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Comparative effectiveness research is needed to optimize treatment of unilateral coronal synostosis. This study compares perioperative morbidity, ophthalmic, and aesthetic outcomes of patients with nonsyndromic unilateral coronal synostosis treated by endoscopic suturectomy or fronto-orbital advancement.From 2004 to 2015, patients with unilateral coronal synostosis were reviewed and data recorded for operative details, reoperations, and aesthetic results using the Whitaker classification, severity of strabismus, and need for surgical correction. Categorical data were analyzed using the Fisher's exact test and continuous data were analyzed using the Wilcoxon rank sum test.Ninety-four patients were treated with endoscopic suturectomy (n = 60) or fronto-orbital advancement (n = 34). Median age at follow-up was 3.5 years (range, 1 to 9 years) for endoscopic suturectomy and 5.0 years (range, 2 to 11 years) for fronto-orbital advancement (p = 0.06). The endoscopic suturectomy group had a lower operative time (42 minutes versus 216 minutes), length of stay (1 day versus 4 days), blood loss (25 cc versus 260 cc), and transfusion rate (0 percent versus 88 percent). Two patients treated with fronto-orbital advancement required secondary intervention for intracranial pressure. Thirty-one percent (16 of 52) of the endoscopic suturectomy group and 65 percent (19 of 29) of the fronto-orbital advancement group developed clinically significant strabismus requiring surgical repair (p = 0.002). In the endoscopic suturectomy group, 88 percent (50 of 57) were Whitaker class I and 5 percent were class II. In the fronto-orbital advancement group, 41 percent (14 of 34) were class I and 44 percent class II. Need for bony revision (two of 57 versus three of 34) and need for secondary cranial procedure (two of 57 versus two of 34) were similar following endoscopic suturectomy and fronto-orbital advancement.For treatment of unilateral coronal synostosis, endoscopic suturectomy is associated with lower morbidity and comparable aesthetics. Patients treated with endoscopic suturectomy are half as likely to require strabismus surgery compared with those treated with fronto-orbital advancement.Therapeutic, III.

Background Endoscopic suturectomy and helmeting represents a successful first-line surgical treatment for bilateral coronal craniosynostosis. Its effect on cranial morphology has not been previously described. Methods Patients were identified who had bilateral coronal craniosynostosis treated with endoscopic suturectomy and postoperative helmeting at Boston Children's Hospital between 2005 and 2013 and who underwent preoperative and postoperative computed tomography. Two normative patient populations were identified from our trauma registry with computed tomographic scans completed at the same age as our pretreatment and posttreatment scans. Craniometric indices were used to quantify the effect of treatment. Results Twenty-seven patients were identified who underwent bilateral coronal suturectomy. Twelve patients had preoperative and postoperative computed tomographic studies. Eight patients (66.7 percent) were syndromic. The average ages for preoperative and postoperative computed tomographic scan were 1.1 months (range, 0.03 to 2.6 months) and 19.6 months (range, 10.8 to 37.5 months). Thirteen patients with an average age of 1.1 months (range, 0.5 to 1.6 months) were identified as a preoperative control group. Fourteen patients with an average age of 18.5 months (range, 15.5 to 22.9 months) were identified as a postoperative control group. The anterior cranial height stabilized with treatment and the anterior cranial base length increased. The anterior cranial height-to-anterior cranial base length ratio significantly decreased with treatment (p = 0.128). Frontal bossing normalized with endoscopic suturectomy (craniosynostosis versus control: preoperatively, p = 0.001; postoperatively, p = 0.8). Cephalic indices also normalized with treatment (craniosynostosis versus control: preoperatively, p = 0.02; postoperatively, p = 0.13). No cases of hydrocephalus were observed. Conclusion Endoscopic suturectomy and helmeting improves anterior turricephaly and corrects frontal bossing and brachycephaly in patients with bilateral coronal craniosynostosis. Clinical question/level of evidence Therapeutic, IV.

