Implementation of Mandibular Distraction Osteogenesis for Patients With Pierre Robin Sequence in a Developing Country Through International Collaboration: A Paradigm for Success.

OBJECTIVE: Though it has made significant strides, Vietnam remains a resource-constrained country of 98 million people. Vietnam National Children's Hospital (VNCH) provides tertiary care to a catchment of 40 million people and is the sole national children's hospital. As such, it is one of the few referral centers in the country equipped to take care of patients diagnosed with Pierre Robin sequence (PRS) as this requires pulmonary, critical care, otolaryngology, and plastic surgery expertise. Before 2015, the only surgical options were tongue lip adhesion or tracheostomy. Only 20% of patients successfully avoided tracheostomy, mechanical ventilation, or death. From 2015 to 2019, mandibular distraction osteogenesis (MDO) was introduced by visiting international surgeons on a short-term basis. Since 2020, local surgeons at VNCH have refined their technique and widely use MDO independently. This report seeks to capitulate their experience and identify factors leading to success. METHODS: A retrospective review was conducted of patients diagnosed with PRS at VNCH from 2015 to 2022. Paper records were digitized, translated, and reviewed for inclusion criteria, including demographics, indications, hospital course, and postoperative outcomes. RESULTS: Complete records satisfying inclusion criteria were available for 53 patients with a diagnosis of PRS who underwent MDO from 2020 to 2022. From 2015 to 2019, there were 19 cases of MDO, though records were incomplete. The median age at the time of MDO was 50 ± 43 days. Forty patients (75.5%) had isolated PRS and 13 (24.5%) were syndromic. Forty-four patients (83%) had a cleft palate. Fifty-one (96.2%) of patients required preoperative supplemental oxygen or mechanical ventilation. The active distraction and consolidation phase was 4.8 ± 1.3 months. The median days to discharge after surgery was 19.0 ± 8.3 days. Median weight at birth, at the time of surgery, and at the time of device removal were 6.8 ± 1.2, 7.7 ± 1.9, and 14.8 ± 2.8 pounds, respectively. Fifty-two patients (98.1%) had obstructive sleep apnea preoperatively with an average Apnea Hypopnea Index of 25.0 ± 10.6. Post-MDO, only 4 (7.5%) had obstructive sleep apnea and the average Apnea Hypopnea Index was 5.2 ± 0.6. No patients (0) required a tracheostomy for a 100% success rate. CONCLUSIONS: The tremendous success of the implementation of MDO by local surgeons in Vietnam after its introduction by visiting international surgeons illustrates a paradigm for capacity-enhancing global surgical endeavors. Mandibular distraction osteogenesis has replaced tongue lip adhesion as the surgical treatment of choice for PRS patients at VNCH. Surgical techniques can be transferred to operating environments with basic infrastructure through collaboration and resource optimization. These results demonstrate that global surgical engagement may be scalable and repeatable with direct benefits for patients in lower-middle-income countries.

Evaluation of Helmeting Therapy Duration After Endoscopic Strip Craniectomy for Metopic and Sagittal Craniosynostosis.

INTRODUCTION: Endoscopic strip craniectomy (ESC) is a minimally invasive option for early surgical treatment of metopic (MC) and sagittal craniosynostosis (SC). For ESC, however, the postoperative duration and compliance of helmet therapy are crucial to correct MC and SC asymmetry. The purpose of this study is to assess the period of postoperative band therapy and determine differences, if any, between MC and SC. METHODS: A single-institution retrospective review was performed for patients with MC and SC who underwent ESC from November 2015 to 2019. Patients received preoperative, postoperative, and post-band 3-dimensional imaging. Factors recorded included patient sex, insurance type, number of helmets needed, age at surgery, time of first helmet, and at time of completion of helmet therapy, cephalic index, interfrontal angle, and cranial vault asymmetry index. RESULTS: Patients with SC and MC had ESC surgery at 3.3 and 3.4 months of age, respectively.Patients with SC were found to have completed banding therapy at a younger age (7.88 versus 10.0 mo), with shorter duration (4.17 versus 6.00 mo), and less number of bands (1.54 versus 2.21) than patients with MC. After regression analysis, suture type was found to be a significant predictor of total time in band therapy (P=0.039) with MC requiring a longer duration of banding therapy when compared with SC. CONCLUSIONS: Suture type directly correlates with duration of helmeting therapy for patients, with patients with MC requiring longer periods of postop helmeting and increased number of bands as compared with SC.

