title: Cranial vault remodeling in microcephalic osteodysplastic primordial dwarfism type II and craniosynostosis.
abstract: This is a survey of the long-term result after various surgical treatments in a child with microcephalic osteodysplastic primordial dwarfism type II (MOPD II) and craniosynostosis. We report a 17-year-old patient with MOPD II but some unusual clinical signs including bilateral knee dislocation, a misplaced upper lobe bronchus, and hypoplasia of the anterior corpus callosum. Because of premature fusion of several cranial sutures, the child developed signs of increased intracranial pressure with somnolence and papilledema. Cranial vault remodeling with fronto-orbital advancement was performed twice at the age of 16 and 21 months to open the abnormally closed suture, increase the intracranial volume, and relieve the elevated intracranial pressure. Following this procedure, the child's neurologic situation recovered significantly. Surgical procedure of fronto-orbital advancement and the performed reoperation in our patient were safe with no major complications intraoperatively and postoperatively with good functional and satisfying aesthetic outcomes in the long-term follow-up, expressed by the patient, his parents, and the surgeons.
authors.affiliation: Department of Oral and Maxillofacial Surgery, University Hospital Heidelberg, Heidelberg, Germany. Michael.engel@med.uni-heidelberg.de
authors.forename: Michael
authors.initials: M
authors.lastname: Engel
authors.forename: Gregor
authors.initials: G
authors.lastname: Castrillon-Oberndorfer
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hoffmann
authors.forename: Marcus
authors.initials: M
authors.lastname: Egermann
authors.forename: Christian
authors.initials: C
authors.lastname: Freudlsperger
authors.forename: Oliver Christian
authors.initials: OC
authors.lastname: Thiele
mesh_terms: D000015:Abnormalities, Multiple; D000293:Adolescent; D003393:Cranial Sutures / Q000000981:diagnostic imaging / Q000601:surgery; D003398:Craniosynostoses / Q000000981:diagnostic imaging / Q000601:surgery*; D003399:Craniotomy / Q000379:methods*; D004392:Dwarfism; D006801:Humans; D008297:Male; D008831:Microcephaly / Q000000981:diagnostic imaging / Q000601:surgery*; D011862:Radiography, Panoramic