Selective nerve transfers to restore shoulder abduction and flexion in acute flaccid myelitis: A case report.

Acute flaccid myelitis (AFM) is a polio-like condition predominantly affecting children that is characterized by acute-onset, asymmetric flaccid paralysis, often preceded by a prodromal fever or viral illness. With prompt diagnosis and early surgical referral, nerve transfers may be performed to improve function. Highly selective nerve transfers are ideal to preserve existing functions while targeting specific deficits. In this report, we present a case of a double fascicular nerve transfer of median and ulnar nerve fascicles to the axillary nerve, combined with selective transfer of the spinal accessory nerve to the supraspinatus branch of the suprascapular nerve, performed for a 5-year-old girl who developed AFM after an upper respiratory infection. Six months after the onset of the patient's symptoms, the patient had continued weakness of shoulder flexion and abduction, atrophy of the deltoid, and supraspinatus muscles, though needle electromyography revealed a functioning infraspinatus muscle. The patient had no post-operative complications and at 2 years of postoperative follow up achieved shoulder abduction and flexion Active Movement Scale scores of 7/7 compared to preoperative scores of 2/7, with no loss of function in the donor nerve domains. The patient showed active shoulder abduction against gravity to 90° from 30° preoperatively and shoulder flexion to 180° from 15° preoperatively. This case report shows that highly selective nerve transfers may preserve existing functions while targeting specific deficits. A double fascicular transfer from the median and ulnar nerves to axillary nerve may provide abundant axons for functional recovery.

Unraveling a Major Burden of Orofacial Clefts Analyses: Classification of Cleft Palate Fistulas by Cleft Surgeons.

OBJECTIVE: The objective of this study was to investigate how cleft surgeons classify palatal fistulas. We focused on three different anatomical locations (ie, hard palate, soft palate, junction hard/soft palate) to analyze agreement/disagreement at various anatomical locations. DESIGN: Cross-sectional survey study. PARTICIPANTS: Participants in an international webinar that focused on palatal fistula treatment were included. INTERVENTION: Participants were presented with a survey pre- and post-webinar. MAIN OUTCOMES: Frequency of used classification systems for classifying oronasal fistulas and the inter-rater reliability of the Pittsburgh classification system. RESULTS: A total of 141 participants completed the questionnaires prior to the webinar and 109 participants completed the survey after the webinar. In total, four classification systems were used (ie, Pittsburgh, Pakistan Comprehensive Fistula Classification [PCFC], anatomical and 'other'). The Pittsburgh classification was the most commonly used system in all cases. However, Pittsburgh inter-rater reliability was low (κ = 0.136 pre-webinar, and κ = 0.174 post-webinar). Surprisingly, a substantial shift was observed from the anatomical to Pittsburgh classification after the webinar, indicating increased awareness of the usability of the Pittsburgh classification system. CONCLUSIONS: This study demonstrates a large heterogeneity with regards to the classification of cleft palate fistulas. Interestingly, a shift was observed from the anatomical to Pittsburgh classification after the webinar. However, the inter-rater reliability for using the Pittsburgh classification was low. Classifying palatal fistulas in a homogenous fashion could enhance comparison of primary palate repair and could improve treatment of palatal fistulas.

Bilateral Cleft lip Simulation.

OBJECTIVE: To evaluate the features, anatomic accuracy, and educational value of a high-fidelity bilateral cleft lip simulator. DESIGN: Evaluation of the simulator by expert cleft surgeons after performing a simulated bilateral cleft lip repair. SETTING: The simulator was evaluated by the surgeons during the Latin American Craniofacial Association meeting. PARTICIPANTS: Eleven experienced cleft surgeons evaluated the simulator. The cleft surgeons were selected based on their availability during the meeting. INTERVENTIONS: The participants performed a simulated bilateral cleft lip repair. They were each provided with a questionnaire assessing the simulator's features, realism and value as a training tool. MAIN OUTCOME MEASURE (S): The main outcome measure are the scores obtained from a Likert-type questionnaire assessing the simulators features, realism and value. RESULTS: Overall, the surgeons agreed with the simulator's realism and anatomic accuracy (average score of 3.7 out of 5). Overall, the surgeons strongly agreed with the value of the simulator as a training tool (average score of 4.6 out of 5). CONCLUSIONS: A high-fidelity bilateral cleft lip simulator was developed that is realistic and valuable as a training tool. The simulator provides a comprehensive training platform to gain hands-on experience in bilateral cleft lip repair before operating on real patients.

