Guide to Pediatric External Fixators

Guide to Pediatric External Fixators

Pediatric Education Committee Contributors Lisa Ipp, MD H. Susan Cha, MD Stephanie Perlman, MD Roger Widmann, MD Lucia Fabrizio, MSN, RN, CPNP Anna Givant, RN Cindy MacDonald, RN Lorraine Montuori, LCSW Jennifer Crane, PT/DPT Michelle Patterson, OTR/L Maureen Suhr, PT/DPT Members of the Patient Education Council ©2009 Hospital for Special Surgery

GUIDE TO PEDIATRIC EXTERNAL FIXATORS This booklet is designed to provide patients with information about the external fixator, and to help parents care for their child at home. During the hospital stay, much of the care will be demonstrated and reviewed in preparation for going home. This guide is intended to supplement the information that the doctors and nurses provide.

Please take the time to read through this booklet so that any questions you may still have can be answered before you leave the hospital. Upon discharge, we will provide information on where to call with questions that may arise during the recovery process.

CONTENTS What is an External Fixator?

Diagnosis Symptoms Treatment Options Non-Surgical Surgical Planning For Your Surgery Post-Operative Course: A Step-by-Step Guide for Patients In Hospital Lengthening or Adjustments Phase (at home) Consolidation Phase (at home) Frame Removal (in hospital) Post-Removal Phase (at home) Post-Operative Instructions: Removal of External Fixator Protection Rehabilitation Pain Complications Physical Therapy Following External Fixator Placement Emergency Checklist

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What is an External Fixator?

An external fixator (Ex Fix) is a device that is surgically attached to the bone from the outside using metal pins, wires, or a combination of both.

It can be a scaffold-like frame that goes around the limb, or it can be a monolateral (one-sided) fixator (see figures 1A and 1B on page 7).

External fixators may be used to correct, equalize, or reshape limb length discrepancies (LLD), limb deformities, and certain types of fractures when a cast cannot be applied. A limb length discrepancy is a difference between the lengths of the upper arms and/or lower arms, or a difference between the lengths of the thighs and/or lower legs (when one is shorter than the other). The Ex Fix allows your surgeon to have much more effective control of the bone alignment during healing; it also allows for the skin to be aerated (exposed to air) and to be bathed daily. A cast does not allow you to shower as easily.

Limb lengthening is a surgical procedure that uses an external fixator to make one limb equal in length to the other (arm or leg). This guide will focus on external fixator use for the lower extremity.

There are many possible causes of LLD: • Previous injury: A previously broken bone may cause LLD if it healed in a shortened or bent position. This can happen if the bone was broken in many pieces (comminuted), or if the skin and muscle tissue around the bone were severely injured and the bone was exposed (open fracture). In children, broken bones may grow faster for several years after healing. This causes the injured bone to become longer. A break in a child’s bone through the growth center (located near the ends of the bone) can cause slower or uneven growth. Bones can heal in a shortened and deformed position (malunion) or sometimes even remain unhealed (non-union).

• Bone infection: Bone infections in growing children, especially infants, may cause significant LLD. • Bone diseases (dysplasias): These may include neurofibromatosis, multiple hereditary exostoses (formations of new bone) and Ollier disease.

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• Idiopathic (cause unknown): Sometimes no cause for an unequal limb can be determined using current diagnostic methods such as physical exam or x-ray.

Sometimes certain conditions are present at birth, but the LLD may not be detectable. As the child grows, the LLD increases and becomes more noticeable. Examples include: • Hemimelia (one sided undergrowth): Underdevelopment of the inner or outer side of the leg is called hemimelia. This is a condition in which one side of the body seems to grow faster than the other. It may be related to cancer of the kidney or liver, Wilms Tumor and/or Beckwith Wiedemann syndrome. One of the two bones between the knee and ankle (tibia or fibula) is abnormally short. There may also be associated foot and knee abnormalities.

