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.

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.

MRI findings of spinal accessory neuropathy.

AIM: To characterise the magnetic resonance imaging (MRI) appearance of patients with spinal accessory nerve (SAN) denervation. MATERIAL AND METHODS: Twelve patients who had SAN denervation on electromyography (EMG) were included. The sternocleidomastoid and trapezius muscles and the SAN were assessed using MRI. RESULTS: Trapezius muscle atrophy was seen in 11 (92%), and of those patients, T2/short tau inversion recovery (STIR) signal hyperintensity was also demonstrated in seven (58%). All three patients with prior neck surgery had scarring around the SAN, and one of these patients demonstrated a neuroma, which was confirmed surgically. CONCLUSION: Features of SAN neuropathy on MRI include atrophy and T2/STIR signal hyperintensity of the trapezius, and in patients who have had posterior triangle neck surgery, scarring may be seen around the nerve.

Sleep restriction increases the neuronal response to unhealthy food in normal-weight individuals.

CONTEXT: Sleep restriction alters responses to food. However, the underlying neural mechanisms for this effect are not well understood. OBJECTIVE: The purpose of this study was to determine whether there is a neural system that is preferentially activated in response to unhealthy compared with healthy foods. PARTICIPANTS: Twenty-five normal-weight individuals, who normally slept 7-9 h per night, completed both phases of this randomized controlled study. INTERVENTION: Each participant was tested after a period of five nights of either 4 or 9 h in bed. Functional magnetic resonance imaging (fMRI) was performed in the fasted state, presenting healthy and unhealthy food stimuli and objects in a block design. Neuronal responses to unhealthy, relative to healthy food stimuli after each sleep period were assessed and compared. RESULTS: After a period of restricted sleep, viewing unhealthy foods led to greater activation in the superior and middle temporal gyri, middle and superior frontal gyri, left inferior parietal lobule, orbitofrontal cortex, and right insula compared with healthy foods. These same stimuli presented after a period of habitual sleep did not produce marked activity patterns specific to unhealthy foods. Further, food intake during restricted sleep increased in association with a relative decrease in brain oxygenation level-dependent (BOLD) activity observed in the right insula. CONCLUSION: This inverse relationship between insula activity and food intake and enhanced activation in brain reward and food-sensitive centers in response to unhealthy foods provides a model of neuronal mechanisms relating short sleep duration to obesity.

Postprandial thermogenesis and substrate oxidation are unaffected by sleep restriction.

BACKGROUND/OBJECTIVES: The extent to which alterations in energy expenditure (EE) in response to sleep restriction contribute to the short sleep-obesity relationship is not clearly defined. Short sleep may induce changes in resting metabolic rate (RMR), thermic effect of food (TEF) and postprandial substrate oxidation. SUBJECTS/METHODS: Ten females (age and body mass index: 22-43 years and 23.4-28 kg m(-2)) completed a randomized, crossover study assessing the effects of short (4 h per night) and habitual (8 h per night) sleep duration on fasting and postprandial RMR and respiratory quotient (RQ). Measurements were taken after three nights using whole-room indirect calorimetry. The TEF was assessed over a 6-h period following consumption of a high-fat liquid meal. RESULTS: Short versus habitual sleep did not affect RMR (1.01±0.05 and 0.97±0.04 kcal min(-1); P=0.23). Fasting RQ was significantly lower after short versus habitual sleep (0.84±0.01 and 0.88±0.01; P=0.028). Postprandial EE (short: 1.13±0.04 and habitual: 1.10±0.04, P=0.09) and RQ (short: 0.88±0.01 and habitual: 0.88±0.01, P=0.50) after the high-fat meal were not different between conditions. TEF was similar between conditions (0.24±0.02 kcal min(-1) in both; P=0.98), as was the ~6-h incremental area under the curve (1.16±0.10 and 1.17±0.09 kcal min(-1) × 356 min after short and habitual sleep, respectively; P=0.92). CONCLUSIONS: Current findings observed in non-obese healthy premenopausal women do not support the hypothesis that alterations in TEF and postprandial substrate oxidation are major contributors to the higher rate of obesity observed in short sleepers. In exploring a role of sleep duration on EE, research should focus on potential alterations in physical activity to explain the increased obesity risk in short sleepers.

Staged rotation advancements provide improved nasal results compared to 1-stage repairs in patients with complete bilateral cleft lip and palate.

After an experience of 32 complete bilateral cleft patients treated with staged rotation advancement to lip repairs and McComb nasal corrections during an 8-year period, improved nasal results with columella and lobule of normal dimensions have been noted, and the author feels that this method can be recommended for general use.

Formation and stability of impurity "snakes" in tokamak plasmas.

