'Z-pattern' craniosynostosis: a novel presentation of trisutural fusion.

The majority of infants with multiple sutural craniosynostosis have identifiable syndromes, which affect both coronal sutures (Apert, Crouzon, Pfeiffer, and so on), and multiple sutural fusions not involving both these sutures are exceeding rare. We report a highly unusual pattern of trisutural fusion, which to our knowledge has not been previously described. A retrospective clinical review was performed of all cases of craniosynostosis, which had presented to the Craniofacial Center in Dallas, over a 15-year period. Two patients were identified with a unique pattern of craniosynostosis involving the left coronal, sagittal, and right lambdoid sutures creating a "Z-pattern." Both patients were treated with staged cranial vault reconstructive procedures with an initial posterior remodeling (3 to 4 months) followed by an anterior repair (7 to 8 months). Both patients developed cerebellar tonsillar herniation, and one symptomatic patient required surgical decompression. With a follow up of greater than 2 years, normocephaly has been maintained with growth, and both children appear to have normal intellectual development. A unique pattern of trisutural "Z-patterned" craniosynostosis is presented. This distinctive pattern of craniosynostosis appears to be associated with cerebellar tonsillar herniation. In this small series, a two-staged procedure successfully normalized the calvarial dysmorphism, and early growth and development appear normal.

Identifying reproducible patterns of calvarial dysmorphology in nonsyndromic sagittal craniosynostosis may affect operative intervention and outcomes assessment.

BACKGROUND: The authors tested the premise that there are four distinctive patterns of calvarial dysmorphology in nonsyndromic sagittal craniosynostosis that can be reproducibly recognized. METHODS: Twenty-nine computed tomographic scan data sets of infants met the following criteria: nonsyndromic sagittal craniosynostosis, age younger than 12 months, and satisfactory computed tomographic data. Osseous reformations were constructed in the anteroposterior, right lateral, and vertex projections for each patient. From these images, four templates--coronal constriction, occipital protuberance, bifrontal bossing, and bitemporal protrusion--were selected as prototypes of the specific dysmorphologies the authors observed in patients with sagittal craniosynostosis. Four residents assigned the 29 calvarial image sets to one of the four templates or, if they were unable to do so, to the group "other." The sortings were then assessed for clustering. The same patient computed tomographic data were reformatted with osseous color images, which were then sorted according to template group by eight senior craniofacial surgeons, who repeated the task approximately 3 months later. The repeatability and assessment of clustering of image sets using the templates was evaluated. RESULTS: In the residents' pilot study, 41 percent (12 of 29) of patients had 100 percent concordance rates, 31 percent (nine of 29) had 75 percent concordance, 24 percent (seven of 29) had 50 percent, and 3 percent (one of 29) had 25 percent concordance. In summary, greater than 70 percent of the patient image sets could be sorted with at least 75 percent concordance by residents. In the senior surgeons' study, 90 percent of patients could be identified as falling into two of five possible groups. Senior raters demonstrated nearly 70 percent repeatability between sortings. CONCLUSION: These findings support the hypothesis that there are identifiable and reproducible patterns of varying calvarial dysmorphology in patients with sagittal craniosynostosis.

Endoscopic carpal tunnel release: a review of 753 cases in 486 patients.

BACKGROUND: Endoscopic carpal tunnel release is gaining increasing acceptance relative to the standard open carpal tunnel release for the treatment of carpal tunnel syndrome. Concerns about endoscopic carpal tunnel release include effectiveness of therapy and complication rates. This study attempted to evaluate outcomes of endoscopic carpal tunnel release in a large patient cohort. METHODS: Four hundred eighty-six patients (753 hands) with carpal tunnel syndrome who underwent endoscopic carpal tunnel release by a single surgeon were reviewed retrospectively. Data included demographics, subjective complaints, prior interventions, preoperative examination findings, and postoperative follow-up. All follow-up data were obtained from a single, independent, occupational therapy clinic. RESULTS: Median patient age was 48 years. Three hundred seventy-seven patients were gainfully employed at presentation, and 206 filed a worker's compensation claim. Median symptom duration was 2 years. Nonoperative therapy was ineffective in 151 patients. Preoperative nerve conduction studies were consistent with carpal tunnel syndrome in 472 patients (97 percent); all patients had either physical examination findings or nerve conduction studies consistent with carpal tunnel syndrome. Four hundred eighty-six patients (100 percent) obtained symptom relief. Complications included one transient median nerve neurapraxia, six complaints of residual pain, and one complaint of hypersensitivity. Worker's compensation patients and non-worker's compensation patients returned to work full-duty at similar times postoperatively. Ninety percent of employed patients returned to their original occupation. CONCLUSIONS: The authors' data indicate that an endoscopic approach for the treatment of carpal tunnel syndrome is safe and effective. Patients demonstrated a high return-to-work rate and an extremely low complication rate. The data challenge the belief that endoscopic carpal tunnel release results in higher complication rates.

Effect of distraction osteogenesis of the mandible on upper airway volume and resistance in children with micrognathia.

Children with craniofacial anomalies often have compromise of the upper airway, a condition with potential for morbidity and mortality. In children with microretrognathia, the diminutive size and retruded position of the mandible reduces the size of the oropharynx, thereby predisposing to glossoptosis and airway obstruction. Although several authors have reported successful use of mandibular distraction osteogenesis to alleviate this type of upper airway obstruction, the physiologic relationship between changes in mandibular shape, size, and position and upper airway dynamics remains undefined. The purpose of this study was to develop methodologies to quantitatively evaluate upper airway dynamics in children with micrognathia both before and after mandibular distraction osteogenesis. The patient population consisted of four children with micrognathia who had successfully undergone upper airway stabilization by bilateral mandibular distraction osteogenesis. The data used were digitally archived computed tomographic scan data from high-resolution, thin-slice head computed tomographic scans obtained before and after mandibular distraction. Upper airway evaluation was performed in two ways: static and dynamic. Static analysis consisted of computer quantification of predistraction and postdistraction mandibular and upper airway volumes using Analyze imaging software. Dynamic analysis consisted of fabrication of rigid stereolithographic hollow cast models of the upper airway produced from computed tomographic scan data. Models were used for characterization of upper airway resistance and flow patterns as related to respiration. After distraction osteogenesis, mandibular total volume increased 32, 32, 18, and 25 percent (mean, 27 percent) and upper airway volume increased by 20, 31, 23, and 71 percent (mean, 37 percent). A significant decrease in flow resistance, both inspiratory and expiratory, was observed in the patient with the greatest upper airway volume increase (71 percent) after distraction. After distraction, the inspiratory resistance was diminished by 51 percent and the expiratory resistance diminished by 85 percent. However, the three patients with more modest upper airway volume increases of 20 to 31 percent demonstrated no statistically significant change in flow resistance after distraction. Results of this study support the conclusion that distraction osteogenesis of the micrognathic mandible increases the volume of the upper airway, roughly paralleling the increase in mandibular volume. In the biomechanical airway model studied, upper airway volume expansion has been shown to be able to decrease the flow resistance over the length of the airway, presumably secondary to an increase in the average cross-sectional area. The artificial rigidity of the stereolithographic "airway" compared with the elasticity of the human upper airway may account for the insensitivity of this model to smaller but clinically significant airway changes.