The Skin Necrosis Conundrum: Examining Long-term Outcomes and Risk Factors in Implant-Based Breast Reconstruction.

BACKGROUND: Mastectomy skin flap necrosis (MSFN) is a common complication following mastectomy that causes significant distress to patients and physicians and also compromises oncologic, surgical, and quality-of-life outcomes. OBJECTIVES: We sought to investigate the long-term outcomes of MSFN following implant-based reconstruction (IBR) and determine the rates and predictors of post-MSFN complications. METHODS: This was a 20-year analysis of consecutive adult (>18 years) patients who developed MSFN following mastectomy and IBR from January 2001 to January 2021. Multivariable analyses were performed to identify factors associated with post-MSFN complications. RESULTS: We identified 148 reconstructions, with a mean follow-up time of 86.6 ± 52.9 months. The mean time from reconstruction to MSFN was 13.3 ± 10.4 days, and most cases (n = 84, 56.8%) were full-thickness injuries. Most cases (63.5%) were severe, 14.9% were moderate, and 21.6% were mild. Forty-six percent (n = 68) developed a breast-related complication, with infection being the most common (24%). An independent predictor of overall complications was longer time from reconstruction to MSFN (odds ratio [OR], 1.66; P = .040). Aging was an independent predictor of overall complications (OR, 1.86; P = .038); infection (OR, 1.72; P = .005); and dehiscence (OR, 6.18; P = .037). Independent predictors of dehiscence were longer interval from reconstruction to MSFN (OR, 3.23; P = .018) and larger expander/implant size (OR, 1.49; P = .024). Independent predictors of explantation were larger expander/implant size (OR, 1.20; P = .006) and nipple-sparing mastectomy (OR, 5.61; P = .005). CONCLUSIONS: MSFN is associated with high risk of complications following IBR. Awareness of the timing and severity of MSFN and the predictors of post-MSFN complications is crucial for guiding evidence-based decision-making and improving outcomes.

Decoding the Mastectomy SKIN Score: An Evaluation of Its Predictive Performance in Immediate Breast Reconstruction.

BACKGROUND: The skin ischemia and necrosis (SKIN) score was introduced to standardize the assessment of mastectomy skin flap necrosis (MSFN) severity and the need for reoperation. We evaluated the association between the SKIN score and the long-term postoperative outcomes of MSFN after mastectomy and immediate breast reconstruction (IBR). METHODS: We conducted a retrospective cohort study of consecutive patients who developed MSFN following mastectomy and IBR from January 2001 to January 2021. Primary outcome was breast-related complications following MSFN. Secondary outcomes were 30-day readmission, operating room (OR) debridement, and reoperation. Study outcomes were correlated with the SKIN composite score. RESULTS: We identified 299 reconstructions in 273 consecutive patients with mean follow-up time of 111.8±3.9 months. Most patients had a composite SKIN score of B2 (25.0%, n=13), followed by D2 (17.3%) and C2 (15.4%). We found no significant difference in rates of OR debridement (p=0.347), 30-day readmission (p=0.167), any complication (p=0.492), or reoperation for a complication (p=0.189) based on the SKIN composite score. The composite skin score was a poor predictor of reoperation, with area under the curve (AUC) of 0.56. A subgroup analysis in patients who underwent implant-based reconstruction revealed no difference in rates of OR debridement (p=0.986), 30-day readmission (p=0.530), any complication (p=0.492), or reoperation for a complication (p=0.655) based on the SKIN composite score. CONCLUSION: The SKIN score was a poor predictor for postoperative MSFN outcomes and reoperation. An individualized risk-assessment tool that incorporates both the anatomical appearance of the breast, imaging data, and patient-level risk factors is needed.

Characterization of Regional Morphological Changes in Sagittal Craniosynostosis Following Endoscopic Strip Craniectomy With Post-operative Helmeting: Predictors for Success.