BACKGROUND The development of surgery in low- and middle-income countries has been limited by a belief that it is too expensive to be sustainable. However, subspecialist surgical care can provide substantial clinical and economic benefits in low-resource settings. The goal of this study is to describe the clinical and economic impact of recurrent short-term plastic surgical trips in low- and middle-income countries. METHODS The authors conducted a retrospective review of clinic and operative logbooks from Hands Across the World's surgical experience in Ecuador. The authors calculated the disability-adjusted life-years averted to estimate the clinical impact of cleft repair and then calculated the economic impact of surgical intervention for cleft disease. RESULTS One thousand one hundred forty-two reconstructive surgical cases were performed over 15 years. Surgery was most commonly performed for scar contractures [449 cases (39.3 percent)], of which burn scars comprised a substantial amount [215 cases (18.8 percent)]. There were 40 postoperative complications within 7 days of operation (3.5 percent), and partial wound dehiscence was the most common complication [16 of 40 (40 percent)]. Cleft disorders constituted 277 cases (24.3 percent), and 102 cases were primary cleft lip and/or palate cases. Between 396 and 1042 total disability-adjusted life-years were averted through surgery for these 102 cases of primary cleft repair. This translates to an economic benefit between $4.7 million (human capital approach) and $27.5 million (value of a statistical life approach). CONCLUSIONS Plastic surgical disease is a significant source of morbidity for patients in resource-limited regions. Dedicated programs that provide essential reconstructive surgery can produce substantial clinical and economic benefits to host countries.

Hearing loss has been described in Apert syndrome but is poorly documented in other craniosynostosis disorders.The authors retrospectively reviewed the audiologic and otologic records of patients with Saethre-Chotzen syndrome to define the incidence, type, and extent of hearing loss. Only patients with documented audiologic examinations were included. Hearing loss was categorized by American Speech-Language-Hearing Association guidelines (i.e., mild, 26 to 40 dB; moderate, 41 to 55 dB; moderate/severe, 56 to 70 dB; severe, 71 to 90 dB; and profound,90 dB).Twenty-nine patients met inclusion criteria. Mean age at initial audiologic evaluation was 6.7 years (range, 0.7 to 24.5 years). Seventeen patients (59 percent) had at least one abnormal audiogram; in 15 patients, the deficit was mild. Eight patients demonstrated sensorineural hearing loss. Five cases resolved and, thus, had been mischaracterized. Six patients had conductive hearing loss on at least one examination; follow-up testing in four patients revealed normal hearing. Two patients had unspecified hearing loss by sound field method. One patient had mixed hearing loss on consecutive audiograms. Twenty-one patients (72 percent) had normal hearing on their last audiogram.Most patients with Saethre-Chotzen syndrome had hearing loss at some point during childhood. This was typically mild and correlated with middle ear abnormality and eustachian tube dysfunction. Usually, the hearing deficit resolved. Early mischaracterization of mixed hearing loss or conductive hearing loss as sensorineural hearing loss was common.

As health care costs rise exponentially in the United States, increasing emphasis is being placed on measuring value, which incorporates both quality and costs. Although the concept of continuous quality improvement has taken a firm foothold in health care, techniques for measuring and continuously improving costs at the patient or system level are lacking.A retrospective, microcosting analysis mapped detailed medical costs over 18 months for 25 patients with nonsyndromic, isolated cleft palate to illustrate the concept of a continuous cost improvement map in a complex, multidisciplinary condition.Care for patients with nonsyndromic, isolated cleft palate was mapped to three timelines based on diagnostic subtype. Patients with Robin sequence requiring early surgical intervention for airway or feeding management (n = 4) had median costs that were triple those of Robin patients managed conservatively (n = 5) ($87,841 versus $27,864, respectively) as compared with patients without Robin sequence (n = 16) ($15,698). Inpatient services accounted for 85 to 95 percent of all costs, which were driven by the operating room, intensive care unit, and inpatient ward. More detailed analysis of each cost driver is reported.The cost improvement map provides a counterpart to the quality improvement map to illustrate how costs may be incorporated into value improvement efforts for complex, multidisciplinary conditions. The transparency and level of detail provided by this methodology are critical for internal improvement efforts and offer valuable insight for health care managers and policy makers, whose decisions should be based on accurate, patient-centered data.

Background: The concept of value-based health care underlies many new improvement initiatives in U.S. health care. To determine value, accurate measures of both outcomes and costs are essential, which may then be compared for the same provider or system over time or between providers, to foster improvement. Although outcomes measurement has received a great deal of attention since the quality movement began in the United States, costing methodologies are lacking. Methods: A basic microcosting methodology was used to obtain direct medical costs, including physician compensation, for individuals with isolated, unilateral cleft lip deformity receiving their full course of care from one surgeon. The authors analyzed costs associated with the timeline of care during the first year of life. Results: The median cost for the first year of life was $13,013 (range, $10,426 to $16,115; n = 12). Ninety-one percent of costs were associated with the cleft lip repair, which occurred at a median age of 3.7 months. The majority of these costs stemmed from time in the operating room and the inpatient stay, which accounted for 68 and 19 percent of first-year costs, respectively. Conclusions: Using a microcosting approach, the authors identified specific cost drivers and outlined a distinct timeline of care for patients with isolated cleft lip in the first year of life. This approach may serve as a template for the cost side of the value equation, for which accurate methodologies are needed. When combined with key outcomes measures, it will be possible to measure and improve value at the patient and provider levels.

Background: The health care debate in the United States centers on a concept that is fundamental to any service-based profession yet minimally integrated into the health care community: value creation. Value in health care has been defined as outcome achieved per dollar spent, and focuses on the patient. Many of the new strategies proposed to restructure health care delivery in the United States aim to study and improve both components of this equation. Indeed, it is a near guarantee that providers will soon be responsible for reporting their outcomes and resource use and will be benchmarked by these metrics. In addition, patients have a right to understand the value they receive from their care providers. Methods: In this report, the authors evaluate the current state of preparedness for the assessment of value in care delivery in the field of cleft lip-cleft palate based on literature review. Results: There has been important progress in the definition and assessment of basic outcomes in cleft lip-cleft palate care, largely through formation of intercenter collaborations. However, many fundamental challenges face the cleft community, especially in North America. Standardization of data collection and outcomes measurement and reporting are particularly lacking. In addition, few data exist regarding the cost of cleft care. Conclusions: The weight of the evidence reveals that the cleft community is not prepared to assess, and thus improve, the value offered to patients. The authors address key challenges and outline future directions.

Background Although most craniosynostoses can be determined by pattern recognition, some patients defy phenotypic diagnosis and require computed tomography to determine sutural fusions. Methods The authors' craniofacial database was reviewed for patients treated for craniosynostosis between 1989 and 2007. Children with single-suture synostosis, isolated bilateral coronal synostosis, and pansynostosis were excluded. Recorded variables included the pattern of sutural fusion, age at presentation, cranial shape, presence of elevated intracranial pressure, genetic testing, and types of operative correction. Results Thirty-nine of 518 patients (7.5 percent) had phenotypically unusual combined forms of craniosynostoses. The most common were bilateral coronal-sagittal (30.8 percent), unilateral coronal-sagittal (10.3 percent), metopic-sagittal (10.3 percent), and bilateral lambdoid-sagittal (7.7 percent). Patients with fusion patterns involving both coronal sutures were more likely to have a mutation than those with other combinations (p = 0.01). Elevated intracranial pressure was noted in 76.9 percent of children. All patients underwent cranial repair; 33.3 percent had staged procedures, including early expansion followed by more definitive remodeling. Patients with bilateral coronal-sagittal or bilateral lambdoid-sagittal fusion had relatively normal head shapes (balanced dysmorphism). This subgroup was more likely to be syndromic and have operative intervention at an older age (p = 0.04). Conclusions Children with phenotypically unusual combined craniosynostoses are likely to have a syndromic diagnosis and elevated intracranial pressure. Cranial expansion was delayed in patients with a balanced dysmorphism. Patients with combined synostoses often require staged procedures because of elevated intracranial pressure or major cranial asymmetry.

