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Meningomyelocele

August 15, 2008 By 1 Comment

In this condition the normally developed spinal cord or cauda equina may be protruded along with the meninges and these structures may be adherent to the posterior aspect of the sac.

Clinical Features

  1. Though myelocele is the most common type of spina bifida (many cases are still born), yet meningomyelocele is the commonest variety of spina bifida in the living children.
  2. The bony defect may usually extend over 3 or more segments.
  3. At the summit of the swelling skin may be absent or atrophic, so here the meninges are exposed. That is why complications like infection and rupture are not uncommon.
  4. In transillumination test the nerves may be seen within the sac (cf. meningocele), though it is a translucent swelling.
  5. Neurological manifestations are almost always present. There may be sensory disturbances alongwith trophic changes in the lower extremities. There may be motor disturbances and in advanced cases one may find extensive paralysis of the legs. Bilateral talipes is quite common in this condition, as also urinary incontinence.
  6. X-ray will show the bony defect. There may be other abnormalities of the vertebrae, like scoliosis or kyphosis or even haemivertebra.

Complications

  1. Infection
  2. Rupture
  3. Urinary obstruction, hydronephrosis and urinary tract infection

Treatment

Operation must be performed as early as possible. Child’s condition should also be considered, as it should withstand operation.

Delay in operation may cause

  1. Infection within the sac and postoperative problems.
  2. The sac may rupture and will make operation extremely difficult with infection spreading throughout the subarachnoid space.
  3. There will be more adhesion of nerves with the wall of the sac, which will be difficult to separate.
  4. Gradually extensive paralysis of the legs and incontinence may occur, so that surgical intervention may become contraindicated.

Steps of Operation

  1. The sac is opened and the redundant membrana is excised. Meticulous care must be taken to separate all the nerve fibres which are adherent to the posterior wall of the sac and then they are replaced into the vertebral canal.
  2. The membranes are sutured in the midline deep to the bony defect.
  3. Adjacent erector spinae muscles and the overlying fasciae are brought in the midline and sutured. Two lateral release incisions are made to minimize tension on the suture line of the muscles. The skin is closed without tension. If difficulty is encountered to bring the skin in the midline, a rotation flap may be used.
  4. Further care of these children may be necessary. Repeated orthopaedic and urological surgery may be necessary to rectify orthopaedic defects and urinary incontinence. C.S.F. shunts may be necessary for hydrocephalus.

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Filed Under: Pediatric Disorders, Spine Tagged With: , , , , , , , , , , , ,

Meningocele

August 13, 2008 By Leave a Comment

In this condition protrusion of meninges occurs through the defect in the neural arch. Such protrusion contains only cerebrospinal fluid. So it gives rise to a cystic swelling.

Usually the duramater stops at the margin of the defect and usually the pia and arachnoid protrude. The overlying skin remains intact.

It is common in the lumbosacral region. Meningocele also occurs in the skull, where it is more common in the occipital region or at the root of the nose.

Clinical Features

  1. It is present since birth
  2. It is a cystic swelling, that means fluctuation test is positive
  3. It is a highly translucent swelling
  4. It is compressible
  5. Expansile impulse is present when the child cries or coughs
  6. The overlying skin is normal and free
  7. On careful palpation the edge of the bony defect is palpable
  8. Neurological manifestations are usually absent (cf. meningo-myelocele, where neurological manifestations are usually present).
  9. This condition may be associated with hydrocephalus and this combination is known as Arnold-Chiari syndrome.
  10. X-ray is confirmatory and will show the bony defect

Complication

  1. Infection
  2. Rupture

Treatment

Operation should be performed as early as possible, but child’s condition and strength should be sufficient to withstand operation. This operation is often performed within a few days of birth. If the operation is delayed are:

  1. The skin and the sac are opened with incisions perpendicular to each other. This will minimize the chance of postoperative C.S.F. leakage.
  2. The redundant part of the sac is excised
  3. The margins of the excised sac are sutured together in the midline
  4. To strengthen the bony gap, the adjacent erector spinae muscle and the overlying fasciae are approximated over the gap with the help of lateral release incisions (to minimize tension in the suture line).
  5. The skin is closed.