• Hemihypertrophy (one-sided overgrowth): Stimulation of growth of one side of the body from an unknown cause is called hemihypertrophy. It is a rare condition. Hemihypertrophy causes overgrowth of both the arm and leg on the same side of the body.

There also may be differences between the two sides of the face.

• Short stature: In patients with skeletal dysplasia, short stature (height) can be very disabling.

Diagnosis Doctors can measure LLD during a physical examination. The doctor may measure the difference between the: • Levels of each side of the pelvis when standing • Lengths from the hips to the ankles If a more precise measurement is needed, the doctor may request an x-ray to measure the length of the bones. In growing children, the physical examination and x-ray may be repeated every six months to one year. This can determine if the LLD has increased or stayed the same.

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Symptoms The effects of LLD are different from patient to patient. Symptoms depend upon the cause of the discrepancy and the size of the difference.

Differences of 3.5-4% of the total length of the lower extremity (1 2/3 inch or 4 cm in an average adult), including the thigh, lower leg, and foot, may cause noticeable abnormalities while walking. The patient may need to use considerably more effort to walk.

A limb length discrepancy may be detected on a screening examination for a curve of the spine (scoliosis); however, LLD does not cause scoliosis.

TREATMENT OPTIONS Non-Surgical Patients with minor LLDs without deformity may not need surgical treatment. Patients with LLD of less than one inch, may benefit from a prescribed shoe lift. A lift fitted to the shoe can often improve walking and running, as well as relieve any back pain that may be caused by the LLD. Shoe lifts are inexpensive and can be removed if they do not help.

Surgical Shortening (Epiphysiodesis) In growing children, legs can be made equal or nearly equal in length with a relatively simple surgical procedure. This procedure slows down the growth of the longer leg at one or two growth sites. Your physician can tell you how much the procedure will be able to make your legs the same length. The procedure is performed under x-ray control through very small incisions in the knee area. The shortening procedure will not cause an immediate correction in length. Instead, the LLD will gradually decrease as the opposite extremity continues to grow and “catch up.�

Gradual Lengthening (External Fixation) In gradual lengthening, the surgeon attaches a scaffold-like frame (external fixator) to the bone with metal pins, wires, or both

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(Figures 1A and 1B). The surgeon gently taps the portion of the bone where the lengthening will occur. By tapping the bone, a split will be created in a circular fashion around the outer (cortex) layer of the bone. At this time, your surgeon will ensure Gradual Lengthening (External Fixation) that the (medullary) blood vessels andthe the In gradual lengthening, surgeon attaches a scaffold-like frame (external fixator) to the bone with metal pins, wires, or both (Figures 1A and 1B). The next layer of bone, the periosteum, remain surgeon gently taps the portion of the bone where the lengthening willLengthening occur. By tapping the bone, a split Gradual (External Fixation) will be inlengthening, a circular fashion aroundattaches the outer Increated gradual the surgeon a intact. This blood supply is vital for healing (cortex) layer of theframe bone.(external At this time, your surgeon scaffold-like fixator) to the bone with will maintain thatwires, the (medullary) blood 1A vessels metal pins, or both (Figures and and 1B). The to take place. The frame willthe next be worn until layer of bone, the periosteum, surgeon gently taps the portion ofremains the boneintact. where the This blood supplywill is vital for healing to take lengthening occur.

By tapping the place. bone, a split will be created in a circular fashion around the outer the gradual lengthening occurs and correct (cortex) layer of the the frame bone. until At this your surgeon The patient will wear thetime, gradual will maintain the (medullary) vessels and lengthening occurs that and correct length isblood achieved. the next ofmember bone, thewill periosteum, remains patient or layer family turn an affixed dialintact. length is achieved. You or a The caregiver will blood supply is vital healingtension. to take The place. on theThis frame several times dailyfor to create tension allows the crack and the gap in the bone to turn an affixed dial on the frame several “spread.”

This process is called “distraction.”

The The patient will wear the frame until the gradual surgeon determines the rate turninglength by taking Xlengthening occurs andofcorrect is achieved. rays every 10 to 14 during office visits.

