: To successfully treat these complex lesions, the plastic surgeon must understand the disease process, the natural history and complications, and the options for treatment.
Giant congenital melanocytic nevi (CMN) are rare, congenital, disfiguring lesions with a risk of degeneration to malignant melanoma. Giant CMN are associated with an increased risk of malignant degeneration. In a minority of cases, patients with giant CMN may have associated neurocutaneous melanosis with leptomeningeal involvement. Giant CMN of the trunk pose difficult diagnostic and reconstructive problems requiring complex multistage treatment. For high-risk cases, diagnostic evaluation in the form of neuro-imaging is an essential component of the planning phase. Although nonsurgical options for the treatment of giant CMN have been advocated, these modalities may decrease the burden of nevus cells but do not result in complete removal of these cells. The ability to monitor nevus cells that remain after nonsurgical management of giant CMN remains questionable. These nonsurgical options include dermabrasion, laser ablation, and chemical peel. In contrast, direct excision of the nevus is the mainstay of treatment of nonsurgical management of giant CMN. There are numerous surgical options to resurface the resultant cutaneous defect after excision of the nevus. The simplest of these options consists of serial excision and direct closure of the defect in stages. However, if the defect cannot be closed by direct cutaneous advancement, other options for wound resurfacing include split- or full-thickness skin graft, tissue expansion, and free tissue transfer. Tissue expansion should be viewed as a category of treatment options because expanders can be used to create an expanded full-thickness skin graft, local expanded flaps adjacent to the lesion, or expansion of a free tissue donor site. Given the diversity of reconstructive options that use tissue expansion, these techniques have evolved as the primary treatment method for giant CMN of the trunk. The authors outline an approach to the evaluation of giant CMN of the trunk, review the risks of melanoma and of neurocutaneous melanosis, describe their preferred treatment regimen, and offer a treatment algorithm for giant CMN of the trunk.
Vascular anomalies can be classified into two unique groups-hemangiomas and vascular malformations-based on their endothelial properties. The present review focuses on vascular malformations rather than hemangiomas. The authors address capillary malformations, lymphatic malformations, venous malformations, and arteriovenous malformations. Diagnostic and therapeutic modalities are discussed with relevant case examples. A MEDLINE search was performed to gather all pertinent references. The physician treating these challenging lesions should ideally use a multidisciplinary team-based approach with multispecialty experience in diagnostic and therapeutic modalities for the management of vascular malformations.
The plastic surgeon treating these challenging lesions must have a solid working knowledge of the disease's histology, its natural history and complications, and the options for treatment.
Objective Studies have shown that the majority of cleft lip and palate (CLP) children have middle ear fluid present at the time of lip repair (3-4 months). Despite hearing loss, the majority of children do not undergo ventilation tube treatment if required until the time of palate repair (9-12 months). We aimed to examine the effectiveness and potential complications of early ventilation tube placement prior to palatoplasty in infants with cleft lip and palate. Data Sources Medline (1946-2015), Embase (1980-2015), and EBM Reviews (Cochrane Central Register of Controlled). Review Methods Data sources were searched for publications that described the results of early ventilation tube placement in children with CLP prior to cleft palate repair. Two independent reviewers appraised the selected studies. Results Of 226 studies identified, 6 studies met the inclusion criteria. Early ventilation tube insertion in CLP gave similar speech and audiology outcomes to non-CLP children undergoing ventilation tube insertion and better outcomes than those children with CLP having later ventilation tube insertion at or after the time of palate closure. The main reported side effect was otorrhea, being higher for children with CLP having early ventilation tube insertion (67% vs 33%), with a reduction in otorrhea with increasing age. Larger studies with longer-term outcome reporting are required to fully address the study objectives. Conclusion Published data are limited but appear to support early insertion of ventilation tubes in children with CLP to restore middle ear function and maximize audiologic and speech outcomes.
