesiology, neonatology, psychosocial support, and bioethics, is essential to guide comprehensive, patient-centered care. Fetal surgery is an evolving field that offers hope for conditions previously considered untreatable before birth. Full article

Congenital cystic adenomatoid malformation (CCAM) is a rare fetal lung anomaly characterized by benign multicystic masses that can lead to severe complications, such as pulmonary hypoplasia, fetal hydrops, and neonatal death. This literature review examines current knowledge on antenatal therapies for CCAM, focusing on pharmacological, procedural, and surgical interventions. Betamethasone, the first-line pharmacological treatment, has shown efficacy in reducing lesion size and resolving hydrops, particularly in microcystic CCAM. Procedural options, such as thoracoamniotic shunting, are effective for macrocystic lesions but carry risks including preterm labor and thoracic deformities. Open fetal surgery remains a last-resort intervention for severe cases, while emerging techniques, like percutaneous laser ablation and sclerotherapy, offer promising minimally invasive alternatives. A proposed treatment algorithm emphasizes individualized care based on lesion type, gestational age, and the presence of complications. The authors searched the US National Library of Medicine Database, Google Scholar, and PubMed Central to gather information on antenatal therapies for CCAM. This review emphasizes that, despite significant advancements, considerable challenges persist, underscoring the need for prospective studies to refine therapeutic protocols and assess long-term outcomes. Full article

Fetal surgery has made significant strides over the past 40 years, facilitated by advances in technology and imaging modalities enabling the diagnosis and treatment of congenital anomalies in utero. The Management of Myelomeningocele Study (MOMS), a multicenter randomized controlled trial, established open fetal myelomeningocele (MMC) repair as the gold standard for improving neurological outcomes compared to postnatal repair. However, this approach is associated with increased maternal complications and preterm birth due to hysterotomy, prompting the exploration of minimally invasive alternatives. Due to the lack of an existing randomized control trial with fetoscopic MMC repair and variations in technique (percutaneous versus laparotomy/transuterine access, different trocar configurations, closure methods, and patch applications) among different fetal centers, more studies are needed to optimize this approach as an alternative to the standard of care. This paper proposes to assess the basics tenets of open fetal MMC repair and to establish guiding principles for a fetoscopic approach that could prove to be equivalent or superior to open fetal MMC repair in maternal and fetal outcomes and lead to clinical implementation. Full article

Renal masses are uncommon during pregnancy; they represent the most frequently encountered urological cancer in pregnant patients and require careful surgical planning. The introduction of robotic surgical systems aims to address these challenges by simplifying intra-corporeal suturing and reducing technical complexity. Robot-assisted laparoscopic renal surgery offers potential benefits over both open surgery and conventional laparoscopy, providing greater precision and reduced invasiveness, particularly in tumor excision and suturing. Although urological tumors during pregnancy are rare, early detection significantly improves outcomes by enabling intervention before the tumor advances and while the uterus remains relatively small. The decision regarding the timing and necessity of surgery in pregnant patients requires a careful assessment of maternal health, fetal development, and the progression of the disease. Risks for adverse pregnancy outcomes should be explained, and the patient’s decision about pregnancy termination should be considered. Radical nephrectomy or nephron-sparing surgery are essential treatments for the management of renal tumors. Effective management demands close collaboration between a multidisciplinary team and the patient to ensure individualized care. The aim of this review was to evaluate the renal tumors during pregnancy in terms of epidemiology, risk factors, diagnosis and the safety of a robot-assisted laparoscopic approach in the management of these tumors. Full article