The patient or days family member will turn Although an affixed dial times daily to create tension.

The tension this lengthening process often daily calledto"stretching," on the frame severalistimes create tension. The the bone is notallows actually thethe very tension the stretched. crack and Instead, the gap in bone to amount ofThis tension that the frame“distraction.” exerts on the The “spread.” process is called allows the crack and the gapsmall in the bone to bone stimulates the bone to bone and fill in Xsurgeon determines thegrow rate new of turning by taking to therays gradually gap.during The surrounding every enlarging 10 to 14 days office visits. Although Figure 1A “spread.” This process is called “distraction.” muscles, skin, process and blood vessels also "stretching," grow. this nerves, lengthening is often called A circular external fixator with wires and The maximum lengthening in children is the very the bone israte notofactually stretched.

Instead, pins 1 mm per day, or 1 inch perthe month. may be small amount of tension that frameItexerts on the to affix the bones to the fixator The surgeon determines theusually rate of turning slowerbone in a stimulates bone that was previously injured or and fill in the bone to grow new bone operated on.gradually enlarging gap. The surrounding to the nerves, skin, and blood vessels also grow. by taking x-rays every 10 to After 14muscles, days during The maximum rate of lengthening is the bone is lengthened, it must healininchildren the usuallyposition.

1 mm per day, or 1 inch month. It may be lengthened This is called the per consolidation office visits. Although this lengthening process in aframe bone that was previously injured or phase.slower Then the is removed.

Under ideal operated conditions, the on. time "in the frame" is approximately to 3 months to complete both the lengthening and is often called “stretching,” 2.5 the bone is not consolidation time varies depending After thephases. bone isThis lengthened, it must heal in upon the the patient’s age, position. general health participation in lengthened This isand called the consolidation etc. the Some activities may beUnder moreideal phase.

Then frame is removed. actually stretched. Instead, rehabilitation, the very small difficult when wearing a frame getting in and out conditions, the time "in the(i.e., frame" is approximately of a car).

Most patients easily both returnthe tolengthening school and and 2.5 to 3 months tocan complete amount of tension that the frame on daily consolidation routines.exerts Whenphases. the surgeon determines that bone upon This time varies depending strength nearly normal again,health the frame, pins, and in theispatient’s age, general and participation wiresto are removed.

Gradual lengthening can achieve rehabilitation, etc. Some activities may be more the bone stimulates the bone grow new large difficult gains in length if the process is repeated several when wearing a frame (i.e., getting in and out years of later, or ifMost it is performed opposite ends the and a car). patients canateasily return to of school same daily boneenlarging at the same time.the This "double level routines.

When surgeon determines that bone bone and fill in to the gradually gap. lengthening" rates strengthachieves is nearlylengthening normal again, thegreater frame,than pins,1and mm per day.are Deformities, such as lengthening malunion (incorrect wires removed. Gradual can achieve The surrounding muscles, nerves, and union), the gains resultskin, oflength a broken bone whichishas healedseveral large in if the process repeated in a “crooked” fashion, also be corrected with theof the years later, or if it may is performed at opposite ends use of an Ex Fix. same bone at the same time.

This "double level blood vessels also grow. The maximum rate lengthening" achieves lengthening rates greater than 1 mm per day. Deformities, such as malunion (incorrect union), the result a broken per bone which has healed of lengthening in children is usually 1ofmm Figure 1B in a “crooked” fashion, may also be corrected with the monolateral external fixator with pins to use of an Ex Fix. day, or 1 inch per month. It may be slower in a Aaffix the bones to the fixator bone that was previously injured or operated on.

After the bone is lengthened, it must heal in the lengthened position. This is called the consolidation phase. Under ideal conditions, the time “in the frame” is approximately 2.5 to 3 months to complete both the lengthening and consolidation phases. This time varies depending upon the patient’s age, general health, and participation in rehabilitation, etc. Some activities may be more difficult when wearing a frame (i.e., getting in and out of a car). Most patients can easily return to school and daily routines.

