Aimen Farouk Shaaban

BACKGROUND AND OBJECTIVES: Myelomeningocele is defined by the presence of a fluid-filled sac that contains the neural placode and cerebrospinal fluid (CSF) at the level of the spinal defect. Alternatively, the term myeloschisis is used when the neural placode is not contained within a CSF-filled sac. While both are eligible for prenatal closure, few studies have characterized the impact of the type of lesion on postnatal outcomes. This study compares the outcomes of these 2 types of open spinal dysraphism using data from the Fetal Myelomeningocele Consortium registry sponsored by the North American Fetal Therapy Network. METHODS: The prospective observational Fetal Myelomeningocele Consortium registry was used to extract deidentified data corresponding to patients who underwent fetal closure of myelomeningocele vs myeloschisis. Demographics, operative characteristics, perioperative complications, and postnatal outcomes were collected. RESULTS: A total of 1327 patients were identified, including 991 (74.7%) with myelomeningocele and 336 (25.3%) with myeloschisis. Compared with myelomeningocele, myeloschisis was associated with lower rates of prenatal talipes (11% vs 24%, P < .001) and higher rates of favorable prenatal movement (94% vs 86%, P < .001). Postnatally, myeloschisis conferred higher rates of leg movement (97% vs 90%, P = .002) and an increased likelihood of ambulation in the household (10.4% vs 4.1%, P = .009) and outside (7.2% vs 1.9%, P = .004) at 1 year. However, myeloschisis was also linked to greater use of skin patches during closure (49% vs 27%, P < .001), higher CSF leak rates among those with wound dehiscence (26% vs 8%, P = .014), and more frequent CSF diversion and tethered cord surgeries at follow-up. CONCLUSION: Myeloschisis and myelomeningocele demonstrate distinct clinical profiles. Although patients with myeloschisis may have favorable motor outcomes, they are also characterized by higher rates of CSF leakage, CSF diversion, and tethered cord surgery. These findings highlight the need for lesion-specific prognostication and may inform surgical planning and parental counseling in the context of prenatal spina bifida closure.

Fetal surgery offers valuable opportunities to address severe congenital disabilities, yet accurate evaluation of fetal physiological changes during in utero procedures to mitigate the risk of operative complications remains an unmet need. Conventional unimodal approaches lack predictive value, specificity and compatibility with minimally invasive interventions. Here we present a bioelectronic system featuring a multimodal, steerable filamentary probe that interfaces directly with the fetus in utero, enabling reliable and minimally invasive monitoring of various physiological parameters. Integrated soft robotic actuators ensure consistent contact through controlled navigation and force delivery, creating a gentle and secure interface with delicate fetal surfaces. In a sheep fetal surgery model, the multifunctional probe effectively monitored in utero conditions during fetoscopic surgeries, detecting fetal bradycardia, hypoxia and hypothermia, potentially informing for early intervention. Experimental results on rodents and large animal fetuses demonstrate potential for direct translation to human use. This system offers continuous, comprehensive fetal monitoring, addressing gaps in current clinical practices, and provides real-time insights during fetal surgeries.

The increasing demand for continuous, comprehensive physiological information captured by skin-interfaced wireless sensors is hindered by their relatively high-power consumption and the associated patient discomfort that can follow from the use of high capacity batteries. This paper presents an adaptive electronics platform and a tri-modal energy harvesting approach to reduce the need for battery power. Specifically, the schemes focus on sensors that involve light in their operation, through use of (i) photometric methods, where ambient light contributes directly to the measurement process, (ii) multijunction photovoltaic cells, where ambient light powers operation and/or charges an integrated battery, and (iii) photoluminescent packaging, where ambient light activates light-emitting species to enhance the first two schemes. Additional features of interest are in (i) in-sensor computational approaches that decrease the bandwidth and thus the energy consumption in wireless data communication and (ii) radio frequency power transfer for battery charging. These ideas have utility across broad other classes of wearable devices as well as small, portable electronic gadgetry.

