Velopharyngeal Insufficiency Following Furlow Versus Straight Line Repair With Intravelar Veloplasty: A Single-institution Experience.

BACKGROUND: Measurements of postoperative velopharyngeal dysfunction (VPD) can be used to determine the efficacy of a palatoplasty operation. Hypernasality and audible nasal air emission are typical manifestations of VPD during speech. We aimed to longitudinally compare VPD outcomes in postpalatoplasty patients who underwent Furlow repair versus straight line repair with intravelar veloplasty (IVVP). Additionally, we examined the relationship between VPD outcomes and select pre-existing patient characteristics. METHODS: Retrospective chart review was performed to identify primary palatoplasty patients treated from April 2012 to March 2021. Variables collected included gender, syndromic status, primary language, Veau cleft type, type of speech assessment, age at time of surgery, degree of hypernasality, presence of audible nasal air emission, and overall adequacy of velopharyngeal function. Pearson χ2 test and multivariable t tests were used to analyze variables. Logistic regression was used to control for statistically significant variables. RESULTS: Of the 118 patients included, 38 received a Furlow procedure and 80 received a straight line with IVVP procedure. Audible nasal air emission was present in 57.3% of straight line with IVVP patients and 42.9% of Furlow patients, with no statistically significant difference between groups. Clinically significant hypernasality was present in 42.1% of straight line with IVVP patients and 22.9% of Furlow patients (P=0.05). Velopharyngeal function was classified as adequate in 63.5% of straight line with IVVP patients and 83.3% of Furlow patients (P=0.03). However, after stratifying by syndromic versus nonsyndromic status, there was no statistically significant difference between straight line with IVVP and Furlow patients for postoperative hypernasality and velopharyngeal function. CONCLUSIONS: This study suggests that there are no statistically significant differences between straight line with IVVP and Furlow palatoplasty techniques regarding speech outcomes including hypernasality, audible nasal air emission, and overall VP function. Furthermore, select patient characteristics such as gender, primary language, syndromic status, age at repair, and Veau cleft type do not significantly impact postoperative speech outcomes.

Cleft Care Packages and the Impact on Postoperative Care Following a Primary Cleft Lip Repair.

OBJECTIVE: To provide caregivers with all the resources needed to care for a surgical site following a primary cleft lip repair and evaluate its efficacy on postoperative care. SETTING/PARTICIPANTS: Caregivers of infants ages 3 to 6 months with a cleft lip and/or palate undergoing a primary repair at the Texas Children's Hospital. METHODS: Packages were given to caregivers at discharge following repair. Packages included instructions and supplies needed for surgical site care. At discharge an advanced practice provider obtained informed consent and a questionnaire that established baseline knowledge of surgical site care. Following the questionnaire, the advanced practice provider demonstrated how to care for the site using the package provided. Assessment of scar healing, nasal stent compliance, and ease of care was evaluated at postoperative follow up. RESULTS: Thirty-two families were enrolled in this study. Our data supports that caregivers who are provided resources to care for the site had increased comfort level, preparedness, and compliance rates following a primary cleft lip repair. Eighty-four percent of respondents strongly agreed that the package provided aided in preparedness for site care with 100% of respondents recommending the resources to future families undergoing a cleft lip repair. CONCLUSIONS: Caregivers feel comfortable and equipped with their ability to care for their child's repaired cleft lip when given the appropriate instructions and supplies. In addition, they would recommend the packages to future families following a repair. Empowering families to be proactive in postoperative care will potentially lead to better outcomes in cleft care.

Screening pediatric surgical patients during the COVID-19 pandemic.

ABSTRACT: SARS-CoV-2 has profoundly affected the way healthcare is delivered and has created significant strain on medical facilities globally. As a result, hospitals have had to continuously adapt in order to provide optimal patient care while minimizing the risk of SARS-CoV-2 transmission, particularly in the surgical setting. Texas Children's Hospital developed a set of protocols for surgical screening and clearance of patients in the context of the COVID-19 pandemic. These screening protocols were designed to mitigate the risk of exposing patients and healthcare providers to SARS-CoV-2 and have evolved significantly as a result of the emerging changes in medicine, technology, and governmental regulations. In this article, we share the reasoning behind the development, implementation, and successive modification of our institutional screening protocols.

