Osteopetrosis (OP) is a rare hereditary disease that causes reduced bone resorption and increased bone density as a result of osteoclastic function defect. Our aim is to review the difficulties, mid-term follow-up results, and literature encountered during the treatment of OP. This is a retrospective and observational study containing data from nine patients with a mean age of 14.1 years (9 to 25; three female, six male) with OP who were treated in our hospital between April 2008 and October 2018 with 20 surgical procedures due to 17 different fractures. Patient data included age, sex, operating time, length of stay, genetic type of the disease, previous surgery, fractures, complications, and comorbidity.Aims
Methods
This article presents a unified clinical theory
that links established facts about the physiology of bone and homeostasis,
with those involved in the healing of fractures and the development
of nonunion. The key to this theory is the concept that the tissue
that forms in and around a fracture should be considered a specific
functional entity. This ‘bone-healing unit’ produces a physiological
response to its biological and mechanical environment, which leads
to the normal healing of bone. This tissue responds to mechanical
forces and functions according to Wolff’s law, Perren’s strain theory
and Frost’s concept of the “mechanostat”. In response to the local
mechanical environment, the bone-healing unit normally changes with
time, producing different tissues that can tolerate various levels
of strain. The normal result is the formation of bone that bridges
the fracture – healing by callus. Nonunion occurs when the bone-healing
unit fails either due to mechanical or biological problems or a
combination of both. In clinical practice, the majority of nonunions
are due to mechanical problems with instability, resulting in too
much strain at the fracture site. In most nonunions, there is an
intact bone-healing unit. We suggest that this maintains its biological
potential to heal, but fails to function due to the mechanical conditions.
The theory predicts the healing pattern of multifragmentary fractures
and the observed morphological characteristics of different nonunions.
It suggests that the majority of nonunions will heal if the correct
mechanical environment is produced by surgery, without the need
for biological adjuncts such as autologous bone graft. Cite this article:
We report the outcome of 84 nonunions involving
long bones which were treated with rhBMP-7, in 84 patients (60 men:
24 women) with a mean age 46 years (18 to 81) between 2003 and 2011.
The patients had undergone a mean of three previous operations (one
to 11) for nonunion which had been present for a mean of 17 months
(4 months to 20 years). The nonunions involved the lower limb in
71 patients and the remainder involved the upper limb. A total of 30
nonunions were septic. Treatment was considered successful when
the nonunion healed without additional procedures. The relationship
between successful union and the time to union was investigated
and various factors including age and gender, the nature of the
nonunion (location, size, type, chronicity, previous procedures,
infection, the condition of the soft tissues) and type of index
procedure (revision of fixation, type of graft, amount of rhBMP-7) were
analysed. The improvement of the patients’ quality of life was estimated
using the Short Form (SF) 12 score. A total of 68 nonunions (80.9%) healed with no need for further
procedures at a mean of 5.4 months (3 to 10) post-operatively. Multivariate
logistic regression analysis of the factors affecting union suggested
that only infection significantly affected the rate of union
(p = 0.004).Time to union was only affected by the number of previous
failed procedures
(p = 0.006). An improvement of 79% and 32.2% in SF-12 physical and
mental score, respectively, was noted within the first post-operative
year. Rh-BMP-7 combined with bone grafts, enabled healing of the nonunion
and improved quality of life in about 80% of patients. Aseptic nonunions
were much more likely to unite than septic ones. The number of previous
failed operations significantly delayed the time to union. Cite this article:
We reviewed 59 bone graft substitutes marketed
by 17 companies currently available for implantation in the United Kingdom,
with the aim of assessing the peer-reviewed literature to facilitate
informed decision-making regarding their use in clinical practice.
After critical analysis of the literature, only 22 products (37%)
had any clinical data. Norian SRS (Synthes), Vitoss (Orthovita),
Cortoss (Orthovita) and Alpha-BSM (Etex) had Level I evidence. We question
the need for so many different products, especially with limited
published clinical evidence for their efficacy, and conclude that
there is a considerable need for further prospective randomised
trials to facilitate informed decision-making with regard to the
use of current and future bone graft substitutes in clinical practice. Cite this article: