Background: Le Fort III distraction for syndromic craniosynostosis is performed using internal or external devices. The authors compared the results of both devices.
Methods: The authors retrospectively evaluated 60 patients with syndromic craniosynostosis treated with Le Fort III distraction (internal or external device) between 2001 and 2021. The authors verified demographic data, surgery-related data, and complications using medical records. For each of the 2 devices, they compared the various factors associated with the device to each other.
Results: For the external device, 32 patients with syndromic craniosynostosis were included. The mean age at surgery was 11.7 years, and the mean elongation length was 20.0 mm. Class III occlusion reoccurred in 11 patients and was significantly associated with age at surgery. Seven complications (device problems and others) were noted. Cranial pin slippage was significantly related to the elongation length. For the internal device, 28 patients with syndromic craniosynostosis were included. The mean age at surgery was 10.4 years, and the mean elongation length was 18.7 mm. There were 15 complications, including device problems, zygomaticomaxillary fractures, and infections. Elongation length was significantly related to these complications. Class III occlusion reoccurred in 9 patients and was significantly related to age at surgery.
Conclusions: This study found that complications are significantly more likely to occur in internal devices than in external devices, especially device infection. The authors' findings identified several factors that may assist surgeons in selecting between external and internal devices. The relationship between the amount of extension and device-related problems found in this study will be beneficial for solving these problems.
Clinical Question/level Of Evidence: Therapeutic, III.
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http://dx.doi.org/10.1097/PRS.0000000000010954 | DOI Listing |
Taiwan J Obstet Gynecol
January 2025
Gynecology and Obstetrics Clinic "Narodni front", Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
Objective: Prenatal detection of complex chromosomal rearrangements (CCR) is extremely rare, but is of great clinical importance, since CCR can be causative of different congenital disorders. We present an exceptionally rare case of prenatally diagnosed Saethre-Chotzen syndrome (SCS) rising as a consequence of chromothripsis involving chromosomes 5, 7 and 11 and deletion of TWIST1 gene.
Case Report: Brachycephaly, hypertelorism, flat face, micrognathia, relative macroglossia and small posterior fossa were noted on ultrasound examination at 28th gestational week.
Cleft Palate Craniofac J
January 2025
Division of Plastic and Reconstructive Surgery, Oregon Health & Science University, Portland, OR, USA.
Craniosynostosis is rarely diagnosed in utero. Prenatal diagnosis has the potential to improve patient outcomes and streamline care, however, and is becoming more feasible as technology improves. The objective of this study is to examine existing literature on prenatal diagnosis of nonsyndromic craniosynostosis.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Basis Dis
January 2025
Shanghai Clinical Research Center of Bone Disease, Department of Osteoporosis and Bone Diseases, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China. Electronic address:
Cole-Carpenter syndrome (CCS) is a rare autosomal-dominant genetic disease characterized by craniosynostosis, ocular proptosis, hydrocephalus, distinctive facial features, and bone fragility. Previous cases of CCS are associated with genetic variations in P4HB, which encodes the protein disulfide isomerase (PDI), a key enzyme in protein folding. Patients with CCS caused by P4HB mutations often present with short stature, limb deformities, and abnormal epiphyseal plates.
View Article and Find Full Text PDFSurg Neurol Int
December 2024
Department of Ophthalmology, Dr. Soetomo General and Academic Hospital, Surabaya, East Java, Indonesia.
Background: Craniosynostosis may result in malformations of the orbit, which can be observed in clinical presentations. Craniosynostosis impairs the normal growth of the skull, which typically occurs perpendicular to the fused suture. Craniosynostosis is classified into non-syndromic and syndromic, with an incidence of 1: 2000-2500 live births.
View Article and Find Full Text PDFJ Craniofac Surg
January 2025
Division of Pediatric Craniofacial Surgery, Nemours Children's Health, Jacksonville, FL.
External rigid distraction is an established method for achieving subcranial Le Fort III advancement in severe syndromic craniosynostosis. Craniofacial surgeons commonly use halo-type devices for these corrections, as they allow for multiple vectors of pull and facilitate larger midfacial advancements. Although most complications related to their use involve pin displacement or infection, rare complications such as skull fractures have been reported.
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