Soft Palate Dysfunction in Children With Microtia.

BACKGROUND: Speech issues in microtia patients have been historically attributed to poor hearing. However, the authors have noted that almost all patients with microtia have palatal dysfunction. The aim of this study is to determine the prevalence of soft palate dysfunction (SPD) and velopharyngeal insufficiency (VPI) in microtia patients. METHODS: A prospective cohort study was performed on consecutive microtia patients from March to June 2017. Clinical characteristics were collected. Palate movement was rated by oral examination and degree of nasal escape by the mirror-fogging test. Correlations between clinical characteristics of microtia and SPD were determined. RESULTS: Ninety-seven (40 unilateral, 27 bilateral, and 30 control) children met inclusion criteria. Among all 67 patients with microtia, 96% (64 patients) showed SPD. Twenty-four unilaterals (60%) and 23 bilaterals (85%) had observable VPI by mirror examination. Of these patients, nasality was noticeable to the examiners in 14 unilaterals (58%) and 21 bilaterals (91%). Sixteen of the 27 bilaterals (59%) showed almost no movement of the soft palate. There was a significant correlation between SPD and mirror-fogging. A less developed middle ear as determined by computed tomography scan was associated with palatal dysfunction (P = 0.007). The severity of mandibular shift (P = 0.048) and presence of a syndrome (P = 0.045) were associated with grade of VPI. The severity of the ear deformity (P = 0.007) and presence of a syndrome (P = 0.034) were also correlated with the presence of SPD. CONCLUSIONS: This is the first study that documents the presence of VPI due to soft palate dysfunction in patients with isolated and nonisolated microtia.

Epidemiology and resource utilization in pediatric facial fractures.

BACKGROUND: Pediatric facial fractures, although uncommon, have a significant impact on public health and the US economy by the coexistence of other injuries and developmental deformities. Violence is one of the most frequent mechanisms leading to facial fracture. Teaching hospitals, while educating future medical professionals, have been linked to greater resource utilization in differing scenarios. This study was designed to compare the differences in patient characteristics and outcomes between teaching and non-teaching hospitals for violence-related pediatric facial fractures. METHODS: Using the 2000-2009 Kids' Inpatient Database, 3881 patients younger than 18 years were identified with facial fracture and external cause of injury code for assault, fight, or abuse. Patients admitted at teaching hospitals were compared to those admitted at non-teaching hospitals in terms of demographics, injuries, and outcomes. RESULTS: Overall, 76.2% of patients had been treated at teaching hospitals. Compared to those treated at non-teaching hospitals, these patients were more likely to be younger, non-white, covered by Medicaid, from lower income zip codes, and have thoracic injuries; but mortality rate was not significantly different. After adjusting for potential confounders, teaching status of the hospital was not found as a predictor of either longer lengths of stay (LOS) or charges. CONCLUSIONS: There is an insignificant difference between LOS and charges at teaching and non-teaching hospitals after controlling for patient demographics. This suggests that the longer LOS observed at teaching hospitals is related to these institutions being more often involved in the care of underserved populations and patients with more severe injuries.

The Surgical Treatment of Robin Sequence.

