Till Koehne1, Anja Köhn2, Reinhard E Friedrich3, Uwe Kordes4, Thorsten Schinke5, Nicole Muschol2, Bärbel Kahl-Nieke6. 1. Department of Orthodontics, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany. tkoehne@uke.de. 2. Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 3. Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 4. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 5. Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. 6. Department of Orthodontics, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany.
Abstract
OBJECTIVE: The aims of this study were to analyze the maxillomandibular morphology of patients with mucopolysaccharidosis (MPS) type I, II, III, IVa and VI and to evaluate the craniofacial effect of hematopoietic stem cell transplantation (HCST) in MPS I. MATERIALS AND METHODS: One hundred head magnetic resonance images were retrospectively analyzed from 41 MPS and 27 control individuals. The width, height and length of the maxilla and mandible were plotted against age and the means of controls, MPS I, MPS II and MPS III were statistically compared. To determine the effect of HSCT in MPS I, jaw morphology was compared between MPS I patients with full donor chimerism versus patients with mixed/no donor chimerism. RESULTS: Maxillary dimensions were not statistically different between the MPS types. The height and length of the mandible were clearly smaller in MPS I as compared to those in controls, MPS II and MPS III. This was associated with progressive resorption of the mandibular condyles in MPS I, which was also observed in MPS II and VI, but not in MPS III or IVa. Whereas the success of HCST did not affect these changes, mandibular width was significantly smaller in MPS I individuals with full donor chimerism. CONCLUSION: MPS I individuals have a smaller mandible as compared to control, MPS II and MPS III individuals due to progressive condylar degeneration. These abnormalities are also evident following successful HSCT. CLINICAL RELEVANCE: Clinicians should be aware of specific differences in mandibular morphology and condylar involvement among the MPS subtypes.
OBJECTIVE: The aims of this study were to analyze the maxillomandibular morphology of patients with mucopolysaccharidosis (MPS) type I, II, III, IVa and VI and to evaluate the craniofacial effect of hematopoietic stem cell transplantation (HCST) in MPS I. MATERIALS AND METHODS: One hundred head magnetic resonance images were retrospectively analyzed from 41 MPS and 27 control individuals. The width, height and length of the maxilla and mandible were plotted against age and the means of controls, MPS I, MPS II and MPS III were statistically compared. To determine the effect of HSCT in MPS I, jaw morphology was compared between MPS Ipatients with full donor chimerism versus patients with mixed/no donor chimerism. RESULTS: Maxillary dimensions were not statistically different between the MPS types. The height and length of the mandible were clearly smaller in MPS I as compared to those in controls, MPS II and MPS III. This was associated with progressive resorption of the mandibular condyles in MPS I, which was also observed in MPS II and VI, but not in MPS III or IVa. Whereas the success of HCST did not affect these changes, mandibular width was significantly smaller in MPS I individuals with full donor chimerism. CONCLUSION:MPS I individuals have a smaller mandible as compared to control, MPS II and MPS III individuals due to progressive condylar degeneration. These abnormalities are also evident following successful HSCT. CLINICAL RELEVANCE: Clinicians should be aware of specific differences in mandibular morphology and condylar involvement among the MPS subtypes.
Entities:
Keywords:
Cephalometry; Craniofacial; Hematopoetic stem cell transplantation; MRI; Mucopolysaccharidosis; OSAS
Authors: Mark J Osborn; Beau R Webber; Ronald T McElmurry; Kyle D Rudser; Anthony P DeFeo; Michael Muradian; Anna Petryk; Benedikt Hallgrimsson; Bruce R Blazar; Jakub Tolar; Elizabeth A Braunlin Journal: J Inherit Metab Dis Date: 2016-10-14 Impact factor: 4.982
Authors: Erlane Marques Ribeiro; Cristiane S R Fonteles; Adriana Bezerra Freitas; Karla Shangela da Silva Alves; André Jalles Monteiro; Carlos Antônio Bruno da Silva Journal: Cleft Palate Craniofac J Date: 2014-06-11