Background: Obesity rates have significantly increased globally, affecting up to 40% of women of childbearing age in the United States. While prenatal repair of open fetal spina bifida has shown improved outcomes, most fetal surgery centers exclude patients with a body mass index (BMI) ≥ 35 kg/m² based on criteria from the Management of Myelomeningocele Study (MOMS) trial. This exclusion raises concerns about healthcare equity and highlights a significant knowledge gap regarding the safety and efficacy of fetal spina bifida repair in patients with obesity. Objective: To review the current state of knowledge regarding open fetal surgery for fetal spina bifida in patients with obesity, focusing on safety, efficacy, and clinical considerations. Methods: A comprehensive literature search was conducted using the PubMed and EMBASE databases, covering articles from the inception of the databases to April 2024. Studies discussing fetal surgery for neural tube defects and documenting BMI measurements and their impact on surgical outcomes, published in peer-reviewed journals, and available in English were included. Quantitative data were extracted into an Excel sheet, and data synthesis was conducted using the R programming language (version 4.3.3). Results: Three retrospective studies examining outcomes of prenatal open spina bifida repair in a total of 43 patients with a BMI ≥ 35 kg/m² were identified. These studies did not report significant adverse maternal or fetal outcomes compared to patients with lower BMIs. Our pooled analysis revealed a perinatal mortality rate of 6.1% (95% CI: 1.76–18.92%), with 28.0% (95% CI: 14.0–48.2%) experiencing the premature rupture of membranes and 82.0% (95% CI: 29.2–98.0%) delivering preterm (<37 weeks). Membrane separation was reported in 10.3% of cases (95% CI: 3.3–27.7%), the mean gestational age at birth was 34.3 weeks (95% CI: 32.3–36.3), and the average birth weight was 2651.5 g (95% CI: 2473.7–2829.4). Additionally, 40.1% (95% CI: 23.1–60.0%) required a ventriculoperitoneal shunt. Conclusion: While current evidence suggests that fetal spina bifida repair may be feasible in patients with obesity, significant limitations in the existing body of research were identified. These include small sample sizes, retrospective designs, and a lack of long-term follow-up data. There is an urgent need for large-scale, prospective, multicenter studies to definitively establish the safety and efficacy of fetal spina bifida repair in patients with obesity. Such research is crucial for developing evidence-based guidelines, improving clinical outcomes, and addressing healthcare disparities in this growing patient population with obesity. Full article

Background: This study aims to evaluate the outcomes of fetal sacrococcygeal teratoma (SCT) submitted to prenatal interventions. Methods: We performed a systematic literature review of fetal SCT patients and compared the outcomes between open fetal surgery and percutaneous intervention. In addition, we also compared the results of SCT fetuses who did not undergo any surgical intervention (NI). Results: We identified 16 cases of open fetal surgery (OS), 48 cases of percutaneous fetal intervention (PI), and 93 NI patients. The survival rate was 56.2% in OS, 45.8% in PI (p = 0.568), and 71.0% in NI patients. The gestational age at delivery was earlier in cases where there was no survival compared to cases where the fetuses did survive across all evaluated cohorts (OS: p = 0.033, PI: p < 0.001, NI: p < 0.001). The gestational weeks at delivery in OS and PI fetuses were more similar; however, OS tended to be performed later on in pregnancy, and the affected fetuses had more severe presented findings. In our evaluation, we determined that the presence of fetal hydrops and cardiac failure had no significant impact on survival in SCT cases. In NI patients, polyhydramnios was much higher in fetuses who did not survive compared to their surviving cohorts (p < 0.001). Conclusions: In conclusion, gestational age at delivery can affect the short-term prognosis of fetuses affected with sacrococcygeal teratomas. Regardless of the mode of delivery or the necessity for intervention during the fetal period, monitoring for complications, including polyhydramnios, can prevent premature delivery. Full article

Stents are tubular meshed endoprostheses implanted mini-invasively through a transcatheter intervention to guarantee the patency of body conduits, mainly in cardiovascular applications. In pediatric cardiology, stenting has become an accepted procedure in the treatment of congenital heart disease (CHD) as an alternative to open-heart surgery. CHD refers to a range of defects affecting the heart’s structure and function arising from abnormal development during pregnancy. While during fetal life, the presence of additional shunts allows for the establishment of parallel circulation and survival of gestation, CHD is not compatible with extrauterine life, and medical intervention is required soon after birth. This review aims to discuss the state of the art of stenting in CHD. Despite the severity of these pathologies, investment from the industry remains limited due to the restricted number of cases, and dedicated devices are still missing. As a consequence, commercially available adult stents are commonly exploited on an off-label basis in newborns without any optimization for the specific anatomy and required function. In this review, a classification of the available stents is provided, resuming the manufacturing technologies, materials, and geometrical aspects to obtain the target biomechanical performance. After analyzing the fetal circulation, different forms of CHD amenable to stenting are considered, collecting the stents currently adopted and discussing the clinical outcomes to outline the features of an ideal device. Full article

Open spina bifida (OSB) is a congenital, non-lethal malformation with multifactorial etiology. Fetal therapy can be offered under certain conditions to parents after accurate prenatal diagnostic and interdisciplinary counseling. Since the advent of prenatal OSB surgery, various modifications of the original surgical techniques have evolved, including laparotomy-assisted fetoscopic repair. After a two-year preparation time, the team at the University of Giessen and Marburg (UKGM) became the first center to provide a three-port, three-layer fetoscopic repair of OSB via a laparotomy-assisted approach in the German-speaking area. We point out that under the guidance of experienced centers and by intensive multidisciplinary preparation and training, a previously described and applied technique could be transferred to a different setting. Full article

Right-sided ligamentum teres (RSLT) is a rare anatomic variant in which the fetal umbilical vein connects to the right portal vein. Patients with RSLT frequently have hepatic vasculature and bile duct anomalies, which increase the risk of complications with hepatectomy. Most patients with RSLT undergo open hepatectomy. Herein, we describe a patient with RSLT and hepatocellular carcinoma who underwent laparoscopic hepatectomy. The patient was a 69-year-old man with hepatocellular carcinoma located in the left liver based on computed tomography (CT) and magnetic resonance imaging. Imaging also demonstrated RSLT. Three-dimensional CT analysis revealed independent right lateral type anomalies of the portal vein and bile duct. A laparoscopic extended left lateral sectionectomy was performed after careful surgical planning. Ultrasonography was used frequently during surgery to avoid damaging the right hepatic vasculature. The left lateral and partial left median sections were removed as planned. The patient’s postoperative recovery was uneventful. Avoiding injury to the right hepatic vasculature is essential when performing left lobectomy, including left lateral sectionectomy, in patients with RSLT. Laparoscopic hepatectomy can be performed safely in patients with RSLT, provided that careful surgical planning is conducted using preoperative three-dimensional CT analysis and intraoperative ultrasonography. Full article

