We investigated the function of biceps in 18 patients (19 shoulders) with lesions of the rotator cuff. Their mean age was 59 years. Another series of 18 patients (19 shoulders) with normal rotator cuffs as seen on MRI acted as a control group. Their mean age was 55 years. A brace was used to maintain contraction of biceps during elevation. Anteroposterior radiographs were obtained with the arm elevated at 0°, 45° and 90° with and without contraction of biceps. The distance between the centre of the head of the humerus and the glenoid was compared in the two groups. We found that in the group with tears there was significantly greater proximal migration of the head of the humerus at 0° and 45° of elevation without contraction of biceps but depression of the head of the humerus at 0°, 45° and 90° when biceps was functioning. We conclude that biceps is an active depressor of the head of the humerus in shoulders with lesions of the rotator cuff

This study evaluates the position of the long head of biceps tendon using ultrasound following simple tenotomy, in patients with arthroscopically repaired rotator cuff tears. In total, 52 patients with a mean age of 60.7 years (45 to 75) underwent arthroscopic repair of the rotator cuff and simple tenotomy of the long head of biceps tendon. At two years post-operatively, ultrasound revealed that the tendon was inside the bicipital groove in 43 patients (82.7%) and outside in nine (17.3%); in six of these it was lying just outside the groove and in the remaining three (5.8%) it was in a remote position with a positive Popeye Sign. A dynamic ultrasound scan revealed that the tenotomised tendons had adhered to the surrounding tissues (autotenodesis).The initial condition of the tendon influenced its final position (p < 0.0005). The presence of a Popeye sign was statistically influenced by the pre-operative co-existence of supraspinatus and subscapularis tears (p < 0.0001). It appears that the natural history of the tenotomised long head of biceps tendon is to tenodese itself inside or just outside the bicipital groove, while its pre-operative condition and coexistent subscapularis tears play a significant role in the occurrence of a Popeye sign

Objectives. Traumatic brachial plexus injury causes severe functional impairment of the arm. Elbow flexion is often affected. Nerve surgery or tendon transfers provide the only means to obtain improved elbow flexion. Unfortunately, the functionality of the arm often remains insufficient. Stem cell therapy could potentially improve muscle strength and avoid muscle-tendon transfer. This pilot study assesses the safety and regenerative potential of autologous bone marrow-derived mononuclear cell injection in partially denervated biceps. Methods. Nine brachial plexus patients with insufficient elbow flexion (i.e., partial denervation) received intramuscular escalating doses of autologous bone marrow-derived mononuclear cells, combined with tendon transfers. Effect parameters included biceps biopsies, motor unit analysis on needle electromyography and computerised muscle tomography, before and after cell therapy. Results. No adverse effects in vital signs, bone marrow aspiration sites, injection sites, or surgical wound were seen. After cell therapy there was a 52% decrease in muscle fibrosis (p = 0.01), an 80% increase in myofibre diameter (p = 0.007), a 50% increase in satellite cells (p = 0.045) and an 83% increase in capillary-to-myofibre ratio (p < 0.001) was shown. CT analysis demonstrated a 48% decrease in mean muscle density (p = 0.009). Motor unit analysis showed a mean increase of 36% in motor unit amplitude (p = 0.045), 22% increase in duration (p = 0.005) and 29% increase in number of phases (p = 0.002). Conclusions. Mononuclear cell injection in partly denervated muscle of brachial plexus patients is safe. The results suggest enhanced muscle reinnervation and regeneration. Cite this article: Bone Joint Res 2014;3:38–47

Between March 1994 and June 2003, 80 patients with brachial plexus palsy underwent a trapezius transfer. There were 11 women and 69 men with a mean age of 31 years (18 to 69). Before operation a full evaluation of muscle function in the affected arm was carried out. A completely flail arm was found in 37 patients (46%). Some peripheral function in the elbow and hand was seen in 43 (54%). No patient had full active movement of the elbow in combination with adequate function of the hand. Patients were followed up for a mean of 2.4 years (0.8 to 8). We performed the operations according to Saha’s technique, with a modification in the last 22 cases. We demonstrated a difference in the results according to the pre-operative status of the muscles and the operative technique. The transfer resulted in an increase of function in all patients and in 74 (95%) a decrease in multidirectional instability of the shoulder. The mean increase in active abduction was from 6° (0 to 45) to 34° (5 to 90) at the last review. The mean forward flexion increased from 12° (0 to 85) to 30° (5 to 90). Abduction (41°) and especially forward flexion (43°) were greater when some residual function of the pectoralis major remained (n = 32). The best results were achieved in those patients with most pre-operative power of the biceps, coracobrachialis and triceps muscles (n = 7), with a mean of 42° of abduction and 56° of forward flexion. Active abduction (28°) and forward flexion (19°) were much less in completely flail shoulders (n = 34). Comparison of the 19 patients with the Saha technique and the 15 with the modified procedure, all with complete paralysis, showed the latter operation to be superior in improving shoulder stability. In all cases a decrease in instability was achieved and inferior subluxation was abolished. The results after trapezius transfer depend on the pre-operative pattern of paralysis and the operative technique. Better results can be achieved in patients who have some function of the biceps, coracobrachialis, pectoralis major and triceps muscles compared with those who have a complete palsy. A simple modification of the operation ensures a decrease in joint instability and an increase in function

We describe an operation to relieve compression of the lateral antebrachial cutaneous nerve at the elbow. Between 1987 and 1997 we operated on seven patients, one with bilateral compression. In two the compression was associated with injury to biceps. A longitudinal or a transverse incision was carried out and the nerve was released from the deep fascia. Partial excision of the biceps aponeurosis was undertaken in the patients who did not have injury to biceps; some additional procedures were required for those patients with injuries. All patients had symptomatic relief

The diagnosis of chronic lesions of the rotator cuff is challenging. We have developed a new index to improve the sonographic diagnosis of chronic tears of the cuff. In a pilot study, we examined 50 asymptomatic healthy volunteers by ultrasound to establish the diameter of the rotator cuff in relation to the tendon of the long head of biceps. Subsequently, the index was calculated in 64 patients who had had shoulder pain for more than three months caused by clinically diagnosed lesions of the rotator cuff. The compensatory hypertrophy of the biceps tendon was quantified sonographically in relation to the diameter of the cuff. Comparison with the contralateral shoulder revealed a significantly higher biceps rotator-cuff ratio (p < 0.05) for patients with torn rotator cuffs. A ratio greater than 0.8 was considered pathological (index positive); the mean ratio in the control group was 0.43. The sensitivity of a positive index was 97.8%, the specificity 63.2%, the positive predictive value 86.3%, and the negative predictive value 92.4% in comparison with surgical findings. Use of the index improves sensitivity in the diagnosis of chronic tears of the cuff by ultrasound

