Suprazygomatic Maxillary Nerve Block in Palatoplasty: A Retrospective Cohort Study of Perioperative Outcomes in Syndromic and Non-Syndromic Children.

OBJECTIVE: This study compares the impact of surgical site infiltration of local anesthesia alone to surgical site infiltration plus suprazygomatic maxillary nerve block (SMB) in non-syndromic and syndromic children undergoing primary palatoplasty. DESIGN: Retrospective cohort study of intra- and post-operative outcomes and opioid utilization in children undergoing palatoplasty by a single surgeon. SETTING: Urban, academic, tertiary care children's hospital. PATIENTS, PARTICIPANTS: Children 24 months or younger undergoing primary palatoplasty were included (n = 102). Exclusion criteria were concurrent painful procedures, history of neonatal abstinence syndrome, and nurse-controlled analgesia (n = 30). INTERVENTIONS: All patients received epinephrine-containing local anesthetic infiltrated at the surgical site. Fifty-seven also underwent placement of ultrasound-guided SMB. MAIN OUTCOME MEASURE(S): Intra-operative opioid requirement, duration of anesthesia, time to wake up, post-operative opioid requirement, hypoxemic episodes, need for respiratory support, FLACC scores, and length of stay. RESULTS: When controlling for syndromic status and cleft phenotype, SMB was associated with a 57% reduction in intraoperative opioid requirements (95% CI = 15-81%, p = 0.024) but also with a 29% (∼5-min) increase in wake-up time post-surgery (95% CI = 3-50%, p = 0.048). Postoperatively, SMB was linked to a 18% reduction in hospital stay length (95% CI = 2-31%, p = 0.027) and a 88% reduction in opioid requirements within 24 h after surgery (p = 0.006). Desaturations and new respiratory support requirements were unaffected by SMB. CONCLUSIONS: Compared to surgical site infiltration of local anesthetic alone, adding SMB reduces intra- and postoperative narcotic requirements and decreases length of stay. These benefits apply to both syndromic and non-syndromic children. SMB does not meaningfully affect respiratory outcomes.

Expanding the Spectrum of Perioral Myogenic Tumors in Pediatric Patients: An SRF::NCOA2 Fused Perivascular Tumor of the Philtrum.

BACKGROUND: Perivascular tumors, which include myopericytoma and myofibroma, are rare benign soft tissue neoplasms composed of perivascular smooth muscle cells. Most demonstrate characteristic morphology and are readily diagnosed. However, a recently identified hypercellular subset shows atypical histologic features and harbor unique SRF gene fusions. These cellular perivascular tumors can mimic other more common sarcomas with myogenic differentiation. METHODS: Clinical, radiological, morphological, immunohistochemical, and molecular findings were reviewed. RESULTS: A slow-growing, fluctuant mass was noted within the philtrum at 16 months. Ultrasonography revealed a well-circumscribed cystic hypoechoic lesion. A small (1.0 cm), tan, well-circumscribed soft-tissue mass was excised after continued growth. Histologically, the encapsulated tumor was hypercellular and composed of spindle cells with predominantly-storiform architecture, focal perivascular condensation, dilated branching thin-walled vessels, increased mitoses, and a smooth muscle immunophenotype. An SRF::NCOA2 fusion was identified. CONCLUSION: We report the first case of an SRF-rearranged cellular myopericytoma in the perioral region in a young child. This case expands the differential diagnosis of perioral soft tissue tumors with myogenic differentiation. We highlight key clinical, pathological, and molecular features. As we illustrate, these rare tumors pose a considerable diagnostic challenge, and risk misdiagnosis as sarcoma, most notably spindle cell rhabdomyosarcoma.

Brief report: Areolar melanosis in a girl with precocious puberty.

We report a case of melanosis of the areola in a 7-year-old girl with early thelarche. Areolar melanosis is a rare condition previously only described in women over 25 years of age, often in the setting of pregnancy. This case supports a theory that hyperpigmentation may be associated with increased sensitivity to hormonal stimulation in areas with greater populations of melanocytes.

Large Vertex Encephaloceles: Management and Outcomes.