BACKGROUND: We present an outcomes analysis of the surgical treatment of Robin sequence including all infants and comorbid conditions treated by tongue-lip adhesion (TLA) or mandibular distraction osteogenesis (MDO). METHODS: A 19-year single-institution, multisurgeon retrospective review of all syndromic and nonsyndromic neonates with Robin sequence treated with TLA (1994-2004) or MDO (2004-2013) was performed. Comorbid conditions were recorded in all patients. Outcomes include incidence of tracheostomy, improvement in obstructive breathing, and surgical complications. Need for repeat distraction and conversion from TLA to MDO were included as secondary end-points. Polysomnography data were recorded preoperatively at 1 month and 1 year as a measure of airway improvement. RESULTS: Seventy-four MDO patients and 15 TLA patients during the study period met inclusion criteria. There was no significant difference in mean age at intervention (32.1 ± 29.0 vs 35.5 ± 32.1 days), birth weight (2.9 ± 0.7 vs 3.2 ± 0.6 kg), prematurity (23.0 vs 35.7%), or intrauterine growth restriction (31.1 vs 15.4%). Central nervous system anomalies (24.3% vs 0.0%; P < 0.04) and gastrostomy tubes (66.2% vs 33.3%; P < 0.03) were present more frequently in MDO patients versus TLA patients. Rates of other organ system anomalies were similar between the groups. The success rate was significantly higher in the MDO group (90.5% vs 60.0%; P < 0.008). Postoperative tracheostomies occurred in 8.1% of the MDO group and 33.3% of the TLA group (P < 0.02). Preoperative apnea-hyponea index was similar between the two groups (38.3 vs 38.1). The apnea-hyponea index was significantly improved in the MDO group at 1 month (4.0 vs 21.7; P < 0.002) and 1 year (5.7 vs 20.5; P < 0.005). Surgical complications were statistically less in the MDO group (20.3 vs 53.3%; P < 0.02). CONCLUSIONS: In the heterogeneous population of Robin sequence, MDO demonstrates superior outcomes measures at 1 month and 1 year compared to TLA. Fewer complications occurred in the MDO group compared to the TLA.

Economic Analysis of Cleft Palate Repair in International Adoptees.

DESIGN: Retrospective cohort study. SETTING: Major international tertiary care referral center for cleft palate repair. PATIENTS: One hundred thirty-eight patients at the Children's Hospital of Philadelphia who had palate repair performed between 2010 and 2013, excluding syndromic patients, patients undergoing palate revision, and patients with incomplete payment information. INTERVENTIONS: None. MAIN OUTCOME MEASURE: Fees and charges for procedures. RESULTS: Surgeon payment was significantly higher for international adoptees (Δ = $2047.51 [$128.35 to $3966.66], P = .038). Medicaid-adjusted surgeon payments averaged $1006 more for adoptees ([-$394.19 to $2406.98], P = .158). CONCLUSIONS: Hospital and anesthesiology costs for adoptee palate repair were highly variable but did not differ significantly from those for nonadoptees. Partly due to payer mix, surgeon reimbursement was somewhat higher for international adoptees. No difference in total payment was found.

An Assessment of 30-Day Complications in Primary Cleft Palate Repair: A Review of the 2012 ACS NSQIP Pediatric.

OBJECTIVE: This study uses the American College of Surgeons Pediatric National Surgical Quality Improvement Program (ACS NSQIP Pediatric), a multicenter database, to identify risk factors for complications after cleft palate repair. DESIGN: Patients undergoing Current Procedural Terminology (CPT) codes 42200 and 42205 were extracted from the 2012 ACS NSQIP Pediatric. Patients older than 36 months or those who had undergone an additional surgery that altered the risk were excluded. Outcome variables were combined to create a complication variable. Fisher's exact, Pearson chi-square, and Wilcoxon rank-sum tests were used for analysis. RESULTS: Eligibility criteria were met by 751 patients. Of these, 192 (25.6%) had unilateral clefts, 146 (19.4%) bilateral, and 413 (55.0%) were unspecified. The average age at time of surgery for those without and with complications was 421.1 ± 184.8 and 433.6 ± 168.0 days, respectively (P = .76). Of the 21 (2.8%) patients with complications, respiratory complications were the most common. Risk factors associated with complications included American Society of Anesthesiologists classification of 3 (P = .003), nutritional support (P = .013), esophageal/gastric/intestinal disease (P = .016), oxygen support (P = .003), structural pulmonary/airway abnormality (P = .011), and impaired cognitive status (P = .009). Patients undergoing concurrent laryngoscopy (P = .048) or other surgeries (P = .047) were also found to be associated with increased complications. The 30-day fistula rate was 0.5%, and the readmission rate was 1.9%. CONCLUSION: Perioperative complications for primary palatoplasty were 2.8% according to the ACS NSQIP Pediatric. Preoperative patient-related factors as well as concurrent surgeries may affect 30-day complication rates. These results help target those at greater risk for complications and allow for appropriate interventions to mitigate risks.

