hinking Outside the Box for Proximal Femoral Focal Deficiency
First-Place Article, 2016 Paper of the Year Competition

by Ruth Byers, OTC

Inside a box of fiberglass are 10 rolls of unlimited potential. While we can use fiberglass to fix fractures, it can also be used as a problem-solving tool for patients and their families. I am an Orthopaedic Technologist who specializes in pediatrics. Pediatrics is a complex discipline. There is the initial orthopaedic problem, and while assessing that, you are also looking at the child’s development mentally and physically. In addition to the patient, you are also taking care of the family, grandparents, caregivers, school administration and regulations. When you meet your patient for the first time, you are sizing them up and they are sizing you up.

This case report is of a male child diagnosed with proximal femoral focal deficiency, his mother, the commitment of the community he lives in, and his surgeon’s persistence to treat the problem. The saying “it takes a community to raise a child” is true and plays a large role in the success of this child’s treatment.

According to research proximal femoral focal deficiency (PFFD) is the third most common limb length deficiency effecting children (1). Fifty percent of the time there is associated coxa vara and 15% of the time it is bilaterally affected (2). There are 4 classes of PFFD. He was diagnosed with class A. He does have a femoral head and he did have some valga deformity of the hip. The surgeons could work with and consider a lengthening in the future.

Case Report
Ralph first appeared in the orthopaedic clinic in 2009 as a 3 year old, referred to us for hip dysplasia. His mother was a poor historian, and English is not her primary language. He was a full-term uncomplicated twin delivery that started walking at 11 months old. He had no other developmental issues. His mother noticed a marked discrepancy in his limb lengths compared to his twin sister. His gait was abnormal. He toe-walked on his right foot to equalize his pelvis to ambulate. X-rays showed that he had severe coxa vara of his hips and shortened femur with extreme femoral bowing. He was given the diagnosis of proximal femoral focal deficiency (PFFD) class A. In March 2009, he was scheduled for a proximal femoral osteotomy (PFO) and a right adductor tenotomy to relocate his hips into a proper place for development of his acetabulum. He spent three weeks in bilateral long leg casts and an abduction bar. He was fit for a wheelchair and then lost to follow up as he and his mother disappeared from care.

Ralph was lost to follow up for six years. He reappeared, as patients occasionally do, in April 2015. His mother, an African immigrant, was overwhelmed by the healthcare system and decided to ignore the problem. She was strongly encouraged by a close neighbor to bring him back to the clinic to be reevaluated and pursue treatment. Ralph had started to experience increased pain in his right leg and foot. His leg length discrepancy had increased to 10.3 cm in difference in those 6 years and his projected growth difference was estimated to be 13cm. Bone age study projected that he has 6 years of growth remaining. In his gait, he compensated with extreme ankle equinus on the right. He had never worn an external shoe lift or device at any time. Ralph personally expressed the desire for limb equalizing surgery. He was tired of working hard to walk and didn’t like being different. His mother expressed concern for increased back problems since he was walking with such an extreme limp. He could run but couldn’t participate in sports. He wanted to be a normal kid. A plan was made to begin the process of limb lengthening, an arduous task that required coordination with multiple services. The process would take at least one year.

It was decided that he would have the following three staged procedure: right hip reconstruction, an ephiphysodesis on the left leg to slow down growth, and start limb lengthening on the right using a Biomet external fixator. We had concerns that the single mother, whose English is limited and time was precious as she had four other children, would be at high risk for postoperative difficulties given the intense postoperative regiment that was going to be required each week for the next 12 months. Ralph needed to be non-weight bearing and use a wheelchair or walker for mobility for the full year. He needed help with all aspects of personal care and development. To complicate matters more, the family lives 45 miles away from the treatment center and he required weekly follow up visits.

This is where the saying “it takes an entire village to raise a child” comes in. The neighbor who brought Ralph and his mother back to the clinic arranged a list of approved neighbors to bring him to his weekly clinic visits. A total of 16 couples were on the list, approved by the mother to bring him to his clinic visits since she financially could not take off work to bring him to the visits. The people on the list come from varied ages, economic and career backgrounds. They all expressed the same innate human desire to help this child develop and be a normal productive individual in society. They saw great potential in this child and his future and were determined to help him overcome his serious leg length discrepancy.