New observations of the formation and dynamics of long-lived impurity-induced helical "snake" modes in tokamak plasmas have recently been carried out on Alcator C-Mod. The snakes form as an asymmetry in the impurity ion density that undergoes a seamless transition from a small helically displaced density to a large crescent-shaped helical structure inside q<1, with a regularly sawtoothing core. The observations show that the conditions for the formation and persistence of a snake cannot be explained by plasma pressure alone. Instead, many features arise naturally from nonlinear interactions in a 3D MHD model that separately evolves the plasma density and temperature.

Effects of magnetic shear on toroidal rotation in tokamak plasmas with lower hybrid current drive.

Application of lower hybrid (LH) current drive in tokamak plasmas can induce both co- and countercurrent directed changes in toroidal rotation, depending on the core q profile. For discharges with q(0) <1, rotation increments in the countercurrent direction are observed. If the LH-driven current is sufficient to suppress sawteeth and increase q(0) above unity, the core toroidal rotation change is in the cocurrent direction. This change in sign of the rotation increment is consistent with a change in sign of the residual stress (the divergence of which constitutes an intrinsic torque that drives the flow) through its dependence on magnetic shear.

Two extraordinarily severe cases of Treacher Collins syndrome.

Here, we report two extraordinarily severe cases of Teacher Collins syndrome. Initially, amniotic bands and plical fold disruption were considered, but downslanting eyes made us consider severe Treacher Collins syndrome. A TCOF1 mutation in exon 24 was identified in Patient 1 (c.4355_4356ins14, resulting in p.1456Thrfs*18). Patient 2, who expired on day 4, is so similar to Patient 1 that severe Treacher Collins syndrome may be inferred in this instance. Neither the TCOF1 mutation nor the well-known variability in the expression in affected families with Treacher Collins syndrome (∼40% of reported cases) can explain the severity of these cases; otherwise, we would be aware of such cases within families from time to time. We are unaware of any recent sporadic cases (∼60% of reported cases) exactly like ours either with a single exception in the case reported by Writzl et al. [2008] with a TCOF1 mutation. The case described by Otto in 1841 is spectacular. We propose several hypotheses to be considered in explaining this developmental amplification, including some promoter effect on the gene, some position effect on the gene, a polymorphism elsewhere in the gene, a point mutation elsewhere in the gene, a polymorphism in another gene, or a point mutation in another gene, such as POLR1C (which maps to 6p21.1) or POLR1D (which maps to13q12.2). We also review the etiology and pathogenesis of Treacher Collins syndrome, and discuss several other severe cases from the past.

"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.

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.

Rotation reversal bifurcation and energy confinement saturation in tokamak Ohmic L-mode plasmas.

Direction reversals of intrinsic toroidal rotation have been observed in diverted Alcator C-Mod Ohmic L-mode plasmas following electron density ramps. For low density discharges, the core rotation is directed cocurrent, and reverses to countercurrent following an increase in the density above a certain threshold. Such reversals occur together with a decrease in density fluctuations with 2 cm(-1)≤k(θ)≤11 cm(-1) and frequencies above 70 kHz. There is a strong correlation between the reversal density and the density at which the Ohmic L-mode energy confinement changes from the linear to the saturated regime.

Breast reduction with dermoglandular flaps: Tessier's "total dermo-mastopexy" and the "yin-yang technique".

The use of dermoglandular flaps in reduction mastopexy was advocated by Paul Tessier, who never published his method, but had actually almost finished the following article before his death in June 2008. Dr. Tessier is acknowledged as the "father" of craniofacial surgery, but he had interest in aesthetic surgery, and was quite proud of the technique he had developed using dermoglandular flaps in reduction mammoplasty. He had literally hundreds of techniques and methods that he had developed but which never found their way into print, both because of his enormous surgical schedule, and perhaps his self-imposed standards for anything that he published, which were almost impossibly high. The technique proposed by Dr. Gargano is similar in some ways to Dr. Tessier, it seemed good that they will be published together.

Platysma-based myocutaneous clavicular island flap for intraoral reconstruction.

The clavicular myocutaneous island flap, with circulation provided by the platysma and superficial cervical fascia, was first performed by Paul Tessier in 1970, taking his motivation from the prior experience of John Barron with subcutaneous island flaps. A manuscript written by Dr. Tessier on his experience of 120 cases using the flap (which we will refer to as the BT, or Barron-Tessier flap) has been translated and is presented, as well the experiences of Matthews and Wolfe, who learned the procedure from Dr. Tessier, and Kamerer, an ENT/Head and Neck surgeon who learned the procedure from Matthews. In aggregate, we will present our joint experience with 443 cases of the BT flap. Because of its ease and speed of harvest, reliability, and provision of thin, pliable skin, we feel that, in many instances, it is equivalent, or even superior to microsurgical free flap for reconstruction of intraoral lining defects.

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.