Refinements in Extended Alar Contour Grafts: Retrograde Approach in Conjunction with Alar Base Surgery.

SUMMARY: Alar contour grafts are an excellent adjunct in both primary and revision rhinoplasty for correction and prevention of alar rim irregularities. They are traditionally placed at the conclusion of a rhinoplasty through a vestibular incision along the underside of the alar rim. Alternatively, alar base resection provides an opportunity for precise retrograde placement of alar contour grafts. The purpose of this article is to describe an efficient, five-step technique for retrograde placement of alar contour grafts in the setting of concomitant alar base surgery.

Soft Tissue Filler for Secondary Nasal Deformities.

SUMMARY: One common indication for soft tissue filler in the nose, or "liquid rhinoplasty," is the temporary treatment of secondary nasal deformities after rhinoplasty. When applied in this manner, there are multiple aspects of the patient evaluation, timing with respect to the prior rhinoplasty and planned revision, and procedural principles and steps that must be carefully considered. Ultimately, when appropriately implemented, the procedure can help avoid unnecessary patient angst and displeasure preceding a formal revision rhinoplasty. The following article reviews the principles and application of soft tissue filler for secondary nasal deformities.

Arteriovenous Malformations of the Hand: Optimizing Outcomes with Palmar Arch Reconstruction.

BACKGROUND: Arteriovenous malformations are abnormal high-flow vascular anomalies that can greatly alter the structure of surrounding tissues. Treatment of symptomatic hand arteriovenous malformations must address the functional and aesthetic considerations of the hand. Nonsurgical treatment such as embolization continues to be plagued by complications such as digital ischemia and secondary need for amputation. In this study, the authors describe their experience treating hand arteriovenous malformations with wide resection and microsurgical reconstruction of the palmar arch. This technique aims to optimize postoperative function and minimize postoperative complications and recurrence. METHODS: Retrospective review identified five patients who underwent surgical excision of hand arteriovenous malformations performed by two of the senior authors (W.C.P. and M.M.) between 2002 and 2020. Four patients underwent reconstruction of the palmar arch, whereas one patient underwent ray amputation of the involved index finger only. One patient required concurrent free flap for soft-tissue coverage following resection. RESULTS: Mean age at the time of surgery was 34.6 years. After an average follow-up of 2.7 years, there were no postoperative complications, functional deficits, or recurrences. The patient who underwent ray amputation only was postoperatively noted to have pulsatile dorsal veins concerning for a persistent high-flow state, although no recurrent mass was seen at limited 1-year follow-up. CONCLUSIONS: Meticulous surgical excision with microvascular reconstruction is an acceptable technique for management of symptomatic hand arteriovenous malformations. Potential soft-tissue loss does not preclude aggressive resection. Wide excision with palmar arch reconstruction minimizes complications, optimizes functionality, and may prevent ischemia that could otherwise trigger recurrence. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

The Challenges of Upper Extremity Intramuscular Hemangioma: A Case Study and Systematic Review.

BACKGROUND: Intramuscular hemangiomas are rare, benign vascular tumors, constituting 0.8 percent of all hemangiomas. Upper extremity intramuscular hemangiomas pose diagnostic and therapeutic challenges because of their rarity, invasive nature, and potential for neurovascular involvement. The authors report a comprehensive systematic review of upper extremity intramuscular hemangioma management and a challenging case report. METHODS: A systematic review was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Electronic databases were used to identify articles describing upper extremity intramuscular hemangiomas through 2019. Patient demographics, clinical presentation, management, complications, and outcomes were reviewed. Based on operative timing, cases were categorized as either "primary" (excision performed at initial diagnosis) or "secondary" (excision performed after failure of conservative treatment). RESULTS: Eighteen articles encompassing 25 patients were included in the authors' systematic review. Of those, 18 underwent primary excision and seven underwent secondary excision. The majority involved the forearm or antecubital region. Complete excision, evaluated by gross examination or pathology, was reported in all primary cases and 71 percent of secondary cases. Primary excisions demonstrated smaller size of mass (19.4 cm 2 versus 165.3 cm 2 ) and superior reported functional outcomes (100 percent versus 33 percent). Complications were reported in 5 percent of the primary excisions compared to 71 percent of the secondary excisions, where one complication was a fatal hematoma. CONCLUSIONS: The literature concerning upper extremity intramuscular hemangioma is limited to mostly case reports and several case series with the potential risk of bias. With careful dissection and microsurgical technique, wide local excision followed by complete reconstruction can be successfully performed at initial diagnosis for upper extremity intramuscular hemangiomas. At early stages, smaller lesion size significantly reduces the risk of functional impairment and complications.

