Melanoma in infants, caused by a gene fusion involving the anaplastic lymphoma kinase (ALK).

We describe the first cases of pediatric melanoma with ALK fusion gene arising within giant congenital melanocytic nevi. Two newborn boys presented with large pigmented nodular plaques and numerous smaller satellite nevi. Additional expansile nodules developed within both nevi and invasive melanomas were diagnosed before 10 months of age in both boys. Oncogenic driver mutations in NRAS and BRAF were absent in both cases. Instead, oncogenic ZEB2::ALK fusion genes were identified in both the nevus and melanoma developing within the nevus. In both cases, tumors were noted by ultrasound in utero, demonstrated significant nodularity at birth, and progressed to melanoma in the first year of life suggesting that congenital nevi with ALK fusion genes may behave more aggressively than those with other mutations. As ALK kinase inhibitors are effective against a range of tumors with similar ALK fusion kinases, identifying ALK fusion genes in congenital melanocytic nevi may provide an opportunity for targeted therapy.

Top Surgery and Chest Dysphoria Among Transmasculine and Nonbinary Adolescents and Young Adults.

Importance: Transgender and nonbinary (TGNB) adolescents and young adults (AYA) designated female at birth (DFAB) experience chest dysphoria, which is associated with depression and anxiety. Top surgery may be performed to treat chest dysphoria. Objective: To determine whether top surgery improves chest dysphoria, gender congruence, and body image in TGNB DFAB AYA. Design, Setting, and Participants: This is a nonrandomized prospective cohort study of patients who underwent top surgery between December 2019 and April 2021 and a matched control group who did not receive surgery. Patients completed outcomes measures preoperatively and 3 months postoperatively. This study took place across 3 institutions in a single, large metropolitan city. Patients aged 13 to 24 years who presented for gender-affirming top surgery were recruited into the treatment arm. Patients in the treatment arm were matched with individuals in the control arm based on age and duration of testosterone therapy. Exposures: Patients in the surgical cohort underwent gender-affirming mastectomy; surgical technique was at the discretion of the surgeon. Main Outcomes and Measures: Patient-reported outcomes were collected at enrollment and 3 months postoperatively or 3 months postbaseline for the control cohort. The primary outcome was the Chest Dysphoria Measure (CDM). Secondary outcomes included the Transgender Congruence Scale (TCS) and Body Image Scale (BIS). Baseline demographic and surgical variables were collected, and descriptive statistics were calculated. Inverse probability of treatment weighting (IPTW) was used to estimate the association of top surgery with outcomes. Probability of treatment was estimated using gradient-boosted machines with the following covariates: baseline outcome score, age, gender identity, race, ethnicity, insurance type, body mass index, testosterone use duration, chest binding, and parental support. Results: Overall, 81 patients were enrolled (mean [SD] age, 18.6 [2.7] years); 11 were lost to follow-up. Thirty-six surgical patients and 34 matched control patients completed the outcomes measures. Weighted absolute standardized mean differences were acceptable between groups with respect to body mass index, but were not comparable with respect to the remaining demographic variables baseline outcome measures. Surgical complications were minimal. IPTW analyses suggest an association between surgery and substantial improvements in CDM (-25.58 points; 95% CI, -29.18 to -21.98), TCS (7.78 points; 95% CI, 6.06-9.50), and BIS (-7.20 points; 95% CI, -11.68 to -2.72) scores. Conclusions and Relevance: Top surgery in TGNB DFAB AYA is associated with low complication rates. Top surgery is associated with improved chest dysphoria, gender congruence, and body image satisfaction in this age group.

Association of Chest Dysphoria With Anxiety and Depression in Transmasculine and Nonbinary Adolescents Seeking Gender-Affirming Care.