When the surgeon determines that bone strength is nearly normal again, the frame, pins, and wires are removed. Gradual lengthening can achieve large gains in length if the process is repeated several years later, or if 7

it is performed at opposite ends of the same bone at the same time. This “double level lengthening� achieves lengthening rates greater than 1 mm per day.

PLANNING FOR YOUR SURGERY 1.

2.

Scheduling: Plan for a hospital stay of two to four days.

Clothing: Make arrangements to wear loose clothing that will fit over the frame. You can purchase pants with snaps down the sides of each leg from most sporting goods stores.

Surgery: Application of the External Fixator The surgery involves the application of the frame, which is attached to the limb through pins and wires. The external fixator is applied to the limb in the operating room while you are under epidural or general anesthesia and cannot feel the procedure. Your doctor will tell you how long he/ she thinks the procedure will take; it typically lasts 1-3 hours. During the surgery, family members may wait in the Family Atrium, located on the 4th floor.

Preparing to Care for Your External Fixator at Home During the recovery process, patients and their families will learn how to care for the external fixator. We will teach you what you need to know about pin care, and how to check for problems that would require calling the doctor. The nurses will demonstrate the care, and pin care will begin in the hospital so that there is time to become comfortable with the procedure before going home. If the fixator was applied for limb lengthening, one of the team members will demonstrate how to do the frame adjustments and will provide a schedule of when to do them. Frame adjustments may be done as often as 3-4 times per day. It is important to closely follow the surgeon’s instructions for frame adjustments.

We encourage other caregivers, such as family members or babysitters, to read through this manual and/or come to the hospital to learn about pin care from our staff to ensure proper care from all of whom may be involved in caring for the patient at home.

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Here is a list of the things that will be needed at home: • Contact card with important and emergency phone numbers • Pillows for positioning and comfort • Sterile saline (salt water solution for washing) • Hydrogen peroxide • Sterile cotton-tipped applicators • Xeroform gauze (yellow sticky gauze) • Bactroban ointment (requires a prescription) • Pain medication (requires a prescription)

POST-OPERATIVE COURSE: A STEP-BY-STEP GUIDE FOR PATIENTS 1. In Hospital Next Day After Surgery • You will be taught the proper position of the leg while in bed to avoid joint contractures (permanent shortening of muscles or tendons).

• You may get a foot splint to support your foot (leg frames).

• You may get a CPM (Continuous Passive Motion) machine to prevent stiffness of the knee.

• You will start your training with a physical therapist and learn how to walk and exercise with the frame. It is important that you put weight on the operated leg (the frame will protect and support your leg).

• Pain control is managed by a special team at HSS. You will have patient-controlled analgesia (PCA), a pain medication pump, so that you will be able to adjust your own dose of pain medicine. This is delivered either via an intravenous (IV) or epidural catheter (tube).

• You will be seen in the hospital by a variety of healthcare professionals, including: your orthopedic surgeon, a specialist assistant, orthopedic residents, physician assistants, medical doctors, nurses, therapists, and social workers or case managers for discharge planning.

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Second Day After Surgery • All of your dressings (bandages) will be removed.

• Pin care will be started; you will be taught the following pin care routine by the nursing staff: 1. Clean pin sites with an equal part mixture of sterile saline and hydrogen peroxide using sterile cotton tipped applicator. Use a new cotton tip for each pin site. Pin care is performed routinely once per day.

2. Apply bactroban to the area around the pin site. Some patients find that applying the bactroban around the pin site keeps the area from “drying out.” Bactroban ointment is used typically for two weeks after surgery before it is discontinued.

3. Cover the pin sites with Xeroform gauze. You can order special Xeroform gauze that fits around your pins from Integrity Medical 888.906.8196 or 866.463.5632. Xeroform gauze can also be ordered via the Internet at WoundCareShop.com with a twoday delivery time.