BACKGROUND: Sagittal craniosynostosis (SC) restricts craniofacial growth perpendicular to the sagittal plane resulting in scaphocephaly. The cranium grows in the anterior-posterior dimension causing disproportionate changes, which can be corrected with either cranial vault reconstruction (CVR) or endoscopic strip craniectomy (ESC) combined with post-operative helmet therapy. ESC is performed at an earlier age, and studies demonstrate benefits in risk profile and morbidity compared to CVR, with comparable results if the post-operative banding protocol is strictly upheld. We aim to identify predictors of successful outcome and, using three-dimensional (3D) imaging, assess cranial changes following ESC with post-banding therapy. MATERIALS AND METHODS: A single institution retrospective review was performed from 2015-19 for patients with SC who underwent ESC. Patients received immediate post-operative 3D photogrammetry for helmet therapy planning and implementation as well as post-therapy 3D imaging. Using these 3D images, the cephalic index (CI) for study patients was calculated before and after helmet therapy. In addition, Deformetrica™ was used to measure volume and shape changes of pre-defined anatomic skull regions (frontal, parietal, temporal, & occipital) based on the pre- and post-therapy 3D imaging results. Fourteen institutional raters evaluated the pre- and post-therapy 3D imaging in order to determine the success of the helmeting therapy. RESULTS: Twenty-one SC patients met our inclusion criteria. Using 3D photogrammetry, 14 raters at our institution rated 16 of the 21 patients to have had successful helmet therapy. There was a significant difference in CI following helmet therapy with both groups, but there was no significant difference in CI between the "successful" and "unsuccessful" groups. Furthermore, the comparative analysis demonstrated that the parietal region had a significantly higher change in mean RMS distance when compared to the frontal or occipital regions. CONCLUSION: For patients with SC, 3D photogrammetry may be able to objectively recognize nuanced findings not readily detectable when using CI alone. The greatest changes in volume were observed in the parietal region, which falls in line with treatment goals for SC. Patients deemed to have unsuccessful outcomes were found to be older at time of surgery and initiation of helmet therapy. This suggests that early diagnosis and management for SC may increase the likelihood of success.

Area Deprivation Index is Associated with Variation in Quality of Life and Psychosocial Well-being Following Breast Cancer Surgery.

BACKGROUND: Neighborhood-level factors have been shown to influence surgical outcomes through material deprivation, psychosocial mechanisms, health behaviors, and access to resources. To date, no study has examined the relationship between area-level deprivation (ADI) and post-mastectomy outcomes. METHODS: A cross-sectional survey of adult female breast cancer patients who underwent lumpectomy or mastectomy between January 2018 to June 2019 was carried out. Patient-specific characteristics and ADI information were abstracted and correlated with postoperative global- (SF-12) and condition-specific (BREAST-Q) quality-of-life performance via multivariable regression. Patients were classified into three ADI terciles: 0-39 (low deprivation), 40-59 (moderate deprivation), and 60-100 (high deprivation). RESULTS: A total of 564 consecutive patients were identified, being mostly white (75%) with mean age of 60.2 ± 12.4 years, median body mass index of 27.8 [interquartile range (IQR) 24.3-32.2) kg/m2, median Charlson Comorbidity Index of 3 (IQR 2-5), and mean ADI of 42.3 ± 25.7. African American and Hispanic patients and those with high BMI were more likely to reside in highly deprived neighborhoods (p = 0.003 and p < 0.001). In adjusted models, patients in highly deprived neighborhoods had significantly lower mean SF-12 physical (44.9 [95% CI, 43.8-46.0] versus 44.9 [95% CI, 43.7-46.1] versus 46.3 [95% CI, 45.3-47.3], p = 0.03) and BREAST-Q psychosocial well-being scores (63.5 [95% CI, 59.32-67.8] versus 69.3 [95% CI, 65.1-73.6] versus 69.7 [95% CI, 66.4-73.1], p = 0.01) relative to moderate- and low-deprivation groups. CONCLUSIONS: Patients residing in the most deprived neighborhoods were identified to have worse psychological well-being and quality-of-life. The ADI should be incorporated into the shared decision-making process and perioperative counseling to engender value-based and personalized care, especially for vulnerable populations.

Ink Rheology Regulates Stability of Bioprinted Strands.