PURPOSE: The purpose of this study was to determine the existence and strength of association between chest dysphoria and mental health in transmasculine and nonbinary adolescents. METHODS: This is a cross-sectional cohort study of transmasculine and nonbinary adolescents designated female at birth between 12 and 18 years old. None had undergone prior top surgery. Patients complete the Chest Dysphoria Measure and Youth Inventory-4 (YI-4) upon presentation to our institution. Outcomes were retrospectively reviewed. The primary outcome of interest was the association between chest dysphoria and anxiety and depression symptom severity, as measured by the YI-4. RESULTS: One hundred fifty-six patients met inclusion criteria. Mean age was 15.3 years (standard deviation [SD] = 1.7). Most patients identified as transmasculine (n = 132); 18 identified as nonbinary and 6 as questioning. Mean (SD) YI-4 symptom severity scores were 10.67 (6.64) for anxiety and 11.99 (7.83) for depression. Mean (SD) Chest Dysphoria Measure composite score was 30.15 (9.95); range 2-49. Chest dysphoria was positively correlated with anxiety (r = .146; p = .002) and depression (r = .207; p < .001). In multivariate linear regression models, chest dysphoria showed a significant, positive association with anxiety and depression, after accounting for gender dysphoria, degree of appearance congruence, and social transition status. CONCLUSIONS: Chest dysphoria is associated with higher anxiety and depression in transmasculine and nonbinary adolescents designated female at birth. This association is independent of level of gender dysphoria, degree of appearance congruence, and social transition status. Treatment options aimed at alleviating chest dysphoria should be made accessible to adolescents and tailored to individual needs.

Mastectomy and Chest Masculinization in Transmasculine Minors: A Case Series and Analysis by Ethical Principles.

ABSTRACT: Mastectomy for chest masculinization is a commonly performed gender-affirming surgery in minor patients, a vulnerable population with unique developmental and psychosocial needs. We aimed to use principles of medical ethics (eg, autonomy, beneficence, nonmaleficence, and justice) as a framework to analyze preoperative clinical decision making by pediatric plastic surgeons who work with transmasculine and nonbinary adolescents designated female at birth presenting for chest masculinization. Two patients were selected for inclusion in this case series based on the senior author's (J.F.C.) clinical experience. A retrospective chart review was conducted to extract relevant psychosocial and clinical information from clinic notes and supplemental documentation (eg, letters from outside providers) available in the electronic medical record. In case A, a 17-year-old patient presented to a plastic surgery clinic with mixed parental support and restrictive insurance requirements. In case B, a 16-year-old patient presented to the emergency room after an attempt to remove his own breasts. The cases highlight the role of the plastic surgeon in advocating for adolescent autonomy and justice by facilitating shared family decision making and navigating barriers to care. In addition, we recommend multidisciplinary care, including trusted, transcompetent mental health professionals, to ensure beneficence and nonmaleficence by providing timely care when appropriate.

Retroperitoneal Approach for Ilioinguinal, Iliohypogastric, and Genitofemoral Neurectomies in the Treatment of Refractory Groin Pain After Inguinal Hernia Repair.

Inguinal hernia repair is one of the most commonly performed surgical procedures. Postoperative neuropathic groin pain is a potentially disabling complication and can be due to a neuroma of the ilioinguinal, iliohypogastric, and/or genitofemoral nerves. In this article, we present our operative technique-a retroperitoneal approach to the ipsilateral ilioinguinal, iliohypogastric, and genitofemoral nerves with neurectomy and proximal transposition of these nerves-for management of neuropathic pain. A retrospective review was performed of 12 of the patients who underwent this surgery at our institution. Three of 12 patients underwent a selective neurectomy, whereas 9 of 12 underwent a triple neurectomy; 3 of the 9 patients who underwent triple neurectomy also had a retroperitoneal peripheral nerve stimulator placed at the time of neurectomy. Pain visual analog scores (VASs) demonstrated significant improvement after neurectomy (preoperative pain VAS of 85 ± 11 vs postoperative pain VAS of 47 ± 32, P = 0.0027). Eight of 12 patients experienced partial or complete pain relief, whereas 4 of 12 patients had no pain relief. There were no major complications, and the minor complication rate was low for all patients and primarily related to peripheral nerve stimulator placement. This retroperitoneal approach to triple neurectomy for treatment of refractory groin pain in postoperative inguinal hernia repair patients is safe and effective for an otherwise devastating problem.

Single Z-Plasty Versus Double-Opposing Z-Plasty: A Cadaveric Study of Palatal Lengthening.

