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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. This study assessed changes with surgery and with growth. Secondary goals were to compare methods of sidewall reconstruction and septoplasty and to identify predictors of relapse.Consecutive patients undergoing repair (n = 102) were assessed. Images were captured preoperatively, postoperatively, and at 5 years of age (when available) using three-dimensional stereophotogrammetry. Standard anthropometric and contemporary shape-based analysis (volume ratio, dorsal deviation, and alar-cheek definition) was performed to assess longitudinal changes. Images of age-matched normal control subjects were used for comparison.Significant changes in anthropometric and morphometric measurements occurred following surgery. Postoperative form was similar to controls immediately after surgery and at 5 years. Nasal corrections were satisfactory, and only two patients have elected to undergo revision. When subjects were grouped according to cleft type, we found the same trends. When comparing different methods of nasal sidewall reconstruction or septoplasty, we found no differences. Alveolar cleft width was a significant predictor of worse preoperative and postoperative form.Significant nasal correction can be achieved by means of reconstruction of nasal foundation, without nasal tip dissection. Preservation of tissue planes may allow for easier secondary revision, if necessary.Therapeutic, IV.

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).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.Forty-three unicoronal synostosis patients and 36 controls were included. The mean bandeau ratio (symmetry = 1) changed from 0.76 (time 0), to 1.13 (time 1), and then to 1.01 (time 2). The median bandeau ratio change from time 1 to time 2 was -9.1 percent and was impacted by the degree of time 1 asymmetry. The odds of a desired symmetric or overcorrected result (bandeau ratio1.0) at time 2 were increased in patients with less severe preoperative asymmetry (OR, 4.2; p = 0.04) and in those who obtained symmetry or overcorrection at surgery (OR, 4.9; p = 0.02). Craniofacial twist did not significantly change after surgery but decreased at time 2. Orbital height ratios were 1.08, 1.00, and then 1.02 at time 2, respectively. The orbital width ratio was not significantly impacted by surgery, remaining at 0.89 at time 2.Overcorrection of the unicoronal synostosis bandeau resulted in these patients being five times more likely to have a desired result at time 2. Patients with a more severe brow presentation were four times more likely to be asymmetric at time 2. Orbital height was improved, but attention should be directed at addressing orbital width.Risk, III.

Introduction Isolated frontosphenoidal synostosis (FS) is a rare cause of fronto-orbital plagiocephaly that can be challenging to distinguish from isolated unicoronal synostosis (UC). The purpose of this paper is to analyze differences in fronto-orbital dysmorphology between the 2 conditions, to describe approaches for surgical correction, and to report surgical outcomes between FS and UC patients in a casecontrol fashion. Methods Patients treated for craniosynostosis over a 12-year period at our institution were retrospectively evaluated under institutional review board approval. Frontosphenoidal synostosis patients who underwent bilateral fronto-orbital correction of anterior plagiocephaly with minimum 2-year follow-up, adequate pre-, and minimum 2-year postoperative computed tomography scans were included in the case-control portion of the study. These patients were randomly age-matched to UC patients meeting the same inclusion criteria. Preoperative and postoperative orbital shape and volumetric analysis was performed using Mimics software. Results Twelve FS patients were treated during the study period. Seven of these patients met casecontrol inclusion criteria with average follow-up of 47.5 months. The characteristic FS orbit was a relatively wide, short, and shallow trapezoid, while the characteristic UC orbit was a relatively narrow, tall, and deep parallelogram. Frontosphenoidal synostosis orbits were significantly wider, shorter, shallower, and smaller than UC orbits. Surgical correction tailored to the differential dysmorphologies resulted in statistical equalization of these differences between affected and contralateral control orbits at follow-up, with the exception of UC orbital width, which remained significantly narrower than unaffected contralateral control. One patient in each group required cranioplasty for skull defects at follow-up, while no patient underwent surgical readvancement. Conclusions Frontosphenoidal synostosis and UC orbital shape differ significantly, and can be normalized using fronto-orbital advancement tailored to the distinct orbital dysmorphologies of these 2 groups.

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. We observed transmission in 96% of the affected individuals, and thus penetrance, epigenetics, and oligogenic factors need to be considered when recommending genetic testing in patients with nonsyndromic craniosynostosis.

Panfacial injury refers to fractures present in the cranio-orbital (upper third), orbitozygomaticomaxillary (middle third), as well as the mandibular (lower third) portions of the craniofacial skeleton. These injuries present challenges in surgical management to avoid long-term sequelae of inadequate correction, including increased facial width, enophthalmos, facial retrusion, and malocclusion. The purpose of this chapter is to provide an algorithm for management of these panfacial injuries in order to optimize outcomes.

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. We observed transmission in 96% of the affected individuals, and thus penetrance, epigenetics, and oligogenic factors need to be considered when recommending genetic testing in patients with nonsyndromic craniosynostosis.