An Assessment of 30-Day Complications in Primary Cleft Lip Repair: A Review of the 2012 ACS NSQIP Pediatric.

OBJECTIVE: The aim of this study is to identify risk factors associated with complications and readmissions following cleft lip repair using the multicenter American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric. DESIGN: Patients undergoing CPT codes 40700, 40701, and 40702 were extracted from the ACS NSQIP Pediatric. Fisher exact, χ(2), and rank-sum tests were used to evaluate risk factors. RESULTS: Of the 525 patients included, 4.2% had complications, with respiratory complications being the most common. Risk factors included congenital malformations (P = .001), ventilator dependence (P = .002), oxygen support (P = .016), tracheostomy (P = .005), esophageal/gastric/intestinal disease (P = .007), impaired cognitive status (P = .034), acquired central nervous system abnormality (P = .040), nutritional support (P = .001), major and severe cardiac risk factors (P = .011 and P = .005), and an American Society of Anesthesiologists score of 3 (P = .002). In addition, complications were associated with undergoing a one-stage bilateral repair (P = .045) or concomitant ear, nose, and throat procedure (P = .045). The readmission rates for ambulatory patients and inpatients were 2.6% and 4.9% (P = .556), with an overall readmission rate of 4.6%. Ambulatory patients were older (P = .005) and had shorter operative times (P < .001). CONCLUSIONS: Perioperative complications are low following cleft lip repair, with respiratory complications being the most common. Readmission rates of 4.6% are higher than expected, and insight into predictors of complications will allow surgeons to identify patients who could benefit from additional resources.

The prevalence of strabismus in unilateral coronal synostosis.

BACKGROUND: While there is a clear correlation between unilateral coronal synostosis (UCS) and ocular motility abnormalities, the literature provides little information as to the true epidemiology of strabismus, or the underlying etiology of these paralleled pathologies. The purpose of this study is to investigate the rate of oculomotor abnormalities associated with UCS and its management. METHODS: A retrospective review of all patients identified to have single-suture, nonsyndromic UCS treated by fronto-orbital advancement at a tertiary craniofacial referral center from 1977 to 2013 was performed. Inclusion criteria mandated complete medical, surgical, and ophthalmological records. Patients were evaluated for strabismus both preoperatively and postoperatively, and as to whether eye muscle surgery was performed. RESULTS: A total of 181 patients underwent treatment for UCS at our institution during the study period, of which 79 met the inclusion criteria. Twenty-nine patients had strabismus prior to any craniofacial surgical intervention. Following fronto-orbital advancement, 23 patients (46 %) developed a new onset strabismus. Fifty-five patients had no change in their preoperative ocular examination, and one patient had resolution of preoperative strabismus. Of the 51 patients who had postoperative strabismus, 30 went on to have eye muscle surgery. There were no statistically significant differences in gender (p=0.477), race (p=0.395), sidedness of suture involvement (p=0.552), or age at intervention (p=0.66) in comparing the group with new postoperative strabismus and those without. CONCLUSIONS: This study sheds new light on the prevalence of strabismus in UCS, and more importantly, the risk of developing strabismus in the setting of conventional fronto-orbital advancement. This data will allow more accurate preoperative counseling and reinforces the important role of ophthalmologists as members of the multidisciplinary craniofacial team.

Myofascial Closure of Intradural Inclusion Cysts following in utero Myelomeningocele Repair.

