Search

Posterior cranial vault distraction osteogenesis is a powerful, reliable, low-morbidity method to achieve intracranial expansion. It is particularly useful in treating turribrachycephaly seen in syndromic craniosynostosis, allowing for gradual expansion of the bone while stretching the soft tissues over several weeks allowing greater volumetric expansion than conventional techniques. Posterior cranial vault distraction osteogenesis constitutes a more gradual remodeling modality, with infrequent complications. As a first step in intracranial expansion, it preserves the frontal cranium for future frontofacial procedures. A drawback is the need for a second surgery to remove the device, and this must be taken into account during counseling.

Patients with complex, multisutural, and syndromic craniosynostosis (CSO) frequently exhibit intracranial hypertension. The intracranial hypertension cannot be entirely attributed to the craniocephalic disproportion with calvarial restriction because cranial vault expansion has not consistently alleviated elevated intracranial pressure. Evidence has most strongly supported a multifactorial interaction, including venous hypertension along with other pathogenic processes. Patients with CSO exhibit marked venous anomalies, including stenosis of the jugular-sigmoid complex, transverse sinuses, and extensive transosseous venous collaterals. These abnormal intracranial-extracranial occipital venous collaterals might represent anomalous development, with persistence and subsequent enlargement of channels normally present in the fetus, either as a primary defect or as nonregression in response to failure of the development of the jugular-sigmoid complexes. It has been suggested by some investigators that venous hypertension in patients with CSO could be treated directly via jugular foraminoplasty, venous stenting, or jugular venous bypass, although these options are not in common clinical practice. Obstructive sleep apnea, occurring as a consequence of midface hypoplasia, can also contribute to intracranial hypertension in patients with syndromic CSO. Thus, correction of facial deformities, as well as posterior fossa decompression, could also play important roles in the treatment of intracranial hypertension. Determining the precise mechanistic underpinnings underlying intracranial hypertension in any given patient with CSO requires individualized evaluation and management.

Orbital hypertelorism represents lateralization of the orbits, meaning increased interorbital and outer orbital distances. Interorbital hypertelorism represents a failure of medial orbital wall medialization in the setting of normally positioned lateral orbital walls. The etiology and type of hypertelorism influence selection of an operative procedure, whereas the severity of deformity dictates surgical need. Choice of surgical procedure is dictated by anatomic considerations, such as degree of orbital hypertelorism, midfacial proportions, and occlusal status.

In North America, the number of craniofacial surgery fellowship graduates is increasing, yet an analysis of practice settings upon graduation is lacking. We characterize the practice types of recent graduates of craniofacial fellowship programs in the United States and Canada.A 6-year cohort of craniofacial fellows in the United States and Canada (2010-2016) were obtained from craniofacial programs recognized by the American Society of Craniofacial Surgery. Practice setting was determined at 1 and 3 years of postgraduation, and predictors of practice setting were determined.A total of 175 craniofacial surgeons were trained at 35 fellowship programs. At 1 year of postgraduation, 33.6% had an academic craniofacial position and 27.1% were in private practice (p = 0.361). A minority of graduates pursued additional fellowships (16.4%), nonacademic craniofacial positions (10.0%), academic noncraniofacial positions (5.7%), and international practices (7.1%). At 3 years of postgraduation, the percentage of graduates in academic craniofacial positions was unchanged (34.5% vs 33.6%, p = 0.790). The strongest predictors of future academic craniofacial practice were completing plastic surgery residency at a program with a craniofacial fellowship program (odds ratio = 6.78, p0.001) and completing an academic craniofacial fellowship program (odds ratio = 4.48, p = 0.020).A minority of craniofacial fellowship graduates practice academic craniofacial surgery. A strong academic craniofacial surgery background during residency and fellowship is associated with a future career in academic craniofacial surgery. These data may assist trainees choose training programs that align with career goals and educators select future academic surgeons.

In infants with craniofacial disorders, upper airway obstruction is one of the primary causes for morbidity and mortality in the neonatal period. Infants with craniofacial disorders, including Pierre Robin sequence, are at high risk for obstructive sleep apnea syndrome. Because of the complexity of their care, these neonates are usually followed by a multidisciplinary team to ensure timely evaluation and optimal treatment. In addition to history and physical examination, clinical evaluation may include genetic testing, imaging, endoscopy, and polysomnography. There are various treatment options, both surgical and non-surgical, that may be used depending on clinical assessment, underlying condition, and severity of disease. Recent advances have led to better assessment and treatment of these patients, but many questions remain. This review outlines the available literature pertaining to the evaluation and management of upper airway obstruction in the neonate with craniofacial conditions, with a particular focus on Pierre Robin sequence.

