Swelling of the right leg for diagnosis

by. Dr. Giridhar Boyapati

PG.

Dept. of Orthopaedics

CASE REPORT A 14 year old girl presented with chief complaints of pain

and swelling of right leg since 4 months.

Pain in middle third of leg which is insidious in onset, progressive in nature, dull aching continuous type of pain. Aggravated by weight bearing. Relived by rest and medication.

Swelling is gradually progressive in size . Not associated with edema of the limb.

No history of trauma ,fever , chronic cough.

No history of recent significant loss of appetite or weight loss.

No other co-morbid conditions, otherwise a healthy individual.

Family history: no similar complaints in family members. No history of contact with pulmonary tuberculosis .

Menstrual history: attained menarche, no menstrual irregularities.

GENERAL EXAMINATION Pallor present. No clubbing , no icterus no generalized lymphadenopathy.

No similar swellings elsewhere in the body, no thyroid and breast swellings.

CVS/CNS/RS : NAD

Abdomen: soft, non tender, no organomegaly.

No signs of infection or any chronic disease.

LOCAL EXAMINATION

Swelling of size 10x5 cm over the anterior aspect of proximal right leg.

Skin over the swelling is normal, no trophic changes ,no scars and sinuses, no dilated veins.

Firm bony swelling with ill-defined margins, slightly tender.

Local rise of temperature present.

The swelling is continuous with tibia proximally and distally, moving along with tibia.

LOCAL EXAMINATION

No muscle wasting.

Fibula is palpable separately from the swelling.

No pulsations, no bruit heard over the swelling. Movements at knee and ankle joint are normal. Active toe movements present.

No distal neurovascular deficit.

PRE OP PHOTO

Swelling of the Right leg

skin condition normal.

PROVISIONAL CLINICALDIAGNOSIS

Bony swelling arising from diaphysio-metaphyseal junction of right tibia; with out any pressure effects.

1. Aneurysmal bone cyst.

2. Unusual presentation of Simple bone cyst.

3. Osteochondroma.

4. Ewing's sarcoma.

4. Telangiectatic osteosarcoma.

5. Fibrous dysplasia.

INVESTIGATIONS

Normocytic Normochromic anemia.

No infection

Urine routine is normal.

HB 9.0gm%

PCV 27%

TWC 82OOcells/cumm

ESR 15mm 1st hr

BT 2min

CT 4min

CRP NEG

INVESTIGATIONS

Chest X-RAY normal study RBS 92 mg%

SODIUM 140 meq/l

potassium 4.2 meq/l

T.bilurubin 0.5 mg%

B.urea 12 mg%

S.creatinine 0.6 mg%

ALP 40 IU/L

RADIOGRAPH

Standard Anterior- posterior and LATERAL views of the right leg

RADIOLOGY REPORT

Expansile lytic lesion located in postero-lateral aspect of tibia with thin internal septations which are thickened trabecule noted in the diaphysis.

Lesion shows thin cortex.

Finger in balloon sign.

Fibula appears to be normal.

No soft tissue swelling adjacent to the lesion

DIFFERENTIAL DIAGNOSIS

After radiographic study:1.Aneurysmal bone cyst.

2.Unicameral bone cyst.

3. Giant cell tumor of bone.

4. Telangiectatic osteosarcoma.

FURTHER EVALUATION

C.TM.R.IBONE SCANF.N.A.C/BIOPSY

C.T 3D RECONSTRUCTION

CT showing lytic lesion

MRI OF RIGHT LEG

MRI

MRI Expansile lesion arising from right tibia measuring 9x5x4cm

Lesion is multiseptated and multiloculated.

Frank breach seen in the posterior cortex.

T1W heterogenous with signal intensities varying from iso-hyperintense.

T2W significantly hyper intense.

Multiple fluid-fluid levels.

No joint extension.

Minimal edema in adjacent muscles in the posterior part.

CONTRAST MRI

After contrast study, the walls and septations are enhancing.

cyst contents and blood products are not enhancing.

M.R.I IMPRESSION

Bone lesion at upper diaphysis of right tibia is more in favor of

Aneurysmal bone cystother possibilities:

1. Hemorrhagic Simple bone cyst

2.Telengectatic osteosarcoma

BONE SCAN 3 PHASE BONE SCAN

Using Tc-99 MDP.

Photon deficient area with Rim of intense tracer uptake noted in proximal shaft of right tibia – Aneurysmal bone cyst.

No evidence of any other lesion on whole body skeletal survey.

FNAC

Smears show hemorrhagic background with occasional multinucleate giant cells and inflammatory background.

IMPRESSION: cytological features are suggestive of aneurysmal bone cyst.