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Spina Bifida Occulta

August 12, 2008 By 2 Comments

In Spina Bifida Occulta, though there is failure of the neural arches to unite, and a small gap exists in one of the vertebral arches, usually in the lumbar or sacral regions, yet there is no protrusion of the cord or membranes. Frequently only one vertebra is affected. Te gap is filled with fibrous tissue.

As a rule, there is no projection on the surface to indicate this defect, though a local patch of hair, a naevo-lipoma or a depression in the skin may suggest the underlying bony deficiency. [Read more...]

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Spina Bifida-An Overview

August 12, 2008 By 2 Comments

Congenital defect in the posterior bony wall of the spinal canal involving the laminae, is known as spina bifida. This is most commonly seen in the lumbo-sacral region. Sometimes the contents of the canal may protrude through the defect.

Development of the spinal cord:

  1. During the 2nd week of intrauterine life a dorsal groove appears on the surface of the embryo, which is known as the neural groove.
  2. The margins of the neural groove unite so that it becomes converted into a tube, from which the nervous system is developed. This tube is known as neural tube and the lumen, within it, is called the neural canal. This neural canal persists as the central canal. The neural tube becomes separated from the surface by an ingrowth of the mesoderm. [Read more...]

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Acromioclavicular Joint Dislocation or Separated Shoulder

August 11, 2008 By Leave a Comment

Acromioclavicular (AC) joint dislocations and subluxations are commonly known as shoulder separations.

The true incidence is much higher than reported because many patients with low grade injury don’t seek medical attention.

Injuries to the acromioclavicular joint usually are the result of downward force on the acromion.

The most common mechanism of injury is a fall directly onto the shoulder.

The clavicle rests against the first rib, and the rib blocking further displacement of the clavicle. If the result is not a clavicle fracture, then acromioclavicular and coracoclavicular ligaments are ruptured. [Read more...]

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Soft Tissue Bleeding In Hemophilia

August 5, 2008 By Leave a Comment

Soft tissue bleeding is common in hemophila. Following a direct injury, a large hematoma may accumulate in the subcutaneous tissues. This generally is resorbed spontaneously occasionally ulceration occurs, commonly on the forehead, the olecranon process, or the prepatellar area.

Superficial soft-tissue hemorrhage in the form of ecchymosis is common in the severe hemophiliac. It is of no clinical significance.

Intramuscular and Intermuscular Hemorrhage

Quadriceps is most common site of bleeding in lower limb. Other comon sites are triceps surae,adductors of the thigh, hamstrings , and sartorius.

In the upper limb the most common site of bleeding is deltoid, followed by wrist and finger flexors in the forearm, brachio-radialis, biceps, wrist and finger extensors in the forearm, and triceps.

The presenting complaint is pain on movement or at rest. [Read more...]

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Total Joint Replacement and Arthrodesis In Hemophilia

August 4, 2008 By 2 Comments

In uncorrectable painful deformities, it becomes necessary to perform either of two procedures. It is difficult to decide when to perform arthrodesis and when to do total joint replacement. Individual assessment should be carried out to aid in decision making. The treatment options should be discussed with the patient after evaluating his needs and expectation.

Most common indication for surgery is disabling pain. If involvement is unilateral and the opposite lower limb is relatively normal, arthrodesis is a good option.

Total joint replacement in bilateral knees is indicated with Stage IV or Stage V arthropathy when persistent knee pain is presesent.

Total hip replacement is indicated in Stage IV or Stage V hemophilic arthropathy when pain is persistent with severe disability not relieved by conservative measures. Arthroplasty of the elbow has been reported.

Arthrodesis of the ankle, subtalar and midtarsal joints in the foot, shoulder, or knee may be indicated when these joints are destroyed. The surgical technique is the same as in normal patients with the exception that percutaneous pins should not be utilized in hemophiliacs.

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Surgical Treatment of Hemophilia

August 3, 2008 By Leave a Comment

If deformities caused by hemarthrosis cannot be corrected by conservative closed methods, surgical correction is indicated. For example, if equinus deformity is very severe and rigid, tendo Achillis lengthening is indicated.