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. Results: Among 1841 children with clefts, 216 (12 percent) were internationally adopted: 78 percent had cleft lip and palate, 18 percent had cleft lip, and 4 percent had cleft palate. Patients originated predominantly from China (80 percent). Median age at presentation was 31 months, and the rate of new presentations increased five-fold during the study period. Eighty-two percent presented with prior cheiloplasty, and revision was recommended for 64 percent of them. Thirty-seven percent of patients had prior palatoplasty, of which 34 percent presented with a palatal fistula. Secondary palatoplasty/pharyngoplasty was performed more frequently for patients who underwent primary palatoplasty before adoption than after adoption (95 percent CI, 0.20 to 0.77). Overall, adoptees required secondary surgery more often than nonadoptees (49 percent versus 28 percent) regardless of where their primary surgery was performed. Changes in adoptee growth indices suggested improvements in systemic health following adoption. Conclusions: Internationally adopted children with clefts have unique treatment challenges. Children with unrepaired clefts undergo surgery late, and children with prior repairs frequently undergo revision. Compared with nonadoptees, adoptees require more revisions and have a higher fistula rate. Further detailed study is important to optimize care.

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. Most cleft surgeons currently perform a single-stage repair for simplifying patient care. Certain institutions utilize presurgical orthopedics for alignment of the maxillary segments and nasal shaping. Methods for the bilateral cleft lip repair are combined with various open and closed rhinoplasty techniques to achieve improved correction of the primary nasal deformity. There is recent focus on shaping the nose for columellar and tip support, as well as alar contour and alar base position. The authors will present a new technique for closure of the nasal floor to prevent the alveolar cleft fistula. Although the alveolar fistula is closed, alveolar bone grafting is still required at the usual time in dental development to fuse the maxilla. It is paramount to try and minimize the stigmata of secondary deformities that historically have been characteristic of the repaired bilateral cleft lip. A properly planned and executed repair reduces the number of revisions and can spare a child from living with secondary deformities.

Learning objectives After studying this article, the participant should be able to: 1. Diagnose zygomaticomaxillary complex fractures from physical examination and radiographic findings. 2. Plan the necessary surgical approaches for operative treatment of zygomaticomaxillary complex fractures depending on severity. 3. Understand the three-dimensional anatomy of the orbit and zygomaticomaxillary complex and the importance of the zygomaticosphenoid suture along the lateral orbital sidewall. 4. Be aware of pitfalls and associated fractures that can complicate anatomical reduction of zygomaticomaxillary complex fractures. Summary Fractures of the zygoma are some of the most commonly treated facial fractures, yet reconstruction of the three-dimensional structure of the zygomaticomaxillary complex can be challenging, and malunions are common. This article presents an evidence-based, systemic approach to the assessment and treatment of zygoma fractures from the simple to the complex. Anatomy, approaches, techniques, and pitfalls are described in an effort to improve the treatment of these common facial fractures.

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.A 5-year retrospective review of all patients treated with zygomaticomaxillary complex fractures at a level I trauma center was performed. Computed tomographic scans were reviewed to divide patients into those with zygomaticomaxillary complex fractures alone and those with zygomaticomaxillary complex and ipsilateral naso-orbito-ethmoidal fractures. Demographics, treatment protocols, outcomes, complications, reoperations, and length of follow-up were identified for both groups and compared to determine differences between these populations.A total of 245 patients were identified by the Current Procedural Terminology codes for zygomaticomaxillary complex fractures. One hundred eighty-five patients had zygomaticomaxillary complex fractures and 60 patients had zygomaticomaxillary complex/naso-orbito-ethmoidal injuries. The demographics for both populations were similar. There are differences between the groups with regard to mechanism of injury, operative findings, and techniques. The patients with zygomaticomaxillary complex/naso-orbito-ethmoidal fractures had higher rates of postoperative complications and deformities.Patients who sustain a zygomaticomaxillary complex fracture associated with an ipsilateral naso-orbito-ethmoidal fracture have a higher incidence of postoperative complications and deformities. It is important to recognize this fracture pattern early to help minimize postoperative morbidity.Risk, II.

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. Results: Combining the two-center data with the survey results yielded a database of 7328 intracranial and 773 subcranial procedures, for a total of 8101 major craniofacial procedures. The combined intracranial major morbidity rate was less than 0.1 percent, and the combined mortality rate was 0.1 percent. Of the eight perioperative deaths following intracranial procedures, four (50 percent) intracranial mortalities were directly attributed to blood loss. The combined subcranial procedure major morbidity rate was 0.1 percent and the mortality rate was 0.3 percent (airway related). Comparing the earliest published intracranial mortality rate to our current review revealed a statistically significant reduction in incidence (p < 0.001). Conclusions: The incidence rates for serious morbidities and mortalities among major craniofacial procedures have significantly fallen since first published. On the basis of these analyses, the authors believe that a greater focus on protocols for airway management, blood salvage and replacement, age-appropriate deep venous thrombosis prophylaxis, and timing of subcranial midfacial advancements might result in further reduction in craniofacial mortality rates.

Multisutural craniosynostosis that includes bilateral lambdoid and sagittal synostosis (BLSS) results in a very characteristic head shape with frontal bossing, turribrachycephaly, biparietal narrowing, occipital concavity, and inferior displacement of the ears. This entity has been reported both in the genetics literature as craniofacial dyssynostosis and in the surgical literature as "Mercedes Benz" syndrome. Craniofacial dyssynostosis was first described in 1976 by Dr. Neuhauser when he presented a series of seven patients with synostosis of the sagittal and lambdoid sutures, short stature, and developmental delay. Over the past 30 years nine additional patients with craniofacial dyssynostosis have been reported in the literature adding to the growing evidence for a distinct craniosynostosis syndrome. The term "Mercedes Benz" syndrome was coined by Moore et al. in 1998 due to the characteristic appearance of the fused sutures on three-dimensional CT imaging. In contrast to the aforementioned reported cases of craniofacial dyssynostosis, all three patients had normal development. Recently, there have been several case reports of patients with BLSS and distinct chromosomal anomalies. These findings suggest that BLSS is a heterogeneous disorder perhaps with syndromic, chromosomal, and isolated forms. In this manuscript we will present the largest series of patients with BLSS and review clinical, CT, and molecular findings.