Orthognathic Surgical Treatment in a Patient With Hyperimmunoglobulin E Syndrome.

Autosomal-dominant hyperimmunoglobulin E syndrome (HIES), or Job syndrome, is a rare, multisystem, primary immunodeficiency disorder. Additionally, patients may also suffer from connective tissue, dental, and bone malformations. While current management of HIES is directed at prophylactic antibiotics to prevent infections, there is limited work describing surgical considerations for these patients, particularly with respect to hardware placement. Here we report a case of a patient with HIES who underwent orthognathic surgery for maxillary advancement and mandibular setback to address his severe class III malocclusion. The patient's postoperative course was complicated by significant infection, requiring multiple operations and ultimately, hardware removal after bone healing. Although this patient ultimately had a good outcome, the role of orthognathic surgery with implant placement in patients with HIES should be approached with caution and careful consideration.

Speech Outcomes After LeFort I Advancement Among Cleft Lip and Palate Patients.

BACKGROUND: Velopharyngeal insufficiency (VPI) results from incomplete closure of the velopharyngeal (VP) sphincter with oral pressure consonants during speech. Maxillary hypoplasia is common among cleft children and often requires LeFort I advancement. This results in anterior movement of the soft palate with the bony maxillary segment. Consequently, the size of the VP sphincter is increased and may result postoperative VPI or worsening of prior VPI. To better counsel our patients and their families of the risk of VPI after LeFort I advancement, we chose to evaluate our own cohort. METHODS: We conducted an institutional review board-approved prospective review of all cleft children presenting to Texas Children's Hospital who underwent LeFort I advancement after previous palatoplasty between 2013 and 2016 in a three-surgeon, consecutive patient series. Data collected included age, sex, ethnicity, cleft type, prior secondary speech surgery, presence of preoperative fistula, planned distance of advancement, orthognathic surgery performed, and any concurrent procedures performed. Primary outcomes measured included preoperative and postoperative VP function and hypernasality as measured by a certified speech pathologist. RESULTS: Velopharyngeal function was unchanged in 67% of our cohort after LeFort I advancement. Of those patients, 83% had evidence of VPI preoperatively, and 17% had normal speech preoperatively. Twenty-two percent of the patients displayed worsening VP function after surgery, and 6% displayed evidence of improvement. Velopharyngeal function was unable to be assessed in 6% of patients. Nasality ratings worsened in 39% of patients, were unchanged in 39%, and improved in 22%. Of the patients with incompetent VP function after surgery, 50% already received or are currently scheduled for secondary speech surgery, 25% declined secondary surgery, and 25% are pending scheduling. CONCLUSIONS: Although VP function remains unchanged in a majority of patients after LeFort I advancement, VPI should be carefully screened for after surgery. If detected, secondary operations to correct speech should be strongly considered.

Cervical Stenosis in Non-Syndromic Pierre Robin Sequence.

Pierre Robin sequence is a triad of micrognathia, glossoptosis, and airway obstruction that has reported associations with numerous birth defects and anomalies including craniocervical pathologies. Given the number of airway assessments and neck manipulations these neonates experience, undetected cervical instability can result in detrimental consequences. The authors present a case of a neonate with non-syndromic Pierre Robin sequence planned for mandibular distraction osteogenesis that developed abnormal motor activity following intubation. The patient was eventually diagnosed with cervical spine stenosis and craniocervical instability after radiologic investigation. This paper demonstrates the significance of preoperative cervical spine evaluation and the importance of maintaining high level of suspicion when performing neck manipulations in those high-risk infants.

Spontaneous Mediastinal Abscess and Sternal Cleft in the Medically Complex Neonate.