OBJECTIVE: The double-opposing Z-plasty is an effective method of repairing the cleft palate due to its reorientation of the palatal musculature and lengthening of the soft palate. A technique for lengthening the palate with a single oral Z-plasty has also been described. The authors hypothesize that these 2 techniques have equivalent effects on palate length. METHODS: A cadaver study was performed. Ten fresh adult cadaver heads were used.All palates were divided in the midline. In 5 specimens, a modified double-opposing Z-plasty technique was used; 5 other specimens underwent an oral Z-plasty with a straight-line repair of the nasal mucosa. In both groups, the levator veli palatini muscles were separately dissected and reapproximated with an intravelar veloplasty. The velar length, defined in this study as the distance from the posterior nasal spine to the tip of uvula, was measured before and after the surgical procedure. RESULTS: The double-opposing Z-plasty produced a mean increase of 1.0 ±â€Š0.6 cm in velar length (P = 0.023). The single Z-plasty repair resulted in a mean gain of 1.1 ±â€Š0.3 cm (P = 0.001). There was no difference in change in palate length between the 2 procedures (P = 0.941), and no difference in the percentage of soft palate lengthening (24% vs 29%, respectively; P = 0.565). CONCLUSIONS: A single oral Z -plasty provides palatal lengthening equivalent to that of a double-opposing Z-plasty procedure.

Liquid Latex Molding: A Novel Application of 3D Printing to Facilitate Flap Design.

The approach to bony craniofacial reconstruction has been significantly enhanced with the development of patient-specific, computer-aided designed and manufactured (CAD/CAM) implants. This technology, however, has not yet been widely employed for soft tissue reconstruction. While algorithmic approaches based on the size, location, and etiology of the defect are effective in most cases, a patient-specific CAD/CAM approach has benefits in complex reconstructive problems. In this study, we present a patient-specific approach to preoperative flap planning: using three-dimensional (3D) printing and liquid latex to create a flexible model of a flap, and demonstrate its application in planning a complex scalp reconstruction.

Industry Financial Relationships in Plastic Surgery: Analysis of the Sunshine Act Open Payments Database.

BACKGROUND: Limited data exist regarding industry financial relationships in plastic surgery. The Sunshine Act Open Payments Database currently represents the largest repository of these data, but is limited primarily to queries of individual providers. The purpose of this study was to analyze these data and present them in a manner that better delineates these relationships, and to compare plastic surgery with other surgical subspecialties. METHODS: A review of the Open Payments Database was performed for the period from January 1, 2014, to December 31, 2014. These data were analyzed with respect to types of payments, characteristics of plastic surgeons and companies, and comparison with other surgical subspecialties. RESULTS: A total of 49,053 payments from 274 companies were identified that were made to 4812 plastic surgeons (475 academic and 4337 private practice). The total value of payments was $17,091,077. Food and beverage represented the most common type of payment (82.2 percent). Royalties and licensing represented the highest valued type of payment (35.7 percent), but were received by only a minority of plastic surgeons (0.5 percent). No significant differences were identified between academic and private practice plastic surgeons in the value or quantity of payments. Plastic surgery (54.5 percent) exhibited the lowest prevalence of industry financial relationships compared with otolaryngology (57.9 percent), orthopedics (62.4 percent), neurosurgery (87.8 percent), and urology (63.1 percent) (p < 0.001). CONCLUSIONS: Approximately half of all plastic surgeons have industry financial relationships. The prevalence of these relationships is comparatively less than in other surgical subspecialties.

Brachial Artery Aneurysm in a 7-Month-Old Infant: Case Report and Literature Review.

Congenital upper extremity aneurysms are very rare and can be challenging to diagnose and treat. Although they can present as an isolated finding, they are often associated with other systemic conditions. We present a rare case of brachial artery aneurysm in a 7-month-old boy. The patient was evaluated with ultrasound, magnetic resonance angiography, and vein mapping before surgical reconstruction. After excision of the aneurysm, the brachial artery was reconstructed with an interposition saphenous vein graft. Because of potentially associated diagnoses and the possibility of concurrent aneurysms, this condition requires multidisciplinary management.

Remodeling Characteristics and Collagen Distributions of Biologic Scaffold Materials Biopsied From Postmastectomy Breast Reconstruction Sites.

