Surgeons and Patients Don’t Always Agree on Results of Knee Surgery

Young athletes (children and teens) who have stabilization surgery for repeated dislocations of the patella (knee cap) have a 93 per cent success rate following the procedure. “Success” means their pain is less and their function is better. Yet when surveyed, these same patients report a much lower subjective (opinion) level of satisfaction. This study is another one to show a “disconnect” between surgical success and patient’s perception of the results.

Let’s take a closer look. One surgeon performed a patellar realignment procedure on 27 knees. The 24 children and teens in the study were between the ages of eight and 18. They all had at least two (but often more than two) patellar dislocations or subluxations (partial dislocation). They did not respond to conservative (nonoperative) care, which is the usual first-line of treatment for this problem.

The stabilization procedure varies from patient-to-patient. Age, skeletal maturity, and etiology (cause) are important factors in the decision-making process. The surgeon also performs an arthroscopic exam before doing surgery in order to find out what the patellofemoral joint looks like inside. Any unknown or previously unseen problems with the soft tissue structures and joint surface are identified.

The surgeon may perform a lateral release (cuts the soft tissue along the outside edge of the patella) with or without retensioning the medial soft tissue (changing the tension on the other side of the patella closest to the other knee). Other options include an osteotomy (the surgeon removes a wedge of bone to change knee alignment), repair of any damaged patellar ligaments, or a patellar tendon transfer (changes the angle of pull on the patella).

The work may be done from above or below the knee to create the stability needed based on the cause of the problem. In some cases, the patella is reshaped by doing a patellar shaving procedure. Any loose pieces of bone or cartilage found in the joint are removed as well. Most of the children (78 per cent) had more than one thing done during the same surgery — referred to as combined procedures.

Why did patients with an apparent “successful” surgery still report dissatisfaction with the results? Could it be the measures used (standard tests of knee function, symptoms, and activity levels) don’t fit this group? The authors suggest that microscopic damage to the joint and osteoarthritic changes might have something to do with patients’ sense that the knee isn’t stable.

They did find that the younger age groups and patients who had the surgery sooner than later had better outcomes. Those particular outcomes have led the surgeon to recommend patellofemoral reconstruction after only 2 subluxation or dislocation episodes — rather than waiting until the child has had many more than that months to years after the first episode.

There was one other observation that surprised the surgeon: children who had subluxations (partial dislocation) rather than full dislocations actually had worse results. Logically, it would seem than a milder injury would lend itself toward a better final outcome. But that wasn’t the case. So, why not?

The answer is not clear but there are some possible ideas. Perhaps the milder symptoms that accompany subluxations results in more frequent episodes that the patient fails to remember and report. Recovery is often faster with subluxations but damage at the microscopic level may be worse than realized.

And this all ties in with why patients don’t necessarily come back to the surgeon and report dissatisfaction. But when asked by telephone survey later, they do report more problems than the surgeon realized when counting up the number of “successful” procedures. Dislocations don’t occur but mechanical symptoms (e.g., pain with movement) are present and activity level is less than expected.

In summary, the study presented here confirms findings from other studies: patients do not always view their surgical results as successful. But they do not return to the surgeon and report ongoing symptoms or problems. When knee assessment tools like the International Knee Documentation Committee (IKDC) or the Lysholm score are used to measure outcomes, the results look good on paper but do not always provide an accurate view of the patient’s response. More studies are needed to understand all the reasons for less than optimal outcomes reported following patellar realignment surgery.

Is Bracing for Scoliosis Effective?

Bracing continues to be used for children and teens with adolescent idiopathic scoliosis (AIS) but does it work? What’s the evidence for brace treatment and who should it be used with? In this review article, Dr. Paul D. Sponseller from Johns Hopkins University Department of Orthopaedic Surgery brings us up-to-date on the practice of bracing for AIS.

Dr. Sponseller presents both the results of various studies published in the past 25 years on this topic as well as current opinions on the use of bracing from orthopedic surgeons using this treatment tool. Adolescent idiopathic scoliosis refers to a condition of spinal curvature and deformity in children and teens that has no apparent cause.

The word “idiopathic” means “cause unknown”. Another word for the underlying cause of a disease or condition is etiology. There are many theories about the etiology of adolescent idiopathic scoliosis (AIS) but no clear single cause. Most experts consider AIS to have multiple linked causes including genetics, environment and lifestyle, and nervous system dysfunction with biologic and hormonal influences.

Treatment specific to the cause is usually the most effective approach. Without that, the symptoms become the focus and that’s where bracing comes in. The idea is that by placing the spine in an upright position, the forces causing the curvature can be stopped — or at least slowed. Has that ever been the case? How well does it really work?

A review of all the studies done on this topic doesn’t really answer the questions. There are many different study designs so comparing one study to another is like comparing apples to oranges. And there are many different types and styles of braces with the same problem comparing results.

What we do know is that bracing seems to work the best when the brace is worn 16 or more hours a day and that girls with smaller curves (25 to 35 degrees) have the best results. Comparing children who are braced with children who do not wear a brace seems to offer some consistent evidence that observation alone (no bracing) isn’t as effective as bracing. Long-term results (what happens five-to-10 years later) in both groups are unknown.

Waiting too long before using bracing may be a factor. Studies that show a 50 to 60 per cent success rate still leave 40 to 50 per cent of patients turning to surgery for correction. That leads researchers looking for reasons why some patients have a successful outcome in hopes of selecting patients in the future who would be good candidates for bracing.

Before we look at how surgeons decide to use bracing, it should be pointed out that when asked why surgeons use bracing without convincing evidence that it works, there is agreement that the chance to reduce the risk of needing surgery is worth the effort.

Bracing may not improve (decrease) the curve but it appears to keep the curve from progressing (getting worse) in many cases. In 25 per cent of cases, bracing does seem to reduce the risk of surgery. Most likely what is happening is the brace alters the effect of growth on the curve. Parents and even children jump at the chance to be able to do something (anything!) that might create a straight spine. Fear of deformity, pain, and surgery often leads to bracing as a nonoperative option.

That brings us back to the question of when to use bracing. Right now, based on the best evidence available, it looks like bracing should be offered to patients with curves between 25 and 45 degrees. They should be in a phase of rapid bone growth (based on X-rays). Larger curves in children with more mature skeletal growth can be offered bracing but with the warning that there is less chance to really change things.

Patients who are not likely to benefit from bracing include children who are overweight, those who have reached skeletal maturity (no further growth expected), and anyone with a high thoracic curve (above T8). Most girls have reached their peak growth rate around 11 to 12 years old. Boys are a little later at 13 to 14 years old. Curve progression is likely to be the greatest during these years. This requires close monitoring even when wearing a brace.

Other risk factors that predict no change with bracing include 1) the presence of other major health problems that could interfere with wearing the brace, 2) children who do not want to wear a brace, or 3) parents who do not accept the idea of bracing.

Once the decision has been made to use bracing, the next natural question is: which one or what kind? The brace maker (called an orthotist because orthosis is the more modern name for brace) helps guide the decision. This is done in communication with the parent, surgeon, and physical therapist. A team approach is best when looking at the whole child and taking into consideration spine, surrounding soft tissues, general health, and activity level.

Some of the more commonly used braces for adolescent idiopathic scoliosis include the Boston brace, the Wilmington brace, the SpineCor brace, the Milwaukee brace, the Triac brace, the Sforzesco brace, the Charleston brace, the Providence brace, and the Cheneau brace. Does that give you any idea of how many different ways there are to approach this problem?

Some of these braces are intended to derotate the vertebrae. Others force the spine to bend in the opposite direction of the developing curve. The Charleston bending orthosis provides this type of overcorrection and is worn only at night. Most of today’s braces are made of plastic with either metal uprights or velcro or canvas straps to hold them in place. With the exception of the braces intended only for night-use, most braces used for adolescent idiopathic scoliosis are designed for use 23 of each 24 hours. The brace is removed only for bathing, swimming, and dressing.

One of the first questions often asked when getting a brace is “How long do I have to wear this?” Braces are meant to be worn primarily during the growing phases of childhood and adolescence. Once skeletal maturity has been reached (confirmed by X-ray), the brace can be removed. All girls should be at least 2 years past the point of beginning their menstrual cycle (period). The brace can be stopped gradually or all at once. It doesn’t seem to matter but each child is monitored closely to make sure the curve doesn’t start getting worse again.

Dr. Sponseller concludes by reminding us that there is some evidence that bracing can be effective. But it is unpredictable as to how much or who might benefit the most (or at all). There is a great need for more research and especially a way to determine which patients are at risk for surgery but could benefit from bracing.

Hip Replacement After Slipped Capital Femoral Epiphysis

Slipped capital femoral epiphysis (SCFE) is a condition that affects the hip most often in teenagers between the ages of 12 and 16. Cases have been reported as early as age nine years old. In this condition, the growth center of the hip (the capital femoral epiphysis) actually slips backwards on the top of the femur (the thighbone).

Left untreated, this can lead to serious problems in the hip joint later in life. Severity of slipped capital femoral epiphysis (SCFE) can be rated as mild, moderate, or severe. This grading is determined by looking on X-rays at the angle of the epiphysis compared to the other side (if the other hip is normal and not also affected by SCFE).

Another way to classify severity of the condition is by assessing joint stability. Children who can put weight on the hip and walk (sometimes despite pain and/or with or without crutches) are considered to have a stable SCFE. Children with pain so severe that weight bearing and walking are impossible (even with crutches) are considered to have unstable SCFE.

Studies have shown that the more severe the slip, the worse the long-term outcomes. The earlier the diagnosis is made, the more effective the treatment. Fortunately, the condition can be treated and the complications avoided or reduced if recognized early.

