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Relapse in Burkitt's lymphoma.

Of 109 patients with histologically confirmed Burkitt's lymphoma who completed a course of chemotherapy, 86 (79%) achieved complete remission. Forty-five (52%) of patients with initial complete remission relapsed with tumour over an observation period ranging from 2 years to over 5 years. Relapse was more common in patients who initially presented with abdominal or central nervous system (CNS) involvement than in patients who presented with localized facial tumours (p less than 0.01). Anatomical distribution of tumour on relapse differed from that at presentation. Facial bones were much less frequently involved on relapse; on the other hand, the CNS, cranial nerves, orbits and skin were frequent sites of disease on relapse. CNS involvement occurred in 42% (19/45) of patients at the first relapse and in 73% (11/15) of patients with multiple relapses. Prognosis in these patients was poor. Two relapse types were clinically identifiable. Early relapse (remission duration less than 12 weeks) was associated with frequent involvement of the CNS, drug resistance and a generally unfavourable outcome. Patients with late relapse (remission duration greater than 12 weeks) responded much better to secondary treatment. Possible pathogenic mechanisms underlying these two relapse types are discussed.

Abdominal Neoplasms↗

Management of craniofacial development in the Parry-Romberg syndrome: report of two patients.

OBJECTIVE: The aim of this article is to describe the orthodontic therapy for Parry-Romberg syndrome. The therapeutic goal is to minimize the wasting effects of progressive atrophy on facial development of a part of the face. DESIGN: To correct problems affecting craniofacial development of these patients, occurring during puberty, an orthodontic appliance was employed, which helps maintain parallelism of the facial planes, in particular the mandibular plane. SETTING: Orthodontic care was carried out in the Dental Clinic of the Catholic University of the Sacred Heart of Rome. INTERVENTION: Two patients underwent orthodontic therapy for 6 years. Appliances were checked every month and modified periodically so as to adapt to facial bone growth. RESULTS: At the end of craniofacial growth, the mandible was almost symmetric and the problem relating to atrophy remained confined to the initial area. Cephalometric analyses demonstrated that the occlusal plane and the mandibular plane maintained a straight orientation in relation to the bizygomatic plane. The ratio between the left and right side of the ramus and condyle, in the mandible, improved. CONCLUSIONS: The use of orthodontic therapy allows patients affected by hemifacial progressive atrophy to present a more harmonic face at the end of puberty when final reconstruction can be planned. These results provide for a limitation of surgical intervention to the sclerodermic area alone.

Adolescent↗

Primary osteosarcoma of bone. Clinicopathologic investigation of 243 cases, with necropsy studies in 54.

Two hundred forty-three patients who had pathologically verified primary osteosarcomas of bone, treated at The University of Texas System Cancer Center, M. D. Anderson Hospital and Tumor Institute over a 24-year period, form the basis of this study. These cases provide a basis for comparison of those patients currently receiving adjuvant chemotherapy with those who did not receive such treatment. Anatomic findings in 54 cases subjected to complete postmortem examinations were analyzed. Three significant findings emerged: pulmonary metastases were observed in all but one case, lymph-node metastases were found in only four cases, and in all metastases the histologic pattern reproduced that of the primary lesion. In addition, patients less than 26 years old had a significantly earlier appearance of pulmonary metastases than patients past this age. Patients with osteoblastic tumors had the poorest survival rate, followed by those with chondroblastic lesions. Those with fibroblastic tumors survived longest. In the overall study of 243 cases, tumors in the distal end of the femur and the proximal end of the tibia accounted for 147 cases, representing 60.5% of the cases. The highest incidence of the tumor was in the second decade of life, with 76.5% of the cases occurring in patients less than 25 years old. Three-year survival for the series was 21.7%, with only 12.6% surviving five years. Persons with primary lesions in the facial bones had the highest survival rate. Those with lesions in the humerus, tibia, and distal end of the femur had decreasingly lower survival rates. The lowest survival rates were for patients with lesions of the torso. In the femoral cases, size was a factor in survival; no patient with a tumor larger than 10 cm survived longer than five years. The surgery, irradiation, and chemotherapy employed--individually or in combination--did not alter appreciably the mortality rate for this tumor; only 12.6% of the patients survived five years or longer. Survivals were directly attributable to surgical procedures, including resection of pulmonary metastases. The data analyzed in this study provide a firm baseline of experience in analyzing results of treatment for osteosarcoma prior to the use of adjuvant chemotherapy, which is currently producing a vastly improved therapeuttic response.

