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At least 217 records · Page 12Linked to original sources
PubMed2006

Monitoring visual function in children with syndromic craniosynostosis: a comparison of 3 methods.

OBJECTIVE: To compare visual acuity, optic disc appearance, and transient pattern reversal visual evoked potentials as markers of possible visual dysfunction in children with syndromic craniosynostosis. METHODS: Serial visual acuity, optic disc appearance, and pattern reversal visual evoked potential data were recorded in 8 patients with syndromic craniosynostosis before and after cranial vault expansion. The pattern reversal visual evoked potentials were analyzed using linear regression modeling, applied to the N80 to P100 amplitude. RESULTS: Serial optic disc appearances were available for all 8 patients and visual acuities for 7 patients. The visual acuity deteriorated in only 1 patient, improved in 4, and fluctuated in 2, before surgery. Of the 8 patients, 3 showed no papilledema in either eye at any time, 3 showed progressive bilateral swelling before surgery, and 2 exhibited only unilateral disc swelling. In all 8 patients, there was a trend for the N80 to P100 amplitude to decrease before surgery and to increase, in all but 2 patients, after surgery. CONCLUSIONS: This study suggests that neither optic disc appearance nor visual acuity assessment alone is a reliable marker of potential visual dysfunction in children with syndromic craniosynostosis. It also suggests that the pattern reversal visual evoked potential can provide early evidence of visual dysfunction before vault expansion surgery in these children; this dysfunction may recover postoperatively.

Child↗
PubMed2006

Hypertropia associated with superolateral translation of the superior rectus muscle pulley in unilateral coronal synostosis.

OBJECTIVE: To explore the mechanisms underlying the hypertropia associated with unilateral coronal synostosis. METHODS: In 13 patients with unilateral coronal synostosis, we measured gaze-dependent binocular alignment before and after strabismus surgery, assessed the superior rectus muscle (SRM) pulley using computed tomography, and simulated posterior displacement of the trochlea and superolateral displacement of the SRM pulley. RESULTS: All the patients had an ipsilateral hypertropia in primary gaze (3-30 diopters) that increased in contralateral gaze and decreased in ipsilateral gaze and that simulated an inferior oblique muscle overaction. Strabismus surgery fully or partially corrected the hypertropia in only 7 of 11 patients. High-resolution computed tomography demonstrated that the pulley of the SRM relative to the inferior rectus muscle was translated 0 to 11.0 mm laterally and up to 2.7 mm vertically. Lateral translation (up to 10 mm) alone or combined with vertical translation (up to 5 mm) of the SRM pulley in the simulated model produced a hypertropia with lateral incomitance. Posterior translation (15 mm) of the trochlea did not induce a significant hypertropia. CONCLUSION: Superolateral translation of the SRM pulley creates an imbalance of muscle pulling forces that better accounts for the hypertropia than posterior displacement of the trochlea.

Child↗
PubMed1992

Vertically fused tracheal cartilage. An underrecognized anomaly.

Anterior vertical fusion of the tracheal cartilages with normal pars membranacea is a rarely described tracheal anomaly. We report four cases of this anomaly, three associated with craniosynostosis syndromes and one with Goldenhar's syndrome. The anomaly was documented at postmortem examination in one case, both endoscopically and at tracheotomy in two cases, and endoscopically in one case. Documentation from gross postmortem photographs, photomicrographs, and intraoperative endoscopic photographs is presented so that the anatomical aspects of this anomaly can be thoroughly understood and recognized. All four newborn patients developed recurrent lower respiratory tract infections, reactive airway disease, and chronically retained secretions attributable to this anomaly. Neither tracheal stenosis nor stridor was encountered. In the three surviving patients, the infections grew less frequent and less severe in later childhood. These observations suggest that this tracheobronchial anomaly may be associated with a variety of craniofacial syndromes and may alter airflow dynamics in a way that predisposes to retained secretions and chronic pulmonary disease.

Abnormalities, Multiple↗