Myelomeningocele is one of the most common congenital malformations. A randomized controlled trial, known as the Management of Myelomeningocele Study (MOMS), demonstrated that closure during the fetal period can be performed relatively safely and be of significant benefit to patients. However, postnatally, patients can develop resultant symptoms from a tethered cord and inclusion cysts; this often requires surgical treatment. Repeat surgery in this population can be challenging due to the age of the patients, the extent of surgical exposure needed and the need for resection of dermal and epidermal tissues in the midline. We describe our approach for closure of these complex defects using lateral fasciocutaneous flaps with relaxing incisions made in the posterior axillary line, in order to minimize tension and maximize soft tissue coverage of the midline.

In support of using computer-aided design and modeling for periorbital osteotomies.

Three-dimensional virtual surgical planning using computer-aided design and modeling (CAD/CAM) has gained popularity in planning complex orthognathic and osteocutaneous free flap reconstructions of the head and neck because of its ability to guide complex geometric planning in three-dimensional space and save time in the operating room. The purpose of this study was to review our experience using CAD/CAM concepts in periorbital osteotomies. Three complex periorbital osteotomies were planned and performed: 1 case of bilateral vertical and horizontal orbital dystopia, 1 case of a Monobloc-Le Fort II in an Apert patient, and 1 case of recurrent hypertelorism in a patient with craniofrontal nasal dysplasia. The patients' charts were reviewed, including photographs, medical records, and CAD/CAM plans. The CAD/CAM planning sessions were held 3 to 6 weeks preoperatively and lasted approximately 1 hour. Both cutting guides and positioning guides were used, translating to significant precision of both the osteotomy as well as the final position of the orbits. Qualitatively, the cutting and positioning guides were easy to use and improved operating room efficiency. To conclude, in our hands, CAD/CAM virtual surgical planning is safe and effective in the performance of complex periorbital osteotomies. More work is needed to more clearly define surgical indications for this costly, new technology.

Distraction Osteogenesis Versus Conventional Fronto-Orbital Advancement for the Treatment of Unilateral Coronal Synostosis: A Comparison of Perioperative Morbidity and Short-Term Outcomes.

Fronto-orbital advancement and remodeling (FOAR) remains the most widely practiced treatment of unicoronal craniosynostosis (UCS) despite recent studies of ocular dysfunction and aesthetic shortcomings in the long-term. The aim of the study was to compare perioperative morbidity and short-term outcomes of a recently developed, nondevascularizing, distraction-based treatment of UCS with conventional FOAR. To do so, the authors compared the first 6 patients who were treated with a new osteotomy/distraction approach to the last 6 patients who underwent traditional FOAR for the treatment of UCS with regards to demographics, operative details, perioperative morbidity, and short-term outcomes.Between July 2012 and June 2014, 6 patients underwent each procedure. Duration of surgery and length of hospital stay in the distraction group were on average 2 hours 7 minutes and 3.4 days, respectively, significantly less than in the traditional FOAR group (P = 0.039, P = 0.032, respectively). Perioperative blood loss averaged 169 mL, which trended toward less than in the traditional group (mean of 400 mL, P = 0.065). Patients undergoing conventional compared with distraction osteogenesis-mediated FOAR were significantly more likely to develop new-onset strabismus postoperatively (odds ratio 15.4; P = 0.0384). All 12 patients completed therapy without complications and with Whitaker grade I results at latest follow-up.In the perioperative period, distraction-mediated cranial vault remodeling provides similar correction of the aesthetic deformity associated with UCS and an improved morbidity profile. Longer follow-up is needed to determine how distraction compares with FOAR with respect to neuropsychologic and long-term aesthetic outcomes.

New-onset craniosynostosis after posterior vault distraction osteogenesis.