Adolescent↗

Advantage of three-dimensional models in intraoral callus distraction.

Distraction osteogenesis has become an important method of reconstructing hypoplastic facial bones. Intraoral callus distraction is a great aesthetic improvement, because no visible scars are caused. Precise preoperative planning is necessary, however, because the direction of distraction cannot be influenced postoperatively. Nine three-dimensional (3-D) models were used in three intraoral distraction cases: two patients suffered from unilateral hemifacial microsomia and one from micrognathia after bilateral ankylosis caused by multifocal osteomyelitis in childhood. In each case, models of the head were produced and the distraction was simulated preoperatively. The amount of distraction was 18 mm in the two hemifacial microsomia patients and 18 and 14 mm in the ankylosis case. Extension of distraction in the ankylosis patient was shorter on one side in order to achieve a correct midline position. A slight overcorrection of 3-5 mm was allowed to cover relapse and unpredictable future growth deficiency. Three-dimensional models can supply important information when employed in intraoral callus distraction. They allow anticipation of pitfalls and help to achieve a foreseeable result. Further advantages may be expected with complete 3-D enoral distraction in the future.

Child↗

Sevoflurane mask anesthesia for urgent tracheostomy in an uncooperative trauma patient with a difficult airway.

PURPOSE: Proper care of the trauma patient often includes tracheal intubation to insure adequate ventilation and oxygenation, protect the airway from aspiration, and facilitate surgery. Airway management can be particularly complex when there are facial bone fractures, head injury and cervical spine instability. CLINICAL FEATURES: A 29-yr-old intoxicated woman suffered a motor vehicle accident. Injuries consisted of multiple abrasions to her head, forehead, and face, right temporal lobe hemorrhage, and complex mandibular fractures with displacement. Mouth opening was <10 mm. Blood pressure was 106/71 mm Hg, pulse 109, respirations 18, temperature 37.3 degrees C, SpO2 100%. Chest and pelvic radiographs were normal and the there was increased anterior angulation of C4-C5 on the cervical spine film. Drug screen was positive for cocaine and alcohol. The initial plan was to perform awake tracheostomy with local anesthesia. However, the patient was uncooperative despite sedation and infiltration of local anesthesia. Sevoflurane, 1%, inspired in oxygen 100%, was administered via face mask. The concentration of sevoflurane was gradually increased to 4%, and loss of consciousness occurred within one minute. The patient breathed spontaneously and required gentle chin lift and jaw thrust. A cuffed tracheostomy tube was surgically inserted without complication. Blood gas showed pH 7.40, PCO2 35 mm Hg, PO2 396 mm Hg, hematocrit 33.6%. Diagnostic peritoneal lavage was negative. Pulmonary aspiration did not occur. Oxygenation and ventilation were maintained throughout the procedure. CONCLUSION: Continuous mask ventilation with sevoflurane is an appropriate technique when confronted with an uncooperative trauma patient with a difficult airway.

Adult↗

Epidemiology of facial injury in female blunt assault trauma cases.

BACKGROUND: Etiology of trauma in the female population differs from that of the male population. To date, domestic violence has been researched extensively, but little has been published about the epidemiology of facial injury in the female population. OBJECTIVES: To analyze the differences in the circumstances under which males and females are injured, to identify gender-specific patterns of injury, and to assess whether differences in the demographics, health status, and drug-use profile exist between female and male assault cases and whether these factors lead to a difference in outcome. METHODS: A cohort of 91 female assault cases was compared with a control group of 706 males with similar injuries resulting from blunt assault trauma. Information was gathered by retrospective review of 797 consecutive cases requiring admission for facial injuries resulting from blunt assault trauma. Fisher exact, chi 2, and t tests were used to assess statistically significant differences between the male and female cohorts. RESULTS: The female cohort comprised 12% of all cases admitted for blunt assault facial trauma. One third of female blunt assault facial trauma patients were subjects of domestic violence. Statistically significant differences were found between males and females for the type of assault (rape, domestic violence, altercation, etc) with P < .0001. Females were more likely to be admitted with soft tissue injury only but no fracture (P < .05), less likely to be assaulted with a weapon, and unlikely to be involved in an altercation, gang violence, arrest, or robbery. Females were also less likely than males to be injured while intoxicated (P < .05). Incidence of specific injury patterns and outcomes, however, were similar between the 2 groups. CONCLUSION: The present data support the hypothesis that the female blunt assault facial trauma population represents a distinct epidemiological entity, with significant differences in the circumstances of injury, mechanism of assault, and role of intoxication in the incidence of injury. The prevalence of facial trauma in female assault cases makes it critical for the facial plastic surgeon to be vigilant in the evaluation and treatment of these patients.

