Predicting Failure of Conservative Airway Management in Infants with Robin Sequence: The EARN Factors.

OBJECTIVE: Evaluate infants with Robin Sequence (RS) who were successfully treated with conservative airway measures alone vs. those who failed and eventually underwent surgical airway intervention after a protracted course of conservative management. DESIGN: Retrospective review of prospectively gathered database. SETTING: Large tertiary care institution. PATIENTS: Infants diagnosed with RS (n = 122) who underwent primary airway management at a single institution from 1994-2020. MAIN OUTCOME MEASURE: Patient demographics, nutritional and respiratory status, laboratory values, and polysomnographic results were compared between patients who were discharged after successful conservative airway management (Group 1, n = 61) and patients that underwent surgical airway intervention after failing a prolonged course of conservative management (Group 2, n = 61). Receiver operating characteristic (ROC) curve analysis was done to assess continuous variables that may predict failure of conservative airway management. RESULTS: 122 infants with RS were investigated. While several variables were significantly different between groups, the following polysomnographic EARN factors, with cut points, were identified as most predictive of failed conservative airway management: ETCO2 (max) > 49 mmHg, AHI > 16.9 events/hour, OAHI REM >25.9 events/hour, OAHI Non-REM > 23.6 events/hour. CONCLUSIONS: We identified factors in infants with RS that were associated with severe UAO that failed to improve despite weeks of conservative airway management. Our results may expedite earlier definitive treatment of these critical patients and reduce risks for known complications of prolonged UAO.

Defining Age-related OSA Features in Robin Sequence Using Polysomnographic-based Analyses of Respiratory Arousal Responses and Gas-exchange Parameters.

INTRODUCTION: Robin sequence (RS) is a leading cause of obstructive sleep apnea (OSA) in newborns. Most studies have focused on understanding anatomic factors leading to OSA and changes in apnea-hypopnea index (AHI) on polysomnography (PSG) beyond the neonatal period. This study aims to define age-related OSA features between patients with RS, without RS and healthy controls using PSG-based analyses of respiratory arousal responses and gas-exchange parameters. DESIGN: Retrospective comparison of PSG features in a total of 48 children encompassing three groups: (a) infants with RS (n = 24, <1-year old), (b) non-RS older children (1-2 years old) with severe OSA (obstructive AHI (OAHI) of ≥10 events; n = 12), and (c) control infants and children (0-2 years old) without sleep apnea (OAHI ≤1.5/h, n = 12). We examined OSA sleep-stage specific and position-specific indexes, and the relationship between OSA severity and respiratory arousal indexes (OAHI/respiratory arousal indexes). RESULTS: OSA sleep-stage specific indexes (rapid eye movement [REM] vs non-REM[NREM]) as well as position-specific indexes (supine vs nonsupine) were similar in individuals with and without RS. Relative to the non-RS groups, infants with RS have more sustained hypoxemia (time with SpO2 < 90%) and reduced arousal responses to OSA demonstrated by higher OAHI/respiratory arousal indexes. OAHI/respiratory arousal indexes significantly correlated with the severity of hypoxemia in infants with RS. CONCLUSION: Infants with RS and OSA show reduced arousal responses to apneic events, which correlates with higher hypoxemia severity. OAHI/respiratory arousal indexes in RS may identify high-risk individuals with upper airway obstruction and reduced arousal protective responses.

Langerhans cells and SFRP2/Wnt/beta-catenin signalling control adaptation of skin epidermis to mechanical stretching.

