Craniofacial Distraction Osteogenesis.

Distraction osteogenesis (DO) of the craniofacial skeleton has become an effective technique for the treatment of both nonsyndromic and syndromic conditions. The advent of craniofacial DO has allowed for earlier intervention in pediatric patients with less complication risk and morbidity compared to traditional techniques. In this review, we will discuss current application and technique for craniofacial DO by anatomical region and explore future applications in craniofacial surgery.

Revisional Techniques for Secondary Cleft Lip Deformities.

Secondary deformities of repaired cleft lips are an unfortunate complication despite the meticulous approach of modern primary procedures. Most of these surgeries take place in the patient's early life and must be strategically planned to provide optimal cosmesis with minimal interventions. Depending on the level of severity, treatment of the secondary deformities ranges from noninvasive or minimally invasive techniques to complete revision cheiloplasty. Many novel topical, injectable, and laser therapies have allotted physicians more technical flexibility in treating superficial distortions. Nonetheless, surgical techniques such as diamond excision and adjacent tissue transfer remain popular and useful reconstructive modalities. Deformities involving the orbicularis oris must be completely taken down to allow full access to the muscle. Complete revision cheiloplasty requires recreation of the cleft defect and reconstruction similar to the primary repair. Due to the myriad of presentations of these secondary deformities, familiarity with the various treatments available is imperative for any cleft surgeon.

Scar Revisions.

Scars pose a complex challenge to the reconstructive surgeon in the variability of their presentation and their effect on cosmesis and function. Numerous surgical and nonsurgical options exist for scar management, each with their respective advantages and disadvantages. Each treatment modality should be tailored to the patient to ensure enhanced outcomes. In this review, we discuss different scar presentations, scar management options, and the benefits and risks with undergoing these various treatment modalities.

Distraction Osteogenesis: Mandible and Maxilla.

Mandibular and maxillary deformities commonly require surgical intervention. Prior to distraction osteogenesis, traditional modalities involving single-staged translocation and rigid fixation were used to correct these craniofacial anomalies. Distraction osteogenesis has evolved as a compelling alternative for treating aesthetic and functional dentofacial defects. The process of distraction osteogenesis involves three phases-latency, activation, and consolidation-which allow for appropriate translation of the affected craniofacial skeleton. This review will cover the role of distraction for managing congenital and acquired deformities of the mandible and maxilla. This novel technique can be performed at numerous anatomical sites along the craniofacial skeleton to treat a variety of anomalies, which serves as a testament to its adaptability and efficacy. Importantly, distraction osteogenesis also has the ability to simultaneously increase bone length and the overlying soft tissue envelope. This advantage results in larger advancements with reduced relapse rates and improved patient satisfaction. While complications remain a concern, it stands to reason that the measurable benefits observed underscore the power and versatility of distraction osteogenesis.

A Novel Sand Dollar and Staves Technique for Unilateral Lambdoid Craniosynostosis.

BACKGROUND: Unilateral lambdoid synostosis (ULS) is the rarest form of craniosynostosis. Due to the associated cranio-caudal shift seen in ULS, surgical correction is technically challenging from a morphological standpoint. Herein, the authors present a novel "Sand-Dollar and Staves" technique for the repair of ULS. METHODS: A zigzag coronal incision is performed, and an anteriorly-based pericranial flaps are elevated. Prefabricated cutting guides are placed and the calvarium is marked. To treat the flattening on the ipsilateral side, a wedged suturectomy is performed with additional barrel staves. A large circle centered over the bulging on the contralateral side is cut out above the open lambdoid suture. This piece is barrel staved in a radial fashion, leaving the center intact and creating a Sand-Dollar appearance. This disk is then flattened and trimmed. The modified Sand-Dollar is fixed using an absorbable plating system. While gentle pressure is applied to the Sand-Dollar piece as it is being secured, the ipsilateral side demonstrates compensatory filling. Results are evaluated using the Whitaker Classification. RESULTS: Four patients underwent surgical correction with this technique. The procedure was performed at mean age of 11.7 months. The mean operative time was 2.5 hours. Intraoperative blood loss was 50 to 100 ml. Total hospitalization time was 2 to 3 days. No post-operative complications were encountered. Whitaker scores ranged from 1 to 1.5. The mean follow-up was 10 months. CONCLUSION: The Sand-Dollar and Staves procedure is a novel, single-stage approach for the management of ULS with decreased operative time, blood loss, and hospital stay with satisfactory aesthetic outcomes.