The first stage of his surgeries was a triple pelvis osteotomy, hardware removal to remove the hardware from the previous surgery and a valgus producing osteotomy, which he underwent in July 2015. He did well.

In January 2016, he underwent a right femoral osteotomy and a unilateral Biomet external fixator with 6 hydroxipapatite coated pins was placed to facilitate the lengthening. At the same time, he also had a distal femoral epiphysiodesis to slow down growth on the left femur. He required no DVT prophylaxis and he had a basic pediatric narcotic regime for pain management.

At his first postoperative visit, the initial leg lengthening was started. Everything looked fine and we started the physical process of lengthening at 4 “clicks” a day. A click moves the rod a small fraction of a millimeter. Ralph followed up a week later and had x-rays. There was no change in his osteotomy on radiographs. However, his mother started lengthening the wrong way even though hands-on, verbal and written instructions were given to her. She was given further instructions and the lengthening began in the correct direction.

At 4 weeks postoperatively, the second problem developed. The family decided to lengthen it twice as fast as prescribed, which caused his knee to start to a contracture and a foot drop due to the extreme stretching of all the nerves, vessels and skin. This caused several complications, which required thinking outside of the box. With his external fixator in place, a knee immobilizer would not work and an AFO for his foot would take too long to make. I made him a long leg, well-padded, removable splint with his knee in as much extension as I could get. I placed lateral cut outs for the external fixator to allow for lengthening. I used the fiberglass, terry net stockinette, sifoam, adhesive edging and sling on a roll. This splint lasted for more than five weeks. Despite our efforts, at another follow up visit, the physician noticed that he was getting more and more of a flexed knee contracture. It was then discovered that mother made the decision to ignore wearing the custom splint the physician recommended 20 hours a day. The excessive lengthening exacerbated his knee contracture, and caused his knee to have subluxation due to the increased pressure of the lengthening.

In April 2016, it was decided to take him back to surgery to address his knee subluxation issue by extending his external fixator to his tibia. Once in surgery, the initial plan was aborted and it was decided to do a knee manipulation, use a nerve stimulator, and inject 200 units of Botox to his gracilis, semitendinosus, semimembranosus, and biceps femoris. He was fit for a hound dog knee brace while under anesthesia. He would continue to use the custom molded knee immobilizer until the hound dog extension brace arrived. Postoperatively he was able to continue to ambulate with the use of his walker. His post op pain was controlled by oxycodone and Valium.
At his first visit after the knee manipulation and botox injection, his knee continues to have stiffness despite physical therapy. There has been improvement in the motion of his knee with stress. He has had periodic pin site infections. Ralph had increased peroneal nerve pain and was started on Gabapentin at night to reduce the nerve pain. These are not unexpected problems with lengthenings.

The village steps in again. His mother decided that after a year of stress, Ralph should move in with the neighbor, where he gets personal attention, encouragement to do his exercises, help with personal care, pain management, emotional and nutritional support, and help with his schooling. When we saw Ralph after this, his face showed the relief and happiness that having one-on-one attention provides and the caregivers are happy also with the arrangement.

The story of Ralph continues. With children the story never ends until they reach adulthood. Pediatric healthcare is an ongoing evolution and we as health1care professionals have to look at each child as an opportunity to think outside the box. Look for a Ralph update at the end of 2016.

About the Author:
Ruth is a native Illinoisan transplanted to Chapel Hill, North Carolina after college. She has been involved in all aspects of Orthopaedics: from inpatient nursing, operating room scrub tech, to outpatient clinics, over the last thirty-seven years. Ruth has a special interest in pediatric orthopaedics, and has been working in pediatric orthopaedics for the last nineteen years with the University of North Carolina. UNC has enabled Ruth the great opportunity to think outside the box and teach casting and immobilization or the last fifteen years. Ruth’s outside interest include traveling, quilting, cooking and enjoying her friends and loving life.