Sternal cleft (SC) is a rare congenital anomaly, occurring with associated developmental anomalies or in isolation. Surgery to reconstruct the sternum is indicated to protect the visceral organs from trauma, to ensure healthy cardiopulmonary function and growth, and to reconstruct the anterior chest wall. Although infection recognized as a postoperative complication following chest wall reconstruction, spontaneous mediastinal infection is rare. To the authors' knowledge, there is only 1 reported case of spontaneous mediastinal infection with concomitant SC in the literature. Here, the authors present a unique case of a medically complicated infant with a SC who presented with a spontaneous mediastinal abscess.

Insurance Appeals for Pediatric Reconstructive Surgery: A Micro Cost Analysis and How-to Guide.

BACKGROUND: When insurance coverage for pediatric reconstructive procedures is denied, a stressful and time-consuming appeals process ensues. This article discusses the results of a micro cost analysis performed to better understand the time and monetary burden that the insurance process places on our clinic and families. We also advise plastic surgeons on how to appeal insurance denials for necessary reconstructive procedures. METHODS: Our micro cost analysis focused on patients with congenital breast anomalies who were denied insurance coverage during the preservice insurance authorization process. We surveyed staff and family members to determine the steps involved in the insurance process and how long each person spent on each step. We combined this with average compensation data to calculate cost. RESULTS: For the 5 patients included in our analysis, the insurance process took an average of 7.4 hours of institutional time and cost $521.43 per patient. All patients were denied coverage during prior authorization and required a peer-to-peer, and all denials occurred because surgery was deemed cosmetic or not medically necessary. CONCLUSIONS: This analysis estimated the time and monetary cost of the insurance process in our department. Access to care was limited by prior authorization and the opinion of medical directors that these procedures are unnecessary or cosmetic. We encourage plastic surgeons to continue to perform patient-centered outcomes research in their practice to build on literature that proves the functional and psychosocial benefits of reconstructive procedures.

Radioprotection With Amifostine Enhances Bone Strength and Regeneration and Bony Union in a Rat Model of Mandibular Distraction Osteogenesis.

BACKGROUND: Using distraction osteogenesis (DO) to regenerate robust endogenous bone could greatly enhance postoncologic reconstruction of head and neck cancer. However, radiation (XRT) corrosive effects still preclude DO's immense potential. We posit that adjunctive pretreatment with the radioprotectant amifostine (AMF) can optimize wound healing and allow for successful DO with quantifiable enhancements in bony union and strength despite previous surgical bed irradiation. METHODS: Two groups of murine left hemimandibles were exposed to a human equivalent radiation dosage fractionated over 5 daily doses of 7 Gy. AMF-XRT-DO (n = 30) received AMF before radiation, whereas XRT-DO (n = 22) was untreated. All animals underwent left hemimandibular osteotomy and external fixator placement, followed by distraction to a 5.1-mm gap. Left hemimandibles were harvested and mechanically tested for parameters of strength, yield, and breaking load. RESULTS: Radiation-related complications such as severe alopecia were significantly increased in XRT-DO compared with the AMF-treated group (P = 0.001), whereas infection and death were comparable (P = 0.318). Upon dissection, bony defects were grossly visible in XRT-DO distraction gap compared with AMF-XRT-DO, which exhibited significantly more complete unions (P = 0.004). Those results were significantly increased in the specimens prophylactically treated with AMF (yield: 39.41 N vs 21.78 N, P = 0.023; breaking load: 61.74 N vs 34.77 N, P = 0.044; respectively). CONCLUSIONS: Our study revealed that AMF enhances biomechanical strength, regeneration, and bony union after radiation in a murine model of DO. The use of prophylactic AMF in combination with DO offers the promise of an alternative reconstructive option for patients afflicted with head and neck cancer.

Safety Outcomes in Endoscopic Versus Open Repair of Metopic Craniosynostosis.