BACKGROUND: Complex vertex and posterior encephaloceles containing brain tissue have uncertain prognosis and high operative risk. Patients may not be offered operative intervention depending on local and regional specialist expertise. The authors present their experience treating 5 such pediatric patients. METHODS: This is a retrospective review of the surgical assessment, planning, and technique of cranial repairs, as well as surgical outcomes and developmental follow-up regarding adaptive functioning for patients presenting for second opinion for encephalocele of the cranial vertex after having been deemed too high risk at another institution. RESULTS: Five consecutive patients presented between January 2014 and June 2016. One patient was not offered repair. Of 4 patients who underwent reconstruction, average age at time of repair was 2.7 months (range, 0.9-6.7). One presented with ruptured encephalocele, whereas the remaining 3 underwent drainage of the encephalocele (average volume of 1200 mL) at time of surgical resection. Operative time averaged 3.7 hours (range, 2.2-5.3). There were no deaths. One patient had a single seizure postoperatively. Two patients required placement of permanent shunt for hydrocephalus. Two patients completed developmental evaluations, both of whom exhibited delays in adaptive functioning relative to same-aged peers. CONCLUSIONS: Patients with large, complex encephalocele warrant evaluation by an experienced high-volume tertiary care pediatric craniofacial center. The decision to proceed with surgical management should include an interdisciplinary team of surgeons, anesthesiologists, neurologists, and social work. Further study of developmental outcomes in both operated and unoperated patients is necessary to better understand risks and benefits of reconstruction.

Dental Anomalies in Parry-Romberg Syndrome: A 40-Year Retrospective Review.

OBJECTIVE: This study investigates crown and root anomalies in patients with Parry-Romberg Syndrome. DESIGN: This is a retrospective review of patients with Parry-Romberg Syndrome who were evaluated at a tertiary care center from 1980-2020. SETTING: Patients seen in the dental unit from 1980-2020. PATIENTS, PARTICIPANTS: Seventeen patients with documented Parry-Romberg Syndrome were referred for dental evaluation. MAIN OUTCOME MEASURES: All dental anomalies were documented. Root anomalies were assessed using panoramic radiographs and cone beam CT (CBCT) scans to evaluate buccal-lingual, mesio-distal, and axial measurements of hypoplastic teeth, which were compared to those of contralateral teeth. RESULTS: Findings included agenesis (29%, n = 5), hypoplastic teeth (29%, n = 5), delayed canine eruption (24%, n = 4), and mulberry molars (12%, n = 2). Of the five patients with tooth hypoplasia, four had CBCT records and the fifth had panoramic radiographs available for assessment. Axial length was always shorter in hypoplastic teeth relative to contralateral teeth, with differences ranging from 1.2-9.2 mm. Differences in crown size of hypoplastic versus contralateral teeth were unpredictable but always present. CONCLUSIONS: Patients with Parry-Romberg Syndrome can have hypoplastic roots with atypical crown morphology. A patient's specific dental anomaly will influence planning and treatment.

Perioperative Pain Management After Primary Palate Repair: A 3-Surgeon Retrospective Study.

OBJECTIVE: Pain management strategies following palatoplasty vary substantially. Despite efforts to reduce narcotic utilization, specific analgesic regimens are typically guided by surgeon preference. Our aim was to define analgesic variables that affect postoperative narcotic use and time to resumption of oral intake. DESIGN: This is a retrospective review from 2015 to 2018. PATIENTS: Nonsyndromic patients undergoing primary palate repair. MAIN OUTCOMES MEASURES: Analgesic variables included: local anesthetic, pterygopalatine ganglion nerve block, palatal pack, and postoperative use of ketorolac, dexamethasone, and nursing-controlled analgesia (NCA) opioid dosing. Proxy measures for pain included time to resumption of oral intake and morphine equivalence (mg/kg/h) administered. RESULTS: Veau phenotypes for the 111 patients included were: I (28%), II (19%), III (33%), IV (16%), and submucous (4%). Age, weight, local anesthetic, and postoperative use of ketorolac, dexamethasone, and palatal pack had no effect on either proxy measure (P > .05). Postoperative narcotic usage was significantly lower in patients who had an intraoperative suprazygomatic peripheral nerve block and significantly higher when NCA was utilized (P < .05). Neither variable had a significant impact on time to resumption of oral intake (P > .05). CONCLUSION: Several perioperative analgesic strategies lead to comparable postoperative consumption of narcotic and time to resume oral intake. The authors advise careful consideration of NCA due to the potential for increased narcotic utilization that we found in our institution. Based on our promising findings, further studies are warranted to assess risks, benefits, and costs of performing peripheral nerve blocks at the time of palatoplasty.