Surgery for Velopharyngeal Insufficiency Following Cleft Palate Repair: An Audit of Contemporary Practice and Proposed Schema of Techniques and Variations.

OBJECTIVE: Surgical treatment of velopharyngeal insufficiency (VPI) includes a wide array of procedures. The purpose of this study was to develop a classification for VPI procedures and to describe variations in how they are performed.Design/participants/setting/outcomes: We completed an in-depth review of the literature to develop a preliminary schema that encompassed existing VPI procedures. Forty-one cleft surgeons from twelve hospitals across the USA and Canada reviewed the schema and either confirmed that it encompassed all VPI procedures they performed or requested additions. Two surgeons then observed the conduct of the procedures by surgeons at each hospital. Standardized reports were completed with each visit to further explore the literature, refine the schema, and delineate the common and unique aspects of each surgeon's technique. RESULTS: Procedures were divided into three groups: palate-based surgery; pharynx-based surgery; and augmentation. Palate-based operations included straight line mucosal incision with intravelar veloplasty, double-opposing Z-plasty, and palate lengthening with buccal myomucosal flaps. Many surgeons blended maneuvers from these three techniques, so a more descriptive schema was developed classifying the maneuvers employed on the oral mucosa, nasal mucosa, and muscle. Pharynx-based surgery included pharyngeal flap and sphincter pharyngoplasty, with variations in design for each. Augmentation procedures included palate and posterior wall augmentation. CONCLUSIONS: A comprehensive schema for VPI procedures was developed incorporating intentional adaptations in technique. There was substantial variation amongst surgeons in how each procedure was performed. The schema may enable more specific evaluations of surgical outcomes and exploration of the mechanisms through which these procedures improve speech.

Measuring the Unilateral Cleft Lip Nasal Deformity: Lateral Deviation of Subnasale Is a Clinical and Morphologic Index of Unrepaired Severity.

OBJECTIVE: Objective measurement of pre-operative severity is important to optimize evidence-based practices given that the wide spectrum of presentation likely influences outcomes. The purpose of this study was to determine the correlation of objective measures of form with a subjective standard of cleft severity. DESIGN: 3D images were ranked according to severity of nasal deformity by 7 cleft surgeons so that the mean rank could be used as the severity standard. PATIENTS: 45 patients with unilateral cleft lip and 5 normal control subjects. INTERVENTIONS: Each image was assessed using traditional anthropometric analysis, 3D landmark displacements, and shape-based analysis to produce 81 indices for each subject. MAIN OUTCOME: The correlation of objective measurements with the clinical severity standard. RESULTS: Lateral deviation of subnasale from midline was the best predictor of severity (0.86). Other strongly-correlated anthropometric measurements included columellar angle, nostril width ratio, and lateral lip height ratio (0.72, 0.80, 0.79). Almost all shape-based measurements had tight correlation with the severity standard, however, dorsum deviation and point difference nasolabial symmetry were the most predictive (0.84, 0.82). CONCLUSIONS: Quantitative measures of severity transcend cleft type and can be used to grade clinical severity. Lateral deviation of subnasale was the best measure of severity and may be used as a surrogate of uncoupled premaxillary growth; it should be recorded as an index of pre-operative severity with every cleft lip repair. The correlation of other measures evaluated clarify treatment priorities and could potentially be used to grade outcomes.

