Developmental Dysplasia of the Hip (DDH), or hip dysplasia, is the most common hip problem in infants and young children. Our bones develop over time from soft cartilage, and the hip joint (a “ball-and-socket” joint) is particularly underdeveloped at birth. Because of this, babies’ hips can be susceptible to problems when they are newborns. DDH is when a baby has a hip joint that is abnormally loose (the “ball” can move around too much in the “socket”), or completely out of the socket altogether (dislocated). DDH in infancy can cause children to later develop arthritis in the hip joint at a young age, and/or require a hip replacement in young adulthood. This is particularly true if DDH is not caught and treated as early as possible in life.
Kishore Mulpuri and Emily Schaeffer have led the transition of an international study on children with dislocated hips into an International Hip Dysplasia Registry (IHDR) with a truly global impact. This registry enrolls patients at the time of diagnosis of DDH and follows them through their treatment process all the way until they reach 18 years of age and are considered skeletally mature (bones are fully developed). This is the first DDH study of its kind in size and scope, and has placed BC Children’s Hospital as a world leader in the research and care of children’s hip conditions. The registry currently has 18 hospitals participating worldwide, spanning five countries and four continents. The global reach of the study is truly unprecedented in children’s orthopaedics, with developing and underdeveloped countries such as India contributing to this important work. This registry will allow us to better understand how to screen, diagnose and treat infants and children with hip dysplasia around the world, leading to happier, healthier and more mobile lives.
SLIPPED CAPITAL FEMORAL EPIPHYSIS (SCFE)
SCFE is the most common hip problem in teenagers. SCFE occurs when the head of the femur (top of the thigh bone) “slips” off the neck of the femur, causing problems with the alignment of the hip joint, and leading to groin, hip and knee pain that gets worse over time. Surgery is almost always needed to treat teenagers with SCFE; however, there are different surgery options with potential complications associated with each type. SCFE can lead to chronic pain, arthritis of the hip joint, and/or the need for a hip replacement at a young age.
In collaboration with Boston Children’s Hospital, Kishore Mulpuri is the Co-Principal Investigator and Emily Schaeffer is the scientific lead in the development of the largest international prospective study for all children that have been diagnosed with and treated for SCFE with the intention of following them until they reach the age of 18. Most existing information on SCFE has been Development of this registry will help us to better understand the outcomes of different surgeries and treatment approaches, ultimately improving the quality of care and quality of life for these patients.
CHILDREN WITH CEREBRAL PALSY
Children with cerebral palsy (CP) are particularly at risk of having their hips displace or dislocate (come out of the socket) because of muscle imbalances. Hip displacement can be very painful and greatly impact their quality of life. If a child’s hip becomes completely dislocated, or remains out of correct alignment in the socket for a long period of time, the harder it is correct.
Hip Surveillance Programs in Sweden and Australia have been established that enroll all children with CP as early as possible to monitor their hips. These programs have shown that the earlier hip displacement is caught, the easier it is to treat with less invasive surgery options.
Acting as project Medical Lead, Kishore Mulpuri worked in partnership with Child Health BC (CHBC) to establish a similar hip surveillance program for all children with CP across the province of BC, and it is the first formal surveillance program in North America. Currently, all children in the province with a diagnosis of CP are being enrolled in the program by family doctors, pediatricians, community physiotherapists and the CP clinic team here at BC Children’s Hospital.
Additionally, The Hospital for Sick Children in Toronto, Ontario is leading an international study looking at the impact of different treatments for hip displacement on the quality of life for children with CP. Centres around the world are participating in this study, and Kishore Mulpuri and the Orthopaedic research and clinic team here at BC Children’s Hospital are actively contributing to this effort.
And last but not the least, much like the DDH and SCFE registries, we are currently working on the development of a registry for all patients with CP who will be enrolled and followed until the age of 18. This will allow us to more completely understand the impact of all clinical and surgical treatments for CP – including botox injections for muscle spasms, braces and splints, muscle releases and hip surgeries – on the quality of life and functional outcomes for these patients over the long term.
Perthes’ Disease is a hip condition most common in children aged 5 to 9 years and is caused by a lack of blood flow to the head of the femur. Without proper blood flow, the bone tissue “dies”, making the femoral head weak and easy to deform. Perthes can be very painful, and can lead to lifelong hip problems, including limited range of motion, and may lead to arthritis and early hip replacement for these children.
A children’s hospital in Dallas, Texas is leading the IPSG efforts with an international study on these patients, with centres around the world contributing data. Dr. Kishore Mulpuri is the site lead here at BC Children’s Hospital, and we are actively enrolling patients in this study.
FREQUENTLY ASKED QUESTIONS:
1. WHAT ARE THE STATISTICS ON DELAYED HIP DYSPLASIA IN BC?
Comprehensive statistics are not kept specifically for delayed hip dysplasia, mostly because we lack a standard way to define “delayed hip dysplasia”. This lack of agreement in diagnosis, as well as in other areas regarding screening and treatment decisions, is a large part of the reason why hip dysplasia continues to be such a problem not only in BC, but around the world. The majority of patients with severe late hip dysplasia are seen at BC Children’s Hospital by the Pediatric Orthopaedic Surgery Department. In the last year, there have been approximately 60 infants diagnosed with hip dysplasia that required treatment after the age of three months, with the majority of those diagnosed having the most severe form of hip dysplasia – a completely dislocated hip, meaning the head of the femur (thigh bone) is completely outside of the hip socket. This form of hip dysplasia requires the most extensive treatment when caught late, and comes with potential for more long-lasting and worse complications.
