Frequency and Management of Craniofacial Syndromes

Abstract

Background: Craniofacial syndromes occur in approximately 1 in 5600 to 100,000 infants, often resulting in significant morbidity. Due to the heterogeneity of this patient population, no clear consensus consists on optimal treatment modalities and timing. The aim of this study was to analyze the craniofacial syndrome population that were treated at the University Hospital Leuven. Methods: A retrospective analysis of patients with a clinical diagnosis of a craniofacial syndrome was performed. Inclusion criteria were patients with a clinical diagnosis of a craniofacial syndrome and that received treatment between ''2000-2005'' and ''2010-2015.'' Patients with nonsyndromic conditions were excluded. Data regarding patient characteristics, treatment modalities, and treatment outcomes were analyzed. Results: After matching the inclusion criteria, 98 eligible patients, affected by 40 different syndromes were included. In the period of ''2000 to 2005,'' 48 patients were treated, as compared to 50 patients in the period of ''2010 to 2015.'' A statistically significant decrease over time is seen for cleft surgery and orthodontic treatment (P ¼ 0.0017 and P ¼ 0.0015, respectively). No statistically significant differences were found concerning the age at which treatment was received (P ¼ 0.42). Significant associations between treatment modalities were found for orthognathic surgery and distraction osteogenesis (P < 0.0001), orthognathic surgery and orthodontic treatment (P < 0.0001), and between orthodontic treatment and distraction osteogenesis (P ¼ 0.03311). Conclusion:A decline in cleft reconstruction surgery and orthodontic treatment for patients with craniofacial syndromes was seen over time. A significant association was found between distraction osteogenesis and orthognathic surgery, possibly due to higher reintervention rates for patients treated at a young age.

Temporomandibular Joint Dysplasia in Cranio-Maxillofacial Dysplasia: A Retrospective Study. Guideline Treatment Proposal

“…However, the reliability in terms of occlusion is poor and very few cases can be completely treated by this technique alone. 27,28 One of the limitations of the technique is the lack of bone in cases of major defects (classes IIB and III in Pruzansky/Kaban classification). In these cases the bone deficit makes the distraction difficult and sometimes impossible.…”

“…Two surgical procedures are required at each distraction phase, the first one to insert the device, and the second one to remove it. However, the reliability in terms of occlusion is poor and very few cases can be completely treated by this technique alone 27,28 …”

Otolaryngologists and Reproductive Rights

“…Comprehensive counseling strategies for airway anomalies include reassurance regarding parental sense of blame regarding etiology, and anticipatory guidance with discussion of all treatment options, from elective termination to in utero intervention to attended delivery with EXIT procedures, to urgent tracheostomy after an unplanned delivery. In addition to participating in prenatal counseling, we must also be prepared to participate or establish highly skilled, high‐performance teams of obstetricians, anesthesiologists, neonatologists, and surgeons to address the possible increasing number of neonatal airway concerns at the time of delivery and pediatric craniofacial and head and neck pathology 16‐18 . Concerns with regard to legal restrictions on medically appropriate care for pregnant patients, their fetuses and neonates will make this even more complicated.…”

“…In addition to participating in prenatal counseling, we must also be prepared to participate or establish highly skilled, high-performance teams of obstetricians, anesthesiologists, neonatologists, and surgeons to address the possible increasing number of neonatal airway concerns at the time of delivery and pediatric craniofacial and head and neck pathology. [16][17][18] Concerns with regard to legal restrictions on medically appropriate care for pregnant patients, their fetuses and neonates will make this even more complicated.…”