Background Craniosynostosis, the premature fusion of the skull bones, is a congenital deformity that has functional and morphologic implications. Cranial vault reconstructive surgery is required to improve skull shape and increase intracranial volume. Craniosynostosis disorders carry a risk of brain insult and associated neurologic and cognitive dysfunction. This study investigated the long-term effects of craniosynostosis on intelligence in children and adolescents with syndromic and nonsyndromic disorders who had undergone cranial expansion surgery during infancy. Methods Global intellectual evaluations were obtained on 31 children aged 7 to 16 years with mixed syndromic (n = 13) and nonsyndromic (n = 18) craniosynostoses. Results of intellectual assessment were compared with norm-referenced data. Age at surgery and gender comparisons were also made. Results Mean +/- SD general intelligence quotient of the total sample was within the average range (intelligence quotient, 95.6 +/- 21.2). Intellectual functioning was significantly lower in children with syndromic craniosynostosis (mean intelligence quotient, 83.1 +/- 21.9) than nonsyndromic craniosynostosis (mean intelligence quotient, 104.7 +/- 15.8). The majority of children with syndromic craniosynostosis (77 percent) were of normal intelligence. Children with nonsyndromic craniosynostosis did not display obvious evidence of intellectual dysfunction. There were no age or gender differences in intellectual outcomes in this sample. Conclusions Findings are contrary to the historical impression that has regarded syndromic craniosynostosis as synonymous with intellectual disability. Children with nonsyndromic craniosynostosis are of normal intelligence during their school-age years.

Surgical correction of synostotic frontal plagiocephaly (unilateral coronal synostosis) focuses on the asymmetry of the forehead and orbits. However, there is controversy regarding whether nasal angulation should be addressed during primary fronto-orbital advancement in infancy. This prospective study was undertaken to answer that question. Preoperative and postoperative anthropometric measurements were obtained for 19 infants with nonsyndromic synostotic frontal plagiocephaly. The measurements included nasal angulation, nasion-to-endocanthion distance, nasion-to-exocanthion distance, and exocanthion-to-tragion distance. All patients underwent bilateral parallelogrammatic fronto-orbital correction. Closing wedge nasal ostectomy was performed for group I (n = 14) and was not performed for group II (n = 5). The average age at the time of follow-up assessments was 3 years 8 months (range, 1 to 14 years) in group I and 5 years 5 months (range, 2 to 15 years) in group II. A statistically significant change was observed for patients who underwent primary correction of nasal angulation; the change correlated with improved naso-orbital symmetry, as judged with nasion-to-endocanthion and nasion-to-exocanthion measurements (p < 0.01 and p < 0.05, respectively). Group I patients exhibited an average preoperative nasal angulation of 9.15 +/- 0.8 degrees that decreased to 3.1 +/- 0.6 degrees postoperatively (p < 0.01). Group II patients exhibited an average preoperative nasal angulation of 6.4 +/- 0.7 degrees that was unchanged postoperatively at 7.2 +/- 1 degrees. The improvement in nasal angulation in group I was particularly striking because the patients in group II exhibited, on average, a lesser degree of preoperative nasal deviation (p < 0.01). This prospective comparison of fronto-orbital correction of synostotic frontal plagiocephaly with and without nasal correction confirmed an earlier study and demonstrated that angulation of the nasal pyramid does not self-correct within 5 years after traditional bilateral fronto-orbital repair. Closing wedge nasal ostectomy results in improved nasal angulation and naso-orbital symmetry, without evidence of distortion or inhibition of nasal growth.