BACKGROUND: Premature fusion of the metopic suture leads to a range of clinical presentations, with more severe patients presenting characteristically with trigonocephaly. Endoscopic-assisted correction of craniosynostosis is emerging as an alternative to the gold-standard open cranial vault remodeling. While there are several documented benefits of endoscopic correction, the management of craniosynostosis varies widely in the literature with little consensus as to the preferred timing and surgical technique for repair. The purpose of this study was to investigate the safety outcomes of endoscopic versus open surgery at our institution. METHODS: This retrospective cohort study included all patients diagnosed with metopic craniosynostosis who underwent open or closed surgical management and received at least 6 months of follow-up. Data extraction was performed via chart review and included demographic information and safety outcome variables. RESULTS: During the study period, 17 patients who met our inclusion criteria were treated by the multidisciplinary craniofacial team at our institution. Eight patients underwent endoscopic surgery and 9 underwent open surgery. The mean age at surgery was significantly higher in the open surgery group than the endoscopic surgery group (13.7 months versus 2.3 months, respectively). Estimated blood loss, percent estimated blood volume lost, transfusion volume, procedure length, hospital length of stay, and intensive care unit (ICU) length of stay were all significantly higher in the open surgery group compared with the endoscopic group. CONCLUSIONS: In our patient population, endoscopic surgery for metopic craniosynostosis had an improved safety profile versus open surgery based on reduced procedure length, estimated blood loss, volume of blood transfusion, and length of stay in the ICU and hospital.

Shared Medical Appointments for Adolescent Breast Reduction.

Adolescents with macromastia face serious physical, emotional, and social burdens because of their large breast size. Studies have shown that reduction mammoplasty results in improvement in physical symptoms and quality of life for these patients. Shared medical appointments (SMAs), defined as individual patient-physician encounters that occur in a group setting, have been successfully applied to clinics for adult patients seeking breast reduction for this condition. We decided to apply the SMA model to our clinic for preoperative evaluation of adolescent patients with macromastia. The purpose of this article is to describe how our clinic implemented SMAs, evaluate changes in clinic efficiency, measure patient quality of life before surgery, and assess patient and provider satisfaction with the SMA model.From February to June 2016, our department instituted SMAs for preoperative evaluation of female adolescent patients with macromastia. We measured days from referral to appointment for 25 patients who attended SMAs and compared this with a retrospective cohort of 21 patients who attended traditional visits from 2013 to 2015.Clinic efficiency was measured in new patients per hour. During SMAs, we also administered the BREAST-Q, Pediatric Quality of Life Inventory, and patient and provider satisfaction surveys. Mean days between referral and office visit was reduced from 75.3 with traditional visits to 40.3 with shared appointments, although this finding was not statistically significant (P = 0.69). New patients per hour increased from 1.33 with traditional visits to 3 with SMAs, without reducing time spent on education or with the surgeon. The mean preoperative BREAST-Q scores were less than 40 for the 4 tested domains, and the mean (SD) total Pediatric Quality of Life Inventory score was 56.7 (14.8). Patients and the provider reported high satisfaction with SMAs, and the provider wishes to continue using SMAs in the clinic.In conclusion, SMAs resulted in high patient and provider satisfaction and increased clinic efficiency, without sacrificing time spent on education or with the surgeon. Low quality-of-life scores demonstrate a need for these patients to be evaluated and treated for their condition. Measuring patient-reported outcomes with validated surveys and improving clinic efficiency without sacrificing patient care have the potential to increase value at our institution.

Amifostine Treatment Mitigates the Damaging Effects of Radiation on Distraction Osteogenesis in the Murine Mandible.

