Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down&... more Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down's syndrome, and 3 with lymphatic malformation of the tongue, were evaluated for postoperative improvement following partial glossectomy. Symptoms evaluated were speech intelligibility, oral competence at rest, drooling, and normal deglutition. All patients with Down's syndrome and Beckwith-Wiedemann syndrome were improved postoperatively. All patients with Down's syndrome exhibited improved deglutition and reduced drooling. Only 1 patient (33%), however, achieved oral competence. Speech was improved postoperatively in 2 patients (67%) with Down's syndrome. Patients with lymphatic malformation of the tongue did not exhibit consistent long-term improvement postoperatively, suggesting that conservative management of macroglossia in this patient group may be indicated.
Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyn... more Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyngeal flap (n = 75) or a dynamic sphincteroplasty (n = 10) performed between April 1958 and August 1989, and were evaluated preoperatively and postoperatively by a plastic surgeon, speech pathologist, and otolaryngologist. Improvement in speech was noted in 75% (n = 56) of the patients with pharyngeal flaps and 70% (n = 7) of the patients with dynamic sphincteroplasties postoperatively. Thirty percent of the patients in both groups showed no improvement postoperatively in speech. Three patients (4%) who underwent pharyngeal flap procedures developed sleep apnea postoperatively. Persistent velopharyngeal incompetence may be treated effectively with either a pharyngeal flap or a dynamic sphincteroplasty. Either procedure appears to result in improved speech in most patients.
A case is presented in which an indeterminate lung lesion was extracted through an accessory inci... more A case is presented in which an indeterminate lung lesion was extracted through an accessory incision during a video-assisted thoracic surgical lung biopsy. The lesion was malignant, and a completion lobectomy was performed. An incisional recurrence developed 5 months later, and this was treated with a wide chest wall resection and reconstruction. However, there was a second massive chest wall recurrence that proved fatal. We believe that tumor seeding to the chest wall occurred at thoracoscopy. To prevent such tumor seeding, thoracoscopic biopsy specimens should be removed in some sort of receptacle when cancer is suspected.
We report the case of a monomorphic adenoma of the parotid in a neonate. Although solid tumors of... more We report the case of a monomorphic adenoma of the parotid in a neonate. Although solid tumors of the parotid are rare in the pediatric population, these lesions may develop in utero and present at any time after birth. A solid mass in the parotid must be considered malignant until proved otherwise regardless of the age of the patient.
Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, incl... more Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, including osteoclasts. Both osteoclasts and osteoblasts have inducible nitric oxide synthase and produce nitric oxide. Although the direct effect of nitric oxide on osteoblasts in general and osteoblast proliferation in particular has not been delineated, the authors performed studies to clarify the role of nitric oxide on osteoblast proliferation and metabolism. Cultures of human osteoblasts were exposed to 0.1, 1.0, and 10 microM of the nitric oxide releasing agent 3-morpholino sydnonimine (SIN-1) for 7 days. Cells were evaluated for proliferation and production of alkaline phosphatase and osteocalcin. Osteoblasts exhibited decreased proliferation relative to control cultures at 1.0 and 10 microM concentrations of SIN-1 (p < 0.05). Concentrations of 1.0 and 10 microM of SIN-1 effected a decreased production of osteocalcin and alkaline phosphatase (p < 0.05). The results of these studies indicate that nitric oxide may play a critical role by which osteoblasts exhibit self-regulation of mineral metabolism.
Romberg's disease is an uncommon and poorly understood condition manifested by progressive hemifa... more Romberg's disease is an uncommon and poorly understood condition manifested by progressive hemifacial atrophy of skin, soft tissue, and bone. In order to better define the natural history and anatomic variation of this disorder, we evaluated 41 patients by history, physical examination, and facial radiographs. Light microscopic studies were performed on tissue from 19 patients, and ultrastructural analysis was performed on specimens from 6 patients. The average age at inception of the disease was 8.8 years. Atrophy, within one or more trigeminal nerve dermatomes, progressed at a variable rate (mean period of active tissue dissolution = 8.9 +/- 6 years). In 26 patients with skeletal involvement, the mean age of onset was 5.4 years, versus 15.4 years for 15 patients without skeletal involvement, a statistically significant difference (p less than 0.01). However, there was no correlation between the severity of soft-tissue deformity and the age of onset. Electron microscopy demonstrated lymphocytic infiltrates in neurovascular bundles and abnormalities of vascular endothelium and basement membranes. We hypothesize that the pathogenesis of Romberg's disease involves chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis).
