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Of thirty-nine patients with Klippel-Feil syndrome, twenty-five (64 per cent) had significant genitourinary-tract anomalies demonstrated by intravenous urogram and physical examination. The incidence of these anomalies in Feil's three types of the syndrome was essentially the same, unilateral renal agenesis being the most common. A routine intravenous urogram is indicated in patients with this syndrome.
Bony anomalies encountered in the 'no neck' form of Klippel-Feil syndrome (KFS) are a wide, short, fused, bifid, retroflexed spinal canal; craniolacunia, cranium bifidum, and acrania. The only symptom may be mirror movement (MM). The CNS anomalies are hindbrain hernia, hydrocephalus, hydromyelia, syringomyelia, meningocele, myelocele, encephalocele, and anencephalus. In severe KFS, i.e. iniencephalus (IN) and in anencephalus (AN), the inion is in contact with the back. In both there is hindbrain hernia and the left thorax may contain the stomach tethered to an anterior spina bifida. KFS results from distortion of somites by an overdistended neural tube. A neural tube that fails to close cannot overdistend.
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The majority of cervical spine anomalies of clinical significance occur in the occipital, atlas, and axis areas. Below the axis, changes may occur as local solitary anomalies simulating significant disease, or in the form of more widespread segmentation and ossification anomalies categorized as the Klippel-Feil syndrome.
The radiological criteria of juvenile, rheumatic, cervical synostosis discovered in adult life are described and illustrated. These include: involvement of few or many segments, a tendency to bony ankylosis of the diseased intervertebral joints, dysplasias or hypoplasia of the vertebral body and intervertebral disc and dysplasias of the neural arches and hypoplasia of the transverse processes. Pathological ossification may involve the ligamentum flavum, the annulus, or the entire disc. The differential diagnosis of juvenile, rheumatic, cervical synostosis includes congenital block vertebrae, Klippel-Feil syndrome, acquired block vertebrae, juvenile ankylosing spondylitis, synostosing, intervertebral osteochondrosis and myositis ossificans progressiva.
Patients with cleft palates with or without cleft lip need more special attention at birth when the cleft is associated with other malformations. For example, the Pierre-Robin and the Klippel-Feil Syndromes are both well known for the special problems they present. Reported here are three cases, with Crouzon's and two with Franceschetti Syndromes, all of whom have palatal clefts.
There is a multiplicity of birth defects of the spine occurring as isolated anomalies or as aspects of multifaceted syndromes. The clinical significance of these anomalies varies from the asymptomatic to the life threatening. Symptoms and signs may present at birth or not until years later. The optimal clinical result for the patient requires a careful history and physical examination, appropriate routine and specialized roentgenograms, laboratory data, and a high index of suspicion for signs or symptoms of neurological dysfunction. When the diagnosis is made, conservative or surgical treatment may be instituted as appropriate.
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Cervical spinal stenosis occurs at the craniovertebral junction, usually incident to a congenital malformation, or it appears as a developmental defect with diffuse narrowing of the cervical canal. In its acquired form the lesion may be limited to one or two levels, or it may be more extensive and affect three or more segments.
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A unique specimen of unilateral Sprengel's deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. The cervical spine exhibited multiple abnormalities involving both the vertebral centra as well as the posterior elements (Klippel-Feil abnormality). A small spina bifida involving C5 and C6 was present. Abnormalities of the spinous processes included an articulation with a well-formed omovertebral bone that also articulated with the vertebral (infraspinatus) margin of the scapula. Secondary (presumed epiphyseal) ossification was present in the omovertebral bone at the distal end. The scapula was deformed, especially in the supraspinatus portion. The clavicle was shorter and had a different contour.
This review covers conventional radiographic tomography, radioisotopic tomography, and a review of computerized transaxial tomography. Simple, reproducible radiographic tomographic methods are increasing in use, and despite their complexity, the diagnostic results are superior to conventional radiographs. There are many different motions of the X-ray tube and the film which can be employed to create a tomogram. The perfect tomographic motion is partly determined by the geometric shape of the object to be imaged and the thickness of the plane of interest. The undirectional tomographic method blurs a point in a linear fashion; the pleuridirectional method blurs a point over a wider surface. Among the most popular directions used are linear, circular, elliptical, and hypocycloidal. The numerous applications of tomography described in this review are an encouraging, broad-based foundation from which the most clinically useful and economically feasible devices will emerge.
Electrostatic imaging with its capacity for enhancing borders improves radiographic demonstration of gas-soft tissue interfaces in gas myelography. Its wide recording latitude also provides satisfactory delineation through areas of widely varying photon absorption. Xeroradiogrphy surmounts the paradox wherein contrast and latitude are inversely related so that the combination of both high contrast and broad latitude are simultaneously used to advantage. The xerogram, especially in the thoracic region, may be sufficiently diagnostic so that tomography is not necessary. Where additional information is required, tomographic xeroradiography can provide diagnostic information not perceptible on film tomograms.
Association of ocular, cervical, and cardiac anomalies is discussed. A case with severe congenital heart disease, unusual Duane's retraction syndrome, classified here as atypical typical, and Klippel-Feil anomaly is described. To alert the physicians to such an association of congenital malformations and for nosological purposes this entity may be called oculo-cervico-cardiac syndrome. Importance of the association of heart and eye abnormalities is stressed and syndromes with associated ocular and cardiac anomalies are briefly discussed.