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.

Novel Custom Maxillary Disimpaction Splint.

LeFort I, II, and III osteotomies are commonly used in complex craniofacial reconstruction. Patients requiring these procedures typically have a craniofacial cleft, other congenital craniofacial deformities, or severe facial trauma. Both the cleft and traumatized palate have poor bony support, which leads to possible complications when the disimpaction forceps are used during the downfracture of the maxilla. Such potential complications include trauma or formation of a fistula of the palatal, oral, or nasal mucosa; trauma to adjacent teeth; and fracture of the palate and alveolar bone. To help prevent these complications, we developed a custom disimpaction splint. The splint is designed to cover the palate and occlusal surfaces to increase retention and minimize splint movement during the maxillary downfracture portion of the surgical procedure. The base of the splint is fabricated from a two-layered biocryl material, and the palatal area is built with soft-cushion rebase material. This allows for a stable grip of the disimpaction forceps blades and provides protective coverage of the cleft, traumatized palate, or alveolar bone graft site during the downfracture. The custom maxillary disimpaction splint has been routinely used in our clinic from September 2019 to the present for LeFort osteotomies in patients with a compromised primary palate. No surgical complications related to the maxillary downfracture have been noted during this period of time. We conclude that the routine use of a custom maxillary disimpaction splint can result in improved outcomes and decreased complications of LeFort osteotomy procedures in patients with cleft and traumatized palate.

Modified Antia-Buch Flap Incorporating an Extended Temporal Scalp Incision.

The Antia-Buch flap is a popular reconstructive method for full-thickness ear defects involving the helical rim. However, scaphal or conchal resection is often required to prevent ear distortion. Noel et al described a modified technique to the Antia-Buch flap, which includes an incision in the temporal scalp and complete detachment of the preauricular helical root to increase mobility of the flap. Since then, no studies have reported on the use of this modification. We report our experience in implementing Noel et al's modification of the Antia-Buch flap for helical rim defects. Methods: The modified technique differs from the original Antia-Buch flap by completely detaching the root of the helix and adding a vertical incision to the temporal scalp to increase mobility of the flap. No scaphal resection is necessary. After complete elevation of the flaps, the flaps are advanced and inset toward each other followed by closure. Results: In our practice, 10 patients have been treated with Noel et al's modification to the Antia-Buch flap. In each of these patients, acceptable reconstruction of the helical rim was able to be achieved. All the patients were pleased with their reconstructive outcome and ear anatomy was able to be successfully maintained. Conclusions: The modified Antia-Buch flap has shown to be an excellent method for large, helical rim defects, creating versatility by adding the temporal scalp incision. Our outcomes with Noel et al's modification to the original Antia-Buch flap support this method as a versatile technique for wide full-thickness helical rim defects.

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.

A Custom Stabilizing Splint for the Management of BLCP with Protruding Premaxilla.

A severely protruding premaxilla in a patient with bilateral cleft lip and palate prevents functional closure of the orbicularis oris muscle and acceptable reconstruction of the nasolabial components during primary cheiloplasty. This is typically corrected with vomerine osteotomy and premaxillary setback, followed by cheiloplasty and rhinoplasty. Due to the risk of vascular compromise to the prolabium and premaxillary segment, the lip and nose repair is often staged after the vomerine osteotomy and premaxillary setback has healed. Stabilizing the premaxillary segment to allow adequate healing has been a topic of interest. Several methods have been described, but each is associated with varying degrees of compromise of the blood supply to the premaxilla. To combat this, the authors created a custom oral splint that effectively maintained the position of the premaxilla with minimal impingement of the blood supply. The authors present two cases in which a two-stage premaxillary setback with a custom-stabilizing oral splint was performed, followed by primary cheiloplasty and rhinoplasty in an age-appropriate and delayed presentation of bilateral cleft lip and palate and protruding premaxilla.

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.

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.

Frontofacial Reconstruction Technique Modification With Preservation of Blood Supply to the Monobloc Segment.

ABSTRACT: Craniosynostosis syndromes, including Apert Syndrome, Pfeiffer Syndrome, and Crouzon Syndrome, share similar phenotypes, including bicoronal craniosynostosis, midface hypoplasia, hypertelorism, and exorbitism. The standard surgical treatment for these craniofacial abnormalities is monobloc osteotomy with distraction osteogenesis. Complications of this technique include the failure of osteogenesis or resorption of the frontal bone. The authors propose an alternative surgical technique with a frontal arch in continuity with the midface segment to ensure vascularization to anterior and posterior borders of distraction. A case report of an 8-year-old female patient with Apert Syndrome is reported using our technique. Our frontal arch monobloc distraction procedure preserves blood supply to a cranial component of the monobloc segment site that becomes the anterior portion of distraction rather than with the traditional devascularized frontal bone flap. This technique modification should improve osteogenesis outcomes by preventing resorption or failure of bone formation.

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 Large Pediatric Wound Defects Using a Continuous External Tissue Expander.

