Docstoc

FIBROUS DYSPLASIA - DOC

Document Sample
FIBROUS DYSPLASIA - DOC Powered By Docstoc
					               Shaheed Suhrawardy Medical
    College Hospital. Dhaka-1207, Bangladesh.
    Department of Orthopedic & Traumatology.
  A case report on treatment of Shapherd crooks deformity in
                        Firous dysplasia

   Dr. Paritosh Chandra
   Debnath1(Associate Prof,), Dr.Kazi
   Shamimuzzaman2 (Assist. Prof),
   Dr.Subir Hussain3 (Assist.
   Registrar), Dr. Md. Nazrul Islam4
   (Resident Surgeon) , Department of
   Orthpedic & Traumatology.
   Shaheed Suhrawardy Mesdical College
   Hospital. Dhaka-1207, Bangladesh.

Summery

   Fibrous dysplasia the lesion of bone
consisting of irregularly arranged bone
trabeculae in bland fibrous tissue. It
is the cystic lesion of bone that can
weaken    and    lead   to  pathological
fracture. It can involve a single bone
(monostatic fibrous dysplasia), but in
other   it   may   involves many   bones

                                                               1
(polyostotic fibrous dysplasia). The
severity of the disorder ranges from
isolated small lesion             that remains
asymptomatic to lesions that cause
repeated        pathological       fracture    &
deformity of the bone. A young male
patient about 20 years old came to us
with huge cystic lesion in the proximal
femur    with          pathological    fracture,
typically      known as Shepherds          Crook
deformity. The object of this case
report    is        to    highlight   the   huge
deformity with pathological fracture &
emphasis the surgical procedure where
correction of the bony deformity by
osteotomy, bone strengthening and bone
union by mixed bone graft has been
achieved satisfactorily in a single
surgical procedure.
Introduction

 Most authors believe fibrous dysplasia
is a developmental anomaly of bone
formation    that    may   exist   in   a
monostatic or     polyostatic form. In
fibrous dysplasis trabecular bone is
replaced by cellular fibrous tissue. If
the lesions are large the bone is
considerably      weakened     and    the
pathological fracture with progressive
deformity   may    occur.   Small  single

                                               2
lesions are asymptomatic. But patients
with polyostatic disease present in
childhood or adolescent with pain,
limp, bony enlargement, deformity &
pathological       fracture.         Fibrous
dysplasia can occur in the epiphysis,
metaphysic or diaphysis that affects
the ribs, tibia, femur, maxilla, skull,
humerus,   pelvis, radius      and    rarely
spine. Number of bone involvement in
polyostatic fibrous dysplasia varies
from 2-12, the most common involvement
in monostatic variety is femur. It
appears    in     childhood      and     may
progressively enlarge. They are less
active or inactive in skeletal maturity
but rarely can they grow in adult.
Fibrous dysplasia may be associated
with precocious puberty and darkly
pigmented skin lesions called Albright
syndrome. X-Ray of the lesion shows
radio-lucent   cystic    areas     in    the
metaphysic or shaft which contains
fibrous tissue with diffuse spots of
immature   bone,   the    lucent     patches
typically have a slightly hazy or
ground glass appearance. The weight
bearing   bone   may   bend    in    massive
osteolytic   lesion   and    one   of    the
classic feature     is Shepherds       crook
deformity of proximal femur. Malignant

                                           3
transformation to fibrosarcoma occurs
in 5-10% of patients with polyostatic
lesions but only rarely in monostastic
lesions.

Case report

  A 20 years old boy came to us with
difficulty in walking for 7 months. He
could not walk without support. He had
a history of mild trauma to the right
thigh 7months back He noticed mild
aching pain in right upper thigh with
gradual shortening and bending in right
thigh.     His right lower limb was
shortened by 5 cm. of his femoral
component and huge hard swelling was
seen in the right upper thigh. Abnormal
mobility   of  the   upper  femur  with
moderate tenderness was found.
Investigations

    1.     X-Ray of the right upper femur
           shows a huge osteolytic lesion
           involving right upper femur,
           right trochanter, with loss of
           cortical bone and lucent patches
           typically hazy looks like grown
           glass appearance with
           pathological fracture at the
           subtrochantaric region.

                                          4
    2.    Blood for TC,DC,ESR,Hb%--
          Normal
    3.    CXR-P/A-- Normal
    4.    MT-- Normal
    5.    RBS - Normal
    6.    S. creatinine- normal
    7.    Blood urea- normal
            8.             S. calcium -
Normal
          9.              Alkaline
phosphatase- Normal


Operation note
  Through lateral approach upper end of
the femur was exposed. Outer part of
the proximal femur was so thin that it
needs little effort to curette the
cystic areas carefully. After curettage
vulgus wedge osteotomy was done at
subtrochantaric    region   to    correct
deformity, massive irradiated allograft
with fibular autograft was applied to
enhance healing and incorporation of
the cystic bony lesion and fragments
were fixed with proximal femoral nail
(PFN). Wound was closed in layers by
keeping   a  drain   inside   which   was
removed after48 hours. Stiches were
removed after 10th post operative day

                                          5
and   the   healing    was   satisfactory.
Abduction    bar    was    applied   after
completion of the operation to protect
the weakened bone for 2 months. After
removal of the abduction bar at 2
months clinically and radiologically
bone    was      stable     and    uniting
satisfactorily. He was advised to use
crutch for non weight bearing of his
right lower limb upto 3 months.

Histopathological report

Curetted material was sent for
hitopathological study and report shows
fibrous dysplasia.

Pre-operative Radiological Picture

Plane X-ray Rt. Upper femur
(Preoperative)




                                          6
Post Operative radiograph.




                             7
Post operative period with derotation Bar   Follow-up visit




Discussion


Fibrous dysplasia is a developmental
disorder in which trabecular bone are
replaced by cellular fibrous tissue
affects the single bone (monostatic) or
many bones (polystatic). Small single
lesions    are    asymptomatic.   Large

                                                              8
monostatic lesion causes pain & may
develop   pathological     fractures.   The
biopsy may require for establishing
diagnosis. Some lesions may resolves
spontaneously & very large deformed
cystic    lesions     with     pathological
fracture    require    special     surgical
measure for correction of deformity,
fixation     of    bone      by    nailing,
strengthening of bone by bone grafting.
A mixture of cortical & cancellous bone
may provide added strength even this
lesion is not fully eradicated. In this
unusual deformed case we carefully
planned to curette the cystic areas of
bone, lateral wedging for correction of
the varus deformity, proximal femoral
nailing   was    done    for    maintaining
reduction & stability, and erradiated
cancellous bone mixed with fibular
cortical bone were introduced in the
lesion    to    enhance      incorporation,
strengthening & union of the bone. This
is a rare & difficult case to treat.
After   applying    this     procedure   in
Shaheed   Surhawardy     Medical    college
Hospital, union was achieved in usual
time,    bone     graft       was    nicely
incorporated & maximum deformity has
been corrected & shortening was also
overcome following operation.

                                          9
Reference
1. Apley’s System of Orthopaedics and
  fractures.8th edition .2001 . 174-175
2. Campbell’s Operative Orthopaedics.
  10th edition. 2003. 794
3. Turek’s Orthopaedics, Principle &
  their application. 5th edition. 1994.
  299-300.
4. Adam’s J.C. Outline of Orthopaedic.
  12th edition, Churchill Livingston.
  1995. 53-54




                                          10

				
DOCUMENT INFO
Shared By:
Categories:
Tags:
Stats:
views:153
posted:2/6/2010
language:English
pages:10