Surgery is usually necessary to stabilize the hip and prevent the situation from getting worse. Even with treatment, abnormal joint shape and the resulting impingement (soft tissues structures get pinched) can lead to joint degeneration.

The question has been raised: how many children with SCFE end up with a hip replacement because of severe joint destruction? Is it from degenerative arthritis or avascular necrosis (loss of blood to the hip from the disease)? How soon is hip replacement needed? How long does the joint replacement last?

To help answer these questions, surgeons from the Mayo Clinic in Rochester, Minnesota conducted a review of their medical records from 1954 up to 2007. There were 33,000 patients who had hip replacements during that time period. Only 38 were done in hips with degenerative changes or impingement linked with slipped capital femoral epiphysis (SCFE).

A closer look at these patients showed that a severe slip with avascular necrosis was linked most often with the need for joint replacement. There were some patients with SCFE who needed a joint replacement because of impingement rather than degeneration, but the majority were for damage done by the necrosis.

Hip replacement for necrosis occurred early on (mean time was 7.6 years) compared with a delay of over 20 years for patients with joint degeneration or impingement. And the rate of implant failure requiring revision (a second) surgery was fairly high in the necrosis group as well. The reason for implant failure was usually cup loosening or femoral neck fractures. Success of implant revision was good though — 95 per cent of the implants were still in good shape five years later.

The authors say this was the first study of its kind — to show the actual rate of hip replacements in patients who had slipped capital femoral epiphysis (SCFE) as a child. Many of these patients received all of their care over the years at this Mayo Clinic.

What they uncovered with the study was the understanding that hip replacement following a diagnosis of SCFE occurred most often because of hip necrosis not degenerative hip arthritis. Future treatment should be focused on preventing avascular necrosis in severely slipped, unstable hips.

Efforts have already been made to find the best way to accomplish this. Some surgeons have tried cutting the joint capsule while others have performed a stabilization procedure called open reduction and internal fixation (ORIF). Only small studies have been reported so the results are still inconclusive.

The one variable that was difficult to factor into this study was the many changes that have occurred in the way slipped capital femoral ephiphysis has been treated over the years. Implant design and surgical techniques have also changed over the many years between 1954 and 2007.

A closer analysis of these changes might provide additional helpful information for consideration in future cases of slipped capital femoral epiphysis in need of hip replacement. For now, it’s clear that patients with unstable slips that develop necrosis are the ones most likely to need a hip replacement (and often sooner than later).

Setting Parents’ Worries to Rest About Elbow Fractures in Children

When a child fractures a bone and treatment is delayed, parents may worry excessively about the effects of that delayed treatment. Sometimes there just isn’t an orthopedic surgeon available to evaluate and treat that child. The family may have to travel to another hospital or clinic where a physician is on-site. In other situations, the hospital’s operating rooms are full. That means another delay in getting proper care for the fracture.

In this study from the UCLA Orthopaedic Hospital in California, surgeons compared two groups of children with supracondylar humeral fractures (SCHFs). One group had surgery soon after the fracture occurred. The other group was delayed in receiving surgical care. Everyone in both groups had a procedure called closed reduction and percutaneous pinning.

A supracondylar humeral fracture refers to a break just above the elbow at the bottom end of the humerus (upper arm bone). As many as 60 per cent of all elbow fractures in children occur at this location. In order to collect information on the effect of on-time versus delayed treatment, this study was done prospectively. That means the study was set up first, then each child coming into the urgent care center of this hospital with this type of fracture was evaluated.

One advantage of a prospective study over a retrospective study (looking back after the event) is the fact that information collected on each patient is the same. Consistency like this makes it possible to make direct comparisons between the two groups. In this study, data collected included time between injury/fracture and treatment, amount of time in the operating room, and length of hospitalization.

Of greater interest was how many patients developed problems after surgery. The question was raised: does a delay in treatment (pinning the fracture) result in more problems or more severe complications after surgery? The type of problems looked at included loss of blood supply to the bone, fracture healing (malunion, nonunion, delayed union), and infection. Other measurements reflective of the results included elbow range-of-motion, loss of fixation, and final carrying angle of the elbow.

The 144 children who came to the UCLA urgent care center with supracondylar humeral fractures could easily be divided into immediate care and delayed care based on the time between admission to the hospital and the start of surgery. Time between the injury and presentation at the clinic was not used because they didn’t have this information for everyone in the study.

Patients who transferred to the urgent care center from some other hospital, clinic, or facility were in the delayed treatment group. The time delay was at least 21 hours. Patients who came directly to the urgent care center and received evaluation and treatment within eight hours were in the direct treatment group. Since it is not possible to intentionally delay surgery, this was the best way to compare early with delayed treatment for this type of pediatric elbow fracture.

In all cases in this study, the fractures were displaced (separated). Treatment was with closed reduction and fixation. Closed reduction means no incision was needed — the surgeon could use traction to pull the bones apart and line them back up). Fixation refers to pins used to hold the bones together until the fracture healed.

So, what did they find out? Well, basically, that there was no difference in results eight weeks after treatment between the two groups. A delay in treatment for supracondylar humeral fractures is not cause for extreme alarm. The children should be monitored carefully, of course. Any sign of blood loss or nerve damage would be a red flag warning.

But delaying pinning of the fracture 21 hours or more after arriving at the urgent care center was safe and did not mean worse results. Patients (and their families) in the delayed group were just as satisfied with the final outcomes as those in the direct treatment group.

Of course, delays are always to be avoided whenever possible and each child should have treatment based on his or her individual needs. Any child with displaced supracondylar humeral fractures having extreme pain, loss of blood flow, and/or loss of sensation must be treated absolutely as soon as possible. Delayed treatment is not acceptable in this group.

Good News For Children with Complex Regional Pain Syndrome

Children with a condition called complex regional pain syndrome or CRPS often suffer intense pain and swelling of the affected arm and hand or leg and foot. They often experience skin changes (color, texture, hair growth, temperature). The net result is a loss of motion and function along with reduced quality of life. If the condition becomes chronic, dystrophy or deterioration of the bones and muscles in the affected body part may occur.

CRPS occurs most often after an injury as minor as having blood drawn, or a sprained ankle. Other times, it may be the result of a more significant injury such as surgery, a fracture, immobilization with casting or splinting, or in adults, as a result of a stroke.

Risk factors for developing CRPS include immobilization of the affected limb with a cast, splint or sling; smoking; genetics; and psychological factors. The problem is not understood very well. Doctors don’t really know what causes it or why it happens. That makes CRPS a difficult condition to treat effectively.

But a team of health care professionals at Denver Children’s Hospital have some good news. Using a team approach, they have developed a step-by-step plan for the treatment of CRPS in children that is having good success. They start with a review of all the ways the child has already been treated so far. Most often, medications have been prescribed and the child has worked with a physical therapist.

When previous medications (usually pain relievers and/or antiinflammatories) have not worked, a second line of drugs to try are muscle relaxants and anticonvulsants. The child goes back to physical therapy for a more aggressive approach. Failure to achieve pain relief and return of function with these measures results in a referral for a sympathetic (nerve) block.

If the nerve block works, then great! But if it only provides temporary relief from pain, at least it’s clear that the team is on the right track. Inpatient hospitalization is recommended. That’s when the multidisciplinary team gets to work.

The surgeon provides a continuous block to the nerve while the physical therapist works with the child in a total program of sensory modulation, postural alignment, desensitization, motion and movement training and strengthening (as appropriate). During this five-day intense in-patient treatment, a psychologist also offers psychological therapy and behavioral training.

What have the results been so far with this approach? Although their test group was small (37 children under the age of 18), 80 per cent (30 of the 37) were completely cured — no more pain and swelling, no more disabling symptoms.

A closer look at how each child responded as they went along showed that 10 per cent only needed a single nerve block. Another 10 per cent got better with a change in medication and return to physical therapy. That group didn’t need the nerve block. The remaining children finished the full program and gradually experienced improvement and complete resolution of symptoms.

There were two other important finding in this study. The first was the fact that children who responded to the nerve block were still in the first few months of this condition. Children who had CRPS for an average of 22 months when they got their first nerve block were less likely to have a good result. And second, three-fourths of the children were girls. Scientists may want to focus future research on understanding the reason for that. If there is a hormonal imbalance, then perhaps treatment can include some way to address this issue.

For now, the authors share their treatment approach for those who have not yet reached an understanding of the benefits of a multidisciplinary protocol for pediatric complex regional pain syndrome (CRPS).

Rare Ankle Fracture in Children

It can be difficult to advise parents or caretakers of children with traumatic fractures of the talus because this type of fracture is rare. We know how these fractures develop (the mechanism of injury). But other information on talus fractures in children is limited. Who gets these (risk factors), what’s going to happen (natural history), and what can go wrong (complications)?

The talus is located just above the calcaneus (heel bone). The talus has a bit of an odd shape with a main square-shaped body and a small extension of bone coming off the body called the talar neck. It is sandwiched between the calcaneus and the bones you feel on the top of your foot where the end of the tibia (lower leg) meets the foot. The talus is an important bone in ankle motion because it helps create the rocking motion needed for front-to-back and side-to-side movement of the ankle/foot complex. It is the link between the other major joints in the ankle.

In this study from Children’s Hospital in Boston, the records of all children who had a talus fracture over a 10-year period were reviewed. Data was gathered on age of the child at the time of injury, how the injury happened, and the exact type of fracture.

Any injuries to the surrounding soft tissues or other bones were also recorded. X-rays and treatment administered were reviewed. The final piece of important information (and really what the surgeons wanted to know) was the number and type of complications that occurred after treatment.

