JBJS | Kienböck Disease in a Child Who Has Cerebral Palsy. A Case Report*

The Journal of Bone & Joint Surgery, Volume 78, Issue 10

Articles | October 01, 1996

Kienböck Disease in a Child Who Has Cerebral Palsy. A Case Report*

WALTER B. GREENE, M.D.†, CHAPEL HILL, NORTH CAROLINA

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Investigation performed at the Division of Orthopaedics, University of North Carolina School of Medicine, Chapel Hill

The Journal of Bone & Joint Surgery. 1996; 78:1568-73

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Introduction

Introduction | Case Report | Discussion | References

The etiology and treatment of Kienböck disease, or osteonecrosis of the lunate, remain controversial. The condition is frequently associated with repetitive trauma, and the typical patient is twenty to forty years old and has noted an insidious onset of pain and stiffness in the wrist.

Kienböck disease is uncommon in children. In four large series, only five (1 per cent) of 404 patients were less than sixteen years old at the time of diagnosis^7,9,36,44. Furthermore, the children in those reports were involved either in sports activities that had caused repetitive trauma to the wrist, such as gymnastics, or they had sustained an acute injury. A ten-year-old child who had diplegic cerebral palsy and Kienböck disease is described in the present report.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, University of Missouri Hospitals, Columbia, Missouri 65212.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Department of Orthopaedic Surgery, University of Missouri Hospitals, Columbia, Missouri 65212.

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Fig. 1-A: Anteroposterior radiograph of the left wrist, made seven weeks after the onset of symptoms, showing increased density and mild collapse of the lunate. There is questionable sparing of the radial border.

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Fig. 1-B: Anteroposterior radiograph of the right wrist, made when the patient was first seen, for comparison.

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Lateral radiograph of the left wrist, showing collapse to be greater on the volar aspect of the lunate.

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Anteroposterior radiograph of the left wrist, made eleven weeks after the patient was first seen, showing additional increased density involving the entire lunate but no progression of the collapse.

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Anteroposterior radiograph of the left wrist made eight months after the onset of symptoms, showing complete resolution of the osteonecrosis and a normal carpal height ratio of 0.56.

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Anteroposterior radiograph of the left wrist, made when the patient was seventeen years old, showing a normal contour of the lunate and no apparent radiocarpal osteoarthrosis.

Case Report

Introduction | Case Report | Discussion | References

A ten-year and ten-month-old white girl who had moderately severe diplegic cerebral palsy was seen two weeks after the onset of pain and decreased motion of the left wrist. The pain in the wrist was mild, but it had compromised her ability to grasp a walker. The patient reported no recent episodes of trauma to the wrist and had not needed to use analgesics.

The patient was highly motivated and intelligent and had been able to walk independently about the house since the age of seven years. She needed a reverse rolling walker as an aid for walking and, before the onset of pain in the wrist, she had used a wheelchair only for long distances, such as when she went to a shopping mall. Previous operations included iliopsoas recession and adductor myotomy bilaterally when she was three years old and lengthening of the gastrocnemius aponeurosis bilaterally when she was six years old.

Physical examination revealed no obvious swelling and only slight tenderness to palpation over the mid-part of the dorsum of the wrist. Compared with the right side, the range of passive extension of the left wrist had decreased 15 degrees. Radiographs demonstrated slightly increased density of the left lunate. The carpal height ratio, as described by Youm et al.⁴⁹ and refined by Mirabello et al.³⁵, had decreased to 0.48 (normal, 0.54 ± 0.03), indicating some collapse of the lunate. Because of the mild nature of the pain in the wrist, some uncertainty about the diagnosis, and concern that immobilization of the wrist would prevent the patient from walking, observation and a slight modification of activities were recommended.

A repeat evaluation after five weeks revealed increased symptoms, more restricted motion of the wrist, and a decreased ability to walk because aching in the wrist prevented use of the walker. With the fingers flexed, the right wrist had 70 degrees of passive extension, but the left wrist was limited to 20 degrees of passive extension. Radiographs showed increased density and mild collapse of the lunate, particularly on the volar aspect (Figs. 1-A, 1-B, and 1-C). At that time, the wrist was immobilized in a below-the-elbow cast with the wrist in 10 degrees of extension. Immobilization completely eliminated the pain in the wrist and allowed the patient to continue to walk. However, gripping the walker irritated the skin in the thenar web space. This problem was eliminated by removing the loop of cast material in the thenar web space and wrapping an elastic bandage around the palmar aspect of the cast. Radiographs made eleven weeks after the patient was first seen showed greater density of the lunate but no progression of the collapse (Fig. 2).

