Twelve-month Natural History Study of Centrosomal Protein 290 (CEP290)-associated Inherited Retinal Degeneration.

Purpose: To define the clinical characteristics of centrosomal protein 290 (CEP290)-associated inherited retinal degeneration (IRD) and determine which assessments may provide reliable endpoints in future interventional trials. Design: Participants in this natural history study were enrolled into 2 best-corrected visual acuity (BCVA) cohorts: light perception to > 1.0 logarithm of the minimum angle of resolution (logMAR) and 1.0 logMAR to 0.4 logMAR. Each comprised 4 age cohorts (3-5, 6-11, 12-17, and ≥ 18 years). Participants: Patients with CEP290-associated IRD caused by the intron 26 c.2991+1655A>G mutation and BCVA ranging from light perception to 0.4 logMAR. Methods: Best-corrected visual acuity, full-field stimulus threshold (FST) sensitivity, Ora-Visual Navigation Challenge (Ora-VNC) composite score, and OCT-outer nuclear layer (OCT-ONL) average thickness were assessed at screening, baseline, 3 months, 6 months, and 12 months. Main Outcome Measures: Best-corrected visual acuity, FST sensitivity, Ora-VNC composite score, and OCT-ONL average thickness. Results: Twenty-six participants were included in this analysis. Nineteen were female. All participants were White and 4 reported Hispanic ethnicity. At screening, 13 of 16 adult and 9 of 10 pediatric participants had BCVA > 1.0 logMAR. Baseline BCVA was variable (median [range] = 2.0 [0.5, 3.9] logMAR) and was uncorrelated with age, as were VNC composite score, FST sensitivity, and OCT-ONL average thickness. Mean (95% confidence interval [CI]) test-retest variability was -0.04 (-0.09, 0.01) logMAR for BCVA (n = 25); 0.6 (-0.1, 1.3) for VNC composite score (n = 18); and 0.10 (-0.07, 0.27) log cd.s/m2 for red FST (n = 14). A greater than expected test-retest variability (5 [0, 10] µm, n = 14) was observed for OCT-ONL average thickness as nystagmus impacted ability to repeat measures at the same retinal location. Functional assessments were stable over 12 months. Mean (95% CI) change from baseline was 0.06 (-0.17, 0.29) logMAR for BCVA (n = 23); -0.1 (-1.2, 1.0) for VNC composite score (n = 21); and -0.15 (-0.43, 0.14) log cd.s/m2 for red FST (n = 16). Conclusions: Vision was stable over 12 months. Best-corrected visual acuity, FST, and VNC composite score are potentially viable endpoints for future studies in CEP290-associated IRD. Repeatability of OCT measures poses challenges for quantifying anatomical changes in this population. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Gene Editing for CEP290-Associated Retinal Degeneration.