The emphasis on cost reduction and increased efficiency in health care delivery has prompted an increase in outpatient (ambulatory) surgical procedures. A retrospective review of the perioperative management of patients undergoing cleft lip repair at two urban tertiary pediatric hospitals was performed to assess the safety of outpatient cleft lip repair. The hospital database at Childrens Hospital. Los Angeles was searched to find all patients who had been operated on for cleft lip repair during calendar years 1999 and 2000. Two groups were identified from Childrens Hospital Los Angeles: the outpatient cleft lip repair group (patients discharged the same day as the operation; n = 91) and the inpatient cleft lip repair group (n = 14). A data set was acquired from the Royal Children's Hospital in Melbourne, Australia, using the same criteria, for fiscal years 1998 to 2000 (n = 50). All patients from Royal Children's Hospital had operations as inpatients. Parameters considered for each group were age, sex, race, ethnicity, length of hospital stay, preexisting medical conditions or diagnoses, complications, and readmissions or presentation to the emergency department within 4 weeks of operation. The Childrens Hospital Los Angeles outpatient group had three readmissions that were considered to be complications of the operation. The Childrens Hospital Los Angeles inpatient group had one readmission attributable to a complication. The Royal Children's Hospital group also had one readmission for a complication. There was no significant difference in the complication rate of the Childrens Hospital Los Angeles outpatient group and the Royal Children's Hospital group (p > 0.05). There was also no significant difference in the complication rate of both of the Childrens Hospital Los Angeles groups compared with the Royal Children's Hospital group (p > 0.05). This study indicates that cleft lip repair performed in an outpatient setting may be a safe alternative to the inpatient operation. Certain preexisting medical conditions, however, may dictate the need for inpatient hospitalization after repair. (Plast. Reconstr. Surg. 112: 381, 2003.)

A new method of total calvarial remodeling for correcting significant scaphocephaly is described, and experience with the first 30 consecutive patients is presented.Thirty consecutive patients (22 boys and eight girls) with moderate to severe scaphocephaly who underwent total calvarial remodeling using the Melbourne technique were analyzed retrospectively. Mean age at surgery was 7.5 months (range, 4 to 18 months). All had significant frontal and occipital deformities, bitemporal narrowing, a low cranial index, an anteriorly located vertex, and a narrow posteroinferiorly sloping posterior cranium. Clinical photographs, computed tomographic imaging, and cranial index were evaluated preoperatively and postoperatively. A sufficient follow-up period in 17 patients enabled the measurement of changes in cranial indices.A dramatic improvement in head shape was confirmed subjectively by clinical examination and photographs and, in 17 patients, objectively by analyses of the cranial indices. In these 17 patients, mean preoperative cranial index was 64.6 percent preoperatively (range, 56 to 69.3 percent) and increased to a mean of 77.5 percent postoperatively (range, 72 to 81 percent). The mean increase in cranial index was 12.9 percent (range, 9.5 to 19 percent). Among the 30 patients, overall complications were major in one patient (3.3 percent) (presumed air embolus) and minor in three (10 percent).The Melbourne technique of total vault remodeling consistently achieves a virtually normal head shape. After analysis of the first 30 cases, the authors recommend this as their technique of choice for severe scaphocephaly when the full constellation of deformities is present.

