phenylephrine-hydrochloride and Osteogenesis-Imperfecta

The endoscopic transnasal approach to the rostral pediatric spine and craniovertebral junction is a relatively new technique that provides an alternative to the traditional transoral approach to the anterior pediatric spine. In this case series, the authors provide 2 additional examples of patients undergoing endoscopic transnasal odontoidectomies for ventral decompression of the spinal cord. Both patients would have required transection of the palate to undergo an effective transoral operation, which can be a cause of significant morbidity. In one case, transnasal decompression was initially incomplete, and decompression was successfully achieved via a second endoscopic transnasal operation. Both cases resulted in significant neurological recovery and stable long-term spinal alignment. The transnasal approach benefits from entering into the posterior pharynx at an angle that often reduces the length of postoperative intubation and may speed a patient's return to oral intake. Higher reoperation rates are a concern for many endoscopic approaches, but there are insufficient data to conclude if this is the case for this procedure. Further experience with this technique will provide a better understanding of the indications for which it is most effective. Transcervical and transoral endoscopic approaches have also been reported and provide additional options for pediatric anterior cervical spine surgery.

Topics: Arnold-Chiari Malformation; Autistic Disorder; Cervical Vertebrae; Child; Down Syndrome; Endoscopy; Humans; Magnetic Resonance Imaging; Male; Nose; Osteogenesis Imperfecta; Tomography Scanners, X-Ray Computed

Osteogenesis imperfecta (OI) is a rare hereditary disorder of collagen synthesis that results in weak bones that are easily fractured resulting in deformities. Osteogenesis imperfecta refers to a wide array of genetic disorders with variable phenotypic presentation. In general, type I is the mildest form of disease; types IV, III, and II, respectively, increase in severity. There is lack of knowledge about the feasibility of nasal surgery in patients with bony disorders in the literature. Although rhinoplasty is one of the most frequent procedures all over the world, there are only very few case reports about OI patients undergoing rhinoplasty. The authors could find 2 old case reports, published in 1965 and 1977. The only recent publication that could be found was published in 2000, but it was about a follow-up case that had been operated 15 years ago. A 24-year-old woman with OI who has been operated on because of severe airway obstruction and cosmetic reasons is presented. Open rhinoplasty was performed, keeping in mind that bones and cartilages are more fragile, and they heal late in OI. Therefore, greenstick fractures were preferred instead of median osteotomies, and cartilages were fixed to each other at multiple points with nonabsorbable sutures. There were no complications in the postoperative period, and the patient was very satisfied with her breathing and appearance. Current literature is reviewed in accordance with our experience with this unique case. In conclusion, good cosmetic and functional rhinoplasty results may be obtained in OI cases with careful case selection, limited mobilization of the nasal bones, and careful fixation of the nasal cartilages with nonabsorbable sutures.

Topics: Female; Humans; Nasal Obstruction; Nose; Osteogenesis Imperfecta; Osteotomy; Rhinoplasty; Young Adult

Osteogenesis imperfecta (OI) is a genetic disorder whose primary manifestations are fractures, bone deformity and bone pain. Brainstem compression due to basilar invagination is a rare and potentially life-threatening complication of OI. Children with this condition often require significant medical input and multiple admissions to hospital. Traditionally, anterior decompression is carried out through an open trans-oral route. We describe an endoscopic endonasal approach for resection of the peg in a child with OI and basilar invagination. We believe this approach provides an excellent alternative with minimal morbidity and decreased length of stay especially in the paediatric population.

Topics: Adolescent; Brain Stem; Constriction, Pathologic; Decompression, Surgical; Female; Humans; Magnetic Resonance Imaging; Natural Orifice Endoscopic Surgery; Nose; Odontoid Process; Osteogenesis Imperfecta; Spinal Cord Compression; Spinal Fusion; Tomography, X-Ray Computed

Topics: Abnormalities, Drug-Induced; Child; Cleft Palate; Congenital Abnormalities; Female; Goiter; Hearing Disorders; Hearing Tests; Humans; Hyperbilirubinemia, Hereditary; Infant; Infant, Newborn; Iris; Kernicterus; Lacrimal Apparatus; Mandibulofacial Dysostosis; Measles; Meningoencephalitis; Mumps; Nose; Nose Deformities, Acquired; Osteogenesis Imperfecta; Otitis Media; Pierre Robin Syndrome; Pregnancy; Pregnancy Complications; Pregnancy Complications, Infectious; Rubella; School Health Services; Toxicology; Waardenburg Syndrome