A Global Evaluation of Surgical Techniques and Results of Unilateral Cleft Lip Repairs.

BACKGROUND: Variations in the clinical presentation of the unilateral cleft lip resulted in numerous surgical repair techniques used around the world. No universally accepted method exists to evaluate aesthetic surgical results. The purpose of this study is to use the Hubli lip grading system, developed by Smile Train, to evaluate the aesthetic results of unilateral cleft lip repairs based on the surgical technique used. METHODS: A retrospective review was conducted of unilateral cleft lip repair operations performed by Smile Train surgeons throughout Africa, Americas, Asia Pacific, and Europe and Middle East between 2014 and 2018. Using the Hubli lip grading criteria, factoring in cleft severity, acceptable outcomes were identified and compared with the surgical technique used. RESULTS: Eight thousand forty-one unilateral cleft lip repairs were reviewed. The majority utilized the rotational advancement technique (n = 5541, 68.9%) with a statistically significant percentage of acceptable outcomes (92.1%), followed by an acceptance rate of 91.9% with variations of the z-plasty technique, 90.5% with the straight-line repair and its variations, and 88.4% using "other" techniques (P < 0.05). The rotational advancement had the highest acceptable outcomes for complete cleft lips (94.7%, P < 0.0001), associated alveolar clefting (94.2%, P < 0.05), and concomitant clefting of the hard and/or soft palate (94.3%, P < 0.05). CONCLUSION: Regardless of surgical technique used, Smile Train surgeons maintain high rates of successful surgical outcomes. Results of our study will help complement and support existing medical infrastructure and training systems Smile Train employs for surgeons working with comprehensive cleft care teams around the world.

Airway Obstruction Management With Mandibular Distraction and Matthews Device in Pruzansky III Craniofacial Microsomia.

Patients with craniofacial microsomia (CFM) may present with severely malformed mandibles resulting in secondary tongue-based airway obstruction. While surgical management of clinically significant airway obstruction in these patients often involves mandibular distraction osteogenesis (MDO), patients with Pruzansky III CFM typically require additional costochondral bone grafting. In this report, the authors present the successful surgical management of airway obstruction and severe obstructive sleep apnea using a cranio-mandibular fixator (Matthews device) combined with MDO in a 4-year-old female patient with Goldenhar syndrome and associated Pruzansky III CFM. The patient tolerated the procedure without complications and experienced a significant improvement in airway obstruction. With this approach, a severely deformed mandible can be successfully distracted while avoiding tracheostomy and disruption of native TMJ anatomy.

Applications of Fat Grafting in Pediatric Patients.

Autologous fat grafting has become a widely utilized technique for a variety of cosmetic and reconstructive procedures. Its potential for volume restoration and tissue regeneration has made it a popular method for treating soft tissue defects in both adult and pediatric populations. While autologous fat grafting in the pediatric setting is not as well characterized as it is in the adult setting, various reports have demonstrated the safety and utility of its applications in nonadult patient populations. In this article, we present the first comprehensive review of the current applications of autologous fat grafting in pediatric patients. Specific challenges to fat grafting in the pediatric setting and future applications will also be discussed.

Chicken or the Egg: A Case Report of Endoscopic-Assisted Treatment of Concomitant Sagittal Craniosynostosis With Calcified Cephalohematoma.

Nonsyndromic craniosynostosis results in premature fusion of cranial sutures and is rarely found in the context of other craniofacial abnormalities. Here the authors present the case of a 3-month-old male infant with a rare presentation of sagittal craniosynostosis and concomitant calcified cephalohematoma repaired by endoscopic-assisted sagittal strip craniectomy with good cosmetic and functional outcomes. The authors discuss the advantages of endoscopic repair of craniosynostosis when found in the presence of a cephalohematoma and the need for further research to investigate a possible causal relationship between these 2 pathologies.

Spontaneous Mediastinal Abscess and Sternal Cleft in the Medically Complex Neonate.