Objective: To compare the occurrence of fetal bradycardia in open versus fetoscopic fetal spina bifida surgery. Methods: This is a single-institution retrospective cohort study of patients undergoing open (n = 25) or fetoscopic (n = 26) spina bifida repair between 2017 and 2022. From October 2017 to June 2020, spina bifida repairs were performed via an open classical hysterotomy, and from November 2020 to June 2022 fetoscopic repairs were performed following transition to this technique. Fetal heart rate (FHR) in beats per minute (bpm) was recorded via echocardiography every 15 min during the procedure. Cohort characteristics, fetal bradycardia and maternal physiologic parameters were compared between the groups. Results: Fetuses undergoing an open repair more frequently developed bradycardia defined as <110 bpm (32% vs. 3.8%, p = 0.008), and a trend was observed for FHR decreases more than 25 bpm from baseline (20% vs. 3.8%, p = 0.073). Profound bradycardia less than 80 bpm was rare, occurring in only three operations (two in open, one in fetoscopic repair) with two fetuses (one in each group) requiring emergency cesarean delivery. Conclusion: When compared to open fetal surgery, fetal bradycardia occurred less frequently in fetoscopic surgery despite a significantly greater anesthetic exposure and the use of the intraamniotic carbon dioxide insufflation.

Introduction: Fetal thoracoamniotic shunts are common lifesaving interventions but frequently require replacement. Needle fetal thoracoscopy is a technique that uses standard thoracoamniotic shunt introducer sheaths to permit direct visualization and even instrument manipulation during shunt deployment to facilitate optimal positioning and primary shunt function in the most challenging cases. Case Presentation: In this study, 5 patients who underwent needle fetal thoracoscopy-assisted thoracoamniotic shunt placement were reviewed. Three patients with large, macrocystic congenital pulmonary airway malformations (CPAMs) with evidence of worsening mediastinal shift and/or hydrops and 2 patients with large chylothorax with fetal hydrops were treated. Four cases had previous shunts that failed due to poor sonographic visualization during initial placement, cyst septations, shunt obstruction, or dislodgment. Needle fetal thoracoscopy was used to disrupt cyst walls and septations, clear hematoma, and confirm the optimal initial position of the shunt. In this series, 1 severe CPAM patient with a short cervix developed preterm labor postoperatively resulting in neonatal demise. The remaining 4 patients experienced resolution of hydrops and progressed to successful delivery with excellent neonatal outcomes. Conclusion: Needle fetal thoracoscopy is a procedure that may be selectively deployed in challenging thoracoamniotic shunt cases impacted by recurrent failure, poor sonographic windows, and challenging fetal positioning.

Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges. Very few cases of ex-utero-intrapartum-treatment (EXIT) procedures for twin pregnancy have been reported and only in dichorionic pregnancies. We report a singular methodology for EXIT-to-airway procedures in two pregnancies involving monochorionic and dichorionic twins.Two cases of EXIT-to-airway in twin pregnancies were performed in 2018 and 2019 at a regional fetal treatment center. Case 1 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. Case 2 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with preterm labor at 36 weeks. In each case, the polyhydramnios caused the affected twin's amniotic sac to be the presenting sac for the surgical approach. Bronchoscopy and successful intubation was completed after 22 and 10 minutes of uteroplacental bypass, respectively. The bystander twins were delivered second without intubation and resuscitated without perinatal distress.EXIT-to-airway appears to be a reasonable option for twins including monochorionic pregnancies, via delivery of the affected twin first followed by delivery of the bystander twin. Thoughtful preparation and counseling by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies even in an emergent setting.

