Patients with neuromuscular disease and imbalance present a particularly challenging clinical situation for the orthopaedic hip surgeon. The cause of the neuromuscular imbalance may be intrinsic or extrinsic. Intrinsic disorders include those in which the hip is in development, such as cerebral palsy, polio, CVA, and other spinal cord injuries and disease. This can result in subluxation and dislocation of the hip in growing children, and subsequent pain, and difficulty in sitting and perineal care. Extrinsic factors involve previously stable hips and play a secondary role in the development of osteoarthritis and contractures in later life. Examples of extrinsic factors are Parkinson's disease, dyskinesis, athetosis, and multiple sclerosis. Goals of treatment in adults with pain and dysfunction in the setting of neuromuscular imbalance are to treat contractures and to perform salvage procedures to improve function and eliminate pain. Treatment of patients with neuromuscular imbalance may include resection arthroplasty (Girdlestone), arthrodesis, or total hip arthroplasty. Resection arthroplasty is typically reserved for patients that are non-ambulatory, or hips that are felt to be so unstable that arthroplasty would definitely fail due to instability. In modern times arthrodesis has limited use as it negatively impacts function and self-care in patients with neuromuscular disorders. Total hip arthroplasty has the ability to treat pain, relieve contractures, and provide improved function. Due to the increased risk of instability, special considerations must be made during primary total hip arthroplasty in this patient cohort. Risk of instability may be addressed by surgical approach, head size, or use of alternative bearing constructs. Posterior approach may have increased risk of posterior dislocation in this patient group, particularly if a posterior capsular repair is not possible due to the flexion contractures and sitting position in many patients. Surgeons familiar with the approaches may utilise the anterolateral or direct anterior approach judicially. Release of the adductors may be performed in conjunction with primary total hip arthroplasty to help with post-operative range of motion and to decrease risk of instability. In a standard bearing, the selected head size should be the largest that can be utilised for the particular cup size. Rigorous testing of intra-operative impingement, component rotation, and instability is required. If instability cannot be adequately addressed by a standard bearing, the next option is a dual mobility bearing. Multiple studies have shown improved stability with the use of these bearings, but they are also at risk for instability, intraprosthetic dislocation, and fretting and corrosion of the modular connections. Another option is a constrained liner. However, this results in reduced range of motion, and an increased risk for mechanical complications of the construct. The use of a constrained liner in a primary situation should be limited to the most severe instability cases, and the patient should be counseled with the associated risks. If total hip arthroplasty results in repeated instability, revision surgery or Girdlestone arthroplasty may be considered

Introduction. Limb deformity is usually assessed clinically assisted by long leg alignment radiographs and further imaging modalities (MRI and CT). Often decisions are made based on static imaging and simple gait interpretation in clinic. We have assessed the value of gait lab analysis in surgical decision making comparing surgical planning pre and post gait lab assessment. Materials & Methods. Patients were identified from the local limb reconstruction database. Patients were reviewed in the outpatient clinic and long leg alignment radiographs and a CT rotational limb profile were performed. A surgical plan was formulated and documented. All patients then underwent a formal gait lab analysis. The gait lab recommendations were then compared to the initial plan. Results. Twelve patients (8 female) with mean age of 14 (range 12–16) were identified. Nine were developmental torsional malalignments, one arthrogryposis, one hemiparesis secondary to spinal tumour resection and one syndromic limb deficiency. The gait lab recommended conservative management in four patients and agreed with eight surgical plans with one osteotomy level changing. Five patients are post-operative: two bilateral distal tibial osteotomies, two de-rotational femoral osteotomy with de-rotational tibial osteotomies and one bilateral femoral de-rotational osteotomies. Conclusions. Limb deformity correction is major surgery with long rehabilitation and recovery period. Gait lab analysis can identify who would benefit from conservative management rather than surgery with our study showing changes to surgical planning in one third of patients. The gait lab analysis helps to identify patients with functional and neuromuscular imbalances where correcting the bony anatomy may not actually benefit the patient

There has been a reluctance, until relatively recently, to consider replacement of the hip in patients with substantial neuromuscular imbalance. This relates to many factors, including the young age of many (such as cerebral palsy in the older teen and young adult), developmental anatomic abnormality, oft-present poor bone health, neuromuscular imbalance, and the risk of complication; especially dislocation. Mental retardation also introduces challenges with rehabilitation and an increased burden on the family and societal support systems if the outcome is to be maximised. With the development of newer techniques and technology, and the emergence of encouraging outcome studies, these patients can be more easily offered predictable relief of pain, a reasonable chance of improved function, longevity of the reconstruction, and an acceptable risk of complication. A large number of background neurological diagnoses can lead to hip degeneration, or can introduce increased complexity during management of hip degeneration unrelated to that background. Be that as it may, a short list of fundamental questions is common to all and will help guide management:. Important questions to be addressed include:. 1. Did the NV imbalance precede skeletal development? This relates to the dependence of skeletal shape and size on the loads being placed upon it: hence “Form Follows Function”. The shape and size of the hip, and location of the femoral head, will be much different in the young adult with spastic dislocation due to cerebral palsy, when compared with the elderly adult with a late onset CVA-related spasticity superimposed on hip degeneration. 2. Is the muscle tone which will support the hip arthroplasty predominantly spastic or flaccid? In each there is a risk of dislocation, which needs to be addressed at the index procedure, but in spasticity there is the added question as to what tissues need to be released or de-functioned so as to alter the magnitude and direction of the joint reaction forces. 3. Is pain the main reason for consultation? Because pain relief is the most predictable outcome that we can offer, it should guide the indications and timing of intervention. Replacement of the NM hip to improve function, in the absence of pain, should be approached with great caution

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.