CULTURE SENSITIVITY OF ASPIRATE

No bacterial growth after 48hrs. Gram staining and AFB were

negative

. CASE CASE FINDINGSFINDINGS A.B.CA.B.C SIMPLE SIMPLE

BONE BONE CYSTCYST

TELANGIECTATITELANGIECTATICC

OSTEOSARCOMA

GAINT CELL GAINT CELL TUMORTUMOR

AGEAGE 14yr 10-20yr 10-20yr >30yr >30yr

SEX SEX female female male male female

SITESITE DIAPHYSISMETAPHYSIS

RARELY DIAPHYSIS

METAPHYSIS METAPHYSIS EPIPHYSIS

APPEARENCEAPPEARENCE ECCENTRIC ECCENTRIC CENTRAL CENTRAL ECCENTRIC

MRIMRIMULTIPLE

FLUID -FLUID LEVELS

MULTIPLE FLUID-FLUID

LEVELSNO

MULTIPLE FLUID -FLUID

LEVELSNO

GROSS GROSS APPEARANCEAPPEARANCE

BLOOD FILLED CYSTIC SPACES

BLOOD FILLED CYSTIC SPACES

CYST CONTAINING

CLEAR SEROUS FLUID

BLOOD FILLED CYSTIC SPACES

BLOOD FILLED CYSTIC SPACES

MANAGEMENTPatient was advised surgical management.

INDICATION FOR SURGERY 1.To prevent pathological fracture. 2. For open biopsy to rule out malignant tumors.

Informed consent was taken from the patient and her attenders for surgery.

MANAGEMENT

EXTENDED CURETTAGE, FIBULAR BONE GRAFTING AND FIXATION WITH

DYNAMIC COMPRESSION PLATE.

SURGICAL APPROACH

Under tourniquet to reduce blood loss

Anterolateral approach to proximal tibia.

S shaped incision.

Tibialis anterior is retracted laterally to expose the lesion.

EXPOSING THE LESION

Lesion is about 10x5 cm involving proximal tibial shaft.

Soft cystic swelling surrounded by thin cortical bone.

CURETTAGE

Entire lesion is curetted and removed

No surrounding soft tissue involvement

Material sent for histopathology.

FIXATION

After curettage only antero-medial cortex of tibia is left intact.

To prevent any fracture, fixation along with bone grafting is required.

Lesion is not involving the Fibula

OBTAINING FIBULAR GRAFT

Fibular graft is obtained from left leg.

This graft is used to provide additional support for right tibia.

Fibula is split vertically and fixed in medullary canal of tibia.

Fibula graft

POST OPERATIVE X-RAY OF LEFT LEG

fibula graft .

FIXATION

Tibia is fixed using 10 holed D.C.P and screw fixation along with fibular grafting.

POST OPERATIVE X-RAYS

showing tibia fixed with plating and screw fixation and fibular grafting.

POST OPERATIVE PERIOD

Post operative period is uneventful

No h/o fever.

No postoperative wound complications.

After suture removal right leg is immobilized in Above knee synthetic cast.

FOLLOW UP 1MONTH

Fibular graft with implant insitu in tibia.

FOLLOW UP 2 MONTHS

Fibular graft insitu.

patient was allowed partial weight bearing.

LEFT LEG X-RAY

Intact periosteum of the fibula which was excised and used as graft.

FOLLOWUP

Surgical Incision healed by primary intension.

Postoperative knee and ankle movements are normal

No distal neurovascular deficit.

BIOPSY

biopsy report confirmed the diagnosis of ABC

HISTOPATHOLOGY

Histopathology

Large cystic spaces filled with blood and separated by fibrous septa, alternating with solid areas.

Cysts and septa lined by fibroblasts, myo-fibroblasts and histiocytes but not endothelium.

Clusters of osteoclast-like multi-nucleated giant cells with loose spindly stroma to cellular stroma, reactive woven bone.

Variable mitotic figures and hemosiderin. No malignant osteoid, no atypia.

DISCUSSION ABC Aneurysmal bone cyst is a benign osteolytic bone lesion first

described by Jaffe and Lichtenstein in 1942.

ABC is a pseudo-tumoral lesion of unknown etiology accounting for 1% of bone tumors.

They are locally destructive, blood filled lesions.

Demographics: 75% of patients are < 20 yrs.

ABC is most common during the second decade of life and rare in children under 5 years age.

Ratio of female to male is 2:1.

Location

Most common in metaphysis of long bones. Commonly effecting proximal humerus , distal femur and proximal tibia. Most often eccentrically located in the metaphysis. Diaphysis involvement is rare. Epiphyseal lesions are usually intramedullary and associated with chondroblastoma or giant cell tumor. Spinal lesions account for 12-30% of cases. The pelvis accounts for about half of all the flat bones involved.