Early diagnosis of pregnancy- and lactation-associated osteoporosis (PLO) is mandatory for a good outcome. Standard care is not a matter of conventional guidelines, rather it requires an individualized strategy while true overall incidence and pathogeny remain open issues. This is a narrative review based on full-length English articles, published between January 2021 and March 2023 and accessed via PubMed (no traumatic fractures or secondary osteoporosis are included). Our case-sample-based analysis included 836 females with PLO (the largest cohort based on published cases so far) through 12 studies and 24 single case reports. Except for one survey, these involved retrospective cohorts of small size (6–10 females/study) to medium size (23–47 women/study), and large cohorts with >50 subjects per study (a maximum of 379). Age of diagnosis: from 24 to 40 years for case reports (most subjects being over 30 and primigravida), while original studies indicated an average age between 31 and 34.18 years. Type of fractures underlined a most frequent vertebral phenotype (a mean of 2 to 5.8 vertebral fractures per patient) versus a most severe non-vertebral phenotype (hip and femoral neck fractures mostly requiring surgery). Potential contributors varied: smoking (1/3–1/2 of subjects), family history of osteoporosis (1/3), heparin and glucocorticoid use in pregnancy, low body mass index (majority of cases), hypovitaminosis D; and (with a low level of statistical significance) anti-psychotic medication, gestational diabetes, lupus, thrombophilia, anemia, in vitro fertilization (1/3 in one study), twin pregnancy, tocolysis with MgSO4, and postpartum thyroiditis. Most remarkably, up to 50% of PLO patients harbor mutations of LRP5, WNT1, and COL1A1/A2 (more damaged form with potential benefits from osteoanabolic drugs); gene testing might become the new norm in PLO. The low index of clinical suspicion should be supported by performing magnetic resonance imaging (gold standard in pregnancy) with DXA (in lactation). Low bone mineral density is expected (Z-score varying from −2.2 SD to −4 SD, unless normal which does not exclude PLO). Bone turnover markers might be useful in individuals with normal DXA, in pregnancy when DXA cannot be performed, and in following the response to anti-osteoporosis drugs. Alternatively, microarchitecture damage might be reflected by DXA-trabecular bone score and high-resolution peripheral quantitative computed tomography. Specific medical interventions are currently focused on teriparatide (TPT) use (3 studies; n = 99 females treated with TPT and an additional subgroup of 18 patients from the gene-analysis-based study, thus a total of 117 females) which seems to be the therapy of choice as reflected by these new data: 6–24 months, 20 µg/day, no sequential therapy needed; case selection based on high fracture risk is necessary). The first case using romosozumab was reported in 2022. PAO/LAO remains a challenging condition which is a battle for the wellbeing of two individuals, on one hand, considering maternal-fetal outcomes and taking care of the offspring, but it is a battle for a multidisciplinary team, on the other hand, since a standardized approach is lacking. Full article

Fetal and maternal risks associated with open fetal surgery (OFS) in the management of meningomyelocele (MMC) are considerable and necessitate improvement. A modified technique of hysterotomy (without a uterine stapler) and magnesium-free tocolysis (with Sevoflurane as the only uterine muscle relaxant) was implemented in our new magnesium-free tocolysis and classical hysterotomy (MgFTCH) protocol. The aim of the study was to assess the introduction of the MgFTCH protocol in reducing maternal and fetal complications. The prospective study cohort (SC) included 64 OFS performed with MgFTCH at the Fetal Surgery Centre Bytom (FSCB) (2015–2020). Fetal and maternal outcomes were compared with the retrospective cohort (RC; n = 46), and data from the Zurich Center for Fetal Diagnosis and Therapy (ZCFDT; n = 40) and the Children’s Hospital of Philadelphia (CHOP; n = 100), all using traditional tocolysis. The analysis included five major perinatal complications (Clavien-Dindo classification, C-Dc) which developed before the end of 34 weeks of gestation (GA, gestational age). None of the newborns was delivered before 30 GA. Only two women presented with grade 3 complications and none with 4th or 5th grade (C-Dc). The incidence of perinatal death (3.3%) was comparable with the RC (4.3%) and CHOP data (6.1%). MgFTCH lowers the risk of major maternal and fetal complications. Full article

Open spina bifida is one of the most common congenital defects of the central nervous system. Open fetal surgery, which is one of the available therapeutic options, remains the gold standard for prenatal repairs. Fetoscopic closure may lower the number of maternal complications associated with open fetal surgery. Regardless of the approach, the outcome may be compromised by the development of tethered spinal cord (TSC) syndrome. At 24.2 weeks of gestation, a primipara was admitted due to fetal myelomeningocele and was deemed eligible for fetoscopic repair. Fetal surgery was performed at 25.0 weeks of gestation. It was the first complete untethering of the spinal cord and anatomic reconstruction (dura mater, spinal erectors, skin) achieved during a fetoscopic repair of spina bifida. Cesarean section due to placental abruption was performed at 31.1 weeks of gestation. VP shunting, with no need for revision, was performed at 5 weeks postdelivery due to progressing ventriculomegaly. No clinical or radiological signs of secondary tethering were observed. Neurological examination at 11 months postdelivery revealed cranial nerves without any signs of damage, axial hypotonia, decreased muscle tone in the lower extremities, and absent pathological reflexes. Motor development was slightly retarded. Complete untethering of the neural structures should always be performed, regardless of the surgical approach, as it is the only course of action that lowers the risk for developing secondary TSC. Full article