The transfer of part of the ulnar nerve to the musculocutaneous nerve, first described by Oberlin, can restore flexion of the elbow following brachial plexus injury. In this study we evaluated the additional benefits and effectiveness of quantitative electrodiagnosis to select a donor fascicle. Eight patients who had undergone transfer of a simple fascicle of the ulnar nerve to the motor branch of the musculocutaneous nerve were evaluated. In two early patients electrodiagnosis had not been used. In the remaining six patients, however, all fascicles of the ulnar nerve were separated and electrodiagnosis was performed after stimulation with a commercially available electromyographic system. In these procedures, recording electrodes were placed in flexor carpi ulnaris and the first dorsal interosseous. A single fascicle in the flexor carpi ulnaris in which a high amplitude had been recorded was selected as a donor and transferred to the musculocutaneous nerve. In the two patients who had not undergone electrodiagnosis, the recovery of biceps proved insufficient for normal use. Conversely, in the six patients in whom quantitative electrodiagnosis was used, elbow flexion recovered to an M4 level. Quantitative intra-operative electrodiagnosis is an effective method of selecting a favourable donor fascicle during the Oberlin procedure. Moreover, fascicles showing a high-amplitude in reading flexor carpi ulnaris are donor nerves that can restore normal elbow flexion without intrinsic loss

CT arthrography and arthroscopy were used to assess tears of the rotator cuff in 259 shoulders. Tear size was determined in the frontal and sagittal planes according to the classification of the French Arthroscopy Society. CT arthrography had a sensitivity of 99% and a specificity of 100% for the diagnosis of tears of supraspinatus. For infraspinatus these figures were 97.44% and 99.52%, respectively and, for subscapularis, 64.71% and 98.17%. For lesions of the long head of the biceps, the sensitivity was 45.76% and the specificity was 99.57%. Our study showed an excellent correlation between CT arthrography and arthroscopy when assessing the extent of a rotator cuff tear. CT arthrography should, therefore, be an indispensable part of pre-operative assessment. It allows determination of whether a tear is reparable (retraction of the tendon and fatty degeneration of the corresponding muscle) and whether this is possible by arthroscopy (degree of tendon retraction and extension to subscapularis)

We retrospectively identified 18 consecutive patients with synovial chrondromatosis of the shoulder who had arthroscopic treatment between 1989 and 2004. Of these, 15 were available for review at a mean follow-up of 5.3 years (2.3 to 16.5). There were seven patients with primary synovial chondromatosis, but for the remainder, the condition was a result of secondary causes. The mean Constant score showed that pain and activities of daily living were the most affected categories, being only 57% and 65% of the values of the normal side. Surgery resulted in a significant improvement in the mean Constant score in these domains from 8.9 (4 to 15) to 11.3 (2 to 15) and from 12.9 (5 to 20) to 18.7 (11 to 20), respectively (unpaired t-test, p = 0.04 and p < 0.0001, respectively). Movement and strength were not significantly affected. Osteoarthritis was present in eight patients at presentation and in 11 at the final review. Recurrence of the disease with new loose bodies occurred in two patients from the primary group at an interval of three and 12 years post-operatively. In nine patients, loose bodies were also present in the bicipital groove; seven of these underwent an open bicipital debridement and tenodesis. We found that arthroscopic debridement of the glenohumeral joint and open debridement and tenodesis of the long head of biceps, when indicated, are safe and effective in relieving symptoms at medium-term review

Aims

Rotator cuff tear (RCT) is the leading cause of shoulder pain, primarily associated with age-related tendon degeneration. This study aimed to elucidate the potential differential gene expressions in tendons across different age groups, and to investigate their roles in tendon degeneration.

We report the results of performing a pronating osteotomy of the radius, coupled with other soft-tissue procedures, as part of an upper limb functional surgery programme in tetraplegic patients with supination contractures.

In total 12 patients were reviewed with a mean follow-up period of 60 months (12 to 109). Pre-operatively, passive movement ranged from a mean of 19.2° pronation (−70° to 80°) to 95.8° supination (80° to 140°). A pronating osteotomy of the radius was then performed with release of the interosseous membrane. Extension of the elbow was restored postoperatively in 11 patients, with key-pinch reconstruction in nine.

At the final follow-up every patient could stabilise their hand in pronation, with a mean active range of movement of 79.6° (60° to 90°) in pronation and 50.4° (0° to 90°) in supination. No complications were observed. The mean strength of extension of the elbow was 2.7 (2 to 3) MRC grading.

Pronating osteotomy stabilises the hand in pronation while preserving supination, if a complete release of the interosseous membrane is also performed. This technique fits well into surgical programmes for enhancing upper limb function.

We undertook clinical and ultrasonographic examination of the shoulders of 420 asymptomatic volunteers aged between 50 and 79 years. MRI was performed in selected cases. Full-thickness tears of the rotator cuff were detected in 32 subjects (7.6%). The prevalence increased with age as follows: 50 to 59 years, 2.1%; 60 to 69 years, 5.7%; and 70 to 79 years, 15%. The mean size of the tear was less than 3 cm and tear localisation was limited to the supraspinatus tendon in most cases (78%). The strength of flexion was reduced significantly in the group with tears (p = 0.01).

Asymptomatic tears of the rotator cuff should be regarded as part of the normal ageing process in the elderly but may be less common than hitherto believed.

We reviewed 42 consecutive children with a supination deformity of the forearm complicating severe birth lesions of the brachial plexus.

The overall incidence over the study period was 6.9% (48 of 696). It was absent in those in Narakas group I (27.6) and occurred in 5.7% of group II (13 of 229), 9.6% of group III (11 of 114) and 23.4% of group IV (18 of 77).

Concurrent deformities at the shoulder, elbow, wrist and hand were always present because of muscular imbalance from poor recovery of C5 and C7, inconsistent recovery of C8 and T1 and good recovery of C6. Early surgical correction improved the function of the upper limb and hand, but there was a tendency to recurrence. Pronation osteotomy placed the hand in a functional position, and increased the arc of rotation of the forearm. The supination deformity recurred in 40% (17 of 42) of those treated by pronation osteotomy alone, probably because of remodelling of the growing bone.