The First Hybrid International Educational Comprehensive Cleft Care Workshop.

OBJECTIVE: Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. DESIGN: Cross-sectional survey-based evaluation. SETTING: International comprehensive cleft care workshop. PARTICIPANTS: Total of 489 participants. INTERVENTIONS: Three-day simulation-based hybrid comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. RESULTS: The workshop included 489 participants from 5 continents. The response rate was 39.9%. Participants perceived financial factors (30.3%) the most significant barrier and improvement in training (39.8%) as the most important intervention to overcome barriers facing cleft care delivery in low to middle-income countries. All participants reported a high level of satisfaction with the workshop and a strong positive perceived short-term impact on their practice. Importantly, while this was true for both in-person and virtual attendees, in-person attendees reported a significantly higher satisfaction with the workshop (28.63 ± 3.08 vs 27.63 ± 3.93; P = .04) and perceived impact on their clinical practice (22.37 ± 3.42 vs 21.02 ± 3.45 P = .01). CONCLUSION: Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

Partial tibial nerve transfer for foot drop from deep peroneal palsy: Lessons from three pediatric cases.

Peroneal nerve palsy with resultant foot drop has significant impacts on gait and quality of life. Traditional management includes ankle-foot-orthosis, tendon transfer, and arthrodesis-each with certain disadvantages. While nerve transfers for peroneal nerve injury have been reported in adults, with variable results, they have not been described in the pediatric population. We report the use of partial tibial nerve transfer for foot drop from deep peroneal nerve palsy in three pediatric patients. The first sustained a partial common peroneal nerve laceration and underwent transfer of a single tibial nerve branch to deep peroneal nerve 7 months after injury. Robust extensor hallucis longus and extensor digitorum longus reinnervation was obtained without satisfactory tibialis anterior function. The next patient sustained a thigh laceration with partial sciatic nerve injury and underwent transfer of two tibial nerve branches directly to the tibialis anterior component of deep peroneal nerve 9 months after injury. The final patient sustained a blast injury to the posterior knee and similarly underwent a double fascicular transfer directly to tibialis anterior 4 months after injury. The latter two patients obtained sufficient strength (MRC 4-5) at 1 year to discontinue orthosis. In all patients, we used flexor hallucis longus and/or flexor digitorum longus branches as donors without postoperative loss of toe flexion. Overall, our experience suggests that early double fascicular transfer to an isolated tibialis anterior target, combined with decompression, could produce robust innervation. Further study and collaboration are needed to devise new ways to treat lower extremity nerve palsies.

Protocol for a Prospective Observational Study of Revision Palatoplasty Versus Pharyngoplasty for Treatment of Velopharyngeal Insufficiency Following Cleft Palate Repair.

OBJECTIVE: To present the design and methodology for an actively enrolling comparative effectiveness study of revision palatoplasty versus pharyngoplasty for the treatment of velopharyngeal insufficiency (VPI). DESIGN: Prospective observational multicenter study. SETTING: Twelve hospitals across the United States and Canada. PARTICIPANTS: Individuals who are 3-23 years of age with a history of repaired cleft palate and a diagnosis of VPI, with a total enrollment target of 528 participants. INTERVENTIONS: Revision palatoplasty and pharyngoplasty (either pharyngeal flap or sphincter pharyngoplasty), as selected for each participant by their treatment team. MAIN OUTCOME MEASURE(S): The primary outcome is resolution of hypernasality, defined as the absence of consistent hypernasality as determined by blinded perceptual assessment of a standard speech sample recorded twelve months after surgery. The secondary outcome is incidence of new onset obstructive sleep apnea. Statistical analyses will use propensity score matching to control for demographics, medical history, preoperative severity of hypernasality, and preoperative imaging findings. RESULTS: Study recruitment began February 2021. As of September 2022, 148 participants are enrolled, and 78 have undergone VPI surgery. Enrollment is projected to continue into 2025. Collection of postoperative evaluations should be completed by the end of 2026, with dissemination of results soon thereafter. CONCLUSIONS: Patients with VPI following cleft palate repair are being actively enrolled at sites across the US and Canada into a prospective observational study evaluating surgical outcomes. This study will be the largest and most comprehensive study of VPI surgery outcomes to date.