Nonvascularized Toe Joint Transfers to the Hand in Young Children: Technique Revisited.

BACKGROUND: Small joint reconstruction of the hand poses a significant challenge, especially in children where both functional motion and preservation of the epiphysis are desired. Auto-transplantation of whole joints is the only way to reconstruct a functional joint that maintains growth potential. Historically, nonvascularized toe-to-finger joint transfer has been criticized for high rates of avascular necrosis and joint dissolution, while vascularized transfers seemingly resulted in increased durability of the joint space and epiphysis. However, certain populations remain poor candidates for microvascular reconstruction, such as those with congenital deformities or sequelae from trauma or infection. In our case series, we demonstrate that a simplified nonvascularized surgical technique and careful patient selection can produce stable, functional joints. METHODS: Nonvascularized toe-to-finger joint transfer was performed in 3 children between the ages of 4 and 6. Reconstructed joints included 2 proximal interphalangeal (PIP) joints and 1 metacarpophalangeal (MCP) joint. Donor grafts consisted of second toe PIP joints harvested en bloc to include the epiphysis of the middle phalanx, collateral ligaments, volar plate, and a diamond-shaped island of extensor tendon with its central slip attachment. RESULTS: Follow-up ranged from 7 to 29 months. Each patient demonstrated functional improvements in joint motion and stability. Postoperative radiographs confirmed adequate joint alignment and persistence of the joint spaces. Epiphyseal closure was observed in 1 patient as early as 25 months postoperatively. CONCLUSIONS: Nonvascularized joint transfer should remain a practical consideration for small joint reconstruction of the hand in certain pediatric patients.

Update on Thumb Basal Joint Arthritis Surgery.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Comprehend anatomy and biomechanics of the normal and arthritic trapeziometacarpal joint. 2. Evaluate best evidence for diagnosis and for operative and nonoperative treatment of thumb osteoarthritis. 3. Understand treatment pitfalls of basilar joint arthritis and complication avoidance. SUMMARY: Articular and ligamentous anatomy of the trapeziometacarpal joint enables complex motions. Disability from arthritis, common at the trapeziometacarpal joint, is debilitating. Furthering the understanding of how trapeziometacarpal arthritis develops can improve treatment. The authors provide current best evidence for diagnosis and treatment of basilar joint arthritis. Pitfalls in treatment are discussed.

Extremity anomalies associated with Robinow syndrome.

Robinow syndrome, a rare genetic disorder, is characterized by skeletal dysplasia with, among other anomalies, extremity and hand anomalies. There is locus heterogeneity and both dominant and recessive inheritance. A detailed description of associated extremity and hand anomalies does not currently exist due to the rarity of this syndrome. This study seeks to document the hand anomalies present in Robinow syndrome to allow for improved rates of timely and accurate diagnosis. A focused assessment of the extremities and stature was performed using clinical examination and standard photographic images. A total of 13 patients with clinical and molecular diagnosis consistent with dominant Robinow syndrome or recessive Robinow syndrome were evaluated. All patients had limb shortening, the most common of which was mesomelia; however, rhizomelia and micromelia were also seen. These findings are relevant to clinical characterization, particularly as Robinow syndrome has classically been defined as a "mesomelic disorder." A total of eight distinct hand anomalies were identified in 12 patients with both autosomal recessive and dominant forms of Robinow syndrome. One patient did not present with any hand differences. The most common hand findings included brachydactyly, broad thumbs, and clinodactyly. A thorough understanding of the breadth of Robinow syndrome-associated extremity and hand anomalies can aid in early patient identification, improving rates of timely diagnosis and allowing for proactive management of sequelae.

A Case of an Aggressive Intraosseous Arteriovenous Malformation in the Lower Extremity: Special Considerations for Diagnosis and Management.