Location TIBIA 17.5% FEMUR 15.9%

VERTEBRA 11%

PELVIS 11%

HUMERUS 9%

FIBULA 7.3%

FOOT 6.3%

HAND 4.7%

ULNA 3.8%

RADIUS 3.1%

Pathophysiology

Primary ABC:▪ Occurring de-novo , no pre existing lesion.

known to be neoplasms driven by up-regulation of the ubiquitin-specific protease USP6 ( Tre 2) gene on 17p13 .

Secondary ABC:ABC caused by reaction secondary to another bony

lesion. Account for 20- 30% of ABCs Not considered a neoplasm because no known

translocation has been identified

◦

Associated conditions

ABC is associated with other tumors 30% of timeGiant cell tumorChondroblastomaOsteoblastomaFibrous dysplasiaChondromyxoid fibromaNon ossifying fibroma

Pathophysiology

Focal vascular malformation with in the bone, like A-V fistulas, venous blockage

Increased pressure, expansion, erosion, and resorption of surrounding bone.

Local hemorrhage initiate formation of reactive osteolytic lesion.

PresentationSymptoms pain and swelling may present with pathologic fracture in about 8% of cases

Physical examination neurologic deficits possible with spine lesions

NATURAL HISTORY OF ABC

ABC evolve through 4 radiologic stages:

INITIAL PHASE : well defined area of osteolysis with elevation of periosteum

GROWTH PHASE: lesion grows rapidly with progressive destruction of bone. Characteristic BLOWN OUT appearance

STABILIZATION PHASE: maturation of the bony shell giving characteristic SOAP BUBBLE appearance

HEALING PHASE: progressive calcification and ossification of the lesion

STAGING

ENNEKING staging of benign lesions

Stage 1 ( latent ) :intra-compartmental, lesion have well defined cortex

Stage 2 ( active ) : continue to enlarge, lesion have thinned cortex which may be broken but limited to the periosteum

Stage 3 ( progressive ) : lesion penetrate the cortex.

CAPANNA ET AL CLASSIFICATION

Based on radiographic findings:

TYPE 1: central metaphysial presentation

TYPE 2: lesion involve the entire segment of bone.

TYPE 3: eccentric metaphysial location

TYPE 4: sub-periosteal extension

TYPE 5: meta-diaphysial location

Treatment

Non-operative management

Indications

ABC with acute fracture

Indicated until fracture has healed. Once healed, treat as an ABC without fracture unless the fracture has led to spontaneous healing of the ABC

Arterial embolisation

Used to treat vascular bone tumors to limit blood supply at surgery or as definitive therapy when surgery is not feasible.

Transcatheter arterial embolisation.

Various materials, such as springs and foam, have been used to create the emboli.

Arterial embolisation

ADVANTAGES:

1.Able to reach difficult locations.

2.Save joint function when subchondral bone destruction is present.

3. Less bleeding during surgery. Performed within 48 hours before surgery to reduce the amount of hemorrhage.

4 Non-surgical technique that may be effective as the primary treatment but, if it fails intervene surgically .

Intralesional InjectionINDICATIONS

1.Surgical access is difficult

2.Other modalities are contraindicated

CONTRAINDICATIONS

1.Patient has allergies to the injection components

2. A pathologic or impeding fracture

3. Neurologic symptoms, or unbearable symptoms such as pain.

4. Do not use intralesional injection if a better proven treatment is indicated.

5. Uncertain diagnosis.

Intralesional Injection

1.Calcitonin :osteoclastic inhibitory effect and the trabecular bone-stimulating properties

2. Methyl prednisolone inhibitory angiostatic and fibroblastic effects

3. ETHIBLOC :mixture of zein, oleum papaveris, and propylene glycol and acts as a fibrosing agent, and an inflammatory reaction may occur after its administration. Bony healing may take months to years.

4.Aqueous solution of calcium sulphate

Percutaneous sclerotherapy using Polidocanol Polidocanol was injected into the lesion under fluoroscopic

guidance using a bone-marrow aspiration needle. Approximately 1 ml of 3% polidocanol

(Hydroxypolyaethoxydodecan) was injected per 1 cm3 volume of the lesion.

No more than 10 ml of sclerosant was injected into any lesion.

Complications Local recurrence, induration at the site of injection, hypopigmentation, local inflammatory reaction, and an episode of dizziness

POLIDOCANOL

Regular followup after injection

End-point of treatment was defined as the time at which the:

1. Pain had resolved,

2. The cortical thickness of the wall of the cyst had started reforming

3. Lesion had stopped growing in size.

A second injection of sclerosant was given if any one or a combination of the above three parameters was not observed in the first three months after treatment.

