Impact of multidisciplinary engagement in a quality improvement blood conservation protocol for craniosynostosis.

OBJECTIVE: Patients undergoing open cranial vault remodeling for craniosynostosis frequently experience substantial blood loss requiring blood transfusion. Multiple reports in the literature have evaluated the impact of individual blood conservation techniques on blood transfusion rates during craniosynostosis surgery. The authors engaged a multidisciplinary team and assessed the impact of input from multiple stakeholders on the evolution of a comprehensive quality improvement protocol aimed at reducing or eliminating blood transfusion in patients undergoing open surgery for craniosynostosis. METHODS: Over a 4-year period from 2012 to 2016, 39 nonsyndromic patients were operated on by a single craniofacial plastic surgeon. Initially, no clear blood conservation protocol existed, and specific interventions were individually driven. In 2014, a new pediatric neurosurgeon joined the craniofacial team, and additional stakeholders in anesthesiology, transfusion medicine, critical care, and hematology were brought together to evaluate opportunities for developing a comprehensive blood conservation protocol. The initial version of the protocol involved the standardized administration of intraoperative aminocaproic acid (ACA) and the use of a cell saver. In the second version of the protocol, the team implemented the preoperative use of erythropoietin (EPO). In addition, intraoperative and postoperative resuscitation and transfusion guidelines were more clearly defined. The primary outcomes of estimated blood loss (EBL), transfusion rate, and intraoperative transfusion volume were analyzed. The secondary impact of multidisciplinary stakeholder input was inferred by trends in the data obtained with the implementation of the partial and full protocols. RESULTS: Implementing the full quality improvement protocol resulted in a 66% transfusion-free rate at the time of discharge compared to 0% without any conservation protocol and 27% with the intermediate protocol. The administration of EPO significantly increased starting hemoglobin/hematocrit (11.1 g/dl/31.8% to 14.7 g/dl/45.6%, p < 0.05). The group of patients receiving ACA had lower intraoperative EBL than those not receiving ACA, and trends in the final-protocol cohort, which had received both preoperative EPO and intraoperative ACA, demonstrated decreasing transfusion volumes, though the decrease did not reach statistical significance. CONCLUSIONS: Patients undergoing open calvarial vault remodeling procedures benefit from the input of a multidisciplinary stakeholder group in blood conservation protocols. Further research into comprehensive protocols for blood conservation may benefit from input from the full surgical team (plastic surgery, neurosurgery, anesthesiology) as well as additional pediatric subspecialty stakeholders including transfusion medicine, critical care, and hematology.

Innovative Surgical Treatment of Severe Cherubism.

BACKGROUND: Cherubism is an autosomal dominant syndrome characterized by excessive bilateral maxillomandibular bony degeneration and fibrous tissue hyperplasia. Conservative management is the preferred treatment as cherubism has a self-limiting course. Functional or emotional disturbances may, however, demand surgical intervention. We report a patient who underwent surgical intervention. METHOD/DESCRIPTION: He had significant enlargement of lower cheeks and bilateral lower lid scleral show. On computed tomography of the face, the patient had significant fibrous tissue involving bilateral maxilla and mandible. The mandibular tumor was excised. Given normal inferior border, bilateral sagittal split osteotomy was performed to infracture and inset the outer cortex. During the procedure, patient required blood transfusion intraoperatively, so the maxillary portion of the procedure was delayed until 6 months later. For the maxilla, bilateral transconjunctival approach was used to resect parts of the orbital floors that were concave, resulting in 1 × 2 cm defects bilaterally which were reconstructed using resorbable plates. Then the anterior maxillary tumor was excised. RESULTS: The patient and his parents were satisfied with his appearance after surgery. The patient was noted to have improvement in contour and decreased scleral show. He has most recently followed up 15 months after the initial surgery. There were no long-term complications. CONCLUSIONS: Severity of cherubism influences the type of surgical intervention. The present case is innovative because this is the first reported case of recontouring orbital floors with resorbable plates and infracturing of the mandible using sagittal split osteotomies for surgical treatment of cherubism.

Surgical Management of Deep Brain Stimulator Scalp Erosion without Hardware Removal.