Skin can be mechanically stimulated to grow through a clinical procedure called tissue expansion (TE). Using a porcine TE model, we determined that expansion promptly activates transcription of SFRP2 in skin and we revealed that in the epidermis, this protein is secreted by Langerhans cells (LCs). Similar to well-known mechanosensitive genes, the increase in SFRP2 expression was proportional to the magnitude of tension, showing a spike at the apex of the expanded skin. This implies that SFRP2 might be a newly discovered effector of mechanotransduction pathways. In addition, we found that acute stretching induces accumulation of b-catenin in the nuclei of basal keratinocytes (KCs) and LCs, indicating Wnt signalling activation, followed by cell proliferation. Moreover, TE-activated LCs proliferate and migrate into the suprabasal layer of skin, suggesting that LCs rebuild their steady network within the growing epidermis. We demonstrated that in vitro hrSFRP2 treatment on KCs inhibits Wnt/b-catenin signalling and stimulates KC differentiation. In parallel, we observed an accumulation of KRT10 in vivo in the expanded skin, pointing to TE-induced, SFRP2-augmented KC maturation. Overall, our results reveal that a network of LCs delivers SFRP2 across the epidermis to fine-tune Wnt/b-catenin signalling to restore epidermal homeostasis disrupted by TE.

Establishment of Objective Clinical Parameters for Assessment of Trigonocephaly: Are Caliper-Derived Clinical Measures Adequate?

OBJECTIVE: Objective clinical parameters characterizing the severity of trigonocephaly are essential given the concern for computerized tomography (CT) scans and radiation in infants. The present study seeks to develop a clinical tool by which to characterize trigonocephaly. DESIGN: Retrospective cohort study. SETTING: Tertiary academically affiliated children's medical center. PARTICIPANTS: A retrospective review identified patients with trigonocephaly for whom surgery was recommended (group 1) and those with metopic ridging without significant trigonocephaly (group 2). Normal age-matched controls were also evaluated (group 3). INTERVENTIONS: Cranial vault caliper measurements were compared across groups. Two ratios measuring anterior vault constriction were developed: (1) bitemporal width at the mid-forehead to the biparietal width, and (2) bitemporal width at the lateral brow to the biparietal width. MAIN OUTCOME MEASURES: Bitemporal width to biparietal width (ratio). RESULTS: Caliper measures were obtained from 19 patients in group 1, 8 patients in group 2, and 19 patients in group 3 (controls). Cranial indices were not significantly different across groups. The bitemporal width at the mid-forehead to the biparietal width ratio was significantly lower in group 1, with no difference between groups 2 and 3. The bitemporal width at the lateral brow to the biparietal width ratio was significantly different between all 3 groups, with group 1 < group 2 < group 3, respectively. CONCLUSIONS: Bitemporal to biparietal ratios are a quantitative, objective clinical measure that can be used to differentiate patients with significant trigonocephaly from those with metopic ridging but no significant cranial deformity. These findings suggest that caliper-derived indices can assist in characterizing surgically relevant cranial vault deformities secondary to metopic synostosis and may circumvent CT-based analysis.

The Role of Smile Train and the Partner Hospital Model in Surgical Safety, Collaboration, and Quality in the Developing World.

BACKGROUND: The partner hospital model identifies hospitals in the developing world to educate and enable local surgeons to deliver effective cleft care. This study aimed to determine the outcomes of this model on safety, education, and quality of surgical care. METHODS: Twelve partner hospitals, sponsored by Smile Train for 5 or more years and distributed over 4 continents, were selected. Activities at each institution were evaluated using cleft surgical data, and surveys were completed by hospital leadership. RESULTS: A mean of 82% of cleft patients at partner hospitals underwent sponsored surgeries. After partnership, all 12 hospitals implemented preoperative checklists for cleft surgery, and 5 implemented checklists for other surgeries. All hospitals had personnel who received safety training as a result of partnership. There was no change in 30-day reoperations or readmissions. Follow-up rate increased by 18% (P = 0.03). Facilities recruited 1.8 additional cleft surgeons (P < 0.01) and increased the number of cleft surgical trainees by a mean of 13.3 annually (P = 0.012); 2.5 ± 1.7 additional ancillary services were added, resulting in 75% of partner hospitals having a basic multidisciplinary cleft team (surgery, speech, and dental) compared with 25% prior to partnership (P < 0.01). Total cleft surgeries, alveolar bone grafts, and percentage of secondary surgeries increased significantly as length of partnership progressed (P < 0.01). CONCLUSIONS: Smile Train's partner hospital model increases both the volume and quality of cleft care delivered at these institutions. Safety initiatives for cleft care demonstrate effects extending to global surgical care delivered at partner hospitals.