It turns out that many of the injuries occurred during high-impact sports. The ankle/foot was in a position of dorsiflexion (foot flat on the ground, tibia forward over the foot). Other talar fractures were caused by a fall landing on the heels/foot or as a result of a car accident. A force down through the leg too great for the strength of the bone resulted in a fracture.

Children of all ages (from one up to 18) experienced talar fractures. The most common place for a talar fracture was the talar neck. There were fractures of the talus body as well. But there were some children who had more than one area of the talus broken (neck and body). High-energy injuries were more likely to result in damage to other areas of the foot and ankle.

Treatment was with open reduction and internal fixation (ORIF) for about a third of the group. In this procedure, the surgeon makes an open incision and uses hardware such as screws or pins to hold the broken bones together until healing takes place. The majority of the children were treated with immobilization in a cast without additional surgery. Most kids were back up on their feet and engaged in all activities on an average of nine weeks. Displaced fractures (bones separated) took longer to heal than nondisplaced breaks.

What kinds of problems developed after treatment? This is the real focus of the study. Post-traumatic arthritis was the most common complication affecting 17 per cent of the group. Second to arthritis were nerve injuries and avascular necrosis (loss of blood to the bone causing death of bone tissue). Most of the nerve injuries were temporary and healed. Only one patient had residual loss of sensation.

The authors were particularly interested in the low rate of avascular necrosis because this is much more common among adults. They thought perhaps the children had fewer displaced fractures the number of cases of necrosis was lower. Perhaps the thicker periosteum (outer layer of bone) offers some protection. And, of course, supportive cartilage in and around the joint in children is more flexible allowing for more give and bend during trauma.

There were no cases of infection or problems with wound healing and only one fracture that failed to heal. A couple of children/teens needed another surgery to help stabilize the joint. A closer look at those who developed joint problems later showed that these patients had high-energy injuries and a displaced fracture. Likewise the one nonunion and all cases requiring additional surgery were displaced fractures.

In summary, talar fractures in patients under the age of 18 are more common in teens who are involved in sports or driving cars. Younger children are less likely to fracture this bone. When younger children experience a talus fracture, it is often less severe and less involved than in older children and teens. The data from this study does not point to avascular necrosis as a likely complication following treatment in this age group.

Although this is the largest study published on the topic of talus fractures, there were only 29 cases over a 10-year period of time. There is some suspicion that more of these fractures will be seen in the future as more and more children participate in high-impact sports. Having some knowledge of what to expect will help surgeons plan for, evaluate, and treat these injuries when they do occur.

New Surgical Technique to Treat Severe Perthes Hip Disease

In this study, treatment of Legg-Calvé-Perthes Disease (also known as Perthes disease) with triple pelvic osteotomy is evaluated for its effectiveness. That statement will make a lot more sense once we describe Legg-Calvé-Perthes Disease and describe what is a triple pelvic osteotomy.

Perthes disease is a condition that affects the hip in children between the ages of four and eight. The condition three names to honor the three physicians who each separately described the disease.

In this condition, the blood supply to the growth center of the hip (the capital femoral epiphysis) is disturbed, causing the bone in this area to die.

The blood supply eventually returns, and the bone heals. In the process, the joint cartilage softens, the round head of the femur flattens, and subluxation (head of the femur shifts out from inside the hip socket) occurs.

How the bone heals determines how much problem the condition will cause in later life. This condition can lead to premature hip arthritis. That’s why every effort is made to contain (hold) the hip in the socket during the necrosis and revascularization phases. Necrosis refers to the period when loss of blood to the bone results in the death of bone cells. Revascularization is the restoration of normal blood supply.

There are different ways to contain the femoral head. Two of the better known (and most often used) methods are called femoral varus osteotomy and Salter innominate osteotomy. In this study, the triple pelvic osteotomy used the bones on three sides of the femoral head (pubic bone, iliac bone, ischium) to hold the head of the humerus firmly in place.

The surgeon uses tools and instruments to cut the bone in the pelvis, shift the pieces of bone, and reshape the bones to form a holding container around the femoral head. The authors provide drawings and photos of X-rays to show how this is done.

This triple technique is really designed for the more severe cases of Perthes disease. These patients need long periods of time with the hip held in place in order to reshape and stabilize the femoral head. Wearing a cast for months on end has many problems. The hope with this triple osteotomy is to avoid a limp or leg length difference from one side to the other that often occur when only one of the other techniques (varus osteotomy, Salter osteotomy) are used.

Of course, the question remains: how effective is this triple pelvic osteotomy in the treatment of Perthes disease and especially in preventing degenerative arthritis? To find out, results were measured using X-rays, presence of a limp, and leg length differences. Joint motion and activity levels were also compared between those patients who had the triple pelvic osteotomy and those who had one of the other more traditional approaches.

The results were considered fair to good and satisfactory. Remodeling was more successful in younger children (under the age of eight). There were only a few cases where additional surgery was required. The majority of patients went from having a painful hip and limp to a pain free normal gait (walking) pattern. And there were no “poor” results.

The authors conclude that surgical containment of the femoral head in the hip socket is safe and effective in the treatment of severe Legg-Calvé-Perthes disease. Using the triple pelvic osteotomy surgery can reshape the head of the femur into a round sphere that stays in the hip socket.

The final result is a pain free, functional hip that can eliminate differences in leg length, and restore a normal walking pattern without a limp. Surgeons must be careful not to overdo it — too much containment can cause a painful impingement (pinching) problem. But even with this problem, the surgeon can go back in and trim the rim around the hip socket to take care of the impingement.

Review and Update on a Childhood Hip Disorder

If you are looking for information on Legg-Calvé-Perthes Disease, look no further. In this review article, Dr. Harry K. W. Kim from the Center for Excellence in Hip Disorders (Dallas, Texas) provides us with an in-depth update on this hip disorder in children.

Legg-Calvé-Perthes Disease is a condition that affects the hip in children between the ages of four and eight. The condition is also referred to as Perthes disease. It was named in honor of the three physicians who each separately described the disease. Boys are affected five times more often than girls. In 10 to 15 per cent of children with this disease, both hips are affected.

In this condition, the blood supply to the growth center of the hip (the capital femoral epiphysis) is disturbed, causing the bone in this area to die. Hip pain, limited hip motion, and a limp bring the child into the physician’s office for diagnosis. The blood supply eventually returns, and the bone heals. But how the bone heals determines how much problem the condition will cause in later life. This condition can lead to serious problems and even permanent deformity in the hip joint later in life.

Understanding what part of the hip is affected may help in visualizing the problem. The hip joint is where the thighbone (femur) connects to the pelvis. The joint is made up of two parts. The upper end of the femur is shaped like a ball. It is called the femoral head. The femoral head fits into a socket in the pelvis called the acetabulum. This ball and socket joint is what allows us to move our leg in many directions in relation to our body.

In the growing child, there are special structures at the end of most bones called growth plates. The growth plate is sandwiched between two special areas of the bone called the epiphysis and the metaphysis. The growth plate is made of a special type of cartilage that builds bone on top of the end of the metaphysis and lengthens the bone as we grow. In the hip joint, the femoral head is one of the epiphyses of the femur.

The capital femoral epiphysis is somewhat unique. It is one of the few epiphyses in the body that is inside the joint capsule. (The joint capsule is the tissue that surrounds the joint.) The blood vessels that go to the epiphysis run along the side of the femoral neck and are in danger of being torn or pinched off if something happens to the growth plate. This can result in a loss of the blood supply to the epiphysis.

Perthes disease results when the blood supply to the capital femoral epiphysis is blocked. There are many theories about what causes this problem with the blood supply, yet none have been proven. There appears to be some relationship to nutrition. Children who are malnourished are more likely to develop this condition.

Children who have abnormal blood clotting also (a condition called thrombophilia) may have a higher risk of developing Perthes disease. These children have blood that clots easier and quicker than normal. This may lead to blood clotting that blocks the small arteries going to the femoral head.

As a result of new evidence, the certainty of thrombophilia as a cause of Perthes is now under debate. This will remain an area of study until scientists clear up the significance of thrombophilia as a possible cause of Perthes.

There is some new evidence that Perthes disease is genetic as a result of a mutation (abnormal change) in the type II collagen (fibers that make up soft tissue structures). Previously there was no known increase in risk for children whose parent had Perthes disease as a child. But this belief may no longer be accurate.

Studies among Asian families who have many members with this disease have been found with this mutation in the type II collagen gene. Scientists think that the mutation results in weakening of the hip joint cartilage that also affects the blood vessels within the cartilage.

Whatever the true cause of ischemia (lack of blood to the area), the result is bone death (called necrosis) of the femoral head. Without a normal blood supply, the bone loses its strength and shape. The loss of bone density and softening of the head result in a misshapen head. With the hip supporting the weight of the body, tiny microfractures in the necrotic bone fail to heal. This is another reason why normal wear and tear results in deformity.

Treatment is centered around the main goal of preventing deformity of the femoral head. When prevention isn’t possible, then minimizing the damage to this area is the next best approach. Treatment varies depending on the age of the child at the time of diagnosis. Long-term management for adults affected by this condition during childhood is another aspect of treatment.

The surgeon’s challenge is being able to tell which child needs surgery and when. X-rays and MRIs are used to get a handle on where the bone is affected, how much damage is present, and what kind of blood supply is present.

Studies show that surgery is needed when there is a large area of the femoral head and epiphysis affected. Earlier treatment (in children up to eight years old) yields better results. Treatment is more difficult when the femoral head has already fragmented and/or collapsed.

Planning Revision Spinal Surgery in Children with Spinal Deformities

Modern medicine can perform many “miracles” these days. Among them is the ability to surgically straighten a spine that is severely curved from a childhood condition called scoliosis. Pediatric spinal deformities requiring surgery may also occur associated with other developmental problems, neuromuscular diseases, or genetic conditions.