Immobilization in the cast was continued for fifteen weeks. Radiographs made twenty weeks after the initial diagnosis demonstrated slight residual osteonecrosis. For an additional fifteen weeks, the patient wore a removable wrist splint when she used the walker. Radiographs made eight months after the onset of the symptoms showed complete resolution of the osteonecrosis and restoration of the carpal height ratio to 0.56 (Fig. 3).

At a follow-up examination when she was seventeen years old, the patient reported no pain in the left wrist and she had retained her level of walking ability—that is, she used a wheelchair only for long distances. The range of flexion and extension of the left wrist was symmetrical with that on the right side. Active extension of the wrist, which was limited by the spasticity of the wrist flexors, was 10 degrees with the fingers extended and 45 degrees with the fingers flexed. Passive extension of the wrist was 70 degrees. Radiographs demonstrated a normal appearance of the lunate and no evidence of radiocarpal osteoarthrosis (Fig. 4). Ulnar variance was neutral.

Discussion

Introduction | Case Report | Discussion | References

Sequential radiographic changes, bone scintigraphy, magnetic resonance imaging, and open biopsy have confirmed that osteonecrosis is the pathological basis of Kienböck disease^{1,7,17,19,35,40,46}, but the etiology of the osteonecrosis remains controversial. Kienböck^25,26 reasoned that the disorder resulted from an injury causing tears of the ligaments and blood vessels. However, a well defined acute traumatic event has been documented for only a few patients. In the large series described by Nakamura et al.³⁶, preceding trauma was reported by only 12 per cent of 122 patients who had Kienböck disease. Furthermore, osteonecrosis is relatively uncommon after a severe injury such as a dislocation of the lunate, a perilunate dislocation, or a scapholunate dissociation^1,5,7,8,16.

Repetitive trauma is an important factor in the development of Kienböck disease. In several studies, the largest group of patients either was involved in strenuous manual labor or participated in sports activities in which the wrist was loaded repetitively^{1,10,23,34-36,45,47}. Whether repetitive trauma disrupts the arterial blood supply to the lunate, whether it causes subcortical microfractures that result in osteonecrosis, or whether it causes venous congestion that leads to arterial insufficiency is unknown^{12,17,22,29,39}. Although the extraosseous blood supply is abundant, theoretically the intraosseous circulation may be vulnerable, particularly in the 20 to 26 per cent of the population in whom the intraosseous circulation is supplied by a single dorsal or palmar vessel (five of twenty-five subjects in one study³⁹ and fourteen of fifty-three in another²⁹). Certainly, subcortical fractures are present in the later stages of Kienböck disease, but this phenomenon is probably a secondary rather than a primary event. Jensen²² measured the intraosseous pressure in ten patients who were having an operation for Kienböck disease. The pressure in the lunate was considerably higher than that in the radial styloid process and the capitate, and Jensen reasoned that venous congestion rather than arterial insufficiency was a primary factor. However, as in Legg-Calvé-Perthes disease, it is unclear at the present time whether intraosseous hypertension in Kienböck disease is a late finding or a primary cause of the disorder.

Negative ulnar variance (a short ulna) is often cited as a factor contributing to the development of Kienböck disease^{4,13,18,32,35,40}. Operations to restore the relationship between the lengths of the radius and the ulna at the wrist by lengthening the ulna or shortening the radius have been reported as effective methods of treatment^{4,6,40,42,45,48}. Recently, the role of negative ulnar variance in Kienböck disease has been questioned. In 1982, concerns about the findings of previous reports were raised initially by studies^11,38 that demonstrated that the measurement of ulnar variance was altered by the position of the forearm and elbow. In 1991, a study³⁷ of 325 adolescents and adults who had normal wrists showed that age and gender were also important. Increasing age was associated with increasing positive ulnar variance, and female subjects of all ages demonstrated a more positive ulnar variance. Finally, two recent studies^9,37, in which a standardized radiographic position and controls for age and gender were used, showed no difference in ulnar variance between patients who had Kienböck disease and those who did not.

Although normal values for ulnar variance have not been determined for ten-year-old children, negative ulnar variance was probably not a factor in my patient. A radiograph made when she was seventeen years old showed an ulnar variance¹³ of 0.0 millimeter with the wrist in a standard neutral position. This value was within normal limits, as compared with the average ulnar variance of 0.27 ± 1.69 millimeters for white adults¹³ and 0.06 ± 1.11 millimeters in a population of 122 Japanese girls and women who were between the ages of fourteen and thirty years³⁷.