Children should be followed up until skeletal maturity, and the parents counselled on the likelihood of multiple operations.

We have examined the accuracy of 143 consecutive ultrasound scans of patients who subsequently underwent shoulder arthroscopy for rotator-cuff disease. All the scans and subsequent surgery were performed by an orthopaedic surgeon using a portable ultrasound scanner in a one-stop clinic. There were 78 full thickness tears which we confirmed by surgery or MRI. Three moderate-size tears were assessed as partial-thickness at ultrasound scan (false negative) giving a sensitivity of 96.2%. One partially torn and two intact cuffs were over-diagnosed as small full-thickness tears by ultrasound scan (false positive) giving a specificity of 95.4%. This gave a positive predictive value of 96.2% and a negative predictive value of 95.4%. Estimation of tear size was more accurate for large and massive tears at 96.5% than for moderate (88.8%) and small tears (91.6%). These results are equivalent to those obtained by several studies undertaken by experienced radiologists.

We conclude that ultrasound imaging of the shoulder performed by a sufficiently-trained orthopaedic surgeon is a reliable time-saving practice to identify rotator-cuff integrity.

We have conducted a prospective study to assess the mid-term clinical results following arthroscopic repair of the rotator cuff. Patients were evaluated using the Constant score, subjective satisfaction levels and post-operative ultrasound scans.

Of 115 consecutive patients who underwent arthroscopic repair of the rotator cuff at our institution, 102 were available for follow-up. The mean period of follow-up was for 35.8 months (24 to 73). The mean age of the patients was 57.3 years (23 to 78). There were 18 small (≤ 1 cm in diameter), 44 medium (1 cm to 3 cm in diameter), 34 large (3 cm to 5 cm in diameter) and six massive (> 5 cm in diameter) tears. There was a statistically significant increase in the size of the tear with increasing age (p = 0.0048).

The mean pre-operative Constant score was 41.4 points (95% confidence interval, 37.9 to 44.9), which improved to 84.5 (95% confidence interval, 82.2 to 86.9). A significant inverse association (p = 0.0074), was observed between the size of the tear and the post-operative Constant score, with patients having smaller tears attaining higher Constant scores after repair. Post-operatively, 80 patients (78.4%) were able to resume their occupations and 84 (82.4%) returned to their pre-injury leisure activities. Only eight (7.8%) of 102 patients were not satisfied with the outcome.

Recurrent tears were detected by ultrasound in 19 (18.6%) patients, and were generally smaller than the original ones. Patients with recurrent tears experienced a mean improvement of 31.6 points (95% confidence interval, 23.6 to 39.6) in their post-operative Constant scores. Those with intact repairs had significantly improved (p < 0.0001) Constant scores (mean improvement 46.3 points, 95% confidence interval, 41.9 to 50.6). Patient satisfaction was high in 94 cases (92%), irrespective of the outcome of the Constant score. Recurrent tears appear to be linked to age-related degeneration.

Arthroscopic repair of the rotator cuff leads to high rates of satisfaction (92%) and good functional results, albeit with a recurrence rate of 18.6% (19 of 102).

Introduction

The pathogenesis of rotator cuff disease (RCD) is complex and not fully understood. This systematic review set out to summarise the histological and molecular changes that occur throughout the spectrum of RCD.

The aim of this study was to determine the functional outcome and rate of re-tears following mini-open repair of symptomatic large and massive tears of the rotator cuff using a two-row technique.

The 24 patients included in the study were assessed prospectively before and at a mean of 27 months (18 to 53) after surgery using the Constant and the Oxford Shoulder scores. Ultrasound examination was carried out at follow-up to determine the integrity of the repair. Patient satisfaction was assessed using a simple questionnaire.

The mean Constant score improved significantly from 36 before to 68 after operation (p < 0.0001) and the mean Oxford Shoulder score from 39 to 20 (p < 0.0001). Four of the 24 patients (17%) had a re-tear diagnosed by ultrasound. A total of 21 patients (87.5%) were satisfied with the outcome of their surgery. The repair remained intact in 20 patients (83%). However, the small number of re-tears (four patients) in the study did not allow sufficient analysis to show a difference in outcome in relation to the integrity of the repair.

This study reports the clinical and sonographic outcome of arthroscopic rotator cuff repair in patients aged ≥ 70 years and aimed to determine factors associated with re-tear. A total of 69 consecutive repairs were performed in 68 patients with a mean age of 77 years (70 to 86). Constant-Murley scores were collected pre-operatively and at one year post-operatively. The integrity of the repair was assessed using ultrasound. Re-tear was detected in 20 of 62 patients (32%) assessed with ultrasound. Age at operation was significantly associated with re-tear free survival (p = 0.016). The mean pre-operative Constant score was 23 (sd 14), which increased to 58 (sd 20) at one year post-operatively (paired t-test, p < 0.001). Male gender was significantly associated with a higher score at one year (p = 0.019).

We conclude that arthroscopic rotator cuff repair in patients aged ≥ 70 years is a successful procedure. The gender and age of the patient are important factors to consider when planning management.

Cite this article: Bone Joint J 2013;95-B:199–205.

This is a prospective study of 107 repairs of obstetric brachial plexus palsy carried out between January 1990 and December 1999. The results in 100 children are presented. In partial lesions operation was advised when paralysis of abduction of the shoulder and of flexion of the elbow persisted after the age of three months and neurophysiological investigations predicted a poor prognosis. Operation was carried out earlier at about two months in complete lesions showing no sign of clinical recovery and with unfavourable neurophysiological investigations.

Twelve children presented at the age of 12 months or more; in three more repair was undertaken after earlier unsuccessful neurolysis. The median age at operation was four months, the mean seven months and a total of 237 spinal nerves were repaired.

The mean duration of follow-up after operation was 85 months (30 to 152). Good results were obtained in 33% of repairs of C5, in 55% of C6, in 24% of C7 and in 57% of operations on C8 and T1. No statistical difference was seen between a repair of C5 by graft or nerve transfer.

Posterior dislocation of the shoulder was observed in 30 cases. All were successfully relocated after the age of one year. In these children the results of repairs of C5 were reduced by a mean of 0.8 on the Gilbert score and 1.6 on the Mallett score. Pre-operative electrodiagnosis is a reliable indicator of the depth of the lesion and of the outcome after repair. Intra-operative somatosensory evoked potentials were helpful in the detection of occult intradural (pre-ganglionic) injury.