Cleft Lip and Palate in Infants With Prenatal Opioid Exposure.

OBJECTIVE: This study examines phenotypic presentation and perioperative outcomes of cleft-related procedures for infants with cleft lip and/or palate (CL/P) and prenatal opioid exposure. DESIGN: This is a retrospective review of infants with prenatal opioid exposure treated for CL/P from 2008 to 2018. SETTING: Patients cared for at a tertiary center from 2008 to 2018. PATIENTS/PARTICIPANTS: Eighteen patients with documented prenatal opioid exposure and CL/P had primary repairs in our unit. MAIN OUTCOME MEASURE(S): The phenotypes of CL/P were characterized. Demographic data regarding additional exposures, as well as associated medical and social comorbidities were recorded. Outcome variables included operative delays, perioperative complications, and loss of follow-up. RESULTS: Isolated cleft palate (CP; 67%) was overrepresented among patients with prenatal opioid exposure and CL/P, as was Robin sequence (50% in isolated CP). Fifty-six percent had exposure to additional substances. A majority (67%) had other medical conditions or anomalies, and 17% had known genetic syndromes. Seventy-two percent were in state custody. Thirty-nine percent of exposed patients had delays in their planned operative dates due to medical and/or social factors. There were no postoperative readmissions following cleft procedures. Lack of follow-up was noted in 33% of patients. CONCLUSIONS: Infants with CL/P who have prenatal opioid exposure are likely to have additional medical conditions and complex social challenges.

Cleft Lip and Palate in Ectodermal Dysplasia.

OBJECTIVE: Ectodermal dysplasia (ED) comprises multiple syndromes that affect skin, hair, nails, and teeth, and sometimes are associated with orofacial clefting. The purpose of this study is to (1) identify the prevalence and characteristics of cleft lip and/or palate (CL/P) in patients with ED and (2) describe the management and outcomes. DESIGN: Retrospective review from 1990 to 2019. PATIENTS: All patients with ED treated at Boston Children's Hospital. MAIN OUTCOMES MEASURES: Prevalence of CL/P was calculated and clinical details recorded: phenotypic anomalies, cleft type, operative treatment, and results of repair. RESULTS: Of 170 patients with a purported diagnosis of ED, 24 (14%) had CL/P. Anatomic categories were bilateral CL/P (67%), unilateral CL/P (8%), and cleft palate only (25%). The most common ED syndrome (37%) was ectrodactyly, ectodermal dysplasia, and cleft lip/palate (EEC). Pathogenic variants in TP63 were the most frequent finding in the 11 patients who had genetic testing. Aberrations from a typical clinical course included failure of presurgical dentofacial orthopedics, dehiscence of nasolabial adhesion, and total palatal absence requiring free-flap construction. Two patients had prolonged postoperative admission for respiratory infection. High fistula (8%) and velopharyngeal insufficiency (33%) rates reflected the predominance of bilateral complete forms. CONCLUSIONS: As in other types of syndromic CL/P, cleft phenotypic expression in ED is more severe than the general cleft population. Further studies are needed to correlate genotype and phenotype for the distinct syndromes included in the ED spectrum.

Does the Nostril Shape Change After Le Fort I Advancement in Patients With Unilateral Complete Cleft Lip?