Establishing a Clinical Protocol for Velopharyngeal MRI and Interpreting Imaging Findings.

Traditional imaging modalities used to assess velopharyngeal insufficiency (VPI) do not allow for direct visualization of underlying velopharyngeal (VP) structures and musculature which could impact surgical planning. This limitation can be overcome via structural magnetic resonance imaging (MRI), the only current imaging tool that provides direct visualization of salient VP structures. MRI has been used extensively in research; however, it has had limited clinical use. Factors that restrict clinical use of VP MRI include limited access to optimized VP MRI protocols and uncertainty regarding how to interpret VP MRI findings. The purpose of this paper is to outline a framework for establishing a novel VP MRI scan protocol and to detail the process of interpreting scans of the velopharynx at rest and during speech tasks. Additionally, this paper includes common scan parameters needed to allow for visualization of velopharynx and techniques for the elicitation of speech during scans.

Crowdsourcing to Assess Speech Quality Associated With Velopharyngeal Dysfunction.

OBJECTIVE: To assess crowdsourced responses in the evaluation of speech outcomes in children with velopharyngeal dysfunction (VPD). DESIGN: Fifty deidentified speech samples were compiled. Multiple pairwise comparisons obtained by crowdsourcing were used to produce a rank order of speech quality. Ratings of overall and specific speech characteristics were also collected. Twelve speech-language pathologists (SLPs) who specialize in VPD were asked to complete the same tasks. Crowds and experts completed each task on 2 separate occasions at least 1 week apart. SETTING: On-line crowdsourcing platform. PARTICIPANTS: Crowdsource raters were anonymous and at least 18 years of age, North American English speakers with self-reported normal hearing. Speech-language pathologists were recruited from multiple cleft/craniofacial teams. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): Correlation of repeated assessments and comparison of crowd and SLP assessments. RESULTS: We obtained 6331 lay person assessments that met inclusion criteria via crowdsourcing within 8 hours. The crowds provided reproducible Elo rankings of speech quality, ρ(48) = .89; P <.0001, and consistent ratings of intelligibility and acceptability (intraclass correlation coefficient [ICC] = .87 and .92) on repeated assessments. There was a significant correlation of those crowd rankings, ρ(10) = .86; P = .0003, and ratings (ICC = .75 and .79) with those of SLPs. The correlation of more specific speech characteristics by the crowds and SLPs was moderate to weak (ICC < 0.65). CONCLUSIONS: Crowdsourcing shows promise as a rapid way to obtain large numbers of speech assessments. Reliability of repeated assessments was acceptable. Large groups of naive raters yield comparable evaluations of overall speech acceptability, intelligibility, and quality, but are not consistent with expert raters for specific speech characteristics such as resonance and nasal air emission.

A Toolbox of Surgical Techniques for Palatal Fistula Repair.

OBJECTIVE: To provide an inventory of oronasal fistula repair techniques alongside expert commentary on which techniques are appropriate for each fistula type. DESIGN: A 4-stage approach was used to develop a consensus on surgical techniques available for fistula repair: (1) in-person discussion of oronasal fistula cases among cleft surgeons, (2) development of a schema for fistula management using transcripts of the in-person case discussion, (3) evaluation of the preliminary schema via a web-based survey of additional cleft surgeons, and (4) revision of the management schema using survey responses. PARTICIPANTS: Six cleft surgeons participated in the in-person case discussion. Eleven additional surgeons participated in the web-based survey. Participants had diverse training experiences, having completed residency and fellowship at 20 different hospitals. RESULTS: A schema for fistula management was developed, organized by fistula location. The schema catalogues all viable approaches for each location. For fistulae involving the soft palate, the schema stresses the importance of evaluating for velopharyngeal insufficiency (VPI) and incorporating VPI management into fistula repair. For fistulae involving the hard palate, the schema separately enumerates the techniques available for nasal lining repair and for oral lining repair in each region. The schema also catalogues the diversity of approaches to lingual- and labioalveolar fistula, including variation in timing, orthodontic preparation, and simultaneous alveolar bone grafting. CONCLUSIONS: This study employed consensus methods to create a comprehensive inventory of available fistula repair techniques and to identify preferential techniques among a diverse group of surgeons.