BACKGROUND: Arteriovenous malformations (AVMs) are rare, congenital vascular anomalies. Intraosseous AVMS most frequently arise in the head and neck, with only a small fraction occurring in the extremities. Herein, we report the findings of a combined soft tissue and intraosseous AVM involving the lower extremity of a 13-year-old child. This case highlights the necessity of an interdisciplinary approach for the effective treatment and management of these rare vascular anomalies. CASE DESCRIPTION: A 13-year-old female presented with a 4-year history of intermittent pain and swelling over her right lateral malleolus. The patient was evaluated with radiologic imaging revealing an AVM involving the right distal leg, ankle, and hindfoot with intraosseous involvement of the distal tibia and talus. She was then referred to Vascular and Plastic Surgery and an angiogram was performed demonstrating shunting from the anterior tibial, peroneal, and posterior tibial arteries to the AVM. Venous drainage was to the anterior tibial and greater saphenous veins. Three embolizations were performed over the course of 6 months. Following the third embolization, the patient was taken to the operating room where Plastic and Orthopedic Surgery performed total resection of the nidus and involved bone which was then grafted with injectable synthetic bone graft. RESULTS: Successful resection of the nidus was achieved, and the patient had an uncomplicated recovery. Within 6 months postoperatively, the patient demonstrated full range of lower extremity motion and was able to participate in age appropriate gross motor activities. Radiologic evaluation 7 months postoperatively showed no evidence of nidus recurrence. CONCLUSION: Intraosseous involvement of AVMS is rare and presents a therapeutic challenge due to its invasive potential and high incidence of recurrence. Wide local excision with bone grafting and interdisciplinary management are paramount for complete resection.

Total Ear Reconstruction Using Porous Polyethylene.

Total ear reconstruction has been approached by several techniques involving autologous graft, prosthetic implant, and alloplastic implant options. Recent studies have shown the superiority of porous polyethylene (Medpor, Porex Surgical) reconstruction over autologous reconstruction based on improved aesthetic results, earlier age of intervention, shorter surgery times, fewer number of required procedures, and a simpler postoperative recovery process. A durable and permanent option for total ear reconstruction, like Medpor, can help alleviate the cosmetic concerns that patients with auricular deformities may be burdened with on a daily basis. In this article, the authors discuss the advantages of Medpor-based ear reconstruction and discuss recent advances in the surgical techniques involved, such as harvesting a temporoparietal fascia flap and full-thickness skin graft to adequately cover the Medpor framework and decrease extrusion rates.

Syndactyly Release.

Syndactyly is one of the most common congenital hand anomalies treated by pediatric plastic surgeons. Established principles of syndactyly separation dictate the timing and order of syndactyly release, with the goals of surgery being the creation of an anatomically normal webspace, tension-free closure of soft tissue, and return of function to the fingers. Numerous surgical methods have been described, many of which involve the use of local flaps to reconstruct the commissure and full-thickness skin grafts for coverage of raw areas. Recently, reconstructive techniques without the use of skin grafts have been devised, which work well for certain indications. Special considerations are described for complete, complex, and syndromic syndactylies. Outcomes for simple syndactyly release are typically good when surgical principles are followed, whereas complex syndactyly release tends to have less-favorable outcomes and more complications.

The Duplicated Thumb: A Review.

Preaxial polydactyly is a common, often sporadic, congenital anomaly that must be addressed surgically early in life. Ideally, the surgeon seeks to accomplish three goals: construction of a thumb that is adequate in size, preservation of pinch function, and reconstruction of all components in one procedure. Although each case is unique, several classification systems attempt to describe the various types. In this article, the authors discuss the various classification systems, procedures, and outcomes after surgery for pediatric thumb duplication.

Separation of Thoraco-Omphalo-Ischiopagus Conjoined Twins: Surgical Planning, Management, and Outcomes.

BACKGROUND: Conjoined twins are a rare medical phenomenon that offers a unique challenge for medical professionals. The complex anatomy of conjoined twins dictates their survival and amenability to separation, making each case different in terms of medical management, surgical planning, and patient outcomes. Thoraco-omphalo-ischiopagus twins, joined from the thorax to the pelvis, are one of the rarest orientations recorded in the medical literature, and successful separation of this subset of conjoined twins has not been documented. This report presents a novel case of thoraco-omphalo-ischiopagus tetrapus twins who were successfully separated at 10 months of age. The preoperative planning, operative details, and postoperative course are discussed as they relate to the reconstructive effort. METHODS: Three-dimensional medical modeling was pursued early in the planning process and was used to estimate the soft-tissue requirements for reconstruction and to design custom tissue expanders. RESULTS: The reconstructive effort required postponement until respiratory status was optimized. Even with elaborate preoperative planning, primary closure of the abdomen was limited because of tissue edema and other less predictable patient factors. Delayed closure of the abdominal wall was made possible with negative-pressure wound therapy and secondary flap advancements. CONCLUSION: Preoperative coordination with necessary vendors, a multidisciplinary surgical effort, and optimal timing of the surgical intervention all contribute to the successful separation and long-term survival of thoraco-omphalo-ischiopagus conjoined twins. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Common Pediatric Skin Lesions: A Comprehensive Review of the Current Literature.