The aims of this study were to document the incidence of new-onset craniosynostosis (NOC) after posterior vault distraction osteogenesis (PVDO), to determine risk factors for the development of NOC, and to deduce the cranial ramifications of NOC. An institutional review board-approved retrospective review of all patients who underwent PVDO at the Children's Hospital of Philadelphia was performed. Demographics, perioperative data, as well as preoperative and postoperative three-dimensional computed tomographic scans were analyzed. Suture patency preoperatively and postoperatively was recorded.Thirty patients underwent PVDO for suspected increased intracranial pressure and/or severe turribrachicephaly from 2008 to 2013. Twenty-four patients had syndromic diagnoses. The average age at the time of PVDO was 2.03 years. Distraction distances ranged from 19 to 40 mm, with an average of 28.7 mm. Among the 19 patients who had patent lambdoid sutures before PVDO, new-onset lambdoid fusion was seen in 17 patients after PVDO (89.5%), whereas the suture remained open in 2 patients (10.5%). New-onset lambdoid fusion was not significantly associated with age at distraction (P = 0.28), sex (P = 0.47), length of distraction (P = 0.93), or diagnosis (P = 0.61). Similarly, new-onset sagittal synostosis was not associated with age at distraction (P = 0.06), sex (P = 0.64), length of distraction (P = 0.83), or diagnosis (P = 0.25). None of the patients who developed NOC had characteristic head shape changes such as mastoid bulges or scaphocephaly. New-onset lambdoid and sagittal synostoses occur frequently after PVDO. Although the diagnosis of NOC is obvious radiographically, the clinical importance of the diagnosis morphometrically, neurodevelopmentally, and in cranial growth has yet to be fully investigated.

Distinguishing Goldenhar Syndrome from Craniofacial Microsomia.

Goldenhar syndrome is characterized by the typical features of craniofacial microsomia (CFM) with the addition of epibulbar dermoids and vertebral anomalies. The aim of this study is to examine the objective differences between patients carrying a diagnosis of Goldenhar syndrome to those diagnosed with CFM. Thus, we performed an Institutional Review Board-approved retrospective chart review on all patients who presented with a diagnosis of CFM or Goldenhar syndrome from January 1990 to December 2012. Demographic, diagnosis, OMENS+ classification, accompanying diagnoses, and radiographic data were collected. For subjective analysis, subgroups were designed based on the diagnosis Goldenhar syndrome or CFM per history. For objective analysis, subgroups were designed based on the presence of epibulbar dermoids and/or vertebral anomalies. The cohorts were compared with respect to associated medical abnormalities and severity of CFM features. One hundred thirty eight patients met inclusion criteria. Epibulbar dermoids and vertebral anomalies were seen in 17% and 34% of the patients, respectively. Only 10 patients (7.2%) had both epibulbar dermoids and vertebral anomalies. The subjective "Goldenhar" group (N = 44, 32%) was found to have a higher percentage of bilaterally affected patients (P = 0.001), a more severe mandibular deformity (P = <0.001), a more severe soft tissue deformity (P = 0.01), and a higher incidence of macrostomia (P = 0.003). In the objective subgroup analysis, the only significant difference was found in the degree of soft tissue deficiency (P = 0.049). The diagnostic criteria of Goldenhar syndrome remain unclear, thereby making clinical use of the term "Goldenhar" inconsequential. Goldenhar syndrome is over diagnosed subjectively in patients who show more severe CFM features.

Cranial base deviation in hemifacial microsomia by craniometric analysis.

