Unilateral Cleft Lip Nasal Deformity: Foundation-Based Approach to Primary Rhinoplasty.

Background: Cleft lip results in disruption of the nasal foundation and collapse of tip structures. Most approaches to primary rhinoplasty focus on correction of lower lateral cartilages; however, recurrent deformity is common, and secondary revision is frequently required. The authors describe an alternate approach that focuses on the foundation to "upright the nose," without any nasal tip dissection.

Evolution of Bandeau Shape, Orbital Morphology, and Craniofacial Twist after Fronto-Orbital Advancement for Isolated Unilateral Coronal Synostosis: A Case-Control Study of 2-Year Outcomes.

Background: The authors' purpose was to quantify the change in unicoronal synostosis symmetry between presentation (time 0), after fronto-orbital advancement (time 1), and 2 years later (time 2).

Methods: Bandeau/orbital symmetry ratios and skull base/midface twists were measured on computed tomographic scans of consecutive isolated unicoronal synostosis patients. Comparisons were made across three time points and against normal controls.

Single suture craniosynostosis: Identification of rare variants in genes associated with syndromic forms.

We report RNA-Sequencing results on a cohort of patients with single suture craniosynostosis and demonstrate significant enrichment of heterozygous, rare, and damaging variants among key craniosynostosis-related genes. Genetic burden analysis identified a significant increase in damaging variants in ATR, EFNA4, ERF, MEGF8, SCARF2, and TGFBR2. Of 391 participants, 15% were found to have damaging and potentially causal variants in 29 genes.

Safety of Open Cranial Vault Surgery for Single-Suture Craniosynostosis: A Case for the Multidisciplinary Team.

Introduction: Single Suture Craniosynostosis (SSC) occurs in 1 in 2,500 live births and is the most common type of craniosynostosis treated in most centers. Surgical treatment has evolved over the past century and open techniques are tailored to the specific suture type. Additionally, the concept of multi-disciplinary team care has proliferated and is becoming the standard of care for SSC.

Maternofetal Trauma in Craniosynostosis.

Background: Premature cranial suture fusion may prevent neonatal skull malleability during birth, increasing the risk of unplanned cesarean delivery and neonatal birth trauma caused by cephalopelvic disproportion. We sought to determine the incidence of perinatal maternofetal complications in cases of craniosynostosis.

Methods: Records of children presenting with nonsyndromic craniosynostosis to a tertiary pediatric hospital from 1996 to 2012 were reviewed retrospectively with focus on birth history and birth-related complications.

Adopted children with cleft lip and/or palate: a unique and growing population.

Background: Standard clinical pathways are well established for children with cleft lip and/or palate. Treatment of internationally adopted children differs because of the late age at presentation, a newly evolving child-family relationship, and variable extent and quality of previous treatment.

Methods: The authors characterized the presentation and treatment patterns of all internationally adopted children with clefts at their institution between 1997 and 2011.

Contemporary concepts for the bilateral cleft lip and nasal repair.

The bilateral cleft lip and nasal deformity presents a complex challenge for repair. Surgical techniques continue to evolve and are focused on primary anatomic realignment of the tissues. This can be accomplished in a single-stage or two-stage repair early in infancy to provide a foundation for future growth of the lip and nasal tissue.

Oculoauriculofrontonasal syndrome: case series revealing new bony nasal anomalies in an old syndrome.

Frontonasal Dysplasia (FND) and Oculo-auriculo-vertebral spectrum (OAVS) are two well-recognized clinical entities. With features of both FND and OAVS, the term oculoauriculofrontonasal syndrome (OAFNS) was coined in 1981. The OAFNS phenotype combines elements of abnormal morphogenesis of the frontonasal and maxillary process (derived from forebrain neural crest) with abnormal development of the first and second branchial arches (derived from hindbrain neural crest).

Making the diagnosis: metopic ridge versus metopic craniosynostosis.

Introduction: The metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with metopic craniosynostosis. Metopic ridging (MR) is treated nonsurgically while metopic craniosynostosis (MCS) is treated surgically.

Zygomaticomaxillary complex fractures and their association with naso-orbito-ethmoid fractures: a 5-year review.

Background: Zygomaticomaxillary complex fractures associated with ipsilateral naso-orbito-ethmoidal fractures are more complex injuries than isolated zygomaticomaxillary complex fractures. This injury pattern can have significant long-term morbidity if not recognized and treated appropriately during the initial operation. The purpose of this study is to compare mechanisms of injury, treatment, and outcome between patients with zygomaticomaxillary complex fractures and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures.

The importance of accurate, early bony reconstruction in orbital injuries with globe loss.

Patients who sustain facial fractures frequently suffer from visual disturbance. Additionally, orbital fractures often involve ocular injury, which, not infrequently, may require enucleation. Yet an anophthalmic orbit does not obviate the need for aggressive orbital fracture treatment.

Nasal lining mobilization for primary and secondary palatoplasty.

Background: Repair of wide primary cleft palates and secondary cases are challenging. Much literature is dedicated to technique modifications and useful pearls for approaching these patients. Nasal lining is not often highlighted or addressed as a solution to these challenging cleft palate surgeries.

Major morbidity and mortality rates in craniofacial surgery: an analysis of 8101 major procedures.

Background: The first combined evaluation of morbidity and mortality rates in craniofacial surgery was published 30 years ago; many surgeons believe these procedures have since become safer. The authors performed a contemporary evaluation of craniofacial morbidity and mortality rates to help surgeons more accurately counsel families about current risks, and to gain insight into reducing future incidences.

Methods: This study used two methodologies to capture all serious morbidities and mortalities associated with major craniofacial procedures between 1990 and 2008: a comprehensive two-center retrospective review (Dallas and Seattle), and an Internet-based survey sent to all North American craniofacial centers.

Ectopic glioneuronal tissue in the middle cranial fossa region. Report of four cases.

The growth of ectopic glioneuronal tissue in the middle cranial fossa region is an uncommon event, with very few cases reported in the literature. In this paper the authors document 4 cases of ectopic glioneuronal tissue in the middle cranial fossa in children and briefly describe the clinical course and pathology. All of the children presented within the first 6 months of life.

Bupivacaine administration and postoperative pain following anterior iliac crest bone graft for alveolar cleft repair.

Objective: To determine whether placement of a bupivacaine-soaked absorbable sponge (BAS) in addition to bupivacaine infiltration at the anterior iliac crest (AIC) donor site alters postoperative pain for children undergoing alveolar bone grafting (ABG) for cleft lip with or without cleft palate (CL+/-P). The comparison group received only bupivacaine infiltration (NO BAS) at the AIC.

Design: Retrospective cohort.

Symptom outcomes following cranial vault expansion for craniosynostosis in children older than 2 years.

Background: Most craniosynostosis cases are treated by cranial expansion before 1 year of age. Occasionally, patients present at a later age with nonspecific symptoms of increased intracranial pressure. The purpose of this study was to review the symptoms of patients undergoing late cranial vault expansion.