John A. van Aalst

The Spectrum of Orofacial Clefting

LEARNING OBJECTIVES After studying this article, the participant should be able to: 1. Describe the differing types of congenital clefting defects that extend outward from the perioral region. 2. Define the sites of anatomical disruption and deformities that these types of facial clefts cause. 3. Describe the cause and incidence, if known, of orofacial clefts and their inheritance/transmission risks. BACKGROUND Clefts of the orofacial region are among the most common congenital facial defects. The clinical presentation is usually that of a lateral cleft of the lip through the philtrum with or without extension through the palatal shelves. However, atypical forms of clefts with lip involvement also occur in a variety of patterns, some of which are embryologically predictable; others are not. METHODS An overview of the embryology, cause, and incidence of this diverse and interesting group of congenital orofacial clefts is presented. RESULTS Clefts involving the lateral upper lip; median upper lip; and oblique facial, lateral facial, and median mandibular regions are reviewed. CONCLUSIONS This review of orofacial malformations describes clefting anomalies that emanate from the mouth and lips. As the causes of orofacial clefts are better understood, it is becoming clear that a complex interplay between genetic and environmental variables causes these clefts. Future study of orofacial clefts will require increasingly sophisticated methods of elucidating these subtle interactions.

Congenital and acquired pediatric breast anomalies : A review of 20 years' experience

Background: The purpose of this article was to review the senior author’s 20 years of experience in the treatment of pediatric breast abnormalities, to propose a classification system for their treatment, and to provide a synopsis of treatment options. Methods: Congenital and acquired breast anomalies were identified in a retrospective chart review (n = 66). Breast abnormalities were classified as hyperplastic (n = 44), deformational (n = 11), or hypoplastic (n = 11). Hyperplastic abnormalities included gynecomastia, hyperplasia, polythelia, polymastia, and giant fibroadenoma. Deformational abnormalities were categorized as either iatrogenic (previous thoracostomy, thoracotomy, or tumor excision) or traumatic (thermal or penetrating injuries). Hypoplastic abnormalities included athelia, unilateral and bilateral hypoplasia, tuberous breast, and Poland syndrome. Type of surgery, age at initial operation, and number of operations were recorded for all patients. Results: Hyperplastic abnormalities were treated with breast reduction techniques and required the fewest operations per patient (1.14), followed by iatrogenic breast injury (2.1 per patient). The average number of procedures required for hypoplastic abnormalities was 2.45 per patient. The highest reoperation rates were seen in patients with burn injuries to the breast and patients with Poland syndrome. Mean age at initial operation was highest in the deformational group (18.5 years) and lowest in the hyperplastic group (17.4 years). Conclusion: Classification of pediatric breast abnormalities and considerations about timing for surgery and the likely need for staged operations aid in anticipating and optimizing clinical outcomes.

Composite Tissue Engineering on Polycaprolactone Nanofiber Scaffolds

Tissue engineering has largely focused on single tissue-type reconstruction (such as bone); however, the basic unit of healing in any clinically relevant scenario is a compound tissue type (such as bone, periosteum, and skin). Nanofibers are submicron fibrils that mimic the extracellular matrix, promoting cellular adhesion, proliferation, and migration. Stem cell manipulation on nanofiber scaffolds holds significant promise for future tissue engineering. This work represents our initial efforts to create the building blocks for composite tissue reflecting the basic unit of healing. Polycaprolactone (PCL) nanofibers were electrospun using standard techniques. Human foreskin fibroblasts, murine keratinocytes, and periosteal cells (4-mm punch biopsy) harvested from children undergoing palate repair were grown in appropriate media on PCL nanofibers. Human fat-derived mesenchymal stem cells were osteoinduced on PCL nanofibers. Cell growth was assessed with fluorescent viability staining; cocultured cells were differentiated using antibodies to fibroblast- and keratinocyte-specific surface markers. Osteoinduction was assessed with Alizarin red S. PCL nanofiber scaffolds supported robust growth of fibroblasts, keratinocytes, and periosteal cells. Cocultured periosteal cells (with fibroblasts) and keratinocytes showed improved longevity of the keratinocytes, though growth of these cell types was randomly distributed throughout the scaffold. Robust osteoinduction was noted on PCL nanofibers. Composite tissue engineering using PCL nanofiber scaffolds is possible, though the major obstacles to the trilaminar construct are maintaining an appropriate interface between the tissue types and neovascularization of the composite structure.

Nanofiber applications for burn care.

