The mucopolysaccharidoses (MPS) are a group of
inherited lysosomal storage disorders with clinical manifestations relevant
to the orthopaedic surgeon. Our aim was to review the recent advances
in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising
orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include
the recognition of slowly progressive, late presenting subtypes,
developments in life-prolonging systemic treatment and potentially
new indications for surgical treatment. The outcomes of surgery
in these patients are not yet validated and some procedures have
a high rate of complications which differ from those in patients
who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or
young adults with a previously unrecognised dysplasia of the hip.
Surgeons treating patients with a MPS should report their experience
and studies should include the assessment of function and quality
of life to guide treatment. Cite this article:
Primary total knee arthroplasty (TKA) is a reliable
procedure with reproducible long-term results. Nevertheless, there
are conditions related to the type of patient or local conditions
of the knee that can make it a difficult procedure. The most common
scenarios that make it difficult are discussed in this review. These
include patients with many previous operations and incisions, and
those with severe coronal deformities, genu recurvatum, a stiff knee,
extra-articular deformities and those who have previously undergone
osteotomy around the knee and those with chronic dislocation of
the patella. Each condition is analysed according to the characteristics of
the patient, the pre-operative planning and the reported outcomes. When approaching the difficult primary TKA surgeons should use
a systematic approach, which begins with the review of the existing
literature for each specific clinical situation. Cite this article:
