Multidisciplinary Presurgical Education: Clinical Impact on Children With Orofacial Clefts Undergoing Maxillary Distraction via Rigid External Distraction.

OBJECTIVE: Treatment of severe maxillary hypoplasia is commonly addressed via distraction osteogenesis with a rigid external device (RED). While effective, this method can be socially stigmatizing in an already vulnerable patient population. To prepare children and their caregivers for life with a RED and decrease peri-operative anxiety, we instituted a multidisciplinary pre-surgical education session (MPES). This educational team involves our cleft care coordinator, child life specialist, orthodontist and plastic surgeon 2 weeks prior to surgery. We reviewed the impact of this intervention by examining clinical outcomes before and after its implementation. DESIGN: From February 2017 to February 2020, a retrospective chart review was performed to include patients with orofacial clefts and maxillary hypoplasia who underwent maxillary distraction osteogenesis with RED at our center before (28 patients) and after (29 patients) the implementation of MPES. RESULTS: MPES was associated with a significantly shorter length of stay compared to controls who did not receive MPES (3.6 vs 3.1 days, p < 0.03) and significantly decreased usage of inpatient narcotic pain medication compared to controls (16.8 morphine equivalents vs 31.8 morphine equivalents, p < 0.02). Our intervention also demonstrated a trend towards decrease in minor complications but did not achieve statistical significance p = 0.32). CONCLUSIONS: Multidisciplinary presurgical education is a beneficial adjunct in the care of patients with orofacial clefts and maxillary hypoplasia undergoing maxillary advancement with a RED.

Implementation of Telehealth in a Pediatric Plastic Surgery Clinic: A Single Center's Response to COVID-19.

BACKGROUND: With limitation of in-person clinical visits due to the COVID-19 pandemic, telehealth has emerged as an important platform to deliver healthcare. Satisfaction with telehealth has been examined previously in the adult plastic surgery population. However, studies examining its use and satisfaction in the pediatric plastic surgery population are limited. In this study, we explore patient/caregiver satisfaction with the rapid institution of telehealth in our pediatric plastic surgery clinic and factors affecting satisfaction scores. METHODS: From March 2020 to February 2021, families of pediatric patients seen via video telehealth in the pediatric plastic surgery clinic at our center were invited to participate in a posttelehealth session survey. Telehealth visits encompassed both preliminary consultations as well as follow-up visits. After appropriate consent was obtained, patients' families completed a posttelehealth survey conveying demographics and satisfaction with telehealth via a 5-point Likert scale. RESULTS: Seventy eight of 131 eligible families elected to participate. Visit types included the following: initial consultations (23.1%), preoperative visits (10.3%), postoperative visits (29.5%), and follow-up visits (37.1%). A total of 80.3% of the respondents were overall satisfied with their telehealth experience. A total of 85.3% of the respondents agreed that telehealth made it easier for them to see their doctor, and 96.7% of the respondents felt using telehealth saved them time in traveling to the clinic. A total of 89.5% of the respondents felt it important for the state of Utah to have a telehealth program available to pediatric patients. CONCLUSIONS: Video telehealth is a useful adjunct in the care of pediatric plastic surgery patients with high caregiver satisfaction.

Management of sagittal synostosis in the Synostosis Research Group: baseline data and early outcomes.

OBJECTIVE: Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research. METHODS: All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables. RESULTS: Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group. CONCLUSIONS: The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Management of Tessier Type 3 Cleft With a Novel Reverse Palatal Expander.

ABSTRACT: The purpose of this clinical report is to present the novel management of a type Tessier 3 cleft which was treated using a palatal expander in reverse fashion to reapproximate the craniofacial skeleton allowing for closure of the palate and soft tissue of the cleft. Reapproximation of the bony component of the cleft was achieved without osteotomies and allowed for easier and earlier realignment of the bony and soft tissue components of the cleft. To our knowledge, this is the first use of reverse palatal expansion in the treatment of type 3 Tessier cleft. Reverse palatal expansion made management of this cleft more straightforward and should be considered as a useful adjunct in the management of wide facial clefts.

Evaluation of the Patient-Practitioner Consultation on Surgical Treatment Options for Patients With Craniosynostosis.