Although facial asymmetry in hemifacial microsomia (HFM) is well documented in the literature, no studies have concentrated on the morphology of the cranial base. This study aimed to evaluate the endocranial morphology in patients with HFM. Consecutive patients with unilateral HFM treated at a craniofacial center from 2000 to 2012 were included. The patients were grouped according to severity on the basis of the Kaban-Pruzansky classification: mild (0-1), moderate (2a), and severe (2b-3). Skull base angulation and transverse craniometric measures were recorded and then compared with those of age-matched controls. A total of 30 patients (14 males, 16 females) averaging 7.5 years of age (range, 1.1-15.7 y) were included. Four patients were classified as mild; 12, as moderate; and 14, as severe. The mean cranial base angle was found to be between 179 and 181 degrees with no significant difference between the severity groups (P = 0.57). The mean cranial base angle did not differ significantly in the patients compared with the controls(179.6 vs 180.0; P = 0.51) No significant differences between the affected and unaffected sides in the patients were found in distances from the midline to hypoglossal canal, internal acoustic meatus, lateral carotid canal, medial carotid canal, foramen ovale, and rotundum. There were no significant differences in transverse measurements between the severity classes using the same landmarks (P = 0.46, P = 0.30, P = 0.40, P = 0.25, P = 0.57, and P = 0.76, respectively). The cranial base axis is not deviated in the patients with HFM compared with the age-matched controls, and there exists little difference in endocranial morphologic measurements with increasing severity of HFM. These data are interesting, given the role of the cranial base in facial growth and the varying hypotheses regarding the mechanism of disease in HFM.

Simultaneous unicoronal and sagittal distraction osteogenesis for the treatment of nonsyndromic multisutural craniosynostosis.

We present a case of multiplanar distraction osteogenesis for the simultaneous treatment of sagittal and unicoronal craniosynostosis in a nonsyndromic 2-month-old boy. Unilateral fronto-orbital advancement and sagittal suturectomy were performed. Distracters were fixed orthogonally in the sagittal and coronal positions to distract the affected coronal and sagittal sutures. The devices achieved 20 and 22 mm of advancement in the coronal and sagittal locations. A total intracranial volume increase of 62% was noted at 6 months' follow-up. This preliminary report demonstrates the procedure's short-term safety; future investigation is needed over the long term to determine its efficacy.

Perioperative complications associated with intracranial procedures in patients with nonsyndromic single-suture craniosynostosis.

Within the diagnosis "craniosynostosis," there is a subset of patients who present with isolated, nonsyndromic, single-suture involvement. This study evaluates perioperative complications in this specific subset of patients over 4 decades at a single institution. To do so, we performed a retrospective review on consecutive patients undergoing correction of single-suture synostosis from May 1977 to January 2013 at a tertiary pediatric craniofacial center. Demographic information, operative details, and perioperative course were collected. Complications were categorized as either major or minor. A χ(2) test and Fisher exact test were used to compare all categorical variables. Continuous variables were analyzed using Wilcoxon rank-sum and Kruskal-Wallis tests.Seven hundred forty-six patients underwent surgical correction of nonsyndromic craniosynostosis. Of these, there were 307 (41.2%) sagittal, 201 (26.9%) metopic, and 238 (31.9%) unicoronal. Thirty-four patients had complications (4.6%). Eight were considered major (1.1%), including one postoperative mortality in a patient with hypoplastic left-sided heart syndrome. Minor complications occurred in 26 patients (3.5%) and included subgaleal hematoma (n = 3), seroma (n = 4), and superficial wound infection (n = 5). Metopic and sagittal suture involvement was significantly associated with a higher complication rate (P = 0.04). A child with isolated single suture synostosis and any comorbidity had a significantly greater risk of any complication (P < 0.001; odds ratio, 3.8) and specifically an increased risk of major complication (P = 0.031; odds ratio, 6.0). Subclassification of patients by time period yielded no statistically significant changes in perioperative morbidity. To conclude, these data allow us to counsel families more accurately with regard to morbidity and mortality and may potentially serve as a benchmark for future quality improvement work.

New Pattern of Sutural Synostosis Associated With TWIST Gene Mutation and Saethre-Chotzen Syndrome: Peace Sign Synostosis.