BACKGROUND: CEP290-associated inherited retinal degeneration causes severe early-onset vision loss due to pathogenic variants in CEP290. EDIT-101 is a clustered regularly interspaced short palindromic repeats (CRISPR)-CRISPR-associated protein 9 (Cas9) gene-editing complex designed to treat inherited retinal degeneration caused by a specific damaging variant in intron 26 of CEP290 (IVS26 variant). METHODS: We performed a phase 1-2, open-label, single-ascending-dose study in which persons 3 years of age or older with CEP290-associated inherited retinal degeneration caused by a homozygous or compound heterozygous IVS26 variant received a subretinal injection of EDIT-101 in the worse (study) eye. The primary outcome was safety, which included adverse events and dose-limiting toxic effects. Key secondary efficacy outcomes were the change from baseline in the best corrected visual acuity, the retinal sensitivity detected with the use of full-field stimulus testing (FST), the score on the Ora-Visual Navigation Challenge mobility test, and the vision-related quality-of-life score on the National Eye Institute Visual Function Questionnaire-25 (in adults) or the Children's Visual Function Questionnaire (in children). RESULTS: EDIT-101 was injected in 12 adults 17 to 63 years of age (median, 37 years) at a low dose (in 2 participants), an intermediate dose (in 5), or a high dose (in 5) and in 2 children 9 and 14 years of age at the intermediate dose. At baseline, the median best corrected visual acuity in the study eye was 2.4 log10 of the minimum angle of resolution (range, 3.9 to 0.6). No serious adverse events related to the treatment or procedure and no dose-limiting toxic effects were recorded. Six participants had a meaningful improvement from baseline in cone-mediated vision as assessed with the use of FST, of whom 5 had improvement in at least one other key secondary outcome. Nine participants (64%) had a meaningful improvement from baseline in the best corrected visual acuity, the sensitivity to red light as measured with FST, or the score on the mobility test. Six participants had a meaningful improvement from baseline in the vision-related quality-of-life score. CONCLUSIONS: The safety profile and improvements in photoreceptor function after EDIT-101 treatment in this small phase 1-2 study support further research of in vivo CRISPR-Cas9 gene editing to treat inherited retinal degenerations due to the IVS26 variant of CEP290 and other genetic causes. (Funded by Editas Medicine and others; BRILLIANCE ClinicalTrials.gov number, NCT03872479.).

Racial differences in the care of pediatric sagittal craniosynostosis: a single-institution cohort study affecting state Medicaid policy.

OBJECTIVE: Although research has shown the cost-effectiveness of endoscopic versus open repair of sagittal synostosis, few studies have shown how race, insurance status, and area deprivation impact care for these patients. The authors analyzed data from children evaluated for sagittal synostosis at a single institution to assess how socioeconomic factors, race, and insurance status affect the surgical treatment of this population. They hypothesized that race and indicators of disadvantage negatively impact workup and surgical timing for craniosynostosis surgery. METHODS: Medical records of patients treated for sagittal synostosis between 2010 and 2019 were reviewed. Area deprivation index (ADI) and rural-urban commuting area codes, as well as median income by zip code, were used to measure neighborhood disadvantage. Black and White patients were compared as well as patients using Medicaid versus private insurance. RESULTS: Fifty patients were prospectively included in the study. Thirty-one underwent open repair; 19 had endoscopic repair. All 8 (100%) Black patients had open repair, compared to 54.8% of White patients (p = 0.018). Black patients were more likely to use Medicaid compared to White patients (75.0% vs 28.6%, p = 0.019). White patients were younger at surgery (5.5 vs 10.0 months, p = 0.001), and Black patients had longer surgeries (147.5 minutes vs 110.0 minutes, p = 0.021). The median household income by zip code was similar for the two groups. Black patients were generally from areas of greater disadvantage compared to White patients, based on both state and national ADI scores (state: 7.5 vs 4.0, p = 0.013; national: 83.5 vs 60.0, p = 0.013). All (94.7%) but 1 patient undergoing endoscopic repair used private insurance compared to 14 (45.2%) patients in the open repair group (p = 0.001). Patients using Medicaid were from areas of greater disadvantage compared to those using private insurance by both state and national ADI scores (state: 6.0 vs 3.0, p = 0.001; national: 75.0 vs 52.0, p = 0.001). CONCLUSIONS: Because Medicaid in the geographic region of this study did not cover helmeting after endoscopic repair of sagittal synostosis, these patients usually had open repair, resulting in significant racial and socioeconomic disparities in treatment of sagittal synostosis. This research has led to a change in Alabama Medicaid policy to now cover the cost of postoperative helmeting.

A Systematic Review of Microsurgical Pedicle Lengthening in Free Tissue Transfer.