Adult↗

Use of image-guided systems in the reconstruction of the periorbital region.

The conflict in the Middle East has led to an increase in the incidence and severity of facial trauma evaluated at tertiary military medical facilities. The Naval Medical Center San Diego, San Diego, Calif, has treated 5 patients with penetrating injuries to the orbit with associated comminuted fractures. We have used the technology of image-guided surgery to assist in reconstructing these injuries. Image-guided surgery has many applications in the head and neck. This review consists of a series of traumatic applications of the image-guided system with a focus on orbital and midfacial trauma. We will discuss the benefits and drawbacks of this technique compared with previously described techniques.

Adult↗

Orbital neurofibromatosis: involvement of the orbital floor.

Neurofibromatosis has been reported to involve the eye and orbit by causing irregularities of the sphenoid and other facial bones and enlargement of the foramina. To our knowledge, abnormalities of the orbital floor, however, have not been attributed to this disease in the literature until now. Two patients with neurofibromatosis had orbital floor involvement. Multiple neurofibromas eroding the orbital floor threatened vision in one patient. The other case, seen after trauma, had a condition simulating that of a blow-out fracture. Diagnostic workup with adequate roentgenography, especially tomography, helped to establish the diagnosis. We believe this is of importance in modifying the surgical approach, obviating the need for surgical intervention at times.

Female↗

Three-dimensional computed tomography in maxillofacial surgical planning.

Fifty-four maxillofacial three-dimensional computed tomographic examinations were performed during a 12-month period for the purpose of surgical planning. Pathologic entities in the series included trauma, neoplasia, cleft palate, and other developmental anomalies. Computer-assisted mandibular disarticulation was performed routinely after each study to permit direct viewing of the mandible or maxilla in isolation. Three-dimensional computed tomography is a useful technique for maxillofacial surgical planning.

Adolescent↗

Extended access/internal approaches for the management of facial trauma.

The two most significant recent developments in the treatment of facial trauma are the introduction of plating systems, which provide rigid internal fixation and the development of surgical approaches that allow wide exposure of the entire facial skeleton while minimizing external incisions. These approaches (referred to as extended access/internal approaches) are hemicoronal and coronal flaps, the sublabial approach to the midface, the transconjunctival approach to the orbital floor and orbital rim, and the intraoral management of mandibular fractures. These approaches work well, and have become standard techniques for managing facial trauma; however, each one has definite technical points that need to be adhered to to assure their success. Additionally, there are situations where these approaches are not appropriate and, in fact, may even be detrimental. This article outlines our approach to facial trauma using these extended access/internal approaches and discusses the important technical factors of each approach. Our experience in treating 113 patients with 119 fractures and 161 approaches over the last 2 years is presented and the role extended access/internal approaches have played is reviewed. The indications, limitations, and possible complications associated with each approach are outlined.

Facial Bones↗

Epidemiology of facial injury in blunt assault. Determinants of incidence and outcome in 802 patients.

BACKGROUND: Facial assault trauma has reached epidemic proportions. While the biomechanics of injury, methods of repair, and general demography of blunt facial trauma are well known, statistical associations between epidemiological factors, incidence, and outcome are poorly understood. OBJECTIVES: To provide a comprehensive epidemiological survey of this patient population. To assess factors determining outcome and pattern of injury. DESIGN: Retrospective review of 802 patients admitted with blunt assault facial trauma. Statistical analysis of associations between epidemiological factors, incidence, and outcome with chi 2 test, 2-tailed t test, and Fisher exact test. SETTING: Urban university and county hospital. RESULTS: Statistically significant associations are demonstrated between gender and the context of assault, incidence of armed assault, presence of fracture, and rate of intoxication at injury. Poor prognostic indicators for outcome include use of tobacco and presence of a mandible fracture. Open reduction and internal fixation were found to bear a higher complication rate only in mandible fractures. CONCLUSIONS: While choice of treatment carries a statistically significant association with outcome, social variables, including gender, tobacco use, intoxication, and low socioeconomic status, produce dramatic worsening of both injury incidence and outcome.