The authors present a new and unique pattern of sutural fusion "peace sign synostosis" (PSS) characterized by synostosis of the metopic, bicoronal, and sagittal sutures and associated with abnormalities of the TWIST1 gene known to be associated with Saethre-Chotzen syndrome (SCS). To do so, we performed a retrospective review of patients with bicoronal, metopic, and at least partial anterior sagittal synostoses at the Children's Hospital of Philadelphia and Seattle Children's Hospital. Patients' demographics, genetic analysis, perioperative and clinic notes were reviewed. Five patients were identified with PSS and abnormalities of TWIST1 consistent with SCS. One patient, with the longest follow-up of 7 years, underwent 5 intracranial procedures and required a ventriculoperitoneal (VP) shunt. The remaining 4 patients underwent posterior cranial vault distraction as the initial procedure, followed by anterior cranial vault remodeling. Two patients required a VP shunt. To conclude, synostosis of the metopic, bicoronal, and sagittal sutures (PSS) appears to be associated with SCS and produces a characteristic skull morphology that can be readily identified on physical examination. Early data suggest a high rate of reoperation, increased necessity for a VP shunt, and potential complications. Of note, this novel phenotype had not been previously observed at our respective institutions, reported in the literature, or observed in association with TWIST1 abnormalities as described in association with SCS.

The spheno-occipital synchondrosis fuses prematurely in patients with Crouzon syndrome and midface hypoplasia compared with age- and gender-matched controls.

PURPOSE: Premature closure of the spheno-occipital synchondrosis (SOS) has been associated with midface hypoplasia in animal models and patients with specific forms of syndromic craniosynostosis. The present study aimed to characterize SOS fusion in patients with Crouzon syndrome. PATIENTS AND METHODS: A case-control study was performed in patients with Crouzon syndrome treated at the Children's Hospital of Philadelphia from 1984 to 2012. The cases included patients with Crouzon syndrome and at least 1 high-quality computed tomography (CT) scan in which SOS patency could be assessed. Age- and gender-matched control CT scans were identified for comparison. The patient age at the CT scan was evaluated as the predictor, with SOS patency identified as the outcome variable. Three independent reviewers with high inter-rater reliability graded the SOS patency as open, partially fused, or completely fused. The Wilcoxon rank sum test was used to compare the Crouzon group and the controls. RESULTS: During the study period, 30 patients were identified with Crouzon syndrome. A total of 24 patients, all with midface hypoplasia and with 112 cranial CT scans, met the inclusion criteria. Accordingly, 112 age- and gender-matched control CT scans were assessed. No patient in the control group had midface hypoplasia. Within the Crouzon group, the average age at complete closure (14.0 ± 3.4 years) evident on the CT scan was significantly younger than that in the control group (16.6 ± 2.2 years; P = .0152). The average age when the scans showed complete patency of the SOS in the Crouzon group (1.3 ± 1.1 years) was significantly younger than that in the control group (3.2 ± 2.3 years; P = .0001). CONCLUSIONS: The SOS closes significantly earlier in patients with Crouzon syndrome compared with age- and gender-matched controls. The strong statistical correlation supports premature closure of the SOS as a possible mechanistic contributor to midface hypoplasia.

The impact of living with severe lower extremity lymphedema: a utility outcomes score assessment.

BACKGROUND: Debilitating lower extremity lymphedema can be either congenital or acquired. Utility scores are an objective measure used in medicine to quantify degrees of impact on an individual's life. Using standardized utility outcome measures, we aimed to quantify the health state of living with severe unilateral lower extremity lymphedema. METHODS: A utility outcomes assessment using visual analog scale, time trade-off, and standard gamble was used for lower extremity lymphedema, monocular blindness, and binocular blindness from a sample of the general population and medical students. Average utility scores were compared using a paired t test. Linear regression was performed using age, race, and education as independent predictors. RESULTS: A total of 144 prospective participants were included. All measures [visual analog scale, time trade-off, and standard gamble; expressed as mean (SD)] for unilateral lower extremity lymphedema (0.50 ± 0.18, 0.76 ± 0.22, and 0.76 ± 0.21, respectively) were significantly different (P < 0.001) from the corresponding scores for monocular blindness (0.64 ± 0.18, 0.84 ± 0.16, and 0.83 ± 0.17, respectively) and binocular blindness (0.35 ± 0.17, 0.61 ± 0.28, and 0.62 ± 0.26, respectively). CONCLUSIONS: We found that a sample of the general population and medical students, if faced with severe lymphedema, is willing to theoretically trade 8.64 life-years and undergo a procedure with a 24% risk of mortality to restore limb appearance and function to normal. These findings provide a frame of reference regarding the meaning of a diagnosis of severe lower extremity lymphedema to a patient and will allow objective comparison with other health states.