Measuring overweight and obesity in Chinese American children using US, international and ethnic-specific growth charts.

OBJECTIVE: The aim is to determine the disparity between the overweight and obesity prevalence of Chinese American school-aged children and adolescents as measured by the Centers for Disease Control and Prevention (CDC) growth reference and the prevalence as measured by international and ethnic-specific-growth references. DESIGN: This retrospective, cross-sectional study measured overweight and obesity prevalence among a paediatric population using the CDC, International Obesity Task Force (IOTF), World Health Organization (WHO) and an ethnic Chinese growth curve. SETTING: A community health centre in New York City, USA. PARTICIPANTS: Chinese American children aged 6-17 years in 2017 (N 9160). RESULTS: The overweight prevalence was 24 % (CDC), 23 % (IOTF), 30 % (WHO) and 31 % (China). The obesity prevalence was 10 % (CDC), 5 % (IOTF), 10 % (WHO) and 10 % (China). When disaggregated by age and sex, the difference was the most prominent in girls; using the China reference compared with using the CDC reference almost doubles the overweight prevalence (school-aged: 31 v. 17 %, P < 0·001, adolescent: 27 v. 14 %, P < 0·001) and the obesity prevalence (school-aged: 11 v. 5 %, P < 0·001, adolescent: 7 v. 4 %, P < 0·001). CONCLUSIONS: Use of the CDC reference compared with the Chinese ethnic-specific reference results in lower overweight and obesity prevalence in Chinese American girls. Almost half of the girls who were overweight and half of the girls who were obese were not identified using the CDC reference. Using ethnic-specific references or ethnic-specific cut-points may help improve overweight identification for Chinese American children.

Simulation-Based Comprehensive Cleft Care Workshops: A Reproducible Model for Sustainable Education.

OBJECTIVE: Evaluate simulation-based comprehensive cleft care workshops as a reproducible model for education with sustained impact. DESIGN: Cross-sectional survey-based evaluation. SETTING: Simulation-based comprehensive cleft care workshop. PARTICIPANTS: Total of 180 participants. INTERVENTIONS: Three-day simulation-based comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Number of workshop participants stratified by specialty, satisfaction with the workshop, satisfaction with simulation-based workshops as educational tools, impact on cleft surgery procedural confidence, short-term impact on clinical practice, medium-term impact on clinical practice. RESULTS: The workshop included 180 participants from 5 continents. The response rate was 54.5%, with participants reporting high satisfaction with all aspects of the workshop and with simulation-based workshops as educational tools. Participants reported a significant improvement in cleft lip (33.3 ± 5.7 vs 25.7 ± 7.6; P < .001) and palate (32.4 ± 7.1 vs 23.7 ± 6.6; P < .001) surgery procedural confidence following the simulation sessions. Participants also reported a positive short-term and medium-term impact on their clinical practices. CONCLUSION: Simulation-based comprehensive cleft care workshops are well received by participants, lead to improved cleft surgery procedural confidence, and have a sustained positive impact on participants' clinical practices. Future efforts should focus on evaluating and quantifying this perceived positive impact, as well reproducing these efforts in other areas of need.

Speech and Surgical Outcomes in Children With Veau Types III and IV Cleft Palate: A Comparison of Internationally Adopted and Nonadopted Children.