The timely diagnosis and treatment of dermatologic disease in the pediatric population can be challenging. A basic, yet comprehensive knowledge of common lesions is essential for a successful practice in plastic surgery. In this article, the authors describe vascular, cystic, and pigmented cutaneous lesions that are commonly encountered in the pediatric population. Epidemiology, pathogenesis, clinical course, and management options are discussed for each.

Common Adult Skin and Soft Tissue Lesions.

A strong foundational knowledge of dermatologic disease is crucial for a successful practice in plastic surgery. A plastic surgeon should be able to identify and appreciate common dermatologic diseases that may require medical and/or surgical evaluation and management. In this article, the authors describe epidermal/dermal, infectious, pigmented, and malignant cutaneous lesions that are commonly encountered in practice. Descriptions include the epidemiology, pathogenesis, clinical course, and management options for each type of lesion.

Bronchial artery revascularization and en bloc lung transplant in children.

BACKGROUND: Long-term success in pediatric lung transplantation is limited by infection and bronchiolitis obliterans syndrome (BOS). The bilateral sequential lung transplantation (BSLT) technique may result in airway ischemia leading to bronchial stenosis, dehiscence, or loss of small airways. En bloc lung transplant (EBLT) with bronchial artery revascularization (BAR) minimizes airway ischemia, thus promoting superior airway healing. BAR also allows for safe tracheal anastomosis, circumventing the need for bilateral bronchial anastomoses in small children. METHODS: This was a retrospective review of bilateral transplantations from 2005 to 2014. Both techniques were used in parallel. Redo and multiorgan transplants were excluded. RESULTS: There were 119 recipients comprising 88 BSLTs and 31 EBLTs. Follow-up time was 3 years (interquartile range, 1-5 years). Donor ischemic and cardiopulmonary bypass times were not different between techniques (p = 0.48 and p = 0.18, respectively). Degree of graft dysfunction and cellular rejection scores were not different (p = 0.83 and p = 0.93, respectively). There were 3 hospital deaths after BSLT and 2 after EBLT (p = 0.60). Overall survival was 61% for the BSLT group and 77% for the EBLT group (p = 0.54). Freedom from BOS was 71% in the BSLT group and 94% in the EBLT group (p = 0.08). On routine bronchoscopy, 57% BSLT and 16% EBLT patients had 1 or more airway ischemic findings (p < 0.0001). Multivariate analysis showed BSLT was associated with higher ischemic injury (relative risk, 2.86; 95 confidence interval, 1.3-6.5; p = 0.01) and non-airway complications (relative risk, 4.62; 95% confidence interval, 1.1-20.2; p = 0.04) but not airway reinterventions (p = 0.07). Airway dehiscence occurred in 3 BSLT patients. CONCLUSIONS: Pediatric EBLT with BAR can be safely performed without increasing operative or graft ischemic times. Airway ischemia and non-airway complications were significantly reduced when BAR was combined with tracheal anastomosis, potentially diminishing morbidity caused by anastomotic healing complications.

Contemporary Results of Aortic Coarctation Repair Through Left Thoracotomy.

BACKGROUND: Although surgical results for repair of coarctation of the aorta (CoA) have steadily improved, management of this condition remains controversial. The purposes of this study were to analyze the long-term outcomes of patients undergoing CoA repair through left thoracotomy and to define risk factors for reintervention. METHODS: All patients who were less than 18 years old and who underwent initial repair of CoA through left thoracotomy from 1995 to 2013 at Texas Children's Hospital (Houston, TX) were included. Patients were classified into 3 groups: 143 (42%) neonates (0 to 30 days old), 122 (36%) infants (31 days to 1 year old), and 78 (23%) older children (1 to 18 years old). Univariate and multivariate analyses were performed. RESULTS: A total of 343 patients (129 [38%] girls) with median age of 53 days (interquartile range [IQR],12 days to 9 months) and weight of 4.1 kg (IQR, 3.1 to 8.0) underwent repair with extended end-to-end anastomosis (291 patients [85%]), end-to-end anastomosis (44 patients [13%]), interposition graft (2 patients [0.6%]), or subclavian flap (6 patients [2%]). Concomitant diagnoses included genetic abnormalities (48 patients [14%]), isolated ventricular septal defects (58 patients [17%]), small left-sided structures (53 patients,16%), or other complex congenital heart disease (18 patients [5%]). Perioperative mortality was 1% (n = 4, all neonates). At a median follow-up of 6 years (7 days to 19 years), only 14 (4%) patients required reintervention (10 catheter-based procedures, 6 surgical repairs). A postoperative peak velocity of 2.5 m/s or greater was an independent risk factor for reintervention (odds ratio [OR], 4.0; 95% confidence interval [CI], 1.4 to 11.6). Within the cohort, 95 (33%) patients were hypertensive or remained on cardiac medications a median of 12 years (6 months to 19 years) after the surgical procedure. Development of perioperative hypertension was associated with higher risk of chronic hypertension or cardiac medication dependency (OR, 1.9; 95% CI, 1.1 to 3.3). CONCLUSIONS: CoA repair through left thoracotomy is associated with low rates of morbidity, mortality, and reintervention. Aortic arch obstruction should be completely relieved at the time of surgical intervention to minimize the risk of long-term recoarctation.