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. Two children presented with facial masses, 1 with airway obstruction, and another with proptosis of the right eye. Each child underwent a customized surgery dependent on the location and characteristics of the harbored lesion. Ectopic glioneuronal masses in the middle cranial fossa are rare and benign congenital tumors, and affected newborns can present with airway obstruction, feeding difficulties, and facial deformity depending on the lesion location. Determining an appropriate surgical approach and strategy is a significant challenge and may involve a multidisciplinary team of craniofacial plastic surgeons, otolaryngologists, and neurosurgeons. Although these lesions share clinical and anatomical similarities, because of their histopathological heterogeneity, it is unlikely that they represent a single pathological entity. The long-term outcome in these children is still unknown and is an area for future study. The pathogenesis of these lesions also remains unknown and may be revealed in future research.

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. In all cases, the indication for surgery was treatment of the cranial dysmorphology. METHODS The authors performed a retrospective review of all patients (n = 17) undergoing cranial vault expansion from 2000 to 2007 aged older than 2 years (median, 7.4 years) for abnormal head shape who also reported preoperative symptoms consistent with increased intracranial pressure, including headaches, nausea or vomiting, or vision changes. Median follow-up was 30 months; 11 patients had nonsyndromic and six had syndromic craniosynostosis. RESULTS Fourteen patients had headaches, nine had nausea and vomiting, and 11 had vision changes. Ten patients had signs of intracranial pressure elevation, such as a computed tomographic scan findings, papilledema, or both. Twelve of 14 patients had complete resolution of headaches following surgery and four had late mild recurrence. All patients with nausea and vomiting and nine of 11 patients with vision changes had resolution of symptoms. CONCLUSIONS The diagnosis of increased intracranial pressure in older children with craniosynostosis is difficult in the absence of papilledema or computed tomographic findings. Clinical symptoms frequently associated with increased intracranial pressure are improved with late cranial vault expansion and demonstrate a possible benefit beyond improvement in head shape.

Since the first description of a surgical correction of craniosynostosis by Lane in 18921, various techniques have fallen in and out of favor. Initially, high rates of complications caused surgeons to abandon the procedure altogether2. But, gradually, surgical treatment again resurfaced3, mostly in the form of strip craniectomy and removal of the affected suture. Surgeons found, however, that this approach frequently resulted in re-stenosis, inadequate correction, and unacceptable aesthetic results despite a multitude of efforts to prevent re-fusion4. The advent of craniofacial surgery by Paul Tessier5, brought with it a more reliable and reproducible approach which was quickly popularized and has become the standard of care. Over the past decade, craniofacial surgeons have witnessed a resurgence of strip craniectomies for the treatment of single and multi-suture craniosynostosis. The literature is now replete with descriptions of craniosynostosis treatment involving endoscopy7-18, springs19-26 and distraction devices30-35 in conjunction with strip craniectomies. The rationale frequently given for this reversion is shorter, safer operations with less blood loss. The studies which tout “minimally invasive approaches” often denegrate open cranial vault expansion. The current approach of open cranial vault expansion still shares many similarities to Dr. Tessier's descriptions, but it too has evolved over the past decades. What was initially a lengthy, bloody surgery36, 37 with a long recovery and relatively high risk38, 39 has become a routine procedure at many craniofacial centers worldwide. But, previous research into the safety of these procedures has either focused mainly on blood loss, or has relied upon surgeon recall via surveys. Craniofacial teams and the treatment protocols they created have improved the outcomes, shortened the procedures and the recovery, and decreased the risk to the patient. At Seattle Children's Hospital, patients with craniosynostosis are evaluated by a multi-disciplinary team and are offered cranial vault expansion. The type of expansion is tailored to the age of the patient, type of synostotic suture and the shape of the resulting deformity. Those patients who elect to undergo cranial vault expansion are placed on a pathway that is meant to optimize their care from diagnosis through surgery to discharge from the hospital. Since 2002, this pathway has included the same team of pediatricians, surgeons and anesthesiologists who care for this condition. The aim of this study is to describe the multidisciplinary protocol for single suture craniosynostosis care at SCH and more rigorously analyze the safety of open cranial vault expansion in the setting of this paradigm.

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. RESULTS Six hundred eighteen births were reviewed. Rates of cesarean delivery among mothers of children with craniosynostosis [n = 201 (32.5 percent)] exceeded the overall regional rate of 24.5 percent (OR, 1.50; p < 0.0001). Unplanned cesarean delivery occurred in 19.7 percent of births, and were most associated with nulliparous mothers, breech fetal presentations, and lambdoid or multisuture synostosis patterns. Eleven neonates (1.8 percent) exhibited cranial birth trauma, including cephalohematoma and subgaleal hematoma. Neonates with sagittal or multisuture synostosis patterns were more likely to suffer birth trauma and had a higher mean head circumference than those who did not (81st versus 66th percentile, p < 0.05). CONCLUSIONS In the setting of craniosynostosis, birth trauma is increased-for mothers in the form of increased cesarean delivery risk, and for fetuses in the form of subgaleal and subperiosteal perinatal bleeding. Difficult maternal labor may be mediated especially by multisuture or lambdoid synostosis, whereas fetal birth trauma may be mediated to a greater extent by large head size. Prenatal diagnosis of craniosynostosis could influence decision-making in the management of labor. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, IV.

