Solitary median maxillary central incisor syndrome (SMMCI) with congenital nasal puriform aperture stenosis: literature review and case report with comprehensive dental treatment and 14 years follow-up.

BACKGROUND: Solitary median maxillary central incisor syndrome [SMMCI] is an extremely rare anomaly, especially when no other abnormalities are present. The defect is often found together with various nasal abnormalities and short stature with or without decreased levels of growth hormone. In more severe cases, SMMCI has been associated with holoprosencephaly, the CHARGE and the VACTERL association. Also, published sporadic cases have been related with rare variants of ectodermal dysplasia, chromosomal abnormalities, precocious puberty, hypothalamic hamartoma, congenital heart defects, physical/mental retardation, genital hypoplasia and ear abnormalities. For these reasons when the initial diagnosis is made by the paediatric dentist, ENT, neurological and paediatric evaluations are essential. CASE REPORT: A 4-year-old boy with SMMCI was referred for dental treatment. Clinical/radiographic examination revealed a symmetrical primary and permanent SMMCI, a skeletal Class I and a unilateral crossbite. Medical history indicated respiratory distress and surgery soon after birth due to congenital nasal puriform aperture stenosis. Gradual orthodontic treatment started at the age of 4 years and completed at the age of 13 years. Following maxillary expansion, upper lateral segments were moved backwards and anterior space was created for accommodating a second central incisor. Retainers with a supplementary acrylic incisor were provided for aesthetic and functional replacement until the age of 16 years, when a fixed Maryland ceramic bridge was placed. FOLLOW-UP: Two years recall, at the age of 18 years, revealed a satisfactory and stable aesthetic and functional result. CONCLUSION: Successful dental management of SMMCI patients is possible, following a detailed long-lasting treatment plan requiring multidisciplinary paediatric dental, orthodontic and prosthetic approach.

A quantitative study of humeral cartilage in individuals with spinal cord injury.

STUDY DESIGN: Cross-sectional design. OBJECTIVES: In this in vivo study, we investigated the influence of different load situations on humeral cartilage thickness in individuals with paraplegia and quadriplegia. SETTING: ETH Zurich and Swiss Paraplegic Centre Nottwil, Switzerland. METHODS: A previously validated three-dimensional (3D) gradient echo MRI-sequence with selective water excitation was used. Three groups were compared: individuals with paraplegia with high shoulder demand (n=11), individuals with quadriplegia with reduced load on the shoulder joint (n=8) and a control group (n=9). After 3D reconstruction, the mean cartilage thickness, local thicknesses (superior, medial, inferior) and the minimum joint space were calculated. RESULTS: The mean humeral cartilage thickness (1.40+/-0.14 mm) as well as the minimum joint space (2.57+/-0.50 mm) did not differ between groups. In individuals with paraplegia, the superior cartilage thickness was significantly greater than in individuals with quadriplegia (1.47 mm compared to 1.28 mm, P<0.05). In the control group, there was a significant negative correlation between mean cartilage thickness and age (r=-0.81). CONCLUSION: Neither higher shoulder demand nor reduced shoulder loading leads to thinning of humeral cartilage. It is hypothesized that higher local stresses prevent local cartilage degeneration, as in normal load situations humeral cartilage thinning is occurring with age. Furthermore, joint space narrowing was only associated with inferior cartilage thickness and cartilage thickness is not related to shoulder pain.