Nanotechnology is a growing field of manufactured materials with sizes less than 1 &mgr;m, and it is particularly useful in the field of medicine because these applications replicate components of a cell’s in vivo environment. Nanofibers, which mimic collagen fibrils in the extracellular matrix (ECM), can be created from a host of natural and synthetic compounds and have multiple properties that may be beneficial to burn wound care. These properties include a large surface-area-to-volume ratio, high porosity, improved cell adherence, proliferation and migration, and controlled in vivo degradation rates. The large surface area of nanofiber mats allows for increased interaction with compounds and provides a mechanism for sustained release of antibiotics, analgesics, or growth factors into burn wounds; high porosity allows diffusion of nutrients and waste. Improved cell function on these scaffolds will promote healing. Controlled degradation rates of these scaffolds will promote scaffold absorption after its function is no longer required. The objective of this article is to review the current literature describing nanofibers and their potential application to burn care.

Functional Outcomes of Cleft Lip Surgery. Part I: Study Design and Surgeon Ratings of Lip Disability and Need for Lip Revision

Objective: Children with a cleft of the upper lip exhibit obvious facial disfigurement. Many require multiple lip surgeries for an optimal esthetic result. However, because the decision for lip revision is based on subjective clinical criteria, clinicians may disagree on whether these surgeries should be performed. To establish more reliable, functionally relevant outcome criteria for evaluation and treatment planning, a clinical trial currently is in progress. In this article, the design of the clinical trial is described and results of a study on subjective evaluations of facial form by surgeons for or against the need for lip revision surgery are presented. Design: Parallel, three-group, nonrandomized clinical trial and subjective evaluations/ratings of facial views by surgeons. Subjects: For the clinical trial, children with repaired cleft lip and palate scheduled for a secondary lip revision, children with repaired cleft lip and palate who did not have lip revision, and noncleft children. For the subjective evaluations, surgeons’ facial ratings of 21 children with repaired cleft lip. Analysis: Descriptive and Kappa statistics assessing the concordance of surgeons’ ratings of (a) repeated facial views and (b) a recommendation of revision on viewing the prerevision and postrevision views. Results: The surgeons’ consistency in rating repeated views was moderate to excellent; however, agreement among the surgeons when rating individual participants was low to moderate. Conclusions: The findings suggest that the agreement among surgeons was poor and support the need for more objective measures to assess the need for revision surgery.

Achieving Low Cleft Palate Fistula Rates: Surgical Results and Techniques

Objectives To prospectively evaluate and reduce fistula rate after primary cleft palate repair in an academic setting. Methods After noting an institutional palate fistula rate of 35.8%, when a majority of palatoplasties were performed using the Furlow double-opposing Z-plasty, the decision was made to re-evaluate the surgical techniques used for palate repair. As part of our re-evaluation, Furlow and von Langenbeck repairs were limited to clefts less than 8 mm in width. Wider clefts were repaired early in the series with Veau-Wardill-Kilner and later with Bardach two-flap palatoplasties. Half of each palate repair was performed by the residents. Setting Multidisciplinary follow-up was obtained at the University of North Carolina Craniofacial Center. Results A palate fistula was noted in 2 (1.6%) out of 126 cleft palate repairs (both fistulas were located at the anterior hard palate). A split uvula was identified in 2 of 59 patients where the status of the uvula was reported (3.4%). Conclusion This study summarizes one of the lowest overall fistula rates reported in the literature. In a tertiary-care academic setting, plastic surgery residents can actively contribute to palatoplasty with a very low fistula rate. Technical keys to achieving low fistula rate include skeletonization of the vascular pedicle for medialization of the mucoperiosteal flaps, aggressive posterior repositioning of the levator muscle, and meticulous two-layer mattress-suture closure. We recommend Furlow repair for narrower clefts (less than 8 mm wide at the posterior border of the hard palate) and the Bardach two-flap palatoplasty for wider clefts.

Cellular incorporation into electrospun nanofibers: retained viability, proliferation, and function in fibroblasts.

Nanofibers are an emerging scaffold for tissue engineering. To date no one has reported cell incorporation into nanofibers. Human foreskin fibroblasts and human adipose-derived adult stem cells (hADAS) were grown to confluence, resuspended in phosphate-buffered saline, and then solubilized in polyvinyl alcohol (PVA). Nanofibers were created using an electrospinning technique across an electric potential of 20 kV. Cell interaction with nanofibers was assessed with optical microscopic imaging and scanning electron microscopy. PVA nanofibers with incorporated cells were then solubilized in phosphate-buffered saline; cell viability was assessed by trypan blue exclusion. Viable cells were allowed to proliferate. Chondrogenesis in fibroblasts was induced with TGF-&bgr;1. Both fibroblasts and hADAS survived the electrospinning process and were incorporated into PVA nanofibers. hADAS cell proliferation was negligible; however, fibroblasts proliferated and showed retained ability to undergo chondrogenesis. Cells can be incorporated into nanofibers, with maintained viability, proliferation, and function.

Nasoalveolar molding: prevalence of cleft centers offering NAM and who seeks it.

Introduction Nasoalveolar molding (NAM) is a treatment option available for early cleft care. Despite the growing debate about the efficacy of nasoalveolar molding, questions remain regarding its prevalence and the demographic characteristics of families undergoing this technique prior to traditional cleft surgery. Objectives To determine the number of teams currently offering nasoalveolar molding and to identify salient clinical and sociodemographic variables in infants and families who choose nasoalveolar molding compared with those who choose traditional cleft care across three well-established cleft centers. Results Via phone surveys, 89% of the U.S. cleft teams contacted revealed that nasoalveolar molding is available at 37% of these centers. Chart reviews and phone correspondence with caregivers indicate that the average distance to the cleft center was 65.5 miles and caregiver age averaged 30.9 ± 5.7 years. Of families who chose nasoalveolar molding, 85% received total or partial insurance coverage. No difference in caregiver education, income, or distance to the clinic between treatment groups was found. On average, infants receiving nasoalveolar molding and cleft surgery had larger clefts and had more clinic visits than infants receiving traditional cleft surgery. Infants who were firstborn and those who did not have other siblings were more likely to receive nasoalveolar molding than were infants who were residing with other siblings. Conclusions Currently more than one-third of U.S. cleft centers offer nasoalveolar molding. Although the cleft size was larger in the nasoalveolar molding group, no treatment group differences in education, income, and distance to the clinic were found.

MOC-PSSM CME article: Nonsyndromic cleft palate.

Learning Objectives: After reading this article, the participant should be able to: 1. Understand the embryology of normal palate development, and the genetic and environmental causes of clefting. 2. Delineate the anatomy of the normal palate and the cleft palate. 3. Understand the Veau classification for clefts of the palate and techniques used to repair various clefts of the palate. 4. Understand the factors that contribute to complications following palate repair, including fistula formation, velopharyngeal insufficiency, maxillary growth inhibition, and sleep apnea. Summary: Cleft palate is generally an isolated congenital abnormality but can be associated with multiple syndromes. Careful evaluation of an infant with cleft palate by a multidisciplinary team is required to rule out other potential abnormalities. In children with isolated nonsyndromic cleft palate, palate repair is generally performed before 1 year of age. The goals for cleft palate repair include separating the nasal and oral cavities (avoidance of fistulas), establishing normal velopharyngeal function, and preserving maxillofacial growth. This module reviews the incidence, epidemiology, and anatomical classification of cleft palates; and repair techniques, timing, and potential complications associated with palate repair. The Maintenance of Certification module series is designed to help the clinician structure his or her study in specific areas appropriate to his or her clinical practice. This article is prepared to accompany practice-based assessment of preoperative assessment, anesthesia, surgical treatment plan, perioperative management, and outcomes. In this format, the clinician is invited to compare his or her methods of patient assessment and treatment, outcomes, and complications with authoritative, information-based references. This information base is then used for self-assessment and benchmarking in parts II and IV of the Maintenance of Certification process of the American Board of Plastic Surgery. This article is not intended to be an exhaustive treatise on the subject. Rather, it is designed to serve as a reference point for further in-depth study by review of the reference articles presented.

Functional Outcomes of Cleft Lip Surgery. Part II: Quantification of Nasolabial Movement

Objective: To explore nasolabial movements in participants with repaired cleft lip and palate. Design: A parallel, three-group, nonrandomized clinical trial. Subjects: Group 1 = 31 participants with a cleft lip slated for revision surgery (revision), group 2 = 32 participants with a cleft lip who did not have surgery (nonrevision), and group 3 = 37 noncleft control participants. Methods: Three-dimensional movements were assessed using a video-based tracking system that captured movement of 38 landmarks placed at specific sites on the face during instructed maximum smile, cheek puff, lip purse, mouth opening, and natural smile. Measurements were made at two time points at least 1 week and no greater than 3 months apart. Summary measurements were generated for the magnitude of upper lip, lower lip, and lower jaw movements and the asymmetry of upper lip movement. Separate regression models were fitted to each of the summary measurements. Results: Lateral movements of the upper lip were greater than vertical movements. Relative to the noncleft group, the revision and nonrevision groups demonstrated 6% to 28% less upper lip movements, with the smiles having the most restriction in movement and greater asymmetry of upper lip movement. Having an alveolar bone graft further increased the asymmetry, while a bilateral cleft lip decreased the asymmetry. Lower jaw movement caused a small increase in upper lip movement. Conclusions: The objective measurement of movement may be used as an outcome measure for cleft lip surgery.