Introduction: Mandibular distraction is recognized as a treatment of respiratory distress in neonates with microretrognathia as seen in the Pierre Robin syndrome. However, mandibular distraction is a complex and lengthy treatment involving 2 to 4 weeks of distraction and another 4 to 12 weeks for bone consolidation. This study was performed to establish the safety and effectiveness of rapid protocol distraction osteogenesis with recombinant human bone morphogenetic protein 2 (rhBMP-2) in neonates with the Pierre Robin syndrome.
Methods: A retrospective review of all patients treated in our department between February 2003 and February 2008 was performed. Three patients with the Pierre Robin syndrome who underwent distraction osteogenesis with rhBMP-2 were identified. Inpatient and outpatient charts were reviewed for time to completion of distraction, age at distraction, need for tracheostomy, and complications of the mandibular distraction.
Results: Three patients (6 hemimandibles) with Pierre Robin syndrome underwent rapid protocol distraction with rhBMP-2. Mean age at initial distraction was 17.3 days. Mean time from device placement to removal was 89.3 days. The complication rate was 16%, with 1 case of nonunion that required subsequent operative intervention. No patient required tracheostomy.
Conclusions: Rapid protocol distraction with rhBMP-2 allows distraction of the hypoplastic mandible to class III occlusion during the initial operation and avoids the latency and distraction phases of standard mandibular distraction. This case series demonstrates the safety and effectiveness of rapid distraction in neonates with Pierre Robin syndrome. Larger studies and long-term follow-up are necessary; however, this study suggests that rapid protocol distraction with rhBMP-2 is effective in neonates with Pierre Robin syndrome.
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http://dx.doi.org/10.1097/SCS.0b013e3181e47d58 | DOI Listing |
Int J Pediatr Otorhinolaryngol
January 2025
Dr. Elie E. Rebeiz Department of Otolaryngology - Head and Neck Surgery, Tufts Medical Center, Boston, MA, USA. Electronic address:
Background: Internal and external devices may be utilized in mandibular distraction osteogenesis (MDO) for the correction of symptomatic micrognathia in infants and children.
Purpose: To compare the rate and severity of infection between internal and external MDO devices.
Study Design, Setting, Sample: Retrospective cohort study utilizing an institutional database of patients who underwent MDO.
HGG Adv
January 2025
Department of Surgery, Division of Orthopaedics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. Electronic address:
SOX9 encodes an SRY-related transcription factor critical for chondrogenesis and sex determination among other processes. Loss-of-function variants cause campomelic dysplasia and Pierre Robin Sequence, while both gain- and loss-of-function variants cause disorders of sex development. SOX9 has also been linked to scoliosis and cancers, but variants are undetermined.
View Article and Find Full Text PDFJ Craniofac Surg
January 2025
Division of Plastic and Reconstructive Surgery, Children's National Hospital.
Facial nerve dysfunction (FND) is a well-recognized but poorly documented complication of mandibular distraction osteogenesis (MDO) for Robin sequence (RS). This study aims to document the authors' experiences with FND and identify risk factors associated with this adverse event. A retrospective review of a prospectively gathered database was performed to identify patients with RS who underwent MDO at the authors' institution from March 2016 to June 2023.
View Article and Find Full Text PDFNat Commun
January 2025
Center for Integrative Genomics, University of Lausanne, Faculty of Biology and Medicine, Lausanne, Switzerland.
The energetic demands of proliferating cells during tumorigenesis require close coordination between the cell cycle and metabolism. While CDK4 is known for its role in cell proliferation, its metabolic function in cancer, particularly in triple-negative breast cancer (TNBC), remains unclear. Our study, using genetic and pharmacological approaches, reveals that CDK4 inactivation only modestly impacts TNBC cell proliferation and tumor formation.
View Article and Find Full Text PDFCleft Palate Craniofac J
January 2025
Department of Physiology and Medical Biochemistry, Faculty of Medicine, Airlangga University, Surabaya, Indonesia.
Objective: This study compares mandibular distraction osteogenesis (MDO) and tracheostomy in managing severe airway obstruction in patients with the Pierre Robin sequence (PRS).
Design: A systematic review and meta-analysis following PRISMA guidelines was performed. Literature searches were conducted across PubMed, ScienceDirect, Cochrane Library, Scopus, E.
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