Summary Background Craniofacial malformations including cleft lip and/or palate (CL/P) increase risk for obstructive sleep apnea (OSA). While 30% of CL/P occurs in the context of underlying genetic syndromes, few studies have investigated the prevalence of OSA in this high-risk group. This study aims to determine the incidence and risk factors of positive screening for OSA in this complex patient population. Methods The Pediatric Sleep Questionnaire (PSQ) was prospectively administered to all patients cared for by the cleft lip and palate clinic at the Children's Hospital of Philadelphia between January 2011 and August 2013. The PSQ is a 22-item, validated screening tool for OSA with a sensitivity and specificity of 0.83 and 0.87 in detecting an apnea-hypopnea index (AHI) >5/hour in healthy children. The Fisher exact and Chi-square tests were used for purposes of comparison. Results 178 patients with syndromic CL/P completed the PSQ. Mean cohort age was 8.1 ± 4.4 years. Patients were predominately female (53.9%), Caucasian (78.1%), and had Veau Class II cleft (50.6%). Craniofacial syndromes included isolated Pierre Robin Sequence (PRS) (29.8%), 22q11.2 deletion syndrome (14.6%), Van der Woude syndrome (6.7%), and other rare genetic abnormalities (28.8%). The overall incidence of positive OSA screening was 32.0%. Males were at increased risk for positive OSA screening ( P = 0.030), as were non-Caucasians ( P = 0.044). Symptoms with the highest positive predictive value for OSA were "others comment on child appearing sleepy" (76.2%) and "stops breathing during the night" (75.0%). Notably, patients with 22q11.2 deletion syndrome were at highest risk for positive screens (50.0%, P = 0.042). Conclusions Nearly a third of our patients with syndromic CL/P screened positively for OSA (32.0%), highlighting the importance of screening in this at-risk population. Future work will correlate screening results with polysomnograms to help validate these findings. Clinical question/level of evidence Diagnostic, III.

Two new 19-vertex metallaboranes, [B18H20Ni(η5-C5H5)][N(CH3)4] and [B18H19(2-THF)Ni(η5-C5H5)], were isolated from the reaction of anti-B18H22 with nickelocene, [Ni(η5-C5H5)2], in high yields. The complexes were characterized by multinuclear NMR (1H, 13C, 11B), IR and UV–vis spectroscopy. Single-crystal X-ray diffraction analyses of these new complexes confirmed their structures as 19-vertex conjuncto-metallaborane clusters.

Many types of scaffolds have been used in bone tissue engineering, with none emerging as favorites. We propose the use of acellular periosteum as a biologic scaffold to allow for progenitor cell adherence, migration, and proliferation in vitro and to test the construct in vivo in a rat calvarial defect model.Bovine periosteum was processed to remove all antigenic material (RTI Biologics), and its cambial layer was then seeded with adipose-derived stromal cells (ASCs) or periosteal-derived stromal cells (PSCs) and incubated for 14 days. Adherence required a fibronectin coat and was verified for both cell types via scanning electron microscopy and histology. Two 5-mm diameter calvarial defects were created in each of 19 rats. These were filled with xenograft bone chips and covered with acellular periosteum in combination with cells (ASC or PSC), growth factors (vascular endothelial growth factor, bone morphogenetic protein-2, or both), or alone (controls). Rats were killed 56 days postoperatively. Bone deposition was quantified by microcomputed tomography, and viability was determined histologically. Significance was determined through analysis of variance.Acellular allo-periosteum with a fibronectin coat permitted ASC and PSC adherence, migration, and proliferation in vitro. In the rat calvarial defects, the addition of stem cells (P.001) and growth factors (P.001) to the acellular periosteum increased de novo bone growth relative to controls. Although the stem cell source did not influence revitalization (P = .242), the combination of growth factors was more effective (P.001) than either growth factor alone. The interaction indicated that the 2 cell types did not respond equally to growth factors (P = .039).Acellular allo-periosteum is a biomimetic scaffold that permits pleuripotent cell adherence, migration, and proliferation in vitro. The combination of acellular periosteum, xenograft bone, stem cells, and growth factors may prove a viable combination for cranial bone tissue engineering.