Gaby Doumit

Management of sagittal synostosis: a solution to equipoise.

Background In the craniofacial surgery literature, there is a wide disparity of opinions regarding the appropriate treatment of nonsyndromic sagittal synostosis. With the lack of level 1 evidence to support a particular regimen, our study aims to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. Methods An internet-based survey was sent to 102 craniofacial surgeons in 14 countries on 4 continents. Data were collected regarding the following parameters: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated sagittal synostosis with normative intracranial pressure values. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. Results After 2 mailings, the response rate was 58% (59/102). For 63% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management of sagittal craniosynostosis was most commonly performed at 6 months (35%) of age. Total cranial vault remodeling was the most commonly performed procedure (37%). Thirty-five percent of craniofacial surgeons chose an endoscopic surgical approach for patients presenting at younger than 4 months. Only 10% of craniofacial surgeons selected spring-assisted strip craniectomy. Seventy-one percent of polled surgeons performed computed tomographic scans of the skull in all cases, irrespective of presentation. Conclusion Our survey demonstrates that there exists a wide disparity of opinion regarding diagnosis and treatment of nonsyndromic sagittal synostosis. When current practice is compared to findings in the literature, significant discrepancies exist.

Opinion leaders and changes over time: a survey

BackgroundOpinion leaders represent one way to disseminate new knowledge and influence the practice behaviors of physicians. This study explored the stability of opinion leaders over time, whether opinion leaders were polymorphic (i.e., influencing multiple practice areas) or monomorphic (i.e., influencing one practice area), and reach of opinion leaders in their local network.MethodsWe surveyed surgeons and pathologists in Ontario to identify opinion leaders for colorectal cancer in 2003 and 2005 and to identify opinion leaders for breast cancer in 2005. We explored whether opinion leaders for colorectal cancer identified in 2003 were re-identified in 2005. We examined whether opinion leaders were considered polymorphic (nominated in 2005 as opinion leaders for both colorectal and breast cancer) or monomorphic (nominated in 2005 for only one condition). Social-network mapping was used to identify the number of local colleagues identifying opinion leaders.ResultsResponse rates for surgeons were 41% (2003) and 40% (2005); response rates for pathologists were 42% (2003) and 37% (2005). Four (25%) of the surgical opinion leaders identified in 2003 for colorectal cancer were re-identified in 2005. No pathology opinion leaders for colorectal cancer were identified in both 2003 and 2005. Only 29% of surgical opinion leaders and 17% of pathology opinion leaders identified in the 2005 survey were considered influential for both colorectal cancer and breast cancer. Social-network mapping revealed that only a limited number of general surgeons (12%) or pathologists (7%) were connected to the social networks of identified opinion leaders.ConclusionsOpinion leaders identified in this study were not stable over a two-year time period and generally appear to be monomorphic, with clearly demarcated areas of expertise and limited spheres of influence. These findings may limit the practicability of routinely using opinion leaders to influence practice.

Classification and Management of Metopic Craniosynostosis.

Background: In the craniofacial surgery literature, there is a wide disparity of opinions regarding the management of nonsyndromic metopic synostosis. With the lack of level I evidence to support a particular regimen, we aimed to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. Methods: A survey was sent to 102 craniofacial surgeons. The survey featured 2 parts: clinical scenarios and questions regarding the following: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated metopic synostosis. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. Results: The total response rate was 72% (73/102) for the clinical scenarios and 63% (64/102) for the complete survey. There was a large discrepancy when classifying and managing mild metopic synostosis, with between 16% and 35% of surgeons electing to operate on a mild case. All surgeons agreed to operate on moderate and severe cases. For 95% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management was most commonly performed at 6 months (29%) of age. Open frontal orbital advancement was the most commonly performed procedure in mild (27%), moderate (77%), and severe (89%) cases. Endoscopic approaches were more likely to be used in milder cases by 19% of surgeons. Conclusion: Our survey demonstrates that there is a wide disparity of opinion among craniofacial surgeons regarding the diagnosis and management of mild nonsyndromic metopic synostosis. Level of Evidence: Diagnostic, level 5.

Pediatric vascularized composite allotransplantation.