PURPOSE: Patients with unilateral cleft lip and palate (UCLP) may require Le Fort I advancement to correct maxillary hypoplasia after reaching skeletal maturity. The underlying cleft anatomy, previous operations, and scarring can affect nostril changes after maxillary advancement. The purpose of the present study was to determine whether Le Fort I advancement affects the nostril configuration (ie, width, axis, shape) in patients with UCLP. The specific aims were to (1) compare cleft and noncleft nostrils in patients with UCLP after maxillary advancement and (2) compare the changes in nostril configuration in patients with UCLP with those in noncleft controls after Le Fort advancement. PATIENTS AND METHODS: A retrospective case-control study of nonsyndromic, skeletally mature patients with UCLP and a case-matched control group without UCLP who had undergone single-piece Le Fort I advancement with alar cinch suture from 2010 to 2014. Patients were included if they had undergone pre- and postoperative 3-dimensional photogrammetry without intervening nasal revision. Three-dimensional anthropometry was used to evaluate changes in nostril axis and width, soft triangle angle, columellar show, and nasal width after orthognathic correction. RESULTS: The present study included 19 patients with UCLP (11 males; mean age, 18.0 years) and 19 noncleft controls (11 males; mean age, 18.7 years; P = .276). The mean sagittal advancement in the patients with UCLP and noncleft controls was 7.5 mm and 6.3 mm, respectively (P = .143). On average, the nostrils widened, the soft triangles flattened, and the columellar show increased. No significant difference was found in the changes to the nostril configuration between the cleft and noncleft sides in the patients with UCLP. No significant differences were found in the nostril changes between patients with UCLP and noncleft controls. CONCLUSIONS: Baseline nostril asymmetry is not altered by Le Fort osteotomy in patients with UCLP because both nostrils respond similarly to the deforming forces of maxillary advancement. Similarly, no differences were found in the nostril changes between the cleft and noncleft controls. These findings can aid proper surgical planning for cleft nasal revisions.

Development of the Submental Nasal Appearance Scale for the Assessment of Repaired Unilateral Complete Cleft Lip: A Pilot Study.

OBJECTIVE: To develop the "Submental Nasal Appearance Scale" (SNAS), which is an easy-to-use objectified tool to represent a cleft surgeon's standard for assessment of the nasal appearance from the submental perspective. DESIGN: Eighty-five photographs of patients with unilateral complete cleft lip and palate were selected and cropped, displaying the submental view. Sixty-one photographs were used to develop 5 sets of reference photographs. Three cleft surgeons graded 24 photographs with these sets and subjectively graded the overall nasal appearance as well. Internal agreement for both methods was calculated, as well as correlation between them. The SNAS was created, by only using the combination of sets that showed the highest reliability and correlation. SETTING: Boston Children's Hospital, Boston, Massachusetts. PATIENTS: Six- to 9-year-old patients with unilateral complete cleft lip and palate. RESULTS: The intrarater and interrater reliability was 0.84 and 0.79, respectively, for the SNAS and 0.76 and 0.62, respectively, for the overall appearance assessment. The correlation was 0.74 between the methods. CONCLUSIONS: The SNAS is a reliable tool that reflects a cleft surgeon's standard and could be used independently or in combination with existing rating scales using the frontal and/or lateral view, for assessment after cleft lip repair.

Long-term Outcomes After Flap Reconstruction in Pediatric Pressure Ulcers.

BACKGROUND: Pressure ulcers refractory to nonoperative management may undergo flap reconstruction. This study aims to evaluate the long-term outcomes and recurrence rates of flap reconstruction for pediatric pressure ulcers. METHODS: We reviewed the records of patients who underwent flap reconstruction for pressure ulcer(s) from 1995 to 2013. RESULTS: Twenty-four patients with 30 pressure ulcers, requiring 52 flaps were included. Ulcers were stages III and IV and mostly involved either the ischia (15/30) or sacrum (8/30). Flaps were followed for a median of 4.9 years. Twenty-three patients were wheelchair dependent, and 20 had sensory impairment at their ulcer site(s). Ten patients had a history of noncompliance with preoperative management, 8 of whom experienced ulcer recurrence. Twenty-one ulcers had underlying osteomyelitis, associated with increased admissions (P = 0.019) and cumulative length of stay (P = 0.031). Overall, there was a 42% recurrence rate in ulceration after flap reconstruction. Recurrence was associated with a preoperative history of noncompliance with nonoperative therapy (P = 0.030), but not with flap type or location, age, sex, body mass index, osteomyelitis, or urinary/fecal incontinence (P > 0.05, all). CONCLUSIONS: Flap reconstruction can be beneficial in the management of pediatric pressure ulcers. Although high rates of long-term success with this intervention have been reported in children, we found rates of ulcer recurrence similar to that seen in adults. Poor compliance with nonoperative care and failure to modify the biopsychosocial perpetuators of pressure ulcers will likely eventuate in postoperative recurrence. Despite the many comorbidities observed in our patient sample, compliance was the best indicator of long-term skin integrity and flap success.