OBJECTIVE: This study compares speech and surgical outcomes in internationally adopted and nonadopted patients undergoing cleft palate repair, and examines the influence of age at initial palatoplasty. DESIGN: Retrospective cohort study setting: Tertiary Care Children's Hospital. PATIENTS: 70 international adoptees and 211 nonadoptees with Veau type III and IV clefts (without associated syndrome) repaired at our institution. OUTCOME MEASURES: Outcomes included VPI, compensatory misarticulations, intelligibility, nasal air emission, oronasal fistula, and secondary speech surgery. Speech evaluations completed near 5 years of age were gathered from a prospectively collected database. RESULTS: Adoptees underwent palatoplasty 5.2 months after arrival, a mean of 10.4 months later than nonadoptees. Adoptees were significantly more likely to develop moderate/severe VPI and trended toward more frequent need for secondary speech surgery. Oronasal fistula occurred at similar rates. Increased age at initial palatoplasty was a significant predictor of moderate to severe VPI, and need for secondary speech surgery. CONCLUSIONS: International adoptees undergo palatoplasty 10.4 months later than nonadoptees and are significantly more likely to develop moderate/severe VPI, with a trend toward increased secondary speech surgery. An association between treatment delay and moderate/severe VPI and secondary speech surgery has been demonstrated. While a causal relationship between delayed repair and inferior outcomes in international adoptees has not been proven, this data suggests that surgical intervention upon unrepaired cleft palates soon after adoption may be beneficial. The opportunity for a change in practice exists, as half of the 10.4-month relative delay in palate repair occurs postadoption.

Reliability of Oronasal Fistula Classification.

OBJECTIVE: Oronasal fistula is an important complication of cleft palate repair that is frequently used to evaluate surgical quality, yet reliability of fistula classification has never been examined. The objective of this study was to determine the reliability of oronasal fistula classification both within individual surgeons and between multiple surgeons. DESIGN: Using intraoral photographs of children with repaired cleft palate, surgeons rated the location of palatal fistulae using the Pittsburgh Fistula Classification System. Intrarater and interrater reliability scores were calculated for each region of the palate. PARTICIPANTS: Eight cleft surgeons rated photographs obtained from 29 children. RESULTS: Within individual surgeons reliability for each region of the Pittsburgh classification ranged from moderate to almost perfect (κ = .60-.96). By contrast, reliability between surgeons was lower, ranging from fair to substantial (κ = .23-.70). Between-surgeon reliability was lowest for the junction of the soft and hard palates (κ = .23). Within-surgeon and between-surgeon reliability were almost perfect for the more general classification of fistula in the secondary palate (κ = .95 and κ = .83, respectively). CONCLUSIONS: This is the first reliability study of fistula classification. We show that the Pittsburgh Fistula Classification System is reliable when used by an individual surgeon, but less reliable when used among multiple surgeons. Comparisons of fistula occurrence among surgeons may be subject to less bias if they use the more general classification of "presence or absence of fistula of the secondary palate" rather than the Pittsburgh Fistula Classification System.

Trauma care in a multiethnic population: effects of being undocumented.

BACKGROUND: Epidemiologic studies have shown that undocumented immigrants (UIs) display characteristics of having a low socioeconomic status and are primarily of ethnic minorities. These social determinants of health are known to be associated with diminished health care access and poor clinical outcomes. We therefore investigated the impact of documentation status on the clinical outcomes of patients with traumatic injuries. MATERIALS AND METHODS: We conducted a retrospective review of the trauma registry at our safety net institution for all adult patients who were admitted from 2010 to 2014. UIs were identified by the absence of a valid social security number within their medical records. Multivariate regression analysis was used to determine the impact of documentation status on in-hospital mortality, length of stay (LOS), and the odds of rehab placement. RESULTS: 4924 trauma patients met the study criteria, of which 1050 (21.3%) were UIs. There was no significant difference in mortality rates between the two populations. Multivariate regression analyses revealed a longer average LOS and a decreased likelihood for placement in an in-patient rehabilitation facility following hospitalization for UIs, even after accounting for insurance, age, injury severity, and other possible confounders known to affect these outcomes. CONCLUSIONS: There was no association between in-hospital mortality and documentation status; however, UIs had a longer average LOS and were less likely to be placed into rehab following their hospitalization. A longer LOS and a decreased likelihood for rehabilitation placement suggest that disparities in trauma care exist for UIs, putting them at risk for worse clinical and functional outcomes.