According to the American Society of Clinical Oncology, in 2012, more than 53,000 new cases of head and neck cancers (HNCs) were reported in the United States alone and nearly 12,000 deaths occurred relating to HNC. Although radiotherapy (XRT) has increased survival, the adverse effects can be unrelenting and their management is rarely remedial. Current treatment dictates surgical mandibular reconstruction using free tissue transfer. These complex operations entail extended hospitalizations and attendant complications often lead to delays in initiation of adjuvant therapy, jeopardizing prognosis as well as quality of life. The creation of new bone by distraction osteogenesis (DO) generates a replacement of deficient tissue from local substrate and could have immense potential therapeutic ramifications. Radiotherapy drastically impairs bone healing, precluding its use as a reconstructive method for HNC. We posit that the deleterious effects of XRT on bone formation could be pharmacologically mitigated. To test this hypothesis, we used a rodent model of DO and treated with amifostine, a radioprotectant, to assuage the XRT-induced injury on new bone formation. Amifostine had a profound salutary effect on bone regeneration, allowing the successful implementation of DO as a reconstructive technique. The optimization of bone regeneration in the irradiated mandible has immense potential for translation from the bench to the bedside, providing improved therapeutic options for patients subjected to XRT.

Sternal Wound Salvage in Post-Transplant Adolescents: Omental Flap Reconstruction in Patients With Prior Abdominal Surgery.

Sternal wound infections remain a significant cause of morbidity and mortality in patients undergoing complex cardiothoracic surgery. Heart and lung transplant patients presumably face additional risk secondary to their underlying morbidity, postoperative immunosuppression, and difficulty with primary wound closure over large graft size. These patients present a unique challenge to the reconstructive surgeon, as many have a significant past surgical history, which can limit or alter treatment options. This study reports 2 pediatric transplant patients who underwent use of omental flap for sternal wound reconstruction in the context of significant past abdominal surgery. One patient underwent prior heart transplantation and the other patient underwent previous bilateral lung transplantation. Both had significant abdominal surgery prior to transplantation and suffered from sternal wound complications post-transplantation. Each patient was successfully treated with omental flap reconstruction.

Prenatal Identification of Pierre Robin Sequence: A Review of the Literature and Look towards the Future.

Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnostic studies could help the treating medical team as well as families prepare for these early airway emergencies. The medical literature was reviewed for different techniques used to prenatally diagnose PRS radiologically. We have reviewed these techniques and suggested a possible diagnostic pathway to consistently identify patients with PRS prenatally.

Isolated Unilateral Frontosphenoidal Craniosynostosis: A Rare Cause of Anterior Plagiocephaly.

Isolated unilateral frontosphenoidal synostosis is a rare and often misdiagnosed cause of synostotic frontal plagiocephaly. It is important to distinguish the various synostotic causes of frontal plagiocephaly from deformational frontal plagiocephaly because operative intervention is required in synostotic plagiocephaly to avoid progressive deformity, whereas deformational plagiocephaly typically improves without surgery. This study reports a patient with a unique case of anterior plagiocephaly caused by left-sided frontosphenoidal craniosynostosis. The workup should include a thorough history and clinical examination followed by computed tomography scan with three-dimensional reconstruction. Frontosphenoidal synostosis should be suspected in the absence of a coronal synostosis in a child with anterior unilateral plagiocephaly and treated surgically. Good aesthetic and functional results occur with prompt diagnosis and early surgical correction.

Plastic surgeon closure is comparable to orthopedic closure when a perioperative optimization protocol is instituted for pediatric patients with neuromuscular scoliosis.

