2 research outputs found
Bone growth, modeling and remodeling in a supernumerary metatarsal bone associated with segmental gigantism in cutis marmorata telangiectatica congenita
Skeletal structure and processes of bone
growth, modeling and remodeling were studied in a
supernumerary metatarsal surgically removed from a 3-
year-old boy affected by Cutis Marmorata
Telangiectatica Congenita (CMTC), associated with
hypertrophy of the right upper and lower limbs and
postaxial hexadactylism of the homolateral hand and
foot. No other anomalies were observed. The excess of
periosteal growth, due to congenital anomaly, induced an
abnormal development of both modeling and remodeling
processes. In bone modeling, osteoblast activity on the
periosteal surface was not paralleled by osteoclast
resorption along the wall of the medullary canal, and this
enormously increased the cortical thickness. In bone
remodeling, osteoclastic resorption cavities were not
refilled by secondary Haversian systems, thus inducing a
severe bone loss. While the alteration of bone growth
and modeling can be ascribed to the congenital disease,
the unbalanced bone remodeling appears mainly to
depend on mechanical disuse of the supernumerary
metatarsal