Residual muscle weakness in obstetric brachial plexus palsy results in soft-tissue contractures which limit the functional range of movement and lead to progressive glenoid dysplasia and joint instability. We describe the results of surgical treatment in 98 patients (mean age 2.5 years, 0.5 to 9.0) for the correction of active abduction of the shoulder. The patients underwent transfer of the latissimus dorsi and teres major muscles, release of contractures of subscapularis pectoralis major and minor, and axillary nerve decompression and neurolysis (the modified Quad procedure). The transferred muscles were sutured to the teres minor muscle, not to a point of bony insertion. The mean pre-operative active abduction was 45° (20° to 90°). At a mean follow-up of 4.8 years (2.0 to 8.7), the mean active abduction was 162° (100° to 180°) while 77 (78.6%) of the patients had active abduction of 160° or more. No decline in abduction was noted among the 29 patients (29.6%) followed up for six years or more. This procedure involving release of the contracted internal rotators of the shoulder combined with decompression and neurolysis of the axillary nerve greatly improves active abduction in young patients with muscle imbalance secondary to obstetric brachial plexus palsy.

We reviewed 101 patients with injuries of the terminal branches of the infraclavicular brachial plexus sustained between 1997 and 2009. Four patterns of injury were identified: 1) anterior glenohumeral dislocation (n = 55), in which the axillary and ulnar nerves were most commonly injured, but the axillary nerve was ruptured in only two patients (3.6%); 2) axillary nerve injury, with or without injury to other nerves, in the absence of dislocation of the shoulder (n = 20): these had a similar pattern of nerve involvement to those with a known dislocation, but the axillary nerve was ruptured in 14 patients (70%); 3) displaced proximal humeral fracture (n = 15), in which nerve injury resulted from medial displacement of the humeral shaft: the fracture was surgically reduced in 13 patients; and 4) hyperextension of the arm (n = 11): these were characterised by disruption of the musculocutaneous nerve. There was variable involvement of the median and radial nerves with the ulnar nerve being least affected.

Surgical intervention is not needed in most cases of infraclavicular injury associated with dislocation of the shoulder. Early exploration of the nerves should be considered in patients with an axillary nerve palsy without dislocation of the shoulder and for musculocutaneous nerve palsy with median and/or radial nerve palsy. Urgent operation is needed in cases of nerve injury resulting from fracture of the humeral neck to relieve pressure on nerves.

We reviewed the outcome of 28 patients who had been treated using the Aequalis fracture prosthesis for an acute fracture of the proximal humerus at a mean follow-up of 39.3 months (24 to 63). The mean age of the patients at the time of the fracture was 66.3 years (38 to 80). The mean Constant score was 68.2 (37 to 84) for the operated shoulder, which represented 89.5% of the mean score for the uninjured side (p < 0.001). The quality of the reconstruction as shown on the immediate post-operative radiographs was categorised into three types, anatomical, acceptable, and unacceptable, depending on the position of the tuberosities relative to the prosthetic head and the humeral shaft. Anatomical reconstruction was associated with a higher mean Constant score as well as higher mean values of anterior forward elevation, abduction and external rotation than the other types, but the differences were not statistically significant (p > 0.231).

A total of 18 patients had active anterior elevation ≥150°. Their mean active abduction and external rotation were 163.6° and 31.3°, respectively. In seven of the 28 patients, the mean active anterior elevation, abduction and external rotation were 130.7°, 129.2° and 22.8°, respectively. In all, 12 patients were very satisfied with the results, 12 were satisfied, two were dissatisfied and two were disappointed; 26 reported no or only mild pain while only two had moderate pain. In five patients proximal migration of the humeral head was shown on the anteroposterior radiographs of the shoulder. No evidence of loosening was found in any prosthesis.

We undertook this study to determine the minimum amount of coronoid necessary to stabilise an otherwise intact elbow joint. Regan–Morrey types II and III, plus medial and lateral oblique coronoid fractures, collectively termed type IV fractures, were simulated in nine fresh cadavers. An electromagnetic tracking system defined the three-dimensional stability of the ulna relative to the humerus. The coronoid surface area accounts for 59% of the anterior articulation. Alteration in valgus, internal and external rotation occurred only with a type III coronoid fracture, accounting for 68% of the coronoid and 40% of the entire articular surface. A type II fracture removed 42% of the coronoid articulation and 25% of the entire articular surface but was associated with valgus and external rotational changes only when the radial head was removed, thereby removing 67% of the articular surface.

We conclude that all type III fractures, as defined here, are unstable, even with intact ligaments and a radial head. However, a type II deficiency is stable unless the radial head is removed. Our study suggests that isolated medial-oblique or lateral-oblique fractures, and even a type II fracture with intact ligaments and a functional radial head, can be clinically stable, which is consistent with clinical observation.

In order to compare the outcome from surgical repair and physiotherapy, 103 patients with symptomatic small and medium-sized tears of the rotator cuff were randomly allocated to one of the two approaches. The primary outcome measure was the Constant score, and secondary outcome measures included the self-report section of the American Shoulder and Elbow Surgeons score, the Short Form 36 Health Survey and subscores for shoulder movement, pain, strength and patient satisfaction. Scores were taken at baseline and after six and 12 months by a blinded assessor. Nine patients (18%) with insufficient benefit from physiotherapy after at least 15 treatment sessions underwent secondary surgical treatment.

Analysis of between-group differences showed better results for the surgery group on the Constant scale (difference 13.0 points, p − 0.002), on the American Shoulder and Elbow surgeons scale (difference 16.1 points, p < 0.0005), for pain-free abduction (difference 28.8°, p = 0.003) and for reduction in pain (difference on a visual analogue scale −1.7 cm, p < 0.0005).

Frozen shoulder is commonly encountered in general orthopaedic practice. It may arise spontaneously without an obvious predisposing cause, or be associated with a variety of local or systemic disorders. Diagnosis is based upon the recognition of the characteristic features of the pain, and selective limitation of passive external rotation. The macroscopic and histological features of the capsular contracture are well-defined, but the underlying pathological processes remain poorly understood. It may cause protracted disability, and imposes a considerable burden on health service resources. Most patients are still managed by physiotherapy in primary care, and only the more refractory cases are referred for specialist intervention. Targeted therapy is not possible and treatment remains predominantly symptomatic. However, over the last ten years, more active interventions that may shorten the clinical course, such as capsular distension arthrography and arthroscopic capsular release, have become more popular.