(1) Background: The morbidity of gastroschisis is defined by exposure of unprotected intestines to the amniotic fluid leading to inflammatory damage and consecutive intestinal dysmotility, the viscero-abdominal disproportion which results in an abdomen too small to incorporate the herniated and often swollen intestine, and by associated pathologies, such as in complex gastroschisis. To prevent intestinal damage and to provide for growth of the abdominal cavity, fetal interventions such as amnio exchange, gastroschisis repair or covering have been evaluated in several animal models and human trials. This review aims to evaluate the reported techniques for the fetal treatment of gastroschisis by focusing on minimally invasive procedures. (2) Methods: We conducted a systematic database search, quality assessment and analyzed relevant articles which evaluate or describe surgical techniques for the prenatal surgical management of gastroschisis in animal models or human application. (3) Results: Of 96 identified reports, 42 eligible studies were included. Fetal interventions for gastroschisis in humans are only reported for EXIT procedures and amnio exchange. In animal models, particularly in the fetal sheep model, several techniques of open or minimally invasive repair of gastroschisis or covering the intestine have been described, with fetoscopic covering being the most encouraging. (4) Discussion: Although some promising minimally invasive techniques have been demonstrated in human application and animal models, most of them are still associated with relevant fetal morbidity and mortality and barely appear to be currently applicable in humans. Further research on specific procedures, instruments and materials is needed before any human application. Full article

As many meta-analyses comparing pediatric minimally invasive to open surgery can be found in the literature, the aim of this review is to summarize the current state of minimally invasive pediatric surgery and specifically focus on the trends and developments which we expect in the upcoming years. Print and electronic databases were systematically searched for specific keywords, and cross-link searches with references found in the literature were added. Full-text articles were obtained, and eligibility criteria were applied independently. Pediatric minimally invasive surgery is a wide field, ranging from minimally invasive fetal surgery over microlaparoscopy in newborns to robotic surgery in adolescents. New techniques and devices, like natural orifice transluminal endoscopic surgery (NOTES), single-incision and endoscopic surgery, as well as the artificial uterus as a backup for surgery in preterm fetuses, all contribute to the development of less invasive procedures for children. In spite of all promising technical developments which will definitely change the way pediatric surgeons will perform minimally invasive procedures in the upcoming years, one must bear in mind that only hard data of prospective randomized controlled and double-blind trials can validate whether these techniques and devices really improve the surgical outcome of our patients. Full article

Optimising the diet and weight of women prior to and during pregnancy is of paramount importance to both maternal and offspring health. In women who become pregnant after bariatric surgery, evidence suggests a better overall obstetric outcome in comparison to women with severe obesity managed conservatively. Historically, most studies in this population group have monitored supplement adherence or serum concentrations of micronutrients, rather than dietary intake. The aim of this study was to synthesise current knowledge of the dietary intake of women during pregnancy following bariatric surgery. A systematic search of search engines was conducted using the following databases: MEDLINE, Embase, CINAHL, Cochrane database, Scopus, Trip, NHS Evidence, UK Clinical Trials, ClinicalTrials.gov, Prospero, Epistemonikos and Open Grey. Titles and abstracts were screened independently by two reviewers against predefined inclusion and exclusion criteria. After removal of duplicates, 1594 titles were identified, of which 1586 were initially excluded. Following full-text review, four articles were included. In total, across all four studies, data from only 202 bariatric surgery participants were included, the majority of whom had had one type of surgery. Just one study included a control group. Reporting of nutritional outcomes was heterogenous, with none of the studies including complete macro and micronutrient intake results in their articles. An insufficient intake of protein was noted as a concern in two studies and associated with poor fetal growth in one study. Overall, this review has identified a paucity of data about the dietary intake of women during pregnancy after bariatric surgery. Full article