T herapeutic advances are reliant on sound research published in reputable journals with wide readership. The goal of any research is to provide a lasting, retrievable record of the work in the form of a published article (1). Research is often first presented to a peer group in abstract form at an annual meeting; this semiformal informationsharing venue is a critical medium for the communication of new concepts, and enables the presentation of novel or confirmatory information to glean peer feedback. However, many of these presentations are based on partial results, which are modified after final data analysis or during the more rigorous peer-review process required for successful publication. Moreover, these ideas are not effectively disseminated because most surgeon members attend annual general meetings sporadically, if at all (2-4).Unfortunately, many presentations are never published. A 2007 Cochrane review of nearly 30,000 abstracts suggested that 44.5% of abstracts successfully reached publication (4). Data from transplant, orthopedic and urology literature since 2006 have yielded conversion rates of presentation to publication of 33% to 59% (3,5-10). Failure to publish is due to many factors, including inadequate time or resources, lower level study design or poor methodology, and coauthor
Background: Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and respiratory distress. There is no standard clinical classification used in the management of neonatal airway in patients with PRS. The goal of our study was to review the presentation and management of patients with PRS and formulate a clinical grading system and treatment algorithm. Methods: A 10-year retrospective review of all neonates diagnosed with PRS was performed after obtaining institutional ethics approval. Patients were identified using our cleft lip and palate program database. Inclusion criteria were 2 of the following 3 clinical features-glossoptosis, retrognathia, or airway obstruction. We collected demographic data, clinical information (coexisting airway morbidity, maxillary-mandibular discrepancy, type of intervention used, complications, and outcomes (feeding, length of stay, and airway status) during the first year of life. Results: Sixty-three patients met our inclusion criteria. Of these, 55 (87%) had cleft palate and 17 (27%) were syndromic. Forty-eight (76%) patients were managed by prone positioning. Of the 15 surgically managed patients, the initial procedure was floor of mouth release in 7, mandibular distraction osteogenesis (MDO) in 4, and tongue-lip adhesion in 4. Five patients with coexisting airway morbidity needed a second surgery; 2 had MDO and 3 tracheostomies (one patient was later decannulated). Seven (47%) of the surgically managed patients required a gastrostomy tube. Conclusion: At present, there is no consensus on neonatal airway management in infants with PRS. From our review of 63 patients with PRS, we hereby propose a simple 4-point classification system and treatment algorithm, based on clinical features. Résumé Historique : Le syndrome de Pierre-Robin (SPR) désigne une triade de micrognathie, de glossoptose et de détresse respiratoire. Aucune classification clinique standard n'est utilisée pour assurer l'ouverture des voies respiratoires chez les nouveau-nés présentant un SPR. La présenteétude visait à examiner la présentation et la prise en charge des patients ayant un SPR ainsi qu'à formuler un système de classement clinique et un algorithme de traitement. Méthodologie : Après avoir obtenu l'approbation du comité d'éthique de leurétablissement, les chercheurs ont procédé à une analyse rétrospective sur dix ans de tous les nouveau-nés ayant reçu un diagnostic de SPR. Ils ont recensé les patients dans leur base de données de fentes labiales et palatines. Deux des trois caractéristiques cliniques suivantes constituaient les critères d'inclusion : glossoptose, rétrognatie ou obstruction
Congenital midline cervical cleft (CMCC) is a rare developmental defect of the anterior neck normally characterized by an atrophic mucosal plaque with a cranial nipple-like skin tag, a short caudal sinus, and may be attached to a subcutaneous fibrous cord of variable length. Clinically, patients present at an early age with, white females being the most commonly affected population. In addition to aesthetic concerns, CMCC can prevent full extension of the neck, result in micrognathia and torticollis, predispose patients to infection, and can coexist with other clefting defects or cysts. Fewer than 50 cases have been published in the English-language literature. Herein, we report a case of CMCC that also presented with a mild contracture of the right sternohyoid muscle. The embryopathogenesis, histopathology, diagnosis, and treatment of this rare condition are also discussed.
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