Osteoblasts derived from sagittal sutures with premature synostosis, noninvolved coronal sutures,... more Osteoblasts derived from sagittal sutures with premature synostosis, noninvolved coronal sutures, and normal frontal bone were harvested and cultured as cells in an attempt to determine if osteoblasts at the site of premature fusion exhibited altered in vitro cellular dynamics. Basal metabolic parameters of cellular growth and the production of metabolites, including osteocalcin, alkaline phosphatase, platelet-derived growth factor (PDGF), transforming growth factor beta (TGF-beta), PDGF-beta receptors, epidermal growth factor (EGF) receptors, and fibroblast growth factor (FGF) were characterized. Osteoblasts harvested from sagittal sutures (sagittal osteoblasts) exhibited altered cellular growth and indices of cellular metabolism when compared with osteoblasts derived from patent coronal sutures (coronal osteoblasts) and frontal bone (frontal osteoblasts). Sagittal osteoblasts grew at a significantly increased rate and produced significantly more osteocalcin and less alkaline phosphatase than the coronal and frontal osteoblasts (p < 0.05). Significant variations in the production of EGF receptors were also noted between the sagittal osteoblasts and the coronal and frontal osteoblasts (p < 0.05). The osteoblasts from coronal sutures exhibited similarities in cellular growth and cellular metabolism, with the exception of PDGF receptors (p < 0.05), when compared with the osteoblasts obtained from the normal frontal bone. These results support a hypothesis in which a complex cell signaling mechanism regulates morphogenesis of the cranial vault at the sutural sites rather than a set of biomechanical tension forces that are exerted by the underlying brain.
Subperiosteal rejuvenation of the forehead may be performed through several small incisions in th... more Subperiosteal rejuvenation of the forehead may be performed through several small incisions in the brow. Small incisions are placed in the hairline and within the hair-bearing portion of the brow. The procedure provides a safe and reliable way to improve the position of the brow. The addition of resorbable screw fixation improves the stability of the brow position postoperatively and facilitates healing in the desired location. Subperiosteal brow lift may be performed successfully by way of minimally invasive incisions with favorable results.
Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, incl... more Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, including osteoclasts. Both osteoclasts and osteoblasts have inducible nitric oxide synthase and produce nitric oxide. Although the direct effect of nitric oxide on osteoblasts in general and osteoblast proliferation in particular has not been delineated, the authors performed studies to clarify the role of nitric oxide on osteoblast proliferation and metabolism. Cultures of human osteoblasts were exposed to 0.1, 1.0, and 10 microM of the nitric oxide releasing agent 3-morpholino sydnonimine (SIN-1) for 7 days. Cells were evaluated for proliferation and production of alkaline phosphatase and osteocalcin. Osteoblasts exhibited decreased proliferation relative to control cultures at 1.0 and 10 microM concentrations of SIN-1 (p < 0.05). Concentrations of 1.0 and 10 microM of SIN-1 effected a decreased production of osteocalcin and alkaline phosphatase (p < 0.05). The results of these studies indicate that nitric oxide may play a critical role by which osteoblasts exhibit self-regulation of mineral metabolism.
Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down&... more Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down's syndrome, and 3 with lymphatic malformation of the tongue, were evaluated for postoperative improvement following partial glossectomy. Symptoms evaluated were speech intelligibility, oral competence at rest, drooling, and normal deglutition. All patients with Down's syndrome and Beckwith-Wiedemann syndrome were improved postoperatively. All patients with Down's syndrome exhibited improved deglutition and reduced drooling. Only 1 patient (33%), however, achieved oral competence. Speech was improved postoperatively in 2 patients (67%) with Down's syndrome. Patients with lymphatic malformation of the tongue did not exhibit consistent long-term improvement postoperatively, suggesting that conservative management of macroglossia in this patient group may be indicated.
Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyn... more Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyngeal flap (n = 75) or a dynamic sphincteroplasty (n = 10) performed between April 1958 and August 1989, and were evaluated preoperatively and postoperatively by a plastic surgeon, speech pathologist, and otolaryngologist. Improvement in speech was noted in 75% (n = 56) of the patients with pharyngeal flaps and 70% (n = 7) of the patients with dynamic sphincteroplasties postoperatively. Thirty percent of the patients in both groups showed no improvement postoperatively in speech. Three patients (4%) who underwent pharyngeal flap procedures developed sleep apnea postoperatively. Persistent velopharyngeal incompetence may be treated effectively with either a pharyngeal flap or a dynamic sphincteroplasty. Either procedure appears to result in improved speech in most patients.
A case is presented in which an indeterminate lung lesion was extracted through an accessory inci... more A case is presented in which an indeterminate lung lesion was extracted through an accessory incision during a video-assisted thoracic surgical lung biopsy. The lesion was malignant, and a completion lobectomy was performed. An incisional recurrence developed 5 months later, and this was treated with a wide chest wall resection and reconstruction. However, there was a second massive chest wall recurrence that proved fatal. We believe that tumor seeding to the chest wall occurred at thoracoscopy. To prevent such tumor seeding, thoracoscopic biopsy specimens should be removed in some sort of receptacle when cancer is suspected.
We report the case of a monomorphic adenoma of the parotid in a neonate. Although solid tumors of... more We report the case of a monomorphic adenoma of the parotid in a neonate. Although solid tumors of the parotid are rare in the pediatric population, these lesions may develop in utero and present at any time after birth. A solid mass in the parotid must be considered malignant until proved otherwise regardless of the age of the patient.
Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, incl... more Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, including osteoclasts. Both osteoclasts and osteoblasts have inducible nitric oxide synthase and produce nitric oxide. Although the direct effect of nitric oxide on osteoblasts in general and osteoblast proliferation in particular has not been delineated, the authors performed studies to clarify the role of nitric oxide on osteoblast proliferation and metabolism. Cultures of human osteoblasts were exposed to 0.1, 1.0, and 10 microM of the nitric oxide releasing agent 3-morpholino sydnonimine (SIN-1) for 7 days. Cells were evaluated for proliferation and production of alkaline phosphatase and osteocalcin. Osteoblasts exhibited decreased proliferation relative to control cultures at 1.0 and 10 microM concentrations of SIN-1 (p < 0.05). Concentrations of 1.0 and 10 microM of SIN-1 effected a decreased production of osteocalcin and alkaline phosphatase (p < 0.05). The results of these studies indicate that nitric oxide may play a critical role by which osteoblasts exhibit self-regulation of mineral metabolism.
Romberg's disease is an uncommon and poorly understood condition manifested by progressive hemifa... more Romberg's disease is an uncommon and poorly understood condition manifested by progressive hemifacial atrophy of skin, soft tissue, and bone. In order to better define the natural history and anatomic variation of this disorder, we evaluated 41 patients by history, physical examination, and facial radiographs. Light microscopic studies were performed on tissue from 19 patients, and ultrastructural analysis was performed on specimens from 6 patients. The average age at inception of the disease was 8.8 years. Atrophy, within one or more trigeminal nerve dermatomes, progressed at a variable rate (mean period of active tissue dissolution = 8.9 +/- 6 years). In 26 patients with skeletal involvement, the mean age of onset was 5.4 years, versus 15.4 years for 15 patients without skeletal involvement, a statistically significant difference (p less than 0.01). However, there was no correlation between the severity of soft-tissue deformity and the age of onset. Electron microscopy demonstrated lymphocytic infiltrates in neurovascular bundles and abnormalities of vascular endothelium and basement membranes. We hypothesize that the pathogenesis of Romberg's disease involves chronic cell-mediated vascular injury and incomplete endothelial regeneration along branches of the trigeminal nerve (lymphocytic neurovasculitis).
Osteoblasts derived from sagittal sutures with premature synostosis, noninvolved coronal sutures,... more Osteoblasts derived from sagittal sutures with premature synostosis, noninvolved coronal sutures, and normal frontal bone were harvested and cultured as cells in an attempt to determine if osteoblasts at the site of premature fusion exhibited altered in vitro cellular dynamics. Basal metabolic parameters of cellular growth and the production of metabolites, including osteocalcin, alkaline phosphatase, platelet-derived growth factor (PDGF), transforming growth factor beta (TGF-beta), PDGF-beta receptors, epidermal growth factor (EGF) receptors, and fibroblast growth factor (FGF) were characterized. Osteoblasts harvested from sagittal sutures (sagittal osteoblasts) exhibited altered cellular growth and indices of cellular metabolism when compared with osteoblasts derived from patent coronal sutures (coronal osteoblasts) and frontal bone (frontal osteoblasts). Sagittal osteoblasts grew at a significantly increased rate and produced significantly more osteocalcin and less alkaline phosphatase than the coronal and frontal osteoblasts (p < 0.05). Significant variations in the production of EGF receptors were also noted between the sagittal osteoblasts and the coronal and frontal osteoblasts (p < 0.05). The osteoblasts from coronal sutures exhibited similarities in cellular growth and cellular metabolism, with the exception of PDGF receptors (p < 0.05), when compared with the osteoblasts obtained from the normal frontal bone. These results support a hypothesis in which a complex cell signaling mechanism regulates morphogenesis of the cranial vault at the sutural sites rather than a set of biomechanical tension forces that are exerted by the underlying brain.
Subperiosteal rejuvenation of the forehead may be performed through several small incisions in th... more Subperiosteal rejuvenation of the forehead may be performed through several small incisions in the brow. Small incisions are placed in the hairline and within the hair-bearing portion of the brow. The procedure provides a safe and reliable way to improve the position of the brow. The addition of resorbable screw fixation improves the stability of the brow position postoperatively and facilitates healing in the desired location. Subperiosteal brow lift may be performed successfully by way of minimally invasive incisions with favorable results.
Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, incl... more Nitric oxide has recently been shown to inhibit the proliferation of several types of cells, including osteoclasts. Both osteoclasts and osteoblasts have inducible nitric oxide synthase and produce nitric oxide. Although the direct effect of nitric oxide on osteoblasts in general and osteoblast proliferation in particular has not been delineated, the authors performed studies to clarify the role of nitric oxide on osteoblast proliferation and metabolism. Cultures of human osteoblasts were exposed to 0.1, 1.0, and 10 microM of the nitric oxide releasing agent 3-morpholino sydnonimine (SIN-1) for 7 days. Cells were evaluated for proliferation and production of alkaline phosphatase and osteocalcin. Osteoblasts exhibited decreased proliferation relative to control cultures at 1.0 and 10 microM concentrations of SIN-1 (p < 0.05). Concentrations of 1.0 and 10 microM of SIN-1 effected a decreased production of osteocalcin and alkaline phosphatase (p < 0.05). The results of these studies indicate that nitric oxide may play a critical role by which osteoblasts exhibit self-regulation of mineral metabolism.
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