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.

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.

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.

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.

MDCT diagnosis of the child with posterior plagiocephaly.

Objective: In this article, we review the normal anatomy and development of the posterior skull base and describe distinguishing imaging features of the two most common causes of posterior plagiocephaly: posterior deformational plagiocephaly and unilateral lambdoid synostosis. We also describe three unusual cases of posterior plagiocephaly, including asymmetric premature fusion of the anterior and posterior intraoccipital synchondroses, with diagnoses enabled by volume-reformatted MDCT.

Conclusion: Three-dimensional reformatted MDCT enables accurate diagnosis of common and rare causes of posterior plagiocephaly in children.

Skeletal changes in epidermal nevus syndrome: does focal bone disease harbor clues concerning pathogenesis?

Epidermal nevus syndrome (ENS) is a rare, sporadic, congenital disorder of unknown etiology featuring a complex and highly variable phenotype that can include focal or generalized skeletal disease. We describe a young man with ENS manifesting right-sided linear skin lesions, generalized weakness, diffuse osteopenia associated with hypophosphatemic rickets, and distinctive focal bone lesions ipsilateral to the skin findings. Review of the literature concerning ENS-associated skeletal disease suggested such focal bone defects are fibrous dysplasia, but our patient did not have the typical radiographic or histopathologic findings of fibrous dysplasia.

Diagnostic and surgical challenges in the pediatric skull base.

Advances in management of adult skull base pathologies are increasingly being applied in children. Pediatric patients present special challenges because of their smaller anatomy, but potential gains in reduced morbidity make improvements in skull base approaches well worth pursuing.

Early composite cranioplasty in infants with severe aplasia cutis congenita: a report of two cases.

Objective: Severe cutis aplasia congenita has traditionally been treated with initial soft tissue coverage and delayed cranioplasty. We advocate the technique of early composite reconstruction of both bone and soft tissues.

Methods: Two cases of cutis aplasia congenita with large skull defects (6 x 10 cm, 8 x 8 cm) of superficial layers, skull, and dura are presented.

Furlow palatoplasty for management of velopharyngeal insufficiency: a prospective study of 148 consecutive patients.

Background: The objectives of the study were to describe speech outcomes in a large series of patients undergoing Furlow palatoplasty for management of velopharyngeal insufficiency and to test whether preoperative velopharyngeal gap size and other patient characteristics significantly affect those outcomes.

Methods: Data collected included age at the time of surgery, surgeon, type of cleft, syndrome diagnosis, preoperative velopharyngeal gap size as determined by videonasendoscopy, and preoperative and postoperative perceptual speech assessments. Descriptive statistics were generated and ordinal logistic regression on the outcome variable, postoperative velopharyngeal insufficiency severity score, was performed.

Surgical site infections after pediatric intracranial surgery for craniofacial malformations: frequency and risk factors.

Objective: Surgical site infections (SSIs) after pediatric intracranial surgery for craniofacial malformations are relatively common and potentially serious nosocomial infections. Despite this, few studies have been published on this topic. We performed a retrospective study to determine the incidence of and factors associated with SSIs after intracranial surgery for craniofacial malformations at a single multidisciplinary craniofacial center during a 6-year period.

Differential diagnosis of the trapezoid-shaped head.

Objective: To recognize several conditions that result in a trapezoid head shape and review and contrast their various physical findings.

Methods: A detailed review of all patients seen in the Craniofacial Clinic at the Children's Hospital and Regional Medical Center in Seattle, Washington, over a 10-year period from 1991 to 2001, with the diagnosis of craniosynostosis and plagiocephaly was performed. During this period, 690 patients had a surgical correction of craniosynostosis, and 1537 patients had posterior plagiocephaly.