BACKGROUND CONTEXT: Since 2015, plastic multilayer closure (PMC) has been gaining attraction due to improved wound healing outcomes for medically complex patients. Plastic multilayer closure has been readily used for complex spine surgery closures in patients susceptible to wound healing issues (ie, dehiscence, surgical site infection [SSI]). However, PMC requires extensive soft tissue manipulation compared with standard orthopedic spine surgeon closure (SOC) and can result in extended operative times, increased transfusion rates, and more frequent returns to the operating room. PURPOSE: From 2016 to 2019, our institution implemented a perioperative protocol designed to decrease postoperative complication rates in NMS patients. A retrospective cohort study was performed to determine if PMC imparted advantages over SOC above and beyond that from the perioperative protocol. STUDY DESIGN/SETTING: Retrospective study at a single academic institution. PATIENT SAMPLE: Eighty-one pediatric patients with neuromuscular scoliosis undergoing spinal fixation surgery. OUTCOME MEASURES: Postoperative wound complications such as surgical site infection, hematoma, and superficial/deep dehiscence were the main outcome measures. Respiratory and neuromuscular complications along with duration of surgery were also recorded. METHODS: A retrospective review was conducted of NMS patients undergoing spinal fixation at a single academic pediatric hospital over 4 years. Cases were labeled as SOC (n=41) or PMC (n=40) based on the closure technique applied. Reported 90-day complications were evaluated as the primary outcome. RESULTS: Of the 81 reviewed patients, 45 reported complications, roughly equal between the study groups. While we found no statistically significant differences in rates of postoperative complications or SSIs, SOC cases were 30 minutes shorter on average with fewer returns to the operating room for additional surgery. CONCLUSIONS: With the implementation of our perioperative protocol for NMS patients, PMC did not result in fewer complications than SOC but the surgeries did take longer.

Distraction osteogenesis following low-dose hyperfractionated irradiation in the rat mandible.

PURPOSE: The investigators hypothesized that low-dose hyperfractionated radiation would impair mandibular distraction osteogenesis (DO) in a murine mandibular model. MATERIALS AND METHODS: Male Sprague-Dawley rats underwent fractionated radiation (30 Gy) of the left mandible. After a 2-week recovery period, an external frame distractor was applied and gradual distraction of the mandible was performed. Tissue was harvested after a 28-day consolidation period. Gross, radiologic, and histologic evaluations were undertaken. Control animals underwent surgery for an identical time frame without preoperative radiation. RESULTS: Animals subjected to preoperative radiation (n = 10) showed suboptimal bone formation, including bone atrophy, incomplete bridging of the distraction gap, and gross bony defects or nonunion, compared with controls (n = 10). Although physical lengthening was achieved, irradiation consistently led to a detrimental effect on the normal process of DO. CONCLUSION: This set of experiments establishes a valuable rodent model to evaluate the effects of radiation on DO and may help to formulate strategies to optimize DO before it is widely applied in oncologic reconstruction.

Dose-response effect of human equivalent radiation in the mandible.

BACKGROUND: Despite widespread use of adjuvant irradiation for head and neck cancer, the extent of damage to the underlying bone is not fully understood but is associated with pathologic fractures, nonunion, and osteoradionecrosis. The authors' laboratory previously demonstrated that radiation significantly impedes new bone formation in the murine mandible. We hypothesize that the detrimental effects of human equivalent radiation on the murine mandible results in a dose-dependent degradation in traditional micro-computed tomography (micro-CT) metrics. METHODS: Fifteen male Sprague-Dawley rats were randomized into 3 radiation dosage groups: low (5.91 Gy), middle (7 Gy), and high (8.89 Gy), delivered in 5 daily fractions. These dosages approximated 75%, 100%, and 150%, respectively, of the biologically equivalent dose that the human mandible receives during radiation treatment. Hemimandibles were harvested 56 days after radiation and scanned using micro-CT. Bone mineral density, tissue mineral density, and bone volume fraction were measured along with microdensitometry measurements. RESULTS: Animals demonstrated dose-dependent adverse effects of mucositis, alopecia, weight loss, and mandibular atrophy with increasing radiation. Traditional micro-CT parameters were not sensitive enough to demonstrate statistically significant differences between the radiated groups; however, microdensitometry analysis showed clear differences between radiated groups and statistically significant changes between radiated and nonradiated groups. CONCLUSIONS: The authors report dose-dependent and clinically significant adverse effects of fractionated human equivalent radiation to the murine mandible. The authors further report the limited capacity of traditional micro-CT metrics to adequately capture key changes in bone composition and present microdensitometric histogram analysis to demonstrate significant radiation-induced changes in mineralization patterns.