INTRODUCTION: Traumatic injuries that require free tissue flaps for reconstruction may require vascular pedicle extension between the flap and recipient vessels to form a clear anastomosis. Currently, a variety of techniques are used, each with their own potential benefits and harms. In addition, reports in the literature conflict on the reliability of pedicle extensions of vessels in free flap (FF) surgery. The objective of this study is to systematically assess the available literature about outcomes of pedicle extensions in FF reconstruction. METHODS: A comprehensive search was performed for relevant studies published up to January 2020. Study quality was assessed using the Cochrane Collaboration risk of bias assessment tool and a set of predetermined parameters was extracted by 2 investigators independently for further analysis. The literature review yielded 49 studies investigating pedicled extension of FF. Studies meeting inclusion criteria underwent data extraction focusing on demographics, conduit type, microsurgical technique, and postoperative outcomes. RESULTS: The search yielded 22 retrospective studies totaling 855 procedures from 2007 to 2018 in which 159 complications (17.1%) were reported in patients aged between 39 and 78 years. Overall heterogeneity of articles included in this study was high. Free flap failure and thrombosis were the 2 most prevalent major complications noted: vein graft extension technique had the highest rate of flap failure (11%) in comparison with the arterial graft (9%) and arteriovenous loops (8%). Arteriovenous loops had a rate of thrombosis of 5% versus 6% in arterial grafts and 8% in venous grafts. Bone flaps maintained the highest overall complication rates per tissue type at 21%. The overall success rate of pedicle extensions in FFs was 91%. Arteriovenous loop extension resulted in a 63% decrease in the odds of vascular thrombosis and a 27% decrease in the odds of FF failure when compared with venous graft extensions (P < 0.05). Arterial graft extension resulted in a 25% decrease in the odds of venous thrombosis and a 19% decrease in the odds of FF failure when compared with venous graft extensions (P < 0.05). CONCLUSIONS: This systematic review strongly suggests that pedicle extensions of the FF in a high-risk complex setting are a practical and effective option. There may be a benefit to using arterial versus venous conduits, although further examination is warranted given the small number of reconstructions reported in the literature.

Do Socioeconomic Status and Race Impact the Safety and Efficacy of Breast Reconstruction?

INTRODUCTION: Immediate breast reconstruction after mastectomy has increased in recent years when compared with delayed reconstruction. Despite this encouraging trend, racial and socioeconomic disparities in the receipt of postmastectomy breast reconstruction have been well documented. We sought to assess the effect of race, socioeconomic status, and patient comorbidities on muscle sparing transverse rectus abdominis myocutaneous outcomes at our safety net hospital institution in the southeast. METHODS: The database of a tertiary referral center was queried for patients who received free transverse rectus abdominis myocutaneous flaps for immediate reconstruction after mastectomy meeting inclusion criteria from 2006 to 2020. Patient demographics and outcomes were compared based on socioeconomic status. The primary outcome (reconstructive success) was defined as breast reconstruction without flap loss. Statistical analysis included analysis of variance and χ2 tests were appropriate using Rstudio. RESULTS: Three-hundred fourteen patients were included in the study, with 76% White, 16% Black, and 8% other. Overall complication rate at our institution was 17% and reconstructive success was 94%. Non-White race, older age at time of breast cancer diagnosis, higher body mass index, and presence of comorbid conditions including current smoking and hypertension were all associated with low socioeconomic status. Despite this, surgical complication rates were not predicted by non-White race, older age, or presence of diabetes mellitus. When analyzing major and minor complications based on radiation received or reconstructive success, there was no significant difference regardless of radiation treatment with the group overall achieving a 94% success rate (P = 0.229). CONCLUSIONS: This study aimed to characterize the impact of socioeconomic status and race/ethnic status of patients on breast reconstruction outcomes at an institution in the South. We found that despite the greater morbidity in low income and ethnic/minority patients that when treated by a comprehensive safety net institution, they had excellent reconstructive outcomes due to low complications and minimal reoperations.

Pediatric Cranioplasty Patients With Hostile Reconstructive Environments: Split Calvarial Versus Prosthetic Implant.

