Estrogen and progesterone receptors in gynecomastia.

The etiology of gynecomastia is unknown. There seems to be no increased incidence of malignancies in patients with idiopathic gynecomastia; however, patients with Klinefelter syndrome exhibit an increased incidence of malignancy. The authors reviewed the results of 34 patients with gynecomastia diagnosed in adolescence who, following initial evaluation, had a mastectomy. The estrogen and progesterone receptors were analyzed in these patients. Three of the patients were diagnosed with Klinefelter syndrome. These three patients exhibited elevated amounts of estrogen and progesterone receptors. None of the patients who were not diagnosed with this syndrome demonstrated significant elevation of their estrogen or progesterone receptors. The presence of elevated estrogen and progesterone receptors in patients with Klinefelter syndrome provides a potential mechanism by which these patients may develop breast neoplasms. The absence of elevated estrogen and progesterone receptors in patients with idiopathic gynecomastia may serve to clarify why these patients' disease rarely degenerates into malignancy.

Distraction osteogenesis in the hand.

Distraction osteogenesis was used to correct congenital hand deformities in 12 digits. The patients' ages at the time of distraction ranged between 2.5 and 7 years. A total of nine patients underwent lengthening of 12 bones. The average period of distraction was 31.1 +/- 17.6 days. The average length of distraction was 23.6 +/- 7.3 mm. Seventeen percent of the patients after lengthening had an angular deformity that required remodeling of the regenerate. No infections were noted. Distraction osteogenesis provides a useful tool for reliable and predictable correction of osseous defects with vascular bone in patients with congenital anomalies of the hand.

Scaphocephaly: premature closure of the sagittal suture: a localized disorder of cellular metabolism?

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.

Role of nitric oxide in the regulation of osteoblast metabolism.

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.

Symptomatic hemangiomas and venous malformations in infants, children, and young adults: treatment with percutaneous injection of sodium tetradecyl sulfate.

OBJECTIVE: The purpose of this study was to evaluate the efficacy and safety of percutaneous sclerosis therapy using sodium tetradecyl sulfate for treatment of symptomatic hemangiomas and venous malformations in infants, children, and young adults. MATERIALS AND METHODS: Fifty-two sclerosis procedures were performed in 21 patients who were 13 months to 24 years old. Six of these patients had hemangiomas, and the remaining 15 patients had venous malformations. Sodium tetradecyl sulfate was injected solely percutaneously in 49 procedures, by both percutaneous and arterial routes in two procedures, and intraarterially only in one procedure. Thirteen patients had sclerosis therapy alone, and eight patients had sclerosis therapy followed by surgery within 48 hr. RESULTS: Sclerosis therapy alone or sclerosis therapy followed by surgery was judged beneficial in 18 (86%) of 21 patients, including five of the six patients who had hemangiomas and 13 of the 15 patients who had venous malformations. Two patients had an equivocal response to the therapy, and one patient had no apparent benefit. Three of the 21 patients had minor complications (skin ulcers) but no long-term sequelae. CONCLUSION: Percutaneous injection of sodium tetradecyl sulfate, either alone or before surgery, is a safe and effective method of managing symptomatic hemangiomas and venous malformations.

Osteoblast-directed osteoclast metabolism from patients with premature coronal synostosis.

Osteoclasts were harvested from explants of five patients with premature unilateral coronal synostosis and plated on devitalized bovine bone wafers. The planar surface areas of resorption lacunae were quantitated by means of light microscopy and a digital imaging system. Osteoclasts isolated from involved regions of premature synostosis exhibited enhanced resorption when compared with osteoclasts isolated from noninvolved cranial bone in the same patients (p < 0.05). When the involved osteoclasts were exposed to conditioned media from non-involved osteoblasts, the in vitro metabolism was reduced consistent with noninvolved osteoclasts. The results suggest there may be a primary cellular defect and that there appears to be a complex coupling between osteoblast and osteoclast function that may lead to sutural synostosis.

Role of resorbable plates and screws in craniofacial surgery.

