Special devices as aids in the management of child self-mutilation in the Lesch-Nyhan syndrome.

The Lesch-Nyhan syndrome is a rare inborn error of purine metabolism associated with hyperuricacidemia, mental retardation, and an insatiable urge for self-mutilation, especially of the extremities. Insensitivity to pain and severe muscle spasms create very difficult management problems, especially for children being cared for at home. Fortunately this syndrome is rare (1 per 380,000 live births). Four known patients are under treatment in Manitoba, of which two brothers are the subject of this study. In general, the medical management of these patients has been unsuccessful, especially in controlling self-mutilation. It therefore was decided to manage these children by the use of mechanical aids. Through the utilization of custom-designed seating devices control of the self-mutilation has been obtained, reducing the burden of family care. It is now possible for the children to attend regular school on a half-day basis. They are also able to engage in community activities without the fear that a mishap will occur when not under the vigilance of the immediate family.

Orthopaedic treatment of hemangiomatous hypertrophy of the lower extremity.

The triad of varicose veins, hypertrophy of soft tissue and bone, and cutaneous hemangiomas (The Klippel-Trenaunay syndrome) often results in major malformations. A review of eleven children treated for this condition showed that the overgrowth of the limb was a major problem in management. In three patients the altered hemodynamics resulted in cardiac complications. Venous stripping, excision of the arteriovenous fistulae, and radiation therapy should be avoided.

Conversation in the operating theater as a cause of airborne bacterial contamination.

Using microspheres of human albumin sprayed on the face and in the nostrils under the facemask, we studied the role of conversation in contaminating the operating-room environment in addition to the microbial contamination of the air in an empty and in an occupied operating room. Conversation consistently increased the number of microspheres found in the simulated wound. The spatial relationship between the mask and the hood was important in reducing contamination. A complete hood overlapping the mask, with no gaps at the sides or bottom of the mask, was most efficient. The air contamination was increased by the presence of operating-room personnel and varied in different parts of the theater according to the density of traffic. We concluded that conversation contributes to airborne contamination in the operating theater but that contamination from this source can be lessened by wearing a face-mask that extends underneath an overlapping hood.

Preoperative skeletal traction in scoliosis.

To determine the role of preoperative traction in the treatment of spinal curves, ten patients with curves averaging 81 degrees by the Cobb measurement were studied. Each patient was treated in traction by means of a halo or skull tongs and femoral pins for two to three weeks prior to the surgical correction of the scoliosis with instrumentation. Weights were added to the traction apparatus, 1.8 kilograms a day, to a maximum of 18.1 kilograms. Pretraction supine roentgenograms were compared with supine roentgenograms made at three-day intervals throughout the traction period. Preoperative traction resulted in an average improvement of the curves of 34 degrees, or 41 per cent of the initial curve. Most of the correction was obtained within the first week of traction. Further correction during surgery with Harrington instrumentation yielded 13.5 degrees on the average (16 per cent) so that combined correction averaged 47 degrees, or 57 per cent.

Mobility aids for the paraplegic child.

Paraplegic children derive many benefits from upright bracing during their growing years. Physiological advantages include the prevention of joint contractures, decreased osteoporosis, prevention of urinary tract infections, increased cardiopulmonary tolerance, and the development of trunk and head control. The quality of life is enhanced because the child has increased self-esteem and participates in group activities with greater independence. Two new devices, the stand-up wheelchair and the shuttlebug, have been employed with success in the Winnipeg Child Paraplegic Program.

Musculoskeletal manifestations of the Antley-Bixler syndrome.

The Antley-Bixler syndrome is a rare disorder with many musculoskeletal anomalies that demand orthopedic assessment. The syndrome includes skeletal, craniofacial, and urogenital anomalies. The most common skeletal deformities are radiohumeral synostosis, craniosynostosis, multiple joint contractures, and arachnodactyly. Other orthopedic manifestations that may occur are femoral bowing, ulnar bowing, camptodactyly, synostoses of carpal and tarsal bones, clubfoot, vertebral body anomalies, perinatal fractures, and advanced skeletal age. The inheritance pattern is thought to be autosomal recessive. A patient with this syndrome is described, which is the 18th of 24 reports published in the world literature. This case is compared with the other reported cases.