Adolescent↗

New form of platyspondylic lethal chondrodysplasia.

We report on a sporadic case of hitherto unknown lethal skeletal dysplasia. The cardinal clinical manifestations consisted of frontal bossing, cloudy corneae, low nasal ridge, and micrognathia, hypoplastic thorax, and rhizomelic micromelia. Laryngoscopy and neck CT disclosed laryngeal stenosis, and brain CT demonstrated hypoplasia of the corpus callosum. Skeletal survey demonstrated hypoplasia of facial bones and short skull base, extremely severe platyspondyly, hypoplastic ilia, and delayed epiphyseal ossification and rhizomelic shortness of tubular bones. The long bones appeared overtubulated with exaggerated metaphyseal flaring. The humeri were particularly short and bowed. Bowing of the radii and ulnae with subluxation of radial heads presented as a Madelung-like deformity. Unlike the long bones, the short tubular bones were not short and normally modeled. The skeletal changes were superficially similar to those in a group of lethal platyspondylic chondrodysplasias, but were inconsistent with any known subtypes of this group or other lethal skeletal dysplasias.

Abnormalities, Multiple↗

Teratogenic effects of vigabatrin in TO mouse fetuses.

Vigabatrin (VGB) is a relatively recently introduced antiepileptic drug that enhances the brain levels of gamma aminobutyric acid (GABA). Few data on its teratogenic effects appear to have been reported. Our objective was to determine if VGB was teratogenic in the TO mouse. Single doses of 300-600 mg/kg of VGB dissolved in saline were administered intraperitoneally (IP) to groups of TO mice on one of gestation days (GD) 7-12. The controls were saline treated or untreated. No maternal toxic effects were observed in the 300 or 450 mg/kg groups, and the 600 mg/kg dose was totally lethal to the mothers. Fetuses were collected on GD 18. Both 300 and 450 mg/kg doses induced a consistently significant intrauterine growth retardation irrespective of the developmental stage at administration. VGB did not augment the spontaneous incidence of neural tube defects characteristic of this strain, but accelerated destruction of the brain in spontaneous exencephalic embryos. Mandibular and maxillary hypoplasia, arched palate, cleft palate (two cases), limb defects (one case), and exomphalos were observed in the malformed fetuses. The high incidence of exomphalos appears to be a unique result of VGB treatment. Alizarin red-S/alcian blue-stained, skeletons revealed hypoplasia of mid facial bones, stage-dependent increase in the frequency of cervical and lumbar ribs, rib fusion, and sternal and vertebral malformations in the drug-treated fetuses. Middle and distal phalanges of the forepaw and mid phalanges and tarsals of the hindpaw failed to ossify in a significant number of experimental fetuses. Homeotic shift in terms of presacral vertebral number and a high incidence of lumbar and cervical ribs in the treated group are suggestive of treatment-related alterations in gene expression. In view of the paucity of human and animal data on the reproductive toxicologic effects of VGB, the results of the present study assume particular importance and suggest that VGB should be used in pregnancy with extreme caution.

Abnormalities, Drug-Induced↗

New strategy for the diagnosis of parotid gland lesions utilizing three-dimensional sialography.

Three-dimensional (3D) imaging for parotid gland lesions was performed using 3D sialography. This method provided a detailed surface structure of the parotid gland, and made it possible to overcome the disadvantages of X-ray sialography, planar CT, and MRI. Immediately after the conventional sialography via an oral route, CT scanning was performed with a slice-thickness of 3 mm using a helical CT scanner. CT data were analyzed on a workstation to reconstruct 3D images. 3D sialography was found to have the following advantages: (1) The structure of the acinar surface is visualized in detail; (2) The 3D structure of the entire parotid system from Stensen's duct to the gland is shown in one image; (3) The parotid gland can be assessed in the context of the bony architecture of facial bones; (4) The surface structure of the parotid gland can be understood very easily, like a scanning electron micrograph. We conclude that 3D sialography is a useful imaging technique for parotid gland lesions.

Adult↗