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 × 10 cm, 8 × 8 cm) of superficial layers, skull, and dura are presented. In each case, composite reconstruction was undertaken before 2 weeks of age with restoration of bony and soft tissue coverage through autologous, full-thickness cranial bone grafts and scalp flaps. Both children have been followed up over 2 years with clinical examination and computed tomography (CT) scans. Results In both cases, defects were completely repaired postoperatively and remained closed 2 years later. Complete regeneration of calvarial bone graft donor sites were documented by CT scan. Head shape and circumference were normal at 2-year follow up.

Since its first description by Virchow in 1851, craniosynostosis has been known as a potentially serious condition resulting in premature fusion of skull sutures. Traditionally, craniosynostosis has been regarded as an event that occurs early in fetal development, resulting in a skull shape at birth that is determined by the suture or sutures involved. In recent years, a different form of craniosynostosis has been observed. Patients initially come to the attention of physicians because they exhibit midface hypoplasia or occasionally hypertelorism. The affected individuals all have a normal skull shape and open sutures in infancy but develop multiple-suture craniosynostosis postnatally, ultimately requiring surgical correction. These cases are significant because, although the patients do not initially display the physical manifestations of craniosynostosis, they frequently develop increased intracranial pressure, which can have devastating consequences. Unless these patients are recognized and vigilant follow-up monitoring is instituted at an early age, permanent impairment can result. A retrospective chart review study was conducted, and patients with multiple-suture craniosynostosis who developed symptoms of increased intracranial pressure were selected. The patients were divided into two groups, namely, those with normal sutures and/or head shape at birth (progressive craniosynostosis) (n = 15) and those with abnormal head shapes at birth (classic syndromic craniosynostosis) (n = 12). Clinical and radiological findings typically used to monitor the development of increased intracranial pressure were reviewed for both groups and compared. In addition, mutational analyses were performed. All patients with progressive postnatal craniosynostosis demonstrated clinical, radiological, or ophthalmological evidence of increased intracranial pressure, requiring skull expansion. Those patients displayed papilledema, anterior fontanelle bulge, and thumbprinting more often than did the patients with classic craniosynostosis. Thirteen of 15 patients were given the clinical diagnosis of Crouzon syndrome, which raises the question of whether such patients represent a subset of patients with this syndrome. Mutational analyses for the patients with progressive craniosynostosis demonstrated that, of 13 patients tested, 11 had mutations in exon 7 or 9 of FGFR2, which is a common site of mutations in Crouzon syndrome. The traditional indications of increased intracranial pressure used to monitor patients with classic craniosynostosis can be used to monitor patients with progressive postnatal craniosynostosis, particularly anterior fontanelle bulge, papilledema, and thumbprinting. It is thought that regular monitoring of these characteristics may lead to earlier diagnosis and allow for surgical intervention before the development of undesirable outcomes. It is important for clinicians to be aware of this group of patients, because any delay in diagnosis and treatment can result in severe consequences for the patients.

Ballistic trauma to the craniofacial skeleton combines the challenges of complex bone injury and loss with severe soft tissue injury and violation of the naso-orbital or oropharyngeal cavities. The authors report a patient who experienced a unique ballistic injury at 28 months of age that resulted in loss of the mandibular ramus and condyle. A segmental injury to the facial nerve was also identified. Primary costochondral grafting and delayed interpositional nerve grafting was undertaken. After 10 years, the patient has nearly 40 mm of opening, with only slight deviation to the injured side. Her facial nerve regeneration provides complete orbicularis oculi function, oral competence, and only slight facial asymmetry. This traumatic reconstruction differs from that of patients with hemifacial microsomia or post-traumatic/arthritic ankylosis in that the joint space itself was spared. Thus, the costochondral graft benefits from the remaining articular disk and upper disk space and is able to rotate and translate. Function and growth are adequately re-established, even in this young pediatric patient.

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. Results and Conclusions The shape of the head when viewed from the vertex position in an axial plane can be a significant diagnostic aid when evaluating a patient with plagiocephaly. Positional molding causes the vast majority of plagiocephaly. This deformational change results in a parallelogram-shaped head. A much more rare cause of plagiocephaly is lambdoid synostosis. With premature fusion of one of the lambdoid sutures, the head takes on a very characteristic trapezoid shape when viewed from the vertex. Unilateral coronal synostosis that occurs on the same side as either posterior positional molding or unilateral lambdoid synostosis will also result in the trapezoid head shape. Furthermore, on the rare occasion when anterior and posterior deformational plagiocephaly occurs on the same side, the trapezoid head shape may be the consequence. The choice of appropriate treatment modality requires systematic evaluation of the patient with a trapezoid-shaped head to determine the etiology of the deformation.