Background: Selective fetoscopic laser photocoagulation (SFLP) is the preferred intervention for stage II-IV twin-twin transfusion syndrome (TTTS); however, there is no consensus on whether SFLP or expectant management (EM) is the preferred strategy to manage Quintero stage I TTTS. Objective: The objective of this study is to estimate whether SFLP or EM is the cost-effective strategy for management of Quintero stage I TTTS. Study Design: A decision-analysis (DA) model compared SFLP to EM for 1,000 pregnant people with monochorionic-diamniotic twins affected by stage I TTTS. All subjects were assumed to be appropriate candidates for either SFLP or EM. Probabilities, costs, and utilities were derived from the literature. The DA was conducted from a healthcare payor perspective, and the analytic horizon was over the course of an offspring’s lifetime, with primary outcomes of survivorship (i.e., no intrauterine fetal demise or neonatal death) and long-term neurodevelopmental impairment. The model incorporated Markov processes with 4-week cycles throughout pregnancy. Incremental cost-effectiveness ratios (ICER) for each strategy were calculated and compared to estimate marginal cost effectiveness. An ICER of USD 100,000 per quality-adjusted life year was used to define the cost-effectiveness threshold. One-way sensitivity and Monte Carlo analyses (MCA), as well as microsimulations, were performed. Results: For base-case estimates, SFLP was found to be cost-effective compared to EM in the management of stage I TTTS. In one-way sensitivity analysis, varying each variable along pre-specified ranges did not result in changes in the conclusion. MCA projects SFLP as the cost-effective strategy in 100% of runs. Conclusions: With base-case estimates, SFLP is estimated to be the cost-effective strategy for the treatment of Quintero stage I TTTS when compared with EM. This remained true across a wide range of inputs.

Objective: Liver herniation is a known risk factor for increased severity in CDH and is associated with clinically significant pulmonary hypoplasia and pulmonary hypertension. Better studies are needed to understand the growth of the herniated liver compared to the liver that remains in the abdomen and how this liver growth then affects lung development. Serial hi-resolution fetal MRI enables characterization of liver growth throughout gestation and examination of macroscopic features that may regulate liver growth. Here, we hypothesized that the nature of liver herniation affects liver growth and, in turn, affects lung growth.Methods: Clinical data were retrospectively collected from consecutive cases of prenatally diagnosed isolated left-sided or right-sided CDH from June 2006 to August 2021. Only those cases with MRI lung volumetry for both mid-gestation and late-gestation time points were recruited for analysis. Cases with fetal chromosomal abnormalities and other major structural abnormalities were excluded. Fractional liver volume and liver growth was indexed to estimated fetal weight and compared to lung growth.Results: Data was collected from 28 fetuses with a left liver-down CDH (LLD), 37 left liver-up CDH (LLU) and 9 right liver-up CDH (RLU). Overall, RLU fetuses had greater overall and fractional (intra-thoracic vs. intra-abdominal) liver growth when compared to LLD and LLU fetuses. Additionally, intra-thoracic liver growth was consistently slower than intra-abdominal liver growth for either right- or left-sided CDH. When the liver was not herniated, a positive correlation was seen between liver growth and lung growth. However, when the liver was herniated above the diaphragm, this positive correlation was lost.Conclusion: Right-sided CDH fetuses exhibit greater liver growth compared to left-sided CDH. Liver herniation disrupts the normal positive correlation between liver and lung growth that is seen when the liver is entirely within the abdomen.

Purpose of Review In utero hematopoietic cellular transplantation (IUHCT) is a promising intervention for the non-toxic treatment of congenital disease that hinges on the assumption of fetal immunologic immaturity and an inability to reject a hematopoietic allograft. However, clinical IUCHT has failed except in cases where the fetus is severely immunocompromised. The current review examines recent studies of engraftment barriers stemming from either the fetal or maternal immune system. Recent Findings New reports have illuminated roles for maternal humoral and cellular immunity and fetal innate cellular immunity in the resistance to allogeneic IUHCT. These experimental findings have inspired new approaches to overcome these barriers. Despite these advances, postulates regarding a maternal immune barrier to IUHCT provide an inadequate explanation for the well-documented clinical success only in the treatment of fetal immunodeficiency with normal maternal immunity. Summary Characterization of the maternal and fetal immune response to allogeneic IUHCT provides new insight into the complexity of prenatal tolerance. Future work in this area should aim to provide a unifying explanation for the observed patterns of success and failure with clinical IUHCT.