Intellectual development and familial dysautonomia.

The study examined adaptive trends in cognitive development among individuals with familial dysautonomia and sought to establish new base rates of intelligence for the dysautonomic population. Fifty-two subjects, aged 6 to 28 years, were administered the Wechsler scales of intelligence. The results indicate that there is less cognitive impairment than previous research would suggest, and that more dysautonomic children are capable of adjusting to standard school programs than was heretofore thought possible. Specific deficits seen in this population are discussed along with a rationale for deleting the term retarded where most of these individuals are concerned.

Fragmentation of the distal pole of the patella in spastic cerebral palsy.

Of eighty-five consecutive patients, thirteen to twenty years old, with spastic cerebral palsy involving one or both extremities (thirty-five patients seen at one institution and fifty, at another), four had roentgenographic evidence of fragmentation of the distal pole of the patella. In addition, three other patients with six spastic lower extremities, four of them with patellar fragmentation, were also included. In these seven patients, there were nine knees with patellar fragmentation, twelve knees with patella alta, nine with a flexion contracture, five that were painful, and four with changes in the tibial tubercle resembling those found in Osgood-Schlatter disease. Excessive tension in the quadriceps mechanism, usually in the presence of a flexion contracture, appeared to cause the lesions. Four of the fragmented patellae healed after hamstring release and correction of the flexion deformity.

Scoliosis in familial dysautonomia. Operative treatment.

The results of operative treatment of scoliosis were reviewed for twenty-two patients (ten boys and twelve girls) who had familial dysautonomia, an autosomal recessive disorder affecting primarily Ashkenazi Jews. The indication for operative intervention was progressive kyphoscoliosis to 45 degrees or more in a skeletally immature patient for whom bracing had failed. The mean age at the time of the operation was fifteen years and five months (range, eight years and two months to nineteen years). Seventeen patients had a thoracic curve with a mean preoperative Cobb angle of 69 degrees (range, 47 to 112 degrees), and five patients had a double major curve with a mean preoperative Cobb angle of 71 degrees (range, 42 to 87 degrees) for the cephalad curves and 60 degrees (range, 45 to 72 degrees) for the caudad curves. Twenty patients had a rigid kyphosis; in fourteen, the apex was at the seventh thoracic vertebra or more cephalad. Two patients had a lordoscoliosis. The mean preoperative kyphosis was 64 degrees (range, 12 to 110 degrees) in the thirteen patients who had a thoracic curve and for whom information regarding kyphosis was available, and it was 70 degrees (range, 54 to 84 degrees) in the five patients who had a double major curve. Postoperior spinal arthrodesis and instrumentation was performed in all patients. Two patients had an anterior arthrodesis as well because of the severity and rigidity of the curve. Allograft bone was used in eighteen patients. Postoperatively, all patients were managed with a body cast or with a custom-molded thoracolumbar brace.(ABSTRACT TRUNCATED AT 250 WORDS)

Salvage and reinfusion of postoperative sanguineous wound drainage. A preliminary report.

Thirty-five patients who were to have posterior spinal arthrodesis, total hip arthroplasty, or total knee arthroplasty were entered into one of two groups: Group A, to receive unwashed, filtered sanguineous drainage from the wound, or Group B, to receive washed, filtered drainage. The purpose of this prospective study was to evaluate the safety, efficacy, and difficulty of reinfusion of washed compared with unwashed drainage that had been salvaged from the wound after an orthopaedic operation. The sixteen patients in Group A received a mean of 475 milliliters of unwashed drainage for each total knee arthroplasty, 427 milliliters for each total hip arthroplasty, and ten milliliters for the one posterior spinal arthrodesis. The complications included immediate hypotension (two patients), hyperthermia (one patient), and hypotension five hours after reinfusion (one patient). The latter patient died, four days after the operation, of a massive myocardial infarction. The nineteen patients in Group B received a mean of 193 milliliters of washed, filtered drainage for each total knee arthroplasty, 203 milliliters for each total hip arthroplasty, and 179 milliliters for each posterior spinal arthrodesis. Salvage and reinfusion of washed drainage from the wound caused no problems in these patients.

