Characteristics and outcomes of socioeconomically disadvantaged pregnant individuals with adult congenital heart disease presenting to a Cardio-Obstetrics Program.

BACKGROUND: Outcomes of individuals with adult congenital heart disease who are socioeconomically disadvantaged and cared for in cardio-obstetrical programs, are lacking. OBJECTIVE: This study aimed to describe the clinical characteristics, maternal pregnancy outcomes, and contraceptive uptake in individuals with adult congenital heart disease in an urban cardio-obstetrical program. STUDY DESIGN: Retrospective data were collected for individuals with adult congenital heart disease seen in the Maternal Fetal Medicine-Cardiology Joint Program at Montefiore Health System between 2015 and 2021 and compared using modified World Health Organization class I, II vs the modified World Health Organization class ≥II/III. RESULTS: Over 90% of individuals with adult congenital heart disease were pregnant at the time of referral. Modified World Health Organization class I, II (n=77, 62.4% Black or Hispanic/Latina) had a total of 94 pregnancies and modified World Health Organization class ≥II/III (n=49, 49.0% Black or Hispanic/Latina) had a total of 56 pregnancies. Over 25% of individuals in each group had a body mass index ≥30 (P=.78), and very low summary socioeconomic scores. Modified World Health Organization class ≥II/III were more likely to be anticoagulated in the first trimester than modified World Health Organization class I, II (10.7% vs 0.0%, P=.002) and throughout pregnancy (14.3% vs 3.2% P=.02). Modified World Health Organization class ≥II/III were more likely to require arterial monitoring during delivery than modified World Health Organization class I, II (14.3% vs 0.0%, P=.001) or delivery under general anesthesia (8.9% vs 1.1%, P=.03) but had a comparable frequency of cesarean delivery (35.8% vs 41.3%, P=.68). There were no in-hospital maternal deaths. There was no difference in the type of contraception recommended by modified World Health Organization class, however, modified World Health Organization class ≥II/III were more likely to receive long-acting types or permanent sterilization (35.6% vs 54.6%, P=.045). CONCLUSION: In a socioeconomically disadvantaged cohort with adult congenital heart disease from a historically marginalized community, those with modified World Health Organization class ≥II/III had more complex antepartum and intrapartum needs but similar maternal and obstetrical outcomes as modified World Health Organization class I, II. The multidisciplinary approach offered by a cardio-obstetrics program may contribute to successful outcomes in this high-risk cohort, and these data are hypothesis-generating.

"A Simplified Approach for Surgical Correction of Vertical Orbital Dystopia: A 45-year Retrospective Cohort Study."

INTRODUCTION: Vertical Orbital Dystopia (VOD) results in significant facial asymmetry, psychological distress, and poor quality of life in affected patients. The traditional approach (TA) for surgical correction has entailed a standard frontal craniotomy along with circumferential orbital osteotomy, vertical translocation of the orbit, and bone grafting to the lower maxilla. Caution has been expressed regarding its invasive transcranial nature. In this report, we describe the limited approach (LA) for simplified surgical correction of VOD, which obviates the need for a standard frontal craniotomy. METHODS: A 45-year retrospective review was conducted of all patients that underwent surgical correction of VOD, as performed by a single surgeon. Demographic details, procedural characteristics, and complications were compared between patients who were corrected by the TA and those who were corrected by the LA. Complications were defined as CSF leak, infection of the frontal bone, permanent diplopia, permanent ptosis, sudden-onset vision loss, persistent asymmetry, and surgical revision. RESULTS: Forty patients met inclusion criteria for correction of true VOD, of which 18 underwent the TA and 22 underwent the LA. Mean length of hospital stay was 5.3±2.3 days and 4.0±1.5 days for the TA and LA cohorts, respectively. Mean follow-up time was 4.9±7.5 years for the TA and 2.6±3.3 years for the LA. The only reported complications were persistent asymmetry in 2 patients in the TA cohort with one patient requiring surgical revision due to undercorrection, while the LA cohort exhibited no postoperative asymmetry or need for surgical revision. CONCLUSIONS: Both the TA and LA are effective for surgical correction of VOD. The limited craniotomy of the LA reduces exposure of intracranial structures and adequately achieves postoperative symmetry.