Three-dimensional (3D) extrusion bioprinting typically requires an ad hoc trial-and-error optimization of the ink composition toward enhanced resolution. The ink solutions are solidified after leaving cone-shaped or cylindrical nozzles. The presence of ink instability not only hampers the extrusion resolution but also affects the behavior of embedded cellular components. This is a key factor in selecting (bio)inks and bioprinting design parameters for well-established desktop and handheld bioprinters. In this work, we developed an analytical solution for the process of ink deposition and compared its predictions against numerical simulations of the deposition. We estimated the onset of ink instability as a function of ink rheological properties and nozzle geometry. Our analytical results suggest that enhancing the shear-thinning behavior of the ink shortens the toe region of the deposition. Such an extrusion process is often desired, as it leads to faster depositions. However, we demonstrated that such conditions increase the possibility of lateral buckling of the strand once touching the substrate defined as instability in this study. The present study serves as a benchmark for detailed simulations of the extrusion process for optimal bioprinting.

Cardiac amyloidosis mimicking acute coronary syndrome: a case report and literature review.

BACKGROUND: Cardiac amyloidosis, a progressive cardiac disease, results from the accumulation of undegraded proteinaceous substrates in the extracellular matrix of the heart. It may present as acute coronary syndrome (ACS); therefore, a clear distinction remains challenging in clinical practice. We describe a case of cardiac amyloidosis mimicking ACS. CASE SUMMARY: A 72-year-old man experienced chest discomfort for 2 days. He gradually developed dyspnoea during the preceding month. Electrocardiogram (ECG) showed sinus rhythm with right bundle branch block and low voltage. Echocardiography revealed concentric left ventricular thickening, biatrial dilation, and preserved ejection fraction with predominantly left ventricular basal hypokinesis. Serial testing of the cardiac biomarkers showed persistently increased high-sensitive cardiac troponin T levels and normal serum creatine kinase myocardial band levels. He was diagnosed with ACS with haemodynamic stability. However, coronary angiography demonstrated non-obstructive coronary arteries. Furthermore, significant macroglossia and periorbital purpura were noticed. Laboratory investigations revealed elevated serum immunoglobulin free light chain (FLC) kappa and lambda levels with an increased FLC ratio. Histological analysis of the biopsied abdominal skin confirmed amyloidosis. DISCUSSION: Cardiac amyloidosis often presents as restrictive cardiomyopathy. The usual symptoms include dyspnoea and peripheral oedema. Chest pain may manifest rarely, leading to misdiagnosis as coronary artery disease. Some findings suggestive of cardiac amyloidosis include clinical signs such as amyloid deposits, dyspnoea, low ECG voltage, and basal-predominant hypokinesis with relative apical sparing in echocardiography. Serum FLC test and abdominal skin biopsy can confirm the diagnosis of amyloidosis when a myocardial biopsy is not feasible.

Small-Vessel Vasculopathy Due to Aberrant Autophagy in LAMP-2 Deficiency.

Lysosomal associated membrane protein 2 (LAMP2) is physiologically implicated in autophagy. A genetic LAMP2 defect causes Danon disease, which consists of two major phenotypes of myopathy and cardiomyopathy. In addition, arteriopathy may manifest on rare occasions but the pathological basis remains unknown. We encountered two Danon families that developed small-vessel vasculopathy in the coronary or cerebral arteries. To investigate the underlying mechanisms, we characterized the biological features of LAMP-2-deficient mice and cultured cells. LAMP-2-deficient mice at 9-24 months of age showed medial thickening with luminal stenosis due to proliferation of vascular smooth muscle cells (VSMC) in muscular arteries. Ultrastructural analysis of VSMC revealed various autophagic vacuoles scattered throughout the cytoplasm, suggesting impaired autophagy of long-lived metabolites and degraded organelles (i.e., mitochondria). The VSMC in Lamp2 null mice expressed more vimentin but less α-smooth muscle actin (α-SMA), indicating a switch from contractile to synthetic phenotype. Silencing of LAMP2 in cultured human brain VSMC showed the same phenotypic transition with mitochondrial fragmentation, enhanced mitochondrial respiration, and overproduction of reactive oxygen species (ROS). These findings indicate that LAMP-2 deficiency leads to arterial medial hypertrophy with the phenotypic conversion of VSMC, resulting from age-dependent accumulation of cellular waste generated by aberrant autophagy.