The child with posterior plagiocephaly may have positional molding or unilateral lambdoid synostosis. Molding responds to conservative treatment, lambdoid synostosis requires surgical reconstruction. CT is diagnostic, but uses ionizing radiation, may need sedation, and the incidence of lambdoid fusion is only 2–3%. The purpose of this prospective study was to evaluate ultrasound as a screening test of lambdoid sutural patency using CT as the reference standard. In total, 41 children having head CT examinations were enrolled over 6 months. Of those, 29 were referred for abnormal head shape and suspected synostosis, of whom two had lambdoid fusion; 12 were referred for indications not related to head shape and found to have a normal study. Ultrasound scanning and interpretation of the lambdoid sutures was performed blinded to the CT reference standard. The lambdoid suture was read as patent or fused if a hypoechoic gap could or could not be seen between the hyperechoic calvarial bones, respectively. The mean sensitivity and specificity of ultrasound in distinguishing a patent from fused lambdoid suture by three blinded pediatric radiologists was 100% and 89%, respectively. Sonography of the lambdoid sutures shows excellent preliminary promise as a screening test of lambdoid sutural patency.

Anterior cranial base meningiomas are rare tumors in children. Due to the extensive involvement of orbit, paranasal sinuses, midface, and anterior skull base, a multidisciplinary approach is warranted. We present a case of a child with a large planum sphenoidale meningioma extending into subfrontal region, ethmoid and maxillary sinuses inferiorly, and orbits laterally. The patient, a 4-year-old girl, presented with long-standing nasal stuffiness and swelling of the midface. An extended frontobasal approach through a bifrontal craniotomy was used to resect the intracranial portion of this mass. The tumor had eroded through nasal septum, medial orbital walls, and left maxilla, structures which were not readily accessible from a cranial approach alone. A modified Weber-Ferguson incision was used for a transfacial approach to resect the residual mass below the skull base. The advantages of combining the bifrontal craniotomy with a transfacial split provided the added exposure to maximize the extent of resection.

Objective: The primary purpose of this study was to evaluate the long-term result of an onlay bone graft augmentation of the supraorbital ridge at the time of primary correction of coronal suture synostosis. Design: The study is a retrospective review of 62 consecutive patients treated for coronal synostosis from June 1991 through February 1997. The surgical technique utilized involved a standard bilateral fronto-orbital advancement and calvarial reshaping with the addition of an onlay bone graft in the supraorbital region. Setting: All patients were treated at a tertiary care craniofacial center. Results and Conclusion: A total of 62 patients were treated by this technique. Fifty patients underwent primary correction as infants (mean age 9.8 months). An additional 12 patients were older (mean age 8.2 years) and were treated for residual deformity having previously undergone correction by another technique. Results with follow-up as long as 7 years demonstrate stable forehead and orbital symmetry. Complications identified by chart review were minimal and not directly attributable to this modification in surgical technique.

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. The goal of this article was to describe and demonstrate through case examples the simple dissection and significant mobilization of nasal lining as a tool for cleft palate repair. METHODS Retrospective case examples were selected to highlight the technique. RESULTS AND DISCUSSION The surgical technique is standardly used by the senior author and has not been previously published. This technique for tension-free nasal lining closure is detailed, and case examples are provided. This technique for nasal lining mobilization is easily reproducible and is versatile. It can be used as an adjunct to any palatoplasty technique and is particularly useful in the treatment of wide clefts and revision cases.

Neurofibromatosis is common and presents with variable penetrance and manifestations in one in 2500 to one in 3000 live births. The management of these patients is often multidisciplinary because of the complexity of the disease. Plastic surgeons are frequently involved in the surgical management of patients with head and neck involvement.A 20-year retrospective review of patients treated surgically for head and neck neurofibroma was performed. Patients were identified according to International Classification of Diseases, Ninth Revision codes for neurofibromatosis and from the senior author's database.A total of 59 patients with head and neck neurofibroma were identified. These patients were categorized into five distinct, but not exclusive, categories to assist with diagnosis and surgical management. These categories included plexiform, cranioorbital, facial, neck, and parotid/auricular neurofibromatosis.A surgical classification system and clinical characteristics of head and neck neurofibromatosis is presented to assist practitioners with diagnosis and surgical management of this complex disease. The surgical management of the cranioorbital type is discussed in detail in 24 patients. The importance and safety of facial nerve dissection and preservation using intraoperative nerve monitoring were validated in 16 dissections in 15 patients. Massive involvement of the neck extending from the skull base to the mediastinum, frequently considered inoperable, has been safely resected by the use of access osteotomies of the clavicle and sternum, muscle takedown, and brachial plexus dissection and preservation using intraoperative nerve monitoring.Therapeutic, IV.

✓ The authors present the first known reported case of hemifacial microsomia associated with a Chiari I malformation and syrinx. A 14-year-old girl presented with progressive torticollis of 3 years' duration and headaches exacerbated by exercise. Computerized tomography scanning and magnetic resonance imaging revealed extensive craniofacial and vertebral abnormalities, including aplasia of the floor of the left middle fossa and posterior fossa cranium, articulation of the left mandibular condyle with the left temporal lobe, and progressive development of a Chiari I malformation with associated syringomyelia. The patient first underwent posterior fossa decompression, duraplasty, and occipitocervical fusion. This procedure was later followed by reconstruction of the floor of the left middle fossa and temporomandibular joint. The patient's outcome was excellent. In this case report the authors review the complex embryological development of craniofacial and craniovertebral structures, and emphasize the use of a staged approach to treat pathophysiological consequences of this congenital anomaly.