Does breast reconstruction impact the decision of patients to pursue cosmetic surgery?

BACKGROUND: Breast reconstruction is an integral component of breast cancer treatment, often aiding in restoring a patient's sense of femininity. However, many patients choose to have subsequent cosmetic surgery. The purpose of this study is to investigate the reasons that motivate patients to have cosmetic surgery after breast reconstruction. METHODS: The authors performed a retrospective study examining patients who had breast reconstruction and subsequent cosmetic surgery at the University of Pennsylvania Health System between January 2005 and June 2012. This cohort received a questionnaire assessing the influences and impact of their reconstructive and cosmetic procedures. RESULTS: A total of 1,214 patients had breast reconstruction, with 113 patients (9.3%) undergoing cosmetic surgery after reconstruction. Of 42 survey respondents, 35 had autologous breast reconstruction (83.3%). Fifty-two cosmetic procedures were performed in survey respondents, including liposuction (26.9%) and facelift (15.4%). The most common reason for pursuing cosmetic surgery was the desire to improve self-image (n = 26, 61.9%), with 29 (69.0%) patients feeling more self-conscious of appearance after reconstruction. Body image satisfaction was significantly higher after cosmetic surgery (P = 0.0081). Interestingly, a multivariate analysis revealed that patients who experienced an improvement in body image after breast reconstruction were more likely to experience a further improvement after a cosmetic procedure (P = 0.031, OR = 17.83). Patients who were interested in cosmetic surgery prior to reconstruction were also more likely to experience an improvement in body image after cosmetic surgery (P = 0.012, OR = 22.63). CONCLUSION: Cosmetic surgery may improve body image satisfaction of breast reconstruction patients and help to further meet their expectations.

Screening for obstructive sleep apnea in children treated at a major craniofacial center.

Timely diagnosis of obstructive sleep apnea (OSA) in patients with craniofacial disorders may help prevent long-term adverse sequelae of upper airway obstruction, namely pulmonary hypertension, failure to thrive, and impaired neurocognitive development. Currently, little is known about the incidence of OSA in this high-risk population. A prospective study examining the incidence of positive screening for OSA in patients cared for by the craniofacial team at a large, urban referral center was performed. From January 2011 to August 2013, all patient families were asked to complete the Pediatric Sleep Questionnaire. This validated tool has a sensitivity of 85% and specificity of 87% in predicting a positive sleep study when the ratio of positive-total responses is 0.33 or greater. Screening results were evaluated via Chi-squared and Fisher tests according to demographic and clinical variables. A total of 234 children seen in our craniofacial clinic completed the Pediatric Sleep Questionnaire. The mean screening age was 8.38 years, and 47% were male (110/234). Total incidence of positive OSA screening was 28.2% (66/234). Of the total population, 128 patients had an underlying syndrome (54.7%), whereas 106 patients were nonsyndromic (45.3%). Both groups were at equivalent risk for screening positive for OSA (28.1% versus 28.3%, P = 1.0). Among children with a craniofacial diagnosis, patients with a cleft lip and/or palate were at equivalent risk for screening positive for OSA as patients without a cleft (25.5% versus 32.6%, P = 0.24). The OSA symptoms affect almost one third of patients seen by our craniofacial team. Syndromic and nonsyndromic patients seem to be at equivalent risk as those patients with and without an oropharyngeal cleft. Future work will correlate these findings with formal polysomnography and may serve to heighten awareness of OSA in this at-risk population.