But a one-time procedure is unlikely over the lifetime of those children as they move into and through adulthood. Revision surgery is required in up to one-fourth (25 per cent) of all cases. The reasons for revision spinal deformity surgery vary.

It could be there wasn’t enough spinal correction the first time or there is a remaining imbalance in the spine that requires a two- or three-step series of operations. The fusion might not be complete and pseudoarthrosis (a false joint) develops. Loss of correction and an uncorrected second spinal curve getting worse may drive the decision to reoperate.

Sometimes the hardware (rods and screws) used to hold the spine in place break or get dislodged and must be repaired or removed and replaced. Revision surgery is needed then. If infection develops, the surgeon may have to go back in and perform additional procedures. And in some cases, the child or teenager develops pain at the operative site that must be investigated.

Whatever the reason, each case is unique and requires careful consideration and preplanning in order to gain a good result. In this article, Dr. Paul Sponseller, a pediatric orthopedic surgeon from Johns Hopkins University Hospital reviews the issues and complications associated with pediatric revision surgery for spinal deformities.

He reviewed all of the studies and reports on reoperations and summarized the information found on how often revisions occur, age of the patients, and surgical techniques used. Having this information is helpful when counseling patients and their families about what to expect for results with the first spinal correction (usually fusion) and the risk of reoperation.

Trends observed over time were also pointed out. For example, posterior fusions were more likely to require revision than anterior fusions. The overall revision rate for both anterior and posterior fusions has decreased over time. This change is most likely the result of improved surgical techniques and equipment and experience gained by surgeons over time. Most of the revisions required were for adolescents (teens).

It isn’t always clear when revision surgery is required. Sometimes it’s better to wait until the child has stopped growing (or at least isn’t growing so fast). When the decision is made to perform a revision procedure, the surgeon must plan carefully. Every effort is made to prevent further complications.

Surgeons are advised to re-read the patient’s medical record and look carefully for any clues from the previous surgery that might help. Was there quite a bit of bleeding during the index (first) surgery? Were there any other problems during the index procedure? Did the child have any unusual anatomical features to be aware of?

Preoperative imaging including X-rays and CT scans help the surgeon plan his or her correction strategy. CT scans are especially helpful in showing areas of infection or the presence of a pseudoarthrosis (false joint). Every angle and every level of the spine is taken into consideration during the pre-operative planning phase. And it is best to consult with other surgeons about each case in order to avoid missing any important decision points.

For the benefit of pediatric orthopedic surgeons who perform these procedures, the author goes over details of various procedures (e.g., resection, osteotomy, implant removal). Strategies are discussed when connecting one part of the spine to another in the revised fusion. X-rays, photos, and drawings are used to show the surgeon what to do and how to do it.

And a final point of discussion includes various ways to prevent complications such as infection, blood loss, dural tears, and spinal fluid leakage. Dr. Sponseller ends by encouraging all surgeons to aid the patient and family in facing the decision to reoperate. It’s a stressful time. Both patient/family and surgeon should remain open to the thoughts and ideas of others — even if it means both parties seek a second (or third) opinion.

Things That Can Go Wrong in Spinal Corrective Surgery

There’s more to worry about during spinal deformity surgery than just technical problems on the part of the surgeon. Errors can occur involving equipment, medications, communication, and body parts (i.e., operating on the wrong site).

In this literature review, surgeons from the Albany Medical College in New York collected data on complications during orthopedic surgery involving the spine. They specifically focused on problems that were unrelated to the surgeon’s technical skill.

The goal of this study was to improve patient safety by preventing and reducing adverse events (AEs). Adverse events are different from complications. Adverse events refer to any unexpected problems that occur. Complications were defined as adverse events that have a measurable or observable (negative) effect on patient results or outcomes.

Seven key areas were discussed: 1) patient positioning, 2) nutrition, 3) blood loss, 4) other health problems called comorbidities, 5) time in the operating room, 6) pulmonary (lung) problems, and 7) gastrointestinal (GI) problems.

Over time, surgeons have found the ideal position for patients having spinal surgery to correct spine deformities like scoliosis. The patient is placed prone (face down) with the head, neck, and hips supported in a neutral position. The bed is tilted to keep the head elevated slightly above the feet. The arms and legs are supported in a restful position without pressure on the joints.

Using this position has helped improve breathing, reduce pressure on the heart and lungs, lower pressure on the head and face, and decrease blood loss. Even with this good position, new problems such as blindness have cropped up. Pressure on the face and loss of blood supply over a long period of time can lead to this loss of vision.

Blindness is a fairly new complication probably from increased surgical time and more health problems in patients having surgery. The loss of vision can be permanent, so preventing the problem is a high priority. As a result, different head support systems have been developed for use during spinal deformity corrective surgery.

Other problems related to position such as pressure on nerves or kinking of blood vessels can be prevented with closer patient surveillance. This is especially true towards the end of the procedure when the surgeon and anesthesiologist have left the operating room.

Good nutrition before surgery is important to aid the immune system and speed healing. Older and overweight patients must be assessed carefully for possible malnutrition. Serious GI problems from loss of blood supply to the gut can even cause death.

The surgeon has many things to watch for during and after spinal corrective surgery that involves lengthening the spine. Vomiting, dehydration, fluid imbalances, and electrolyte problems are common.

Patients who develop blood clots or air bubbles in the heart or brain after spinal correction surgery have a 50 per cent chance of death. Every effort is made to assess patients for risk factors and to prevent such problems. Patients with heart problems, diabetes, obesity, and those who smoke or abuse alcohol are the most likely to develop complications after any surgery including spinal corrective surgery.

As far as blood loss goes, every effort is made to reduce bleeding during the procedure. The anesthesiologist and surgeon must work together to reduce surgical time. They use every technique possible to prevent blood loss and the need for blood transfusion. Many new methods have been developed to aid in this effort. Specific tools and techniques used are reviewed for surgeons who might be interested in an update in this area.

In summary, the authors of this article identify potential adverse events and complications associated with corrective surgery for spinal deformities. The Academy of Orthopedic Surgeons’ Patient Safety Committee can use this information to provide surgeons with a checklist to follow before, during, and after spinal surgery. The intended outcome is to reduce errors unrelated to the surgery itself.

The next step will be to study the effect any measures taken have in reducing these complications. Surgeons and operating room staff can then focus time and energy on the most effective methods for preventing adverse events that are not directly caused by the surgeon.

Principles of Treatment for Fractures in Adolescents

Treatment of bone fractures in teenagers should not be viewed the same as for children. So say orthopedic surgeons from Cincinnati Children’s Hospital Medical Center. Not only that, but they shouldn’t be treated the same as adults either. The principles of treatment for fractures in adolescents requires a unique and individual approach.

In this instructional review, the management of fractures in patients between childhood and adult age is presented. The patient’s bone age must be determined using X-rays. Actual age and bone age often differ. Without this piece of information, it is impossible to tell if the teen has stopped growing yet.

Concerns about damage to the physis growth plate, deformity, and leg length difference drive the need to estimate skeletal maturity as accurately as possible. Treatment varies depending on whether the growth plate is still wide open (allowing further growth), partially open, or closed. And because the anatomy is different for each bone, there are separate classification systems for each one.

Using photos of X-rays and descriptions, the authors provide a review of each classification scheme. Details are provided in how to look at each X-ray. Additional imaging studies such as CT scans and MRIs may be needed if there is any suspicion of injury to the joint or soft tissues around the bone and joint.

Two sets of principles for the treatment of fractures in teens are presented. The first are directed toward fractures affecting the physis (growth plate). The second deal with nonphyseal fractures in this age group. Specific bone fractures discussed include the clavical (collar bone), radius and ulna (bones in the forearm), femur (thigh bone), and tibia (lower leg bone or shin).

To give you an idea of the types of principles offered, the authors tell surgeons treating physeal fractures in teens:

  • If possible, use traction and gentle hands-on manipulation to pull the fractured ends of the bone apart in an attempt to realign the bones. This procedure is referred to as reduction of a displaced fracture. If this doesn’t work easily, then open incision and surgical reduction is required.
  • Physeal fractures that are two millimeters apart (or less) must be reduced with a closed technique within the first 10 days after injury, otherwise surgery will be needed (open reduction). This is because most physeal fractures will be well on their way to healing by then.
  • When using pins or screws to hold the fracture together, the hardware must be put in parallel to the physis (not perpendicular or at an angle). When fixation devices must cross the physis, then they should be made of smooth material and taken out as soon as possible.
  • No matter what kind of treatment is provided, patients with physeal fractures must be followed closely for at least six months (or until skeletal maturity is reached). During this time, the surgeon will be watching carefully for any signs of growth disturbance.

    That’s just a partial list of the recommendations made for surgeons to follow when treating physeal fractures in teens who have not yet finished growing. What about teens with fractures of the bones named that do not affect the physis (growth plate)? What kind of principles or recommendations for treatment should be followed for fractures of the bone shaft?

  • Treatment is usually more like what is given to adults. The need for reduction follows an adult model.
  • All decisions are made based on the age and weight of the teenager.
  • Fixation hardware for fractures of the long bones are slightly elastic (flexible) and can be put in place through the skin (percutaneously) without making an open incision. The pin goes down through the shaft of the bone lengthwise. This is referred to as an intramedullary pin.
  • The end of the pin is left slightly outside the skin so that it can be pulled out easily when healing has occurred.
  • Most of the time, the patient who has intramedullary fixation is also placed in a cast, splint, or brace to give external support until healing takes place.