Surgical Therapy

1. Intralesional curettage.

2. Intralesional excision. The difference between curettage and excision is that excision involves wide unroofing of the lesion through a cortical window by careful abrasion of all the surfaces with a high-speed burr and, possibly, local adjuvants.

3.En bloc or wide excision is typically reserved for ABCs that are not amenable to intralesional excision

Aggressive curettage and bone grafting

Indicated in symptomatic ABC without acute fracture.

20% recur after curettage, so aggressive curettage with bone grafting or en bloc resection is recommended.

If no coexistent lesion is identified ,lesions are managed by simple curettage and bone grafting.

If a more aggressive lesion is present, treatment must be directed toward that component.

Adjuvant therapy

Extends the area of treatment beyond that which can be physically excised.

Adjuvants involve the use of chemical, freezing, or thermal means to cause bone necrosis and microvascular damage to the walls of the physically excised cyst, disrupting the possible etiology.

Compared with en bloc and regional resection, the use of adjuvants leaves more bone intact, and an increased area is treated compared with that treated with intralesional resection alone.

Liquid nitrogen Most popular adjuvant

After the ABC is exposed and a window is opened, liquid nitrogen may be applied by pouring it into the cyst through a funnel or by using a machine that is designed to spray the liquid onto the walls of the lesion.

A total of 2 or 3 cycles of freezing and thawing should be used to obtain maximum bone necrosis.

The surrounding tissue, especially the neurovascular bundles, must be protected to ensure these structures are not damaged.

Avoid the use of a tourniquet with cryotherapy is to keep the surrounding tissue vascularized, making it more resistant to freezing.

Liquid nitrogen

COMPLICATIONS OF LIQUID NITROGEN Gas Embolism

Late fracture

Wound necrosis

Damage to the surrounding tissue (eg, neurovascular bundles, physis)

Phenol Less often used as an adjuvant. Poor penetration of bony tissue compared

with that of liquid nitrogen. Easy to use. Phenol is simply applied to the

mechanically removed walls by using soaked swabs.

Any remaining phenol is removed with suction, and the cavity is filled with absolute alcohol.

Finally, the cavity is irrigated with isotonic sodium chloride solution.

PMMA ( BONE CEMENT) Thermal properties in causing bone necrosis.

Advantages : Immediate stability In case of a large lesion. Easy to recognize a local recurrence.

If PMMA is used in a subchondral location, the joint surface should be protected by cancellous grafts or Gelfoam placed before cementation.

Argon beam coagulation

Surgical treatment with curettage and adjuvant argon beam coagulation is an effective treatment option for ABC

Reducing recurrence

The primary complication was fracture.

MEGAVOLTAGE RADIOTHERAPY

For recurrent tumors, or tumors for which surgery would result in significant functional morbidity, radiotherapy (RT) provide a safe and effective alternative for local control.

Prescribed tumor dose of 25–30 Gy.CO60 or equivalent megavoltage X-rays are

used.Produce rapid ossification of the cyst.Potential for malignant transformation

Endoscopic approach to the treatment of ABC

Minimally invasive.

Technically difficult

FOLLOW-UP Recurrence usually occurs within the first year after surgery,

and almost all episodes occur within 2 years.

Patients should still be monitored on a regular basis for 5 years.

It is beneficial to detect recurrence early when the lesion is still small and easier to treat.

Children should be monitored until they have reached maturity to ensure that any possible recurrence does not cause deformity or interfere with their growth.

Any patients that have received radiation should be monitored for life because of the risk of secondary sarcoma.

RECURRENCE

.

recurrence

Curettage and bone grafting 10-20%

Curettage and cryotherapy 12.8%

Curettage and irradiation 14.3%

Resection 0%

Radiotherapy 16%

Embolization 10.6%

COMPLICATIONSUniversal complications that have been described with surgery include the following: • Recurrence • Blood loss • Wound infection • Wound slough • Wound hematoma • Osteomyelitis • Damage to the surrounding tissue • Possible physis damage • Pulmonary embolism

Outcome and Prognosis The prognosis for an ABC is generally excellent, although

some patients need repeated treatments because of recurrence, which is the most common problem encountered when treating an ABC.

The overall cure rate is 90-95%

Increased risk of recurrence.

1.Younger age,

2.Open growth plates,

3.Metaphyseal location of the lesion

The stage of the ABC has not been shown to influence the rate of recurrence; however, most clinicians believe that stage 3 lesions have the highest recurrence rate.

UNTREATED CASES

May involute spontaneously and develop a heavy shell of reactive bone at periphery. This involution process is hastened by surgical curettage and bone grafting.

May lead to pathological fracture.