Twenty patients underwent craniomaxillofacial fixation procedures with the use of the LactoSorb (Walter Lorenz Surgical, Jacksonville, FL) resorbable plating system. The patients ranged in age from 4 months to 67 years. No significant problems from the use of this plating system were noted. The resorbable plating system offers several distinct advantages over traditional metallic systems.

Craniofacial gliomas.

Ten patients with gliomas were treated between 1977 and 1993. Three of the lesions (30 percent) exhibited intracranial extension. Fifty percent (2 of 4) of the intranasal lesions exhibited intracranial extension. Effective removal of the lesion required manipulation of nasal bones in intranasal lesions and extranasal lesions with intranasal extension. Gliomas with an intracranial component were best addressed through a combined intracranial and extracranial approach.

Late correction of sagittal synostosis in children.

Twelve patients with sagittal synostosis underwent correction between 8 months and 8 years of age. The patients underwent expansion in the parietal region of 4 cm and had a reduction in length of 3 cm following dural plication. Average follow-up was 2.2 +/- 0.8 years (mean +/- SD). While optimal treatment for sagittal synostosis may be obtained in the first few months of life, late correction of sagittal synostosis may be obtained in a safe and effective manner.

Skeletal distraction of the hypoplastic mandible.

Six patients underwent percutaneous pin placement, 17 intraoral corticotomies, and application of an Orthofix lengthening device to facilitate complex multiplanar distraction as mandated by the patient's specific deformity. The patients underwent gradual bony and soft-tissue distraction at the rate of .25 mm four times a day. Average distraction was 18.3 +/- 5.5 mm (mean +/- SD). After distraction, a 2-day period of stabilization for each 1 mm of lengthening was used (36.4 +/- 10.7 days) (M +/- SD). All the patients had marked improvement in their occlusion postoperatively and significant amelioration of their preoperative respiratory and feeding difficulties. The patients all exhibited dramatic aesthetic improvement. Intraoral corticotomies performed in conjunction with gradual skeletal distraction appears to offer significant advantages over classical treatment for micrognathia in a very specific group of patients.

Thoracoscopic implantation of cancer with a fatal outcome.

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.

Cranial bone grafting in children.

Computed tomographic scans of 96 patients from newborn to 21 years of age were reviewed to assess the thickness of the skull and diploic space. Cranial thickness of the parietal bone used for calvarial bone harvest was assessed. Analysis of the data revealed that (1) cranial bone thickness may be predicted reliably as a function of age, (2) the growth velocity of increasing thickness of the parietal bone decreased with increasing age, and (3) presence of a diploic space may be predicted reliably as a function of age. On the basis of this analysis, we recommend performing split cranial bone grafting after the age of 3 years. In situ cranial bone grafting should not be planned prior to the age of 9 years.

Disseminated Rhizopus infection in a premature infant.

A 16-day-old premature male infant developed rapidly expanding necrotic facial plaques. Histology and cultures of tissue biopsy specimens revealed infection with Rhizopus arrhizius. The patient was treated aggressively with surgical debridement, amphotericin B, and rifampin, but had a progressive spread of the cutaneous infection and a fatal clinical course. He had gastrointestinal and pulmonary involvement on autopsy. Zygomycoses are rapidly progressive infections which generally affect immunocompromised hosts. Premature infants should be included as individuals at risk for disseminated zygomycotic disease.

Ocular abnormalities associated with unilateral coronal synostosis.

Forty-five patients with premature fusion of one coronal suture were treated between June 1985 and June 1991. Thirty-three patients were evaluated with pre- and postoperative photographs, computed tomographic scans, and serial radiographs and were assessed by the ophthalmological and plastic surgical services before and after surgery. The patients' mean age was 11.5 +/- 3.2 months at the time of surgery. Two patients (6%) were noted to have facial abnormalities preoperatively. Seventeen patients (52%) exhibited a disorder of extraocular muscle function preoperatively. After craniofacial repair, 21% of patients exhibited a distinct change from their preoperative ocular examination. A substantial majority of patients with premature fusion of the unilateral coronal suture (72%) will present with an abnormal examination postoperatively. In addition, a significant percentage of these patients will exhibit a change in ophthalmological status after craniofacial surgery; therefore, surgery on the extraocular musculature should, in general, be deferred until after bony repositioning.