An Oncoplastic Approach to Primary Pediatric Pterygomaxillary Osteosarcoma.

Osteosarcomas arising within the pterygomaxillary/infratemporal fossa region are rare among the pediatric population. Survival rates are most influenced by tumor resection with negative margins, which can be dependent on surgical accessibility of the tumor site. The pterygomaxillary/infratemporal fossa location poses several challenges to safe and adequate tumor resection, including proximity of the facial nerve and great vessels and scarring associated with traditional transfacial approaches. In this article, we present the case of a 6-year-old boy with an osteosarcoma of the left pterygomaxillary/infratemporal fossa region successfully managed with an "oncoplastic" approach, incorporating the use of CAD/CAM and mixed reality technologies.

Gingivosupraperiosteoplasty following Presurgical Maxillary Orthopedics Is Associated with Normal Midface Growth in Complete Unilateral and Bilateral Cleft Patients at Mixed Dentition.

BACKGROUND: Passive orthodontic appliances and gingivosupraperiosteoplasty are adjuncts that can be used by surgeons at the time of primary cleft lip repair. These treatments, along with the surgical technique of cleft lip and palate repair, may impact midface growth. The objective of this study was to describe the authors' protocol for unilateral and bilateral cleft lip repair and to evaluate midfacial growth in a cohort of patients at mixed dentition who had undergone presurgical passive orthodontic appliance therapy and gingivosupraperiosteoplasty at the time of unilateral and bilateral cleft lip repair. METHODS: Fifteen complete unilateral and 15 complete bilateral cleft lip and palate patients underwent passive orthodontic appliance treatment and primary lip repair with gingivosupraperiosteoplasty. Lateral cephalograms were analyzed by three blinded reviewers. Mean cephalometric measurements at mixed dentition were compared to cephalometric values for noncleft patients, unilateral cleft lip and palate patients who did not undergo gingivoperiosteoplasty or presurgical treatment, and unilateral cleft lip and palate patients who underwent gingivoperiosteoplasty/nasoalveolar molding with independent samples t tests. RESULTS: Mean cephalometric values were within age-specific normal values for sella-nasion-A point, sella-nasion-B point, A point-nasion-B point, and facial axis. Eighty-seven (13/15) percent of unilateral cleft lip and palate patients and 93 percent (14/15) of bilateral cleft lip and palate patients did not exhibit skeletal class III malocclusion. There was no significant difference between cephalometric values for our patients and patients who did not receive gingivosupraperiosteoplasty or presurgical treatment or who underwent the gingivoperiosteoplasty/nasoalveolar molding protocol. CONCLUSIONS: Presurgical passive orthodontic appliances, combined with gingivosupraperiosteoplasty at the time of lip repair, leads to normal maxillary development in most patients at mixed dentition. Assessment of midface growth at skeletal maturity is required. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Early Postmarket Results with EmboTrap II Stent Retriever for Mechanical Thrombectomy: A Multicenter Experience.

BACKGROUND AND PURPOSE: EmboTrap II is a novel stent retriever with a dual-layer design and distal mesh designed for acute ischemic stroke emergent large-vessel occlusions. We present the first postmarket prospective multicenter experience with the EmboTrap II stent retriever. MATERIALS AND METHODS: A prospective registry of patients treated with EmboTrap II at 7 centers following FDA approval was maintained with baseline patient characteristics, treatment details, and clinical/radiographic follow-up. RESULTS: Seventy patients were treated with EmboTrap II (mean age, 69.9 years; 48.6% women). Intravenous thrombolysis was given in 34.3%, and emergent large-vessel occlusions were located in the ICA (n = 18), M1 (n = 38), M2 or M3 (n = 13), and basilar artery (n = 1). The 5 × 33 mm device was used in 88% of cases. TICI ≥ 2b recanalization was achieved in 95.7% (82.3% in EmboTrap II-only cases), and first-pass efficacy was achieved in 35.7%. The NIHSS score improved from a preoperative average of 16.3 to 12.1 postprocedure and to 10.5 at discharge. An average of 2.5 [SD, 1.8] passes was recorded per treatment, including non-EmboTrap attempts. Definitive treatment was performed with an alternative device (aspiration or stent retriever) in 9 cases (12.9%). Some hemorrhagic conversion was noted in 22.9% of cases, of which 4.3% were symptomatic. There were no device-related complications. CONCLUSIONS: Initial postmarket results with the EmboTrap II stent retriever are favorable and comparable with those of other commercially available stent retrievers. Compared with EmboTrap II, the first-generation EmboTrap may have a higher first-pass efficacy; however, data are limited by retrospective case analysis, incomplete clinical follow-up, and small sample size, necessitating future trials.