4. Wrap 2” white gauze bandage around pin sites. White rolls of gauze bandage are available at most drugstores.

Discharge from the Hospital • Prescriptions for pain medications, antibiotics, and physical therapy will be provided. Call your doctor immediately if there are signs of a pin infection. Signs of infection include increased redness or drainage at the pin site, increased pain, fever, or chills.

• Avoid agents that slow bone growth such as smoking and nicotine, as well as Non-Steroidal Anti-inflammatory Drugs (NSAIDs) such as: Motrin, Aleve, aspirin and Naprosyn.

• Eat a well-balanced diet that includes protein and calcium to encourage bone growth. Daily calcium supplementation is recommended. Check with your doctor or nutritionist for the recommended daily dosage, as it varies with age.

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• You may start to shower approximately one week after your surgery if your surgeon gives you permission to do so. Remove all bandages; you do not need to cover the frame. Use an anti-bacterial soap.

• If your surgeon allows, you may be able to swim in a chlorinated pool; this is a great way to keep your pin sites clean and healthy.

Avoid hot tubs, bath tubs, lakes, and ocean swimming.

2. Lengthening or Adjustments Phase (at home) Begins approximately 7 to 10 days after surgery and continues until the length or full correction is achieved.

• One of the team members will teach you how to do adjustments of the frame and give you a schedule. Frame adjustments are typically performed 3-4 times per day.

• You will continue with the pin care and physical therapy sessions.

The most important task for you during this phase is to follow the adjustments schedule and prevent joints from getting stiff. While you may need to elevate the leg to prevent swelling, walking and putting weight on the leg is encouraged, as long as it is not painful.

• Physical therapy to maintain mobility and prevent stiffness of the knee and ankle is done 3-5 times per week. Physical therapy can be done at a local facility, or you may find a physical therapist that comes to your home.

3. Consolidation Phase (at home) Allows new bone to mature and become strong. During this phase, the more you walk and put full weight on the operated leg, the faster the bone will heal.

4. Frame Removal (in hospital) Removal of the frame is done in the operating room under sedation.

The procedure is approximately 15 minutes, after which a cast or a brace may be applied to the leg for protection of the new bone.

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5. Post-Removal Phase (at home) Requires slow and gradual return to full weight bearing, usually within 4 weeks.

Post-Operative Instructions: Removal of External Fixator 1. Protection Your bone has just lost the support of your external frame, and now it must be protected. You must “take a step back” and limit your weight bearing to 50% of body weight. Use the crutches at all times. This protective period typically lasts 2 weeks.

You may be in a cast or a brace during waking hours. You may remove your dressing in 2 days. At that time, you may shower and wet the wounds. Do not soak the wounds under water - no baths, no pools, no lakes, and no ocean. Use Band-Aids® if needed to cover any draining wounds. Wounds should not drain for more than 3 days.

2. Rehabilitation Continue to be active and maintain some independence. Try to achieve a good balance, do not push yourself too hard, but do not lie in bed all day either. If you have a brace, then remove it for gentle joint motion exercises. Formal physical therapy will start after your post-operative office visit.

3. Pain Many patients ask about the amount of pain associated with limb lengthening. There is some discomfort associated with any surgery.

Pain medicine is given as needed while the patient is in the hospital (usually 2 to 3 days). The doctor will also prescribe pain medicine as needed when you leave the hospital (for example: Tylenol with codeine, vicodin, or percocet). Low levels of pain are expected once the patient is home and the lengthening process is underway. If there is a sudden increase in pain, contact your surgeon immediately. Pain may be a warning sign of a possible problem and should be brought to your doctor’s attention quickly.

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4. Complications • The bone may heal too rapidly (premature consolidation) and need to be gently adjusted again to continue the lengthening process.

• The bone may heal too slowly (delayed union). This can require that you wear the fixator for extra time, use an external bone stimulator or undergo more surgery, such as insertion of a bone graft.

• The pins or wire sites can become infected. If untreated, infection can spread to the bone. To minimize this risk, the staff will provide detailed instructions on how the patient and family should carefully clean the pins and wires.

