Abstract
Cerebral palsy (CP) arises due to an injury in the immature brain, and—even in patients in whom the disorder is considered to be nonprogressive—the resulting disability is lifelong. During the growth of a child with CP, deformities may appear, get worse, and lead to decreased function and ability to perform daily life activities, depending on the orthopedic and general management scheme implemented. CP is therefore largely perceived as a childhood disease, and the need for early management is well known. Long-term therapy strategies are required, because the groundwork for the optimal performance of activities of daily living in adulthood is laid in childhood.
Many adults with CP report decreases in activity and body functions [1–7]. Pain, increasing musculoskeletal problems, fatigue, and bladder and bowel problems are common in adult patients with CP [8–11]. Nevertheless, their levels of life satisfaction are similar to those of the average population [3]. In contrast to the general opinion, they may experience a good quality of life despite their severe handicap [12].
Well-being and self-perceived health are related to pain and deteriorating function, but are not related to the level of functioning itself [6, 13]. According to the opinions of adult CP patients, the most important issues to them are communication, daily life activities, mobility, and walking [14].
Focusing more specifically on the CP hip, its physiopathology is directly related to its severity, i.e., delayed or nonacquired weight-bearing. Hip subluxation and dislocation occur in nonwalking patients, with femoral and acetabular dysplasia arising due to the nonphysiological constraints on the hip joint present during persistent sitting with muscle imbalance, along with persistent femoral anteversion and coxa valga, as well as posterolateral acetabular dysplasia (Fig. 1).
3D CT reconstruction: posterolateral dysplasia in a nonwalking GMFCS 5 CP patient due to an underlying muscle imbalance, bony dysplasia, and persistent sitting
Therefore, in nonambulatory adult CP, hip pain is one of the main complaints—either provoked, linked to position, or spontaneous [15]—and it may also occur in reduced hips. This is one of the most important disabling disorders seen in CP adolescents or adults. It conditions the ability to stand for transfers [16] and walk, or to sit comfortably. Therefore, we need to establish the long-term consequences of unadapted treatments and hurdles for adults with CP. With increasing body age, patients face osteoarthritis, leading to seating instability or disabilities, diffculties with care, and decubital ulcers (Fig. 2). For women, there can be problems with menstruation, sexual activity, or pregnancy. Major hip pain increases muscle tone and decreases upper limb use, finally leading to communication problems and withdrawal from the outside world.
Twenty-eight-year-old adult with a history of left femoral head neck resection with major pain, inability to sit, and skin problems. In this GMFCS 5 CP patient, the decision to perform this type of surgery was made based on no patient walking potential. There was no evaluation and integration of long-term general health, sitting potential, and comfort during daily life during the initial surgical decision-making process
The treatment strategy must be adapted for severely involved patients with hip problems. Recent advances in seating technology (Fig. 3), adapted focal tone treatment (Fig. 4), and the integration of surgical techniques such as total hip prosthesis [16] may be proposed (Fig. 5). These surgeries must always be integrated into a general therapy program, such as regular gastrointestinal, nutritional, and respiratory checkups and treatment.
Twenty-six-year-old GMFCS 5 CP patient with major dystonia, athetosis, and a painful dislocated left hip who was initially installed in a rigid seating system in a hip-abduction/external-rotation position. The pain subsided when the patient was later installed in an elastic carbon-fiber seating system with free internal hip rotation
The treatment approach for the hips of a child with CP should focus on their expected activities of daily life during adulthood, and should relate to the severity of the pathology of the patient, rather than the deformity itself. It is essential to find intervention strategies that promote good health over the course of the patient's lifetime. It was recently pointed out that the most important challenge is to meet the needs arising from functional problems such as body deterioration, as well as those relating to self-esteem and aspects of autonomy [17].
With regard to long-term treatment strategy planning, the International Classification of Functioning, Disability and Health (ICF) [18] can aid in the creation of an evaluation scheme for all CP patients, and when establishing therapy objectives regarding daily living. The ICF introduces the concepts of activity and participation in the context of personal and environmental factors.
In this context, activity means the execution of a task, and participation is the involvement of the patient in a life situation. These are objective concepts that depend on personal factors and each patient's environment. The patient may experience difficulties in executing certain activities that may create problems in situations encountered in daily life. There are two qualifiers for both activity and participation: capacity and performance. Capacity is the aptitude of the patient when executing a task, and performance is the act of executing a certain activity in daily life.
The concept of quality of life (QL) evaluation is not clearly integrated into the ICF. Health-related quality of life is multidimensional, and considers the following aspects according to the concept of health proposed by the World Health Organisation (WHO) [19]: physical (autonomy, physical capacities, capacities to execute daily life activities, pain, …); psychological (well-being, anxiety, emotion, …); and social (relationships to family, friends, professionals, …) [20–22].
QL is a subjective measure. It attempts to quantify the subjective feeling reported by the subject him/herself.
Considering daily life, and reviewing the literature in this field, other concepts may also be important and should therefore be evaluated, such as independence and caregiver burden/enjoyment.
It is impossible for the orthopedic surgeon to take into account all of these aspects and integrate them all into the solution of a specific orthopedic problem. However, it may be interesting to integrate some of the concepts (Table 1) into the discussions with the family and the caregiving team, who are facing the daily functional issues arising from the hip problem in the CP patient and expect a tangible treatment strategy.
Questionnaires employed to explore activity and participation with regard to capacity and performance
Type (of questionnaire): 1 = child/adolescent self-questionnaire, 2 = parent questionnaire, 3 = care-giver questionnaire
For both walking and nonwalking young CP patients, from the long-term perspective of performing activities of daily living, it is important to keep the hips well centered with a level pelvis in order to achieve good sitting comfort, prevent pain, and avoid biomechanical conditions that can allow scoliosis to evolve. Surgery of a dislocated hip may not cure sitting and positioning asymmetry [23], and may even worsen it when performed unilaterally [24]. Bilateral surgery may therefore be needed to correct a pelvic imbalance that would hinder activities of daily living.
In older CP patients with femoral head deformity, as long as the femoral physis is open, reduction can result in a painless, functional hip provided that it is taken care of properly [16].
Reconstructive hip surgery in severely dysplastic hips or in the presence of osteoarthritis should be first weighed against its potential complications and the need for a complete preoperative work-up in the severely involved CP patient to decrease complications. Considering daily life activities, reducing spasticity using focal hip objective-based toxin injections to improve comfort and maintain symmetric motion for daily care may be more useful than having a located hip as documented on an X-ray [25].
For reliable orthopedic management of hip problems in CP patients, it is most important to be aware of long-term issues of daily living. It's essential to ask the “good” questions to be able to propose a daily living objective based therapy program.
Daily living has to be the basis for long-term decision-making regarding CP hip problems.