Surgical Management of Facial Port-Wine Stain in Sturge Weber Syndrome.

Sturge Weber Syndrome is characterized by the classic triad of a facial port-wine stain, leptomeningeal angiomatosis, and glaucoma. The resultant facial vascular anomaly can lead to soft tissue and bone irregularities, causing psychosocial distress and mental health morbidity. When severe, patients can opt for multi-staged surgical intervention by reconstructive surgeons to restore normal symmetry and improve the aesthetic appearance of the face. This study reports a case of surgical correction for severe facial vascular malformation resulting in poor outcomes due to the associated mental comorbidities seen in Sturge Weber Syndrome. A 37-year-old male with previously diagnosed Sturge Weber Syndrome presented to the outpatient craniofacial clinic for surgical evaluation of a large facial tuberous hemangioma. The patient underwent multiple operations for facial reconstruction including a staged full-thickness skin graft, facial recontouring, and extracranial correction of vertical orbital dystopia. The case was complicated by the patient's poorly controlled seizure disorder and psychosocial illness, resulting in self-mutilation of the repair and poor follow-up. Over the span of 10 years, the patient's mental illness caused him to fail numerous attempts at facial restoration and ultimately led to a poor final result. The psychosocial distress seen in patients with Sturge Weber Syndrome can adversely affect surgical outcomes. Physicians should be mindful of the possible complications that can arise in these patients and have the clinical means to address them.

Surgical Management of a Mild Case of Frontonasal Dysplasia: A Case Report and Review of Literature.

Frontonasal dysplasia (FND) is a rare congenital craniofacial cleft syndrome associated with a spectrum of midline facial bone and soft-tissue malformations. When present, the physical features of FND are often obvious and classified at birth. The resultant facial deformities have the potential to negatively influence psychosocial health and quality of life. Reconstructive surgical intervention in early childhood can serve to restore facial contour and alleviate psychological stress. In this report, a case of a 14-year-old female with previously undiagnosed mild form of FND presented for reconstructive surgery evaluation and underwent several procedures including sliding advanced genioplasty, submucosal resection of the nasal turbinates, open rhinoplasty, and bilateral transnasal medial canthopexies. The patient had subsequent nasal tip recontouring for persistent supratip fullness. The patient achieved an acceptable esthetic outcome and was satisfied with her physical appearance. This case emphasizes the subtle presentation and reconstructive surgical options of a mild case of FND that was diagnosed at a later age, unlike the more severe phenotypes of the syndrome and other common craniofacial anomalies that are usually diagnosed and treated in early childhood. Multidisciplinary craniofacial care teams should be able to correctly diagnose and implement the appropriate surgical interventions in patients with milder forms of FND.

The arrhinias: Proboscis lateralis literature review and surgical update.

Proboscis lateralis (PL) is a rare malformation, reported for the first time in 1861 by Forster in his monograph on congenital malformations of the human body. The abnormal side of the nose is represented by a tube-like rudimentary nasal structure, attached at any point along the embryonic fusion line between the anterior maxilla and the frontonasal processes. As clefts of the lip (and alveolus) are bilateral or unilateral, an arrhinia can be bilateral (total) or unilateral. In this case it is a 'hemi-arrhinia' (or heminasal agenesis. The arrhinias represent three groups of anomalies, each with different levels of clinical severity, some involving association with the labio-palatal cleft or agenesia of the premaxilla (1). In PL the nasal cavity on the affected side is replaced by a tubular appendage located off-center from the midline of the face, arising commonly from the medial aspect of the roof of the orbit (2). It is usually associated with heminasal aplasia or hypoplasia, microphthalmia, and - less commonly - with midline clefting. Associated brain and cranial vault anomalies are seen in 19% of these patients. PL is usually unilateral, with very few symmetrical/bilateral cases being reported (3). Morpho-aesthetic and psychological problems are frequent concerns for the patients and their families. In this study, the authors describe a clinical case and the chosen surgical technique, as well as reviewing the alternative techniques present in the literature.