This review describes the clinical and pathological features of frozen shoulder. We also outline the current treatment options, review the published results and present our own treatment algorithm.

We present a review of claims made to the NHS Litigation Authority (NHSLA) by patients with conditions affecting the shoulder and elbow, and identify areas of dissatisfaction and potential improvement. Between 1995 and 2012, the NHSLA recorded 811 claims related to the shoulder and elbow, 581 of which were settled. This comprised 364 shoulder (64%), and 217 elbow (36%) claims. A total of £18.2 million was paid out in settled claims. Overall diagnosis, mismanagement and intra-operative nerve injury were the most common reasons for litigation. The highest cost paid out resulted from claims dealing with incorrect, missed or delayed diagnosis, with just under £6 million paid out overall. Fractures and dislocations around the shoulder and elbow were common injuries in this category. All 11 claims following wrong-site surgery that were settled led to successful payouts.

This study highlights the diagnoses and procedures that need to be treated with particular vigilance. Having an awareness of the areas that lead to litigation in shoulder and elbow surgery will help to reduce inadvertent risks to patients and prevent dissatisfaction and possible litigation.

Cite this article: Bone Joint J 2014; 96-B:574–9.

After establishing anatomical feasibility, functional reconstruction to replace the anterolateral part of the deltoid was performed in 20 consecutive patients with irreversible deltoid paralysis using the sternoclavicular portion of the pectoralis major muscle. The indication for reconstruction was deltoid deficiency combined with massive rotator cuff tear in 11 patients, brachial plexus palsy in seven, and an isolated axillary nerve lesion in two. All patients were followed clinically and radiologically for a mean of 70 months (24 to 125). The mean gender-adjusted Constant score increased from 28% (15% to 54%) to 51% (19% to 83%). Forward elevation improved by a mean of 37°, abduction by 30° and external rotation by 9°.

The pectoralis inverse plasty may be used as a salvage procedure in irreversible deltoid deficiency, providing subjectively satisfying results. Active forward elevation and abduction can be significantly improved.

In adults with brachial plexus injuries, lack of active external rotation at the shoulder is one of the most common residual deficits, significantly compromising upper limb function. There is a paucity of evidence to address this complex issue. We present our experience of isolated latissimus dorsi (LD) muscle transfer to achieve active external rotation. This is a retrospective review of 24 adult post-traumatic plexopathy patients who underwent isolated latissimus dorsi muscle transfer to restore external rotation of the shoulder between 1997 and 2010. All patients were male with a mean age of 34 years (21 to 57). All the patients underwent isolated LD muscle transfer using a standard technique to correct external rotational deficit. Outcome was assessed for improvement in active external rotation, arc of movement, muscle strength and return to work. The mean improvement in active external rotation from neutral was 24° (10° to 50°). The mean increase in arc of rotation was 52° (38° to 55°). Mean power of the external rotators was 3.5 Medical Research Council (MRC) grades (2 to 5).

A total of 21 patients (88%) were back in work by the time of last follow up. Of these, 13 had returned to their pre-injury occupation. Isolated latissimus dorsi muscle transfer provides a simple and reliable method of restoring useful active external rotation in adults with brachial plexus injuries with internal rotational deformity.

Cite this article: Bone Joint J 2013;95-B:660–3.

Surgical repair of posterosuperior rotator cuff tears has a poorer outcome and a higher rate of failure compared with repairs of supraspinatus tears. In this prospective cohort study 28 consecutive patients with an irreparable posterosuperior rotator cuff tear after failed conservative or surgical treatment underwent teres major tendon transfer. Their mean age was 60 years (48 to 71) and the mean follow-up was 25 months (12 to 80). The mean active abduction improved from 79° (0° to 150°) pre-operatively to 105° (20° to 180°) post-operatively (p = 0.011). The mean active external rotation in 90° abduction improved from 25° (0° to 70°) pre-operatively to 55° (0° to 90°) post-operatively (p < 0.001). The mean Constant score improved from 43 (18 to 78) pre-operatively to 65 (30 to 86) post-operatively (p < 0.001). The median post-operative VAS (0 to 100) for pain decreased from 63 (0 to 96) pre-operatively to 5 (0 to 56) post-operatively (p < 0.001).

In conclusion, teres major transfer effectively restores function and relieves pain in patients with irreparable posterosuperior rotator cuff tears and leads to an overall clinical improvement in a relatively young and active patient group with limited treatment options.

Cite this article: Bone Joint J 2013;95-B:523–9.

We compared the outcome of peri-operative humeral condylar fractures in patients undergoing a Coonrad-Morrey semiconstrained total elbow replacement with that of patients with rheumatoid arthritis undergoing the same procedure without fractures. In a consecutive series of 40 elbows in 33 patients, 13 elbows had a fracture in either condyle peri-operatively, and 27 elbows were intact. The fractured condyle was either fixed internally or excised. We found no statistical difference in the patients’ background, such as age, length of follow-up, immobilisation period, Larsen’s radiological grade, or Steinbrocker’s stage and functional class. There was also no statistical difference between the groups in relation to the Mayo Elbow Performance Score, muscle strength, range of movement, or radiolucency around the implants at a mean of 4.8 years (1.1 to 8.0) follow-up.

We conclude that fractured condyles can be successfully treated with either internal fixation or excision, and cause no harmful effect.

Objectives

To report the five-year results of a randomised controlled trial examining the effectiveness of arthroscopic acromioplasty in the treatment of stage II shoulder impingement syndrome.

In a prospective, randomised study on the repair of tears of the rotator cuff we compared the clinical results of two suture techniques for which different suture materials were used.

We prospectively randomised 100 patients with tears of the rotator cuff into two groups. Group 1 had transosseous repair with No. 3 Ethibond using modified Mason-Allen sutures and group 2 had transosseous repair with 1.0 mm polydioxanone cord using modified Kessler sutures. After 24 to 30 months the patients were evaluated clinically using the Constant score and by ultrasonography.

Of the 100 patients, 92 completed the study. No significant statistical difference was seen between the two groups: Constant score, 91% vs 92%; rate of further tear, 18% vs 22%; and revision, 4% vs 4%. In cases of further tear the outcome in group 2 did not differ from that for the intact repairs (91% vs 91%), but in group 1 it was significantly worse (94% vs 77%, p = 0.005).