Both internal and external tissue expanders take advantage of the innate adaptive mechanisms the skin exerts in response to mechanical tension, known as the stress-relaxation phenomenon. Internal tissue expander use is time-consuming and can be complicated by infection and extrusion. In this case series, continuous external tissue expanders used to manage large pediatric wounds were assessed. Fourteen patients (ages: 4 days to 17 years) with large wounds underwent continuous external tissue expansion intraoperatively. The success of wound closure was assessed. In addition, the size of the patient's wounds, duration of device application, and postoperative complications were evaluated. The continuous external tissue expander was applied to wound sizes ranging from 14.7 to 560 cm2 for 5 to 10 days until the wound was amenable for direct closure. In 11 of the 14 patients, delayed primary closure was achieved. The device significantly reduced the wound sizes of the remaining three cases (average 80% size reduction). There was no incidence of wound dehiscence or infection. This case series demonstrates the benefit of the continuous external tissue expansion in managing pediatric wounds that would not otherwise be amenable to primary closure. The method allows for timely closure with limited risk of infection or extrusion, and should be in the armamentarium of reconstructive plastic surgeons.

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.

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.

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.

Serial Posterior Cranial Vault Distraction for the Treatment of Complex Craniosynostosis.

ABSTRACT: Posterior cranial vault distraction is an important modality in the management of craniosynostosis. This surgical technique increases intracranial volume and improves cranial aesthetics. A single procedure is often inadequate in patients with complex multisuture craniosynostosis, as some will go on to develop intracranial hypertension despite the operation. Considering the negative effects of intracranial hypertension, some patients may warrant 2 planned distractions to prevent this scenario from ever occurring. Three patients with complex multiple-suture synostosis and severe intracranial volume restriction (occipital frontal head circumferences [OFCs] <1st percentile) were treated with 2 planned serial posterior cranial vault distractions at the institution between 2013 and 2018. Demographics, intraoperative data, and postoperative distraction data were collected. The OFC was recorded pre- and postdistraction, at 3- and 6-month follow-up appointments. Patients had a corrected average age of 18 weeks at the time of their initial procedure. There was an average of 38 weeks between the end of consolidation and the time for their 2nd distraction procedure. There was an average age of 79 weeks at the time of the 2nd procedure. All patients had a substantial increase in OFC and improvement of the posterior calvarium shape. The average increase in OFC was 5.2 cm after first distraction and 4.3 cm after 2nd distraction. No postoperative complications were encountered. Planned serial posterior cranial vault distraction is a safe and effective strategy for increasing intracranial volume, improving aesthetic appearance, and preventing the consequences of intracranial hypertension in patients with multisuture craniosynostosis and severe intracranial volume restriction.

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.

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.

Sinus Pericranii in Posterior Cranial Vault Distraction.

Anomalous venous connections between the extracranial and intracranial systems are relatively rare. These connections are also known as sinus pericranii (SP) have been reported previously in the setting of various cranial malformations including craniosynostosis. Angiography may be useful in the diagnosis of SP also allowing for differentiation of the dominance of the venous systems. Several methods have been described in the treatment of SP. In isolated cases both endovascular and open approaches have been successful in the treatment of SP. In cases wherein there is synostosis present, both single- and multiple-staged operations have been described. In this article, we describe 2 cases of posterior cranial distraction in the setting of sinus peri cranii.

Maxillary Distraction Osteogenesis in a Patient With Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is characterized by brittle bones, premature hearing loss, blue sclera, dental abnormalities, and short stature. Maxillofacial pathology is marked in many OI patients and includes a high incidence of class III malocclusion secondary to a retrusive maxilla relative to both the mandible and cranial base.Review of literature shows that most of the orthognathic surgeries performed in the setting of OI are double jaw surgeries, in the form of maxillary advancement and mandibular setback. However, severe maxillary hypoplasia is usually not correctable with single-stage maxillary advancement. Distraction osteogenesis (DO) is a technique that relies on the normal healing process that occurs between controlled, surgically osteotomized bone segments and it is a relatively widely used technique in modern management of craniofacial conditions.Distraction osteogenesis has been reported in only several patients with OI. There is only 1 previously documented case of maxillary distraction in the craniofacial literature. The authors present here the successful management of a patient with OI and severe class III malocclusion using LeFort I osteotomy and DO with an external rigid distractor.At 12 months follow-up, the patient had no complications and maintained stable maxillary position with normal occlusion, improvement of facial appearance, obstructive airway symptoms, speech, and chewing.This case serves to reinforce the safety and efficacy of DO in patients with OI. The authors did not significantly change our distraction protocol and did not have any complications, therefore the authors believe that DO should be the preferable treatment technique for severe malocclusion in OI patient population.

Use of Bone Morphogenetic Protein in Pediatric Cranioplasty With Pre- and Postoperative Radiation Therapy.

INTRODUCTION: Bone morphogenetic protein (BMP) is a signaling protein that has proven efficacy in the setting of bone repair. It has been widely used in orthopedic surgery and is being implemented more in the field of craniofacial surgery, although there is limited report on its use in pediatric patients. CASE: A 6-year-old female with stage IV neuroblastoma with metastasis to the parietal parasagittal calvarium, which had failed to respond to multiple medical therapies, including radiation therapy. The tumor was excised and the defect was replaced with a combination of split calvarial bone graft and rhBMP-2. The patient received post-operative radiation therapy with no reports of complications of the defect site on immediate and long term follow up. CONCLUSION: The use of BMP has the potential to aid in bone generation for high-risk calvarial defects. It can be safe and efficacious to use in the pediatric population; however, future studies should be done to determine the safest and most effective dosing of BMP.