INTRODUCTION: Pediatric cranial defects can be preceded by prior infection, radiation therapy, failed prior cranioplasty, or cerebrospinal fluid leak, leading to a complex reconstructive environment. The primary aim of this study was to investigate differences in outcomes between pediatric patients with hostile reconstructive environments who received split-calvarial autologous grafts as opposed to prosthetic grafts in cranioplasty. METHODS: We performed an institutional review board-approved retrospective chart review of 51 patients younger than 18 years who underwent cranioplasty with a hostile setting between 1998 and 2020. Patients were then stratified into prosthetic (45%) and autologous groups (54%). The primary outcome measured was postoperative complication, defined as requirement of a subsequent surgery or revision. RESULTS: Overall, there were no significant differences in age, sex, type of hostile setting, etiology of cranial defect, or side of the cranial defect between the 2 groups. Complication rate among the 2 graft groups was 18%. However, there were no significant differences in complications, defined as infection, failure or resorption of the graft, wound breakdown or necrosis, resulting bone defect, or hematoma, between the 2 populations. There was a significant difference in etiology between patients with complications, with patients who required a cranioplasty due to previous hemicraniectomy being nearly 5 times as likely to face a complication ( P = 0.045). CONCLUSIONS: In our study, there was no significant difference observed in complications between prosthetic and split-thickness autologous grafts in pediatric patients with hostile settings. It does, however, seem that patients who had a previous hemicraniectomy are more likely to face complications as a result of cranioplasty.

Repair of Tracheal Dehiscence After Heart-Lung Transplant Complicated by SARS-CoV-2 Infection.

Airway complications are a major cause of morbidity after thoracic transplantation. Airway ischemia, necrosis, and tracheobronchial anastomotic dehiscence are associated with early mortality. We describe a case of tracheal anastomotic dehiscence after en bloc heart-lung transplant complicated by severe acute respiratory syndrome coronavirus 2 infection. Timely surgical management and reconstruction with a bovine pericardial patch and double muscle flap were performed. After 8 months of follow-up, there are no airway complications and normalized allograft function.

The Safety of Fat Grafting: An Institutional Retrospective Review.

BACKGROUND: Autologous fat grafting is a popular technique for volume replacement in the breast and face. The efficacy, safety, and complication rate of this technique at the division of plastic surgery at the University of Alabama at Birmingham will be described in this review. METHODS: An institutional review board-approved retrospective review of patients undergoing fat grafting procedures from January 2015 to July 2018 was performed. Records were reviewed for fat graft recipient site, donor site, amount grafted, and complications. Continuous variables were compared using either a t test or one-way analysis of variance test. Categorical data were compared using χ2 test. A P value of 0.05 or less was considered statistically significant for all comparisons. RESULTS: A total of 396 patients who underwent fat grafting procedures of the face and body from January 2015 through July 2018 met inclusion criteria. Average amount of fat grafted for all grafts was 124.4 +/- 6.74 grams. Two hundred fifty of the grafts (62.7%) involved the bilateral breasts with an average of 140.6 +/- 93.97 g used, 70 per side. Of the 396 patients, 110 (27.8%) experienced complications. Forty three of the complications (10.9%) were considered to be major, which included hematomas/seromas, fat necrosis, dermatitis/cellulitis, and infection. No statistical differences were seen among recipient site complication rate. Types of minor complications were statistically significant per recipient sites with bilateral breasts more likely to experience asymmetry than the other recipient sites (20% for bilateral breasts vs 16% overall, P < 0.05). Fifty nine of the 110 patients (53.6%) had the complications reported to be resolved. CONCLUSIONS: Fat grafting is a reliable method for volumization of the breasts and face. Minor complications were not infrequent in this case series; however, no life-threatening complications were observed. Continued work needs to be done to use fat grafting beyond traditional measures.

Comorbidity Trends in Patients Requiring Sternectomy and Reconstruction: Updated Data Analysis From 2005 to 2020.