Overall, seven patients had complications which required revision surgery, in four for pain (two in each group) and in three for infection (two in group 1 and one in group 2).

Replacement of the shoulder in juvenile idiopathic arthritis is not often performed and there have been no published series to date.

We present nine glenohumeral hemiarthroplasties in eight patients with systemic or polyarticular juvenile idiopathic arthritis. The mean follow-up was six years (59 to 89 months). The mean age at the time of surgery was 32 years. Surgery took place at a mean of 27 years after diagnosis. The results indicated excellent relief from pain. There was restoration of useful function which deteriorated with time, in part because of progression of the systemic disease in this severely affected group. No patient has required revision to date and there has been no radiological evidence of loosening or osteolysis around the implants.

We discuss the pathoanatomical challenges unique to this group. There was very little space for a prosthetic joint and, in some cases, bony deformity and the small size necessitated the use of custom-made implants.

Objectives

Cadaveric models of the shoulder evaluate discrete motion segments using the glenohumeral joint in isolation over a defined trajectory. The aim of this study was to design, manufacture and validate a robotic system to accurately create three-dimensional movement of the upper body and capture it using high-speed motion cameras.

We prospectively evaluated 61 patients treated arthroscopically for anterior instability of the shoulder at a mean follow-up of 44.5 months (24 to 100) using the Rowe scale. Those with post-operative dislocation or subluxation were considered to be failures. Logistic regression analysis was used to identify patients at increased risk of recurrence in order to develop a suitable selection system.

The mean Rowe score improved from 45 pre-operatively to 86 at follow-up (p < 0.001). At least one episode of post-operative instability occurred in 11 patients (18%), although their stability improved (p = 0.018), and only three required revision. Subjectively, eight patients were dissatisfied. Age younger than 28 years, ligamentous laxity, the presence of a fracture of the glenoid rim involving more than 15% of the articular surface, and post-operative participation in contact or overhead sports were associated with a higher risk of recurrence, and scored 1, 1, 5 and 1 point, respectively. Those patients with a total score of two or more points had a relative risk of recurrence of 43% and should be treated by open surgery.

We have previously described the short-term outcome of the use of reverse shoulder arthroplasty in the treatment of acute complex proximal humeral fractures in the elderly. We now report the clinical and radiological outcome of 36 fractures at a mean of 6.6 years (1 to 16). Previously, at a mean follow-up of 6 years (1 to 12) the mean Constant score was 58.5; this was reduced to 53 points with the further follow-up. A total of 23 patients (63%) had radiological evidence of loosening of the glenoid component. Nevertheless, only one patient had aseptic loosening of the baseplate at 12 years’ follow-up.

The reduction in the mean Constant score with longer follow-up and the further development of scapular notching is worrying.

New developments in design, bearing surfaces and surgical technique, and further follow-up, will determine whether reverse shoulder arthroplasty has a place in the management of complex proximal humeral fractures in the elderly.

We report the long-term clinical and radiological outcomes of the Aequalis total shoulder replacement with a cemented all-polyethylene flat-back keeled glenoid component implanted for primary osteoarthritis between 1991 and 2003 in nine European centres. A total of 226 shoulders in 210 patients were retrospectively reviewed at a mean of 122.7 months (61 to 219) or at revision. Clinical outcome was assessed using the Constant score, patient satisfaction score and range of movement. Kaplan-Meier survivorship analysis was performed with glenoid revision for loosening and radiological glenoid loosening (sd) as endpoints. The Constant score was found to improve from a mean of 26.8 (sd 10.3) pre-operatively to 57.6 (sd 20.0) post-operatively (p < 0.001). Active forward flexion improved from a mean of 85.3° (sd 27.4) pre-operatively to 125° (sd 37.3) postoperatively (p < 0.001). External rotation improved from a mean of 7° (sd 6.5) pre-operatively to 30.3° (sd 21.8°) post-operatively (p < 0.001). Survivorship with revision of the glenoid component as the endpoint was 99.1% at five years, 94.5% at ten years and 79.4% at 15 years. Survivorship with radiological loosening as the endpoint was 99.1% at five years, 80.3% at ten years and 33.6% at 15 years.

Younger patient age and the curettage technique for glenoid preparation correlated with loosening. The rate of glenoid revision and radiological loosening increased with duration of follow-up, but not until a follow-up of five years. Therefore, we recommend that future studies reporting radiological outcomes of new glenoid designs should report follow-up of at least five to ten years.

We prospectively studied 26 consecutive patients with clinically documented sensory or motor deficiency of a peripheral nerve due to trauma or entrapment using ultrasound, and in 19 cases surgical exploration of the nerves was undertaken. The ultrasonographic diagnoses were correlated with neurological examination and the surgical findings. Reliable visualisation of injured nerves on ultrasonography was achieved in all patients. Axonal swelling and hypoechogenity of the nerve was diagnosed in 15 cases, loss of continuity of a nerve bundle in 17, the formation of a neuroma of a stump in six, and partial laceration of a nerve with loss of the normal fascicular pattern in five. The ultrasonographic findings were confirmed at operation in those who had surgery.

Ultrasound may be used for the evaluation of peripheral nerve injuries in the upper limb. High-resolution ultrasound can show the exact location, extent and type of lesion, yielding important information that might not be obtainable by other diagnostic aids.

Inability to actively supinate the forearm makes common activities of daily living and certain vocational activities awkward or impossible to perform. A total of 11 patients with deficient supination of the arm underwent transfer of the tendon of flexor carpi ulnaris to the split tendon of brachioradialis with its bony insertion into the radial styloid left intact. Active supination beyond neutral rotation was a mean of 37.2° (25° to 49.5°) at a minimum follow-up of three years, representing a significant improvement (95% confidence interval 25 to 50, p < 0.001). Functional evaluation of the hand after this transfer showed excellent and good results in ten patients and fair in one.

The split tendon of brachioradialis as an insertion for transfer of the flexor carpi ulnaris appears to provide adequate supination of the forearm without altering the available pronation and avoids the domination of wrist extension sometimes associated with transfers of the flexor carpi ulnaris to the radial extensors of the wrist.