    After giving general treatment guidelines for physeal and shaft fractures, the authors offered some specific details for individual fractures starting with clavicular (collarbone) fractures and working their way through all the long bones. Some changes have occurred over the years in the way various fractures are treated.

    Fractures of the clavicle (shaft, not the ends) used to always be nonoperative. But studies have shown that realigning a displaced fracture and using hardware to hold it in place has a better outcome than leaving it to heal on its own where ever the broken ends might happen to be (matched up or not). Now the adolescents who are active athletes and sports participants can get back in full swing within three months rather than four. Minimally displaced fractures are still treated conservatively (without surgery).

    Forearm fractures may do better when repaired surgically. X-rays and other imaging studies are needed to check the bone(s) for any sign of angulation Angulation means the bones are not healing straight but with a bend somewhere along the shaft. The amount of angulation that can be treated with a cast depends on where the fracture and angle is located in the bone (e.g., top one-third of the shaft toward the elbow, middle portion, lower third toward the wrist).

    The bones of the forearm and clavicle are long but fairly thin so the placement of the pin or nail requires some care when placing it. The surgeon must avoid pushing the pin into tendons, blood vessels, nerves, or other vital soft tissues. Sometimes both bones of the forearm are broken at the same time. That creates a more complex injury to treat. There is a greater risk for delayed union, malunion, or nonunion. Other potential problems include loss of reduction, infection, injury to the physis, and migration (unintended movement) of the nail.

    The authors provided a detailed discussion covering the treatment of femoral shaft, tibial fractures, and ankle fractures. Advantages and disadvantages of surgical versus nonsurgical treatment are discussed for each fracture location. There are many different treatment options for these fractures. The first concern is always for the growth plate. Age is always a factor, too. For example, in the case of femoral fractures in children 11 and older, there are more choices in terms of the kinds of hardware (metal plates, elastic nails) that can be used compared with younger children.

    A few final instructions were given for the use of flexible nails for fracture fixation (especially of the femur). Two nails must be selected that are the same diameter and don’t fill more than 80 per cent of the shaft. The nails are pre-bent before insertion so that once they are threaded down into the center of the bone shaft, the bend is right where the fracture is located.

    A different type of nail is needed for older children who are overweight and/or who have an unstable fracture site. There are many different nail systems to choose from. Each surgeon must learn the types of nails available, when to use each one, and how to use them.

    In summary, the management of fractures in teenagers can be very challenging for a number of different reasons. Skeletal age is more important than actual (chronological) age. Bone quality, location, and severity of the fracture are added variables. The surgeon must make the determination of what type of treatment is best based on principles outlined for physeal versus nonphyseal fractures.

    The goals of treatment are to obtain the best fracture healing with as little deformity as possible. Preventing complications from the use of intramedullary pins used to hold the broken pieces in place until healing takes place is another goal. All management decisions are made keeping in mind the child’s emotional and psychological needs with every effort to minimize time away from school and sports.

  • Concussions in High School Athletes

    If you ever watched the Three Stooges on television, at least once per episode, Moe would take Curly and Larry and smack their heads together. It’s a wonder they didn’t suffer from a concussion. That type of head-to-head collision is what accounts for three-fourths of all sports concussions.

    Recent attention on sports concussions has resulted in a database called the High School Reporting Information Online (HS RIO) surveillance system. Athletic trainers at 100 pre-selected high schools from around the United States enter data each week on all sports-related injuries.

    Information is entered on any athlete (male or female) participating in football, soccer, basketball, wrestling, baseball, volleyball, or softball who suffers an injury during their sport activity.

    Not just concussions, but any injury is reported along with the date it occurred, age and sex of the player, and type of injury. Information on the specific symptoms, date the athlete returned to play, and medical treatment received is also recorded.

    In this study, physicians from the Sports Concussion Clinic at Children’s Hospital in Boston, Massachusetts report on the 544 concussions reported to the surveillance system. All cases occurred among high school athletes for the 2008-2009 year. After analyzing the data they could see that injuries of this type occur equally in every year of the high school experience (freshman through senior years).

    Most concussions occured during competitive play (rather than during practice). More than half were the result of football action. The most common mechanisms of injury was contact with another player (three-fourths of all concussions), contact with the field or playing surface (15.5 per cent), or collision with a piece of playing equipment (e.g., goal posts, bleachers).

    How can you tell if an athlete has suffered a concussion? The first clue comes from symptoms such as headache, blurred vision, nausea and vomiting, sleepiness and dizziness. Other symptoms can include difficulty concentrating, feeling in a fog or feeling slowed down, ringing in the ears, irritability, and sensitivity to noise.

    Loss of consciousness is a very important signal that something serious has happened. But being “knocked out” isn’t necessary to have a concussion. Headache is the most common symptom but some players aren’t aware of any symptoms.

    Symptoms resolve within the first 24 to 36 hours for most athletes. Symptoms persisting beyond a week are not uncommon. These usually clear up within a month’s time. Only a small percentage of athletes (1.5 per cent) report symptoms lasting more than 30 days.

    The immediate question after a collision is whether or not that player can return-to-play right away and if not, when can he or she get back into action? Symptoms offer some guidance but there are reports of deaths among athletes who failed to report symptoms and went back to play right away.

    To avoid the deadly consequences of concussion, medical experts recommend computerized neuropsychologic (NP) testing. This type of test includes questions that evaluate the athlete’s brain function including memory, attention, language, and visual-spatial skills. The NP test provides a comparison to expected norms for each task and a baseline from which to measure changes or progress after injury.

    Data from the surveillance system for the 2008-2009 year showed that players who took the neuropsych (NP) test were less likely to return to sports play the same day as the injury. In fact, they didn’t return to their sport for the first week after their injury. And a curious pattern emerged from this data: football players (the sports athlete most likely to sustain a concussion) were the least likely to be tested.

    That last bit of news raised more than a few eyebrows. There is concern that football players are more likely than any other sports athletes to avoid reporting symptoms in an effort to stay in the game.

    If this is true, there is much that needs to be done to protect athletes possibly through mandatory testing or even just education about the seriousness of this injury. Long-term studies are needed to see if there are later effects of concussions that don’t show up for 5, to 10 to 20 years (or longer).

    The safety of the player has to be the number one priority. Athletes should not be allowed to return to play until it is safe to do so. Neuropsychologic (NP) testing is one way to assure that players get the protection and treatment they need after a significant head injury.

    Results of Treatment of Growing Rods for Scoliosis in Children

    Scoliosis (curvature of the spine) can be a serious problem in young children. The spine can curve so much that the lungs and heart are compressed causing deadly complications. When scoliosis is present early (between birth and age five), the chances of a fast growing curve are much higher than when the curvature develops in the teen years.

    In this report, researchers at the San Diego Center for Spinal Disorders in California take a closer look at complications following growing rod treatment for scoliosis. These expandable rods hold the spine in neutral alignment (as much as possible).

    As the child grows, the rod can be lengthened. This type of treatment prevents removing the rods and replacing them as growth occurs. You can see the advantages of such treatment in cases of early-onset scoliosis. When the child stops growing, then spinal fusion can be done.

    But there can be complications, too. By studying who is affected and possibly answering the question, Why do these problems develop?, it might be possible to prevent such problems.

    The main factors reviewed in this study included 1) age of the child at the time of surgery, 2) where the rods are placed (under the skin above the fascia versus below the fascia), 3) single or dual rods (placed on one side versus both sides of the spine), and 4) the number of surgeries performed. Fascia is another word for the connective tissue that surrounds and supports the soft tissues (e.g., muscles).

    The information gathered on children comes from The Growing Spine Study Group. This is a computer database with information downloaded from around the world. Various spine centers treating children with early-onset-scoliosis provide information about results of treatment using these growing rods.

    Over the years, this group has been able to show that early spinal fusion (before age seven) is not a good idea. These are the kids who end up with cosmetic deformities and difficulty breathing. This same group was also able to show that growing rods have more complications than previously appreciated.

    Different rod designs with growth expansion at the ends versus in the center of the rod have been tried. The single-rod on one side of the spine has been compared with using dual-rods (one on each side of the spine). Data has also been collected and analyzed on the different ways the rods are attached to the spine (e.g., screws, hooks, connecting bars at the top and bottom).

    There was a whole host of different complications. Infections, blood loss, rods breaking, painful scars, hook or screw pullout, and rods poking through the skin give you some idea of what was happening. There were also cases of lung, heart, and/or intestinal problems.

    In the database, there were 140 patients who had a total of 897 growing-rod procedures. More than half (58 per cent) of those 140 children had at least one complication. The rate of complications was higher in children with the single-rod support or subcutaneous placement. They also found that the more surgeries the child had, the greater the risk of problems developing.

    For early-onset scoliosis, the surgeons advise putting off surgery for as long as possible. Bracing or casting may be used to delay surgery. When surgery is finally done, dual rods should be considered over single rods. The deeper the rods are placed, the better the results. And the fewer times the rods are lengthened, the fewer the complications.

    Children with curves that are growing too fast to hold with conservative measures must have the growing-rod treatment. Careful monitoring is essential to get the best results with the fewest problems. The goal is to prevent deformities while still allowing for growth and development of the spine and trunk.

    Parents of children with early-onset scoliosis that is progressively getting worse must be prepared for changes in treatment. Casting or bracing may be used at first and then replaced with surgery. At the same time, every child with a growing rod must be using a special plastic brace called a thoracolumbar sacral orthosis (TLSO). Complications, problems, and additional (often unplanned) surgeries are to be expected.

    In the future, it may be possible to lengthen the rods without doing surgery. The internal rods could have an external remote. The remote could be used to allow for expansion without opening up the spine to adjust the rods by hand. This type of technology could reduce the number of procedures (and complications) until spinal fusion is possible.