Reducing excess cardiac biomarker testing at an academic medical center.

BACKGROUND: Elimination of wasteful diagnostic testing will improve value for the United States health care system. OBJECTIVE: Design and implement a multimodal intervention to improve evidence-based ordering of cardiac biomarkers for the diagnosis of acute coronary syndrome (ACS). DESIGN: Interrupted times series. SUBJECTS: A total of 60,494 adult inpatient admissions from January 2009 through July 2011 (pre-intervention) and 24,341 admissions from November 2011 through October 2012 (post-intervention) at an academic medical center in Baltimore, Maryland. INTERVENTION: Multimodal intervention introduced August through October 2011 that included dissemination of an institutional guideline and changes to the computerized provider order entry system. MAIN MEASURES: The primary outcome was percentage of patients with guideline-concordant ordering of cardiac biomarkers, defined as three or fewer troponin tests and zero CK-MB tests in patients without a diagnosis of ACS. Secondary outcomes included counts of tests ordered per patient, incidence of diagnosis of ACS, and estimated change in charges for cardiac biomarker tests in the post-intervention period. KEY RESULTS: Twelve months following the intervention, we estimated that guideline-concordant ordering of cardiac biomarkers increased from 57.1 % to 95.5 %, an absolute increase of 38.4 % (95 % CI, 36.4 % to 40.4 %). We estimated that the intervention led to a 66 % reduction in the number of tests ordered, and a $1.25 million decrease in charges over the first year. At 12 months, there was an estimated absolute increase in incidence of primary diagnosis of ACS of 0.3 % (95 % CI, 0.0 % to 0.5 %) compared with the expected baseline rate. CONCLUSIONS: We implemented a multimodal intervention that significantly increased guideline-concordant ordering of cardiac biomarker testing, leading to substantial reductions in tests ordered without impacting diagnostic yield. A trial of this approach at other institutions and for other diagnostic tests is warranted and if successful, would represent a framework for eliminating wasteful diagnostic testing.

Comprehensive sequencing of PALB2 in patients with breast cancer suggests PALB2 mutations explain a subset of hereditary breast cancer.

BACKGROUND: This study sought to determine the prevalence of PALB2 mutations in a cohort referred for diagnostic testing for hereditary breast cancer. METHODS: Sanger sequencing was used to analyze the entire coding region and flanking introns of PALB2 in anonymized DNA samples from 1479 patients. Samples were stratified into a "high-risk" group, 955 samples from individuals predicted to have a high probability of carrying a mutation in BRCA1 or BRCA2 based on their personal and family history, and a "lower-risk" group consisting of 524 samples from patients with breast cancer, but fewer risk factors for being a BRCA1 or BRCA2 mutation carrier. All patients were known to be negative for deleterious sequence mutations and large rearrangements in BRCA1 and BRCA2. RESULTS: We identified 12 disease-associated PALB2 mutations among the 1479 patients (0.8%). The PALB2 mutations included 8 nonsense, 3 frameshift mutations and a splice-site mutation. The mutation prevalence for the high-risk population was 1.05% (95% CI = 0.5-1.92), whereas that for the lower-risk population was 0.38% (95% CI = 0.05-1.37). We identified 59 PALB2 variants of uncertain significance (VUS) among 57 of the 1479 patients (3.9%). CONCLUSIONS: These results suggest that PALB2 mutations occur at a frequency of ~1% in patients with hereditary breast cancer.