Preoperative personality characteristics as predictors of postoperative physical and psychological patterns in scoliosis.

Four adaptive personality characteristics (trait anxiety, active--independent orientation, emotional poise, and level of cognitive development) were evaluated preoperatively in 50 adolescent idiopathic scoliosis patients undergoing spinal surgery. Postoperatively, three indices of physical recovery (quantity of analgesics taken, elimination difficulties, and number of days to discharge) and two of psychological state (anxiety and depression) were rated. Two of the four adaptive personality characteristics correlated significantly with postoperative physical and psychological reactions. Quantity of analgesics taken, elimination difficulties, anxiety, and depression were significantly related to (viz., predicted by) preoperative trait anxiety. Quantity of analgesics taken and anxiety were significantly related to level of cognitive development. Preoperative degree of curvature was significantly related to postoperative quantity of analgesics taken. Duration of the scoliosis was significantly associated with level of cognitive development, but not with postoperative physical or psychological adjustment.

Osteoporosis in Scheuermann's disease.

A prospective study was undertaken to evaluate osteoporosis in Scheuermann's disease. Ten consecutive untreated patients with thoracic Scheuermann's were studied. The mean age was 16 years, 1 month, and the mean kyphosis was 64 degrees. Osteoporosis was quantitated by single and dual photon absorptiometry. Seven age-, sex-, height-, and weight-matched subjects were used as controls. The mean bone mineral density (BMD) of the lumbar spine was 0.975, compared with 1.130 for the control group, significant at P less than 0.025. For patients with a kyphosis greater than 45 degrees, the BMD was 0.913, significant at P less than 0.005. The mean BMD of the femoral neck was 1.00, compared with 1.22, significant at P less than 0.005. For patients with a kyphosis greater than 45 degrees, the femoral neck BMD is 0.983, significant at P less than 0.005. The BMD of the radius by single photon absorptiometry was 0.689, compared with 0.748 in the controls, which was not significant. In patients with a kyphosis greater than 45 degrees, the BMD was 0.655, which is significant at P less than 0.01. A highly significant association exists between osteoporosis and Scheuermann's disease. Further investigation is indicated to determine the role of medical management in the treatment of these patients.

Long-term psychological sequelae of surgically versus nonsurgically treated scoliosis.

Forty-six female scoliosis patients (21-34 years of age) were tested psychologically 4 or more years following treatment. Twenty-three had been treated by bracing (Group A) and 23 by posterior spinal fusion (Group B). Variables evaluated were: self-esteem, capacity for intimacy, sexual satisfaction, and mental representation of the patient's own body (body image). The prediction that normal controls would show better psychological adjustment than scoliotic women--surgically treated or not--was only partially borne out. Contrary to expectations, both groups of scoliotics showed higher levels of sexual satisfaction than controls. Group B showed a greater need for intimacy and better sexual adjustment, self-esteem, and body image than Group A.

The effects of internal fixation on the articular cartilage of unfused canine facet joint cartilage.

Custom-made distraction instrumentation was placed in the lumbar spine of eight large dogs with care taken to preserve the integrity of two intervening apophyseal joints. Histologic staining of immobilized joint cartilage showed varying degrees of chondrolysis, cloning, invasion of the tide mark, and loss of proteoglycans as early as 2 months postoperatively. Every specimen had significant degenerative changes characteristic of osteoarthritis. The joints one segment caudal to the lower hooks also were examined and were found to have similar histologic evidence of degeneration. Three dogs had the instrumentation removed and were then sacrificed 1.5-3 months later. The degenerative changes were not reversible following instrumentation removal.

Percutaneous computed-tomography-guided biopsy of the thoracic and lumbar spine.