INTRODUCTION: Comorbidity trends after median sternectomy were studied at our institution by Vasconze et al (Comorbidity trends in patients requiring sternectomy and reconstruction. Ann Plast Surg. 2005;54:5). Although techniques for sternal reconstruction have remained unchanged, the patient population has become more complex in recent years. This study offers insight into changing trends in this patient population. METHODS: A retrospective review was performed of patients who underwent median sternectomy followed by flap reconstruction at out institution between 2005 and 2020. Comorbidities, reconstruction method, average laboratory values, and complications were analyzed. RESULTS: A total of 105 patients were identified. Comorbidities noted were diabetes (27%), immunosuppression (16%), hypertension (58%), renal insufficiency (23%), chronic obstructive pulmonary disease (16%), and tobacco utilization (24%). The most common reconstruction methods were omentum (45%) or pectoralis major flaps (34%). Thirty-day mortality rates were 10%, and presence of at least 1 complication was 34% (hematoma, seroma, osteomyelitis, dehiscence, wound infection, flap failure, and graft exposure). Univariate analysis demonstrated that sex (P = 0.048), renal insufficiency, surgical site complication, wound dehiscence, and flap failure (P < 0.05) had statistically significant associations with mortality. In addition, body mass index, creatinine, and albumin had a significant univariate association with mortality (P < 0.05). CONCLUSIONS: Similar to the original study, there is an association between renal insufficiency and mortality. However, the mortality rate is decreased to 10%, likely because of improved medical management of patients with increasing comorbidities (80% with greater than one comorbidity). This has led to the increased use of omentum as a first-line option. Subsequent wound dehiscence and flap failure demonstrate an association with mortality, suggesting that increasingly complex patients are requiring a method of reconstruction once used a last resort as a first-line option.

Outcomes of Cranioplasty Strategies for High-Risk Complex Cranial Defects: A 10-Year Experience.

INTRODUCTION: Although the literature contains reports of the risks and complications of calvarial vault reconstruction for acquired defects, there are few publications addressing the specific patient population who require such reconstructions in cases preceded by prior infection, radiation, massive associated soft tissue trauma, and so on. We define such clinical presentations as a hostile environment for large surface area reconstruction. Our objective is to compare the safety and efficacy of autologous bone and alloplastic reconstruction in hostile cranial defects. METHODS: An institutional review board-approved retrospective review of patients who underwent cranioplasty of a hostile site at the University of Alabama at Birmingham between January 2008 and December 2018 was performed. The patients were stratified into 3 groups based on the type of implant used: autogenous (bone), alloplastic (polyetheretherketone [PEEK], titanium, polymethyl methacrylate), or mixed (combination of bone and prosthetic). The primary outcome metric was a complication in the year after cranioplasty, identified by implant failure, necrosis, or infection. Statistical analysis included t tests and χ2 tests where appropriate using SPSS. RESULTS: There were 55 total cases in this period: 27 autogenous, 23 alloplastic, and 5 mixed. The purely autogenous group had the highest complication rate (44%), and the alloplastic group had the lowest complication rate (38%), which was not statistically different between the 3 groups (P = 0.121). When stratified by specific material used for reconstruction (27 bone, 14 PEEK, 10 titanium, and 5 polymethyl methacrylate), overall complication rate was statistically significant (P = 0.009, χ2 test), with PEEK implants having the lowest complication rate (21%). CONCLUSIONS: This analysis interestingly found that in the setting of hostile cranial defects, cranioplasties would benefit from the use of prosthetic implants instead of autologous bone grafts, not only for avoidance of donor site morbidity but also for a decrease in overall complications.

Comparison of aesthetic outcomes between open and endoscopically treated sagittal craniosynostosis.