The recent report of a transforming growth factor-beta 3 (TGF-beta 3) knock-out mouse in which 100 percent of the homozygous pups have cleft palate raised the question as to the potential usefulness of these animals as a model for cleft palate research. The specific aim in this study was to carefully document the anatomy of the cleft palate in the TGF-beta 3 knock-out mice as compared with wild type controls. Special attention was paid to the levator veli palatini muscle, the tensor veli palatini muscle, and their respective innervation. Because the TGF-beta 3 knock-out is lethal in the early perinatal period and because the heterozygotes are phenotypically normal, polymerase chain reaction was required to genotype the animals before mating. Time-mated pregnancies between proven heterozygotes were then delivered by cesarean section at gestational day 18.5 to prevent maternal cannibalism of homozygote pups. All delivered pups were killed and their tails processed by polymerase chain reaction to verify genotype. The heads were then fixed and sectioned in axial, coronal, or sagittal planes. Sections were stained with hematoxylin and eosin or processed for immunohistochemistry with nerve specific protein gene product 9.5 and calcitonin gene-related peptide antibodies. Sections were analyzed in a serial fashion. Nine wild type control animals were analyzed along with nine TGF-beta 3 knock-out homozygotes. Time matings between proven heterozygotes yielded wild type pups, heterozygote pups, and homozygote knock-out pups in the expected mendelian ratios (28 percent to 46 percent to 26 percent; n = 43). The results demonstrated 100 percent clefting in the homozygous TGF-beta 3 knock-out pups. Complete clefting of the secondary palate was seen in four of nine and incomplete clefting was seen in five of nine. The levator veli palatini and tensor veli palatini muscles were demonstrated coursing parallel to the cleft margin in all cleft mice. The orientation of these muscles differs from the normal transverse sling of the levator veli palatini muscle and the normal palatine aponeurosis of the tensor veli palatini muscle at the soft palate in control animals. Innervation of the levator veli palatini muscle by cranial nerve IX and the tensor veli palatini muscle by cranial nerve V were demonstrated in both cleft and control animals by use of immunohistochemistry with nerve-specific antibodies. Demonstration of a teratogen-free, reproducible animal model of clefting of the palate with a known, single-gene etiology is an important step in the systematic understanding of a congenital defect whose multifactorial etiology has hampered previous research efforts. This study presents a detailed anatomic description of such a model, including a description of the muscular anatomy and the innervation of the muscles of the palate. Because of early perinatal mortality, this model has limited applications for postnatal studies.

A retrospective study was undertaken to assess speech outcomes in patients undergoing Furlow palatoplasty. Since 1994, the authors have used the position of the levator veli palatini musculature to determine type of surgical intervention recommended for the management of velopharyngeal insufficiency. Furlow palatoplasty has been used in patients with clinical evidence of sagittally oriented levator veli palatini musculature. Forty-eight patients who underwent a Furlow palatoplasty between June of 1994 and August of 1998 were included. All patients underwent preoperative and postoperative perceptual speech analyses to describe velopharyngeal insufficiency severity, nasal air emissions, and resonance, and preoperative nasendoscopy to assess velopharyngeal gap size and palatal and lateral pharyngeal wall movement. Other patient characteristics considered included gender, age at time of surgery, previously repaired cleft palate, submucous cleft palate, and syndrome diagnosis. Speech outcomes were determined on the basis of postoperative perceptual speech analyses and were categorized in one of three ways: (1) complete resolution of velopharyngeal insufficiency, (2) substantial improvement of velopharyngeal insufficiency, and (3) audible residual velopharyngeal insufficiency. Complete resolution of velopharyngeal insufficiency was defined as normal resonance and an absence of nasal air emissions. Substantial improvement of velopharyngeal insufficiency was defined as an improvement of at least two categories in velopharyngeal insufficiency severity in those patients without complete resolution. Audible residual velopharyngeal insufficiency refers to patients with postoperative velopharyngeal insufficiency severity ratings of mild, moderate, or severe. The male:female ratio in the study was 27:21. Twelve patients were syndromic; three had velocardiofacial syndrome. The median age at surgery was 6.5 years (range, 2 to 22 years). The average duration of follow-up was 14.7 months (range, 1.3 to 58.6 months). Postoperatively, the severity of velopharyngeal insufficiency was rated as none in 19 of the 48 patients (39.6 percent), minimal in eight (16.7 percent), mild in six (12.5 percent), moderate in nine (18.75 percent), and severe in six (12.5 percent). Substantial improvement was seen in seven of the 29 patients without complete resolution. There was a significant association between male gender and complete resolution of velopharyngeal insufficiency (p < 0.05). Presence of syndrome and female gender was associated with audible residual velopharyngeal insufficiency (p < 0.05). The main complication was palatal fistula (two cases). In conclusion, most patients who underwent a Furlow palatoplasty had a complete resolution or substantial improvement of velopharyngeal insufficiency postoperatively, and there were few surgical complications.

Hemifacial hypertrophy is a rare condition characterized by unilateral enlargement of all tissues of the face. We describe three patients who exhibit hemifacial hyperplasia of the muscles of facial expression with no other organ system involvement. These three cases, in addition to six other cases identified in the literature, describe a unique constellation of characteristics that place these patients into a distinct syndrome. We suggest that the term “hemifacial myohyperplasia” be used to describe this specific and unique condition. © 2001 Wiley-Liss, Inc.

Craniosynostosis refers to the premature fusion of one of the six major sutures of the cranial vault. Functionally, craniosynostosis may be defined as the premature conversion of the dynamic region of growth and resorption between two adjacent bones of the cranium into a static region of bony union. Molecular analysis has blurred the traditional categories of nonsyndromic and syndromic synostosis to some extent, but, in general, the distinctions between the two groups still hold true. The complexity of the congenital anomalies may be limited with the former, whereas the latter usually requires reoperations and correction of the facial skeleton. This article briefly outlines the characteristic deformities produced from nonsyndromic craniosynostosis. Various approaches to surgical correction of the deformities are described. Finally, new biomaterials that are used in the correction of nonsyndromic craniosynostosis are reviewed.