Molecular, Morphological, and Functional Characterization of Corticotropin-Releasing Factor Receptor 1-Expressing Neurons in the Central Nucleus of the Amygdala.

The central nucleus of the amygdala (CeA) is a brain region implicated in anxiety, stress-related disorders and the reinforcing effects of drugs of abuse. Corticotropin-releasing factor (CRF, Crh) acting at cognate type 1 receptors (CRF1, Crhr1) modulates inhibitory and excitatory synaptic transmission in the CeA. Here, we used CRF1:GFP reporter mice to characterize the morphological, neurochemical and electrophysiological properties of CRF1-expressing (CRF1+) and CRF1-non-expressing (CRF1-) neurons in the CeA. We assessed these two neuronal populations for distinctions in the expression of GABAergic subpopulation markers and neuropeptides, dendritic spine density and morphology, and excitatory transmission. We observed that CeA CRF1+ neurons are GABAergic but do not segregate with calbindin (CB), calretinin (CR), parvalbumin (PV), or protein kinase C-δ (PKCδ). Among the neuropeptides analyzed, Penk and Sst had the highest percentage of co-expression with Crhr1 in both the medial and lateral CeA subdivisions. Additionally, CeA CRF1+ neurons had a lower density of dendritic spines, which was offset by a higher proportion of mature spines compared to neighboring CRF1- neurons. Accordingly, there was no difference in basal spontaneous glutamatergic transmission between the two populations. Application of CRF increased overall vesicular glutamate release onto both CRF1+ and CRF1- neurons and does not affect amplitude or kinetics of EPSCs in either population. These novel data highlight important differences in the neurochemical make-up and morphology of CRF1+ compared to CRF1- neurons, which may have important implications for the transduction of CRF signaling in the CeA.

Autogenous Bone Cranioplasty: Review of a 42-Year Experience by a Single Surgeon.

BACKGROUND: Autogenous bone is frequently espoused as the gold standard material for cranioplasty procedures, yet alloplastic cranioplasty continues to persist in the search, presumably, for a simpler technique. Although short-term outcomes can be successful using foreign materials, long-term follow-up in these patients often demonstrates increased rates of failure because of exposure or late infection. Autogenous bone grafts, however, integrate and revascularize, and are thus more resistant to infection than alloplastic materials. METHODS: This is a retrospective review of all patients that underwent reconstructive cranioplasty for full-thickness defects, as performed by the senior author (S.A.W.) between 1975 and 2018. All procedures were performed with autogenous bone. RESULTS: One hundred fifty-four patients met criteria for inclusion in the report. Cranioplasties were performed for both congenital and secondary indications. Split calvaria was used in 115 patients (74.7 percent), rib graft was used in 12 patients (7.8 percent), iliac crest graft was used in 10 patients (6.5 percent), and combinations of donor-site grafts were used in 17 patients (11.0 percent). In the entire series, none of the patients suffered from complications related to infection of either the donor site or transferred bone graft. None of the patients required secondary operations to fill in defects created by the postoperative resorption. CONCLUSIONS: Although autologous bone is widely considered the gold standard material for cranioplasty procedures, some argue against its use, mainly citing unpredictable resorption as the purported disadvantage. However, it is less susceptible to infection, and results in fewer long-term complications than alloplastic materials. There is no alloplastic material that has matched these outcomes, and thus autogenous bone should be considered as the primary option for cranioplasty procedures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Squamosal Suture Synostosis: Incidence, Associations, and Implications for Treatment.