OBJECTIVE: In the last several decades, there has been much debate regarding the ideal treatment for sagittal synostosis. The purpose of this study was to compare perioperative, anthropometric, and subjective assessments of cosmetic outcomes between open and endoscopic management of isolated sagittal synostosis. METHODS: At their routine postoperative follow-up, pediatric patients with sagittal craniosynostosis were recruited to undergo digital cranial measurement and standardized photography for objective and subjective assessments of perioperative outcomes. Age-normalized z-scores for cephalic index, head circumference, euryon-euryon diameter (Eu-Eu), and glabella-opisthocranion diameter (G-Op) were calculated for each patient. Faculty surgeons, surgical trainees, nurses, and laypersons were asked to rate the normalcy of craniofacial appearances using a 5-point Likert scale. Outcomes were compared between patients treated with endoscopic correction and those treated with open repair. RESULTS: A total of 50 patients were included in the study. Thirty-one had undergone open surgical correction, and 19 had undergone endoscopic treatment. Endoscopic repair involved significantly lower operative time, blood loss, transfusion rate, and hospital length of stay than those with open repair (p < 0.001). There was no significant difference between groups in terms of z-scores for head circumference (p = 0.22), cephalic index (p = 0.25), or Eu-Eu (p = 0.38). Endoscopic treatment was associated with a significantly lower G-Op (p = 0.009). Additionally, the average subjective rating of head shape was higher for endoscopic treatment when corrected for age, gender, and ethnicity (p = 0.02). CONCLUSIONS: The study findings suggest that patients who are treated endoscopically may have an overall more normal appearance in skull morphology and cosmesis, although these results are limited by poor reliability.

Mandibular Distraction Osteogenesis as a Primary Intervention in Infants With Pierre Robin Sequence.

BACKGROUND: Pierre Robin sequence (PRS) is a triad of congenital facial abnormalities that can present as a syndrome (syndromic PRS [sPRS]) or an isolated entity (isolated PRS [iPRS]). Patients with PRS can develop airway and feeding problems that may result in failure to thrive. Mandibular distraction osteogenesis (MDO) is a method for improving the functional issues associated with breathing and feeding. There is a Paucity of literature evaluating the outcomes of MDO between sPRS and iPRS patients. METHODS: An institutional review board-approved retrospective review of PRS patients managed by a single surgeon and treated with MDO between January 2015 and December 2019 at a tertiary referral hospital was performed. The patients were stratified into iPRS or sPRS based on gene testing. Airway outcome measures included avoidance of tracheostomy, relief of sleep apnea, and oxygen saturation improvement. Primary feeding measures included achievement of full oral feeds and growth/weight gain. Statistical analysis included t tests and χ2 tests where appropriate using SPSS. RESULTS: Over the study period, of the 29 infants with PRS, 55% identified as iPRS and 45% as sPRS. There were no significant differences in the patient characteristics, apnea-hypoxia index (22.27 ± 12.27) and laryngeal view (3 ± 0.79) pre-MDO. After MDO, 83% of the subjects achieved a positive feeding outcome and 86% achieved a positive airway outcome with no statistical significance between sPRS and iPRS (P = 0.4369). There was a statistically significant change post-MDO in apnea-hypoxia index (5.24 ± 4.50, P = 0.02) and laryngeal view (1.59 ± 1.00, P = 0.01). CONCLUSIONS: Our recent experience would lead us to believe that sPRS patients have greater morbidities and challenging clinical developments that, when properly evaluated, can be managed by MDO. There is a potential role for MDO in reducing the need for traditional surgical interventions for respiratory and feeding problems in both iPRS and sPRS patients.

Reconstructive Management of Gunshot Wounds to the Frontal Sinus: An Urban Trauma Center's Perspective.