Surgical tongue reduction is often performed in Beckwith-Wiedemann syndrome when macroglossia results in abnormal tongue function or cosmesis; however, no published studies have examined the long-term outcomes of this procedure.Patients older than 18 years with Beckwith-Wiedemann syndrome who had previously undergone surgical tongue reduction at the Royal Children's Hospital in Melbourne underwent assessment of speech and tongue function, mobility, sensation, and cosmesis. These assessments were performed by questionnaire on 11 subjects and by formal medical and speech pathology review in four of these 11 subjects.Eleven patients aged 19 to 31 years completed responses by means of questionnaire. The most common self-reported abnormalities were continued disproportionate tongue bulk (91 percent), abnormal tongue appearance (82 percent), specific speech sound errors (73 percent), and a short tongue tip (55 percent). Formal assessments investigated speech, swallowing, taste, and tongue mobility in four quite different subjects. One of these four subjects had no detectable deficits in tongue function. All 11 patients were of normal intelligence and did not report significant difficulties in their day-to-day life that were attributable to their macroglossia or tongue reduction surgery.Pediatric patients with symptomatic macroglossia requiring surgical tongue reduction may not achieve complete normality in tongue function and appearance in adulthood. It is important that surgical tongue reduction addresses the global nature of the macroglossia and aims to retain a tapered tongue tip with length sufficient to permit normal tongue movements.

Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign skin neoplasm that arises from hair follicle matrix cells. Pilomatrixoma is a common skin neoplasm in the pediatric population that is often misdiagnosed as other skin conditions. This study reviews an 11-year experience at a tertiary children's hospital, examining the cause, clinical and histopathological presentation, management, and treatment outcomes of pilomatrixoma. A review of the pathology database at Children's Hospital Los Angeles revealed 346 pilomatrixomas excised from 336 patients between 1991 and 2001. The hospital charts, pathology records, and plastic surgery clinic charts were reviewed with respect to variables such as sex, age at the time of presentation, clinical and histopathological presentation, preoperative diagnosis, management, recurrence, and treatment outcome. The main presenting symptom was a hard, subcutaneous, slowly growing mass. The preoperative diagnosis was accurate and consistent with the pathological diagnosis of pilomatrixoma in only 100 cases (28.9 percent). This entity should be considered with other benign or malignant conditions in the clinical differential diagnosis of solitary firm skin nodules, especially those on the head, neck, or upper limbs. The diagnosis can generally be made with a clinical examination. Imaging studies are not required unless symptoms or the location of the lesion warrants such diagnostic assessments. The treatment of choice is surgical excision, and the recurrence rate is low.

Hemifacial microsomia is the most common facial congenital disability after cleft lip and palate, but as yet its pathogenesis remains unknown. Clinical classification systems have evolved over the last 30 years from those classifying only single components of the disorder, to those classifying according to the combination of deformities, to the most recent systems that grade each anatomical component separately, such as the Orbit, Mandible, Ear, Nerve, and Soft tissue (OMENS) system. The aim of the present study was to review the classification of patients with hemifacial microsomia treated by the Melbourne Craniofacial Unit at the Royal Children's Hospital using the OMENS-Plus system of classification and to correlate the findings with data from other centers. Records of patients treated by the craniofacial unit were reviewed and included in the study if adequate clinical records, photographs, and radiographs (anteroposterior, lateral, basal cephalometry, panoramic views) were available. The data were entered into a database file developed for this purpose. Seventy-one patients were identified from the hospital database, of which six were excluded because of incomplete data. Of the 65 patients, there were 31 (48 percent) with right-sided microsomia, 25 (38 percent) with left-sided microsomia, and nine (14 percent) with bilateral microsomia, with an overall male-to-female ratio of 1.2:1. The majority of patients had a normal orbit (77 percent), mildly hypoplastic mandibular ramus-condyle with functioning temporomandibular joint (57 percent with type M1 or M2a), normal facial nerve (76 percent), and mild soft-tissue hypoplasia (73 percent). There was a similar proportion of patients with mild ear anomalies (53 percent with grade 0 or 1) compared with those with more severe anomalies (47 percent with grade 2 or 3). Correlative analysis demonstrated a slight but positive correlation between the severity gradings of the five individual components. The correlation was lowest between the grading of the nerve and ear and that of the mandible and nerve. The data demonstrate the phenotypic variability of hemifacial microsomia and suggest a degree of relationship among the components of hemifacial microsomia. The OMENS-Plus system has provided a major advancement in the classification of hemifacial microsomia. The authors suggest refinements to the grading of the orbit and nerve components.