Squamosal suture craniosynostosis is thought to be a relatively rare entity. In the authors' experience, it is underreported in imaging examinations and the existing literature. The authors sought to determine the incidence of squamosal synostosis, whether it is increasing in frequency, and its relationship with synostosis of the major calvarial sutures.Patients undergoing computed tomography imaging for suspected craniosynostosis over a 15-year period were reviewed by a plastic surgeon and pediatric neuroradiologist. Patients with synostosis of the squamosal sutures were identified and involvement of additional sutures, gender, and the presence of a known syndromic diagnosis were recorded. Patients greater than 4 years of age or those with prior craniofacial surgery were excluded.One hundred twenty-five patients met inclusion criteria, 26 of whom had squamosal suture synostosis (26/125, 20.8%). Squamosal synostosis was found in isolation in 3 patients (3/26, 11.5%), with 1 additional major suture in 10 patients (10/26, 38.5%), and ≥2 major sutures in 13 patients (13/26, 50%). Squamosal synostosis was more common in patients with a syndromic diagnosis (11/26 syndromic, 15/99 nonsyndromic, P < 0.001). Eleven of 26 patients with squamosal synostosis were identified in the radiology report (42.3%).Craniosynostosis of the squamosal suture is much more common than previously reported and can contribute to abnormal head shape in isolation, or in combination with major sutures. Squamosal suture synostosis is underdiagnosed clinically and radiologically, although insufficient evidence exists to determine if its true incidence is increasing.

Temperature gradient scale length measurement: A high accuracy application of electron cyclotron emission without calibration.

Calibration is a crucial procedure in electron temperature (Te) inference from a typical electron cyclotron emission (ECE) diagnostic on tokamaks. Although the calibration provides an important multiplying factor for an individual ECE channel, the parameter ΔTe/Te is independent of any calibration. Since an ECE channel measures the cyclotron emission for a particular flux surface, a non-perturbing change in toroidal magnetic field changes the view of that channel. Hence the calibration-free parameter is a measure of Te gradient. BT-jog technique is presented here which employs the parameter and the raw ECE signals for direct measurement of electron temperature gradient scale length.

ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis

"BACKGROUND: Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF), with nutritional status strongly associated with pulmonary function and survival. Nutritional management is therefore standard of care in CF patients. ESPEN, ESPGHAN and ECFS recommended guidelines to cover nutritional management of patients with CF. METHODS: The guidelines were developed by an international multidisciplinary working group in accordance with officially accepted standards. The GRADE system was used for determining grades of evidence and strength of recommendation. Statements were discussed, submitted to Delphi rounds, reviewed by ESPGHAN and ECFS and accepted in an online survey among ESPEN members. RESULTS: The Working Group recommends that initiation of nutritional management should begin as early as possible after diagnosis, with subsequent regular follow up and patient/family education. Exclusive breast feeding is recommended but if not possible a regular formula is to be used. Energy intake should be adapted to achieve normal weight and height for age. When indicated, pancreatic enzyme and fat soluble vitamin treatment should be introduced early and monitored regularly. Pancreatic sufficient patients should have an annual assessment including fecal pancreatic elastase measurement. Sodium supplementation is recommended and a urinary sodium:creatinine ratio should be measured, corresponding to the fractional excretion of sodium. If iron deficiency is suspected, the underlying inflammation should be addressed. Glucose tolerance testing should be introduced at 10 years of age. Bone mineral density examination should be performed from age 8-10 years. Oral nutritional supplements followed by polymeric enteral tube feeding are recommended when growth or nutritional status is impaired. Zinc supplementation may be considered according to the clinical situation. Further studies are required before essential fatty acids, anti-osteoporotic agents, growth hormone, appetite stimulants and probiotics can be recommended. CONCLUSION: Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers."

Feedback system for divertor impurity seeding based on real-time measurements of surface heat flux in the Alcator C-Mod tokamak.

Mitigation of the intense heat flux to the divertor is one of the outstanding problems in fusion energy. One technique that has shown promise is impurity seeding, i.e., the injection of low-Z gaseous impurities (typically N2 or Ne) to radiate and dissipate the power before it arrives to the divertor target plate. To this end, the Alcator C-Mod team has created a first-of-its-kind feedback system to control the injection of seed gas based on real-time surface heat flux measurements. Surface thermocouples provide real-time measurements of the surface temperature response to the plasma heat flux. The surface temperature measurements are inputted into an analog computer that "solves" the 1-D heat transport equation to deliver accurate, real-time signals of the surface heat flux. The surface heat flux signals are sent to the C-Mod digital plasma control system, which uses a proportional-integral-derivative (PID) algorithm to control the duty cycle demand to a pulse width modulated piezo valve, which in turn controls the injection of gas into the private flux region of the C-Mod divertor. This paper presents the design and implementation of this new feedback system as well as initial results using it to control divertor heat flux.