Axial computed tomographic scans were used to guide percutaneous needle biopsies in 76 patients with thoracic and lumbar spinal lesions. Prebiopsy evaluation included spine radiographs, radionuclide bone scans, computed tomographic scans, magnetic resonance imaging scans in some cases, and coagulation studies. Forty-five patients were diagnosed as having metastatic lesions, 11 infection, and 12 primary bone tumors. Of all patients, 34 had lytic vertebral lesions with significant collapse and questionable spinal stability. Six of those had a concomitant paravertebral mass. A clinical and pathologic correlation was completed for each of the cases studied. Histologic diagnosis confirming the clinical suspicion was obtained on the first biopsy attempt in 65 (86%) of the 76 cases.

Physical therapy management of familial dysautonomia.

This paper describes physical therapy programs to assist physical therapists in the rehabilitation of patients with familial dysautonomia. There have been no reports in the literature about a physical therapy program for patients with this disease. A retrospective analysis of the clinical manifestations in 80 patients in a dysautonomia clinic was performed. Scoliosis and kyphosis were found in 92 percent of the patients, 93 percent had ataxia, 74 percent had feeding difficulties, 69 percent had frequent pneumonias, and 63 percent exhibited delayed developmental milestones. Cardiovascular, gastrointestinal, pulmonary, musculoskeletal, and neurological symptoms and treatments are discussed.

Optimizing hospital reimbursement through physician awareness: a step toward better patient care.

We instituted a training program to improve the overall accuracy of medical record coding through greater physician awareness to enhance hospital reimbursement and maintain quality patient care. A physician-targeted course reviewed the prospective payment system, diagnosis-related group guidelines, ambulatory surgery reimbursement, and the relationship between accurate physician documentation and medical record coding. Annual increases in charges from prospective surgical case assignment, proper conversion of outpatient to inpatient status, and more accurate coding of inpatient comorbidities and complications led to an estimated increase in hospital charges of $1.6 million.

A feasibility study for removing tissue contamination from porous implants.

Porous implants that are unpackaged in the operating room but not implanted are discarded because they must be considered potentially contaminated with tissue. To reduce this waste, a method was developed to decontaminate these implants so that they can be resterilized and implanted. This method consists of ultrasonic scrubbing, sequentially, in aqueous solutions of dishwashing detergent, 7% nitric acid, and 5.25% sodium hypochlorite. The effectiveness of the method was tested by contaminating samples of porous implants with tissue, subjecting them to the decontamination method, and then using the following techniques to determine whether any tissue remained. The weights of samples after decontamination were compared with their weights before contamination. The rate of removal of labeled protein contamination from samples was measured. The capacity of decontaminated samples to activate immune system cells was assayed. Bioburden evaluations were performed on decontaminated samples. Within the measurement capabilities of each technique, no tissue was detected in any sample after decontamination.

The Hospital for the Ruptured and Crippled moves east on 42nd Street 1912 to 1925.

In 1912, the Hospital for the Ruptured and Crippled (R & C) moved to its third location since its founding in 1863. It was a newly constructed six-story building on 42nd Street between Second Avenue and First Avenue. At the time Dr. Virgil Gibney, the second Surgeon-in-Chief, was 65 years of age and had already served 25 years in that position. The building housed a New York City public school since the length of stay of the children, many afflicted with tuberculosis and poliomyelitis, might be over 1-2 years. The large number of immigrants in the city led to very challenging social conditions that saw changes made in the first two decades of the twentieth century. When this country entered World War I in 1917, the physician staff was significantly affected as many volunteered for military service. Soldiers, sailors, and marines were treated at R & C, and military physicians were educated in orthopedics and hernia care on the wards and in the outpatient department. Because of declining health in 1924, Dr. Gibney retired and was replaced as Surgeon-in-Chief by his long-term friend and colleague, Dr. William B. Coley in January 1925.