PMC6394545_01
Clinical manifestations of SMS in the family. a A pacemaker generator, sternotomy wires, and a prosthetic mitral valve are present. b Calcification of the abdominal aorta is observed and the lumbar vertebral bodies show low bone mineral density. c Joint deformities are evident due to metacarpophalangeal joint subluxation. d Calcification is seen at the insertion of the Achilles tendon and the plantar fascia. e Toenails are hypoplastic and subcutaneous calcium deposition is evident over the fourth toe
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Fig1
AV block led to the placement of a dual-chamber pacemaker, and at age 52 years, mechanical mitral valve replacement was undertaken due to severe calcification (Fig. 1a); a single-vessel CABG was performed to relieve a 50% occlusion of the right coronary artery. A chest radiograph at this time revealed a tortuous and calcified thoracic aorta. Upon evaluation at age 59 years, she had marked calcification of the abdominal aorta (Fig. 1b), ulnar deviation of the fingers (Fig. 1c), and calcification of the Achilles tendons and plantar fascia (Fig. 1d).
Dental manifestations of SMS. Clinical photography of maxillary a and mandibular b teeth showing failure of eruption of many permanent teeth. A dental panoramic radiograph c confirmed that most of these permanent teeth were impacted in the bone. d Anterior periapical radiograph showing very short roots. e Periapical view of upper left quadrant teeth revealing absent periodontal ligament space consistent with ankylosis
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Fig2
He has worn a full upper denture over the partially erupted teeth since the age of 15 years (Fig. 2a-b). Radiographically, there appeared to be a normal number of developing permanent teeth, most of which remained subgingival and partially impacted in the bone (Fig. 2c). Tooth crown development appeared normal; however, the roots were very short (Fig. 2d) and tapered with closed root apices. While the pulp chamber was visible to suggest the submerged teeth were vital, the periodontal ligament space could not be discerned (Fig. 2e) consistent with root ankylosis.