phenylephrine-hydrochloride and Dysostoses

Topics: Abnormalities, Multiple; Dysostoses; Forehead; Humans; Nose

Topics: Bone Diseases, Developmental; Craniofacial Dysostosis; Craniosynostoses; Dysostoses; Facial Bones; Female; Frontal Bone; Humans; Infant; Nose; Poland Syndrome

Topics: Cannula; Dysostoses; Humans; Nose; Oxygen

To explore a new accurate way for the treatment of congenital midfacial dysplasia in facial cleft patients.. Between November 2015 and November 2016, 8 patients with nasal deformity and midfacial dysplasia (Tessier Nos. 3-11 cleft) were collected (median age, years; range = 15-20 years). Expanded frontal flap for nasal reconstruction and image-guided navigation-assisted surgery for modified nasal-maxillary-hard palatine osteotomy to advance the peri-pyriform bone structure were performed in all the patients. After 6 to 12 months of follow-up, the authors analyzed the differences between preoperative planning and postoperative results through computed tomography data.. Patients were satisfied with surgery, and computed tomography data showed that there was little difference between preoperative planning and postoperative results with the navigation-assisted surgery.. Using expanded frontal flap with navigation-assisted surgery for peri-pyriform advancement, the authors could treat congenital nasal deformity and midfacial dysplasia effectively, accurately, and safely in craniofacial cleft patients.

Topics: Adolescent; Adult; Craniofacial Abnormalities; Dysostoses; Facial Bones; Humans; Nose; Surgery, Computer-Assisted; Surgical Flaps; Tomography, X-Ray Computed; Young Adult

Acrodysostosis is a rare syndrome characterized by peripheral dysostosis, nasal hypoplasia and frequently mental retardation. Only one adult case of acrodysostosis has been reported to have neurologic symptoms. We report one further adult case of acrodysostosis with severe neurologic findings including myelopathy and spastic paraparesis due to diffuse spinal stenosis and recurrent deep vein thrombosis possibly caused by neurologic deficits.. We report a 43-year-old woman who had back and neck pain with weakness in the extremities of several years. 1~year before admission to our hospital, she had been treated with a missed diagnosis of sero (-) spondyloarthropathy but had not benefited. She became unable to walk, thereafter she underwent decompression surgery with a diagnosis of degenerative spinal stenosis. She presented at our outpatient department complaining of lowback pain and difficulty walking. She had marked facial and peripheral appearance of acrodysostosis. Spinal MRI revealed extensive spinal stenosis. A diagnosis was made through the genetic investigation, clinical and radiological findings. Spastic paraparesis were detected. There was widespread neuropathic pain. 15 days after admission, she developed swelling and redness of the left lower extremity and the venous doppler ultrasonography showed left acute and right past DVT. We treated DVT with anticoagulant therapy. Gabapentin and Baclofen were initiated for neuropathic pain and spasticity. A conventional rehabilitation program was performed. She left walking with a walker without pain and spasticity.. We would like to remind physicians to be aware of peripheral malformations as signs of skeletal dysplasias and to consider acrodysostosis in the differential diagnosis. Although it is a rare condition, if diagnosed early, possible complications can be treated and outcomes may be improved.

Topics: Adult; Anticoagulants; Dysostoses; Female; Hand Deformities, Congenital; Humans; Intellectual Disability; Nose; Paraparesis, Spastic; Spinal Stenosis; Syndrome; Venous Thrombosis

We report a 13-year-old boy with peripheral dysostosis, nasal hypoplasia, brachydactyly and a tracheal stenosis of unknown origin. Acrodysostosis is usually caused by an autosomal gene, but in this case it was probably due to a new mutation. The frequently described mental retardation is not essential feature. Ectopic calcification has not been previously reported.

Topics: Adolescent; Chromosome Aberrations; Chromosome Disorders; Craniofacial Dysostosis; Dysostoses; Foot; Genes, Dominant; Hand; Humans; Male; Nose; Radiography; Tracheal Stenosis