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Bone Aug 2023Osteopetrosis (OPT) denotes the consequences from failure of osteoclasts to resorb bone and chondroclasts to remove calcified physeal cartilage throughout growth....
Osteopetrosis (OPT) denotes the consequences from failure of osteoclasts to resorb bone and chondroclasts to remove calcified physeal cartilage throughout growth. Resulting impairment of skeletal modeling, remodeling, and growth compromises widening of medullary spaces, formation of the skull, and expansion of cranial foramina. Thus, myelophthisic anemia, raised intracranial pressure, and cranial nerve palsies complicate OPT when severe. Osteopetrotic bones fracture due to misshaping, failure of remodeling to weave the collagenous matrix of cortical osteons and trabeculae, persistence of mineralized growth plate cartilage, "hardening" of hydroxyapatite crystals, and delayed healing of skeletal microcracks. Teeth may fail to erupt. Now it is widely appreciated that OPT is caused by germline loss-of-function mutation(s) usually of genes involved in osteoclast function, but especially rarely of genes necessary for osteoclast formation. Additionally, however, in 2003 we published a case report demonstrating that prolonged excessive dosing during childhood of the antiresorptive aminobisphosphonate pamidronate can sufficiently block osteoclast and chondroclast activity to recapitulate the skeletal features of OPT. Herein, we include further evidence of drug-induced OPT by illustrating osteopetrotic skeletal changes from repeated administration of high doses of the aminobisphosphonate zoledronic acid (zoledronate) given to children with osteogenesis imperfecta.
Topics: Child; Humans; Osteopetrosis; Osteoclasts; Zoledronic Acid; Fractures, Bone; Skull
PubMed: 37172883
DOI: 10.1016/j.bone.2023.116788 -
Oral and Maxillofacial Surgery Clinics... Nov 2023Pediatric panfacial trauma is a rare occurrence with poorly understood implications for the growing child. Treatment algorithms largely mirror adult panfacial protocols... (Review)
Review
Pediatric panfacial trauma is a rare occurrence with poorly understood implications for the growing child. Treatment algorithms largely mirror adult panfacial protocols with notable exceptions including augmented healing and remodeling capacities that favor nonoperative management, limited exposure to avoid disruption of osseous suture and synchondroses growth centers, and creative fracture fixation techniques in the setting of an immature craniomaxillofacial skeleton. The following article provides a review of our institutional philosophy in the management of these challenges injuries with important anatomic, epidemiologic, examination, sequencing, and postoperative considerations.
Topics: Adult; Child; Humans; Facial Bones; Fracture Fixation; Skull Fractures
PubMed: 37280142
DOI: 10.1016/j.coms.2023.04.006 -
Oral and Maxillofacial Surgery Clinics... Nov 2023The unique anatomy and physiology of the growing craniofacial skeleton predispose children to different fracture patterns as compared to adults. Diagnosis and treatment... (Review)
Review
The unique anatomy and physiology of the growing craniofacial skeleton predispose children to different fracture patterns as compared to adults. Diagnosis and treatment of pediatric orbital fractures can be challenging. A thorough history and physical examination are essential for the diagnosis of pediatric orbital fractures. Physicians should be aware of symptoms and signs suggestive of trapdoor fractures with soft tissue entrapment including symptomatic diplopia with positive forced ductions, restricted ocular motility (regardless of conjunctival abnormalities), nausea/vomiting, bradycardia, vertical orbital dystopia, enophthalmos, and hypoglobus. Equivocal radiologic evidence of soft tissue entrapment should not withhold surgery. A multidisciplinary approach is recommended for the accurate diagnosis and proper management of pediatric orbital fractures.
Topics: Adult; Child; Humans; Orbital Fractures; Tomography, X-Ray Computed; Nausea; Vomiting; Physical Examination; Enophthalmos
PubMed: 37302946
DOI: 10.1016/j.coms.2023.05.002