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case.

BACKGROUND: Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors. OBSERVATIONS: The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing. LESSONS: Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

Prevalence of Prohibited Questions during Plastic Surgery Residency Interviews.

Despite rules set forth by the National Resident Matching Program and American Association of Medical Colleges (AAMC), prohibited questions during the residency interview process are well documented. This study describes the prevalence of these encounters by surveying residency applicants to integrated plastic and reconstructive surgery (PRS) programs for the 2022 match cycle. Methods: An anonymous 16-question REDCap survey was distributed to 2022 cycle applicants of a single PRS program. The applicants were queried about demographic information, interview experience, and questions deemed illegal by the AAMC/NRMP guidelines. Results: One hundred survey responses were attained for a 33.1% response rate. The majority of respondents were aged 26-30 (76%), women (53%), and white (53%); 33% received 15+ interviews for the application cycle. Seventy-eight percent of respondents reported being asked a prohibited question during at least one interview, with the most common "illegal" question categories being number/ranking of interviews (42%), marital status (33%), career balance (25%), and race/ethnicity (22%). Only 25.6% of applicants considered the subject matter inappropriate, whereas 42.3% were unsure. Although no applicant took action to report the potentially illegal scenarios, 30% said that their experiences influenced their rank list. Conclusions: Our survey study revealed that prohibited interview questions in PRS residency interviews are common. Permissible lines of questioning and discussion between programs and applicants during residency interviews have been defined by AAMC. Institutions should provide guidance and training to all participants. Applicants should be made aware of and empowered to utilize available anonymous reporting tools.

Epithelioid Sarcoma in a Young Child: A Case Report and Literature Review.

Epithelioid sarcoma is a rare, high-grade malignant soft tissue tumor that is often misdiagnosed. Classified as a mesenchymal malignancy, it exhibits both mesenchymal and epithelial markers. Occurrence in children under age 10 is extremely rare. This report describes the clinical course and management of a 5-year-old girl who presented with epithelioid sarcoma in the distal extremity. The lesion was initially misdiagnosed and treated for over a year as a common wart.

Secondary Suture Fusion after Primary Correction of Nonsyndromic Craniosynostosis: Recognition of the Problem and Identification of Risk Factors.

BACKGROUND: Secondary fusion of initially patent cranial sutures after primary correction of nonsyndromic craniosynostosis is rarely reported. This study's aim is to report the incidence and analyze whether there are variables that may predispose to such fusion. METHODS: A single-institution, retrospective, case-control study was conducted of all nonsyndromic patients who underwent operative treatment for craniosynostosis from April of 2008 to May of 2017. Patients with less than 1 year of follow-up and/or without a 1-year postoperative computed tomographic scan were excluded. Preoperative, intraoperative, and postoperative variables were analyzed using univariate and multivariate analyses. RESULTS: Sixty-six patients were included in the study, with a mean 2.57-year postoperative follow-up. Six patients (8.8 percent) were found to have secondary craniosynostosis, all of whom had fusion of sutures that were initially patent and refusion of the primary pathologic suture(s). Fifty percent of secondary fusions presented as pansynostosis. On univariate analysis, suturectomy with barrel staving (p < 0.01) was significantly associated with secondary suture fusion. On multivariate analysis, bilambdoid suture involvement (p = 0.03) and suturectomy with barrel staving (p = 0.01) were significantly associated with secondary suture fusion. CONCLUSIONS: Secondary cranial suture fusion may be a relatively common complication after primary craniosynostosis correction. Suturectomy with barrel staving was independently associated with secondary craniosynostosis. Wide surgical separation of the dura from the cranium and osteotomies across patent sutures may predispose to secondary craniosynostosis. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Abnormal Cranial Shape Preceding Radiographic Evidence of Craniosynostosis.

