The kleeblattschädel anomaly in Apert syndrome: intracranial anatomy, surgical correction, and subsequent cranial vault development.

We present a case of Apert syndrome in which intracranial anomalies of the cranial base were localized to the lesser wings of the sphenoid and sphenoid ridge. The lesser wings of the sphenoid were displaced superiorly to follow the fused coronal sutures bilaterally, where they met at a single point on the skull vertex. Careful preoperative study of the intracranial anatomy in the kleeblattschädel anomaly led to a surgical plan for early correction of the anomaly. The present report indicates that an aggressive approach to the correction of the kleeblattschädel anomaly beginning early in infancy can result in normalization of the trilobar skull configuration. Although this approach can correct the kleeblattschädel anomaly, 3.5-year follow-up in this patient with Apert syndrome demonstrates progressive turricephaly despite repeated cranial vault remodeling. Although the trilobar skull configuration can be corrected through early surgical intervention, the long-term correction of progressive turricephaly in patients with Apert syndrome remains an unsolved problem.

Frontofacial osteotomies, advancement, and remodeling by distraction: an extended application of the technique.

The purpose of this clinical report is to present the distraction technique for advancement of the frontofacial skeleton as a unit. Our 14-year-old patient was diagnosed with Carpenter's syndrome and kleblattschädel deformity at birth. At other centers the patient underwent corrective surgeries, including repeated fronto-orbital advancement in an attempt to correct the residual deformity. This has resulted in bony malunion and recurrent deformity, and it has left the patient with no available donor sites for harvesting of bone graft. The patient had class III malocclusion, severe midfacial and frontal deficiency, and relative turricephaly. We performed frontofacial osteotomies and placement of the distraction devices. Distraction of 20 mm was accomplished, correcting the exophthalmos and midface retrusion and producing class I dental occlusion. We conclude that distraction is an optional surgical method that can be applied in selected cases for advancement of the entire frontofacial skeleton.

A Pentium personal computer-based craniofacial three-dimensional imaging and analysis system.

Personal computer (PC)-based computing is now ubiquitous in common consumer applications. Although PCs have equaled or surpassed engineering workstations in basic computing power and economy, there is still strong workstation dependency for imaging applications. This article demonstrates that a complete system, based on a Pentium PC (Intel Corporation, Santa Clara, CA) and readily available and inexpensive software, can be built very economically for effective execution of most of the commonly used three-dimensional imaging operations. For the craniofacial application, the Pentium system offers a twofold speed advantage over a Sparc 20 system using similar three-dimensional processing software. The Pentium system allows interactive fuzzy volume rendering, and manipulation and analysis of complex hard and soft-tissue structures.

Neuroelectric stimulation in cerebral palsy: long-term quantitative assessment.

Results from multiple tests including somatosensory evoked potentials, passive resistance to motion, upper extremity motor skills evaluation, neuromuscular examination, and parental interview were evaluated in 13 children with cerebral palsy (CP) who received chronic cerebellar stimulation (CCS) for reduction of spasticity and movement disorders during the past 14 years. The prospective study included immediate postoperative follow-up data as well as longer term results from the quantitative test series. Although CCS was effective in reducing hypertonicity in CP children during the immediate short-term, the diminishment waned significantly 3-5 years postoperatively.

Traumatic facial injuries with steering wheel loading.

This study was conducted to evaluate the biomechanics of facial fractures caused by steering wheel loading. Twelve intact fresh human cadaver heads were impacted onto standard or energy-absorbing steering wheels with a custom-designed and validated vertical-drop apparatus. Either zygoma was impacted once at a velocity of 2.0-6.9 m/s. The specimens were oriented to permit a direct comparison between pretest and posttest radiography, and two-dimensional and three-dimensional CT images. Bone mineral content was determined, and biomechanical forces, accelerations, and deformations were recorded. More severe fractures were associated with higher forces on the zygoma. With increasing velocities, fractures initiated at the zygomatic region propagated to other unilateral regions such as the mandible and orbit or to the contralateral side. Less facial trauma was observed with energy-absorbing steering wheels compared with standard wheels at similar impact velocities. Bone mineral content did not correlate well with specimen age or with fracture severity. Clinically significant fractures were identifiable on 3-D CT images. The flexibility of 3-D CT in evaluating the spatial extent of facial abnormalities in different orientations may have significant impact in planning surgical procedures.

Analysis of neuroelectric implant integrity.