Double Supercharged Jejunal Interposition for Late Salvage of Long-gap Esophageal Atresia.

BACKGROUND: A variety of surgical techniques exist to manage long-gap esophageal atresia (LGEA), including gastric pull-up (GPU), colonic interposition (CI), jejunal interposition (JI), and distraction lengthening. Salvage reconstruction for late failure of any conduit type is a complex surgical problem fraught with technical difficulty and significant risk. Jejunal interposition can be used as a salvage procedure in the management of LGEA. However, the opposing requirements of conduit length and adequate perfusion make the procedure technically challenging. Chronic comorbidities and abdominal and thoracic adhesions may further complicate these cases. METHODS: We report a technique for the management of 3 late treatment failures of LGEA using pedicled JI in conjunction with 2 additional arterial and venous anastomoses, or double supercharging. For 2 patients who presented with failed CI, pedicled JI was performed and supercharged to internal mammary vessels as well as vasculature preserved from the prior colonic flap mesentery. The third patient presented with failed GPU and underwent pedicled JI that was supercharged caudally to the gastroepiploic vessels and cranially to the left common carotid artery. RESULTS: No flaps were lost in any patients. Median operation time was 16.5 hours. Patients were monitored postoperatively in the intensive care unit for a median of 23 days, extubated after 14 days, and discharged at 41 days. Postoperatively, all patients tolerated an oral diet by discharge and continue to enjoy oral intake of all food consistencies without dysphagia or aspiration. Follow-up time spanned 2 to 4 years (average, 3.3 years). One patient required dilatations and temporary stent for stricture, and another required removal of prominent sternal wires; otherwise, no additional procedures were performed. CONCLUSIONS: Although technically difficult, double supercharged JI should be considered as a salvage operation to restore esophageal continuity after CI or GPU failure for LGEA, when there are otherwise limited reconstructive options.

Cleft Lip and Palate in CHARGE Syndrome: Phenotypic Features That Influence Management.

OBJECTIVE: Infants with syndromic cleft lip and/or cleft palate (CL/P) often require more complex care than their nonsyndromic counterparts. Our purpose was to (1) determine the prevalence of CL/P in patients with CHARGE syndrome and (2) highlight factors that affect management in this subset of children. DESIGN: This is a retrospective review from 1998 to 2016. PATIENTS: Patients with CHARGE syndrome were diagnosed clinically and genetically. MAIN OUTCOMES MEASURES: Prevalence of CL/P was determined and clinical details tabulated: phenotypic anomalies, cleft types, operative treatment, and results of repair. RESULTS: CHARGE syndrome was confirmed in 44 patients: 11 (25%) had cleft lip and palate and 1 had cleft palate only. Surgical treatment followed our usual protocols. Two patients with cardiac anomalies had prolonged recovery following surgical correction, necessitating palatal closure prior to nasolabial repair. One of these patients was too old for dentofacial orthopedics and underwent combined premaxillary setback and palatoplasty, prior to labial closure. Velopharyngeal insufficiency was frequent (n = 3/7). All patients had feeding difficulty and required a gastrostomy tube. All patients had neurosensory hearing loss; anomalies of the semicircular canals were frequent (n = 3/4). External auricular anomalies, colobomas, and cardiovascular anomalies were also common (n = 8/11). Other associated anomalies were choanal atresia (n = 4/11) and tracheoesophageal fistula (n = 2/11). CONCLUSIONS: CHARGE syndrome is an under-recognized genetic cause of cleft lip and palate. Hearing loss and speech and feeding difficulties often occur in these infants. Diagnosis can be delayed if the child presents with covert phenotypic features, such as chorioretinal colobomas, semicircular canal hypoplasia, and unilateral choanal atresia.

Understanding Hematoma Risk: Study of Patient and Perioperative Factors in a Large Cohort of Young Women Undergoing Reduction Mammaplasty.