Premature fusion of a cranial suture is known to result in characteristic changes in the head shape, even when the synostosis involves only part of the suture. We report an unusual case of a patient seen at the age of 2 months for an abnormal head shape that was present at birth. The phenotype was consistent with an isolated fusion of the frontosphenoidal suture, but the suture was open on a high-resolution computed tomography scan finding. There was no improvement in cranial form after 6 months of helmet therapy, and a follow-up computed tomography scan image taken at age 10 months showed the development of bilateral isolated frontosphenoidal synostosis. This case highlights that an abnormal head shape may, in some patients, predate radiographic evidence of craniosynostosis.

Volumetric changes in cranial vault expansion: comparison of fronto-orbital advancement and posterior cranial vault distraction osteogenesis.

BACKGROUND: Posterior cranial vault distraction osteogenesis has recently been introduced to treat patients with multisuture syndromic craniosynostosis and is believed to provide greater gains in intracranial volume. This study provides volumetric analysis to determine the gains in intracranial volume produced by this modality. METHODS: This was a two-center retrospective study of preprocedure and postprocedure computed tomography scans of two groups of 15 patients each with syndromic multisuture craniosynostosis treated with either fronto-orbital advancement or posterior cranial vault distraction osteogenesis. Scan data were analyzed volumetrically with Mimics software. Volumetric gains attributable to growth between scans were controlled for. RESULTS: The mean advancements were 12.5 mm for fronto-orbital advancement and 24.8 mm for distraction osteogenesis. The mean difference in volume between the preoperative and postoperative scans was 144 cm(3) for fronto-orbital advancement and 274 cm(3) for (p = 0.009). After controlling for growth, the corrected mean volume difference was 66 cm(3) for fronto-orbital advancement and 142 cm(3) for distraction osteogenesis (p = 0.0017). The corrected mean volume difference per millimeter of advancement was 4.6 cm(3) for fronto-orbital advancement and 5.8 cm(3) for distraction (p = 0.357). CONCLUSIONS: In this retrospective study, posterior cranial vault distraction osteogenesis provided statistically greater intracranial volume expansion than fronto-orbital advancement. The volume gains per millimeter advancement were similar between groups, with a trend toward greater gains per millimeter with distraction osteogenesis. Gradual expansion of the overlying soft tissues with posterior cranial vault distraction osteogenesis appears to be the primary mechanism for greater volume gains with this technique. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Is nasal mucoperiosteal closure necessary in cleft palate repair?

BACKGROUND: The goals of successful palate repair include optimizing speech and feeding, mitigating adverse maxillary growth effect, and avoiding fistulae. The necessity of vomerine and/or nasal-side mucosa repair has not been tested. The purpose of this study was to compare the outcome of palate repairs with and without nasal mucoperiosteal closure. The authors used the null hypothesis. METHODS: This was a retrospective analysis of consecutive cleft palate repairs performed between 2001 and 2004. Group 1 underwent two-layer repair (oral and nasal/vomerine mucoperiosteal flaps), and group 2 underwent one-layer closure (oral mucoperiosteal flaps) only. Both groups underwent double-opposing Z-plasty posteriorly. Demographic and perioperative outcome variables were recorded and compared statistically. RESULTS: Group 1 consisted of 51 children (23 boys and 28 girls), and 80 percent were nonsyndromic. Group 2 included 29 patients (15 boys and 14 girls), and 72 percent were without an associated diagnosis. Age at repair was similar (20.80 and 15.17 months, respectively). Operative time was less for one-layer repair (84 versus 135 minutes) (p = 0.0001). Complications, length of stay, and follow-up length were similar between the two cohorts. Velopharyngeal dysfunction was rare in both groups. A single fistula occurred in each group. Anthropometric data revealed larger maxillary arc and tragus-subnasale lengths in group 2. Growth velocities were similar in both groups. CONCLUSIONS: The goals of cleft palate repair can be efficiently achieved using a one-sided oral mucoperiosteal repair only. Omitting the nasal-side and vomer repair does not increase fistula formation or prove detrimental to velopharyngeal function, and may facilitate maxillary growth.