Sternal cleft (SC) is a rare congenital anomaly, occurring with associated developmental anomalies or in isolation. Surgery to reconstruct the sternum is indicated to protect the visceral organs from trauma, to ensure healthy cardiopulmonary function and growth, and to reconstruct the anterior chest wall. Although infection recognized as a postoperative complication following chest wall reconstruction, spontaneous mediastinal infection is rare. To the authors' knowledge, there is only 1 reported case of spontaneous mediastinal infection with concomitant SC in the literature. Here, the authors present a unique case of a medically complicated infant with a SC who presented with a spontaneous mediastinal abscess.

Nonsyndromic Craniosynostosis.

Nonsyndromic craniosynostosis is significantly more common than syndromic craniosynostosis, affecting the sagittal, coronal, metopic, and lambdoid sutures in decreasing order of frequency. Nonsyndromic craniosynostosis is most frequently associated with only 1 fused suture, creating a predictable head shape. Repair of craniosynostosis is recommended to avoid potential neurodevelopmental delay. Early intervention at 3 to 4 months of age allows minimally invasive approaches, but requires postoperative molding helmet therapy and good family compliance. Open techniques are deferred until the child is older to better tolerate the associated surgical stress. Cranial vault remodeling is generally well-tolerated with a low rate of complications.

Pediatric Craniomaxillofacial Oncologic Reconstruction.

Reconstruction of defects of the head and face in the pediatric population requires special consideration for future growth, and at times temporization in anticipation for skeletal maturity followed by subsequent reoperation at an appropriate age. Additional challenges include more limited donor sites, smaller anastomoses, and unpredictable postoperative compliance compared with their adult counterparts. Nonetheless, successful composite bony and soft tissue, and isolated soft tissue defects in children are safely reconstructed using existing local tissue and microsurgical techniques.

Cranial Anchored Mandible Distraction Osteogenesis.

Multiple or large distance mandibular distraction osteogenesis (MDO) in the older child is often complicated by iatrogenic temporomandibular joint (TMJ) pathology. The transmission of significant force to the TMJ in these particular patients is due to the greater distance of distraction required and the relative inelasticity of the soft tissue envelope. The authors present a clinical report of a successful asymmetrically vectored large distance MDO in a 13-year-old female with bilateral craniofacial microsomia with Goldenhar syndrome. During distraction, the TMJ joints were effectively unloaded from the forces of distraction using external bilateral cranial anchored devices (Cranio-Mandibular Fixator; KLS Martin, Jacksonville, FL). Angle's occlusion, facial angle, and evidence of TMJ pathology were assessed.

Speech Outcomes After LeFort I Advancement Among Cleft Lip and Palate Patients.

BACKGROUND: Velopharyngeal insufficiency (VPI) results from incomplete closure of the velopharyngeal (VP) sphincter with oral pressure consonants during speech. Maxillary hypoplasia is common among cleft children and often requires LeFort I advancement. This results in anterior movement of the soft palate with the bony maxillary segment. Consequently, the size of the VP sphincter is increased and may result postoperative VPI or worsening of prior VPI. To better counsel our patients and their families of the risk of VPI after LeFort I advancement, we chose to evaluate our own cohort. METHODS: We conducted an institutional review board-approved prospective review of all cleft children presenting to Texas Children's Hospital who underwent LeFort I advancement after previous palatoplasty between 2013 and 2016 in a three-surgeon, consecutive patient series. Data collected included age, sex, ethnicity, cleft type, prior secondary speech surgery, presence of preoperative fistula, planned distance of advancement, orthognathic surgery performed, and any concurrent procedures performed. Primary outcomes measured included preoperative and postoperative VP function and hypernasality as measured by a certified speech pathologist. RESULTS: Velopharyngeal function was unchanged in 67% of our cohort after LeFort I advancement. Of those patients, 83% had evidence of VPI preoperatively, and 17% had normal speech preoperatively. Twenty-two percent of the patients displayed worsening VP function after surgery, and 6% displayed evidence of improvement. Velopharyngeal function was unable to be assessed in 6% of patients. Nasality ratings worsened in 39% of patients, were unchanged in 39%, and improved in 22%. Of the patients with incompetent VP function after surgery, 50% already received or are currently scheduled for secondary speech surgery, 25% declined secondary surgery, and 25% are pending scheduling. CONCLUSIONS: Although VP function remains unchanged in a majority of patients after LeFort I advancement, VPI should be carefully screened for after surgery. If detected, secondary operations to correct speech should be strongly considered.