    Sleeve Fractures of the Kneecap in Children

    You may not be familiar with the term sleeve fracture of the patella (kneecap). That’s not surprising since this is a very rare injury. Of all the bone breaks children have, the kneecap is only involved in about one per cent of the cases. And sleeve fractures make up about half of those patellar injuries.

    What’s a sleeve fracture? A little anatomy will help explain what happens. The patella or kneecap sits in front of the knee joint. It isn’t attached by a piece of bone or bone bridge. Instead, it moves freely up and down, gliding along a set pathway or patellar track. The kneecap is held in the track by the quadriceps tendon.

    The quadriceps tendon is wrapped around the kneecap to hold it in place. At the upper end, the tendon is attached to the large four-part quadriceps muscle along the front of the thigh. Its job is to straighten the knee. The quadriceps tendon continues down below the knee cap where it inserts or attaches to the tibia (lower leg bone).

    With a sleeve fracture, the quadriceps tendon is torn so severely, it separates from the muscle and takes a piece of the cartilaginous patella with it. It also takes the top layer of bone called the periosteum. When the periosteum is peeled away with a fragment of the underlying bone still attached, it is called a sleeve avulsion.

    Sleeve fractures of the patella can actually occur at the top of the kneecap (called the superior pole) or at the bottom (inferior pole). Most sleeve fractures involve the inferior pole.

    With an inferior pole injury, the trauma occurs when the knee is bent or flexed. Superior pole sleeve fractures are more likely to be caused by a sudden, forceful contraction of the quadriceps while the knee is bent but trying to straighten. This movement is called an eccentric contraction (a fully contracted muscle is releasing). Direct trauma to the tendon can also cause this type of fracture.

    In children who are not fully grown yet (we say they are skeletally immature), the patella is still more cartilage than bone. The softer cartilaginous patella in the skeletally immature child tears more easily than solid, hardened bone in a skeletally mature individual.

    Sleeve fractures were first described in the literature in 1979. Boys are affected five times more often than girls. Most are between the ages of eight and 16 years old. Increased high-intensity sports activity may be one reason this type of injury has started to show up. Like this report, most articles published in medical journals on this topic involve isolated cases (only one child affected). There’s been only one article based on a group of 47 cases.

    The case that led to this report and review of the literature involved a 10-year-old-boy who slipped while jumping off a diving board. In the process, his knee was flexed and moving toward extension (eccentric contraction) when he felt a “pop” and immediate, severe pain. A physical exam, X-rays, and MRI confirmed the diagnosis of a superior pole sleeve fracture of the patella.

    The authors used this case to describe sleeve fractures, discuss how they are diagnosed, and offer suggestions for treatment. All other cases of sleeve fractures were reviewed and summarized as well. The results of the various cases, as well as the final outcome for this child were also presented.

    It’s important to note that X-rays don’t always tell the whole story. In many cases, it wasn’t until further imaging studies (CT scans, MRIs, ultrasound) were done that the full extent of the injury was discovered. And the surgeon really needs all the details of the injury to form the best plan for each child.

    Surgery is usually needed to bring the pieces of the patella back together (reduction) and hold them in place with pins or screws (internal fixation) until healing takes place. The procedure is called open reduction and internal fixation or ORIF. The leg is put in a cast with the knee straight for about six weeks. Physical therapy begins as soon as the cast is removed. Restoring full knee motion and strength are the two main goals of therapy.

    The authors conclude that with careful placement of the sleeve fracture during surgery, normal quadriceps function is possible. Improper treatment can result in deformity and poor timing of the quadriceps’ ability to contract and release normally.

    Conservative (nonoperative) care may be possible in skeletally mature patients if there is no change in the fragment position as the knee bends and straightens. In order to know if the fragment moves, the knee must be observed under fluoroscopy, a special type of 3-D X-rays that allow the surgeon to see the joint as it moves.

    Without surgery, the patella may end up shifting location (moving up or down depending on which type of sleeve fracture occurred). The quadriceps may develop an extensor lag and start to atrophy (weaken and waste away). An extensor lag means the quadriceps tendon that straightens the joint doesn’t pull back far enough to get full knee extension. The knee remains slightly flexed no matter how hard the person tries to straighten it.

    Most children (including the boy in this report) are able to recover fully. They resume full participation in all activities and sports. There may be some occasional knee pain with certain activities like running and jumping. But for the most part, the fracture heals, the kneecap tracks normally, and the quadriceps muscle bulks up again.

    Treatment for Children with Spinal Cord Injuries

    Fifty years have gone by since the first study published on spinal cord injuries in children. Although this injury doesn’t happen very often, when it does occur, surgeons must be ready to provide the best treatment possible.

    Systematic reviews like this one are important because they summarize evidence to support best practice. Researching all studies published on a subject like pediatric spinal cord injuries gives everyone a better idea of who suffers these injuries and how they should be treated.

    What do we know about who is spinal cord injured at an early age? Most of the children are in their teens. Only 10 per cent of all pediatric spinal cord injuries affect children younger than 15 years.

    Their injuries come from car accidents, falls, sports, diving, gunshot wounds, and pedestrian injuries. Boys (males) are injured more often than girls (females).

    Car accidents rank as the number one cause of spinal injuries. Just slightly more than half of all spinal cord traumas are the result of car accidents for all ages. And two thirds of these injuries occur when the child was not wearing a seat belt.

    What’s the best way to treat these injuries? The first problem surgeons face is the stabilization of the spine. Broken vertebral bones must be put back together and held in place so that they don’t do further damage to the spinal cord.

    Taking pressure off the spinal cord is referred to as a decompression procedure. Using wires, screws, metal plates, or rods to hold the spine in place is referred to as internal fixation or instrumentation.

    The timing of the surgery (how soon after the accident) and the methods used (fixation or not? type of instrumentation?) are two of the biggest debates. Most of the research done so far has been on adults. As you can imagine (especially with young children), the small size of the bones and immature skeletal structures presents unique challenges.

    Applying treatment techniques designed for spinal cord injured adults on children often yields better results than when using them on adults. Results are measured in terms of fusion rates and complications.

    That discovery raises the question, Are there even better ways to treat pediatric cases of spinal cord injury? Improved spinal cord monitoring techniques and Stereotactic and CT guidance systems used during surgery are now available.

    Are there improved results because of them? Hopefully future studies of children will bring about some answers to these questions. For now, the evidence suggests that unstable spinal fractures should be surgically corrected. Realigning the bones protects the nerve tissues.

    What about spinal deformities that develop later? What’s the best way to treat scoliosis (curvature of the spine) in children who are still growing? Is more surgery needed? What about the use of back braces?

    Studies show that almost all children who have not completed growth at the time of the spinal cord injury will develop a spinal curvature. Trunk deformity is most common in girls during a growth spurt. This occurs most often before the age of 12 when trunk growth slows to a stop. The same is true for boys but growth slows to a stop at a slightly older age (12 to 14).

    Everyone on the team (parents or family, physicians, physical therapists, nurses) monitors the child closely for the first sign of spinal deformity. Bracing right away is strongly encouraged to keep the curve from getting worse and perhaps prevent the need for spinal fusion.

    When bracing doesn’t prevent worsening of the deformity, then surgery may be needed to fuse the spine and hold it in place. There is very low evidence to support this recommendation. It is how treatment is carried out right now. This approach will likely continue as such until studies are done to show whether there is a better way to approach this problem.

    In summary, use of instrumentation is strongly recommended for unstable spinal fractures in children. Protecting the spinal cord is a high priority. Traditional treatment with bracing and physical therapy remains the approach to spinal deformities (scoliosis).

    Future studies are needed to further examine both of these issues. High-quality evidence is needed to support continued treatment as it is now provided or to offer guidance in a different direction — one that will yield better results.

    Rare Case of Viral-Induced Trigger Finger in a Child

    Here’s something pediatricians and parents of young children will want to know. Viral infections can cause trigger finger. It doesn’t happen very often but it should be a consideration when evaluating cases of unexplained trigger finger.

    What is trigger finger? Trigger finger (and trigger thumb) are conditions affecting the movement of the tendons as they bend the fingers or thumb toward the palm of the hand. This movement is called flexion.

    The tendons that move the fingers are held in place on the bones by a series of ligaments called pulleys. These ligaments form an arch on the surface of the bone that creates a sort of tunnel for the tendon to run in along the bone. To keep the tendons moving smoothly under the ligaments, the tendons are wrapped in a slippery coating called tenosynovium. The tenosynovium reduces the friction and allows the flexor tendons to glide through the tunnel formed by the pulleys as the hand is used to grasp objects.

    Triggering is usually the result of a thickening in the tendon that forms a nodule, or knob. The pulley ligament may thicken as well. The constant irritation from the tendon repeatedly sliding through the pulley causes the tendon to swell in this area and create the nodule.

    What causes trigger finger? In children, triggering can be caused by a congenital defect that forms a nodule in the tendon. Type 1 diabetes has also been linked with trigger finger in children and teens. In adults, rheumatoid arthritis, partial tendon lacerations, repeated trauma from pistol-gripped power tools, or long hours grasping a steering wheel can cause triggering.

    In the case of the seven-year-old who is the subject of this report, viral infection caused damage to the synovium and a rounded swelling (nodule) to form in the tendon. The authors wrote this case up to help highlight the fact that viral infections can indeed be a triggering mechanism for trigger finger. It’s a rare, but important, factor to keep in mind when evaluating a child with trigger finger.

    What does trigger finger look like? The symptoms of trigger finger can include pain and a funny clicking sensation when the finger is bent and straightened. Tenderness usually occurs over the area of the nodule, at the bottom of the finger or thumb.

