Ramon L. Ruiz

Treacher Collins syndrome: Current evaluation, treatment, and future directions

OBJECTIVE Treacher Collins syndrome (TCS) is an inherited disorder in which there are general bilateral symmetric anomalies of the structures within the first and second branchial arches. In general, there is complete penetrance and variable expressivity of the trait. The craniofacial rehabilitation of a child with TCS is tailored to the extent of the deformities involved: the orbitozygomatic region, the maxillomandibular region, the nose, facial soft tissues, and external and middle ear structures. CONCLUSION This article reviews the range of clinical features and specific dysmorphology observed in TCS. Functional and aesthetic objectives are discussed, and a comprehensive staged reconstructive approach is outlined, which may be used as a roadmap for treatment planning.

The Craniofacial Dysostosis Syndromes: Current Surgical Thinking and Future Directions

Craniofacial dysostosis is the term applied to familial forms of craniosynostosis in which the sutural involvement generally includes the cranial vault, cranial base, and midfacial skeletal structures. The syndromic forms of craniofacial dysostosis were initially described by Carpenter, Apert, Crouzon, Saethre and Chotzen, Pfeiffer, and others. In addition to the dysmorphic cranial features, affected individuals may have profound alterations in facial skeletal development. Surgical reconstruction requires thoughtfully sequenced and staged procedures with consideration for the individuals specific malformations, craniofacial growth patterns, and psychosocial needs. Management of the craniofacial dysostosis syndromes is surgical, but the indications and the timing, type, and effectiveness of each stage of reconstruction have not been well evaluated and remains as much an art as a science. This article reviews the specific characteristic clinical features of the craniofacial dysostosis syndromes and presents current philosophy and rationale for the staging of reconstruction.

Cleft Lip and Palate Surgery: An Update of Clinical Outcomes for Primary Repair

The comprehensive management of cleft lip and palate has received significant attention in the surgical literature over the last half century. It is the most common congenital facial malformation and has a significant developmental, physical, and psychological impact on those with the deformity and their families. In the United States, current estimates place the prevalence of cleft lip and palate or isolated cleft lip at approximately 1 in 600. There is significant phenotypic variation in the specific presentation of facial clefts. Understanding outcome data is important when making clinical decisions for patients with clefts. This article provides an update on current primary cleft lip and palate outcome data.

Survival of microvascular free flaps in mandibular reconstruction: A systematic review and meta-analysis

Free tissue transfer is commonly used in the reconstruction of post‐ablative defects of the mandible. Due to lack of statistical power, comparing the survival of various free flaps, even in large studies, is challenging. The purpose of this study was to perform a meta‐analysis comparing the survival of the most commonly used free flaps for mandibular reconstruction.

Surgical correction of midface deficiency in cleft lip and palate malformation

Timothy A. Turvey, DDS*, Ramon L. Ruiz, DMD, MD, Bernard J. Costello, DMD, MD Department of Oral and Maxillofacial Surgery, University of North Carolina at Chapel Hill, Brauer Hall, CB #7450, Chapel Hill, NC 27599-7450, USA Department of Pediatrics, University of North Carolina at Chapel Hill, Brauer Hall, CB #7450, Chapel Hill, NC 27599-7450, USA Children’s Hospital of North Carolina, Chapel Hill, NC, USA University of North Carolina Craniofacial Center, Chapel Hill, NC, USA Departments of Oral and Maxillofacial Surgery, Pediatric Dentistry, and Pediatric Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA Magee-Women’s Hospital, Pittsburgh, PA, USA Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

Craniofacial and Orbital Dermoids in Children

Dermoid cysts are congenital lesions that commonly arise from nondisjunction of surface ectoderm from deeper neuroectodermal structures. They tend to be found along planes of embryonic closure. Classification by site is helpful for diagnostic planning and surgical treatment. A distinction can be made between frontotemporal, orbital, frontoethmoidal, and calvarial lesions. The risk of extension into deeper tissues must be determined before surgical intervention. Simple lesions are amenable to direct excision. Deeper lesions often require a coordinated surgical approach between a neurosurgeon and craniofacial surgeon after thorough radiographic imaging. Follow-up through the developmental years is recommended for complex dermoid lesions.

Temporary Skeletal Anchorage Devices for Orthodontics

This article discusses the recent advances and basic concepts of skeletal anchorage devices of various types and reviews the current literature on their use. Temporary skeletal anchorage devices allow orthodontic movements that were previously thought to be difficult if not impossible. Much like the concepts introduced during the beginnings of orthognathic dentofacial teams, treatment that uses skeletal anchorage requires interdisciplinary collaboration and planning with regular interaction, continuing education, and a regular review of the latest relevant literature.

Microvascular Free Tissue Transfer for Head and Neck Reconstruction in Children: Part I.

Background:Limited outcome data exist regarding the survival of microvascular free flaps for head and neck reconstruction in children. The objectives of this study were to perform a systematic review of the literature and meta-analysis comparing the survival of the most commonly used free flaps used for head and neck reconstruction in children. Methods:A systematic search of PubMed, Embase, and Scopus was conducted using various keywords up to January 1, 2015. Meta-analysis was used to compare the survival of the most commonly used free flaps. The primary predictor variable was free flap type. The primary outcome variable was flap failure. The pooled relative risk (RR) with 95% confidence intervals (CIs) was estimated using a Mantel-Haenszel, fixed-effects model. Results:The authors reviewed 25,303 abstracts. Five studies met inclusion criteria. A total of 646 children received a total of 694 free flaps. The pooled survival rate among all free flaps was 96.4%. The fibula free flap (fibula) and subscapular system free flaps (scapula) were the most commonly used flaps. There was no difference in survival when comparing the scapula (RR = 0.59, 95% CI: 0.26, 1.56, P = 0.29), or fibula (RR = 1.91, 95% CI: 0.55, 6.65, P = 0.31) to other free flaps, or when comparing the scapula to the fibula (RR = 2.29; 95% CI: 0.40, 13.08, P = 0.35). Conclusions:Free tissue transfer is highly successful in children. Although data are limited, there appears to be no difference in survival among various free flaps used for head and neck reconstruction in children.

Management of Cleft Lip and Palate and Cleft Orthognathic Considerations

Cleft lip and palate are among the most common congenital anomalies in humans. The treatment of this group of patients is best conducted by a multidisciplinary team approach. This article discusses the accepted treatment algorithm and timeline, as well as special considerations for this patient group when performing orthognathic surgery. Patients with cleft lip and palate often present with significantly more technical and challenging procedures, so clinicians should familiarize themselves with these special considerations before attempting to care for these individuals.

The Coronal Scalp Flap: Surgical Technique

Ramon L. Ruiz, DMD, MD*, Jogi V. Pattisapu, MD, Bernard J. Costello, DMD, MD, Brent Golden, DDS, MD Arnold Palmer Hospital for Children, 83 West Columbia Street, Orlando, FL 32806, USA University of Central Florida College of Medicine, Health Sciences Campus at Lake Nona, 6850 Lake Nona Boulevard, Orlando, FL 32827, USA Oral & Maxillofacial Surgery, University of Pittsburgh College of Dental Medicine, Pittsburgh, PA, USA Oral & Maxillofacial Surgery Residency, University of Pittsburgh College of Dental Medicine, Pittsburgh, PA, USA Pediatric Cleft & Craniofacial Surgery Fellowship Program, University of Pittsburgh College of Dental Medicine, Pittsburgh, PA, USA