OBJECTIVE: The study purpose was to evaluate the associations between patient characteristics and the histologic remodeling scores of acellular dermal matrices (ADMs) biopsied from breast reconstruction sites in the first attempt to generate a multivariable risk prediction model of nonconstructive remodeling. It was hypothesized that host characteristics and surgical site assessments predict the degree of graft remodeling for ADMs used during breast reconstruction. METHODS: The ADMs were biopsied from the breast reconstruction sites of n = 62 patients during a subsequent breast procedure, stained with hematoxylin-eosin, and evaluated according to a semi-quantitative scoring system for remodeling characteristics (cell types, cell infiltration, extracellular matrix deposition, scaffold degradation, fibrous encapsulation, and neovascularization) and a mean composite score. Biopsies were stained with Sirius Red and Fast Green, and analyzed to determine the collagen I:III ratio. Based on univariate analyses between subject clinical characteristics and the histologic remodeling scores, cohort variables were selected for multivariable regression models using a P value of 0.20 or less. RESULTS: The composite score model yielded 3 variables: pack-year history, corticosteroid use, and radiation timing (r pseudo = 0.81). The model for collagen I yielded 2 variables: corticosteroid use and reason for reoperation (r pseudo = 0.78). The model for collagen III yielded 1 variable: reason for reoperation (r pseudo = 0.35). CONCLUSIONS: These preliminary results constitute the first steps in generating a risk prediction model that predicts the patients and clinical circumstances most likely to experience nonconstructive remodeling of biologic grafts used to reconstruct the breast.

The impact of chemotherapy and radiation therapy on the remodeling of acellular dermal matrices in staged, prosthetic breast reconstruction.

BACKGROUND: An acellular dermal matrix will typically incorporate, in time, with the overlying mastectomy skin flap. This remodeling process may be adversely impacted in patients who require chemotherapy and radiation, which influence neovascularization and cellular proliferation. METHODS: Multiple biopsy specimens were procured from 86 women (n = 94 breasts) undergoing exchange of a tissue expander for a breast implant. These were divided by biopsy location: submuscular capsule (control) as well as superiorly, centrally, and inferiorly along the paramedian acellular dermis. Specimens were assessed for cellular infiltration, cell type, fibrous encapsulation, scaffold degradation, extracellular matrix deposition, neovascularization, mean composite remodeling score, and type I and III collagen. Patients were compared based on five oncologic treatment groups: no adjuvant therapy (untreated), neoadjuvant chemotherapy with or without radiation, and chemotherapy with or without radiation. RESULTS: Biopsy specimens were procured 45 to 1805 days after implantation and demonstrated a significant reduction in type I collagen over time. Chemotherapy adversely impacted fibrous encapsulation (p = 0.03). Chemotherapy with or without radiation adversely impacted type I collagen (p = 0.02), cellular infiltration (p < 0.01), extracellular matrix deposition (p < 0.04), and neovascularization (p < 0.01). Radiation exacerbated the adverse impact of chemotherapy for several remodeling parameters. Neoadjuvant chemotherapy also caused a reduction in type I (p = 0.01) and III collagen (p = 0.05), extracellular matrix deposition (p = 0.03), and scaffold degradation (p = 0.02). CONCLUSION: Chemotherapy and radiation therapy limit acellular dermal matrix remodeling. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Point of maximum width: a new measure for anthropometric outcomes in patients with sagittal synostosis.

The esthetic success of sagittal synostosis reconstruction is measured by cephalic index (CI). This limited measure does not fully account for the abnormal head shape in sagittal synostosis. In this retrospective study, we investigate a new objective measure, point of maximum width (PMW) of the skull from a vertex view, to determine where the head is widest for children with sagittal synostosis as compared with normal controls. Preoperative computed tomography (CT) scans of 27 children with sagittal synostosis and 14 postoperative CT scans at least 8 months after surgery were obtained. Normal CT scans were matched for age, sex, and race. Three-dimensional renderings were standardized for orientation. Mean (SE) PMW in patients with sagittal synostosis was 53% (1%) compared with 57% (1%) in controls (P < 0.001). Mean (SE) CI in patients with sagittal synostosis was 66.8% (0.8%) compared with 83.3% (1.0%) in controls (P < 0.001). The correlation between PMW and CI was weak in both controls (r2 = 0.002, P = 0.824) and uncorrected cases (r2 = 0.083, P = 0.145). After surgical correction, both CI and PMW significantly improved. Mean (SE) PMW in patients after surgical release of sagittal synostosis was 58% (1%) compared with 58% (1%) in controls (P = 0.986). The PMW is not a surrogate for CI but is a novel, valid measure of skull shape, which aids in quantifying the widest region of the skull. It is significantly more anterior in children with sagittal synostosis and exhibits a consistent posterior shift along the cranium after surgery, showing no difference compared with healthy children.