We have used Fourier transform infrared spectroscopy (FTIR) to characterise the chemical and structural composition of the tendons of the rotator cuff and to identify structural differences among anatomically distinct tears. Such information may help to identify biomarkers of tears and to provide insight into the rates of healing of different sizes of tear. The infrared spectra of 81 partial, small, medium, large and massive tears were measured using FTIR and compared with 11 uninjured control tendons. All the spectra were classified using standard techniques of multivariate analysis.

FTIR readily differentiates between normal and torn tendons, and different sizes of tear. We identified the key discriminating molecules and spectra altered in torn tendons to be carbohydrates/phospholipids (1030 cm⁻¹ to 1200 cm⁻¹), collagen (1300 cm⁻¹ to 1700 cm⁻¹ and 3000 cm⁻¹ to 3350 cm⁻¹) and lipids (2800 cm⁻¹ to 3000 cm⁻¹).

Our study has shown that FTIR spectroscopy can identify tears of the rotator cuff of varying size based upon distinguishable chemical and structural features. The onset of a tear is mainly associated with altered structural arrangements of collagen, with changes in lipids and carbohydrates. The approach described is rapid and has the potential to be used peri-operatively to determine the quality of the tendon and the extent of the disease, thus guiding surgical repair.

The treatment of a chronic posterior dislocation of the shoulder is often determined by the size of the associated impression fracture of the humeral head. Our hypothesis was that patients with a chronic unreduced posterior dislocation of the shoulder and a defect in the humeral head involving between 25% to 50% of the articular surface, would do better if reconstructed with an allograft from the femoral head rather than treated by a non-anatomical reconstruction. We reviewed ten men and three women with a mean age of 42 years (36 to 51) at a mean follow-up of 54 months (41 to 64) who had this procedure. At follow-up, nine had no pain or restriction of activities of daily living. Their mean Constant-Murley shoulder score was 86.8 (43 to 98). No patient had symptoms of instability of the shoulder.

Reconstruction of the defect in the humeral head with an allograft provides good pain relief, stability and function for patients with a locked, chronic posterior dislocation where the defect involves between 25% and 50% of the circumference of the articular surface.

Fractures of the proximal humerus can lead to malalignment of the humeral head, necrosis and post-traumatic osteoarthritis. In such cases surface replacement might be a promising option.

A total of 28 shoulders with glenohumeral arthritis subsequent to a fracture underwent surface replacement arthroplasty of the humeral head in patients with a mean age of 60 years (35 to 83). On the basis of the inclination of the impacted head, post-traumatic arthritis was divided into three types: type 1, an impacted fracture of the head in an anatomical position (seven cases); type 2, a valgus impacted fracture (13 cases); type 3, a varus impacted fracture (eight cases). The outcome was measured by means of the Constant score.

According to the Boileau classification of the sequelae of fractures of the proximal humerus, all 28 patients had a final result of intra-capsular category 1. The mean Constant score for the 28 shoulders increased from 23.2 points (2 to 45) pre-operatively to 55.1 points (20 to 89) at a mean of 31 months (24 to 66) post-operatively. Valgus impacted fractures had significantly better results (p < 0.039).

Surface replacement arthroplasty can provide good results for patients with post-traumatic osteoarthritis of the shoulder. Their use avoids post-operative complications of the humeral shaft, such as peri-prosthetic fractures. Further surgery can be undertaken more easily as the bone stock is preserved.

Arthrolysis and dynamic splinting have been used in the treatment of elbow contractures, but there is no standardised protocol for treatment of severe contractures with an arc of flexion < 30°. We present our results of radical arthrolysis with twin incisions with the use of a monolateral hinged fixator to treat very severe extra-articular contracture of the elbow. This retrospective study included 26 patients (15 males and 11 females) with a mean age of 30 years (12 to 60). The mean duration of stiffness was 9.1 months (5.4 to 18) with mean follow-up of 5.2 years (3.5 to 9.4). The mean pre-operative arc of movement was 15.6° (0° to 30°), with mean pre-operative flexion of 64.1° (30° to 120°) and mean pre-operative extension of 52.1° (10° to 90°). Post-operatively the mean arc improved to 102.4° (60° to 135°), the mean flexion improved to 119.1° (90° to 140°) and mean extension improved to 16.8° (0° to 30°) (p < 0.001). The Mayo elbow score improved from a mean of 45 (30 to 65) to 89 (75 to 100) points, and 13 had excellent, nine had good, three had fair and one had a poor result. We had one case of severe instability and one wound dehiscence which responded well to treatment. One case had deep infection with poor results which responded well to treatment.

Our findings indicate that this method is very effective in the treatment of severe elbow contracture; however, a randomised controlled study is necessary for further evaluation.

In a prospective study between 2000 and 2005, 22 patients with primary osteoarthritis of the shoulder had a total shoulder arthroplasty with a standard five-pegged glenoid component, 12 with non-offset humeral head and ten with offset humeral head components. Over a period of 24 months the relative movement of the glenoid component with respect to the scapula was measured using radiostereometric analysis.

Nine glenoids needed reaming for erosion. There was a significant increase in rotation about all three axes with time (p < 0.001), the largest occurring about the longitudinal axis (anteversion-retroversion), with mean values of 3.8° and 1.9° for the non-offset and offset humeral head eroded subgroups, respectively. There was also a significant difference in rotation about the anteversion-retroversion axis (p = 0.01) and the varus-valgus (p < 0.001) z-axis between the two groups. The offset humeral head group reached a plateau at early follow-up with rotation about the z-axis, whereas the mean of the non-offset humeral head group at 24 months was three times greater than that of the offset group accounting for the highly significant difference between them.

We retrospectively reviewed 26 patients who underwent reconstruction of the shoulder for a medial rotation contracture after birth injury of the brachial plexus. Of these, 13 patients with a mean age of 5.8 years (2.8 to 12.9) received an injection of botulinum toxin type A into the pectoralis major as a surgical adjunct. They were matched with 13 patients with a mean age of 4.0 years (1.9 to 7.2) who underwent an identical operation before the introduction of botulinum toxin therapy to our unit.

Pre-operatively, there was no significant difference (p = 0.093) in the modified Gilbert shoulder scores for the two groups. Post-operatively, the patients who received the botulinum toxin had significantly better Gilbert shoulder scores (p = 0.012) at a mean follow-up of three years (1.5 to 9.8).

It appears that botulinum toxin type A produces benefits which are sustained beyond the period for which the toxin is recognised to be active. We suggest that by temporarily weakening some of the power of medial rotation, afferent signals to the brain are reduced and cortical recruitment for the injured nerves is improved.