Paraspinal Muscle Activity During Sports.

Using sophisticated telemetry techniques, the authors studied electrical activity in the paraspinal muscles and found implications not only for analyzing athletic performance, but also for patients with low back pain.

Computer corner #16. Computer as an aid in orthopaedic slide production.

The state of the art in the field of graphics presentation is increasing almost monthly. At the present time, however, there is the software capability to allow orthopaedic surgeons to become quite competent in the production of quality graphic material, especially the production of 35-mm slides of a quality that can be presented at any orthopaedic meeting for a modest cost and minimal production time.

Degloving injuries in children.

Degloving injuries are uncommon but serious and are being encountered with increasing frequency in children. A 5-year review of experience with degloving injuries on the Orthopaedic Service at the Winnipeg (Manitoba, Canada) Children's Hospital revealed 16 patients who had sustained this trauma in association with fractures. Twelve of these children had typical anatomical degloving. However, six patients had an associated concealed degloving or so-called "physiological degloving," with disruption of the underlying skin vasculature but no actual disruption of the skin surface. The most common causes of degloving injuries were being run over by a motor vehicle and farm machinery accidents. The diagnosis and proper management of the degloved extremity, especially when accompanied by underlying fracture, are essential in children if morbidity and limb loss are to be minimized.

The hidden adolescent ankle fracture.

Ankle injuries are common in adolescence, seldom initially seen by orthopaedists, and often treated primarily by emergency department personnel. It is often not appreciated that the distal tibial growth plate fuses in a mediolateral direction, leaving it open and vulnerable to growth plate injuries for a longer period during adolescence. Fractures of the lateral plafond of the tibia are often not appreciated due to overlap of the fibula. A review of the injuries at the Winnipeg Children's Hospital has revealed these are not as uncommon as often supposed. A 3 year experience yielded 26 patients with so-called "Tillaux fractures" of which 9 could be diagnosed only by the oblique view and 5 were initially missed. Knowledge of the anatomical behavior of the fusing tibial growth plate in the ankle-injured adolescent plus mandatory oblique views of the ankle in this age group should enable the casualty officer in a busy emergency department to bring this fracture out of hiding and make the diagnosis in every instance.

Computer corner #15. The AAOS: request for computer system proposal.

The Committee on Computer Science and Practice Management of the American Academy of Orthopaedic Surgeons has provided an excellent format to ensure a happy relationship between the vendor and the orthopaedic office using this package. Problems in communication and understanding between the vendor and the orthopaedic surgeon will undoubtedly be significantly reduced. How the system will work is the vendor's problem, but what you want it to do for you is your problem, and both of these issues are addressed in these questionnaires. It is recommended in the computer package that three vendor proposals be invited. Since the most frequent cause of computer problems is improper evaluation of what functions the computer system must perform in order to meet the needs of the orthopaedic office, this series of questionnaires is important. In addition to recommending this computer package, I would also emphasize that if at all possible--in spite of what may appear to be an excellent system on paper--try to visit an office in which the system has been in use over the past six to 12 months.

Cervical spondylolysis in children: is it posttraumatic?

Cervical spondylolysis is a rare defect of unknown etiology. Five cases of cervical spondylolysis as well as two cases of fractures of the pedicles of C2 in infants are presented. Comparison of the cases suggests that a fracture at birth or in infancy may be the cause of some cases of cervical spondylolysis.

Pediatric update #7. The orthopaedic manifestations of head injury in children.