INTRODUCTION: Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options. METHODS: An online survey was developed and distributed to 31 providers. The response rate was 84% (26/31). RESULTS: Twenty-six (100%) respondents offer a minimally invasive surgical option for sagittal craniosynostosis, 21 (81%) for coronal, 20 (77%) for metopic, 18 (69%) for lambdoid, and 12 (46%) for multi-suture. Social issues considered in determining whether to offer a minimally invasive option include anticipated likelihood of compliance (23 = 88%), distance traveled for care (16 = 62%) and financial considerations (6 = 23%). Common tools to explain options include verbal discussion (25 = 96%), 3D reconstructed CT scans (17 = 65%), handouts (13 = 50%), 3D models (12 = 46%), hand drawings (11 = 42%) and slides (10 = 38%). Some respondents strongly (7 = 27%) or somewhat (3 = 12%) encourage a minimally invasive option over open repair. Others indicate they remain neutral (7 = 27%) or tailor their approach to meet perceived needs (8 = 31%). One (4%) somewhat encourages open repair. Despite this variation, all completely (17 = 65%), strongly (5 = 19%) or somewhat agree (4 = 15%) they use shared decision making in presenting surgical options. CONCLUSION: This survey highlights the range of practice patterns in presenting surgical options to families and reveals possible discrepancies in the extent providers believe they use shared decision making and the extent it is actually used.

Early Results on the Efficacy of Demineralized Bone Matrix, Bone Morphogenic Protein, and Freeze-dried Bone Chips in Alveolar Cleft Repair.

Background: Conventional treatment for alveolar cleft repair is done using autologous iliac crest alveolar bone graft (ABG). However, this method may not be ideal in all patients. Analysis of the efficacy of a mixture of demineralized bone matrix (DBX), bone morphogenic protein (rhBMP-2), and freeze-dried bone chips (FDBC) as an alternative for alveolar cleft repair was performed. Methods: Consecutive patients from August 2019 to June 2022 undergoing early alveolar cleft repair, concomitant hard palate and alveolar cleft repair, secondary alveolar cleft repair, and regrafting from a previously failed ABG were analyzed. Computed tomography scans were performed to evaluate graft take at least 6 months postoperatively. Images were reviewed and scored. Alveolar graft height and graft thickness were recorded. A standardized scoring system was developed, with a score of 0 representing no graft take and 3 representing best possible graft take. Results: Fifty-five consecutive alveolar clefts (43 patients) were identified as having undergone ABG and satisfied all the other inclusion criteria. Of these, 29 underwent first time ABG and 26 underwent redo ABG. The mean graft height and graft thickness recorded for all clefts was 2.2 and 2.0, respectively. Conclusions: Early results evaluating the efficacy of ABG using DBX, rhBMP-2, and FDBC show feasibility in regard to both graft height and thickness when using a maxillary computed tomography scan to measure the bone graft take. These results suggest that DBX, rhBMP-2, and FDBC may act as a versatile bone graft material in cleft care, although further studies are needed to determine long-term outcomes.

Mandibular Myofibroma and Severe Trismus: A Complex Case and Review of Complications.

A female child was investigated for insidious onset of temporomandibular joint dysfunction and trismus in the setting of a mandibular myofibroma. Myofibromas, benign mesenchymal neoplasms composed of spindle cells, are rarely found in the oral cavity, most commonly in the mandible. These lesions are historically described as indolent with a high cure rate and minimal recurrence rates following surgical resection. The patient initially presented with concerns regarding snoring, retrognathia, and jaw ankylosis, as well as a history of trouble latching as an infant but without obvious physical deformities. Imaging revealed a large expansile lytic mass of the mandible, but no temporomandibular joint involvement; surgical biopsy evidenced myofibroma, and the lesion was resected. Over the course of disease, the lesion continued to expand, and the patient's maximal incisal opening continued to decrease despite conservative management with jaw physiotherapy; eventually she could not open her mouth despite the absence of joint involvement. Re-exploration along with formal jaw physiotherapy was achieved and optimal jaw opening was maintained. Myofibromas are rare benign desmoid tumors that can present anywhere in the body in solitary and multicentric forms, and previously did not present significant challenges to surgical and medical management. Tumors of the mandible may present with trismus and soft tissue ankylosis, which can mimic temporomandibular joint dysfunction in the absence of joint involvement. Physical therapy, rehabilitation, and soft tissue contracture release are key to management and improving outcomes in oral cancer patients, regardless of tumor pathology.

A multi-institutional survey on calvarial vault remodeling techniques for sagittal synostosis and outcomes analysis for patients treated at 12 months and older.