BACKGROUND: Scalp erosion in patients with deep brain stimulation (DBS) hardware is an uncommon complication that lacks a clearly defined management strategy. Previous studies have described various therapies including conservative treatment with antibiotics and surgical debridement with or without hardware removal. OBJECTIVES: The aim of this study was to review the efficacy of a hardware-sparing management strategy for the treatment of scalp erosion. METHODS: Five patients with previous DBS implantation presented with scalp erosion and visible hardware exposure at the calvarial burr hole site, and underwent tension-free, vascularized, rotational scalp flap, with preservation of the leads under the pericranium. Two of the procedures were performed after an unsuccessful attempt at primary closure and 3 as a primary procedure. Each patient was followed clinically for at least 14 months postoperatively to evaluate for wound-healing and adverse effects. RESULTS: The median duration from initial DBS hardware implantation to erosion and revision surgery was 12 months (range 1.5-62 months). Three patients were documented to have positive intraoperative cultures in spite of the absence of purulence. At the last follow-up, all patients were noted to have complete wound-healing and no evidence of infection or erosion. CONCLUSIONS: DBS scalp erosion can be managed by rotational scalp flap without hardware removal, even in cases where infection is identified.

Impact of a Cleft and Craniofacial Center on an Academic Health System.

BACKGROUND: The contributions of all physician specialties and ancillary services involved in cleft and craniofacial center care must be evaluated to fairly assess the financial impact of a cleft and craniofacial center. The authors hypothesized that the cleft and craniofacial center generates profitable downstream productivity for the academic health system. METHODS: This was a retrospective cohort study of all patients who presented to a cleft and craniofacial center in the first quarter of 2011. Analysis included all health system encounters for each patient over a 2-year period using the electronic medical record and health system financial database. RESULTS: Sixty-two patients were seen (mean age, 11.4 years; 38 boys and 24 girls; 18 new and 44 established patients). Over 2 years, there were 618 health system encounters (599 outpatient and 19 inpatient encounters), 68 hospital days, and 110 procedures. The most common physician specialty was plastic surgery [312 encounters (50.5 percent)] and the most common ancillary service was speech therapy [256 encounters (41.4 percent)]. The overall reimbursement rate was 39.9 percent, with a majority payor-mix of government payors (62.1 percent). The total profit margin percentage from all encounters was 13.7 percent, which was greater for managed care compared with government payor (38.9 percent versus -10.8 percent; p = 0.022), inpatient compared to outpatient (24.5 percent versus -2.8 percent; p < 0.001), and plastic surgery compared to other specialty encounters (19.7 percent versus 8.7 percent; p = 0.003). CONCLUSIONS: The cleft and craniofacial center generated profitable downstream productivity for the academic health system with referrals to 39 different physician and nonphysician specialties. Health system providers and the business team should align to analyze the center, enhance patient outcomes, and improve specialty care access for patients.

Cleft and Craniofacial Clinic Formats in the United States: National and Institutional Survey.

BACKGROUND: Craniofacial teams employ multidisciplinary clinics to optimize patient care. Different clinic formats exist among teams. Formats include providers rotating from room to room as separate specialties, patients rotating from room to room to either separate specialties or as 1 group, as well as providers rotating together as 1 group. Surveys were used to study family preferences between the different formats and to compare them with trends of national practices. METHODS: Families of the authors' team clinic patients were surveyed from November 2012 to February 2013, after a clinic format change from patients moving between rooms to see providers, to providers moving between rooms to see patients. This survey focused on patient satisfaction, clinic format preference, and their perception of efficiency. A second, national survey was distributed to 161 American craniofacial teams approved by the American Cleft Palate-Craniofacial Association to survey clinic formats, provider satisfaction, and experience with other formats. Institutional survey data were tabulated as percentages and further analyzed using the Mann-Whitney Test. The national survey data was then tabulated and compared with authors' institutional results. RESULTS: Thirty-nine of 54 (72.2%) families responded to the institutional survey. Providers moving between rooms were associated with greater patient satisfaction (mean 4.8 of 5, 5 being most satisfied) (0<0.0001), shorter perceived clinic time (76.9%), and an increased sense of comfort (84.6%). The difference in satisfaction rates was statistically significant (P <0.0001) between the primary clinic formats of providers rotating (mean of 4.8) and patients rotating (mean of 2.4).The national survey had 93 responses of 161 (57.7%). 54.9% of respondents have providers rotating between examination rooms, and 32.3% have patients moving between rooms. Other formats included the entire team moving as a group between rooms (10.8%) and specialties sitting together in 1 room while patients rotate (9.7%). Respondents were satisfied with current formats (mean 4.24 of 5, 5 being most satisfied). 22.2% had tried a different format previously. CONCLUSION: The most common American cleft and craniofacial clinic format is providers moving between rooms; however, all formats have high provider satisfaction. At our institution, patients prefer when providers move between rooms. Our study suggests that clinic formats do not need to be standardized, and the clinic format utilized should be tailored to the individual needs of the institution.

