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In the 1960s, effective techniques were developed for shunting hydrocephalus and for early closure of neural tube defects. As a result, orthopaedic surgeons were presented with the challenge of managing an emerging population of children who had myelomeningocele. Initially, the musculoskeletal problems in these children were treated with the modalities and expectations that had been learned from the treatment of poliomyelitis. However, it soon became apparent that the management of children who have myelomeningocele was not so simple. Additional factors include a decrease or loss of sensation affecting some or all parts of the lower extremities, associated congenital anomalies of the spine and lower extremities, and muscle imbalance that affects skeletal development over the entire period of growth. Furthermore, some patients who have myelomeningocele have a static encephalopathy that impairs coordination and results in the loss of strength of the lower and upper extremities29,30,45. Also, progressive neurological deterioration may occur because of tethered cord syndrome or syringomyelia2. As a result, the evaluation and treatment of musculoskeletal problems in these patients can be quite difficult. The purpose of this Instructional Course Lecture is to review the natural history of myelomeningocele as well as the types of deformity that are associated with it, the treatment options that are available, and the expected results of treatment of hip and knee problems related to myelomeningocele. Although other factors must be considered, the neurological level is the key to understanding the hip and knee deformities seen in these patients. Unless otherwise specified, a modification of the classification system described by Asher and Olson3 will be used to define the neurological level (Table I). This classification is based on muscle strength, is simple to use, and, in my experience, has been helpful in predicting gross motor function …

The etiology and treatment of Kienbock 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. Kienbock 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 diagnosis7,9,36,44. 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 Kienbock disease is described in the present report. 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 …

The results of five patients who had hemophilia and a history of recurrent hemarthrosis and hypertrophic synovitis and who had been managed with a synovectomy of the ankle were studied at an average age of nine years (range, four years and seven months to nineteen years). Compared with the complications encountered after synovectomy of the knee or the elbow, the rehabilitation process after synovectomy of the ankle was relatively easy, even for the three youngest children in this series. The average duration of follow-up was five years (range, one to nine years). By the latest follow-up examination, the range of motion of the ankle had increased an average of 10 degrees (range, -5 to 15 degrees). The rate of hemarthrosis episodes requiring transfusion was reduced from an average of 3.4 per month (range, 0.3 to 5.0 per month) for the six months before the synovectomy to 0.1 per month (range, zero to 0.2 per month) for the twelve months before the latest follow-up examination.

We reviewed the cases of fifteen obese patients (twenty-one extremities) who had had adolescent tibia vara and had been followed for at least two years. Of the nine patients (eleven extremities) who had been initially managed with lateral tibial hemiepiphyseodesis, eight (ten extremities) were skeletally mature at the time of the review (mean duration of follow-up, five years). The mechanical alignment was judged to be excellent in three of these ten extremities, fair in three, and poor in four. Excellent mechanical alignment was defined as a value within the reported normal range of 5 degrees of varus to 2 degrees of valgus. A poor result was defined as alignment that was more than 5 degrees outside the normal range. After secondary operative procedures, three of the extremities for which the result had been poor and one for which it had been fair had excellent alignment. Five of the nine patients had bilateral involvement. Two of them were managed with bilateral tibial hemiepiphyseodesis; two, with contralateral proximal tibial osteotomy; and one had a mild deformity on the contralateral side that was not treated. Six extremities in six patients (two of whom had a contralateral hemiepiphyseodesis) were managed primarily with proximal tibial osteotomy and were evaluated an average of seven years postoperatively. Two additional patients were managed with proximal tibial osteotomy because of residual varus deformity after the hemiepiphyseodesis.(ABSTRACT TRUNCATED AT 250 WORDS)

The records of all thirteen patients for whom a diagnosis of cloacal exstrophy had been recorded in our hospitals were analyzed for evidence of musculoskeletal problems. All thirteen patients had spina bifida, four had congenital scoliosis, two had congenital kyphosis, and three had non-congenital scoliosis. All had a lipomeningocele, and eleven had paralysis of the lower extremities. Hydrocephalus and its associated problems were not found, but tethered-cord syndrome was diagnosed in eleven patients. Persistent diastasis of the symphysis pubis was found in all patients. Abduction and external rotation of the hips were more than normal. Mild dysplasia was seen in six of the twenty-six hips. Deformities of the foot were common, and twelve feet had been operated on for correction. Recurrent equinovarus deformity of the foot was associated with tethered-cord syndrome in two patients.

Thirty patients who had hemophilia and were seropositive for the human immunodeficiency virus were evaluated. The preoperative CD4 lymphocyte count was decreased to an average of 336 x 10(9) per liter (range, 27 to 708 x 10(9) per liter). After twenty-six orthopaedic operations in patients who had no previous bacterial infection, a nosocomial infection (cellulitis in the forearm, at the site of an intravenous catheter) developed in only one patient, but five patients had an abnormal postoperative fever that was not accompanied by the expected increase in the white blood-cell count. The preoperative CD4 lymphocyte count was significantly reduced in the patients who had an abnormal elevation in body temperature (p less than 0.004). The functional result or outcome after operation was similar to that in hemophilic patients treated before 1982. Subsequent progression of infection with the human immunodeficiency virus, as determined by the CD4 lymphocyte count and the Walter Reed classification system, occurred in most patients. Acquired immunodeficiency syndrome was diagnosed in six patients. A more rapid progression to acquired immunodeficiency syndrome was seen in the patients who had a lower CD4 lymphocyte count preoperatively. Preoperative evaluation of the CD4 lymphocyte count and the response to intradermal skin-test antigens in patients who are at risk for infection postoperatively provides additional information concerning immunological competence. With these data, the possible risk of infection in patients who are seropositive for the human immunodeficiency virus can be estimated more accurately.

The Board of Trustees of The Journal of Bone and Joint Surgery has appointed James D. Heckman, M.D, as our new Editor-in-Chief, beginning January 1, 2000. His selection was the result of an intensive national search for a successor to Henry R. Cowell, M.D. Ph.D., who has been Editor since 1985. We believe that Dr. James Heckman is abundantly and uniquely qualified to lead The Journal into the future. He is an educator with outstanding academic credentials, who, until recently, served as Professor and Chairman of the Department of Orthopaedics and Director of the Orthopaedic Residency …

In 105 knees, the roentgenographic classifications of hemophilic arthropathy of Arnold and Hilgartner, of Pettersson et al., and of a new system were compared. The amount of interobserver agreement (between two observers) was good for all three systems and, for all three, there was a good correlation between progressive severity of the lesion and decreasing function of the joint. The system of Pettersson et al. was better than that of Arnold and Hilgartner for grading severe arthropathy, but it was not better than the new, simplified system. The new system is a four-sign, 7-point classification, in contrast to the eight-sign, 13-point system of Pettersson et al.

The rationale for the use of prophylactic antibiotics in clean orthopaedic surgery was explored by means of an experimental model suited to the investigation of (1) antibiotic penetration and persistence in operative hematomas in bone and (2) the effect of cephaloridine administration on standard wound infections. Cephaloridine was found to penetrate hematomas in bone readily and to persist there in bacteriocidal concentrations. When cephaloridine was given preoperatively, all wounds were converted to bacteriological sterility and did not become clinically infected. When cephaloridine was begun six hours or later after contamination, infection occurred uniformly.