BACKGROUND: Despite high satisfaction rates, reduction mammaplasty can have complications such as hematoma. Factors such as age, tobacco use, and comorbidities are known contributors, whereas the influence of race, BMI, certain medications, and blood pressure (BP) remain contentious. This study investigates hematoma risk factors in young women undergoing reduction mammaplasty. STUDY DESIGN: A retrospective review was conducted including all female patients who underwent bilateral reduction mammaplasty at a single institution between 2012 and 2022. Data on demographics, BMI, medical comorbidities, surgical techniques, medications, and perioperative BP were collected. Differences between patients who developed a hematoma and those who did not were assessed using chi-square, Fisher's exact, and t -tests. The relationship between perioperative BP and hematoma formation was assessed using logistic regression. RESULTS: Of 1,754 consecutive patients, 3% developed postoperative hematoma of any kind, with 1.8% returning to the operating room. Age (odds ratio [OR] 1.14, p = 0.01) and ketorolac use (OR 3.93, p = 0.01) were associated with hematoma development. Controlling for baseline BP, each 10 mmHg incremental increase in peak intraoperative BP (systolic BP [SBP]: OR 1.24, p = 0.03; mean arterial pressure: OR 1.24, p = 0.01) and postoperative BP (SBP: OR 1.41, p = 0.01; mean arterial pressure: OR 1.49, p = 0.01) escalated the odds of hematoma. Postoperative SBP variability also incrementally increased hematoma odds (OR 1.48, p < 0.01). Other factors, including race and surgical technique, were not significantly influential. CONCLUSIONS: Age, ketorolac use, and intra- and postoperative BP peaks and variability are risk factors for hematoma in reduction mammaplasty. This emphasizes the importance of perioperative BP management and optimizing pain management protocols.

Is a Picture Worth a Thousand Words? A Scoping Review of the Impact of Visual Aids on Patients Undergoing Surgery.

OBJECTIVE: While graphics are commonly used by clinicians to communicate information to patients, the impact of using visual media on surgical patients is not understood. This review seeks to understand the current landscape of research analyzing impact of using visual aids to communicate with patients undergoing surgery, as well as gaps in the present literature. DESIGN: A comprehensive literature search was performed across 4 databases. Search terms included: visual aids, diagrams, graphics, surgery, patient education, informed consent, and decision making. Inclusion criteria were (i) full-text, peer-reviewed articles in English; (ii) evaluation of a nonelectronic visual aid(s); and (iii) surgical patient population. RESULTS: There were 1402 articles identified; 21 met study criteria. Fifteen were randomized control trials and 6 were prospective cohort studies. Visual media assessed comprised of diagrams as informed consent adjuncts (n = 6), graphics for shared decision-making conversations (n = 3), other preoperative educational graphics (n = 8), and postoperative educational materials (n = 4). There was statistically significant improvement in patient comprehension, with an increase in objective knowledge recall (7.8%-29.6%) using illustrated educational materials (n = 10 of 15). Other studies noted increased satisfaction (n = 4 of 6), improvement in shared decision-making (n = 2 of 4), and reduction in patient anxiety (n = 3 of 6). For behavioral outcomes, visual aids improved postoperative medication compliance (n = 2) and lowered postoperative analgesia requirements (n = 2). CONCLUSIONS: The use of visual aids to enhance the surgical patient experience is promising in improving knowledge retention, satisfaction, and reducing anxiety. Future studies ought to consider visual aid format, and readability, as well as patient language, race, and healthcare literacy.

Art in Surgery: A Review of Art-based Medical Humanities Curricula in Surgical Residency.