INTRODUCTION: In the last decade, we have seen a steady increase in the incidence of frontal sinus trauma due to gunshot wounds and a decrease in motor vehicle trauma. Penetrating gunshot wounds to the frontal sinus present a unique challenge to the reconstructive surgeon because they require careful consideration of the management principles of plastic surgery. Despite previous reviews on frontal sinus trauma, there are no studies examining the management techniques of frontal sinus fractures due specifically to gunshot wounds. In this study, we aim to retrospectively evaluate the use of a variety of tissue flaps in intervention and associated outcomes. METHODS: A retrospective chart review was completed on all patients with gunshot wound(s) to the frontal sinus from January 2010 to January 2018 at a single institution. The patients were classified based on the fracture pattern (anterior vs posterior table vs both), degree of displacement, presence of nasofrontal outflow tract injury, and evidence of cerebrospinal fluid leak. Patients were then stratified according to the type of reconstruction performed (cranialization, obliteration and need for free flap) and evaluated for major and minor complications after reconstruction. RESULTS: In this study, we present outcome data from 28 cases of frontal sinus trauma due to gunshot wounds. There was a statistically significant difference (P = 0.049) in the type reconstructive strategy employed with each type of flap, with pericranial flaps primarily used in cranialization, temporal grafts were more likely to be used in obliteration, and free flaps were more likely to be used in cranialization. The overall major complication rate was 52% (P = 0.248), with the most common acute major complication was cerebrospinal fluid leak (39%) and major chronic was abscess (23.5%). CONCLUSIONS: This report explores the management of frontal sinus trauma and presents short-term outcomes of treatment for penetrating gunshot wounds at a tertiary referral center.

Microsurgical management of a brachial artery pseudoaneurysm in a 41-day-old infant.

A pseudoaneurysm of the proximal right brachial artery is rare, with most caused by penetrating or blunt trauma. We report the case of a 41-day-old patient with a large iatrogenic pseudoaneurysm of the right brachial artery that had been induced by a puncture lesion during peripherally inserted central catheter placement for treatment of Lennox-Gastaut syndrome. The patient was successfully treated with a multidisciplinary approach, that consisted of direct excision of the pseudoaneurysm, followed by microvascular direct anastomosis. The patient was discharged with no complications, and complete exclusion of the pseudoaneurysm was confirmed at the 2-year follow-up examination.

Objective Craniometric Versus Subjective Outcome Ratings in Endoscopic and Open Sagittal Synostosis Surgery.

BACKGROUND: Despite advances in surgical treatments and assessments of objective outcomes in surgery for sagittal synostosis, there is no agreement regarding the optimal assessment of postoperative outcomes. Additionally, few studies have evaluated subjective assessments of cranial morphology after surgical correction. This study sought to evaluate the utility of subjective aesthetic outcome assessment and compare these assessments to established craniometric outcomes in patients undergoing surgery for isolated sagittal synostosis. METHODS: Nineteen raters (5 parents, 4 surgeons, 5 trainees and 5 nurses) evaluated fifty patients who underwent surgical correction of isolated sagittal synostosis using standardized postoperative patient photos and a five-point Likert scale. Previously established anthropomorphic measurements were recorded postoperatively in these same patients and comparisons were made between the objective anthropomorphic and subjective outcome evaluations. RESULTS: There were no statistically significant correlations between age-controlled cephalic index, head circumference, or euryon-euryon diameter and subjective aesthetic scores. Lay persons assigned a significantly lower proportion of scores (37.9%) as middle values (2, 3, or 4) compared with faculty (64.8%). There was a statistically significant association between high scores given by surgical faculty and laypersons (P < 0.001). CONCLUSIONS: Subjective measurement of cosmetic outcome is a useful metric in surgical correction of craniosynostosis. Although no correlations were found between objective measurements and subjective aesthetic scores, cosmetic assessments by surgeons demonstrated strong correlation with lay perception, indicating that these ratings may be a good gauge of overall cosmetic outcome. When used in combination, objective and subjective measurements provide unique value to assess outcomes after surgery for craniosynostosis.

Extending the Reach of Craniofacial Free Flaps Using the Descending Branch of the Lateral Circumflex Femoral Vessels.