A 10-year follow-up study of neuroelectric implant integrity has been completed for 27 subjects who were treated for movement disorders associated with cerebral palsy. Data used for analysis included X-ray material, clinical data, and results from electrophysiological testing. Of the 21 subjects with subcutaneous receivers placed in the thoracic region, 81% experienced wire (67%) or receiver (14%) failure. Of the 6 subjects with subcutaneous receivers placed in the occipital region, there were no wire failures and one (17%) receiver failure. The occipital units lasted a minimum of 5.2 years without complications, whereas the thoracic units failed as early as 6 months after surgery, and lasted an average of 3.8 years. All wire fractures occurred between C1 and T1. Two types of fracture occurred, one consisting of a clean break and the other consisting of a scenario of bending and kinking, then thinning and fraying, and finally progressive multiple fragmentation. With a few exceptions the clean breaks occurred between C1 and C3, while the fraying scenario occurred between C6 and T1. Mechanisms for failure are discussed, as are results from material tests of wire samples.

CT of dry skulls with craniofacial deformities: accuracy of three-dimensional reconstruction.

Dry skulls from patients with Crouzon syndrome or orbital neurofibromatosis were studied using three-dimensional reconstruction of computed tomography data. The images were compared with one another and with the actual skulls. It was concluded that the use of dry skulls is helpful in pointing out errors of inclusion or exclusion. Thinner sections permit more accurate representation. Since reconstructed data do not appear to be significantly enhanced by using overlapping sections, radiation can be reduced by using abutting sections.

Immediate effects of spinal cord stimulation in spinal spasticity.

Six patients with intractable spasms after spinal cord injury underwent implantation of an epidural spinal cord stimulation system. All the patients experienced good relief postoperatively. In three patients spinal cord stimulation consistently produced immediate inhibition of the spasms. This was evident within less than 1 minute of stimulation. Conversely, the spasms reappeared within less than 1 minute after cessation of the stimulation. The clinical observations were confirmed by polygraphic electromyographic recordings.

Skeletal morphology of anterior encephaloceles defined through the use of three-dimensional reconstruction of computed tomography.

We have used three-dimensional reconstruction of computed tomography to study the morphology of anterior encephaloceles. The findings based on living patients rather than cadaver specimens confirm previous findings suggesting 'blow out' defects which displace otherwise normal osseous structures.

Three-dimensional reconstruction of craniofacial deformity using computed tomography.

The computed tomographic studies obtained routinely in the examination of patients with congenital or acquired defects of the skull and facial bones can be utilized as a substrate to provide an accurate three-dimensional representation of osseous abnormalities. The total dose of x-irradiation is reduced as other means of radiological examination are eliminated. Osseous structures are faithfully reproduced. Complete inspection of the reproduced structure can be made from any viewpoint, including internal inspection.

The computed tomographic appearance of the spinal cord in diastematomyelia.

The myelographic and computed tomographic findings in 10 patients with diastematomyelia were reviewed. In all patients, including seven with osseous spurs, the hemicords were smaller than the normal cord above or below the cleft. The narrow hemicords distinguish diastematomyelia from diplomyelia.

Evoked potentials in experimental myelopathy.

Evoked potentials recorded from the cerebral cortex and spinal cord secondary to peripheral stimulation are reversibly reduced in amplitude by both pathologic distraction and pathologic flexion of the vertebral column. While the cerebral responses are lost within two minutes after complete occlusion of the ascending aorta, the responses recorded from the spinal cord persist without change for approximately ten minutes and then gradually disappear. During the first few minutes after aortic occlusion, changes produced by spinal distraction and spinal flexion are indistinguishable from those produced when the same maneuvers are made with the aorta patent. The responses mediated by dorsal columns and corticospinal tracts are affected in the same way by flexion and distraction, suggesting that somatosensory evoked potential recordings should be a reliable means of detecting spinal cord dysfunction during surgical procedures affecting the spinal cord. It may also be possible to differentiate a mechanical from a vascular insult by the time required for the evoked potential to become abnormal following a particular surgical maneuver.

Gas myelography in spinal cord injury.

Using polytomographic techniques, gas myelography was performed in 162 patients with post-traumatic neurologic deficit. The type, extent, and location of lesions were easily demonstrated, allowing adequate preoperative surgical planning. In cases in which expected neurologic improvement did not follow surgery, postoperative myelography provided information so that remedial measures could be taken. No neurologic deterioration was noted in any of the patients and furthermore, arachnoiditis, which may accompany positive contrast myelography, has not been detected in any of the patients in the series reported.