OBJECTIVE: Numerous programs integrate arts and humanities methods to advance medical education competencies. Despite the highly visual and technical nature of the field of surgery, the current state of art utilization in surgical training is unclear. The purpose of this review is to gain a comprehensive understanding of how art has been utilized in surgical training, to investigate the purpose of such interventions, and to assess how art interventions may benefit surgeons. DESIGN: A systematic literature review using PRISMA methodology was conducted to identify articles published prior to February 2022 that investigated or described using art in surgical resident training. Qualitative themes were developed upon full review of the literature and categorized based on fundamental aspects of surgical education. The data was summarized by a narrative approach. RESULTS: Six hundred seventy-four unique articles were initially identified, thirteen of which met inclusion criteria. Twelve studies employed drawing or sculpture in surgical residency training; one discussed art observation to foster mindfulness, teambuilding, and empathy. Eight articles utilized art as an evaluation tool, 2 for didactic and archival purposes, one employed exercises in art analysis to improve empathy and physician wellbeing, and 2 described courses in which art making was treated as a foundational skill. No articles discussed use of art for honing diagnostic skills, observation, or patient communication - competencies that have been addressed in other fields. CONCLUSIONS: This review highlights the small number of examples in the medical literature about visual arts in surgical training. The existing art-based surgical humanities studies identify opportunities for curricular innovation within surgical training.

Prevalence of visual art education in medical school curricula: a national survey of US medical schools.

Background: The Association of American Medical Colleges (AAMC) has recommended integrating medically-relevant arts and humanities curricula into medical student education in order promote physician skills development. An analysis of the state of existing visual arts-based medical school pedagogies was conducted to inform future implementation strategies.Methodology: An electronic survey was distributed to representatives of US medical schools to describe the prevalence and characteristics of visual arts-based medical school curricula. Official courses, informal events, cross-registration opportunities, and established art museum partnerships were assessed.Results: Survey response rates were 65% for US allopathic medical schools and 56% for osteopathic medical schools. A majority (79%) of responding institutions incorporate or support medical student art experiences in some format. Thirty-one percent (n = 36) of schools offer stand-alone humanities courses using visual arts. These were primarily allopathic programs (n = 35; 37% of allopathic programs) and only one responding osteopathic program (n = 1; 5% of osteopathic programs). Schools without dedicated courses are less likely to report other curricular and extracurricular visual arts engagement. Most visual art medical courses are offered at medical schools located in the Northeastern United States.Conclusions: Many but not all medical schools are incorporating the visual arts into their medical education curriculum. Opportunities to promote increased uptake, more effective implementation, and collaboration strategies for the AAMC recommendations are proposed.

Comparative Study of Pharyngeal Flap Outcomes between Children With 22q11.2 Deletion Syndrome and Non-Syndromic Cleft Lip and Palate.

BACKGROUND: Management of velopharyngeal insufficiency (VPI) in 22q11.2 deletion syndrome (22q) is challenging. This study compares pharyngeal flap outcomes in children with 22q to those with non-syndromic cleft lip and palate (CLP) to assess risk of poor speech outcomes and negative sequelae. METHODS: Children with 22q or CLP treated with pharyngeal flap through a multidisciplinary VPI clinic between 2009 and 2020 were retrospectively reviewed. Pre- and postoperative speech assessments, perioperative characteristics, and complications were identified. RESULTS: 36 children with ​22q and 40 with CLP were included. Age at surgery (p=0.121), pre-operative velopharyngeal competence score (VPC) (p=0.702), and pre-operative resonance (p=0.999) were similar between groups. Pharyngeal flaps were wider (p=0.038*) and length of stay longer in the 22q group (p=0.031*). On short term follow 4 months after surgery, similar speech outcomes were seen between groups. At long term follow up >12 months after surgery, 86.7% 22q v. 100% CLP (p=0.122) had improvement in velopharyngeal function, however fewer children with 22q (60.0%) achieved a completely "competent" VPC score compared to those with CLP (92.6%) (p=0.016*). Nasal regurgitation improved for both groups, with a greater improvement in those with 22q (p=0.026*). Revision rate (p=0.609) and new onset OSA (0.999) were similar between groups. CONCLUSION: Children with 22q have improved speech after pharyngeal flap, but may be less likely to reach normal velopharyngeal function over the long term than those with CLP; however, negative sequelae do not differ. Improvement in nasal regurgitation is a uniquely positive outcome in this population.

Use of 3-Dimensional Stereophotogrammetry to Detect Disease Progression in Craniofacial Morphea.