The fate of developing teeth in facial trauma: tooth buds in the line of mandibular fractures in children.

Mandibular fractures in children present the surgeon with a unique set of considerations. Tooth buds within the body and ramus of the mandible restrict the type of surgical intervention that may be used. In addition, the fate of nonerupted dentition in the direct line of the fracture remains unclear. Between July 1985 and June 1990, 30 patients in deciduous or mixed dentition presented with a total of 45 mandibular fractures. Of these 45 fractures, 33% (n = 15) involved 16 nonerupted teeth. Age at the time of fracture was 6.0 +/- 2.3 years (mean +/- SD). Thirty-three percent of patients were treated with open reduction and internal fixation of fractures; 67% of patients were treated with closed reduction and either intermaxillary fixation or a lingual splint. Normal eruption was exhibited by 82% (n = 9) of the tooth buds in the line of mandibular fracture. The results of this study indicate the following: (1) Mandibular fractures in children involve unerupted teeth directly in one-third of cases. (2) The majority of tooth buds (82%) in the line of mandibular fractures eventually erupt normally. And (3) a significant percentage of dentition (18%) may exhibit either delayed eruption or noneruption with resorption of the tooth bud if directly involved in the line of fracture.

Vascularized muscle flaps for life-threatening mediastinal wounds in children.

Vascularized muscle flaps have been shown to be highly effective in adults for complicated mediastinal and thoracic wounds. They result in infection control, wound coverage, and preservation of life. Minimal experience exists with the use of these techniques in children. We review our experience with vascularized muscle flaps between 1985 and 1993 in treating life-threatening mediastinal wounds in 8 children. The 8 patients ranged in age from 1 day to 10 years; mean age was 2 years. Two patients had mediastinitis; one had an exposed Gore-Tex graft, and the other had an exposed homograft conduit. One patient had a midline defect with exposed right ventricle immediately beneath the skin. One patient had tracheal dehiscence after repair of an acquired tracheoesophageal fistula from battery erosion. One patient with Marfan's syndrome had a pectus deformity and an unstable sternum after emergent reoperation for a false aortic aneurysm. Two neonates had their sternum left open with a temporary Silastic skin patch after orthotopic cardiac transplantation because of swelling of the donor heart. One transplant patient had wound dehiscence. Vascularized muscle flaps used to heal the mediastinal wounds in this group included pectoralis major (10), rectus abdominis (3), and cervical strap (1). One infant with asplenia and an exposed Gore-Tex graft that had a delayed vascularized muscle flap died of Candida sepsis. Seven of 8 patients (85%) healed their mediastinal wound. Prompt recognition of thoracic wound complications facilitates immediate surgical intervention to obtain primary closure with vascularized muscle flaps, which promotes rapid healing in these children.

Plagiocephaly: premature unilateral closure of the coronal suture: a potentially localized disorder of cellular metabolism.

Osteoblasts from involved and noninvolved sutures and normal membranous bone in patients with premature unilateral coronal synostosis were harvested and grown in tissue culture. The cultures were characterized to establish specific basal metabolic parameters of cellular growth and the production of metabolites, including osteocalcin, alkaline phosphatase, platelet-derived growth factor, epidermal growth factor, tumor necrosis factor-alpha, and DNA. In addition, metabolic responses to two provocative bone trophic agents, parathyroid hormone and 1,25dihydroxyvitamin-D3 were ascertained. Osteoblasts from involved sutures (involved bone) exhibited altered indices of cellular metabolism when compared with osteoblasts derived from noninvolved sutures (noninvolved bone) in a basal state and when exposed to parathyroid hormone and vitamin D3 (p < or = 0.05). Involved osteoblasts produced significantly more osteocalcin and significantly less alkaline phosphatase than noninvolved osteoblast-derived cultures (p < or = 0.05). Secretion of platelet-derived growth factor and epidermal growth factor was also altered in the involved osteoblasts compared with the noninvolved osteoblasts (p < or = 0.05). Secretion of tumor necrosis factor-alpha was not significantly different between involved and noninvolved osteoblast-derived cultures.