Open surgery has become relatively safe, provided the clotting mechanism is restored to near normal by the administration of antihemophilic factor. This should be continued for three weeks. Sutures should be removed on the fourteenth to sixteenth day postoperatively.

Wounds heal normally in hemophilic patients.

Hematologic Management

During surgery and the first postoperative day, the factor level should be raised to 100 percent by infusion of factor concentrate. During the first postoperative week, the factor level is maintained at 50 percent, and subsequently for the first postoperative month at 30 to 40 percent by daily infusions of factor concentrate.

Synovectomy

Synovectomy is done to prevent progression of hemophilic arthropathy. It helps by

  • Decreases the vulnerability to trauma of the highly vascular synovial tissue i
  • Hemophilic synovial tissue has a high level of fibrinolytic activity that tends to prolong the bleeding episodes.
  • Hypertrophic synovial tissue in hemophilia contains increased levels of acid phosphatase and cathepsin D.
  • Hemosiderin deposition in the synovium interferes with the production of collagenases, which may cause death of chondrocytes.

Indications

  • History of severe recurrent hemarthrosis -two or three major bleeding episodes per month
  • Patients who fail to respond to aggressive medical management for a period of at least six months.
  • Failure of response to orthopedic nonsurgical treatment
  • Radiographic Stage II or Stage III hemophilic arthropathy

In stages IV and V synovectomy is not effective and contraindicated.

Complications

  • loss of range of joint motion due to adhesions
  • Massive bleeding.

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Non Surgical Management of Deformities In Hemophila

August 2, 2008 By Leave a Comment

In spite all the measures, there would be cases in haemophilia where deformities would develop. Before surgical correction is taken nonoperative measures should always be employed.

Traction

In flexion deformities of the knee and hip, a period of continuous traction is effective in relieving muscle spasm and increasing range of motion. Initially traction forces are in the line of deformity and are gradually altered to achieve correction.

Prophylactic protection with antihemophilic factor is usually not required while the patient is in traction. Once a neutral or a nearly neutral position is obtained, well-padded plastic splints are used to maintain the part in the corrected position.

Active exercises are begun to increase muscle power and range of motion of the joints.

Plaster Cast

If functional range of motion is not achieved after two or three weeks of traction, a wedging cast is applied. The antihemophilic factor is administered when the cast is wedged.

When full joint motion is achieved the knee is immobilized for a period of seven to ten days and maintained in plastic splint

Physical therapy in the form of active exercises is begun and increased gradually.

If bleeding occurs during physiotherapy, it is controlled by intravenous administration of antihemophilic factor.

Forceful manipulation of a joint under general anesthesia is not recommended.

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Subacute and Chronic Hemophilic Arthropathy

August 2, 2008 By Leave a Comment

Subacute Hemophilic Arthropathy

Subacute Hemophilic Arthropathy occurs when repeated episodes of bleeding into a joint occur in a relatively short time. This results in synovial hypertrophy and persistent effusion. Subacute Hemophilic Arthropathy is best managed by immobilization of the joint, factor replacement and followed by physical therapy.

Most of subacute hemarthroses resolve over a period of three to four weeks.

If the subacute hemarthrosis fails to respond to three weeks of partial immobilization, physical therapy, and factor replacement, prednisone or a similar steroid is given for one week in the appropriate dosage.

Prolonged immobilization of the affected joint should be avoided, as this results in marked muscle atrophy and restriction of joint motion.

Support of the lower limb in orthotic devices is indicated when mechanical insufficiency of the lower limb predisposes the child to fall and sustain repeated injury.

Chronic Hemophilic Arthropathy

This condition results after repeated acute hemophilic arhropathy. Repeated acute episodes result in intra-articular fibrosis, cartilage destruction and joint stiffness.This condition is preventable in most cases by effective and immediate treatment of acute hemarthrosis.

In the management of chronic hemophilic arthropathy, physical therapy, traction ,orthoses, traction and surgery play an important role. Treatment needs to be individualized. The objective is to correct joint deformity and to restore function.

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Filed Under: Arthritis Tagged With: , , , ,
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