A Novel Single-Staged Technique for the Treatment of Soft Tissue in an Oblique Facial Cleft.

INTRODUCTION: Oblique facial clefts are extremely rare and cause significant morbidity. Treatment of these clefts is complex and requires a fundamental understanding of cleft classification and techniques used for treatment of clefts. METHODS: We describe a novel single-staged technique to repair the Tessier no. 4 soft tissue cleft and reconstruct the buccal sulcus and bilaminar lower eyelid by preserving normally excised tissue combined with standard procedures. We also present a case report demonstrating the technique in an adolescent female. The procedure incorporates turnover flaps from soft tissue preservation within the cleft, a Mustarde cheek advancement flap, an anatomical subunit lip repair, a dorsal nasal Rieger flap for ala repositioning, and a lateral nasal flag flap. RESULTS: The single-staged soft tissue repair eliminated the Tessier no. 4 cleft while simultaneously reconstructing the bilaminar lower eyelid and buccal lining. Our patient had no complications within the perioperative period. CONCLUSIONS: This novel single-staged technique for the treatment of the soft tissue Tessier no. 4 cleft not only repairs the cleft but also reconstructs the buccal sulcus and bilaminar lower eyelid with turnover flaps preserved from the normally discarded excess soft tissue within the cleft. The novel repair allows for the creation of a deeper fornix to aid with placement of an orbital prosthesis and is ideal for use in underserved or remote locations.

Social and Support Services Offered by Cleft and Craniofacial Teams: A National Survey and Institutional Experience.

BACKGROUND: A multidisciplinary approach to patients with craniofacial abnormalities is the standard of care by the American Cleft Palate-Craniofacial Association (ACPA). The standards of team care, however, do not require provision of social support services beyond access to a social worker. The purpose of this investigation is to study social support services provided by ACPA teams, funding sources for services, and family interest in services. METHODS: A survey was submitted to ACPA cleft and craniofacial team leaders (N = 161), which evaluated the provision of potentially beneficial social support services, and their funding sources. A second survey administered to patient families at our institution gauged their level of interest in these services. Statistical analysis evaluated the level of interest among services. RESULTS: Seventy-five of 161 (47%) teams and 39 of 54 (72%) families responded to the surveys. Services provided included scholarships (4%), summer camp (25%), social media (32%), patient support groups (36%), parties (42%), parent support groups (46%), other opportunities (56%), and social workers (90%). The majority of funding for social workers was by the institution (61%) whereas funding for ancillary services varied (institution, team, fundraisers, grants, and other sources). Families indicated an average interest of 2.4 ±â€Š1.41 for support groups, 2.5 ±â€Š1.63 for summer camps, 2.92 ±â€Š1.66 for parties, 3.16 ±â€Š1.65 for social media, and 3.95 ±â€Š1.60 for scholarships (P value <0.05). CONCLUSIONS: The ACPA standards of team care do not require teams to provide social support services beyond access to a social worker. Among our survey respondents, the authors found that in addition to a social worker, teams offered social support services, which were not required. The social worker position is usually institutionally funded, whereas funding sources for additional services varied. Respondents at our center desired additional social support services. The authors recommend a hybrid model of hospital and nonhospital funding to provide social and support services to patients with craniofacial deformities.

Postnatal Pancraniosynostosis in a Patient With Infantile Hypophosphatasia.