SUMMARY: Craniofacial free tissue transfer is sometimes complicated by insufficient pedicle length and/or paucity of recipient vessels. A saphenous vein graft can be used to reach the high-flow, large-caliber vessels of the neck, but because of the vein's taper and thick wall, there is often a mismatch. Following the principle of like-for-like, the authors prefer the descending branch of the lateral circumflex femoral vessels to achieve a more anatomical pedicle extension for free tissue transfer in complex craniofacial reconstruction. The authors' experience using the descending branch of the lateral circumflex femoral pedicle extender from 2010 to 2019 was reviewed. Indications, patient characteristics, reconstruction site, flap type, pedicle length, recipient vessels, and vascular complications were noted. The authors reviewed two strategies for implementation of the pedicle extender: in some cases, the flap was first transferred and allowed to perfuse on the descending branch of the lateral circumflex femoral vessels in the thigh, and then transferred to the recipient vessels (double-ischemia transfer); and in other cases, the flap and pedicle extender were transferred such that the flap underwent a single period of ischemia (single-ischemia transfer). The descending branch of the lateral circumflex femoral pedicle extender was used in 17 craniofacial cases. Indications included tumor, trauma, osteoradionecrosis, and congenital. Double-ischemia transfer was used in eight cases and single-ischemia transfer in nine. The longest pedicle extender in the series was 15 cm. Arterial thrombosis occurred in one case. This case series demonstrates that the descending branch of the lateral circumflex femoral pedicle extender is a viable option for complex craniofacial free tissue transfer cases. It provides ample length and excellent vessel match, following the like-for-like principle. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Pedicle flap coverage for infected ventricular assist device augmented with dissolving antibiotic beads: Creation of an antibacterial pocket.

Infectious complications following left ventricular assist device implantation can carry significant morbidity and mortality. The main tenet of treatment is source control which entails local wound care, intravenous antimicrobial therapy, surgical debridement, and at times, soft tissue flap coverage. The mode of therapy depends on the severity, etiology, and location of infection as well as the clinical status of the patient. We describe a case of a 46-year-old male who underwent left ventricular assist device placement complicated by pump thrombosis, recurrent infection, and hardware exposure who was successfully treated with a novel method of staged, soft tissue reconstruction.

Subcutaneous Furosemide in Heart Failure: Pharmacokinetic Characteristics of a Newly Buffered Solution.

Parenteral diuretics form the cornerstone of decongestion in heart failure. However, parenteral therapy routinely requires emergency room or inpatient care. A novel buffered furosemide formulation with neutral pH was developed to offer "hospital-strength" diuresis for outpatient use, including self-administration at home. Subcutaneous infusion using a biphasic delivery profile resulted in complete bioavailability (99.65%) and equivalent diuresis when compared with intravenous administration. Subcutaneous administration of buffered furosemide was well tolerated with no evidence of any drug-induced skin reactions. Subcutaneous infusion of buffered furosemide in the outpatient setting or home may help to reduce the burden of heart failure.

Deep Venous Thrombosis in Teen With Crouzon Syndrome Post-Le Fort III Osteotomy With Rigid External Distraction.

Venous thromboembolic events are rare in pediatric patients. Risk factors associated with the development of venous thromboembolic events in pediatric patients include the use of central venous catheters, hospitalization, cancer, sepsis, trauma, surgery, and congenital prothrombotic disorders.The authors present the case of a 14-year-old man with Crouzon syndrome who required Le Fort III osteotomy with rigid external distraction for significant midface hypoplasia who presented postoperatively with an extensive deep venous thrombosis. This is the first reported case of symptomatic venous thrombosis post-Le Fort III osteotomy and rigid external distraction. Although rare, surgeons should be aware of this potential complication.

The Millard Rotation-Advancement Cleft Lip Repair: 50 Years of Modification.

Since its inception in 1955, Millard's rotation-advancement repair has been one of the most popular techniques used in the care of patients with a cleft lip. Over the past half century, Millard's repair has evolved and laid the foundation for many other repair techniques that have followed in its footsteps. This publication compares Millard's rotation-advancement technique to the various repairs used today. The purpose of this article is to lend perspective as to the impact of Millard repair over the past 50 years in the treatment of cleft lip.