Pierre robin sequence.

Pierre Robin sequence (PRS) is classically described as a triad of micrognathia, glossoptosis, and airway obstruction. Infants frequently present at birth with a hypoplastic mandible and difficulty breathing. The smaller mandible displaces the tongue posteriorly, resulting in obstruction of the airway. Typically, a wide U-shaped cleft palate is also associated with this phenomenon. PRS is not a syndrome in itself, but rather a sequence of disorders, with one abnormality resulting in the next. However, it is related to several other craniofacial anomalies and may appear in conjunction with a syndromic diagnosis, such as velocardiofacial and Stickler syndromes. Infants with PRS should be evaluated by a multidisciplinary team to assess the anatomic findings, delineate the source of airway obstruction, and address airway and feeding issues. Positioning will resolve the airway obstruction in ~70% of cases. In the correct position, most children will also be able to feed normally. If the infant continues to show evidence of desaturation, then placement of a nasopharyngeal tube is indicated. Early feeding via a nasogastric tube may also reduce the amount of energy needed and allow for early weight gain. A proportion of PRS infants do not respond to conservative measures and will require further intervention. Prior to considering any surgical procedure, the clinician should first rule out any sources of obstruction below the base of the tongue that would necessitate a tracheostomy. The two most common procedures for treatment, tongue-lip adhesion and distraction osteogenesis of the mandible, are discussed.

Outcomes of laparoscopic paraesophageal hernia repair in elderly and high-risk patients.

BACKGROUND: This study examines the impact of age and comorbidities on complications and outcomes of laparoscopic (Lap) paraesophageal hernia (PEH) repair. METHODS: Data were collected prospectively on all patients who underwent Lap PEH repair from January 1995 through June 2005. Pre- and postoperative variables including complications were analyzed. Patients were stratified by age (Group [Gr.] 1, <65 years; Gr. 2, 65 to 74 years; Gr. 3, >/=75 years) and American Society of Anesthesiology (ASA) class (1 and 2 vs 3 and 4). Statistical analysis was performed using 1-way ANOVA, chi-square, and Fisher exact test. RESULTS: Overall, 171 patients underwent Lap PEH repair. Mean patient age was 65 +/- 15 years, mean ASA class 2.4 +/- 0.5, gender 72% female, and mean operating time 173 +/- 49 min. Patients in Gr. 3 had a significantly higher ASA class (Gr. 1, 2.3 +/- 0.6; Gr. 2, 2.5 +/- 0.5; Gr. 3, 2.6 +/- 0.5) and longer postoperative length of stay (LOS) compared with Gr. 1 (P < 0.05). Esophageal lengthening was required in 10.4% of patients in Gr. 3 versus 2.6% in Gr. 1 and 2.1% in Gr. 2 (P = 0.079). Total complication rates were 17.1% in Gr. 1, 22.4% in Gr. 2, and 27.7% in Gr. 3 (P = not significant [NS]). Most complications were minor; grade 2 or higher complications occurred in 10.5% of patients in Gr. 1, 8.3% in Gr. 2, and 8.5% in Gr. 3 (P = NS). There was 1 death (Gr. 2) on postoperative day 18 due to a myocardial infarction (mortality rate = 0.6%). Mean follow-up was 25.3 +/- 20.6 months. Postoperative symptoms of heartburn and regurgitation were similar between groups as was antisecretory medication use. Anatomic failure of the repair occurred in 23.7% of patients with adequate follow-up: 26.7% in Gr. 1, 15.4% in Gr. 2, and 27.8% in Gr. 3 (P = NS). Reoperation was performed in 1 of 32 (3.1%) failures. CONCLUSIONS: Lap PEH repair is safe in elderly and properly selected high-risk patients, although complication rates are higher than in younger patients. Most patients have a good symptomatic outcome irrespective of their age, but the anatomic recurrence rates remain a concern for all age groups.