    The clicking sensation occurs when the nodule moves through the tunnel formed by the pulley ligaments. With the finger straight, the nodule is at the far edge of the surrounding ligament. When the finger is flexed, the nodule passes under the ligament and causes the clicking sensation. If the nodule becomes too large it may pass under the ligament, but it gets stuck at the near edge. The nodule cannot move back through the tunnel, and the finger is locked in the flexed trigger position.

    How does the physician know a viral infection is the cause of trigger finger? In this case, the child had been seen first for hip pain and limping. There was no fever and no history of trauma. Lab tests showed an elevated erythrocyte sedimentation rate (ESR or “sed” rate). The sed rate is a sign of inflammation. Ten days later, he came in to the clinic with trigger finger affecting the ring finger of both hands. Anytime a problem occurs on both sides (called bilateral), the physician has cause to suspect an underlying systemic problem.

    What’s the treatment for viral-induced trigger finger? Like any viral infection, rest and plenty of fluids are advised. Antibiotics are not helpful as there is no bacterial component to the illness. And since the trigger finger symptoms went away after a month’s time, the authors felt their plan of care was right on target. This result to a wait-and-see approach is important because most cases of trigger finger (caused by something other than a virus) usually require surgery.

    Thus the trigger finger condition caused by a viral infection is considered benign (not life-threatening), transient (symptoms come and go or move around as in this case), and self-limiting (temporary and will go away). Why only the hip on one side and both ring fingers were affected in this case remains unknown.

    Orthopedic Surgeons Offer Their Preferred Treatment for OCD of the Elbow

    Overhead throwing athletes (especially pitchers) of all ages are at risk for elbow and shoulder problems. In this review, orthopedic surgeons from Harvard Medical School, Wellesley Hospital, and Tufts University walk us through all aspects of osteochondritis dissecans (OCD) of the elbow in pediatric patients.

    What is osteochondritis dissecans? OCD is a condition in which a piece of cartilage and the underlying bone have been damaged. In some cases, the damaged fragment separates from the bone and floats freely within the joint.

    The problem can develop in the elbow as a result of trauma (injury) but more often, it occurs when there is repetitive compression of the radiocapitellar joint. Athletes affected most often include baseball pitchers, weight lifters, tennis players, cheerleaders, and female gymnasts.

    The radiocapitellar joint is located where the radius (bone in the forearm) joins the bottom of the humerus (upper arm bone) to form part of the elbow joint. Osteochondritis dissecans (OCD) of the elbow doesn’t occur in immature throwing athletes very often so there isn’t a lot of information about it to help guide treatment.

    What do we know about this condition? Over time, OCD lesions can lead to further degenerative changes in the elbow. It is not self-limiting or in other words, it doesn’t get better on its own. But other than that bit of information, the natural history (what happens over time) and the best way to treat this condition isn’t known.

    Shear stresses from repeated motions probably start the problem. Poor mechanics and fatigue of the muscles and ligaments are added to the shear load. Combined together, these forces cause the cartilage to separate from the bone, taking a piece of the underlying layer of bone with it.

    How can the orthopedic surgeon tell if someone has osteochondritis dissecans? Of course, the patient history helps — for one, participation in any of the sports mentioned is a red flag. The symptoms reported are usually pain along the outside of the elbow that gets better with rest. Stiffness, locking, catching, and loss of full elbow extension complete the picture.

    To confirm the diagnosis, the physician relies on X-rays, MRIs, and sometimes CT scans. Once it has been determined where the damage is located, how severe the lesion is, and how stable (or unstable) the elbow is, then a management plan can be formed.

    The first step is to rest for three to six weeks. Athletes must learn how to change the way they do things or the problem will come right back. This process is called activity modification.

    A physical therapist will prescribe exercises to stretch and strengthen appropriate muscles. The physician may prescribe medications such as nonsteroidal antiinflammatories (NSAIDs). There aren’t enough studies to show that this is really needed or beneficial. Specific guidelines regarding dosage (how much) and duration (how long) these medications should be used are not available.

    Athletes most likely to recover nicely with conservative (nonoperative) care are younger and have early (mild) disease. Patients who have completed six months of conservative care but who still have symptoms are considered candidates for surgery.

    Surgery is also considered when there are fragments of cartilage and/or bone inside the joint. These are called loose bodies. Patients who have loose bodies are most likely to develop the catching and locking symptoms of the elbow reported.

    What can the surgeon do for this condition? The authors’ suggest drilling for lesions that are stable. Stable means there are no loose fragments or unstable bits of cartilage that could get torn off and form a loose body.

    Drilling refers to the practice of putting tiny holes in the surface of the cartilage down through the layer of bone underneath the cartilage and right through to the bone marrow. Tiny drops of blood seep up from the bone into the defect and stimulate a healing response. This type of bone marrow stimulation has good short- to medium-term results. Long-term data (especially about return-to-sports status) is still needed.

    When there are loose bodies or an unstable cap, the authors prefer to use debridement first, then bone marrow stimulation. Debridement involves removing any fragments and smoothing down any remaining rough edges. If the lesions are large (more than half of the cartilage in the radiocapitellar joint is damaged), then osteochondral autograft transplantation (OAT) is advised.

    The OAT technique is a two-step process. First, normal, healthy plugs of articular cartilage and bone are harvested. Because the radiocapitellar joint is so small, surgeons must rely on another joint as the donor site.

    Usually the femoral condyle (end of the thigh bone forming the upper half of the knee joint) is the main source of graft plugs. The plugs are then transferred to the damaged area of the elbow joint and inserted.

    As with bone marrow stimulation, the OAT approach has not been studied enough to know how well the plug works in the long-run. There are questions and concerns about the match-up between knee-to-elbow cartilage because knee cartilage is flatter and thicker than the cartilage in the radiocapitellar joint.

    The authors advise careful inspection of the entire elbow joint using arthroscopy. Different portals (insertion sites for the scope) must be used to view the front, sides, and back of the joint. For all surgical procedures discussed, there is a detailed description of the patient’s position during surgery, location of incisions or portals, and exact surgical techniques used.

    In summary, physicians treating young patients for osteochondritis dissecans of the (elbow) capitellum have much to consider when trying to determine the best plan of care. The athlete’s interest is in getting back to their sport of choice as soon as possible.

    The surgeon wants to provide treatment that will give the athlete a stable joint that won’t develop degenerative arthritis later. Right now, there isn’t enough evidence to create standard treatment guidelines.

    Given the current evidence and surgeons’ experience treating this condition, the authors’ suggestions for conservative versus surgical care are what they call their “preferred treatment”. More studies (especially long-term studies) are clearly needed to find out what works best for each type of athlete given the location and severity of the damage done to the radiocapitellar joint.

    Elbow Pain in Young Boys: Panner Disease or Osteochondritis Dissecans?

    What do we know these days about osteochondritis dissecans of the elbow in young athletes? What causes this condition? Can it be cured? These are some of the questions answered in this review article written by sports medicine orthopedic surgeons from Rush University in Chicago, Illinois.

    Osteochondritis dissecans (OCD) is a problem encountered most often by male adolescents (teens) involved in repetitive overhead throwing activities. Young girls participating in gymnastics are the second group affected most often. Gymnasts can spend quite a bit of time engaged in activities that require repeated weight-bearing on the arms leading to OCD.

    What is OCD and what causes it? In this condition, repetitive microtrauma from repeated motions of the elbow causes the articular cartilage that lines the elbow joint to separate and break into pieces. When the cartilage pulls away from the joint, it takes a layer of subchondral bone with it. Subchondral just means “under the cartilage,” which describes the first layer of bone next to the articular cartilage.

    Any bone within the elbow can be affected. But the most commonly involved bone is the capitellum. Here’s a quick review of elbow anatomy to help you picture the capitellum. The elbow is the connection of the humerus (upper arm bone) and the two bones of the forearm (the ulna and the radius).

    The joint where the humerus meets the radius is called the humeroradial joint. This joint is formed by a knob and a shallow cup. The knob on the end of the humerus is called the capitellum. The capitellum fits into the cup-shaped end of the radius, also called the head of the radius.

    When the head of the radius spins on the capitellum, the forearm rotates so that the palm faces up toward the ceiling (supination) or down toward the floor (pronation). The joint also hinges as the elbow bends and straightens.

    When making the diagnosis, the orthopedic surgeon must distinguish between OCD and another problem called Panner disease. Although these two conditions are considered separate problems, some experts view them as two stages of the same thing.

    Both affect the capitellum but Panner disease causes fragmentation of the entire capitellum. OCD is usually more of an isolated lesion that breaks away from the main bone causing a loose body to float inside the joint.

    Panner disease tends to develop in young boys between the ages of 5 and 10 who aren’t involved in repetitive motions that cause trauma to the joint. For unknown reasons, normal growth in the outer edge of the elbow is disrupted, which causes the small area of bone to flatten out.

    Symptoms of diffuse elbow pain are common with both Panner disease and OCD. Diffuse means throughout the entire elbow. Pain can occur along the outside or lateral aspect of the elbow. The pain is present with activity and there’s a loss of extension. The child cannot straighten the elbow all the way. There may be stiffness, swelling, and when there is a loose body associated with OCD, clicking, catching, and/or locking of the elbow can occur.

    How does the physician tell the difference between OCD and Panner disease? The child’s age and activity level help sort this out. X-rays, MRIs, and the most definitive method: arthroscopy shows the type, location, and severity of cartilage and subchondral damage. MRIs are especially good at showing early changes when X-rays appear otherwise normal.

    Once the diagnosis has been made, then a plan of care is developed. One main difference between Panner disease and OCD is that Panner disease is self-limiting. That means it will go away with rest and doesn’t require additional treatment. Over a period of one to two years, the bone slowly rebuilds itself. During this time, symptoms gradually disappear, although the elbow may never fully straighten out.