OBJECTIVE: Surgical treatment of sagittal craniosynostosis is challenging in older patients. This study aimed to assess the effect of increasing age on open surgical technique selection and patient outcomes using the multi-institutional Synostosis Research Group (SynRG) collaboration. METHODS: Surgeons in SynRG were surveyed for key influences on their preferred open calvarial vault remodeling techniques at various patient ages: < 6, 6-12, and > 12 months. The SynRG database was then queried for open repairs of nonsyndromic sagittal craniosynostosis performed for patients older than 12 months of age. Perioperative measures, complications, and preoperative and postoperative cephalic indices were reviewed. RESULTS: All surgeons preferred to treat patients at an earlier age, and most (89%) believed that less-optimal outcomes were achieved at ages older than 12 months. The modified pi procedure was the dominant technique in those younger than 12 months, while more involved open surgical techniques were performed for older patients, with a wide variety of open calvarial vault remodeling techniques used. Forty-four patients met inclusion criteria, with a mean (± SD) age at surgery of 29 ± 16 months. Eleven patients underwent parietal reshaping, 10 parietal-occipital switch, 9 clamshell craniotomy, 7 geometric parietal expansion, 6 modified pi procedure, and 1 parietal distraction. There were no readmissions, complications, or mortality within 30 days postoperatively. Patients' cephalic indices improved a mean of 6.4% ± 4.0%, with a mean postoperative cephalic index of 74.2% ± 4.9%. Differences in postoperative cephalic index (p < 0.04) and hospital length of stay (p = 0.01) were significant between technique cohorts. Post hoc Tukey-Kramer analysis identified the parietal reshaping technique as being significantly associated with a reduced hospital length of stay. CONCLUSIONS: Patient age is an important driver in technique selection, with surgeons selecting a more involved calvarial vault remodeling technique in older children. A variety of surgical techniques were analyzed, with the parietal reshaping technique being significantly associated with reduced length of stay; however, multiple perioperative factors may be contributory and require further analysis. When performed at high-volume centers by experienced pediatric neurosurgeons and craniofacial surgeons, open calvarial vault techniques can be a safe method for treating sagittal craniosynostosis in older children.

Preoperative imaging patterns and intracranial findings in single-suture craniosynostosis: a study from the Synostosis Research Group.

OBJECTIVE: The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort. METHODS: In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type. RESULTS: A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring. CONCLUSIONS: The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.

Alveolar Bone Graft Stabilization with Custom Maxillary Splints.

For secondary alveolar bone grafting in cleft patients, the success of bone graft take is dependent upon creating an ideal environment for both bony and soft tissue healing. This is particularly challenging in patients with existing fistulas, wide clefts, and bilateral alveolar clefts, where large soft tissue mobilization is required to get a tensionless repair, and micro-motion around the bone graft is significantly higher. Herein we describe our method for manufacture and placement of a custom postoperative maxillary splint following secondary alveolar bone grafting. Our splint encompasses the palate and alveolus to stabilize the maxillary arch and protect the incision lines during healing. We find our splint to be a useful adjunct to facilitate postoperative healing following secondary alveolar bone grafting.

Layered Closure of Lumbosacral Myelomeningocele Defects with Bilateral Paraspinous Muscle and Composite Fasciocutaneous Flaps.

Robust, reliable, and reproducible closure of lumbosacral myelomeningocele defects remains a challenge. In infants with lumbosacral myelomeningocele defects, multiple methods of soft tissue coverage have been described. These include various cutaneous, fascial, and muscle flaps and grafts. This is done with relative ease when ample soft tissue is present but becomes extremely difficult for large and distally located defects. We present here our closure technique of lumbosacral myelomeningocele defects in newborns, with associated short- and medium-term outcomes. We demonstrate the anatomy of this technique with fresh cadaver dissection and present a review of demographic and outcome data of 12 consecutive patients treated with this method from June 2014 to August 2019. No major intra- or postoperative complications have been encountered, with a mean follow-up of 22.2 months and median follow up of 18 months. After the neurosurgical repair of lumbosacral myelomeningocele, bilateral composite fascial flaps composed of thoracolumbar and gluteus maximus fascia are elevated in continuity. The paraspinous muscle flaps are then elevated, disinserted distally, and medialized to provide complete muscular coverage of the dural repair. The bilateral composite fascial flaps are medialized and closed over the deep paraspinous muscle flap repair. Two patients experienced areas of small, superficial skin necrosis, one of which healed by secondary intention and the other by debridement and full-thickness skin grafting. Use of bilateral paraspinous muscle flaps and bilateral composite fascial flaps composed of thoracolumbar and gluteus maximus fascia provides robust coverage of lumbosacral defects following myelomeningocele repair in infants.

Management of deep wound complications with vacuum-assisted therapy after Berlin Heart EXCOR ventricular assist device placement in the pediatric population.

Wound complications after ventricular assist device (VAD) placement remain a formidable challenge to surgeons. The Berlin Heart EXCOR VAD is a versatile pulsatile system that has been successful in pediatric patients of all ages and sizes. Prevention of device-related complications such as infection, particularly in pediatric patients, remains an essential issue in minimizing patient morbidity and mortality. The introduction of vacuum-assisted wound closure (VAC) therapy and its application in VAD-related wound complications provide an efficient and effective method for wound healing. We report our experience in the management of deep wound complications in two pediatric patients after placement of the Berlin Heart EXCOR VAD. The wound VAC system proved to achieve complete wound healing without any infectious complications.

Pyoderma Gangrenosum-like Wounds in Leukocyte Adhesion Deficiency: Case Report and Review of Literature.