A multitude of operations have been prescribed for the treatment of Kienböck disease. These include lengthening of the ulna, shortening of the radius, excision of the lunate, proximal-row carpectomy, silicone replacement arthroplasty, excisional arthroplasty with a tendon implant, various arthrodeses of the surrounding carpal bones, revascularization procedures, total wrist arthrodesis, and total wrist arthroplasty. Furthermore, the results of these operations are well documented^{1,3,4,6,7,9,14,15,18-21,23,24,30-34,40-42,45,47,48}. In comparison, the results and indications for non-operative management are not well documented⁴⁴. In 1947, Ståhl advocated prolonged immobilization; he observed better results when patients who had Kienböck disease had been managed with a cast for more than two months. However, that study did not provide a precise understanding of either long-term symptoms or function. In contrast, Lichtman et al.³² reported on twenty-two patients who had been managed with a cast or splint for two to fourteen months. Progressive collapse of the lunate developed in seventeen patients, and only three of the other five patients had a satisfactory result. The study by Kristensen et al.²⁷ has been cited often as one that supports the use of immobilization for the treatment of Kienböck disease. However, the primary purpose of that report was the evaluation of ulnar variance, and neither symptoms nor function were assessed objectively.

Authors of review articles and of textbooks on surgery of the hand, who have limited information but a perception that immobilization usually is not successful, typically have recommended that immobilization be restricted to stage-I Kienböck disease (normal findings on radiographs, with the exception of a possible crescent sign)^1,2,5,28. Operative intervention with a procedure to restore the relationship between the lengths of the radius and ulna at the wrist or a revascularization procedure is considered a better alternative for stage-II disease (increased density of the lunate but no substantial alteration in the anatomical relationship of the carpal bones). Whether children who have stage-II Kienböck disease should be managed differently is unclear, but Amadio and Taleisnik⁵ noted that a procedure to restore the relationship between the lengths of the radius and ulna at the wrist or a revascularization procedure should be considered for skeletally immature patients. Furthermore, other investigators who have advocated operative intervention have included children (age range, nine to fifteen years) in their series^31,36,45.

Osteonecrotic lesions should heal more rapidly in a child than in an adult, particularly if the extraosseous blood supply has not been disrupted. Indeed, that was the situation in my patient who had radiographic findings of slight residual osteonecrosis four and one-half months after the onset of symptoms and no evidence of residual disease eight months after the onset. Because of the relatively small volume of bone that has to be reformed and remodeled in Kienböck disease and the fact that the prevalence of individuals who have both a palmar and a dorsal vessel supplying the intraosseous circulation has been reported to be 74 per cent (thirty-nine of fifty-three²⁹) to 80 per cent (twenty of twenty-five subjects³⁹), the good result after non-operative therapy in my preadolescent patient was not surprising.

Immobilization in a cast certainly alleviated the pain in the wrist in my patient and made it easier for her to use a walker. In retrospect, immobilization should have been instituted when she was first seen. Theoretically, immobilizing the wrist in slight extension may have advanced the process of bone repair by decreasing venous congestion that could have resulted from the chronic posture of wrist flexion. Whether immobilization in a cast enhances healing of osteonecrotic lesions cannot be proved in a single case report. However, because of the good result in my patient and the paucity of well controlled studies, I believe that the role of cast immobilization in the treatment of stage-I and II Kienböck disease in children and adults should be evaluated further.

Rooker and Goodfellow⁴³ noted Kienböck disease in five patients who had cerebral palsy. However, the patients were adults (between the ages of seventeen and twenty-eight years) who had severe quadriplegic cerebral palsy and kept the wrist in an extreme position of flexion. My patient had diplegic cerebral palsy, but the involvement of the upper extremity was more severe than is usually observed with that type of the disorder. My patient could feed herself and write in a limited fashion, but she had only 20 to 30 degrees of active extension of the wrist and that motion was accompanied by ulnar deviation. Her high motivation to walk coupled with her need to grip the reverse walker in the face of the mild spasticity of the wrist and of the finger flexors, and perhaps an unrecognized injury of the wrist, probably triggered the chain of events that caused the osteonecrosis of the lunate. Finally, specific inquiries about pain in the wrist during routine evaluations of patients who have cerebral palsy may reveal that osteonecrosis of the lunate occurs more frequently than previously recognized. This may be true not only for patients with spastic diplegia who repetitively traumatize the wrist when gripping a walker but also for those who have spastic quadriplegia and constantly position the wrist in flexion.

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