Importance: Objectively determining disease progression in craniofacial morphea (CM) is challenging, as clinical findings of disease activity are often lacking. Objective: To evaluate the utility of 3-dimensional (3D) stereophotogrammetry in detecting disease progression in CM over time. Design, Setting, and Participants: This prospective cohort study included 27 pediatric and adult patients with CM from 2 hospitals in Boston (Boston Children's Hospital and Brigham & Women's Hospital) consecutively enrolled from April 1, 2019, to March 1, 2023. Review of 3D stereophotogrammetry images and data analysis occurred from March 1 to April 1, 2023. Main Outcomes and Measures: Clinical and 3D stereophotogrammetry assessments were performed at 2- to 12-month intervals, depending on the clinical context. The 3D stereophotogrammetry images were then qualitatively rated as demonstrating no progression or definitive progression by an expert (board-certified plastic craniofacial surgeon) and nonexpert (board-certified dermatologist) in 3D stereophotogrammetry. In addition, κ coefficients were calculated for interrater reliability. Results: Of 27 patients with CM (19 female; median age, 14 [range, 5-40] years) and 3D stereophotogrammetry images obtained from a minimum of 2 time points (median, 4 [range, 2-10] images) spaced a median of 3 (range, 2-12) months apart, 10 experienced progression of their disease based on clinical assessments performed during the study period. In all cases in which clinical progression was favored, blinded qualitative assessment of 3D stereophotogrammetry images also favored progression with substantial interrater reliability (κ = 0.80 [95% CI, 0.61-0.99]). Furthermore, review of 3D stereophotogrammetry detected occult progression of asymmetry not noted on clinical examination in 3 additional patients. Conclusions and Relevance: In this prospective cohort study, blinded assessment of sequential 3D stereophotogrammetry images in patients with CM not only corroborated clinical assessment of disease progression but also detected occult progression of facial asymmetry not appreciable on clinical examination alone. Therefore, 3D stereophotogrammetry may serve as a useful adjunct to clinical examination of patients with CM over time. Future investigations are warranted to validate 3D stereophotogrammetry as an outcome measure in CM.

Barriers to care in juvenile localized and systemic scleroderma: an exploratory survey study of caregivers' perspectives.

BACKGROUND: Juvenile localized scleroderma (LS) and systemic sclerosis (SSc) are rare pediatric conditions often associated with severe morbidities. Delays in diagnosis are common, increasing the risk for permanent damage and worse outcomes. This study explored caregiver perspectives on barriers they encountered while navigating diagnosis and care for their child's scleroderma. METHODS: In this cross-sectional study, caregivers of juvenile LS or SSc patients were recruited from a virtual family scleroderma educational conference and a juvenile scleroderma online interest group. The survey queried respondents about their child's condition and factors affecting diagnosis and treatment. RESULTS: The response rate was 61% (73/120), with 38 parents of LS patients and 31 parents of SSc patients. Most patients were female (80%) and over half were non-Hispanic white (55%). Most families had at least one person with a college education or higher (87%), traveled ≤ 2 h to see their rheumatologist (83%), and had private insurance (75%). Almost half had an annual household income ≥ $100,000 (46%). Families identified the following factors as barriers to care: lack of knowledge about scleroderma in the medical community, finding reliable information about pediatric scleroderma, long wait times/distances for a rheumatology/specialist appointment, balance of school/work and child's healthcare needs, medication side effects, and identifying effective medications. The barrier most identified as a major problem was the lack of knowledge about juvenile scleroderma in the medical community. Public insurance, household income less than $100,000, and Hispanic ethnicity were associated with specific barriers to care. Lower socioeconomic status was associated with longer travel times to see the rheumatologist/specialist. Diagnosis and systemic treatment initiation occurred at greater than one year from initial presentation for approximately 28% and 36% of patients, respectively. Families of LS patients were commonly given erroneous information about the disease, including on the need and importance of treating active disease with systemic immunosuppressants in patients with deep tissue or rapidly progressive disease. CONCLUSION: Caregivers of children with LS or SSc reported numerous common barriers to the diagnosis, treatment, and ongoing care of juvenile scleroderma. The major problem highlighted was the lack of knowledge of scleroderma within the general medical community. Given that most of the caregiver respondents to the survey had relatively high socioeconomic status, additional studies are needed to reach a broader audience, including caregivers with limited English proficiency, geographical limitations, and financial constraints, to determine if the identified problems are generalizable. Identifying key care barriers will help direct efforts to address needs, reduce disparities in care, and improve patient outcomes.