Leukocyte adhesion deficiency (LAD) is a rare primary immunodeficiency characterized by impairment of leukocyte migration during an inflammatory response. LAD patients can experience recurrent neutrophilic wounds similar to pyoderma gangrenosum (PG), predominantly of the skin and mucosal surfaces. There have been only a few reports addressing the management of extensive, life-threatening wounds in LAD patients. We describe here both the systemic and local management employed to successfully treat a severe PG-like cutaneous lesion in the setting of LAD in a 9-year-old female. A comprehensive literature review was performed to identify previously reported similar cases. Under aggressive systemic and local management, the wound was stabilized and complete epithelialization was achieved in 8 months. Eight studies documenting 11 patients with LAD and PG-like lesions were identified in our review of the literature. The complexity of wounds associated with LAD requires an aggressive, multidisciplinary approach. Involvement of pediatrics, immunology, plastic surgery, infectious disease, and physical therapy is essential to obtaining a positive outcome. In the setting of LAD with PG-like lesions, the only viable option is allowing for closure by secondary epithelialization. This was achieved in our patient once the wound was stabilized with the systemic administration of infliximab and topical administration of tacrolimus.

Variation in the management of isolated craniosynostosis: a survey of the Synostosis Research Group.

OBJECTIVEThe authors created a collaborative network, the Synostosis Research Group (SynRG), to facilitate multicenter clinical research on craniosynostosis. To identify common and differing practice patterns within the network, they assessed the SynRG surgeons' management preferences for sagittal synostosis. These results will be incorporated into planning cooperative studies.METHODSThe SynRG consists of 12 surgeons at 5 clinical sites. An email survey was distributed to SynRG surgeons in late 2016, and responses were collected through early 2017. Responses were collated and analyzed descriptively.RESULTSAll of the surgeons-7 plastic/craniofacial surgeons and 5 neurosurgeons-completed the survey. They varied in both experience (1-24 years) and sagittal synostosis case volume in the preceding year (5-45 cases). Three sites routinely perform preoperative CT scans. The preferred surgical technique for children younger than 3 months is strip craniectomy (10/12 surgeons), whereas children older than 6 months are all treated with open cranial vault surgery. Pre-incision cefazolin, preoperative complete blood count panels, and an arterial line were used by most surgeons, but tranexamic acid was used routinely at 3 sites and never at the other 2 sites. Among surgeons performing endoscopic strip craniectomy surgery (SCS), most create a 5-cm-wide craniectomy, whereas 2 surgeons create a 2-cm strip. Four surgeons routinely send endoscopic SCS patients to the intensive care unit after surgery. Two of the 5 sites routinely obtain a CT scan within the 1st year after surgery.CONCLUSIONSThe SynRG surgeons vary substantially in the use of imaging, the choice of surgical procedure and technique, and follow-up. A collaborative network will provide the opportunity to study different practice patterns, reduce variation, and contribute multicenter data on the management of children with craniosynostosis.

Minimizing bone gaps when using custom pediatric cranial implants is associated with implant success.

OBJECT: Occasionally after a craniotomy, the bone flap is discarded (as in the case of osteomyelitis) or is resorbed (especially after trauma), and an artificial implant must be inserted in a delayed fashion. Polyetheretherketone (PEEK) implants and hard-tissue replacement patient-matched implants (HTR-PMI) are both commonly used in such cases. This study sought to compare the failure rate of these 2 implants and identify risk factors of artificial implant failure in pediatric patients. METHODS: This was a retrospective cohort study examining all pediatric patients who received PEEK or HTR-PMI cranioplasty implants from 2000 to 2013 at a single institution. The authors examined the following variables: age, sex, race, mechanism, surgeon, posttraumatic hydrocephalus, time to cranioplasty, bone gap width, and implant type. The primary outcome of interest was implant failure, defined as subsequent removal and replacement of the implant. These variables were analyzed in a bivariate statistical fashion and in a multivariate logistic regression model for the significant variables. RESULTS: The authors found that 78.3% (54/69) of implants were successful. The mean patient age was 8.2 years, and a majority of patients were male (73%, 50/69); the mean follow-up for the cohort was 33.3 months. The success rate of the 41 HTR-PMI implants was 78.1%, and the success rate of the 28 PEEK implants was 78.6% (p = 0.96). Implants with a bone gap of > 6 mm were successful in 33.3% of cases, whereas implants with a gap of < 6 mm had a success rate of 82.5% (p = 0.02). In a multivariate model with custom-type implants, previous failed custom cranial implants, time elapsed from previous cranioplasty attempt, and bone gap size, the only independent risk factor for implant failure was a bone gap > 6 mm (odds ratio 8.3, 95% confidence interval 1.2-55.9). CONCLUSIONS: PEEK and HTR-PMI implants appear to be equally successful when custom implantation is required. A bone gap of > 6 mm with a custom implant in children results in significantly higher artificial implant failure.