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Biomedical subjects

S E Kopits

Publications and source records attributed to S E Kopits.

At least 19 recordsLinked to original sources
PubMed1994

Total craniospinal decompression in achondroplastic stenosis.

We describe our experience with total craniospinal decompression along the entire neuraxis, extending from the brain stem to the cauda equina, in seven patients with achondroplasia. These patients presented with clinically significant compression at multiple levels. In these patients, there were focal areas of complete myelographic block, typically at the cervicothoracic or thoracolumbar junction, as well as diffuse narrowing of the entire spinal subarachnoid space. In some, there were further complications of basilar impression, Arnold-Chiari malformation, or syringomyelia. Total craniospinal decompression was completed in either one or two stages. Only a small minority of our patients with achondroplasia had critical stenosis over this many levels, requiring total craniospinal decompression. However, with proper preparation and technique, we found that patients can tolerate even such an extensive decompressive procedure and benefit from surgery without suffering postoperative spinal instability.

Achondroplasia↗
PubMed1987

Cervicomedullary compression in young patients with achondroplasia: value of comprehensive neurologic and respiratory evaluation.

We studied prospectively 26 young patients with achondroplasia to test two hypotheses: that respiratory problems may be the result of occult spinal cord compression, and that achondroplastic patients with cord compression might have occult respiratory abnormalities. Respiratory abnormalities were present in 85%, the majority caused by a primary problem of the pulmonary system, such as small thoracic cage or obstructed airway. Three patients had hypoxemia, recurrent cyanotic spells, and episodes of respiratory distress explainable only by cervicomedullary cord compression; in each patient, respiratory problems were alleviated by decompressive surgery. Another six patients with cervicomedullary compression had, in addition, at least one primary pulmonary cause of respiratory problems. After decompressive surgery the respiratory problems improved in three and were unchanged in three. Reconstructed sagittal CT images proved the most sensitive technique for detecting craniocervical stenosis as a cause of cervicomedullary cord compression, although some degree of stenosis was present in nearly all of the patients.

Achondroplasia↗
PubMed1986

Multiplanar (MPR) imaging of the hip.

Multiplanar CT expands the ability of the radiologist to define the extent of disease, to suggest appropriate surgery, and to offer a probable prognosis.

Acetabulum↗
PubMed1985

Spondyloepiphyseal dysplasia congenita. Light and electron microscopic studies of the eye.

A 5-month-old infant with spondyloepiphyseal dysplasia congenita (SEDC) died after an anoxic episode. Ophthalmologic examination one month before death revealed marked myopia and a normal ophthalmoscopic examination, without evidence of retinal detachment, lattice degeneration, or vitreoretinal changes. On postmortem histopathologic and electron microscopic examination of the eyes, we found the vitreous to have central liquefaction, to be detached in multifocal areas, and exerting traction on the retina. The internal limiting membrane of the retina was thin throughout and displayed many areas of discontinuity. There were multiple areas of preretinal cellular proliferation and a few small areas of retinoschisis. Our observation of extensive vitreoretinal degeneration with traction of the retina indicates that eyes of patients with SEDC are at an increased risk for the development of retinal detachment.

Eye↗
PubMed1983

Respiratory complications of achondroplasia.

Nine patients with achondroplasia who were seen over a three-year period developed significant respiratory complications. Eight had sleep-disordered breathing, including obstructive sleep apnea in five, for which two required tracheostomy. Of the seven patients with significant hypoxemia, five had clinical evidence of cor pulmonale and recurrent pulmonary infiltrates. Two patients died, one with autopsy findings of compression of the medulla at the level of the foramen magnum and one with respiratory and cardiac failure. Appropriate therapy for our patients depended on recognition of the mechanisms that led to the respiratory complications, including (1) chest deformity, (2) upper airway obstruction and sleep-disordered breathing, (3) neurologic complications, and (4) coincidental chronic pulmonary conditions such as asthma.

Achondroplasia↗
PubMed1978

Proximal femoral focal deficiency.

Proximal femoral focal deficiency, a distinct clinical and radiographic entity that results in leg length discrepancy, has received little attention in the radiographic literature. Thirteen patients were studied and the degree of deficiency established using radiographic criteria. The full extent of hip instability often could not be ascertained on radiographs obtained during the first year of life. Hip arthrograms combined with cine radiography can establish the presence of an unossified femoral head and aid in evaluating the degree of hip instability. Unlike other causes of leg length discrepancy, such as congenital idiopathic coxa vara, proximal femoral focal deficiency is often associated with other congenital bony anomalies, most commonly ipsilateral absent or hypoplastic fibula.

Bone Diseases↗
PubMed1977

Primary chronic sclerosing osteomyelitis.

The case of a ten-year-old white male with longstanding, painful swelling and severe limitation of left elbow motion is reported. The lack of symptoms and signs characteristic of infection and the pseudosarcomatous radiological findings coupled with the pathological findings of chronic osteomyelitis outline the diagnosis of primary chronic sclerosing osteomyelitis. A detailed description is given of the clinical course to illustrate the diagnostic difficulties encountered in a case of this nature. The outcome was favorable.

Bone Resorption↗