• Joint stiffness (contractures) may occur during lengthening. This is especially true for longer lengthenings. If joint stiffness happens, the lengthening may need to be stopped or further surgery may be needed. Participation in prescribed physical therapy and home exercises will minimize the chances of joint problems.

• Fractures of the new bone may occur when the external fixator is removed. Initially, the new bone is not as strong as the original bone. If the new bone breaks, the surgeon may apply a cast, reapply the fixator, or restrict the patient’s physical activities.

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PHYSICAL THERAPY FOLLOWING EXTERNAL FIXATOR PLACEMENT

Rehabilitation involving physical therapy usually follows external fixator placement. At first, your joints may feel stiff and sore after being placed in an external fixator. Your therapist will help you move your joints to improve your circulation, reduce swelling, improve movement, and ease discomfort. You will learn exercises to regain movement, strength, flexibility, coordination, and function of your joints. Your physical therapist can also make recommendations on how to protect your joints during your daily activities.

Throughout recovery, physical therapy plays an important role in keeping your joints flexible and in maintaining muscle strength. To speed up bone healing, gradual weight-bearing is encouraged. Your therapist will help you learn how to walk with a walker or crutches while you are in the hospital. You will also learn how to go up and down stairs either using crutches or by bumping up and down the stairs on your bottom.

A difference in leg length can cause the pelvis and spine to tilt and bend.

This can affect the way the muscles work and can make moving difficult.

Physical therapy will help strengthen weak muscles and stretch tight muscles. Physical therapy is hard work and will take a lot of time. Sometimes it will hurt, but it is important to do your exercises. Here is a list of commonly taught exercises to help you recover after placement of your external fixator.

While you are in the hospital, your physical therapist will teach you many of these exercises to keep your hip, knee, and ankle moving.

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1. Passive Positioning in Knee Extension Proper positioning is crucial. Place your ankle on a pillow, allowing your knee to straighten. Rest with your knee in this position for 10-20 minutes, three times per day.

2. Ankle Pumps and Circles

Move your foot and ankle up and down, left and right, and in a circle.

Repeat 10 times in each direction, every hour.

3. Active-Assisted Knee Flexion

Sit on the edge of a bed or chair. Have someone help you bend your knee as much as possible. Hold for 30 seconds and repeat 5 times, every hour.

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4. Knee Extension Place a towel roll under your knee. Use your muscles to push your knee down into the towel and hold for 5 seconds.

Repeat 10 times, every hour.

After your discharge home, the physical therapist may add many or all of the following exercises to strengthen your legs. It is imperative to continue to perform range of motion exercises following your discharge from the hospital.

1. Knee Extension Sit on the edge of a bed or a chair.

Straighten your leg as much as possible.

Repeat 10 times, three times per day.

2. Straight Leg Raise While lying on your back, keep your knee straight and lift your leg up.

Repeat 10 times, three times per day.

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3. Hip Extension While lying on your stomach, keep your knee straight and lift your leg up.

Repeat 10 times, three times per day.

4. Hip Abduction While lying on your side, keep your knee straight and lift your leg up.

Repeat 10 times, three times per day.

5. Knee Flexion

While lying on your back, bend your knee while sliding your heel along the bed. Repeat 10 times, three times per day.

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Please do not hesitate to contact your physical therapist with any questions or concerns at 212.606.1368.

We hope this information has been helpful. The health care team is here to answer your questions, and we encourage you to speak with us about any questions or concerns you may have.

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EMERGENCY CHECKLIST

CALL YOUR DOCTOR OR NURSE IMMEDIATELY IF ANY OF THE FOLLOWING OCCURS: • • • •

Sudden increase in pain Fever Blue discoloration of the affected limb Redness, swelling, drainage, or pain at a pin site

IMPORTANT PHONE NUMBERS Child’s Name HSS Doctor’s Name Number Pediatrician’s Name Number Emergency Contacts Name Number Name Number 19

Hospital for Special Surgery is an affiliate Weill Cornell Medical College.

535 East 70th Street New York, NY 10021 tel 212.606.1000 www.hss.edu