The incidence of significant head injury has been estimated at 220/100,000 children. Over 90% of these will recover with little residual disability. The purpose of this study is to re-examine the small but significant percentage of head injured children with permanent total body involvement to identify patterns of deformity and temporal sequences and to develop preventive treatment regimens. Sixteen head injured children with residual spastic quadriplegia were examined. All exhibited musculoskeletal abnormalities. Hip adduction contractures were most common leading to hip subluxation in eight children, followed by pes equinus, scoliosis, pelvic obliquity, and knee flexion contracture. The average time to onset of permanent foot deformity was 11 months after head injury, for scoliosis an average of 22 months postinjury, and for hip subluxation an average of 31 months postinjury. Hamstring contractures occurred later, at an average of 37 months, but caused the most interference with good seating. The physical problems of the head injured child have some similarities to those of the child with cerebral palsy, but with distinct differences. An aggressive surgical approach to prevention and treatment of fixed deformity in these children is recommended at an early stage with postoperative orthotic management and stable seating in abduction.

Fractures of the neck of the talus in children.

A review of children with talar injuries treated at the Winnipeg Children's Hospital yielded 12 patients that had been treated for fractures of the neck of the talus between 1960 and 1978, inclusive. Three developed avascular necrosis of the body of the talus; two of these had their fractures recognized only after avascular necrosis had become radiologically evident. The children ranged in age from 1 year 7 months to 13 years 11 months at the time of the injury. Fracture of the neck of the talus does occur in children and may be associated with avascular necrosis of the body. This injury should be considered and the talus should be examined in all children sustaining trauma secondary to falls from a height or motor vehicle trauma.

Skeletal diseases associated with angiomatosis.

Basically, there are two conditions in which angiomatosis is associated with underlying skeletal disease. The first is Maffucci's syndrome in which angiomatosis is associated with multiple enchondromatosis. Two patients with this disease are presented and its clinical and radiologic features are reviewed. The second is "congenital angiectatic hypertrophy" in which angiomatosis is associated with localized hypertrophy of underlying bones, soft tissues and occasionally internal vercera. Four patients with this condition are presented, illustrating the subtypes of closely related diseases within a broad spectrum.

Delayed paresis following spinal fusion with Harrington instrumentation.

Immediate postoperative paresis following spinal fusion for scoliosis is uncommon. This neurological disaster can occur either within hours or days after surgical correction of the curvature. Paresis of the right lower extremity occurred on the third postoperative day in a 15-year-old boy who underwent spinal fusion for a pseudarthrosis of a previous fusion and in whom a Harrington rod was inserted for stability but not correction. The rod was removed within four hours of recognition of the paresis; within 18 months the child made an almost complete recovery of all motor function; slight residual weakness of the anterior dorsiflexors of the foot still remain.

Gunshot wounds of the extremities in children.

A ten-year review of gunshot trauma in Manitoba was prompted by an increasing incidence in gunshot trauma in children. Of 54 children treated from 1964 through 1973 60% sustained injury to one or more extremities. Fifteen children sustained long bone fractures, usually complete and often comminuted. All fractures united and there was only one instance of osteomyelitis. The major complication in gunshot wounds to the skeletally immature was limb length inequality secondary to epiphyseal plate injuries. This complication occurred in nine children. In three children there was in epiphyseal growth disturbance, even though the missile did not appear to pass through the plate but only in close proximity to it. Joint contractures secondary to skin loss and resulting subsequent skin contracture were also common. The amount of debridement required following gunshot trauma in children appears to be somewhat less than is recommended for similar injuries sustained by adults in time of war.

The vacuum splint: an aid in emergency splinting of fractures.

The vacuum splint has been shown to be a simple, safe and effective method of emergency splinting of fractured extremities. The splint is simply constructed from clear vinyl sheeting and contains 2-mm. expanded polystyrene balls. Evacuation of air causes the splint to become rigid, thereby providing stability and immobilization of the limb. The splint is radiolucent, containing no obstructive metal components that would interfere with the radiographic appearance of the injured limb. The ease of application of this splint makes it especially effective for the emergency splinting of fractures in children.