BackgroundVascularized composite allotransplantation (VCA) has experienced a growing acceptance, which has led to a debate centered on extending the indications of the procedure to include pediatric patients. The aim of this article was to discuss such indications based on the evidence in pediatric solid organ transplantation, reconstructive surgery in children, and VCA in adult patients. MethodsPapers published on the outcomes of pediatric solid organ transplantation, growth after replantation of extremities, vascularized autologous tissue transfer, craniofacial surgery, orthognathic procedures, facial fractures, and outcomes after repair of peripheral nerves in children were reviewed. ResultsAlthough the outcomes of solid organ transplantation in children have improved, the transplanted organs continue to have a limited lifespan. Long-term immunosuppressive therapy exposes the patients to an increased lifetime risk of infections, diabetes, hypertension, dyslipidemia, cardiovascular disease, and malignancy. Growth impairment and learning disabilities are other relevant drawbacks, which affect the pediatric recipients. Nonadherence to medication is a common cause of graft dysfunction and loss among the adolescent transplant recipients. Rejection episodes, hospitalizations, and medication adverse effects contribute negatively to the quality of life of the patients. Although normal growth after limb transplantation could be expected, pediatric facial transplant recipients may present with arrest of growth of transplanted midfacial skeleton. ConclusionsConsidering the non–life-threatening nature of the conditions that lead to eligibility for VCA, it is suggested that it is premature to extend the indications of VCA to include pediatric patients under the currently available immunosuppressive protocols.

Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome

Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance.

Squamosal suture craniosynostosis in Muenke syndrome.

AbstractMuenke syndrome caused by point mutation (C749G) in the FGFR3 gene affects 1 in 30,000 newborns and accounts for 25% to 30% of genetic causes of craniosynostosis. Anomalies in patients with Muenke syndrome include craniosynostosis, hypertelorism, sensorineural hearing loss, and developmental delay, among others. Most craniosynostoses in patients with Muenke syndrome involve bicoronal suture fusion. This article reports, for the first time, the existence of squamosal craniosynostosis in patients with Muenke syndrome.

Microsurgical Anatomy of the Terminal Hypoglossal Nerve Relevant for Neurostimulation in Obstructive Sleep Apnea.

Neurostimulation of the hypoglossal nerve has shown promising results in the treatment of obstructive sleep apnea. This anatomic study describes the detailed topography of the hypoglossal nerves motor points as a premise for super‐selective neurostimulation in order to optimize results and minimize the risk of complications related to main nerve trunk manipulation.

Opinion leaders and evidence-based medicine in craniofacial surgery.

BackgroundIn health care, it is widely known that evidence-based medicine (EBM) has a significant impact on clinical practice, and opinion leaders can enhance the clinician’s application of EBM in various disciplines. In this article, we examine the existence and impact of opinion leaders in craniofacial surgery as well as barriers to evidence-based practice. MethodsWe compiled the answers of an Internet questionnaire, which was sent to 102 craniofacial surgeons. ResultsOur results demonstrate that opinion leaders most definitely can be identified in craniofacial surgery. They are tightly connected to their field’s social network and promote EBM. In this survey, 44% of craniofacial surgeons reported that their greatest obstacle to clinical decision making in the management of nonsyndromic synostosis was lack of surgical consensus. In addition, craniofacial surgeons stated that EBM and opinion leaders are the most influential factors that caused them to change their management of craniosynostosis. ConclusionsWe expect that the use of opinion leaders can further enhance the uptake of EBM in craniofacial surgery.

Fat embolism after liposuction in Klippel-Trenaunay syndrome.

AbstractFat embolism syndrome (FES) is a rare but potentially fatal postoperative complication from liposuction. We present the case of a 24-year-old woman with Klippel-Trenaunay syndrome who developed FES as a complication of lower extremity liposuction. There may be an increased risk of FES in patients with vascular malformations undergoing liposuction.

Establishing the Feasibility of Face Transplantation in Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA; formerly Wegeners granulomatosis) is a rare vasculitis that commonly starts in the craniofacial region. We report a case that was masked by prior facial trauma and associated with pyoderma gangrenosum (PG). Disease progression and aggressive debridements led to severe facial tissue loss. The decision to perform a face transplant was controversial because of the risk of disease relapse on the facial allograft. We reviewed renal transplant outcomes in GPA for possible relevance. A PubMed search retrieved 29 studies. Patient and graft survival, relapse, morbidity, mortality, rejection and immunosuppression were assessed. Ten‐year patient survival and graft survival were 84.4% and 72.6%, respectively. GPA relapse occurred in 31.5%, and upper airway/ocular relapse occurred in 17.8% (resolved in 76.9%). Mortality was 12.3%. Acute and chronic rejection rates were 14.9% and 6.8%, respectively. Traditional posttransplant immunosuppression was effective. Our review suggests that GPA renal transplant outcomes are comparable to general renal transplant cohorts. Furthermore, transplanted GPA patients exhibit lower disease relapse secondary to lifelong immunosuppression. This supported our decision to perform a face transplant in this patient, which has been successful up to the present time (1‐year posttransplantation). Untreated GPA and PG are potential causes of worse surgical outcomes in the craniofacial region.