This study prospectively evaluated the outcome of manipulation under anaesthesia and hydrodilatation as treatments for adhesive capsulitis. A total of 36 patients (38 shoulders) were randomised to receive either method, with all patients being treated in stage II of the disease process.

The mean age of the patients was 55.2 years (44 to 70) and the mean duration of symptoms was 33.7 weeks (12 to 76). Eighteen shoulders (17 patients) underwent manipulation under anaesthesia and 20 (19 patients) had hydrodilatation. There were three insulin-dependent diabetics in each group. The mean visual analogue score in the manipulation under anaesthesia group was 5.7 (3 to 8.5; n = 18) before treatment, 4.7 (0 to 8.5; n = 16) at two months (paired t-test p = 0.02), and 2.7 (0 to 9; n = 16) at six months (paired t-test, p = 0.0006). The mean score in the hydrodilatation group was 6.1 (4 to 10; n = 20) before treatment, 2.4 (0 to 8; n = 18) at two months (paired t-test, p = 0.001), and 1.7 (0 to 7; n = 18) at six months (paired t-test, p = 0.0006). The visual analogue scores in the hydrodilatation group were significantly better than in the manipulation under anaesthesia group over the six-month follow-up period (p < 0.0001).

The mean Constant score in those manipulated was 36 (26 to 66) before treatment, 58.5 (24 to 90) at two months (paired t-test, p = 0.001) and 59.5 (23 to 85) at six months (paired t-test, p = 0.0006). In the hydrodilatation group it was 28.8 (18 to 55) before treatment, 57.4 (17 to 80) at two months (paired t-test, p = 0.0004) and 65.9 (28 to 92) at six months (paired t-test, p = 0.0005). The Constant scores in the hydrodilatation group were significantly better than in the manipulated group over the six-month period of follow-up (p = 0.02).

The range of movement improved in all patients over the six months, but was not significantly different between the groups. At the final follow-up, 94% of patients (17 of 18) were satisfied or very satisfied after hydrodilatation compared with 81% (13 of 16) of those receiving a manipulation.

Most of our patients were treated successfully, but those undergoing hydrodilatation did better than those who were manipulated.

This study examined the role of vitamin D as a factor accounting for fatty degeneration and muscle function in the rotator cuff. There were 366 patients with disorders of the shoulder. A total of 228 patients had a full-thickness tear (group 1) and 138 patients had no tear (group 2). All underwent magnetic resonance arthrography and an isokinetic muscle performance test. The serum concentrations of vitamin D (25(OH)D₃) were measured.

In general, a lower serum level of vitamin D was related to higher fatty degeneration in the muscles of the cuff. Spearman’s correlation coefficients were 0.173 (p = 0.001), −0.181 (p = 0.001), and −0.117 (p = 0.026) for supraspinatus, infraspinatus and subscapularis, respectively. In group 1, multivariate linear regression analysis revealed that the serum level of vitamin D was an independent variable for fatty degeneration of the supraspinatus and infraspinatus.

The serum vitamin D level has a significant negative correlation with the fatty degeneration of the cuff muscle and a positive correlation with isokinetic muscle torque.

Reversed shoulder prostheses are increasingly being used for the treatment of glenohumeral arthropathy associated with a deficient rotator cuff. These non-anatomical implants attempt to balance the joint forces by means of a semi-constrained articular surface and a medialised centre of rotation. A finite element model was used to compare a reversed prosthesis with an anatomical implant. Active abduction was simulated from 0° to 150° of elevation. With the anatomical prosthesis, the joint force almost reached the equivalence of body weight. The joint force was half this for the reversed prosthesis. The direction of force was much more vertically aligned for the reverse prosthesis, in the first 90° of abduction. With the reversed prosthesis, abduction was possible without rotator cuff muscles and required 20% less deltoid force to achieve it.

This force analysis confirms the potential mechanical advantage of reversed prostheses when rotator cuff muscles are deficient.

We have investigated the outcome of arthroscopic revision surgery for recurrent instability of the shoulder after failed primary anterior stabilisation. We identified 40 patients with failed primary open or arthroscopic anterior stabilisation of the shoulder who had been treated by revision arthroscopic capsulolabral reconstruction and followed up for a mean of 36 months (12 to 87). There were 34 men and six women with a mean age of 33.1 years (15 to 48). Details of the patients, the technique of the primary procedure, the operative findings at revision and the clinical outcome were evaluated by reviewing the medical records, physical examination and the use of the Western Ontario shoulder instability index score, the American Shoulder and Elbow Surgeons score and the health status questionnaire 12.

Recurrent instability persisted in four patients after the revision arthroscopic procedure. At the final follow-up, the mean American Shoulder and Elbow Surgeons score was 81.1 (17.5 to 99.5) and the mean Western Ontario shoulder instability index score was 68.2 (20 to 98.2). Quality-of-life scoring showed good to excellent results in most patients.

Arthroscopic revision capsulolabral reconstruction can provide a satisfactory outcome in selected patients for recurrent instability of the shoulder provided that no large Hill-Sachs lesion is present.

We studied 21 patients with a spontaneous palsy of the anterior interosseous nerve. There were 11 men and 10 women with a mean age at onset of 39 years (17 to 65).

Pain around the elbow or another region (forearm, shoulder, upper arm, systemic arthralgia) was present in 17 patients and typically lasted for two to three weeks. It had settled within six weeks in every case. In ten cases the palsy developed as the pain settled. A complete palsy of flexor pollicis longus and flexor digitorum profundus to the index finger was seen in 13 cases and an isolated palsy of flexor pollicis longus in five. All patients were treated without operation. The mean time to initial muscle contraction was nine months (2 to 18) in palsy of the flexor digitorum profundus to the index finger, and ten months (1 to 24) for a complete palsy of flexor pollicis longus. An improvement in muscle strength to British Medical Research Council grade 4 or better was seen in all 15 patients with a complete palsy of the flexor digitorum profundus and in 16 of 18 with a complete palsy of flexor pollicis longus.

There was no significant correlation between the duration of pain and either the time to initial muscle contraction or final muscle strength. Prolonged pain was not always associated with a poor outcome but the age of the patient when the palsy developed was strongly correlated. Recovery occurred within 12 months in patients under the age of 40 years who achieved a final British Medical Research Council grade of 4 or better. Surgical decompression does not appear to be indicated for young patients with this condition.