Desmoplastic fibroma is an exceedingly rare primary bone tumor. Previous reports have not included the presentation of this tumor associated with the zygoma. This report describes the unusual presentation and surgical management of a desmoplastic fibroma of the zygoma.

The management of orbital fractures has evolved with the advent of craniofacial techniques, computed tomography scanning, and rigid fixation. Accurate diagnosis followed by early and aggressive treatment of these injuries can limit the late complications often associated with orbital trauma. The fundamental principles in the management of facial fractures are adequate surgical exposure, anatomic reduction, rigid fixation, and reconstruction using autogenous bone. In this article, these key principles along with the pertinent anatomy, important technical points, and common pitfalls related to the management of orbital fractures are discussed. Special considerations regarding pediatric patients are also reviewed.

Objectives To assess the speech outcomes and complications in children who had undergone sphincter pharyngoplasty (SP) for management of velopharyngeal insufficiency (VPI). Study design Retrospective chart review. Methods Charts from patients who had sphincter pharyngoplasty between January 1993 and June 1996 were reviewed. Syndrome diagnosis and presence of repaired cleft palate were reviewed. Preoperative speech assessment, videofluoroscopic and nasopharyngoscopic evaluations, age at time of surgery, and postoperative speech assessments were reviewed for all patients. Postoperative videofluoroscopy and nasopharyngoscopy were performed for those patients who had persistent VPI. Obstructive sleep symptoms were also assessed. Results Thirty patients were identified; six patients had no follow-up evaluation, leaving 24 patients included in this study. Average follow-up was 11.7 months (range, 2-35 mo). Velocardiofacial syndrome (VCFS) was the most commonly identified syndrome. Postoperatively, 15 of 24 patients (62.5%) had complete resolution of their VPI; five of 24 (20.8%) had significant improvement; one of 24 (4.2%) had minimal to no change; and three of 24 (12.5%) were hyponasal. Of the six patients with some degree of persistent VPI, three underwent revision surgery. All three patients had complete resolution of their VPI after revision surgery, resulting in an overall success rate of 18 of 24 (75%). Conclusions Sphincter pharyngoplasty has wide application in the management of children with VPI, including those with VCFS. The procedure is readily modified to accommodate an individual patient's needs as determined by preoperative VPI evaluation. A modification of the procedure is described to minimize the risk of postoperative airway obstruction and hyponasality, both regarded as airway complications of sphincter pharyngoplasty.

The correct differential diagnosis of an abnormal head shape in an infant or a child is vital to the management of this common condition. Establishing the presence of craniosynostosis, which warrants surgical correction, versus non-synostotic causes of head deformity, which do not, is not always straightforward. This paper deals with three groups of abnormal head shape that may cause diagnostic confusion: the spectrum of metopic synostosis; the dolicho-cephaly of prematurity versus sagittal synostosis; and the differential diagnosis of plagiocephaly. Special emphasis has been placed on the problem of posterior plagiocephaly, in the light of recent evidence demonstrating that lambdoid synostosis has been overdiagnosed. Metopic synostosis presents as a wide spectrum of severity. Although only severe forms of the disorder are corrected surgically, all cases should be monitored for evidence of developmental problems. The dolichocephalic head shape of preterm infants is non-synostotic in origin and is managed nonsurgically. The scaphocephalic head shape resulting from sagittal synostosis requires surgical intervention for correction. Posterior plagiocephaly may be due to unilambdoid synostosis or positional molding, which have very different clinical and imaging features. True lambdoid synostosis is rare. Most cases of posterior plagiocephaly are due to positional molding, which can usually be managed nonsurgically. Regardless of the suture(s) involved, all children with confirmed craniosynostosis should be monitored for increased intracranial pressure and developmental problems.

Objective Congenital bony syngnathia is an extremely rare condition characterized by bony fusion of the jaws. Five new cases are described and the existent literature is reviewed. A classification system is proposed and treatment recommendations based on this classification are presented.

Craniodiaphyseal dysplasia is a rare, sporadic form of craniotubular bone dysplasia, characterized by massive generalized hyperostosis and sclerosis, particularly of the skull and facial bones, leading to severe deformity. The clinical course is typically characterized by progressive encroachment of the craniofacial foramina and brain by the relentless deposition of bone. Compression of cranial nerves, the foramen magnum, and intracranial contents commonly leads to blindness, loss of hearing, and death. This report describes a unique case of craniodiaphyseal dysplasia manifesting with asymmetric craniofacial and axial hyperostosis. The tubular bones demonstrated the characteristic diaphyseal endostosis, undertubulation, and relative overgrowth on the involved side. Significant brain compression with signs and symptoms of increased intracranial pressure was managed successfully with decompressing craniectomy at age 12 years, enlarging the anterior and middle fossae. Calvarial thickness measured nearly 4 cm. Further calvarial, midfacial, and mandibular recontouring were performed 6 and 22 months later. Follow-up in our case indicates that close observation is mandatory to manage further progression of the disease.