Hypophosphatasia is a rare metabolic bone disorder that predisposes patients to craniosynostosis. Typically, patients born with hypophosphatasia will exhibit fused cranial sutures at birth. This is the first reported case of delayed onset of pancraniosynostosis in a patient with infantile hypophosphatasia. The severity of onset and delayed presentation in this patient are of interest and should give pause to those care providers who treat and evaluate patients with hypophosphatasia.

45 Years of Simultaneous Le Fort III and Le Fort I Osteotomies: A Systematic Literature Review.

OBJECTIVE: To review and collectively summarize our knowledge of simultaneous Le Fort III and Le Fort I osteotomies. DESIGN: A PubMed search using "Le Fort III," "simultaneous Le Fort III and Le Fort I," "combined Le Fort III and Le Fort I," "dual midface," and "segmental midface" was performed. Articles with relevant abstracts were obtained for formal review. A new case of simultaneous Le Fort III and Le Fort I is presented to describe and discuss specific operative indications and surgical decisions. RESULTS: There were 14 articles that met inclusion criteria with reports of simultaneous Le Fort III and Le Fort I osteotomies. A total of 20 cases were present in the literature. No major complications were reported. We performed combined Le Fort III with Le Fort I osteotomies in a 25-year-old patient with Crouzon syndrome who had undergone a previous Le Fort III at the age of 4 years. The patient tolerated the procedure well, and postoperatively, her exophthalmos and class III malocclusion were corrected. CONCLUSIONS: Simultaneous Le Fort III and Le Fort I can correct differential upper and lower midface hypoplasia and is a well-tolerated procedure in the mature facial skeleton. This systematic review improves our understanding of the surgical technique and indications for a procedure that can correct complex midfacial deformities.

Le Fort II osteotomy.

BACKGROUND: In comparison with the abundant literature on Le Fort I and III osteotomies, there is scant information on the Le Fort II osteotomy. Our goal in this study was to define the indications and techniques of the elective Le Fort II osteotomy. We reviewed our 30-year experience, which is the longest series of patients treated with Le Fort II osteotomies at a single institution. METHODS: A review of the records of 13 consecutive patients at our institution with a diagnosis of Le Fort II was conducted. Data analyzed included demographic information, indications, techniques, complications, and combined procedures. On the basis of surgical outcomes, a treatment algorithm was created. RESULTS: Thirteen patients had a diagnosis of Le Fort II at our institution. Five were excluded because of incomplete records or Le Fort II fracture repair. Three patients were male, and 5 were female. Their average age was 23 years, and the average follow-up was 60 months. Indications included nasomaxillary deviation related to unilateral coronal synostosis, hemifacial microsomia, Romberg disease, and noncleft nasomaxillary hypoplasia. The average estimated blood loss was 950 mL. Complications were persistent nasal deviation, temporary nasal obstruction, and a hematoma. Complementary procedures included bilateral sagittal split osteotomies, bone grafts, Le Fort I osteotomy, and repositioning of the zygoma. CONCLUSIONS: On the basis of this review, we have elucidated the indications and less invasive techniques of the elective Le Fort II osteotomy. No major complications were observed. A management algorithm for performing the Le Fort II osteotomy is presented.

Orthognathic surgery for obstructive sleep apnea: applying the principles to new horizons in craniofacial surgery.

This article is dedicated to the senior author Dr. Henry K. Kawamoto, Jr, who pioneered the use of orthognathic surgery to treat severe obstructive sleep apnea in 1981. Since that time, his techniques for maxillomandibular advancement have been revised and expanded for improved surgical success. Obstructive sleep apnea is a growing public health concern because it can cause hypertension, cardiac arrhythmias, heart attack, stroke, and, in rare circumstances, sudden death if untreated. When less invasive options fail such as weight loss or dental devices for mandibular repositioning, maxillomandibular advancement is a valuable treatment option for severe obstructive sleep apnea.

Genetic and epigenetic influences of twins on the pathogenesis of craniosynostosis: a meta-analysis.