    OCD may respond to rest, which removes the compressive load and shear forces long enough to allow healing. The use of antiinflammatory medications and a physical therapy program of stretching and strengthening exercises are also recommended.

    OCD does not always improve with conservative care. With more advanced (more severe, unstable) lesions, surgery might be needed to help the cartilage heal. There are many different surgical procedures that have been used to help aid healing in OCD. These include drilling, grafting, chondrocyte implantation, and osteotomy.

    Surgery is necessary when there are loose fragments of cartilage and subchondral bone floating inside the joint. Various techniques are described to reconnect the loose fragment (e.g., wiring, screw fixation, stapling). Sometimes the fragment just has to be removed.

    Studies of the various surgical treatments have resulted in a wide range of results. There isn’t one technique that works the best for everyone. The surgeon must decide what might work best given the location and severity of the defect. Sometimes there’s more than one lesion to be considered.

    To help surgeons who are treating OCD surgically, the authors provide many color photographs taken with an arthroscope of various cases they have treated. They describe the technique used to insert the scope into the joint to get the best possible view of what’s going on inside there. Different sized scopes can be used depending on the size of the child’s elbow.

    Their preferred treatment is removal of small fragments and fixation of large loose pieces. Special surgical tools are used to shave any uneven edges of cartilage and create a healthy, smooth rim of cartilage. Whenever possible, osteochondral reconstruction is avoided if other less involved surgery can be done to aid in the healing process.

    Most young athletes can expect to return to the sports activity of their choice. But there’s an extended period of time of physical therapy, rehab, and recovery. When full, pain free elbow motion is possible, then strengthening begins and progresses to include sports-specific training.

    The prognosis for osteochondritis dissecans of the capitellum is not always good. Studies show that at least half of the children affected by this condition end up with arthritis and continued elbow pain, stiffness, and limitations. The prognosis seems poorest for those patients with the most severe, unstable lesions. New treatment techniques are undergoing study with hope for more promising long-term results.

    Factors For Developing Osteonecrosis in Children with SCFE

    Slipped capital femoral epiphysis (SCFE) is a condition that affects the hip in teenagers between the ages of 12 and 16 most often. Cases have been reported as early as age nine years old. In this condition, the growth center of the hip (the capital femoral epiphysis) actually slips backwards on the top of the femur (the thighbone).

    If untreated this can lead to serious problems in the hip joint later in life. Fortunately, the condition can be treated and the complications avoided or reduced if recognized early. Surgery is usually necessary to stabilize the hip and prevent the situation from getting worse.

    Even with treatment, there is a risk of developing a condition called osteonecrosis (death of bone). What causes osteonecrosis to occur? A little understanding of the anatomy of the child’s hip will help explain this phenomenon.

    In the growing child, there are special structures at the end of most bones called growth plates. The growth plate is sandwiched between two areas of the bone called the epiphysis and the metaphysis. The growth plate is made of a special type of cartilage that builds bone on top of the end of the metaphysis and lengthens the bone as we grow. In the hip joint, the femoral head is one of the epiphyses of the femur.

    The capital femoral epiphysis is somewhat unique. It is one of the few epiphyses in the body that is inside the joint capsule. The joint capsule is the tissue that surrounds the joint. Here’s the key to osteonecrosis developing: the blood vessels that go to the epiphysis run along the side of the femoral neck and are in danger of being torn or pinched off if something happens to the growth plate. This can result in a loss of the blood supply to the epiphysis and then death of the bone (osteonecrosis).

    Why do some children develop osteonecrosis and others don’t? And how often does this really happen? Reports in the medical literature range from 10 to 60 per cent. That’s quite a broad range. To help answer some of these questions, pediatric orthopedic surgeons from The Children’s Hospital of Philadelphia (CHOP) reviewed 70 of their cases to see what they could find out.

    One unique characteristic that all 70 children had in common was an unstable slipped capital femoral epiphysis (SCFE). Unstable means they were unable to put any weight on the affected leg. The pain was so severe that even with crutches or other supportive aids, they were unable to walk.

    By going back and reading the medical records and comparing children with unstable SCFE who developed osteonecrosis with those who didn’t, they hoped to shed some light on this situation. They also compared the type of surgery performed and individual patient factors. Those factors included age, weight, how long symptoms were present before diagnosis, and length of time between diagnosis and treatment.

    X-rays taken before surgery were used to measure two things: the slip angle and change in translation. These measurements helped give the surgeons an idea of how severe the slip was before surgery and how much reduction took place as a result of the surgery.

    They quickly saw that the children fell into one of three groups based on the type of surgical treatment. Group one consisted of children who had the slipped epiphysis held in place with a screw. This procedure is called in situ screw fixation.

    Group two had a closed reduction. Reduction means the slipped epiphysis went back into place. Closed tells us this happened without open surgery with an incision. Sometimes just positioning the hip in a certain way will reduce or realign the hip. This can happen while moving the child or placing him or her on the operating table.

    And group three had open surgery to put the slipped epiphysis back in place and hold it there with a long pin (screw) placed through the bone. This procedure is called an open reduction and internal fixation (ORIF).

    After looking over all the information collected and analyzing the data, they found three factors that might be significant. The first was age. Younger children with very little warning symptoms before the problem was diagnosed had a higher incidence of osteonecrosis after surgery. The second was severity of slippage at the time of diagnosis. It’s likely that the more severe the problem and the greater the instability, the shorter the time before symptoms develop.

    The third significant factor was the type of surgery that was done. Group two (closed reduction) had the largest number of cases of osteonecrosis (26 per cent). Group one (in-situ fixation) had the second highest incidence (19 per cent).

    Group three (open reduction and fixation) had only one patient develop osteonecrosis. For all the children in the study, the more severe the slip was before surgery, the greater the risk of developing osteonecrosis after surgery. The overall incidence of osteonecrosis for the entire group (all 70 children) was around 20 per cent.

    The authors agree with other experts that treatment for slipped capital femoral epiphysis should be done as quickly as possible. Parents (and patients who are old enough) should be advised about the risk of osteonecrosis after surgery.

    The authors further note that more study is needed to evaluate the role of treatment type in the development of SCFE. Even with 70 patients enrolled in this study, it wasn’t enough to generate statistically significant findings in some areas. They concluded that the true difference among their three groups with different treatment approaches wasn’t as clear as if there had been more people in the study.

    Help in Diagnosing Loeys-Dietz Syndrome in Children

    There’s a new disorder among children called the Loeys-Dietz syndrome. Physicians and geneticists from The Johns Hopkins University write about it in this article. They studied 65 children who had a diagnosis of Loeys-Dietz confirmed with genetic testing.

    What is Loeys-Dietz syndrome? It’s a disorder that affects multiple body systems including the skeletal system, the heart (cardiac) and blood vessels (circulatory system), and the skin. This problem is caused by a genetic mutation. They’ve even found the gene that has gone bad: TGF-ßR1 or TGF-ßR2. This particular gene is responsible for coding the growth factor-beta receptor.

    That probably doesn’t mean much to the average reader. But for the affected child it can mean the formation of aneurysms (weak blood vessel walls that can burst and bleed), scoliosis (spinal curvature), very thin skin, chest deformities, and severe foot deformities (clubfoot). Other problems observed in children with Loeys-Dietz include an unstable cervical spine (neck), small jaw, cleft palate, hip dysplasia, and many other skeletal deformities.

    Some features of this disorder are very distinctive (e.g., cleft palate, clubfoot). There may be a condition called hypertelorism, which is an abnormal distance between the eyes. Craniosynostosis is another telltale skeletal feature. This refers to the premature fusion of the cranial (skull) bones. Early fusion of this type prevents the brain from growing normally. The child ends up with an abnormally shaped head and face. Delays in development (especially motor skills) are also common when brain growth is restricted.

    There are several other conditions that have similar effects (e.g., Ehlers-Danlos syndrome, Marfan syndrome, Shprintzen-Goldberg syndrome) so the diagnosis isn’t always straight forward. That’s why the authors put this information together. By studying 65 children with Loeys-Dietz syndrome and comparing them to these other connective tissue disorders, they were able to better define this newly recognized syndrome.

    For example, two distinctive signs of Marfan syndrome are also present in up to one-fourth of all children with Loeys-Dietz syndrome. One is the positive thumb or Steinberg sign. The other is the positive wrist or Walker-Murdoch sign. If the patient puts the thumb across the palm and closes the fingers and the thumb pokes out the other (little finger) side, there’s a positive thumb sign. If the patient can circle the forearm with the thumb and little finger of the other hand and cover the entire nail of the little finger with the thumb, there is a positive wrist sign.

    Of course, the condition can be confirmed with genetic testing and that may be important given the seriousness of the aneurysms (they can be life-threatening). Early treatment to control the amount of blood pressure against the thin-walled vessels can help prevent rupture and hemorrhage. Malformations of the upper cervical spine may require fusion surgery to stabilize the head and neck and keep pressure off the spinal cord. Scoliosis may be treated with spinal bracing and/or fusion surgery when the curvature is large enough to compromise lung function (breathing) or digestion.

    The authors conclude that the risks of untreated problems in Loeys-Dietz such as aneurysms and neck instability make it imperative that this problem is diagnosed early. In the past, children with the clinical features of Loeys-Dietz as described here would have been considered a subgroup of the Marfan syndrome. The discovery that there are mutations in a specific gene (TGF-ßR1 or TGF-ßR2) puts this syndrome in a category by itself. Pediatricians and primary care physicians who see children with combinations of any of the features mentioned should refer the patient to a genetic specialist as soon as possible.