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. Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. The goal of this study is to describe the physical examination and CT scan characteristics which may help to differentiate between physiological closure of the metopic suture with ridging (MR) and MCS.A retrospective chart review of all patients seen at Seattle Children's Hospital between 2004 and 2009 with the diagnosis of either MCS or MR (n = 282) was performed. Physical examination characteristics described by diagnosing practitioners were analyzed. Clinical photos were assessed by 3 expert raters to determine the importance of these characteristics. CT scan findings were abstracted and compared between the two diagnoses.The "classic" triad of narrow forehead, biparietal widening, and hypotelorism was present in only 14% of patients with MCS. Ninety-eight percent of patients in both groups had a palpable metopic ridge. The photographic finding of narrow forehead and pterional constriction was present in all patients with MCS, but only in 11.2% and 2.8% of patients with MR. On CT scan, the presence of 3 or more MCS findings was diagnostic of MCS in 96% of patients. Patients with MCS were more likely to present before 6 months of age (66% vs. 32%).Patients with MCS tend to present earlier than those with MR. Upon physical examination, the relationship between the lateral frontal bone and the lateral orbit is important in distinguishing between the two diagnoses. A CT scan can be helpful in making the diagnosis not to confirm a closed suture but to identify 3 or more MCS characteristics.

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). We present a case series of 33 children with OAFNS ascertained from a comprehensive review of the literature and report an additional retrospective series of eight patients displaying features consistent with OAFNS. Notably, in a subset of our cases, we have observed abnormalities in nasal ossification and bony structures of the maxilla that have not previously described in OAFNS and are not seen in either FND or OAVS. We present the phenotype and novel naso-maxillary findings and explore potential etiologic and developmental pathways for OAFNS. We highlight the differences in phenotypic characteristics of OAFNS compared to OAVS and FND. These observations support the classification of OAFNS as a discrete syndrome. Further phenotypic refinements of OAFNS are needed to understand pathogenesis of this syndrome and the newly described nasal malformation may help identify the etiology. © 2013 Wiley Periodicals, Inc.

There is controversy regarding whether the frontal bossing associated with sagittal synostosis requires direct surgical correction or spontaneously remodels after isolated posterior cranial expansion. The authors retrospectively measured changes in frontal bone morphology in patients with isolated sagittal synostosis 2 years after open posterior and midvault cranial expansion and compared these changes with those occurring in age-comparable healthy control groups.Forty-three patients age 1 year or younger (mean, 6 months) with sagittal synostosis underwent computed tomography scan digital analysis immediately after and 2 years after posterior-middle cranial vault expansion. Quantitative angular and linear measures were taken along the midsagittal and axial planes to capture both aspects of frontal bossing. The change in values over the 2 years were compared with healthy controls with normal computed tomography scans taken to rule out head trauma.All measures indicative of frontal bossing decreased significantly from the time of posterior-middle vault expansion to 2 years postoperatively. Whereas the majority of patients at time of the operation had frontal bossing measures greater than two standard deviations outside the age-comparable control mean, almost all patients were within two standard deviations of the norm 2 years later. Lateral forehead bossing and anterior cranial growth was greater the older the patient was at the time of the operation, suggesting that the more time that passed before the operation, the more compensatory anterior fossa growth occurred. Central forehead position relative to the anterior cranial base was greatest in the younger patients at the time of operation, suggesting that a central forehead bulge was an early compensatory response to premature sagittal fusion.As a group, patients with sagittal synostosis start to normalize their forehead morphology within 2 years if an isolated posterior operation is performed at 1 year of age or younger, and this occurs by a combination of restriction of growth and reduction relative to patients without synostosis. This protocol decreases the risks of intraoperative positioning, forehead contour deformities, and two-stage operations.Therapeutic, III.

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. In fact, treatment of the sequelae of the anophthalmic orbit can be difficult and require multiple surgeries. Intraoperative use of a conformer after accurate bony reduction and orbital reconstruction with bone grafts or orbital implants are essential steps to allow for prosthetic rehabilitation of the anophthalmic orbit.

A triad of facial palsy, orofacial edema, and furrowed tongue constitutes an uncommon condition known as Melkersson-Rosenthal syndrome (MRS). We report on 14 patients with Melkersson-Rosenthal syndrome. Two patients had facial palsy, 12 had orofacial edema, and 1 patient had a furrowed tongue. Nine patients were treated medically. Intralesional steroid therapy had a 75 percent recurrence rate. Systemic steroid therapy resulted in remission in two of three patients. Surgical excision and reconstruction were carried out in five patients with chronic lip or eyelid edema. This provided relief in all cases. The etiology, clinical presentation, and histologic features are discussed. Three illustrative cases are presented. An algorithm is provided that guides the surgeon with regard to both the medical and surgical treatment of the patient with Melkersson-Rosenthal syndrome.

A stereotactic system has been designed to address the problem of achieving symmetry in complex and extensive craniofacial defects. Preliminary testing suggests that such a system, which allows for the intraoperative application of preoperative CT planning, will be useful in guiding the reconstruction of congenital or acquired bony time, is being used to investigate the correlation of intraoperative globe position following enophthalmos correction with long-term outcome, particularly as it relates to the size and location of the orbital defect, and the timing of the procedure.

The aim of treatment of combined injuries of the cranium and face is the correct anatomic restoration of the maxilla in relation to the cranial base above and the mandible below and the reconstruction of any associated craniofacial, naso-orbitoethmoidal, and zygomatic fractures. The plethora of techniques described in this article for the management of these injuries attests to the controversy and confusion surrounding the management of these patients.