BACKGROUND: The pathoetiology of craniosynostosis is not well understood. It likely results from a combination of genetic and epigenetic phenomena, such as intrauterine constraint from multiple gestations. Information on craniosynostosis in twins is limited to case reports and series. The authors conducted a systematic review and meta-analysis of the literature to elucidate the genetic and nongenetic influences of twins on the pathogenesis of craniosynostosis. METHODS: PubMed and Ovid databases were reviewed for the key terms "craniosynostosis and twins." Data analyzed included demographical information, incidence rates, concordance, and phenotypic variability. Risk factors for craniosynostosis, concordance, and phenotypic variability were assessed by univariate and multivariate analyses. A case series was presented. RESULTS: Data were extracted from 34 journal articles, including the authors' five patients, and representing a total of 199 twins with craniosynostosis. Twinning was 2.62 times greater in patients with craniosynostosis (6.29 percent) compared with unaffected controls (2.4 percent; p < 0.0001). Boys were affected more than girls (65.30 versus 34.70 percent, respectively; p < 0.0001). Monozygotic concordance rates were greater than dizygotic (60.90 versus 5.30 percent, respectively; p < 0.0001) but were not 100 percent. Phenotypic variability was present in 62 percent of monozygotic twin sets (p < 0.05). CONCLUSIONS: Increased concordance rates among monozygotic compared with dizygotic twins confirm the genetic role of twins on craniosynostosis. Evidence to support the epigenetic influence of twinning on the pathogenesis of craniosynostosis includes the elevated incidence of twins among a craniosynostotic population compared with unaffected twins in the general population and male gender predominance, as well as monozygotic phenotypic variability and discordance. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, IV.

The Muenke syndrome mutation (FgfR3P244R) causes cranial base shortening associated with growth plate dysfunction and premature perichondrial ossification in murine basicranial synchondroses.

Muenke syndrome caused by the FGFR3(P250R) mutation is an autosomal dominant disorder mostly identified with coronal suture synostosis, but it also presents with other craniofacial phenotypes that include mild to moderate midface hypoplasia. The Muenke syndrome mutation is thought to dysregulate intramembranous ossification at the cranial suture without disturbing endochondral bone formation in the skull. We show in this study that knock-in mice harboring the mutation responsible for the Muenke syndrome (FgfR3(P244R)) display postnatal shortening of the cranial base along with synchondrosis growth plate dysfunction characterized by loss of resting, proliferating and hypertrophic chondrocyte zones and decreased Ihh expression. Furthermore, premature conversion of resting chondrocytes along the perichondrium into prehypertrophic chondrocytes leads to perichondrial bony bridge formation, effectively terminating the postnatal growth of the cranial base. Thus, we conclude that the Muenke syndrome mutation disturbs endochondral and perichondrial ossification in the cranial base, explaining the midface hypoplasia in patients.

Angiosarcoma of the breast.

BACKGROUND: Angiosarcoma of the breast is a rare entity. The objectives of this study were to evaluate prognostic factors and determine outcomes in a large contemporary series of patients. METHODS: Clinical and pathologic factors were analyzed in all patients with angiosarcoma of the breast treated between 1990 and 2003. Overall survival (OS) and disease-free survival (DFS) were estimated using the methods of Kaplan and Meier. Multivariate analyses were performed to evaluate prognostic factors. RESULTS: Fifty-five women with angiosarcoma of the breast were identified. The median age was 49.1 years, and the median follow-up time was 3.7 years. The median OS and DFS were 2.96 years and 2.26 years, respectively. For the 32 patients with primary angiosarcoma of the breast 5-year OS was 59%. Twenty-three (42%) patients had received prior radiation therapy for the treatment of breast cancer. These patients with radiation therapy-associated angiosarcoma were on average 30 years older and less likely to present with distant metastatic disease than patients presenting with radiation-naive angiosarcoma of the breast. Although radiation-naive patients appeared to have had better early DFS and OS, the Kaplan-Meier curves were not statistically different between patients with radiation therapy-associated disease and radiation therapy-naive patients. On multivariate analysis, tumor recurrence (P = 0.006) was the only significant adverse prognostic factor noted for OS. CONCLUSIONS: In this series of 55 patients with angiosarcoma of the breast, radiation therapy-naive angiosarcomas occurred in younger patients, but they behaved similarly to radiation therapy-associated angiosarcomas.