Gregory E. Lakin

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

AbstractThis 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.

Le Fort II osteotomy.

BackgroundIn 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. MethodsA 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. ResultsThirteen 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. ConclusionsOn 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.

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 ± 1.41 for support groups, 2.5 ± 1.63 for summer camps, 2.92 ± 1.66 for parties, 3.16 ± 1.65 for social media, and 3.95 ± 1.60 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.

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.

Commentary on "Nonsyndromic craniosynostosis: the Rady Children's Hospital approach".

The objective of craniosynostosis repair is to prevent potential brain injury due to increased intracranial pressure and to improve the cosmetic appearance of the skull by establishing normal calvarial shape and contour. In most centers around the world, these goals are achieved with the traditional open techniques described by Tessier and his students. Some surgeons, however, are able to achieve excellent results via minimally invasive approaches that use endoscopic imaging to facilitate surgical dissection and obviate the need for widely open exposure. Dr. Cohen, originally trained in open techniques by Dr. Kawamoto, here discusses the history, diagnosis, and current treatment options for nonsyndromic craniosynostosis. He specifically compares his experience with open techniques for sagittal, metopic, and coronal craniosynostosis repair to his success with endoscopic approaches to the same clinical problems. Dr. Cohen should be commended on his achievements. He has presented long-term outcomes data on patients treated for nonsyndromic craniosynostosis using both open and endoscopic modalities. With a significant clinical experience in both methods, Dr. Cohen and his colleagues have a unique perspective on the ‘‘ideal’’ patient for either type of procedure. They have demonstrated that in a select population of younger patients with less severe deformities, the endoscopic technique is a safe and effective procedure, reducing blood loss, transfusion requirement, operative time, postoperative recovery, and length of hospital stay. In their hands, the technique yields primarily good to excellent aesthetic results compared to standard open approaches. These results are consistent with other centers that have published their experience with endoscopic craniosynostosis repair. It is important to note, however, that the endoscopic approach to craniosynostosis repair is not ideal for every patient. Because of his extensive experience with both open and endoscopic techniques, Dr. Cohen has been able to establish screening parameters to identify patients who are best suited to the latter. The endoscopic approach is recommended to treat mild to moderate presentation of nonsyndromic craniosynostosis in patients younger than 4 months of age, while the calvarial bones are malleable and thin, and susceptible to the molding forces of a rapidly growing brain and a conforming orthotic helmet. For more severe deformities or for patients who present later in the first year of life, Dr. Cohen recommends a traditional open approach. It would be of interest to compare Dr. Cohen’s results to published reports on the role of spring-assisted cranioplasty in the treatment of nonsyndromic craniosynostosis. Despite requiring a second procedure to remove the springs once the desired cranial morphology is obtained, this technique offers similar advantages to endoscopic approaches in terms of decreased blood loss, hospital stay, and operative time. It is also worth mentioning that the learning curve associated with the endoscopic technique can be an issue in determining successful outcomes: the technical challenges inherent to a limited exposure when engaging in intracranial dissection, particularly around the sagittal sinus, require a high level of surgical expertise. What Dr. Cohen’s experience underscores is the need to focus on the fundamental principles associated with craniosynostosis repair, regardless of technique: 1. Timing of surgery: Early repair is required for endoscopic techniques that capitalize on thin calvarial bones for execution and a subsequent period of rapid brain growth to obtain the desired final result. While the preferred age for the open technique remains a matter of some debate, it has been shown that earlier procedures are associated with a higher risk of relapse of the original deformity. The risk of persistent full thickness defects also increases when surgery is delayed beyond 12 months of age, as the osteogenic potential of the dura decreases along with the rate of brain growth. Of course, early surgery may be dictated by the severity of the presentation, with evidence of exorbitism and corneal exposure or elevated intracranial pressure mandating earlier intervention. 2. Blood loss: Minimally invasive techniques limit the amount of intraoperative hemorrhage and the need for transfusion. However, numerous strategies can help decrease a patient’s risk of exposure to transfusion of banked blood products, even during open procedures. These include preoperative erythropoietin injection, donor directed units collected from family members, intraoperative use of cell saver, controlled hypotension, and of course meticulous surgical technique. Communication between the surgical and anesthesia teams must of course be seamless and ongoing throughout the case. 3. Fixation: In order to ensure a predictable and stable result, rigid fixation is a central component of the repair. It has greater importance in the open procedures, where postoperative helmet therapy is not anticipated. Currently, resorbable plates and screws are the preferred method of achieving fixation. 4. Efficiency: Regardless of the technique used, craniosynostosis repair poses a tremendous physiologic burden on pediatric patients. Success in the operating room is dependent upon thorough presurgical planning, working with an experienced surgical team, immediate availability of all necessary instruments, and elimination of extraneous movements during surgery. DISCUSSION

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.

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.