2. WHAT HAPPENS TO A CHILD WHO IS NOT DIAGNOSED AT BIRTH?
When a child is diagnosed at birth or shortly after, they can be successfully treated for hip dysplasia without having to undergo any surgery most of the time (~95% of cases). Instead, babies diagnosed early are most often put in a brace or harness to help their hips develop in the correct position as they grow, and no further treatment is necessary. When a child is not diagnosed right away because their hip dysplasia is missed early on, quite often the child will require more complex forms of care, including potential multiple operations, to correct the hip dysplasia. The surgeries needed when diagnosis is delayed are often far more invasive, and the child often still needs more surgeries later in childhood, as a teenager, or as a young adult.
3. HOW MUCH DO YOU THINK IT’S COSTING THE BC HEALTH CARE SYSTEM (YEARLY) AS A RESULT OF NOT BEING ABLE TO DIAGNOSE AT BIRTH?
The true cost of delayed hip dysplasia is difficult to estimate given the issues around diagnosis and screening. However, as an example of both the excess hospital costs, we have a patient at BC Children’s Hospital who was diagnosed with hip dysplasia very late, at 3 years old. She is now 17 years old, and has had 15 surgeries throughout her childhood and adolescence. Even with those 15 surgeries, she will still require a total hip replacement surgery in her early-to-mid-20s. Total hip replacements are expensive surgeries, and the number being done on younger patients is consistently on the rise. More of these hip replacements than we can currently appreciate may be directly due to undiagnosed hip dysplasia in infancy and childhood.
Beyond the high costs associated with any individual significant surgical procedure, there are also additional costs associated with multiple clinic visits that happen pre- and post-operatively for each surgery. For each surgical procedure, the patient attends at the very least, one pre-operative clinical appointment to discuss options and the procedure with the orthopaedic surgeon. Depending upon the complexity and recovery time of the surgery, there will also be multiple post-operative visits directly as a result of the surgical procedure to check recovery progress.
Besides the direct costs incurred to the BC health care system as a direct result of required clinical care, there are also a number of indirect social and economic costs to the family that are extremely impactful, and far more difficult to put a number on. Particularly in a geographically diverse region like BC, families may need to travel quite a distance to come to their appointments at BC Children’s Hospital. With that comes associated travel and accommodation costs, as well as costs for lost wages due to parents needing to take time off work to attend clinic visits. There is also the potential long-term costs of the child’s physical disability resulting from the lengthy care process.
4. TELL US WHAT A CHILD GOES THROUGH AS A RESULT OF DELAYED DIAGNOSIS?
As in question 3, a child who receives a delayed diagnosis may need to go through many surgeries throughout their childhood, and these surgeries are often very invasive and painful. Surgery for hip dysplasia that is diagnosed late often involves reshaping of the pelvis and cutting and changing the angle of the femur (thigh bone). These surgeries can have long recoveries, potential painful complications and can result in a child needing to wear a partial body cast during the healing stages. The surgeries themselves can also leave the child with limb length discrepancies – where one leg is shorter than the other. This can lead to additional surgeries and/or difficulty walking or participating fully in physical activities. Delayed diagnosis also increases the chances that they will develop arthritis in their hip joint in early adulthood, or even require a total hip replacement in their 20s or 30s.
5. AREN’T BABIES CURRENTLY TESTED AT BIRTH? WHY IS THIS NOT ENOUGH?
Yes, currently all babies should be tested by a physical examination at birth to check for hip dislocation or instability. However, not all forms of hip dysplasia can be detected by physical exam; additionally, there is quite a lot of variability in expertise and awareness of screening and examination for hip dysplasia depending on where the child is born. A lack of awareness of screening practices, a lack of expertise in performing the physical exam, and the difficulty in diagnosing potentially borderline cases result in delayed or missed diagnoses that lead to the issues described above. Consequently, more must be done to improve our current screening practices and provide the best care possible to children throughout British Columbia, Canada and the rest of the world.
6. WHY IS IT IMPORTANT TO HAVE A STANDARDIZED METHOD OF TESTING?
It is extremely important to have a standardized method of testing to ensure that all children, regardless of location or socioeconomic status receive the same quality of care. Standardized testing methods will help to decrease the variability in screening practice, and increase our ability to create strong clinical practice guidelines to help health care workers that may not have the same access to resources and training in this area. With standardized testing methods, we should be able to cut down on the number of children who are delayed in their diagnosis of hip dysplasia; however, we should also be able to cut down on a number of unnecessary tests and treatments that can happen with the current testing methods.
7. IS THERE AN URGENCY TO DEVELOPING THIS STANDARDIZED METHOD OF TESTING?
Yes. The current method of testing here in BC is for every baby to receive a physical exam at birth, and for babies thought to be at high risk for hip dysplasia to also be tested by an ultrasound exam. These screening procedures are based on the latest clinical practice guidelines for hip dysplasia; however, this program is not necessarily easy to apply for geographically spread out regions like BC. Additionally, ultrasound is not even available for ready use, or lacks trained personnel in some areas of British Columbia, and is not available at all as a screening option in many developing and underdeveloped areas of the world. Developing standardized testing methods while also adapting to local resources will be extremely important to improve care not only here in British Columbia, but also around the world, particularly in areas with large populations such as India, China and Africa.