Measuring Symmetry in Children With Cleft Lip. Part 3: Quantifying Nasal Symmetry and Nasal Normalcy Before and After Unilateral Cleft Lip Repair.

OBJECTIVE: The purpose of this project was to develop objective computer-based methods to measure nasal asymmetry and abnormality in children undergoing treatment of unilateral cleft lip (UCL) and to determine the correlation of these measures to clinical expectations. PARTICIPANTS: Thirty infants with UCL undergoing cleft lip repair; 27 children with UCL aged 8 to 10 years who had previously undergone cleft lip repair; 3 control infants; 3 control children aged 8 to 10 years. INTERVENTIONS: To measure nasal symmetry, we used a process of depth mapping and calculated the Depth Area Difference. To measure abnormality, we used the reconstruction error from Principle Component Analysis (PCA) that was based upon characteristics of a dataset of over 2000 images of normal control subjects. MAIN OUTCOME MEASURES: Depth Area Difference and PCA Reconstruction Error for cleft type, changes with surgery, and individual subjects ranked according to cleft severity were assessed. RESULTS: Significant differences in Depth Area Difference and PCA Reconstruction Error were found between cleft types and found before and after surgery. Nasal symmetry and normalcy scores for infants with UCL approached those of controls after surgery, and there was a strong correlation with ranked cleft severity. For older children, measures of nasal symmetry and abnormality were better than infants prior to repair but worse than infants following UCL repair. CONCLUSIONS: Our computer-based 3D analysis of nasal symmetry and normalcy correlated with clinical expectations. Automated processing made measurement convenient. Use of these measures may help to objectively measure cleft severity and treatment outcome.

Harlequin Ichthyosis: A Surgical Perspective.

Limb constriction or encasement in patients with harlequin ichthyosis can cause tissue injury resulting in necrosis and auto-amputation. Surgical release of constrictive plaques has been previously demonstrated, but the perioperative and intraoperative considerations surrounding this infrequent intervention have not been discussed in detail. This report documents a case of harlequin ichthyosis requiring surgical treatment, focusing on the importance of early surgical consultation, risks of surgery, indications for and timing of surgical intervention, and the details of the operation.

Measuring Symmetry in Children With Cleft Lip. Part 2: Quantification of Nasolabial Symmetry Before and After Cleft Lip Repair.

OBJECTIVE: The first part of this study validated an automated computer-based method of identifying the three-dimensional midfacial plane in children with unrepaired cleft lip. The purpose of this second part is to develop computer-based methods to quantify symmetry and to determine the correlation of these measures to clinical expectations. PARTICIPANTS: A total of 35 infants with unrepaired unilateral cleft lip and 14 infant controls. INTERVENTIONS: Six computer-based methods of quantifying symmetry were developed and applied to the three-dimensional images of infants with unilateral cleft lip before and after cleft lip repair and to those of controls. MAIN OUTCOME MEASURE: Symmetry scores for cleft type, changes with surgery, and individual subjects ranked according to cleft severity were assessed. RESULTS: Significant differences in symmetry scores were found between cleft types and found before and after surgery. Symmetry scores for infants with unilateral cleft lip approached those of controls after surgery, and there was a strong correlation with ranked cleft severity. CONCLUSIONS: Our computer-based three-dimensional analysis of nasolabial symmetry correlated with clinical expectations. Automated processing made measurement convenient. Use of these measures may help to objectively measure cleft severity and treatment outcome.